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'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
{{Infobox_Disease |
{{Infobox_Disease |
   |Name          = Pulmonary atresia  
   |Name          = Pulmonary atresia  
  |Image          = Pulmonary-artery-atresia.jpg
  |Caption        = Only an aorta can be seen originating from this pathology specimen. No pulmonary artery is present.
   |DiseasesDB    =  
   |DiseasesDB    =  
   |ICD10          = {{ICD10|Q|25|5|q|20}}  
   |ICD10          = {{ICD10|Q|25|5|q|20}}  
Line 9: Line 8:
   |OMIM          =  
   |OMIM          =  
   |MedlinePlus    = 001091  
   |MedlinePlus    = 001091  
   |eMedicineSubj  = ped
   |eMedicineSubj  =  
   |eMedicineTopic = 2526
   |eMedicineTopic =
   |eMedicine_mult = {{eMedicine2|ped|2898}}
   |eMedicine_mult =  
   |MeshName      = Pulmonary+Atresia  
   |MeshName      = Pulmonary+Atresia  
   |MeshNumber    = C14.240.670  
   |MeshNumber    = C14.240.670  
}}
}}
{{SI}}
{{Pulmonary atresia}}
{{WikiDoc Cardiology Network Infobox}}
{{CMG}}; {{AE}} : {{mwaq}}


{{CMG}}
{{SK}}  


'''Associate Editor-in-Chief:''' Keri Shafer, M.D. [mailto:kshafer@bidmc.harvard.edu]
==[[Xyz overview|Overview]]==


{{Editor Join}}
==[[Xyz historical perspective|Historical Perspective]]==


==Overview==
==[[Xyz classification|Classification]]==
'''Pulmonary atresia''' is a [[congenital]] malformation of the [[pulmonary valve]] in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the [[heart]] to the [[lungs]].


==Pathophysiology==
==[[Xyz pathophysiology|Pathophysiology]]==
The [[pulmonary valve]] is located on the right side of the heart between the [[right ventricle]] and [[pulmonary artery]]. In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close like one way doors. As these flaps open and close they force blood to flow forward into the [[pulmonary artery]] and backward into the [[right ventricle]] then forward again to the lungs where the blood becomes oxygenated. With the disease pulmonary atresia, the flap-like openings are completely covered by a layer of tissue, thus preventing the ability of blood flow to the lungs to become oxygenated. The body requires oxygenated blood for survival. Pulmonary atresia is not threatening to a developing fetus however, because the mother's placenta provides the needed oxygen since the baby's lungs are not yet functional. Once the baby is born its lungs must now provide the oxygen needed for survival, but with Pulmonary atresia there is no opening on the [[pulmonary valve]] for blood to get to the lungs and become oxygenated. Due to this, the newborn baby is blue in color and pulmonary atresia can usually be diagnosed within hours or minutes after birth.


==Classification==
==[[Xyz causes|Causes]]==
There are two forms of pulmonary atresia. 
===Pulmonary atresia with intact ventricular septum (PA-IVS)===
PA-IVS is a rare [[congenital]] [[lesion]].  This lesion can be diagnosed during the fetal stage of life by using fetal echocardiography, which is a test that uses sound waves to look at the structure of an unborn baby's heart.  PA-IVS involves complete blockage of the [[pulmonary valve]] located on the right side of the heart.  This blockage thus prevents the flow of blood to the lungs.  Because of this lack of blood flowing through the right side of the heart, the structures on that side, such as the [[pulmonary valve]] and the [[tricuspid valve]], are abnormally small.  The genetic cause of PA-IVS is unknown.  It is rare, occurring in around 7.1-8.1 per every 100,000 live births.


===Pulmonary atresia with ventricular septal defect or VSD===
==[[Xyz differential diagnosis|Differentiating Xyz from other Diseases]]==
PA-VSD is identified by underdevelopment of the [[right ventricle]].  It is a second opening in the ventricular wall.  This opening provides a way out for blood in the [[right ventricle]].  When this second opening ([[VSD]]) does not exist, very little blood goes to the right ventricle, which is what causes it to be underdeveloped.  This defect can also be determined before birth.  Out of all congenital cardiac malformations, it is estimated that PA-VSD occurs in 2.5-3.4% of the cases.  The genetic cause of PA-VSD is not known; it is found however, that children whose parents have PA-VSD are at a higher risk for congenital heart lesions.


Images shown below is courtesy of Professor Peter Anderson DVM PhD and published with permission. [http://www.peir.net © PEIR, University of Alabama at Birmingham, Department of Pathology]
==[[Xyz epidemiology and demographics|Epidemiology and Demographics]]==


<div align="left">
==[[Xyz risk factors|Risk Factors]]==
<gallery heights="175" widths="175">
 
Image:Pulmonary atresia.jpg|Only an aorta can be seen originating from this pathology specimen. No pulmonary artery is present.
==[[Xyz screening|Screening]]==
</gallery>
 
</div>
==Diagnosis==
[[Xyz diagnostic study of choice|Diagnostic study of choice]] | [[Xyz history and symptoms|History and Symptoms]] | [[Xyz physical examination|Physical Examination]] | [[Xyz laboratory findings|Laboratory Findings]] | [[Xyz electrocardiogram|Electrocardiogram]] | [[Xyz x ray|X-Ray Findings]] | [[Xyz echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Xyz CT scan|CT-Scan Findings]] | [[Xyz MRI|MRI Findings]] | [[Xyz other imaging findings|Other Imaging Findings]] | [[Xyz other diagnostic studies|Other Diagnostic Studies]]


==Treatment==
==Treatment==
At first, the child will need to be admitted to the [[intensive care unit]] and possibly placed on oxygen and a [[ventilator]] that will help the child breathe.  Also, IV medications that assist the heart and lung function may be given.  An IV medication called prostaglandin E1 is used for treatment of pulmonary atresia.  This medication helps the blood circulate through the body, but it is extremely dangerous because it causes [[apnea]].  Another example of preliminary treatment is heart catheterization to evaluate the defect or defects of the heart; this procedure is much more invasive.  Ultimately, however, the patient will need to have a series of surgeries to improve the blood flow permanently.  The first surgery will likely be performed shortly after birth.  A [[shunt]] can be created between the aorta and the pulmonary artery to help increase blood flow to the lungs.  As the child grows, so does the heart and the shunt may need revised in order to meet the body's requirements.
[[Xyz medical therapy|Medical Therapy]] | [[Xyz interventions|Interventions]] | [[Xyz surgery|Surgery]] | [[Xyz primary prevention|Primary Prevention]] | [[Xyz secondary prevention|Secondary Prevention]] | [[Xyz cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Xyz future or investigational therapies|Future or Investigational Therapies]]
Children with pulmonary atresia will need to be seen regularly throughout their lifetime by a cardiologist to be sure that their heart is functioning properly.  Due to their increased risk of [[endocarditis]], children diagnosed with pulmonary atresia should have amoxicillin before any type of dental work or other types of surgeries to help prevent this infection from occurring.
 
==Case Studies==
[[Xyz case study one|Case #1]]
 
[[Category: (name of the system)]]


The type of surgery recommended depends on the size of the [[right ventricle]] and the [[pulmonary artery]].
==[[Pulmonary atresia epidemiology and demographics|Epidemiology and demographics]]==
If they are normal in size and the right ventricle is able to pump blood, [[open heart surgery]] can be performed to make blood flow through the heart in a normal pattern.  
* While there is no difference in the incidence of Pulmonary atresia in male or female, it is found that pulmonary atresia with VSD ( PA-VSD ) is slightly more prevalent in males than in females. <ref>https://www.uptodate.com/contents/pulmonary-atresia-with-intact-ventricular-septum-pa-ivs?search=pulmonary%20atresia&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H172915282][https://emedicine.medscape.com/article/905119-overview#a5</ref>
If the right ventricle is small and unable to act as a pump, doctors may perform another type of operation called the [[Fontan procedure]]. In this two-stage procedure, the [[right atrium]] is disconnected from the pulmonary circulation. The systemic venous return goes directly to the lungs, by-passing the heart.


==Prognosis==
1) The prevalence of Pulmonary Atresia with VSD is estimated to be around 0.07 per 1000 live breath. and 2.5-3.4 % among all congenital heart diseases.[https://emedicine.medscape.com/article/905119-overview#a5]
The outcome varies for every child. If the condition is left uncorrected it is fatal, but the prognosis has greatly improved over the years for babies with pulmonary atresia. Some factors that affect how well the child does include how well the heart is beating, the condition of the blood vessels that supply the heart, and how leaky the other heart valves are. Most cases of pulmonary atresia can be helped with surgery. If the patient's [[right ventricle]] is exceptionally small, many surgeries will be needed in order to help stimulate normal circulation of blood to the heart.


If uncorrected, babies with this type of [[congenital heart disease]] will only survive for the first few days of life while the fetal shunts between left and right circulations remain patent.
2) The overall incidence of PA-IVSD is under estimated as most of the fetus are spontaneously aborted due to the underlying other congenital malformations or are diagnosed on routine antenatal ultrasound and results in elective termination.  
Many children with Pulmonary Atresia will go on to lead 'normal' lives.


Doctors are unsure of the cause of congenital heart defects, but there are some medical conditions that have been found to increase the risk of having a baby with a heart defect such as congenital heart disease in the mother, father, brother, or sister of the baby, a diabetic mother, use of drugs or alcohol or over the counter prescriptions during pregnancy. These conditions do not mean that the baby will definitely be born with a heart defect, but that it runs a higher chance. Sometimes babies are born without any of these conditions; the cause is unknown. All pregnant women should do all that they can to ensure and maintain the health of their unborn child.
- The reported incidence is 6-8 per 100,000 live births and 1-3% of all congenital heart disease. [https://www.uptodate.com/contents/pulmonary-atresia-with-intact-ventricular-septum-pa-ivs?search=pulmonary%20atresia&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H172915282][https://emedicine.medscape.com/article/898167-overview#a6]
 
==[[Pulmonary atresia classifications|Classification]]==
 
==[[Pulmonary atresia natural history|Natural history, Complications, and Prognosis]]==
 
==[[Pulmonary atresia causes|Causes]]==
 
==Diagnosis==
[[Pulmonary atresia history and symptoms|History and Symptoms]] | [[Pulmonary atresia physical examination|Physical Examination]] | [[Pulmonary atresia laboratory tests|Laboratory Tests]] | [[Pulmonary atresia electrocardiogram | Electrocardiogram]] | [[Pulmonary atresia chest x ray|Chest X Ray]] | [[Pulmonary atresia MRI|MRI]] | [[Pulmonary atresia CT|CT]] | [[Pulmonary atresia echocardiography|Echocardiography]] | [[Pulmonary atresia other imaging findings|Other Imaging Findings]] | [[Pulmonary atresia other diagnostic studies|Other Diagnostic Studies]]
 
==Treatment==
==References==
{{reflist|2}}


==External links==
==External links==
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* [http://www.childrenshospital.org/az/Site509/mainpageS509P0.html from Children's Hospital Boston]
* [http://www.childrenshospital.org/az/Site509/mainpageS509P0.html from Children's Hospital Boston]
* [http://www.americanheart.org/presenter.jhtml?identifier=1303 from American Heart Association]
* [http://www.americanheart.org/presenter.jhtml?identifier=1303 from American Heart Association]
* [http://www.emedicine.com/ped/topic2898.htm] [http://www.emedicine.com/ped/topic2526.htm from eMedicine from WebMD]
* [http://www.ctsnet.org/file/paivs_LETTER_06-25-03.pdf from Congenital Heart Surgeons' Society]
* [http://www.ctsnet.org/file/paivs_LETTER_06-25-03.pdf from Congenital Heart Surgeons' Society]
* [http://www.nlm.nih.gov/medlineplus/ency/article/001091.htm from Medline Plus]
* [http://www.nlm.nih.gov/medlineplus/ency/article/001091.htm from Medline Plus]
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{{Congenital malformations and deformations of circulatory system}}
{{Congenital malformations and deformations of circulatory system}}
{{SIB}}
 
[[Category:Congenital heart disease]]
[[Category:Congenital heart disease]]
[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Pediatrics]]
[[Category:Mature chapter]]


[[fr:Sténose et atrésie pulmonaire]]
[[fr:Sténose et atrésie pulmonaire]]

Latest revision as of 21:08, 14 July 2020

For patient information click here

Pulmonary atresia
ICD-10 Q25.5
ICD-9 747.3
MedlinePlus 001091
MeSH C14.240.670

Pulmonary atresia Microchapters

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

Synonyms and keywords:

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Xyz from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Interventions | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Epidemiology and demographics

  • While there is no difference in the incidence of Pulmonary atresia in male or female, it is found that pulmonary atresia with VSD ( PA-VSD ) is slightly more prevalent in males than in females. [1]

1) The prevalence of Pulmonary Atresia with VSD is estimated to be around 0.07 per 1000 live breath. and 2.5-3.4 % among all congenital heart diseases.[3]

2) The overall incidence of PA-IVSD is under estimated as most of the fetus are spontaneously aborted due to the underlying other congenital malformations or are diagnosed on routine antenatal ultrasound and results in elective termination.

- The reported incidence is 6-8 per 100,000 live births and 1-3% of all congenital heart disease. [4][5]

Classification

Natural history, Complications, and Prognosis

Causes

Diagnosis

History and Symptoms | Physical Examination | Laboratory Tests | Electrocardiogram | Chest X Ray | MRI | CT | Echocardiography | Other Imaging Findings | Other Diagnostic Studies

Treatment

References

  1. ↑ https://www.uptodate.com/contents/pulmonary-atresia-with-intact-ventricular-septum-pa-ivs?search=pulmonary%20atresia&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H172915282][https://emedicine.medscape.com/article/905119-overview#a5

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