Pulmonary valve stenosis: Difference between revisions

	Pulmonary valve stenosis;
	Pulmonary valve stenosis
ICD-10	I37.0, I37.2, Q22.1
ICD-9	424.3, 746.02
OMIM	265500
DiseasesDB	11025
MedlinePlus	001096

VisualWikitext

Latest revision as of 21:22, 15 December 2022

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aravind Kuchkuntla, M.B.B.S [2]

Synonyms and keywords: Valvular Pulmonary Stenosis, Pulmonic Stenosis, Right Ventricular Outlet Obstruction, supravalvular pulmonic stenosis, infundibular pulmonic stenosis, Narrowing of pulmonary valve, PS

Overview

Pulmonary valve stenosis accounts for 8% of all congenital heart disease and worldwide the prevalence of pulmonary valve stenosis is 1 per 2000 births.^[1] The pulmonic valve stenosis is classified into 3 different subtypes based on the location of the stenosis. Isolated valvular stenosis is the most common sub-type, with dome shaped morphology and dysplastic valves. Patients with mild stenosis usually have a beningn course and do not progress, patients with moderate to severe stenosis manifest symptoms of dyspnea, chest pain, fatigue and syncope. If left untreated patients progress to right heart failure. 2D Echo is the standard diagnostic test to identify the location and to assess the severity of the stenosis. Symptomatic patients undergo valvulotomy or balloon valvuloplasty based on the morphology of the affected valves. Timely intervention in patients with valvular stenosis has good outcomes and excellent prognosis. Guidelines for evaluation, approach and treatment are well-defined.

Historical Perspective

The pulmonary valve and its function of allowing blood to the lungs for nourishment was first described by Hippocrates. ^[2]
Erasistratus, mentioned the function of the pulmonary valve in the unidirectional flow. ^[3]
Galen described the membranes of the valves and named them as "semilunar". ^[4]
Mondino de Luzzi designed the sketch of the pulmonary valves in the anatomical position for the first time. ^[5]
Realdo Colombo described the pulmonary circulation for the first time. ^[6]
Aortic stenosis was probably first described by Lazare Riviere (1589-1655), a French physician in 1663. ^[7]
The first transcatheter aortic valve replacement procedure in the world was performed on 16 April 2002 in a 57-year-old inoperable patient with severe aortic stenosis. The procedure was done by the Interventional Cardiologist Professor Alain Cribier at the Charles Nicolle University Hospital in Rouen, France.

Classification

Based on the anatomic location

Pulmonic stenosis is classified into valvular, subvalvular (infundibular) and supravalvular based on the location of the stenosis in relation to the pulmonary valve. Valvular stenosis is most common of the three sub-types.^[8] ^[9]

Sub-valvular stenosis: It can be infudibular or sub-infundibular. Infundibular stenosis is a feature of tetralogy of Fallot. Sub-infundibular pulmonic stenosis is known as ‘double chambered right ventricle’ dividing the right ventricle into a high pressure inlet and a low pressure outlet causing a progressive right ventricular outflow tract obstruction.^[10]
Valvular stenosis: It is the most common cause of pulmonic stenosis. The valves are usually dome shaped or dysplastic affecting the movement of the cusps. It can be isolated or associated with other congenital heart diseases such as atrial septal defect, Ebstein’s anomaly, double outlet right ventricle, and transposition of the great arteries.^[9]
Supravalvular stenosis: The obstruction is usually in the common pulmonary trunk or in the bifurcation or the pulmonary branches. It is commonly associated with other congenital syndromes such as Williams–Beuren, Noonan, Allagile syndrome, DiGeorge, and Leopard syndrome. ^[9]

Based on the severity of the stenosis

Severity of pulmonary stenosis is classified based on the estimated peak velocity and peak resting gradient calculated using modified Bernoulli equation. It is classified into:^[11]

Mild: Peak velocity less than 3m/s and peak gradient is less than 36 mm Hg.^[11]
Moderate: Peak velocity is 3 to 4m/s and peak gradient is 36 to 64mm Hg.
Severe: Peak velocity is greater than 4m/s and peak gradient is greater than 64mm Hg. ^[9]

According to 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease, stages of severe pulmonic stenosis are defined as follows: This form of aortic stenosis presents later in life, usually after the age of 75. ^[12]

Stage	Definition	Valve Anatomy	Valve Hemodynamics	Hemodynamic Consequences	Symptoms
C,D	Severe pulmonic stenosis	Thickened, distorted, possibly calcified leaflets with systolic doming and/or reduced excursion Other anatomic abnormalities may be present, such as narrowed RVOT.	Vmax >4 m/s; peak instantaneous gradient >64 mm Hg	Right ventricular hypertrophy Possible RV, RA enlargement Poststenotic enlargement of main pulmonary artery	None or variable and dependent on severity of obstruction

According to 2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease, progression of valvular heart disease (VHD) are defined as follows:^[13]

Stage	Definition	Description
A	At risk	Patients with risk factors for development of valvular heart disease
B	Progressive	Patients with progressive valvular heart disease (mild-to-moderate severity and asymptomatic)
C	Asymptomatic severe	Asymptomatic patients who have the criteria for severe valvular heart disease: C1: Asymptomatic patients with severe VHD in whom the left or right ventricle remains compensated C2: Asymptomatic patients with severe VHD with decompensation of the left or right ventricle
D	Symptomatic severe	Patients who have developed symptoms as a result of valvular heart disease

Pathophysiology

Pathogenesis

Pulmonic valve stenosis with fused commisures affect the flexibility of the valve causing obstruction of the outflow tract. In patients with dysplastic valves, the cusps are not fused but they are rigid from intrinsic thickening resulting in the narrowing of the outflow tract. The valve problems develop 5 - 10 years after the rheumatic fever, a tiny nodule forms along the valve leaflets. The degree of leaflet thickening and calcification and the severity of chordal involvement are variable. Rheumatic fever is becoming rare in the United States, so mitral stenosis is also less common.^[14]
These morphological changes affect the complete opening of the pulmonic valve during the ventricular systole causing elevated right ventricular systolic pressures and leading to right ventricular remodelling.^[15]
The obstruction leads to increased pressure overload on the right ventricle as it has to push the blood against resistance. ^[9]

Genetics

These are a common genetic disorders associated with pulmonic stenosis:^[16]

Syndrome	Genetic Defect	Cardiac features	Other features
Noonan syndrome^[17]	PTPN11, SOS1 Heterogeneous trait Aberrant RAS-MAPK-signaling	Dysplastic pulmonary valve stenosis Supravalvular pulmonary stenosis Hypertrophic cardiomyopathy	Short stature Hypertelorism Downward eye slant Low set ears
Williams Beuren^[18]	7Q11.23 deletions Autosomal dominant trait	Supravalvular aortic or pulmonary stenosis	Elfin face Short stature Impaired cognition and development Endocrine disorders and genitourinary abnormalities
Leopard syndrome^[19]	PTPN11, RAF-1 Autosomal dominant trait	Electrocardiographic abnormalities Supravalvular or valvular pulmonary stenosis	Lentigines^[20] Ocular hypertelorism Abnormal genitalia Growth retardation Deafness
DiGeorge syndrome^[21]	22Q11 deletion Autosomal dominant trait	Conotruncal defects such as tetralogy of Fallot Interrupted aortic arch Truncus arteriosus Vascular rings ASD/VSD	Hypertelorism Low set and posteriorly rotated ears Palatal abnormalities Micrognathia Developmental delay Hypoplastic thymus Hypocalcaemia Immunological abnormalities
Allagile^[22]	AG-1, NOTCH-2 Autosomal dominant trait	Peripheral pulmonary stenosis^[23]	Facial dysmorphias (triangular face, wide nasal bridge, deep set eyes) Intrahepatic cholestasis Butterfly [vertebra]]
Keutel^[24]	MGP mutations Autosomal recessive trait	Multiple peripheral pulmonary stenosis	Abnormal cartilage calcifications Brachytelephalangy Subnormal IQ and hearing loss
Congenital rubella syndrome^[25]	N/A	Peripheral pulmonary stenosis Open ductus Botalli	Congenital cataract/glaucoma Deafness Pigmentary retinopathy

Associated conditions

A rare association of pulmonic stenosis with an unrepaired ASD is reported.^[26] ^[27] ^[28]

Epidemiology and Demographics

Pulmonary stenosis accounts for 8% of all congenital heart disease. ^[29]

Worldwide, the prevalence of pulmonic stenosis is 1 per 2000 births.^[30]
The prevalence of pulmonic stenosis and tetralogy of Fallot is higher in asian countries.^[31] ^[32]

Causes

Pulmonary valve stenosis is due to a structural changes resulting from thickening and fusion of the pulmonary valve. The valve pathology can be congenital or acquired. The following is the list of causes: ^[33]^[34].

Congenital causes

These account for 95% of the cases with pulmonic stenosis which include isolated pulmonic valve pathologies and its associations with other congenital heart diseases.^[35]

Associated with congenital heart disease

Tetralogy of Fallot^[36] ^[37] ^[38]
Double outlet right ventricle ^[39]
Univentricular atrio-ventricular connection ^[40]
Atrioventricular canal defect ^[41]
Bicuspid pulmonary valve^[42]
Quadricuspid pulmonary valve: Benign and an incidental finding^[43]
Isolated pulmonic stenosis^[44]
- Acommissural pulmonary valves: Valve has a prominent systolic doming of the cusps and an eccentric orifice.^[45]^[46]
- Dysplastic pulmonary valves: Thickened and deformed cusps with no commissural fusion. It is a common finding associated with Noonan syndrome.^[47]
- Unicommissural pulmonary valve
- Bicuspid valve with fused commissures

Acquired Causes

These are less frequent and account for less than 5% of the cases. ^[48]

Carcinoid Syndrome: most common acquired cause^[49]
Post infectious: Infective endocarditis
Calcification of the pulmonary valve^[50]
Rheumatic heart disease^[51]
Ross procedure^[52]
Functional pulmonic stenosis: Primary cardiac tumors obstructing the right ventricular outflow tract such as leiomyosarcoma.^[53]

Differentiating from other diseases

Right ventricular outflow tract obstruction must be distinguished from an ASD, a small VSD, aortic stenosis, and acyanotic or pink tetralogy of Fallot.^[54]

Atrial septal defect: Presence of systolic ejection murmur, wide fixed split S2, EKG showing RVH. In ASD the split of the S2 is fixed, there is no ejection click. ^[55]
Small Ventricular septal defect: Amyl nitrate increases venous return and increases the murmur of pulmonary stenosis, in VSD the murmur becomes softer.
Mild left-sided outflow obstruction: With valsalva maneuver the murmur of aortic stenosis becomes softer after about 5 beats, with pulmonary stenosis it becomes softer within 3 beats.
Acyanotic or pink tetralogy of Fallot: with amyl nitrate and increased venous return the murmur of PS increases, and the murmur of tetralogy of Fallot decreases because of peripheral vasodilation and an increase in right to left shunting. ^[33]

Risk Factors

Common risk factors in the development of congenital heart disease apply for pulmonic stenosis and include:^[56]

Maternal gestational diabetes mellitus
Consanguineous marriage^[57]
Phenylketonuria
Febrile illness
Vitamin A use ^[58]
Marijuana use ^[59]
Exposure to organic solvents ^[60]

Natural History, Complications and Prognosis

Natural History

Patients with congenital pulmonary stenosis manifest clinical features few hours after birth, in childhood or in adulthood. Manifestation of symptoms, symptom severity and the outcomes are dependent on the severity of stenosis. Patients with mild pulmonic stenosis have a benign course and do not progress and patients with moderate and severe stenosis have dyspnea with exertion and syncope.^[61]

Prognosis

Patients with moderate to severe pulmonic valve stenosis are managed well with surgery or balloon valvuloplasty and have very good prognosis. ^[62]

Complications

If left untreated, patients with moderate to severe stenosis progress to develop tricuspid regurgitation and right ventricular dysfunction leading to right ventricular failure and arrhythmias.^[63]

Diagnosis

Gold standard diagnosis of pulmonic stenosis and assessment of severity is done by 2D echocardiography.^[64]

History and Symptoms

The severity of symptoms and age of symptom onset depends on the severity of the stenosis. Clinical presentations vary as follows:

Critical pulmonary stenosis:It presents in first few hours to days of life with cyanosis. It is a condition with a very small or pin-hole orifice in the pulmonary valve which can be diagnosed prenatally. These patients have an intact interventricular septum, poorly complaint hypoplastic right ventricle and areductus dependent. Cyanosis in these patients is due to the right to left shunting at the level of the foramen ovale.^[65]
Mild Pulmonic Stenosis: Patients with mild stenosis are asymptomatic and are diagnosed by routine examination with an ejection systolic murmur.
Moderate Pulmonic Stenosis: Patients present with exertional dyspnea and fatigue. ^[66]
Severe Pulmonic Stenosis: Patients present with exertional dyspnea, chest pain and syncope. ^[67]
Untreated patients develop features of right ventricular failure which include:
- Exercise intolerance
- Fatigue
- Shortness of breath
- Swelling of the feet or ankles
- Abdominal discomfort
Patients with subinfundibular/infundibular PS can be asymptomatic or they may present with angina, dyspnea, dizziness, or syncope.
Patients with supravalvular PS may be asymptomatic or have symptoms of dyspnea and fatigue on exertion.

	Mild valvular PS	Moderate Valvular PS	Severe Valvular PS	Infundibular Pulmonic Stenosis	Supravalvular Pulmonic Stenosis	Right Heart Failure
Clinical Features	Asymptomatic	Exertional dyspnea Fatigue	Exertional dyspnea Chest pain Syncope	Angina Dyspnea Dizziness Syncope	Dyspnea Fatigue on exertion	Exercise intolerance Fatigue Shortness of breath Swelling of the feet or ankles Abdominal discomfort

Physical Examination

The common examination findings include:

Patients with isolated pulmonary stenosis usually appear normal. In patients diagnosed with syndromes associated with pulmonic stenosis syndrome specific physical examination findings are demonstrated.^[68]
- Fabry's disease^[69]
Cardiac examination findings are dependent on the degree of the pulmonary stenosis, the pathology of the valve and associated cardiac lesions. The common findings include as follows: ^[70]
- In mild stenosis findings include normal jugular venous pulse, absent right ventricle lift, ejection click in the pulmonary area which decreases with inspiration, ejection systolic murmur in the pulmonary area heard in the ending of mid systole increasing in intensity during inspiration.^[71]
- In severe stenosis findings include:
  - Elevated JVP with a prominent "a" wave
  - Right ventricular heave.
  - Louder and longer ejection murmur in the left parasternal area in second and third intercostal space.
  - Ejection click is softer and absent with increasing severity.
  - Wide split S2 with reduced or absent P2 component.
  - Right sided S4 can be audible. ^[72]

	Mild valvular PS	Severe Valvular PS
Physical Examination Findings	Normal jugular venous pulse Absent right ventricle lift Ejection click in the pulmonary area which decreases with inspiration Ejection systolic murmur in the pulmonary area heard in the ending of mid systole increasing in intensity during inspiration	Elevated JVP with a prominent "a" wave Right ventricular heave Louder and longer ejection murmur in the left parasternal area in second and third intercostal space Ejection click is softer and absent with increasing severity Wide split S2 with reduced or absent P2 component Right sided S4 can be audible.

EKG

Patients with mild stenosis usually do not show any EKG changes excepting for right axis deviation of -100° to -110° which is considered normal in children and adults.
In case of severe stenosis the following changes can be noted, which include:^[73]

Features of right ventricular hypertrophy
Rightward axis deviation ^[74]
High R wave amplitude in lead V1.
Deep S waves in the left precordial leads with <1 R:S ratio in lead V6

Chest X-Ray

Vascular fullness in the left lung base greater than the right lung base.
Dilation of the main pulmonary artery is a more common finding in doming pulmonary valve stenosis when compared to dysplastic valves.
Calcification can be rarely seen.

Echocardiography

Transthoracic 2D Echo and Doppler imaging is the standard diagnostic test to detect and assess the severity of the stenosis.^[75]

Echo shows thickened and dome shaped valves, peak and mean gradients to assess the severity can be measured by Doppler imaging. ^[76]
Dysplastic valves are well visualized on echo.^[77]
Always calculate the tricuspid regurgitation gradient to rule out overestimation of the pulmonary stenosis gradient.^[78]
Right ventricular function and ejection fraction is better measured by a 3D echo when compared to a 2D echo.^[79]

Pulmonic Stenosis 1

Pulmonic Stenosis 2

MRI

Cardiac MRI is very useful to study the anatomy of the right ventricular outflow tract, pulmonary artery and to locate the exact level of stenosis.^[80]

Cardiac Catheterization

Cardiac catheterization is useful to measure the pressure gradients directly, but its not performed on a regular basis as echo is a reliable and non-invasive test to measure the pressure gradients.^[81]

Dual-Source Computed Tomography

It is an accurate imaging technique to evaluate the function and anatomy of the pulmonary valve.^[82]

Evaluation of Pulmonary Stenosis in Adolescents and Young Adults

According to 2008 ACC/AHA guidelines, following are the guidelines for evaluation of patients with pulmonary stenosis:

Class I
"1. An ECG is recommended for the initial evaluation of pulmonic stenosis in adolescent and young adult patients and serially every 5 to 10 years for follow-up examinations.(Level of Evidence: C) "
"2.Transthoracic Doppler echocardiography is recommended for the initial evaluation of pulmonic stenosis in adolescent and young adult patients, and serially every 5 to 10 years for follow-up examinations.(Level of Evidence: C)"
"3.Cardiac catheterization is recommended in the adoles- cent or young adult with pulmonic stenosis for evalu- ation of the valvular gradient if the Doppler peak jet velocity is greater than 3 m per second (estimated peak gradient greater than 36 mm Hg) and balloon dilation can be performed if indicated.(Level of Evidence: C) "

Class III
"1.Diagnostic cardiac catheterization is not recommended for the initial diagnostic evaluation of pulmonic stenosis in adolescent and young adult patients.(Level of Evidence: C).(Level of Evidence: C) "

Treatment

Medical Therapy

There is no specific medical therapy for the treatment of pulmonic stenosis. However, patients diagnosed with right heart failure diuretics are recommended to decrease the fluid overload. ^[83]

Surgery

Indications For Intervention

Surgical correction is recommended based on the peak gradient and other associated clinical features:^[84] ^[85]

Surgery is advised regardless of the symptoms if the doppler derived peak instantaneous gradient greater than 64 mm Hg (peak velocity >4 m/s). ^[86]
In patients with doppler derived peak instantaneous gradient less than 64 mm Hg (peak velocity>4 m/s), surgery is advised if any of the following is present:^[83]
- Symptomatic patient
- Decreased right ventricular function
- Double chambered right ventricle
- Arrhythmia
- Right to left shunting via the VSD or ASD
Asymptomatic patients with a systolic RV pressure greater than 80 mm Hg (TR jet velocity >4.3 m/s).

2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines^[87]

Recommendations for Valvular Pulmonary Stenosis

Class I
1. In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of HF, cyanosis from interatrial right-to-left communication, and/or exercise intolerance, balloon valvuloplasty is recommended..(Level of Evidence: B-NR)
2.In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of HF, cyanosis, and/ or exercise intolerance who are ineligible for or who failed balloon valvuloplasty, surgical repair is recommended(Level of Evidence: B-NR)

Class IIa
1.In asymptomatic adults with severe valvular pulmonary stenosis, intervention is reasonable. (Level of Evidence: C-EO)"

According to 2010, ESC Guidelines for the management of grown-up congenital heart disease, Indications for intervention in Right Ventricular Outlet Obstruction are as follows:^[88]

Class I
"1.RVOTO at any level should be repaired regardless of the symptoms when the doppler peak gradient is >64mm Hg(peak velocity >4.0m/s), provided that the RV function is normal and no valve substitute is required(Level of Evidence: C)"
"2.In valvular PS, balloon valvulotomy should be the intervention of choice.(Level of Evidence: C) "
"3.In asymptomatic patients in whom balloon valvulotomy is ineffective and the surgical valve replacement is the only option, surgery should be performed in the presence of a systolic right ventricular pressure greater than 80mm Hg ( TR velocity >4.3m/s)(Level of Evidence: C) "

Class IIa
"1.Intervention in patients with gradient <64 mm Hg should be considered in the presence of: Symptoms related to PS or Decreased RV function or Double chambered RV ( which is usually progressive) or Important arrythmias (Level of Evidence: C)"
"2.Peripheral PS, regardless of the symptoms, should be considered for repair if : >50% diameter narrowing and RV systolic pressure >50 mm of Hg and/or Lung perfusion abnormalities are present.(Level of Evidence: C)"

Intervention Options

Percutaneous balloon pulmonary valvulotomy (BPV) has widely replaced surgical valvulotomy as a treatment option for pulmonary valve stenosis.^[89]^[90]
Catheter intervention is recommended for patients with doming valves which are not dysplastic.^[91]
Surgery is recommended for patients with:
- Subinfundibular or infundibular pulmonic stenosis and hypoplastic pulmonary annulus
- Dysplastic pulmonary valves
- Patients with associated lesions which need a surgical approach, such as severe PR or severe TR.
In patients with significant residual PS after BPV, a redo BPV can be performed with a larger balloon to avoid valve replacement.
In patients with hemodynamically significant pulmonary regurgitation after valvulotomy or BPV, surgical valve replacement is recommended.

Intervention Outcome

Surgical outcomes in patients with valvular stenosis is good with survival rate of 90 to 96% 25 years after the surgery when its done in the childhood.^[92]^[93]
Survival is around 70% at 25 years when the surgery is performed in adulthood.
BPV has shown to have good outcomes in long term follow up with very low rate of re-intervention requirement.^[94]^[95]^[96]
BPV has shown to have sub-optimal results in patients with dysplastic valves when compared to doming valves.^[97]

Complications

Post procedural pulmonary regurgitation is a common complication and occurs in 10 to 40% patients. Majority of the patients remain asymptomatic and only few patients develop hemodynamically significant pulmonary regurgitation.^[98]
Bradycardia and hypotension at the time of balloon inflation
Transient permanent right bundle branch block or atrioventricular block
Higher mortality rates reported after surgical decompression in patients with RV-dependent coronary circulation.^[99]
Balloon rupture
Tricuspid papillary muscle rupture
Perforation of the RV outflow tract

Follow up

Patients with pulmonic stenosis are recommended for a regular echocardiography to evaluate the degree of pulmonary regurgitation, RV pressure, RV function and tricuspid regurgitation. The frequency of visits is dependent on the degree of stenosis and is as follows:^[100]

Mild untreated or residual pulmonic stenosis: Follow up once every 5 years.
Moderate pulmonic stenosis: Annual visit with echocardiography every 2 years.

ACC / AHA Guidelines - Indications for balloon valvotomy in Pulmonary Stenosis (DO NOT EDIT)

According to 2008 ACC/AHA guidelines^[100], following are the indications for balloon valvotomy in pulmonary stenosis:

Class I
"1.Balloon valvotomy is recommended in adolescent and young adult patients with pulmonic stenosis who have exertional dyspnea, angina, syncope, or presyncope and an RV–to–pulmonary artery peak-to-peak gradient greater than 30 mm Hg at catheterization.(Level of Evidence: C) "
"2.Balloon valvotomy is recommended in asymptomatic adolescent and young adult patients with pulmonic stenosis and RV–to–pulmonary artery peak-to-peak gradient greater than 40 mm Hg at catheterization.(Level of Evidence: C) "

Class III
"1.Balloon valvotomy is not recommended in asymptomatic adolescent and young adult patients with pulmonic stenosis and RV–to–pulmonary artery peak-to-peak gradient less than 30 mm Hg at catheterization.(Level of Evidence: C) "

Class IIb
"1.Balloon valvotomy may be reasonable in asymptomatic adolescent and young adult patients with pulmonic stenosis and an RV–to–pulmonary artery peak-to-peak gradient 30 to 39 mm Hg at catheterization.(Level of Evidence: C) "

Recommendations For Pulmonary Valvuloplasty

According to 2011, Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease, A Scientific Statement From the American Heart Association.^[101]

Class I
"1. Pulmonary valvuloplasty is indicated for a patient with critical valvar pulmonary stenosis (defined as pulmonary stenosis present at birth with cyanosis and evidence of patent ductus arteriosus dependency), valvar pulmonic stenosis, and a peak-to-peak catheter gradient or echocardiographic peak instantaneous gradient of >40 mm Hg or clinically significant pulmonary valvar obstruction in the presence of RV dysfunction.(Level of Evidence: A) "

Class IIa
"1. It is reasonable to perform pulmonary valvuloplasty on a patient with valvar pulmonic stenosis who meets the above criteria in the setting of a dysplastic pulmonary valve.(Level of Evidence: C) "
"2. It is reasonable to perform pulmonary valvuloplasty in newborns with pulmonary valve atresia and intact ventricular septum who have favorable anatomy that includes the exclusion of RV-dependent coronary circulation.(Level of Evidence: C) "

Class IIb
"1. Pulmonary valvuloplasty may be considered as a palliative procedure in a patient with complex cyanotic CHD, including some rare cases of tetralogy of Fallot.(Level of Evidence: C) "

Class III
"1. Pulmonary valvuloplasty should not be performed in patients with pulmonary atresia and RV-dependent coronary circulation.(Level of Evidence: B) "

Prevention

There are no specific primary preventive measures.
Patients with diagnosed pulmonary valvar stenosis are not candidates for infective endocarditis prophylaxis.
Infective endocarditis prophylaxis is recommended only in patients with prosthetic valves.

Participation In Sports

According to 2005 Task Force 2: Congenital Heart Disease, guidelines for participation in sports are as follows:^[102]

Pulmonary valve stenosis in untreated patients

"1.Athletes with a peak systolic gradient less than 40 mm Hg and normal right ventricular function can participate in all competitive sports if no symptoms are present. Annual re-evaluation is recommended. "
"2.Athletes with a peak systolic gradient greater than 40 mm Hg can participate in low-intensity competitive sports (classes IA and IB). Patients in this category usually are referred for balloon valvuloplasty or operative valvotomy before sports participation. "

Pulmonary valve stenosis treated by operation or balloon valvuloplasty

"1.Athletes with no or only residual mild PS and normal ventricular function without symptoms can participate in all competitive sports. Participation in sports can begin two to four weeks after balloon valvuloplasty. After operation, an interval of approximately three months is suggested before resuming sports participation. "
"2.Athletes with a persistent peak systolic gradient greater than 40 mm Hg should follow the same recommendations as those for patients before treatment."
"3.Athletes with severe pulmonary incompetence characterized by a marked right ventricular enlargement can participate in class IA and IB competitive sports."

SupraValvular Pulmonic Stenosis

According to 2008 ACC/AHA Guidelines, Recommendations for Evaluation of Patients With Supravalvular, Branch, and Peripheral Pulmonary Stenosis (DO NOT EDIT)^[103]

Class I
"1. Patients with suspected supravalvular, branch, or peripheral PS should have baseline imaging with echocardiography- Doppler plus 1 of the following: MRI angiography, CT angiography, or contrast angiography. (Level of Evidence: C)"
"2. Once the diagnosis is established, follow-up echocardiography- Doppler to assess RV systolic pressure should be performed periodically, depending on severity. (Level of Evidence: C)"

Recommendations for Interventional Therapy in the Management of Branch and Peripheral Pulmonary Stenosis (DO NOT EDIT)^[103]

Class I
"1. Percutaneous interventional therapy is recommended as the treatment of choice in the management of appropriate focal branch and/or peripheral pulmonary artery stenosis with greater than 50% diameter narrowing, an elevated RV systolic pressure greater than 50 mm Hg, and/or symptoms. (Level of Evidence: B)"
"2. In patients with the above indications for intervention, surgeons with training and expertise in CHD should perform operations for management of branch pulmonary artery stenosis not anatomically amenable to percutaneous interventional therapy. (Level of Evidence: B)"

Recommendations for Evaluation and Follow-Up (DO NOT EDIT)^[103]

Class I
"1. Patients with peripheral PS should be followed up every 1 to 2 years, on the basis of severity, with a clinical evaluation and echocardiography-Doppler to evaluate RV systolic pressure and RV function. (Level of Evidence: C)"
"2. Discussion with a cardiac surgeon with expertise in CHD should take place before percutaneous peripheral pulmonary artery interventions are undertaken. (Level of Evidence: C)"

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v t e Congenital malformations and deformations of circulatory system (Q20-Q28, 745-747)
Cardiac chambers and connections	Persistent truncus arteriosus - Double outlet right ventricle (Taussig-Bing syndrome) - Transposition of the great vessels (dextro, levo)
Cardiac septa	Ventricular septal defect - Atrial septal defect (Lutembacher's syndrome) - Atrioventricular septal defect (Ostium primum) - Tetralogy of Fallot - Eisenmenger's syndrome
Right: pulmonary and tricuspid valves	pulmonary valves (stenosis, insufficiency) - tricuspid valves (stenosis, atresia) - Ebstein's anomaly
Left: aortic and mitral valves	aortic valves (stenosis, insufficiency, bicuspid) - mitral valves (stenosis, regurgitation) - Hypoplastic left heart syndrome
Other congenital malformations of heart	Dextrocardia - Levocardia - Cor triatriatum
Great arteries	aorta (Patent ductus arteriosus, Aortic coarctation, Interrupted aortic arch, Overriding aorta, Aneurysm of sinus of Valsalva, Vascular ring) - Pulmonary atresia
Great veins	Persistent left superior vena cava - Total anomalous pulmonary venous connection - Scimitar syndrome
Other	Arteriovenous malformation (Cerebral arteriovenous malformation)
See also non-congenital conditions (I, 390-459)

@@ Line 15: / Line 15: @@
    MeshID         = |
 }}
 {{CMG}}; {{AE}} {{AKI}}
 {{SK}} Valvular Pulmonary Stenosis, Pulmonic Stenosis, Right Ventricular Outlet Obstruction, supravalvular pulmonic stenosis, infundibular pulmonic stenosis,  Narrowing of pulmonary valve, PS
@@ Line 23: / Line 23: @@
 ==Historical Perspective==
-*The [[pulmonary valve]] and its function of allowing blood to the [[lungs]] for nourishment was first described by [[Hippocrates]].<ref name="ParaskevasKoutsouflianiotis2017">{{cite journal|last1=Paraskevas|first1=G.|last2=Koutsouflianiotis|first2=K.|last3=Iliou|first3=K.|title=The first descriptions of various anatomical structures and embryological remnants of the heart: A systematic overview|journal=International Journal of Cardiology|volume=227|year=2017|pages=674–690|issn=01675273|doi=10.1016/j.ijcard.2016.10.077}}</ref>
+*The [[pulmonary valve]] and its function of allowing blood to the [[lungs]] for nourishment was first described by [[Hippocrates]]. <ref name="pmid18628928">{{cite journal |vauthors=Roberts WC, Ko JM |title=Some observations on mitral and aortic valve disease |journal=Proc (Bayl Univ Med Cent) |volume=21 |issue=3 |pages=282–99 |date=July 2008 |pmid=18628928 |pmc=2446420 |doi=10.1080/08998280.2008.11928412 |url=}}</ref>
-*Erasistratus, mentioned the function of the [[pulmonary valve]] in the unidirectional flow.<ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Felt V, Nedvídková J, Hynie S, Mosinger B, Vavrinková M, Järvisalo J, Saris NE |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1016/0006-2952(75)90009-x |url=}}</ref>
+*Erasistratus, mentioned the function of the [[pulmonary valve]] in the unidirectional flow. <ref name="pmid19065003">{{cite journal| author=Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP et al.| title=Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. | journal=Eur J Echocardiogr | year= 2009 | volume= 10 | issue= 1 | pages= 1-25 | pmid=19065003 | doi=10.1093/ejechocard/jen303 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19065003  }} </ref>
-*Galen described the membranes of the [[valves]] and named them as "semilunar".
+*Galen described the membranes of the [[valves]] and named them as "semilunar". <ref name="pmid7720297">{{cite journal| author=Waller BF, Howard J, Fess S| title=Pathology of tricuspid valve stenosis and pure tricuspid regurgitation--Part I. | journal=Clin Cardiol | year= 1995 | volume= 18 | issue= 2 | pages= 97-102 | pmid=7720297 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7720297  }} </ref>
-*Mondino de Luzzi designed the sketch of the [[pulmonary valves]] in the anatomical position for the first time.
+*Mondino de Luzzi designed the sketch of the [[pulmonary valves]] in the anatomical position for the first time. <ref name="pmid7681733">{{cite journal |vauthors=Pellikka PA, Tajik AJ, Khandheria BK, Seward JB, Callahan JA, Pitot HC, Kvols LK |title=Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients |journal=Circulation |volume=87 |issue=4 |pages=1188–96 |date=April 1993 |pmid=7681733 |doi=10.1161/01.cir.87.4.1188 |url=}}</ref>
-*Realdo Colombo described the [[pulmonary circulation]] for the first time.
+*Realdo Colombo described the [[pulmonary circulation]] for the first time. <ref name="pmid24661289">{{cite journal |vauthors=Gur AK, Odabasi D, Kunt AG, Kunt AS |title=Isolated tricuspid valve repair for Libman-Sacks endocarditis |journal=Echocardiography |volume=31 |issue=6 |pages=E166–8 |date=July 2014 |pmid=24661289 |doi=10.1111/echo.12558 |url=}}</ref>
-*Aortic stenosis was probably first described by Lazare Riviere (1589-1655), a French physician in 1663. <ref name="pmid8480616">{{cite journal| author=Vaslef SN, Roberts WC| title=Early descriptions of aortic valve stenosis. | journal=Am Heart J | year= 1993 | volume= 125 | issue= 5 Pt 1 | pages= 1465-74 | pmid=8480616 | doi=10.1016/0002-8703(93)91036-e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8480616  }} </ref>.
+*Aortic stenosis was probably first described by Lazare Riviere (1589-1655), a French physician in 1663. <ref name="pmid21365261">{{cite journal |vauthors=Muraru D, Badano LP, Sarais C, Soldà E, Iliceto S |title=Evaluation of tricuspid valve morphology and function by transthoracic three-dimensional echocardiography |journal=Curr Cardiol Rep |volume=13 |issue=3 |pages=242–9 |date=June 2011 |pmid=21365261 |doi=10.1007/s11886-011-0176-3 |url=}}</ref>
+*The first transcatheter aortic valve replacement procedure in the world was performed on 16 April 2002 in a 57-year-old inoperable patient with severe aortic stenosis. The procedure was done by the Interventional Cardiologist Professor Alain Cribier at the Charles Nicolle University Hospital in Rouen, France.
 ==Classification==
 ===Based on the anatomic location===
-[[Pulmonic stenosis]] is classified into valvular, subvalvular (infundibular) and supravalvular based on the location of the [[stenosis]] in relation to the pulmonary valve. Valvular stenosis is most common of the three sub-types. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Sinelnikova EM, Dvoretskova TV, Kagan ZS, Odom DG, Brady JT, Kenyon G, Carter DC, Mekler LB, Dougherty EM, Vaughn JL, Reichelderfer CF |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1007/bf01732356 |url=}}</ref>
+[[Pulmonic stenosis]] is classified into valvular, subvalvular (infundibular) and supravalvular based on the location of the [[stenosis]] in relation to the pulmonary valve. Valvular stenosis is most common of the three sub-types.<ref name="pmid3337000">{{cite journal| author=Kelly TA, Rothbart RM, Cooper CM, Kaiser DL, Smucker ML, Gibson RS| title=Comparison of outcome of asymptomatic to symptomatic patients older than 20 years of age with valvular aortic stenosis. | journal=Am J Cardiol | year= 1988 | volume= 61 | issue= 1 | pages= 123-30 | pmid=3337000 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3337000  }} </ref> <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Felt V, Nedvídková J, Hynie S, Mosinger B, Vavrinková M, Järvisalo J, Saris NE |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1016/0006-2952(75)90009-x |url=}}</ref>
 *'''Sub-valvular stenosis''': It can be infudibular or sub-infundibular. Infundibular stenosis is a feature of [[tetralogy of Fallot]]. Sub-infundibular pulmonic stenosis is known as ‘[[Double outlet right ventricle|double]] chambered right ventricle’ dividing the right ventricle into a high pressure inlet and a low pressure outlet causing a progressive [[right ventricular outflow tract obstruction]].<ref name="pmid7588901">{{cite journal| author=Cabrera A, Martinez P, Rumoroso JR, Alcibar J, Arriola J, Pastor E et al.| title=Double-chambered right ventricle. | journal=Eur Heart J | year= 1995 | volume= 16 | issue= 5 | pages= 682-6 | pmid=7588901 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7588901  }}</ref>
 *'''Valvular stenosis''': It is the most common cause of pulmonic stenosis. The valves are usually dome shaped or dysplastic affecting the movement of the cusps. It can be isolated or associated with other [[congenital heart diseases]] such as [[atrial septal defect]], [[Ebstein’s anomaly|Ebstein’s]] anomaly, [[double outlet right ventricle]], and transposition of the great arteries.<ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Mills GC, Alperin JB, Trimmer KB, Dooren LJ, de Koning J, Kamphuis RP, Uittenbogaart CH, Brubakk AM, Vossen JM, Marniemi J, Parkki MG, Shute CC, Anderson TR, Slotkin TA |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1016/0006-2944(75)90084-8 |url=}}</ref>
 *'''Supravalvular stenosis''': The obstruction is usually in the common pulmonary trunk or in the bifurcation or the pulmonary branches. It is commonly associated with other congenital syndromes  such as Williams–Beuren, [[Noonan syndrome|Noonan]], Allagile syndrome, [[DiGeorges Syndrome|DiGeorge]], and [[Leopard syndrome|Leopard]] syndrome. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Price J, Sandhu SS, Nelson P, Warren WJ, Nakamura M, Romrell LJ, Hall PF |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1016/0009-8981(75)90259-4 |url=}}</ref>
 ===Based on the severity of the stenosis===
-Severity of pulmonary stenosis is classified based on the estimated peak velocity and peak resting gradient calculated using modified [[Bernoulli equation|Bernoulli equatio]]<nowiki/>n. It is classified into:<ref name="pmid19130998">{{cite journal| author=Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP et al.| title=Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. | journal=J Am Soc Echocardiogr | year= 2009 | volume= 22 | issue= 1 | pages= 1-23; quiz 101-2 | pmid=19130998 | doi=10.1016/j.echo.2008.11.029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19130998  }}</ref> <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Robinson JV, James AL, Sondén A, Rocksén D, Riddez L, Davidsson J, Persson JK, Gryth D, Bursell J, Arborelius UP, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Meyer WJ, Gidwitz S, Ayers VK, Schoepp RJ, Johnston RE |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1097/TA.0b013e3181a5b0e1 |url=}}</ref>
+Severity of pulmonary stenosis is classified based on the estimated peak velocity and peak resting gradient calculated using modified [[Bernoulli equation|Bernoulli equatio]]<nowiki/>n. It is classified into:<ref name="pmid19130998">{{cite journal| author=Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP et al.| title=Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. | journal=J Am Soc Echocardiogr | year= 2009 | volume= 22 | issue= 1 | pages= 1-23; quiz 101-2 | pmid=19130998 | doi=10.1016/j.echo.2008.11.029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19130998  }}</ref>
-*'''Mild:''' Peak velocity less than 3m/s and peak [[gradient]] is less than 36 mm Hg.
+*'''Mild:''' Peak velocity less than 3m/s and peak [[gradient]] is less than 36 mm Hg.<ref name="pmid19130998">{{cite journal| author=Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP et al.| title=Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. | journal=J Am Soc Echocardiogr | year= 2009 | volume= 22 | issue= 1 | pages= 1-23; quiz 101-2 | pmid=19130998 | doi=10.1016/j.echo.2008.11.029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19130998  }}</ref>
 *'''Moderate:''' Peak velocity is 3 to 4m/s and peak [[gradient]] is 36 to 64mm Hg.
-*'''Severe''': Peak velocity is greater than 4m/s and peak gradient is greater than 64mm Hg.
+*'''Severe''': Peak velocity is greater than 4m/s and peak gradient is greater than 64mm Hg. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Robinson JV, James AL, Sondén A, Rocksén D, Riddez L, Davidsson J, Persson JK, Gryth D, Bursell J, Arborelius UP, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Meyer WJ, Gidwitz S, Ayers VK, Schoepp RJ, Johnston RE |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1097/TA.0b013e3181a5b0e1 |url=}}</ref>
+*According to 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease, stages of severe [[pulmonic stenosis]] are defined as follows: This form of aortic stenosis presents later in life, usually after the age of 75. <ref>Townsend CM, et al. Sabiston Textbook of Surgery. 18th ed. Saunders; 2008:1841-1844.</ref>
-*According to 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease, stages of severe [[pulmonic stenosis]] are defined as follows:<ref name="pmid24603192">{{cite journal |vauthors=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA, O'Gara PT, Ruiz CE, Skubas NJ, Sorajja P, Sundt TM, Thomas JD |title=2014 AHA/ACC guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines |journal=J. Am. Coll. Cardiol. |volume=63 |issue=22 |pages=2438–88 |year=2014 |pmid=24603192 |doi=10.1016/j.jacc.2014.02.537 |url=}}</ref> <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref>
 {| class="wikitable"
 !Stage
@@ Line 94: / Line 99: @@
 ==Pathophysiology==
 ===Pathogenesis===
-*Pulmonic valve stenosis with fused [[commisures]] affect the flexibility of the [[valve]] causing obstruction of the outflow tract. In patients with dysplastic valves, the [[cusps]] are not fused but they are rigid from intrinsic thickening resulting in the narrowing of the outflow tract.<ref name="pmid18848134">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to revise the 1998 guidelines for the management of patients with valvular heart disease). Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 13 | pages= e1-142 | pmid=18848134 | doi=10.1016/j.jacc.2008.05.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18848134  }} </ref> <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Sanchez G, Alderete JF, Leal KW, Friel PN, Rapport RL, Wilensky AJ, Akamatsu N, Nakajima H, Ono M, Miura Y, Hattori M, Frazier J, Miles HT, Bose KS, Sarma RH |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1016/s0300-9629(75)80013-2 |url=}}</ref>
+*Pulmonic valve stenosis with fused [[commisures]] affect the flexibility of the [[valve]] causing obstruction of the outflow tract. In patients with dysplastic valves, the [[cusps]] are not fused but they are rigid from intrinsic thickening resulting in the narrowing of the outflow tract. The valve problems develop 5 - 10 years after the rheumatic fever, a tiny nodule forms along the valve leaflets. The degree of leaflet thickening and [[calcification]] and the severity of chordal involvement are variable. Rheumatic fever is becoming rare in the United States, so mitral stenosis is also less common.<ref name="agabegi2nd-ch1">Chapter 1: Diseases of the Cardiovascular system > Section: Valvular Heart Disease in: {{cite book |author=Elizabeth D Agabegi; Agabegi, Steven S. |title=Step-Up to Medicine (Step-Up Series) |publisher=Lippincott Williams & Wilkins |location=Hagerstwon, MD |year=2008 |pages= |isbn=0-7817-7153-6 |oclc= |doi= |accessdate=}}</ref>
 *These morphological changes affect the complete opening of the [[pulmonic valve]] during the [[ventricular systole]] causing elevated right ventricular systolic pressures and leading to right ventricular remodelling.<ref name="pmid25771982">{{cite journal| author=Borgdorff MA, Dickinson MG, Berger RM, Bartelds B| title=Right ventricular failure due to chronic pressure load: What have we learned in animal models since the NIH working group statement? | journal=Heart Fail Rev | year= 2015 | volume= 20 | issue= 4 | pages= 475-91 | pmid=25771982 | doi=10.1007/s10741-015-9479-6 | pmc=4463984 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25771982  }}</ref>
-*The obstruction leads to increased pressure overload on the [[right ventricle]] as it has to push the blood against resistance. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Price J, Philipps GR, Chiemprasert T |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1016/0009-8981(75)90259-4 |url=}}</ref> <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Holder AA, Wootton JC, Baron AJ, Chambers GK, Fincham JR |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1042/bj1490757 |url=}}</ref>
+*The obstruction leads to increased pressure overload on the [[right ventricle]] as it has to push the blood against resistance. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Price J, Philipps GR, Chiemprasert T |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1016/0009-8981(75)90259-4 |url=}}</ref>
 ===Genetics===
-These are a common genetic disorders associated with pulmonic stenosis:<ref name="pmid17519398">{{cite journal| author=Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E et al.| title=Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. | journal=Circulation | year= 2007 | volume= 115 | issue= 23 | pages= 3015-38 | pmid=17519398 | doi=10.1161/CIRCULATIONAHA.106.183056 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17519398  }} </ref> <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref>
+These are a common genetic disorders associated with pulmonic stenosis:<ref name="pmid17519398">{{cite journal| author=Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E et al.| title=Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. | journal=Circulation | year= 2007 | volume= 115 | issue= 23 | pages= 3015-38 | pmid=17519398 | doi=10.1161/CIRCULATIONAHA.106.183056 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17519398  }} </ref>
 {| class="wikitable"
 !Syndrome
@@ Line 106: / Line 111: @@
 !Other features
 |-
 |[[Noonan syndrome]]<ref name="pmid20301303">{{cite journal |vauthors=Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Fong CT, Mefford HC, Smith RJH, Stephens K, Allanson JE, Roberts AE |title= |journal= |volume= |issue= |pages= |year= |pmid=20301303 |doi= |url=}}</ref>
 |
 *PTPN11, SOS1
@@ Line 190: / Line 195: @@
 *Subnormal [[IQ]] and [[hearing loss]]
 |-
 |[[Congenital rubella syndrome]]<ref name="pmid4589966">{{cite journal| author=Rowe RD| title=Cardiovascular disease in the rubella syndrome. | journal=Cardiovasc Clin | year= 1973 | volume= 5 | issue= 1 | pages= 61-80 | pmid=4589966 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4589966  }}</ref>
 |   N/A
 |
@@ Line 202: / Line 207: @@
 === Associated conditions ===
-A rare association of pulmonic stenosis with an unrepaired [[ASD]] is reported.<ref name="pmid26901261">{{cite journal| author=Zampi G, Pergolini A, Celestini A, Benvissuto F, Tinti MD, Ortenzi M et al.| title=[Pulmonary stenosis and atrial septal defect: a rare association in the elderly]. | journal=G Ital Cardiol (Rome) | year= 2016 | volume= 17 | issue= 1 | pages= 62-3 | pmid=26901261 | doi=10.1714/2140.23196 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26901261  }}</ref>
+A rare association of pulmonic stenosis with an unrepaired [[ASD]] is reported.<ref name="pmid26901261">{{cite journal| author=Zampi G, Pergolini A, Celestini A, Benvissuto F, Tinti MD, Ortenzi M et al.| title=[Pulmonary stenosis and atrial septal defect: a rare association in the elderly]. | journal=G Ital Cardiol (Rome) | year= 2016 | volume= 17 | issue= 1 | pages= 62-3 | pmid=26901261 | doi=10.1714/2140.23196 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26901261  }}</ref> <ref name="pmid23489526">{{cite journal| author=Park SJ, Enriquez-Sarano M, Chang SA, Choi JO, Lee SC, Park SW et al.| title=Hemodynamic patterns for symptomatic presentations of severe aortic stenosis. | journal=JACC Cardiovasc Imaging | year= 2013 | volume= 6 | issue= 2 | pages= 137-46 | pmid=23489526 | doi=10.1016/j.jcmg.2012.10.013 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23489526  }} </ref> <ref name="pmid10901521">{{cite journal| author=Nazari S, Carli F, Salvi S, Banfi C, Aluffi A, Mourad Z et al.| title=Patterns of systolic stress distribution on mitral valve anterior leaflet chordal apparatus. A structural mechanical theoretical analysis. | journal=J Cardiovasc Surg (Torino) | year= 2000 | volume= 41 | issue= 2 | pages= 193-202 | pmid=10901521 | doi= | pmc= | url= }} </ref>
 ==Epidemiology and Demographics==
-*Pulmonary stenosis accounts for 8% of all [[congenital heart disease]].
+*Pulmonary stenosis accounts for 8% of all [[congenital heart disease]]. <ref name="pmid24986049">{{cite journal| author=Iung B, Vahanian A| title=Epidemiology of acquired valvular heart disease. | journal=Can J Cardiol | year= 2014 | volume= 30 | issue= 9 | pages= 962-70 | pmid=24986049 | doi=10.1016/j.cjca.2014.03.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24986049  }} </ref>
-*Worldwide, the [[prevalence]] of pulmonic stenosis is 1 per 2000 births.<ref name="pmid22078432">{{cite journal| author=van der Linde D, Konings EE, Slager MA, Witsenburg M, Helbing WA, Takkenberg JJ et al.| title=Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 21 | pages= 2241-7 | pmid=22078432 | doi=10.1016/j.jacc.2011.08.025 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22078432  }} </ref>
-*The [[prevalence]] of pulmonic stenosis and [[tetralogy of Fallot]] is higher in asian countries.<ref name="pmid10754087">{{cite journal| author=Jacobs EG, Leung MP, Karlberg J| title=Distribution of symptomatic congenital heart disease in Hong Kong. | journal=Pediatr Cardiol | year= 2000 | volume= 21 | issue= 2 | pages= 148-57 | pmid=10754087 | doi=10.1007/s002469910025 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10754087  }} </ref>
+*Worldwide, the [[prevalence]] of pulmonic stenosis is 1 per 2000 births.<ref name="pmid25140960">{{cite journal| author=Otto CM, Prendergast B| title=Aortic-valve stenosis--from patients at risk to severe valve obstruction. | journal=N Engl J Med | year= 2014 | volume= 371 | issue= 8 | pages= 744-56 | pmid=25140960 | doi=10.1056/NEJMra1313875 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25140960  }} </ref>
+*The [[prevalence]] of pulmonic stenosis and [[tetralogy of Fallot]] is higher in asian countries.<ref name="pmid10754087">{{cite journal| author=Jacobs EG, Leung MP, Karlberg J| title=Distribution of symptomatic congenital heart disease in Hong Kong. | journal=Pediatr Cardiol | year= 2000 | volume= 21 | issue= 2 | pages= 148-57 | pmid=10754087 | doi=10.1007/s002469910025 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10754087  }} </ref> <ref name="pmid18222317">{{cite journal |vauthors=Shah PM, Raney AA |title=Tricuspid valve disease |journal=Curr Probl Cardiol |volume=33 |issue=2 |pages=47–84 |date=February 2008 |pmid=18222317 |doi=10.1016/j.cpcardiol.2007.10.004 |url=}}</ref>
 ==Causes==
-Pulmonary valve stenosis is due to a structural changes resulting from thickening and fusion of the [[pulmonary valve]]. The [[valve]] pathology can be [[congenital]] or acquired. The following is the list of causes:
+Pulmonary valve stenosis is due to a structural changes resulting from thickening and fusion of the [[pulmonary valve]]. The [[valve]] pathology can be [[congenital]] or acquired. The following is the list of causes: <ref name="pmid28236633">{{cite journal |vauthors=Farag M, Arif R, Sabashnikov A, Zeriouh M, Popov AF, Ruhparwar A, Schmack B, Dohmen PM, Szabó G, Karck M, Weymann A |title=Repair or Replacement for Isolated Tricuspid Valve Pathology? Insights from a Surgical Analysis on Long-Term Survival |journal=Med. Sci. Monit. |volume=23 |issue= |pages=1017–1025 |date=February 2017 |pmid=28236633 |pmc=5338566 |doi=10.12659/msm.900841 |url=}}</ref><ref name="pmid30311884">{{cite journal |vauthors=Salem A, Abdelgawad AME, Elshemy A |title=Early and Midterm Outcomes of Rheumatic Mitral Valve Repair |journal=Heart Surg Forum |volume=21 |issue=5 |pages=E352–E358 |date=August 2018 |pmid=30311884 |doi=10.1532/hsf.1978 |url=}}</ref>.
 ====Congenital causes====
 These account for 95% of the cases with pulmonic stenosis which include isolated pulmonic valve pathologies and its associations with other [[congenital heart diseases]].<ref name="pmid2593721">{{cite journal| author=Altrichter PM, Olson LJ, Edwards WD, Puga FJ, Danielson GK| title=Surgical pathology of the pulmonary valve: a study of 116 cases spanning 15 years. | journal=Mayo Clin Proc | year= 1989 | volume= 64 | issue= 11 | pages= 1352-60 | pmid=2593721 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2593721  }}</ref>
 '''Associated with congenital heart disease'''
-*[[Tetralogy of Fallot]]<ref name="pmid9388286">{{cite journal| author=Greenberg SB, Crisci KL, Koenig P, Robinson B, Anisman P, Russo P| title=Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery. | journal=Pediatr Radiol | year= 1997 | volume= 27 | issue= 12 | pages= 932-5 | pmid=9388286 | doi=10.1007/s002470050275 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9388286  }}</ref>
+*[[Tetralogy of Fallot]]<ref name="pmid9388286">{{cite journal| author=Greenberg SB, Crisci KL, Koenig P, Robinson B, Anisman P, Russo P| title=Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery. | journal=Pediatr Radiol | year= 1997 | volume= 27 | issue= 12 | pages= 932-5 | pmid=9388286 | doi=10.1007/s002470050275 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9388286  }}</ref> <ref name="urlValvular Heart Disease - ScienceDirect">{{cite web |url=+https://www.sciencedirect.com/science/article/pii/B9780124202191000124?via%3Dihub |title=Valvular Heart Disease - ScienceDirect |format= |work= |accessdate=}}</ref> <ref name="pmid8459080">{{cite journal |author=Lindroos M, Kupari M, Heikkilä J, Tilvis R |title=Prevalence of aortic valve abnormalities in the elderly: an echocardiographic study of a random population sample |journal=[[Journal of the American College of Cardiology]] |volume=21 |issue=5 |pages=1220–5 |year=1993 |month=April |pmid=8459080 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/0735-1097(93)90249-Z |accessdate=2012-04-11}}</ref>
-*[[Double outlet right ventricle]]
+*[[Double outlet right ventricle]] <ref name="pmid9060903">{{cite journal |author=Stewart BF, Siscovick D, Lind BK, Gardin JM, Gottdiener JS, Smith VE, Kitzman DW, Otto CM |title=Clinical factors associated with calcific aortic valve disease. Cardiovascular Health Study |journal=[[Journal of the American College of Cardiology]] |volume=29 |issue=3 |pages=630–4 |year=1997 |month=March |pmid=9060903 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0735109796005633 |accessdate=2012-04-11}}</ref>
-*Univentricular atrio-ventricular connection
+*Univentricular atrio-ventricular connection <ref name="pmid16825001">{{cite journal| author=Movahed MR, Ahmadi-Kashani M, Kasravi B, Saito Y| title=Increased prevalence of mitral stenosis in women. | journal=J Am Soc Echocardiogr | year= 2006 | volume= 19 | issue= 7 | pages= 911-3 | pmid=16825001 | doi=10.1016/j.echo.2006.01.017 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16825001  }} </ref>
-*Atrioventricular canal defect
+*Atrioventricular canal defect <ref name="urlHarold on History: The Evolution of Transcatheter Aortic Valve Replacement - American College of Cardiology">{{cite web |url=https://www.acc.org/latest-in-cardiology/articles/2017/07/19/15/42/the-evolution-of-transcatheter-aortic-valve-replacement |title=Harold on History: The Evolution of Transcatheter Aortic Valve Replacement - American College of Cardiology |format= |work= |accessdate=}}</ref>
 *Bicuspid pulmonary valve<ref name="pmid19557994">{{cite journal| author=Jashari R, Van Hoeck B, Goffin Y, Vanderkelen A| title=The incidence of congenital bicuspid or bileaflet and quadricuspid or quadrileaflet arterial valves in 3,861 donor hearts in the European Homograft Bank. | journal=J Heart Valve Dis | year= 2009 | volume= 18 | issue= 3 | pages= 337-44 | pmid=19557994 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19557994  }}</ref>
 *Quadricuspid pulmonary valve: Benign and an incidental finding<ref name="pmid19017322">{{cite journal| author=Fernández-Armenta J, Villagómez D, Fernández-Vivancos C, Vázquez R, Pastor L| title=Quadricuspid pulmonary valve identified by transthoracic echocardiography. | journal=Echocardiography | year= 2009 | volume= 26 | issue= 3 | pages= 288-90 | pmid=19017322 | doi=10.1111/j.1540-8175.2008.00798.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19017322  }}</ref>
 *Isolated pulmonic stenosis<ref name="pmid2957652">{{cite journal| author=Gikonyo BM, Lucas RV, Edwards JE| title=Anatomic features of congenital pulmonary valvar stenosis. | journal=Pediatr Cardiol | year= 1987 | volume= 8 | issue= 2 | pages= 109-16 | pmid=2957652 | doi=10.1007/BF02079465 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2957652  }}</ref>
 **Acommissural pulmonary valves: Valve has a prominent systolic doming of the cusps and an eccentric orifice.<ref name="pmid4889601">{{cite journal| author=Snellen HA, Hartman H, Buis-Liem TN, Kole EH, Rohmer J| title=Pulmonic stenosis. | journal=Circulation | year= 1968 | volume= 38 | issue= 1 Suppl | pages= 93-101 | pmid=4889601 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4889601  }}</ref><ref name="pmid26656195">{{cite journal| author=Jonas SN, Kligerman SJ, Burke AP, Frazier AA, White CS| title=Pulmonary Valve Anatomy and Abnormalities: A Pictorial Essay of Radiography, Computed Tomography (CT), and Magnetic Resonance Imaging (MRI). | journal=J Thorac Imaging | year= 2016 | volume= 31 | issue= 1 | pages= W4-12 | pmid=26656195 | doi=10.1097/RTI.0000000000000182 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26656195  }}</ref>
-**Dysplastic [[pulmonary valves]]: Thickened and deformed cusps with no commissural fusion.<ref name="pmid5792996">{{cite journal| author=Koretzky ED, Moller JH, Korns ME, Schwartz CJ, Edwards JE| title=Congenital pulmonary stenosis resulting from dysplasia of valve. | journal=Circulation | year= 1969 | volume= 40 | issue= 1 | pages= 43-53 | pmid=5792996 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5792996  }}</ref> It is a common finding associated with [[Noonan syndrome]].<ref name="pmid57929962">{{cite journal| author=Koretzky ED, Moller JH, Korns ME, Schwartz CJ, Edwards JE| title=Congenital pulmonary stenosis resulting from dysplasia of valve. | journal=Circulation | year= 1969 | volume= 40 | issue= 1 | pages= 43-53 | pmid=5792996 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5792996  }}</ref>
+**Dysplastic [[pulmonary valves]]: Thickened and deformed cusps with no commissural fusion. It is a common finding associated with [[Noonan syndrome]].<ref name="pmid57929962">{{cite journal| author=Koretzky ED, Moller JH, Korns ME, Schwartz CJ, Edwards JE| title=Congenital pulmonary stenosis resulting from dysplasia of valve. | journal=Circulation | year= 1969 | volume= 40 | issue= 1 | pages= 43-53 | pmid=5792996 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5792996  }}</ref>
 **Unicommissural [[pulmonary valve]]
 **Bicuspid valve with fused [[commissures]]
 ====Acquired Causes====
-These are less frequent and account for less than 5% of the cases
+These are less frequent and account for less than 5% of the cases. <ref name="url15 Years in TAVI">{{cite web |url=https://www.pcronline.com/About-PCR/40-years-angioplasty/15-years-TAVI |title=15 Years in TAVI |format= |work= |accessdate=}}</ref>
-*[[Carcinoid syndrome|Carcinoid Syndrome]]: most common acquired cause<ref name="pmid6391843">{{cite journal| author=Waller BF| title=Morphological aspects of valvular heart disease: Part II. | journal=Curr Probl Cardiol | year= 1984 | volume= 9 | issue= 8 | pages= 1-74 | pmid=6391843 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6391843  }}</ref><ref name="pmid11838647">{{cite journal| author=Simula DV, Edwards WD, Tazelaar HD, Connolly HM, Schaff HV| title=Surgical pathology of carcinoid heart disease: a study of 139 valves from 75 patients spanning 20 years. | journal=Mayo Clin Proc | year= 2002 | volume= 77 | issue= 2 | pages= 139-47 | pmid=11838647 | doi=10.4065/77.2.139 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11838647  }}</ref><ref name="pmid25427026">{{cite journal| author=Paredes A, Valdebenito M, Gabrielli L, Castro P, Zalaquett R| title=[Tricuspid and pulmonary valve involvement in carcinoid syndrome. Report of two cases]. | journal=Rev Med Chil | year= 2014 | volume= 142 | issue= 5 | pages= 662-6 | pmid=25427026 | doi=10.4067/S0034-98872014000500017 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25427026  }}</ref>
+*[[Carcinoid syndrome|Carcinoid Syndrome]]: most common acquired cause<ref name="pmid6391843">{{cite journal| author=Waller BF| title=Morphological aspects of valvular heart disease: Part II. | journal=Curr Probl Cardiol | year= 1984 | volume= 9 | issue= 8 | pages= 1-74 | pmid=6391843 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6391843  }}</ref>
 *Post infectious: [[Infective endocarditis]]
 *[[Calcification]] of the [[pulmonary valve]]<ref name="pmid5448727">{{cite journal| author=Gabriele OF, Scatliff JH| title=Pulmonary valve calcification. | journal=Am Heart J | year= 1970 | volume= 80 | issue= 3 | pages= 299-302 | pmid=5448727 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5448727  }}</ref>
@@ Line 237: / Line 243: @@
 == Differentiating from other diseases ==
-Right ventricular outflow tract obstruction must be distinguished from an [[ASD]],  a small [[VSD]], [[aortic stenosis]], and acyanotic or pink [[tetralogy of Fallot]].
+Right ventricular outflow tract obstruction must be distinguished from an [[ASD]],  a small [[VSD]], [[aortic stenosis]], and acyanotic or pink [[tetralogy of Fallot]].<ref name="pmid21386976">{{cite journal| author=Seckeler MD, Hoke TR| title=The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease. | journal=Clin Epidemiol | year= 2011 | volume= 3 | issue=  | pages= 67-84 | pmid=21386976 | doi=10.2147/CLEP.S12977 | pmc=PMC3046187 | url= }} </ref>
-* [[Atrial septal defect]]: Presence of [[systolic ejection murmur]], wide fixed split S2, [[EKG]] showing [[RVH]]. In [[ASD]] the split of the S2 is fixed, there is no ejection click.
+* [[Atrial septal defect]]: Presence of [[systolic ejection murmur]], wide fixed split S2, [[EKG]] showing [[RVH]]. In [[ASD]] the split of the S2 is fixed, there is no ejection click. <ref name="pmid12633546">{{cite journal |author=Cleland JG, Swedberg K, Follath F, Komajda M, Cohen-Solal A, Aguilar JC, Dietz R, Gavazzi A, Hobbs R, Korewicki J, Madeira HC, Moiseyev VS, Preda I, van Gilst WH, Widimsky J, Freemantle N, Eastaugh J, Mason J |title=The EuroHeart Failure survey programme-- a survey on the quality of care among patients with heart failure in Europe. Part 1: patient characteristics and diagnosis |journal=[[European Heart Journal]] |volume=24 |issue=5 |pages=442–63 |year=2003 |month=March |pmid=12633546 |doi= |url=http://eurheartj.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=12633546 |accessdate=2012-04-11}}</ref>
 *Small [[Ventricular septal defect]]: [[Amyl nitrate]] increases venous return and increases the murmur of [[pulmonary stenosis]], in [[VSD]] the [[murmur]] becomes softer.
 *Mild left-sided outflow obstruction: With [[valsalva maneuver]] the murmur of [[aortic stenosis]] becomes softer after about 5 beats, with [[pulmonary stenosis]] it becomes softer within 3 beats.
-*Acyanotic or pink [[tetralogy of Fallot]]: with [[amyl nitrate]] and increased [[venous return]] the [[murmur]] of PS increases, and the [[murmur]] of [[tetralogy of Fallot]] decreases because of peripheral vasodilation and an increase in right to left shunting.
+*Acyanotic or pink [[tetralogy of Fallot]]: with [[amyl nitrate]] and increased [[venous return]] the [[murmur]] of PS increases, and the [[murmur]] of [[tetralogy of Fallot]] decreases because of peripheral vasodilation and an increase in right to left shunting. <ref name="pmid28236633">{{cite journal |vauthors=Farag M, Arif R, Sabashnikov A, Zeriouh M, Popov AF, Ruhparwar A, Schmack B, Dohmen PM, Szabó G, Karck M, Weymann A |title=Repair or Replacement for Isolated Tricuspid Valve Pathology? Insights from a Surgical Analysis on Long-Term Survival |journal=Med. Sci. Monit. |volume=23 |issue= |pages=1017–1025 |date=February 2017 |pmid=28236633 |pmc=5338566 |doi=10.12659/msm.900841 |url=}}</ref>
 ==Risk Factors==
 Common [[risk factors]] in the development of [[congenital heart disease]] apply for pulmonic stenosis and include:<ref name="pmid220784322">{{cite journal| author=van der Linde D, Konings EE, Slager MA, Witsenburg M, Helbing WA, Takkenberg JJ et al.| title=Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 21 | pages= 2241-7 | pmid=22078432 | doi=10.1016/j.jacc.2011.08.025 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22078432  }}</ref>
 *Maternal [[gestational diabetes mellitus]]
 *[[Consanguineous]] marriage<ref name="pmid570260">{{cite journal| author=Naderi S| title=Congenital abnormalities in newborns of consanguineous and nonconsanguineous parents. | journal=Obstet Gynecol | year= 1979 | volume= 53 | issue= 2 | pages= 195-9 | pmid=570260 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=570260  }} </ref>
 *[[Phenylketonuria]]
 *Febrile illness
-*[[Vitamin A]] use
+*[[Vitamin A]] use <ref name="Aronow-2013">{{Cite journal | last1 = Aronow | first1 = WS. | title = A review of the pathophysiology, diagnosis, and treatment of aortic valve stenosis in elderly patients. | journal = Hosp Pract (1995) | volume = 41 | issue = 4 | pages = 66-77 | month = Oct | year = 2013 | doi = 10.3810/hp.2013.10.1082 | PMID = 24145591 }}</ref>
-*[[Marijuana]] use
+*[[Marijuana]] use <ref name="pmid17921746">{{cite journal| author=Moura LM, Maganti K, Puthumana JJ, Rocha-Gonçalves F, Rajamannan NM| title=New understanding about calcific aortic stenosis and opportunities for pharmacologic intervention. | journal=Curr Opin Cardiol | year= 2007 | volume= 22 | issue= 6 | pages= 572-7 | pmid=17921746 | doi=10.1097/HCO.0b013e3282f0dae6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17921746  }} </ref>
-*Exposure to [[organic solvents]]
+*Exposure to [[organic solvents]]  <ref name="pmid22100375">{{cite journal| author=Hannoush H, Introne WJ, Chen MY, Lee SJ, O'Brien K, Suwannarat P et al.| title=Aortic stenosis and vascular calcifications in alkaptonuria. | journal=Mol Genet Metab | year= 2012 | volume= 105 | issue= 2 | pages= 198-202 | pmid=22100375 | doi=10.1016/j.ymgme.2011.10.017 | pmc=PMC3276068 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22100375 }} </ref>
 ==Natural History, Complications and Prognosis==
 '''Natural History'''
-Patients with [[congenital]] pulmonary stenosis manifest clinical features few hours after birth, in childhood or in adulthood. Manifestation of symptoms, symptom severity and the outcomes are dependent on the severity of [[stenosis]].<ref name="pmid8425320">{{cite journal| author=Hayes CJ, Gersony WM, Driscoll DJ, Keane JF, Kidd L, O'Fallon WM et al.| title=Second natural history study of congenital heart defects. Results of treatment of patients with pulmonary valvar stenosis. | journal=Circulation | year= 1993 | volume= 87 | issue= 2 Suppl | pages= I28-37 | pmid=8425320 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8425320  }}</ref> Patients with mild pulmonic stenosis have a benign course and do not progress and patients with moderate and severe stenosis have [[dyspnea]] with exertion and [[syncope]].<ref name="pmid1163423">{{cite journal| author=Mody MR| title=The natural history of uncomplicated valvular pulmonic stenosis. | journal=Am Heart J | year= 1975 | volume= 90 | issue= 3 | pages= 317-21 | pmid=1163423 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1163423  }}</ref>
+Patients with [[congenital]] pulmonary stenosis manifest clinical features few hours after birth, in childhood or in adulthood. Manifestation of symptoms, symptom severity and the outcomes are dependent on the severity of [[stenosis]]. Patients with mild pulmonic stenosis have a benign course and do not progress and patients with moderate and severe stenosis have [[dyspnea]] with exertion and [[syncope]].<ref name="pmid1163423">{{cite journal| author=Mody MR| title=The natural history of uncomplicated valvular pulmonic stenosis. | journal=Am Heart J | year= 1975 | volume= 90 | issue= 3 | pages= 317-21 | pmid=1163423 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1163423  }}</ref>
 '''Prognosis'''
-Patients with moderate to severe pulmonic valve stenosis are managed well with [[surgery]] or [[balloon valvuloplasty]] and have very good prognosis.
+Patients with moderate to severe pulmonic valve stenosis are managed well with [[surgery]] or [[balloon valvuloplasty]] and have very good prognosis. <ref name="pmid18820172">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=Circulation | year= 2008 | volume= 118 | issue= 15 | pages= e523-661 | pmid=18820172 | doi=10.1161/CIRCULATIONAHA.108.190748 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18820172  }} </ref>
 '''Complications'''
@@ Line 269: / Line 276: @@
 Gold standard diagnosis of pulmonic stenosis and assessment of severity is done by [[Two dimensional echocardiography|2D echocardiography]].<ref name="pmid23560138">{{cite journal| author=Kim DH, Park SJ, Jung JW, Kim NK, Choi JY| title=The Comparison between the Echocardiographic Data to the Cardiac Catheterization Data on the Diagnosis, Treatment, and Follow-Up in Patients Diagnosed as Pulmonary Valve Stenosis. | journal=J Cardiovasc Ultrasound | year= 2013 | volume= 21 | issue= 1 | pages= 18-22 | pmid=23560138 | doi=10.4250/jcu.2013.21.1.18 | pmc=3611114 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23560138  }}</ref>
-===History and Symptoms===
+== History and Symptoms ==
 The severity of symptoms and age of symptom onset depends on the severity of the stenosis. Clinical presentations vary as follows:
-*Critical pulmonary stenosis:It presents in first few hours to days of life with [[cyanosis]].  It is a condition with a very small or pin-hole orifice in the [[pulmonary valve]] which can be diagnosed prenatally. These patients have an intact [[interventricular septum]], poorly complaint [[hypoplastic right ventricle]] and are[[ ductus]] dependent. [[Cyanosis]] in these patients is due to the right to left shunting at the level of the [[foramen ovale]].<ref name="pmid11460987">{{cite journal| author=Hornberger LK, Barrea C| title=Diagnosis, natural history, and outcome of fetal heart disease. | journal=Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu | year= 2001 | volume= 4 | issue=  | pages= 229-43 | pmid=11460987 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11460987  }} </ref><ref name="pmid27646419">{{cite journal| author=Capuruço CA, Vercosa NC, Lopes RM| title=EP08.08: Critical pulmonary valve stenosis in dichorionic and diamniotic twins: prenatal diagnosis, pregnancy outcomes and postnatal development. | journal=Ultrasound Obstet Gynecol | year= 2016 | volume= 48 Suppl 1 | issue=  | pages= 297-8 | pmid=27646419 | doi=10.1002/uog.16888 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27646419  }}</ref>
+*Critical pulmonary stenosis:It presents in first few hours to days of life with [[cyanosis]].  It is a condition with a very small or pin-hole orifice in the [[pulmonary valve]] which can be diagnosed prenatally. These patients have an intact [[interventricular septum]], poorly complaint [[hypoplastic right ventricle]] and are[[ ductus]] dependent. [[Cyanosis]] in these patients is due to the right to left shunting at the level of the [[foramen ovale]].<ref name="pmid11460987">{{cite journal| author=Hornberger LK, Barrea C| title=Diagnosis, natural history, and outcome of fetal heart disease. | journal=Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu | year= 2001 | volume= 4 | issue=  | pages= 229-43 | pmid=11460987 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11460987  }} </ref>
 *Mild Pulmonic Stenosis: Patients with mild stenosis are asymptomatic and are diagnosed by routine examination with an ejection systolic murmur.
-*Moderate Pulmonic Stenosis: Patients present with exertional dyspnea and fatigue.
+*Moderate Pulmonic Stenosis: Patients present with exertional dyspnea and fatigue. <ref name="pmid4552598">{{cite journal| author=Selzer A, Cohn KE| title=Natural history of mitral stenosis: a review. | journal=Circulation | year= 1972 | volume= 45 | issue= 4 | pages= 878-90 | pmid=4552598 | doi= | pmc= | url= }} </ref>
-*Severe Pulmonic Stenosis: Patients present with exertional dyspnea, chest pain and syncope.
+*Severe Pulmonic Stenosis: Patients present with exertional dyspnea, chest pain and syncope. <ref name="pmid14439687">{{cite journal| author=ROWE JC, BLAND EF, SPRAGUE HB, WHITE PD| title=The course of mitral stenosis without surgery: ten- and twenty-year perspectives. | journal=Ann Intern Med | year= 1960 | volume= 52 | issue=  | pages= 741-9 | pmid=14439687 | doi= | pmc= | url= }} </ref>
 *Untreated patients develop features of [[right ventricular failure]] which include:
 **[[Exercise intolerance]]
@@ Line 324: / Line 331: @@
 ===Physical Examination===
 The common examination findings include:
-*Patients with isolated pulmonary stenosis usually appear normal. In patients diagnosed with syndromes associated with pulmonic stenosis syndrome specific physical examination findings are demonstrated.
+*Patients with isolated pulmonary stenosis usually appear normal. In patients diagnosed with syndromes associated with pulmonic stenosis syndrome specific physical examination findings are demonstrated.<ref name="REEVE-1964">{{Cite journal | last1 = REEVE | first1 = R. | last2 = ROBINSON | first2 = SJ. | title = HYPOPLASTIC ANNULUS--AN UNUSUAL TYPE OF AORTIC STENOSIS: A REPORT OF THREE CASES IN CHILDREN. | journal = Dis Chest | volume = 45 | issue = | pages = 99-102 | month = Jan | year = 1964 | doi = | PMID = 14114644 }}</ref>
-*Cardiac examination findings are dependent on the degree of the [[pulmonary stenosis]], the pathology of the [[valve]] and associated cardiac lesions. The common findings include as follows:
+**[[Fabry's disease]]<ref name="pmid7596372">{{cite journal| author=Nakao S, Takenaka T, Maeda M, Kodama C, Tanaka A, Tahara M et al.| title=An atypical variant of Fabry's disease in men with left ventricular hypertrophy. | journal=N Engl J Med | year= 1995 | volume= 333 | issue= 5 | pages= 288-93 | pmid=7596372 | doi=10.1056/NEJM199508033330504 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7596372  }} </ref>
+*Cardiac examination findings are dependent on the degree of the [[pulmonary stenosis]], the pathology of the [[valve]] and associated cardiac lesions. The common findings include as follows: <ref name="pmid27278482">{{cite journal| author=Yamagishi Y, Yuda S, Tsuchihashi K, Saitoh S, Miura T, Ura N et al.| title=Quadricuspid aortic valve associated with aortic stenosis and regurgitation: report of a case and a review of the literature. | journal=J Med Ultrason (2001) | year= 2007 | volume= 34 | issue= 4 | pages= 197-200 | pmid=27278482 | doi=10.1007/s10396-007-0148-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27278482  }} </ref>
 **In mild stenosis findings include normal [[jugular venous pulse]], absent [[right ventricle]] lift, [[ejection click]] in the pulmonary area which decreases with [[inspiration]], [[ejection systolic murmur]] in the pulmonary area heard in the ending of mid systole increasing in intensity during inspiration.<ref name="pmid5377205">{{cite journal |vauthors=Hultgren HN, Reeve R, Cohn K, McLeod R |title=The ejection click of valvular pulmonic stenosis |journal=Circulation |volume=40 |issue=5 |pages=631–40 |year=1969 |pmid=5377205 |doi= |url=}}</ref>
 **In severe stenosis findings include:
 ***Elevated [[Jugular venous pressure|JVP]] with a prominent "a" wave
-***Right ventricular heave
+***Right ventricular heave.
-***Louder and longer [[ejection murmur]] in the left parasternal area in second and third [[intercostal space]]
+***Louder and longer [[ejection murmur]] in the left parasternal area in second and third [[intercostal space]].
-***Ejection click is softer and absent with increasing severity
+***Ejection click is softer and absent with increasing severity.
-***Wide split [[S2 splitting|S2]] with reduced or absent [[Heart sounds|P2]] component
+***Wide split [[S2 splitting|S2]] with reduced or absent [[Heart sounds|P2]] component.
-***Right sided [[S4]] can be audible.
+***Right sided [[S4]] can be audible. <ref name="pmid27721957">{{cite journal| author=Kwon HJ, Park JH, Kim SS, Sun BJ, Jin SA, Kim JH et al.| title=Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male. | journal=J Cardiovasc Ultrasound | year= 2016 | volume= 24 | issue= 3 | pages= 247-250 | pmid=27721957 | doi=10.4250/jcu.2016.24.3.247 | pmc=5050315 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27721957  }} </ref>
@@ Line 360: / Line 369: @@
 ===EKG===
 Patients with mild stenosis usually do not show any [[EKG]] changes excepting for [[right axis deviation]] of -100° to -110° which is considered normal in children and adults.<br>
 In case of severe stenosis the following changes can be noted, which include:<ref name="pmid23303481">{{cite journal| author=Cuypers JA, Witsenburg M, van der Linde D, Roos-Hesselink JW| title=Pulmonary stenosis: update on diagnosis and therapeutic options. | journal=Heart | year= 2013 | volume= 99 | issue= 5 | pages= 339-47 | pmid=23303481 | doi=10.1136/heartjnl-2012-301964 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23303481  }} </ref>
-*Features of [[right ventricular hypertrophy]]
-*[[Rightward axis deviation]]
+*Features of [[right ventricular hypertrophy]]
-*High [[R wave]] amplitude in lead V1
+*[[Rightward axis deviation]] <ref name="pmid26805657">{{cite journal| author=Petit CJ, Gao K, Goldstein BH, Lang SM, Gillespie SE, Kim SI et al.| title=Relation of Aortic Valve Morphologic Characteristics to Aortic Valve Insufficiency and Residual Stenosis in Children With Congenital Aortic Stenosis Undergoing Balloon Valvuloplasty. | journal=Am J Cardiol | year= 2016 | volume= 117 | issue= 6 | pages= 972-9 | pmid=26805657 | doi=10.1016/j.amjcard.2015.12.034 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26805657  }} </ref>
+*High [[R wave]] amplitude in lead V1.
 *Deep S waves in the left [[precordial leads]] with <1 R:S ratio in lead V6
 ===Chest X-Ray===
-*Vascular fullness in the left [[lung]] base greater than the right [[lung]] base.<ref name="pmid5823902">{{cite journal| author=Chen JT, Robinson AE, Goodrich JK, Lester RG| title=Uneven distribution of pulmonary blood flow between left and right lungs in isolated valvular pulmonary stenosis. | journal=Am J Roentgenol Radium Ther Nucl Med | year= 1969 | volume= 107 | issue= 2 | pages= 343-50 | pmid=5823902 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5823902  }} </ref>
+*Vascular fullness in the left [[lung]] base greater than the right [[lung]] base.
 *Dilation of the main [[pulmonary artery]] is a more common finding in doming [[pulmonary valve]] [[stenosis]] when compared to dysplastic valves.
 *[[Calcification]] can be rarely seen.
 ===Echocardiography===
 Transthoracic 2D Echo and [[Doppler]] imaging is the standard diagnostic test to detect and assess the severity of the stenosis.<ref name="pmid912836">{{cite journal| author=Weyman AE, Hurwitz RA, Girod DA, Dillon JC, Feigenbaum H, Green D| title=Cross-sectional echocardiographic visualization of the stenotic pulmonary valve. | journal=Circulation | year= 1977 | volume= 56 | issue= 5 | pages= 769-74 | pmid=912836 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=912836  }}</ref>
-*Echo shows thickened and dome shaped valves, peak and mean gradients to assess the severity can be measured by [[Doppler]] imaging.
+*Echo shows thickened and dome shaped valves, peak and mean gradients to assess the severity can be measured by [[Doppler]] imaging. <ref name="pmid12835667">{{cite journal |author=Zoghbi WA, Enriquez-Sarano M, Foster E, Grayburn PA, Kraft CD, Levine RA, Nihoyannopoulos P, Otto CM, Quinones MA, Rakowski H, Stewart WJ, Waggoner A, Weissman NJ |title=Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography |journal=[[Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography]] |volume=16 |issue=7 |pages=777–802 |year=2003 |month=July |pmid=12835667 |doi=10.1016/S0894-7317(03)00335-3 |url=http://linkinghub.elsevier.com/retrieve/pii/S0894731703003353 |accessdate=2011-03-02}}</ref>
 *Dysplastic valves are well visualized on echo.<ref name="pmid2953383">{{cite journal| author=Musewe NN, Robertson MA, Benson LN, Smallhorn JF, Burrows PE, Freedom RM et al.| title=The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation. | journal=Br Heart J | year= 1987 | volume= 57 | issue= 4 | pages= 364-70 | pmid=2953383 | doi= | pmc=1277176 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2953383  }}</ref>
 *Always calculate the [[tricuspid regurgitation]] gradient to rule out overestimation of the pulmonary stenosis gradient.<ref name="pmid16275521">{{cite journal| author=Silvilairat S, Cabalka AK, Cetta F, Hagler DJ, O'Leary PW| title=Echocardiographic assessment of isolated pulmonary valve stenosis: which outpatient Doppler gradient has the most clinical validity? | journal=J Am Soc Echocardiogr | year= 2005 | volume= 18 | issue= 11 | pages= 1137-42 | pmid=16275521 | doi=10.1016/j.echo.2005.03.041 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16275521  }}</ref>
@@ Line 385: / Line 395: @@
 ===MRI===
 Cardiac [[Magnetic resonance imaging|MRI]] is very useful to study the anatomy of the [[right ventricular outflow tract]], [[pulmonary artery]] and to locate the exact level of [[stenosis]].<ref name="pmid24582177">{{cite journal| author=Rajiah P, Nazarian J, Vogelius E, Gilkeson RC| title=CT and MRI of pulmonary valvular abnormalities. | journal=Clin Radiol | year= 2014 | volume= 69 | issue= 6 | pages= 630-8 | pmid=24582177 | doi=10.1016/j.crad.2014.01.019 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24582177  }}</ref>
 ===Cardiac Catheterization===
 [[Cardiac catheterization]] is useful to measure the [[pressure gradients]] directly, but its not performed on a regular basis as [[echo]] is a reliable and non-invasive test to measure the [[pressure gradients]].<ref name="pmid18997168">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). | journal=Circulation | year= 2008 | volume= 118 | issue= 23 | pages= 2395-451 | pmid=18997168 | doi=10.1161/CIRCULATIONAHA.108.190811 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18997168  }}</ref>
 ===Dual-Source Computed Tomography===
@@ Line 394: / Line 404: @@
 === Evaluation of Pulmonary Stenosis in Adolescents and Young Adults ===
-According to 2008 ACC/[[American Heart Association|AHA]] guidelines<ref name="pmid18848134" />, following are the guidelines for evaluation of patients with pulmonary stenosis:
+According to 2008 ACC/[[American Heart Association|AHA]] guidelines, following are the guidelines for evaluation of patients with pulmonary stenosis:
 {|class="wikitable" width:60%
 |-
@@ Line 414: / Line 424: @@
 ==Treatment==
 ===Medical Therapy===
-There is no specific medical therapy for the treatment of pulmonic stenosis. However, patients diagnosed with right heart failure [[diuretics]] are recommended to decrease the [[fluid overload]].
+There is no specific medical therapy for the treatment of pulmonic stenosis. However, patients diagnosed with right heart failure [[diuretics]] are recommended to decrease the [[fluid overload]]. <ref name="pmid8000584">{{cite journal |author=Schön HR, Dorn R, Barthel P, Schömig A |title=Effects of 12 months quinapril therapy in asymptomatic patients with chronic aortic regurgitation |journal=[[The Journal of Heart Valve Disease]] |volume=3 |issue=5 |pages=500–9 |year=1994 |month=September |pmid=8000584 |doi= |url= |accessdate=2011-03-23}}</ref>
 ===Surgery===
 ====Indications For Intervention====
-Surgical correction is recommended based on the [[peak gradient]] and other associated clinical features:<ref name="pmid18997169">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). | journal=Circulation | year= 2008 | volume= 118 | issue= 23 | pages= e714-833 | pmid=18997169 | doi=10.1161/CIRCULATIONAHA.108.190690 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18997169  }}</ref>
+Surgical correction is recommended based on the [[peak gradient]] and other associated clinical features:<ref name="pmid18997169">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). | journal=Circulation | year= 2008 | volume= 118 | issue= 23 | pages= e714-833 | pmid=18997169 | doi=10.1161/CIRCULATIONAHA.108.190690 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18997169  }}</ref> <ref name="pmid7930196">{{cite journal |author=Lin M, Chiang HT, Lin SL, Chang MS, Chiang BN, Kuo HW, Cheitlin MD |title=Vasodilator therapy in chronic asymptomatic aortic regurgitation: enalapril versus hydralazine therapy |journal=[[Journal of the American College of Cardiology]] |volume=24 |issue=4 |pages=1046–53 |year=1994 |month=October |pmid=7930196 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/0735-1097(94)90868-0 |accessdate=2011-03-23}}</ref>
-*Surgery is advised regardless of the symptoms if the doppler derived peak instantaneous [[gradient]] greater than 64 mm Hg (peak velocity >4 m/s).
+*Surgery is advised regardless of the symptoms if the doppler derived peak instantaneous [[gradient]] greater than 64 mm Hg (peak velocity >4 m/s). <ref name="pmid3289791">{{cite journal |author=Greenberg B, Massie B, Bristow JD, Cheitlin M, Siemienczuk D, Topic N, Wilson RA, Szlachcic J, Thomas D |title=Long-term vasodilator therapy of chronic AR. A randomized double-blinded, placebo-controlled clinical trial |journal=[[Circulation]] |volume=78 |issue=1 |pages=92–103 |year=1988 |month=July |pmid=3289791 |doi= |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=3289791 |accessdate=2011-03-23}}</ref>
-*In patients with doppler derived peak instantaneous [[gradient]] less than 64 mm Hg ([[peak velocity]]>4 m/s), surgery is advised if any of the following is present:
+*In patients with doppler derived peak instantaneous [[gradient]] less than 64 mm Hg ([[peak velocity]]>4 m/s), surgery is advised if any of the following is present:<ref name="pmid8000584">{{cite journal |author=Schön HR, Dorn R, Barthel P, Schömig A |title=Effects of 12 months quinapril therapy in asymptomatic patients with chronic aortic regurgitation |journal=[[The Journal of Heart Valve Disease]] |volume=3 |issue=5 |pages=500–9 |year=1994 |month=September |pmid=8000584 |doi= |url= |accessdate=2011-03-23}}</ref>
 **Symptomatic patient
 **Decreased right [[ventricular function]]
@@ Line 428: / Line 438: @@
 *Asymptomatic patients with a systolic [[RV]] pressure greater than 80 mm Hg ([[TR]] jet velocity >4.3 m/s).
+== 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines<ref name="pmid30121240">{{cite journal| author=Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM | display-authors=etal| title=2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. | journal=J Am Coll Cardiol | year= 2019 | volume= 73 | issue= 12 | pages= 1494-1563 | pmid=30121240 | doi=10.1016/j.jacc.2018.08.1028 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30121240  }}</ref> ==
+=== Recommendations for Valvular Pulmonary Stenosis ===
+{| class="wikitable" width:60%
+|-
+| colspan="1" style="text-align:center; background:LightGreen" |[[ACC AHA guidelines classification scheme#Classification of Recommendations |Class I]]
+|-
+| bgcolor="LightGreen" |'''1.''' In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of HF, cyanosis from interatrial right-to-left communication, and/or exercise intolerance, balloon valvuloplasty is recommended..''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
+|-
+| bgcolor="LightGreen" |'''2.'''In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of HF, cyanosis, and/ or exercise intolerance who are ineligible for or who failed balloon valvuloplasty, surgical repair is recommended''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
+|}
+{| class="wikitable"
+|-
+| colspan="1" style="text-align:center; background:LemonChiffon" |[[ESC Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
+|-
+| bgcolor="LemonChiffon" |'''1.'''In asymptomatic adults with severe valvular pulmonary stenosis, intervention is reasonable. ([[ESC Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C-EO]])''<nowiki>"</nowiki> ''
+|}
 ==According to 2010, ESC Guidelines for the management of grown-up congenital heart disease, Indications for intervention in Right Ventricular Outlet Obstruction are as follows:<ref name="pmid20801927">{{cite journal| author=Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N et al.| title=ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). | journal=Eur Heart J | year= 2010 | volume= 31 | issue= 23 | pages= 2915-57 | pmid=20801927 | doi=10.1093/eurheartj/ehq249 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20801927  }}</ref>==
 {|class="wikitable"
@@ Line 479: / Line 506: @@
 ====Follow up====
-Patients with [[pulmonic stenosis]] are recommended for a regular echocardiography to evaluate the degree of pulmonary regurgitation, RV pressure, RV function and [[tricuspid regurgitation]]. The frequency of visits is dependent on the degree of stenosis and is as follows:<ref name="pmid18848134" />
+Patients with [[pulmonic stenosis]] are recommended for a regular echocardiography to evaluate the degree of pulmonary regurgitation, RV pressure, RV function and [[tricuspid regurgitation]]. The frequency of visits is dependent on the degree of stenosis and is as follows:<ref name="pmid18848134">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to revise the 1998 guidelines for the management of patients with valvular heart disease). Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 13 | pages= e1-142 | pmid=18848134 | doi=10.1016/j.jacc.2008.05.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18848134  }} </ref>
 *Mild untreated or residual [[pulmonic stenosis]]: Follow up once every 5 years.
 *Moderate [[pulmonic stenosis]]: Annual visit with echocardiography every 2 years.
@@ Line 516: / Line 543: @@
 {|class="wikitable"
 |-
-| colspan="1" style="text-align:center; background:LightCoral"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
+| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
 |-
-| bgcolor="LightCoral"|<nowiki>"</nowiki>'''1.''' It is reasonable to perform pulmonary valvuloplasty on a patient with valvar pulmonic stenosis who meets the above criteria in the setting of a dysplastic pulmonary valve.''([[ACC AHA guidelines classification scheme#|Level of Evidence: C]])'' <nowiki>"</nowiki>
+| bgcolor="LemonChiffon" |<nowiki>"</nowiki>'''1.''' It is reasonable to perform pulmonary valvuloplasty on a patient with valvar pulmonic stenosis who meets the above criteria in the setting of a dysplastic pulmonary valve.''([[ACC AHA guidelines classification scheme#|Level of Evidence: C]])'' <nowiki>"</nowiki>
 |-
-| bgcolor="LightCoral"|<nowiki>"</nowiki>'''2.''' It is reasonable to perform pulmonary valvuloplasty in newborns with pulmonary valve atresia and intact ventricular septum who have favorable anatomy that includes the exclusion of RV-dependent coronary circulation.''([[ACC AHA guidelines classification scheme#|Level of Evidence: C]])'' <nowiki>"</nowiki>
+| bgcolor="LemonChiffon" |<nowiki>"</nowiki>'''2.''' It is reasonable to perform pulmonary valvuloplasty in newborns with pulmonary valve atresia and intact ventricular septum who have favorable anatomy that includes the exclusion of RV-dependent coronary circulation.''([[ACC AHA guidelines classification scheme#|Level of Evidence: C]])'' <nowiki>"</nowiki>
 |}
 {|class="wikitable"
@@ Line 538: / Line 565: @@
 *There are no specific primary preventive measures.
 *Patients with diagnosed pulmonary valvar stenosis are not candidates for infective endocarditis prophylaxis.
-*Infective endocarditis prophylaxis is recommended only in patients with prosthetic valves.<ref name="pmid18820172">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=Circulation | year= 2008 | volume= 118 | issue= 15 | pages= e523-661 | pmid=18820172 | doi=10.1161/CIRCULATIONAHA.108.190748 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18820172  }}</ref>
+*Infective endocarditis prophylaxis is recommended only in patients with prosthetic valves.
 ==Participation In Sports==


Pulmonary valve stenosis
Pulmonary valve stenosis
ICD-10	I37.0, I37.2, Q22.1
ICD-9	424.3, 746.02
OMIM	265500
DiseasesDB	11025
MedlinePlus	001096