Sandbox: Pulmonary Valve regurgitation: Difference between revisions

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==Historical Perspective==
==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>
*Erasistratus, mentioned the involvement of the pulmonary valve in the unidirectional flow.<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>
*Galen described the membranes of the valves and named them as "semilunar".<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>
*Mondino de Luzzi designed the sketch of the pulmonary valves in the anatomical position for the first time.<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>
*Realdo Colombo described the pulmonary circulation for the first time.<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>


==Epidemiology and Demographics==
==Epidemiology and Demographics==
*The prevalence of mild pulmonary regurgutation is present in 40% to 78% of patients with normal pulmonary valve anatomy.<ref name="pmid2784023">{{cite journal| author=Choong CY, Abascal VM, Weyman J, Levine RA, Gentile F, Thomas JD et al.| title=Prevalence of valvular regurgitation by Doppler echocardiography in patients with structurally normal hearts by two-dimensional echocardiography. | journal=Am Heart J | year= 1989 | volume= 117 | issue= 3 | pages= 636-42 | pmid=2784023 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2784023  }} </ref><ref name="pmid3382565">{{cite journal| author=Takao S, Miyatake K, Izumi S, Okamoto M, Kinoshita N, Nakagawa H et al.| title=Clinical implications of pulmonary regurgitation in healthy individuals: detection by cross sectional pulsed Doppler echocardiography. | journal=Br Heart J | year= 1988 | volume= 59 | issue= 5 | pages= 542-50 | pmid=3382565 | doi= | pmc=1276894 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3382565  }} </ref>
*The prevalence of mild pulmonary regurgutation is present in 40% to 78% of patients with normal pulmonary valve anatomy.<ref name="pmid2784023">{{cite journal| author=Choong CY, Abascal VM, Weyman J, Levine RA, Gentile F, Thomas JD et al.| title=Prevalence of valvular regurgitation by Doppler echocardiography in patients with structurally normal hearts by two-dimensional echocardiography. | journal=Am Heart J | year= 1989 | volume= 117 | issue= 3 | pages= 636-42 | pmid=2784023 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2784023  }} </ref><ref name="pmid3382565">{{cite journal| author=Takao S, Miyatake K, Izumi S, Okamoto M, Kinoshita N, Nakagawa H et al.| title=Clinical implications of pulmonary regurgitation in healthy individuals: detection by cross sectional pulsed Doppler echocardiography. | journal=Br Heart J | year= 1988 | volume= 59 | issue= 5 | pages= 542-50 | pmid=3382565 | doi= | pmc=1276894 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3382565 }} </ref><ref name="pmid2310593">{{cite journal| author=Klein AL, Burstow DJ, Tajik AJ, Zachariah PK, Taliercio CP, Taylor CL et al.| title=Age-related prevalence of valvular regurgitation in normal subjects: a comprehensive color flow examination of 118 volunteers. | journal=J Am Soc Echocardiogr | year= 1990 | volume= 3 | issue= 1 | pages= 54-63 | pmid=2310593 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2310593 }} </ref>
*In patients born with congenital heart disease, 20% of patients have associated abnormalities of the pulmonary valve or the right ventricular outlet obstruction.
*In patients born with congenital heart disease, 20% of patients have associated abnormalities of the pulmonary valve or the right ventricular outlet obstruction.<ref name="pmid2784023">{{cite journal| author=Choong CY, Abascal VM, Weyman J, Levine RA, Gentile F, Thomas JD et al.| title=Prevalence of valvular regurgitation by Doppler echocardiography in patients with structurally normal hearts by two-dimensional echocardiography. | journal=Am Heart J | year= 1989 | volume= 117 | issue= 3 | pages= 636-42 | pmid=2784023 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2784023  }} </ref>
*There is increased prevalence of pulmonary regurgitation with increasing age.<ref name="pmid2784023">{{cite journal| author=Choong CY, Abascal VM, Weyman J, Levine RA, Gentile F, Thomas JD et al.| title=Prevalence of valvular regurgitation by Doppler echocardiography in patients with structurally normal hearts by two-dimensional echocardiography. | journal=Am Heart J | year= 1989 | volume= 117 | issue= 3 | pages= 636-42 | pmid=2784023 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2784023  }} </ref><ref name="pmid2310593">{{cite journal| author=Klein AL, Burstow DJ, Tajik AJ, Zachariah PK, Taliercio CP, Taylor CL et al.| title=Age-related prevalence of valvular regurgitation in normal subjects: a comprehensive color flow examination of 118 volunteers. | journal=J Am Soc Echocardiogr | year= 1990 | volume= 3 | issue= 1 | pages= 54-63 | pmid=2310593 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2310593  }} </ref>
*There is no racial or ethnic predilection for pulmonary regurgitation.


==Classification==
==Classification==
'''Based on the pulmonary Valve Morphology'''<br>
Pulmonary valve is classified into the following types based on the morphology of the valve and severity of the disease:
===Based on the Pulmonary Valve Morphology===
Pulmonary Regurgitation is classified into primary and secondary types based on the involvement of the pulmonary Valve.
Pulmonary Regurgitation is classified into primary and secondary types based on the involvement of the pulmonary Valve.
*Primary Pulmonary Regurgitation:The pulmonary valve morphology is affected. Isolated PR is very rare and is most commonly associated with other congenital heart diseases.  
*'''Primary Pulmonary Regurgitation''':The pulmonary valve morphology is affected. Isolated PR is very rare and is most commonly associated with other congenital heart diseases.<ref name="pmid17569817">{{cite journal| author=Chaturvedi RR, Redington AN| title=Pulmonary regurgitation in congenital heart disease. | journal=Heart | year= 2007 | volume= 93 | issue= 7 | pages= 880-9 | pmid=17569817 | doi=10.1136/hrt.2005.075234 | pmc=1994453 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17569817  }} </ref>
*Secondary or Functional Pulmonary Regurgitation: The pulmonary valve function is normal. Conditions such as pulmonary hypertension and PA aneurym cause dilation of the valve annulus leading to regurgitation.
*'''Secondary or Functional Pulmonary Regurgitation''': The pulmonary valve function is normal. Conditions such as pulmonary hypertension and PA aneursym cause dilation of the valve annulus leading to regurgitation.<ref name="pmid23922549">{{cite journal| author=Di Lullo L, Floccari F, Rivera R, Barbera V, Granata A, Otranto G et al.| title=Pulmonary Hypertension and Right Heart Failure in Chronic Kidney Disease: New Challenge for 21st-Century Cardionephrologists. | journal=Cardiorenal Med | year= 2013 | volume= 3 | issue= 2 | pages= 96-103 | pmid=23922549 | doi=10.1159/000350952 | pmc=3721135 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23922549  }} </ref>


'''Based on the Severity'''<br>
===Based on the Severity===
Pulmonary regurgitation is classified into three categories based on the severity of the regurgitant jet demonstrated on the 2D echo.
Pulmonary regurgitation is classified into three categories based on the severity of the regurgitant jet demonstrated on the 2D echo.<ref name="pmid20620859">{{cite journal| author=Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K et al.| title=Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. | journal=J Am Soc Echocardiogr | year= 2010 | volume= 23 | issue= 7 | pages= 685-713; quiz 786-8 | pmid=20620859 | doi=10.1016/j.echo.2010.05.010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20620859  }} </ref>
*'''Mild''': Normal valve morphology and usually asymptomatic
*'''Moderate''': Normal to abnormal valve morphology with mild symptoms
*'''Severe''': Abnormal valve morphology with significant symptomatology


==Natural History, Prognosis, Complications==
==Natural History, Prognosis, Complications==
'''Natural History'''
'''Natural History'''
*Mild PR is a very common finding on 2D echo.
*Mild PR is a very common finding on 2D echo.<ref name="pmid19038677">{{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). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 23 | pages= e143-263 | pmid=19038677 | doi=10.1016/j.jacc.2008.10.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19038677  }} </ref>
*Majority of patients with mild PR are asymptomatic and have a beningn course, not progressing to chronic PR.
*Majority of patients with mild PR are asymptomatic and have a beningn course, not progressing to chronic PR.
*Patients tolerate severe chronic PR for a long period of time and begin to develop symptoms when the right ventricle function begins to decline.
*Patients tolerate severe chronic PR for a long period of time and begin to develop symptoms when the right ventricle function begins to decline.
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*Patients with acute worsening of PR should be evaluated for  associated conditions such as pulmonary hypertension which increase the pressure gradient.
*Patients with acute worsening of PR should be evaluated for  associated conditions such as pulmonary hypertension which increase the pressure gradient.
'''Prognosis'''
'''Prognosis'''
*Symptomatic patients are treated with pulmonary valve replacement and have a good prognosis.
*Symptomatic patients are treated with pulmonary valve replacement and have a good prognosis.<ref name="pmid22921969">{{cite journal| author=Lee C, Kim YM, Lee CH, Kwak JG, Park CS, Song JY et al.| title=Outcomes of pulmonary valve replacement in 170 patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction: implications for optimal timing of pulmonary valve replacement. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 11 | pages= 1005-14 | pmid=22921969 | doi=10.1016/j.jacc.2012.03.077 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22921969  }} </ref>
'''Complications'''
'''Complications'''
*Progressive right ventricular dilation increases the risk of ventricular arrhythmias and sudden cardiac death. Patients with tetralogy of Fallot are at increased risk of developing these complications compared to patients with isolated PR.
*Progressive right ventricular dilation increases the risk of ventricular arrhythmias and sudden cardiac death. Patients with tetralogy of Fallot are at increased risk of developing these complications compared to patients with isolated PR.<ref name="pmid17601398">{{cite journal| author=Gregg D, Foster E| title=Pulmonary insufficiency is the nexus of late complications in tetralogy of Fallot. | journal=Curr Cardiol Rep | year= 2007 | volume= 9 | issue= 4 | pages= 315-22 | pmid=17601398 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17601398  }} </ref><ref name="pmid12381647">{{cite journal| author=Helbing WA, Roest AA, Niezen RA, Vliegen HW, Hazekamp MG, Ottenkamp J et al.| title=ECG predictors of ventricular arrhythmias and biventricular size and wall mass in tetralogy of Fallot with pulmonary regurgitation. | journal=Heart | year= 2002 | volume= 88 | issue= 5 | pages= 515-9 | pmid=12381647 | doi= | pmc=1767425 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12381647  }} </ref>


==Pathophysiology==
==Pathophysiology==
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**Afterload of the RV
**Afterload of the RV
**RV diastolic complaince
**RV diastolic complaince
**Duration of RV diastole According to 2014, ACC/AHA valvular heart disease guidelines the stages of severe pulmonary regurgitation are described as follows:
**Duration of RV diastole
 
==== According to 2014, ACC/AHA valvular heart disease guidelines the stages of severe pulmonary regurgitation are described as follows:<ref name="pmid24603191">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| title=2014 AHA/ACC guideline 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. | journal=J Am Coll Cardiol | year= 2014 | volume= 63 | issue= 22 | pages= e57-185 | pmid=24603191 | doi=10.1016/j.jacc.2014.02.536 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24603191  }} </ref> ====
{| class="wikitable"
{| class="wikitable"
!Stage
!Stage
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*Following balloon dilation of critical pulmonary stenosis
*Following balloon dilation of critical pulmonary stenosis
*Perforation of valvar pulmonary atresia
*Perforation of valvar pulmonary atresia
|}
==Differentiating Pulmonary Regurgitation From Other Diseases==
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
|+
! style="background: #4479BA; width: 120px;" | {{fontcolor|#FFF|Disease}}
! style="background: #4479BA; width: 550px;" | {{fontcolor|#FFF|Findings}}
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Pulmonary Valve Regurgitation]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*PR is best heard over the left second and third interspaces and increases with inspiration
*Usually secondary to repair of tetralogy of Fallot or pulmonic Valve stenosis.
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Aortic Regurgitation]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Patients present with dyspnea and fatigability as a consequence of reduced cardiac reserve in the fourth or fifth decade<ref name="GoldschlagerPfeifer1973">{{citejournal|last1=Goldschlager|first1=Nora|last2=Pfeifer|first2=James|last3=Cohn|first3=Keith|last4=Popper|first4=Robert|last5=Selzer|first5=Arthur|title=The natural history of aortic regurgitation|journal=The American Journal of Medicine|volume=54|issue=5|year=1973|pages=577–588|issn=00029343|doi=10.1016/0002-9343(73)90115-0}}</ref>
*AR is heard over left sternal border or over the right second interspace and radiates to the neck
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Tricuspid Regurgitation causing RV enlargement]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Pansystolic murmur accentuating with inspiration<ref name="SepulvedaLukas1955">{{cite journal|last1=Sepulveda|first1=G.|last2=Lukas|first2=D. S.|title=The Diagnosis of Tricuspid Insufficiency: Clinical Features in 60 Cases with Associated Mitral Valve Disease|journal=Circulation|volume=11|issue=4|year=1955|pages=552–563|issn=0009-7322|doi=10.1161/01.CIR.11.4.552}}</ref>
*RV heave
*Gaint "V" wave seen on JVP examination
*Hepatomegaly is seen in 90% of patients
*Quantification of severity of TR is done by colour flow doppler imaging<ref name="Zoghbi2003">{{cite journal|last1=Zoghbi|first1=W|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|volume=16|issue=7|year=2003|pages=777–802|issn=08947317|doi=10.1016/S0894-7317(03)00335-3}}</ref>
|-
| style="padding: 5px 5px; background: #DCDCDC;" |'''Left to Right Shunt causing RV enlargement'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Usually seen in children with acyanotic congenital disease such as ASD
*Fixed splitting of S2 is present
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Arrthmogenic Right Ventricular Cardiomyopathy]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Gradual replacement of normal functional myocardium with adipose or fibroadipose tissue<ref name="pmid27828830">{{cite journal| author=Graziosi M, Rapezzi C| title=Right ventricular arrhythmogenic cardiomyopathy: genetic and MR for modern clinical diagnosis. | journal=J Cardiovasc Med (Hagerstown) | year= 2016 | volume=  | issue=  | pages=  | pmid=27828830 | doi=10.2459/JCM.0000000000000470 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27828830  }} </ref>
*Age of onset is 7 to 40years
*Patients are usually asymptomatic, present with occasional palpitations
*EKG shows negative "T" waves and epsilon waves with selective "S" wave delay in V1 to V3
*RV is dilated and hypokinetic on echocardiography
*Holter is the diagnostic test to diagnose hyperkinetic ventricular arrythmias
|-
|}
|}


==Diagnosis==
==Diagnosis==
===History and Symptoms===
===History and Symptoms===
Clinical presentation of pulmonary regurgitation varies on the severity of the regurgitation and the right ventricular function.
Clinical presentation of pulmonary regurgitation varies on the severity of the regurgitation and the right ventricular function.<ref name="pmid19038677">{{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). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 23 | pages= e143-263 | pmid=19038677 | doi=10.1016/j.jacc.2008.10.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19038677  }} </ref><ref name="pmid6207619">{{cite journal| author=Shimazaki Y, Blackstone EH, Kirklin JW| title=The natural history of isolated congenital pulmonary valve incompetence: surgical implications. | journal=Thorac Cardiovasc Surg | year= 1984 | volume= 32 | issue= 4 | pages= 257-9 | pmid=6207619 | doi=10.1055/s-2007-1023399 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6207619  }} </ref>
*Isolated pulmonary regurgitation is usually asymptomatic and is an incidental finding on 2D echo even when the regurgitation is severe.
*Isolated pulmonary regurgitation is usually asymptomatic and is an incidental finding on 2D echo even when the regurgitation is severe.
*Patients with chronic PR develop right heart failure and present with the following symptoms:
*Patients with chronic PR develop right heart failure and present with the following symptoms:
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====Neck====
====Neck====
* Increased [[JVP]]
* Increased [[JVP]]
* Prominent "[[a wave]]" may be present
* Prominent "[[a wave]]" may be present.
* Prominent "[[v wave]]" may be present in presence of [[tricuspid regurgitation]]
* Prominent "[[v wave]]" may be present in presence of [[tricuspid regurgitation]].
* A palpable impulse (lift or heave) is usually present at the left lower sternal border because of right ventricular dilation.
* A palpable impulse (lift or heave) is usually present at the left lower sternal border because of right ventricular dilation.
====Auscultation====
====Auscultation====
* Pulmonic regurgitation is associated with wide splitting of S<sub>2</sub>.
* Pulmonic regurgitation is associated with wide splitting of S<sub>2</sub>.
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* A right-sided S<sub>3</sub> may be audible and may also be accentuated with inspiration.
* A right-sided S<sub>3</sub> may be audible and may also be accentuated with inspiration.
* Likewise, a right-sided [[S4]] may also be audible and accentuated with inspiration.
* Likewise, a right-sided [[S4]] may also be audible and accentuated with inspiration.
* Murmur of residual pulmonic regurgitation after [[Tetralogy of Fallot]] repair:
* Murmur of residual pulmonic regurgitation after [[Tetralogy of Fallot]] repair:<ref name="BousvarosDeuchar19612">{{cite journal|last1=Bousvaros|first1=GeorgeA.|last2=Deuchar|first2=DennisC.|title=THE MURMUR OF PULMONARY REGURGITATION WHICH IS NOT ASSOCIATED WITH PULMONARY HYPERTENSION|journal=The Lancet|volume=278|issue=7209|year=1961|pages=962–964|issn=01406736|doi=10.1016/S0140-6736(61)90798-X}}</ref>
** It is a low-pitched and soft murmur.
** It is a low-pitched and soft murmur.
** Best heard along the second or third intercostal spaces adjacent to the left sternal border.
** Best heard along the second or third intercostal spaces adjacent to the left sternal border.
** It is accentuated by squatting and inspiration.
** It is accentuated by squatting and inspiration.
** It is made softer by Valsalva maneuvers or expiration.
** It is made softer by Valsalva maneuvers or expiration.
* Murmur of pulmonic regurgitation associated with [[pulmonary hypertension|Pulmonic hypertension]]:
* Murmur of pulmonic regurgitation associated with [[pulmonary hypertension|Pulmonic hypertension]]:<ref name="pmid2662241">{{cite journal| author=Würtemberger G, Dinkel E, Joos A, Matthys H| title=[Pulmonary hypertension. Clinical picture and therapy]. | journal=Radiologe | year= 1989 | volume= 29 | issue= 6 | pages= 263-6 | pmid=2662241 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2662241  }} </ref>
** When the pulmonary artery systolic pressure exceeds 60 mm Hg, dilatation of the pulmonary artery ring may then result in Graham-Steell's murmur.
** When the pulmonary artery systolic pressure exceeds 60 mm Hg, dilatation of the pulmonary artery ring may then result in Graham-Steell's murmur.
** It is a high-pitched, "blowing", early diastolic decrescendo murmur like that of [[aortic regurgitation]].
** It is a high-pitched, "blowing", early diastolic decrescendo murmur like that of [[aortic regurgitation]].
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==== Vena Contracta Width ====
==== Vena Contracta Width ====
It is a more accurate method to assess the severity of PR, but it lacks validation studies.  
It is a more accurate method to assess the severity of PR, but it lacks validation studies.<ref name="pmid206208592">{{cite journal| author=Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K et al.| title=Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. | journal=J Am Soc Echocardiogr | year= 2010 | volume= 23 | issue= 7 | pages= 685-713; quiz 786-8 | pmid=20620859 | doi=10.1016/j.echo.2010.05.010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20620859  }}</ref>


==== Flow convergence method ====
==== Flow convergence method ====
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==== '''Exercise Echocardiography''' ====
==== '''Exercise Echocardiography''' ====
It is used to unmask latent RV dysfunction and is a helpful investigation to assess the RV function in patients who have underwent an intervention for significant PR.
It is used to unmask latent RV dysfunction and is a helpful investigation to assess the RV function in patients who have underwent an intervention for significant PR.
==== Determination of severity of PR based on the findings on echocardiography: ====
==== Determination of severity of PR based on the findings on echocardiography:<ref name="pmid206208593">{{cite journal| author=Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K et al.| title=Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. | journal=J Am Soc Echocardiogr | year= 2010 | volume= 23 | issue= 7 | pages= 685-713; quiz 786-8 | pmid=20620859 | doi=10.1016/j.echo.2010.05.010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20620859  }}</ref> ====
{| class="wikitable"
{| class="wikitable"
!Parameters
!Parameters
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|Greatly increased
|Greatly increased
|}
|}
===EKG===
===EKG===
*EKG findings in chronic PR are non specific.
*EKG findings in chronic PR are non specific.
*In patients with tetralogy of Fallot increased QRS duration with widened QRS complex reflects the severity of PR and right ventricular dilation predisposing the patients to develop malignant arrythmias. <ref name="pmid7600655">{{cite journal| author=Gatzoulis MA, Till JA, Somerville J, Redington AN| title=Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. | journal=Circulation | year= 1995 | volume= 92 | issue= 2 | pages= 231-7 | pmid=7600655 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7600655  }}</ref>
*In patients with tetralogy of Fallot increased QRS duration with widened QRS complex reflects the severity of PR and right ventricular dilation predisposing the patients to develop malignant arrythmias. <ref name="pmid7600655">{{cite journal| author=Gatzoulis MA, Till JA, Somerville J, Redington AN| title=Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. | journal=Circulation | year= 1995 | volume= 92 | issue= 2 | pages= 231-7 | pmid=7600655 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7600655 }}</ref><ref name="pmid10995413">{{cite journal| author=Abd El Rahman MY, Abdul-Khaliq H, Vogel M, Alexi-Meskishvili V, Gutberlet M, Lange PE| title=Relation between right ventricular enlargement, QRS duration, and right ventricular function in patients with tetralogy of Fallot and pulmonary regurgitation after surgical repair. | journal=Heart | year= 2000 | volume= 84 | issue= 4 | pages= 416-20 | pmid=10995413 | doi= | pmc=1729453 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10995413 }} </ref>
*In patients with RV volume overload and isolated PR, QRS prolongation with rSr morphology can be seen in right precordial leads.
*In patients with RV volume overload and isolated PR, QRS prolongation with rSr morphology can be seen in right precordial leads.
*RBB is common in majority of patients who have tetralogy of Fallot repair with right ventriculotomy.
*RBB is common in majority of patients who have tetralogy of Fallot repair with right ventriculotomy.
===Chest X-Ray===
===Chest X-Ray===
Chest X-Ray in chronic PR the following findings can be demonstrated:
Chest X-Ray in chronic PR the following findings can be demonstrated:
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*CMR is the diagnostic modality preffered to determine the requirement of reintervention in patients with repaired tetralogy of Fallot and to assess the ventricular function and dimensions.
*CMR is the diagnostic modality preffered to determine the requirement of reintervention in patients with repaired tetralogy of Fallot and to assess the ventricular function and dimensions.
===Cardiac Catheterization===
===Cardiac Catheterization===
Echo and CMR the investigations of choice in patients to detect and diagnose the severity of PR. Cardiac Catheterization is not a recommended for diagnosis of PR. In patients with poor echocardiographic windows and with pacemakers, CMR cannot be used, catheterization in such patients is a good alternative.
Echo and CMR the investigations of choice in patients to detect and diagnose the severity of PR. Cardiac Catheterization is not a recommended for diagnosis of PR. In patients with poor echocardiographic windows and with pacemakers, CMR cannot be used, catheterization in such patients is a good alternative.<ref name="pmid26104675">{{cite journal| author=Rommel JJ, Yadav PK, Stouffer GA| title=Causes and hemodynamic findings in chronic severe pulmonary regurgitation. | journal=Catheter Cardiovasc Interv | year= 2015 | volume=  | issue=  | pages=  | pmid=26104675 | doi=10.1002/ccd.26073 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26104675  }} </ref><br>
Findings of significant PR include:
*Low pulmonary artery end-diastolic pressure, resulting in wide pulse pressure
*Increased right ventricular end-diastolic pressure
*Equalization of RV and PA diastolic pressures


==Treatment==
==Treatment==
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*There are no specific medical measures for management of PR.
*There are no specific medical measures for management of PR.
*Diuretics are recommended in patients with RV dysfunction for maintenance of fluid balance.
*Diuretics are recommended in patients with RV dysfunction for maintenance of fluid balance.
*In patients with repaired tetralogy of Fallot, ACE inhibitors or beta-blockers are used to reverse the neuroharmonal activation and improve the symptoms.
*In patients with repaired tetralogy of Fallot, ACE inhibitors or beta-blockers are used to reverse the neuroharmonal activation and improve the symptoms.<ref name="pmid12093776">{{cite journal| author=Bolger AP, Sharma R, Li W, Leenarts M, Kalra PR, Kemp M et al.| title=Neurohormonal activation and the chronic heart failure syndrome in adults with congenital heart disease. | journal=Circulation | year= 2002 | volume= 106 | issue= 1 | pages= 92-9 | pmid=12093776 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12093776  }} </ref><ref name="pmid12354712">{{cite journal| author=Davos CH, Davlouros PA, Wensel R, Francis D, Davies LC, Kilner PJ et al.| title=Global impairment of cardiac autonomic nervous activity late after repair of tetralogy of Fallot. | journal=Circulation | year= 2002 | volume= 106 | issue= 12 Suppl 1 | pages= I69-75 | pmid=12354712 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12354712  }} </ref>
 
===Surgical Therapy===
===Surgical Therapy===
====Indications for Surgery====
====Indications for Surgery====
Indications for Pulmonary Valve Replacement include:  
Indications for Pulmonary Valve Replacement include:<ref name="pmid16638542">{{cite journal| author=Geva T| title=Indications and timing of pulmonary valve replacement after tetralogy of Fallot repair. | journal=Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu | year= 2006 | volume=  | issue=  | pages= 11-22 | pmid=16638542 | doi=10.1053/j.pcsu.2006.02.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16638542  }}</ref>
*Symptomatic patients with arrythmias or NYHA class higher than II
*Symptomatic patients with arrythmias or NYHA class higher than II
*Ejection Fraction of less than 40% when assessed with CMR
*Ejection Fraction of less than 40% when assessed with CMR
Line 228: Line 289:
*Timing of pulmonary valve replacement is not well defined as in aortic and mitral regurgitation. However timely intervention is advised before the onset of RV dysfunction.<ref name="pmid15757612">{{cite journal| author=Therrien J, Provost Y, Merchant N, Williams W, Colman J, Webb G| title=Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair. | journal=Am J Cardiol | year= 2005 | volume= 95 | issue= 6 | pages= 779-82 | pmid=15757612 | doi=10.1016/j.amjcard.2004.11.037 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15757612  }} </ref>
*Timing of pulmonary valve replacement is not well defined as in aortic and mitral regurgitation. However timely intervention is advised before the onset of RV dysfunction.<ref name="pmid15757612">{{cite journal| author=Therrien J, Provost Y, Merchant N, Williams W, Colman J, Webb G| title=Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair. | journal=Am J Cardiol | year= 2005 | volume= 95 | issue= 6 | pages= 779-82 | pmid=15757612 | doi=10.1016/j.amjcard.2004.11.037 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15757612  }} </ref>
*Delayed intervention has shown to have poor outcomes and higher rate of re-intervention.
*Delayed intervention has shown to have poor outcomes and higher rate of re-intervention.
*The prime goals of pulmonary valve replacement include improved functional class and quality of life, maintenance of right (and left) ventricular function, risk modification of arrhythmia and sudden cardiac death.
*The prime goals of pulmonary valve replacement include improved functional class and quality of life, maintenance of right (and left) ventricular function, risk modification of arrhythmia and sudden cardiac death.<ref name="pmid11174741">{{cite journal| author=Discigil B, Dearani JA, Puga FJ, Schaff HV, Hagler DJ, Warnes CA et al.| title=Late pulmonary valve replacement after repair of tetralogy of Fallot. | journal=J Thorac Cardiovasc Surg | year= 2001 | volume= 121 | issue= 2 | pages= 344-51 | pmid=11174741 | doi=10.1067/mtc.2001.111209 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11174741  }} </ref>


====Surgical Options====
====Surgical Options====
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====Surgical Valve Implantation====
====Surgical Valve Implantation====
*Various valved conduits are placed to replace the pulmonic valve which include Homografts from cadavers, valved conduits, and the Contegra bovine jugular vein graft or a bioprosthetic valve implanted directly in the RV outflow tract.<ref name="pmid11082375">{{cite journal| author=Tweddell JS, Pelech AN, Frommelt PC, Mussatto KA, Wyman JD, Fedderly RT et al.| title=Factors affecting longevity of homograft valves used in right ventricular outflow tract reconstruction for congenital heart disease. | journal=Circulation | year= 2000 | volume= 102 | issue= 19 Suppl 3 | pages= III130-5 | pmid=11082375 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11082375  }} </ref>
*Various valved conduits are placed to replace the pulmonic valve which include Homografts from cadavers, valved conduits, and the Contegra bovine jugular vein graft or a bioprosthetic valve implanted directly in the RV outflow tract.<ref name="pmid11082375">{{cite journal| author=Tweddell JS, Pelech AN, Frommelt PC, Mussatto KA, Wyman JD, Fedderly RT et al.| title=Factors affecting longevity of homograft valves used in right ventricular outflow tract reconstruction for congenital heart disease. | journal=Circulation | year= 2000 | volume= 102 | issue= 19 Suppl 3 | pages= III130-5 | pmid=11082375 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11082375  }} </ref>
*Bioprosthetic valves are usually preffered over mechanical valve prosthesis and have a longevity of around 15years.<ref name="pmid22921969">{{cite journal| author=Lee C, Kim YM, Lee CH, Kwak JG, Park CS, Song JY et al.| title=Outcomes of pulmonary valve replacement in 170 patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction: implications for optimal timing of pulmonary valve replacement. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 11 | pages= 1005-14 | pmid=22921969 | doi=10.1016/j.jacc.2012.03.077 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22921969  }} </ref><ref name="pmid22561653">{{cite journal| author=Jang W, Kim YJ, Choi K, Lim HG, Kim WH, Lee JR| title=Mid-term results of bioprosthetic pulmonary valve replacement in pulmonary regurgitation after tetralogy of Fallot repair. | journal=Eur J Cardiothorac Surg | year= 2012 | volume= 42 | issue= 1 | pages= e1-8 | pmid=22561653 | doi=10.1093/ejcts/ezs219 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22561653  }} </ref>
*Bioprosthetic valves are usually preffered over mechanical valve prosthesis and have a longevity of around 15years.<ref name="pmid22921969">{{cite journal| author=Lee C, Kim YM, Lee CH, Kwak JG, Park CS, Song JY et al.| title=Outcomes of pulmonary valve replacement in 170 patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction: implications for optimal timing of pulmonary valve replacement. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 11 | pages= 1005-14 | pmid=22921969 | doi=10.1016/j.jacc.2012.03.077 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22921969  }} </ref><ref name="pmid22561653">{{cite journal| author=Jang W, Kim YJ, Choi K, Lim HG, Kim WH, Lee JR| title=Mid-term results of bioprosthetic pulmonary valve replacement in pulmonary regurgitation after tetralogy of Fallot repair. | journal=Eur J Cardiothorac Surg | year= 2012 | volume= 42 | issue= 1 | pages= e1-8 | pmid=22561653 | doi=10.1093/ejcts/ezs219 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22561653 }} </ref><ref name="pmid21444054">{{cite journal| author=Burchill LJ, Wald RM, Harris L, Colman JM, Silversides CK| title=Pulmonary valve replacement in adults with repaired tetralogy of Fallot. | journal=Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu | year= 2011 | volume= 14 | issue= 1 | pages= 92-7 | pmid=21444054 | doi=10.1053/j.pcsu.2011.01.016 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21444054  }} </ref><ref name="pmid19764864">{{cite journal| author=Oosterhof T, Hazekamp MG, Mulder BJ| title=Opportunities in pulmonary valve replacement. | journal=Expert Rev Cardiovasc Ther | year= 2009 | volume= 7 | issue= 9 | pages= 1117-22 | pmid=19764864 | doi=10.1586/erc.09.89 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19764864 }} </ref>
*Mechanical valves are preffered in patients who are at high risk of reoperation such as patients with RV dysfunction.
*Mechanical valves are preffered in patients who are at high risk of reoperation such as patients with RV dysfunction.<ref name="pmid16730181">{{cite journal| author=Waterbolk TW, Hoendermis ES, den Hamer IJ, Ebels T| title=Pulmonary valve replacement with a mechanical prosthesis. Promising results of 28 procedures in patients with congenital heart disease. | journal=Eur J Cardiothorac Surg | year= 2006 | volume= 30 | issue= 1 | pages= 28-32 | pmid=16730181 | doi=10.1016/j.ejcts.2006.02.069 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16730181  }} </ref>
*Stenosis of the conduit is the major limitation and 25% of patients have to undergo a repeat intervention.
*Stenosis of the conduit is the major limitation and 25% of patients have to undergo a repeat intervention.
=====Transcatheter Pulmonary Valve Replacement=====
=====Transcatheter Pulmonary Valve Replacement=====
*The Melody transcatheter pulmonary valve(Medtronic) is approved by FDA in 2010.<ref name="pmid20644013">{{cite journal| author=McElhinney DB, Hellenbrand WE, Zahn EM, Jones TK, Cheatham JP, Lock JE et al.| title=Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. | journal=Circulation | year= 2010 | volume= 122 | issue= 5 | pages= 507-16 | pmid=20644013 | doi=10.1161/CIRCULATIONAHA.109.921692 | pmc=4240270 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20644013  }} </ref>
*The Melody transcatheter pulmonary valve(Medtronic) is approved by FDA in 2010.<ref name="pmid20644013">{{cite journal| author=McElhinney DB, Hellenbrand WE, Zahn EM, Jones TK, Cheatham JP, Lock JE et al.| title=Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. | journal=Circulation | year= 2010 | volume= 122 | issue= 5 | pages= 507-16 | pmid=20644013 | doi=10.1161/CIRCULATIONAHA.109.921692 | pmc=4240270 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20644013  }} </ref>
*The current transcatheter valves are designed to treat conduit and bioprosthetic valve failure only.<ref name="pmid19850214">{{cite journal| author=Zahn EM, Hellenbrand WE, Lock JE, McElhinney DB| title=Implantation of the melody transcatheter pulmonary valve in patients with a dysfunctional right ventricular outflow tract conduit early results from the u.s. Clinical trial. | journal=J Am Coll Cardiol | year= 2009 | volume= 54 | issue= 18 | pages= 1722-9 | pmid=19850214 | doi=10.1016/j.jacc.2009.06.034 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19850214  }} </ref><ref name="pmid16103239">{{cite journal| author=Khambadkone S, Coats L, Taylor A, Boudjemline Y, Derrick G, Tsang V et al.| title=Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. | journal=Circulation | year= 2005 | volume= 112 | issue= 8 | pages= 1189-97 | pmid=16103239 | doi=10.1161/CIRCULATIONAHA.104.523266 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16103239  }} </ref><ref name="pmid19540390">{{cite journal| author=Romeih S, Kroft LJ, Bokenkamp R, Schalij MJ, Grotenhuis H, Hazekamp MG et al.| title=Delayed improvement of right ventricular diastolic function and regression of right ventricular mass after percutaneous pulmonary valve implantation in patients with congenital heart disease. | journal=Am Heart J | year= 2009 | volume= 158 | issue= 1 | pages= 40-6 | pmid=19540390 | doi=10.1016/j.ahj.2009.04.023 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19540390  }} </ref><ref name="pmid20398873">{{cite journal| author=Vezmar M, Chaturvedi R, Lee KJ, Almeida C, Manlhiot C, McCrindle BW et al.| title=Percutaneous pulmonary valve implantation in the young 2-year follow-up. | journal=JACC Cardiovasc Interv | year= 2010 | volume= 3 | issue= 4 | pages= 439-48 | pmid=20398873 | doi=10.1016/j.jcin.2010.02.003 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20398873 }} </ref><ref name="pmid19850214">{{cite journal| author=Zahn EM, Hellenbrand WE, Lock JE, McElhinney DB| title=Implantation of the melody transcatheter pulmonary valve in patients with a dysfunctional right ventricular outflow tract conduit early results from the u.s. Clinical trial. | journal=J Am Coll Cardiol | year= 2009 | volume= 54 | issue= 18 | pages= 1722-9 | pmid=19850214 | doi=10.1016/j.jacc.2009.06.034 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19850214 }} </ref>
*The current transcatheter valves are designed to treat conduit and bioprosthetic valve failure only.<ref name="pmid19850214">{{cite journal| author=Zahn EM, Hellenbrand WE, Lock JE, McElhinney DB| title=Implantation of the melody transcatheter pulmonary valve in patients with a dysfunctional right ventricular outflow tract conduit early results from the u.s. Clinical trial. | journal=J Am Coll Cardiol | year= 2009 | volume= 54 | issue= 18 | pages= 1722-9 | pmid=19850214 | doi=10.1016/j.jacc.2009.06.034 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19850214  }} </ref><ref name="pmid16103239">{{cite journal| author=Khambadkone S, Coats L, Taylor A, Boudjemline Y, Derrick G, Tsang V et al.| title=Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. | journal=Circulation | year= 2005 | volume= 112 | issue= 8 | pages= 1189-97 | pmid=16103239 | doi=10.1161/CIRCULATIONAHA.104.523266 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16103239  }} </ref><ref name="pmid19540390">{{cite journal| author=Romeih S, Kroft LJ, Bokenkamp R, Schalij MJ, Grotenhuis H, Hazekamp MG et al.| title=Delayed improvement of right ventricular diastolic function and regression of right ventricular mass after percutaneous pulmonary valve implantation in patients with congenital heart disease. | journal=Am Heart J | year= 2009 | volume= 158 | issue= 1 | pages= 40-6 | pmid=19540390 | doi=10.1016/j.ahj.2009.04.023 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19540390  }} </ref><ref name="pmid20398873">{{cite journal| author=Vezmar M, Chaturvedi R, Lee KJ, Almeida C, Manlhiot C, McCrindle BW et al.| title=Percutaneous pulmonary valve implantation in the young 2-year follow-up. | journal=JACC Cardiovasc Interv | year= 2010 | volume= 3 | issue= 4 | pages= 439-48 | pmid=20398873 | doi=10.1016/j.jcin.2010.02.003 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20398873  }} </ref>
*They are not useful to treat patients who had a RVOT reconstruction by transannular patching.
*They are not useful to treat patients who had a RVOT reconstruction by transannular patching.
======Complications======
======Complications======
*Stent fracture:It leads to an increase in RV outflow tract gradient and RV pressure and its incidence is around 21% in 1 series that used the Melody valve and was the major reason for a repeat intervention.<ref name="pmid17339542">{{cite journal| author=Nordmeyer J, Khambadkone S, Coats L, Schievano S, Lurz P, Parenzan G et al.| title=Risk stratification, systematic classification, and anticipatory management strategies for stent fracture after percutaneous pulmonary valve implantation. | journal=Circulation | year= 2007 | volume= 115 | issue= 11 | pages= 1392-7 | pmid=17339542 | doi=10.1161/CIRCULATIONAHA.106.674259 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17339542  }} </ref><ref name="pmid18391109">{{cite journal| author=Lurz P, Coats L, Khambadkone S, Nordmeyer J, Boudjemline Y, Schievano S et al.| title=Percutaneous pulmonary valve implantation: impact of evolving technology and learning curve on clinical outcome. | journal=Circulation | year= 2008 | volume= 117 | issue= 15 | pages= 1964-72 | pmid=18391109 | doi=10.1161/CIRCULATIONAHA.107.735779 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18391109  }} </ref>
*Stent fracture:It leads to an increase in RV outflow tract gradient and RV pressure and its incidence is around 21% in 1 series that used the Melody valve and was the major reason for a repeat intervention.<ref name="pmid17339542">{{cite journal| author=Nordmeyer J, Khambadkone S, Coats L, Schievano S, Lurz P, Parenzan G et al.| title=Risk stratification, systematic classification, and anticipatory management strategies for stent fracture after percutaneous pulmonary valve implantation. | journal=Circulation | year= 2007 | volume= 115 | issue= 11 | pages= 1392-7 | pmid=17339542 | doi=10.1161/CIRCULATIONAHA.106.674259 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17339542  }} </ref><ref name="pmid18391109">{{cite journal| author=Lurz P, Coats L, Khambadkone S, Nordmeyer J, Boudjemline Y, Schievano S et al.| title=Percutaneous pulmonary valve implantation: impact of evolving technology and learning curve on clinical outcome. | journal=Circulation | year= 2008 | volume= 117 | issue= 15 | pages= 1964-72 | pmid=18391109 | doi=10.1161/CIRCULATIONAHA.107.735779 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18391109  }} </ref>
*Device instability and dislodgement
*Device instability and dislodgement<ref name="pmid18255307">{{cite journal| author=Kostolny M, Tsang V, Nordmeyer J, Van Doorn C, Frigiola A, Khambadkone S et al.| title=Rescue surgery following percutaneous pulmonary valve implantation. | journal=Eur J Cardiothorac Surg | year= 2008 | volume= 33 | issue= 4 | pages= 607-12 | pmid=18255307 | doi=10.1016/j.ejcts.2007.12.034 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18255307  }} </ref>
*Coronary compression due to stent placement
*Coronary compression due to stent placement<ref name="pmid21536996">{{cite journal| author=Feltes TF, Bacha E, Beekman RH, Cheatham JP, Feinstein JA, Gomes AS et al.| title=Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. | journal=Circulation | year= 2011 | volume= 123 | issue= 22 | pages= 2607-52 | pmid=21536996 | doi=10.1161/CIR.0b013e31821b1f10 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21536996  }}</ref>
*Pulmonary Artery obstruction
*Pulmonary Artery obstruction
====Outcomes====
====Outcomes====
*Patients with percutaneous pulmonary valve replacement have good outcome and are free of reintervention at 1year.
*Patients with percutaneous pulmonary valve replacement have good outcome and are free of reintervention at 1year.<ref name="pmid22958883">{{cite journal| author=Boudjemline Y, Brugada G, Van-Aerschot I, Patel M, Basquin A, Bonnet C et al.| title=Outcomes and safety of transcatheter pulmonary valve replacement in patients with large patched right ventricular outflow tracts. | journal=Arch Cardiovasc Dis | year= 2012 | volume= 105 | issue= 8-9 | pages= 404-13 | pmid=22958883 | doi=10.1016/j.acvd.2012.05.002 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22958883  }} </ref>
*Patients CMR derived pre operative right ventricular end diastolic volume index of less than 160ml/m²  and end systolic volume index of less than 80ml/m² showed better outcomes. <ref name="pmid22921969">{{cite journal| author=Lee C, Kim YM, Lee CH, Kwak JG, Park CS, Song JY et al.| title=Outcomes of pulmonary valve replacement in 170 patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction: implications for optimal timing of pulmonary valve replacement. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 11 | pages= 1005-14 | pmid=22921969 | doi=10.1016/j.jacc.2012.03.077 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22921969  }} </ref><ref name="pmid17620511">{{cite journal| author=Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk AP, Spijkerboer AM et al.| title=Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. | journal=Circulation | year= 2007 | volume= 116 | issue= 5 | pages= 545-51 | pmid=17620511 | doi=10.1161/CIRCULATIONAHA.106.659664 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17620511  }} </ref>
*Patients with CMR derived pre operative right ventricular end diastolic volume index of less than 160ml/m²  and end systolic volume index of less than 80ml/m² showed better outcomes. <ref name="pmid22921969">{{cite journal| author=Lee C, Kim YM, Lee CH, Kwak JG, Park CS, Song JY et al.| title=Outcomes of pulmonary valve replacement in 170 patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction: implications for optimal timing of pulmonary valve replacement. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 11 | pages= 1005-14 | pmid=22921969 | doi=10.1016/j.jacc.2012.03.077 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22921969  }} </ref><ref name="pmid17620511">{{cite journal| author=Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk AP, Spijkerboer AM et al.| title=Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. | journal=Circulation | year= 2007 | volume= 116 | issue= 5 | pages= 545-51 | pmid=17620511 | doi=10.1161/CIRCULATIONAHA.106.659664 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17620511 }} </ref><ref name="pmid15028368">{{cite journal| author=Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ| title=Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging. | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 6 | pages= 1068-74 | pmid=15028368 | doi=10.1016/j.jacc.2003.10.045 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15028368 }}</ref>


====Follow Up====
====Follow Up====
Line 255: Line 317:
*Anticoagulation is recommended in patients with mechanical valves and aspirin for patients with bioprosthetic valves.<ref name="pmid22561653">{{cite journal| author=Jang W, Kim YJ, Choi K, Lim HG, Kim WH, Lee JR| title=Mid-term results of bioprosthetic pulmonary valve replacement in pulmonary regurgitation after tetralogy of Fallot repair. | journal=Eur J Cardiothorac Surg | year= 2012 | volume= 42 | issue= 1 | pages= e1-8 | pmid=22561653 | doi=10.1093/ejcts/ezs219 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22561653  }} </ref>
*Anticoagulation is recommended in patients with mechanical valves and aspirin for patients with bioprosthetic valves.<ref name="pmid22561653">{{cite journal| author=Jang W, Kim YJ, Choi K, Lim HG, Kim WH, Lee JR| title=Mid-term results of bioprosthetic pulmonary valve replacement in pulmonary regurgitation after tetralogy of Fallot repair. | journal=Eur J Cardiothorac Surg | year= 2012 | volume= 42 | issue= 1 | pages= e1-8 | pmid=22561653 | doi=10.1093/ejcts/ezs219 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22561653  }} </ref>
*Oral anticoagulation in patients with bioprosthetic valves is recommended only when other indications such as atrial arrhythmia or prior thromoembolic event are present.
*Oral anticoagulation in patients with bioprosthetic valves is recommended only when other indications such as atrial arrhythmia or prior thromoembolic event are present.
*All patients are adviced for a lifelong follow up to assess the valve and systolic function.
*All patients are adviced for a lifelong follow up to assess the valve morphology and RV systolic function.


==Prevention==
==Prevention==
There are no primary preventive measures but all the patients with mechanical valve prosthesis are recommended for infective endocarditis prophylaxis.<ref name="pmid24589852">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| 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=Circulation | year= 2014 | volume= 129 | issue= 23 | pages= 2440-92 | pmid=24589852 | doi=10.1161/CIR.0000000000000029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24589852  }} </ref>
There are no primary preventive measures but all the patients with mechanical valve prosthesis are recommended for infective endocarditis prophylaxis.<ref name="pmid24589852">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| 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=Circulation | year= 2014 | volume= 129 | issue= 23 | pages= 2440-92 | pmid=24589852 | doi=10.1161/CIR.0000000000000029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24589852  }} </ref>


==Recommendations==
==ACC / AHA Guidelines - Recommendations for Surgery for Adults With Previous Repair of Tetralogy of Fallot(DO NOT EDIT)==
Recommendations for Surgery for Adults With Previous Repair of Tetralogy of Fallot
According to 2008 ACC/AHA guidelines, ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease<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>
* Class I: Pulmonary valve replacement is indicated for severe pulmonary regurgitation and symptoms or decreased exercise tolerance. (Level of Evidence: B)
{|class="wikitable"
* Class IIa : Pulmonary valve replacement is reasonable in adults with previous tetralogy of Fallot, severe pulmonary regurgitation, and any of the following:
|-
**Moderate to severe RV dysfunction. (Level of Evidence: B)
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
**Moderate to severe RV enlargement. (Level of Evidence: B)
|-
**Development of symptomatic or sustained atrial and/or ventricular arrhythmias. (Level of Evidence: C)
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Pulmonary valve replacement is indicated for severe pulmonary regurgitation and symptoms or decreased exercise tolerance.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>
**Moderate to severe TR. (Level of Evidence: C)
|}


{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
| bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' Pulmonary valve replacement is reasonable in adults with previous tetralogy of Fallot, severe pulmonary regurgitation, and any of the following:
**Moderate to severe RV dysfunction.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
**Moderate to severe RV enlargement. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
**Development of symptomatic or sustained atrial and/or ventricular arrhythmias. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''
**Moderate to severe TR.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}


According to,
==Recommendations for Percutaneous Pulmonary Valve Replacement==
 
According to 2011, Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease, A Scientific Statement From the American Heart Association.<ref name="pmid21536996">{{cite journal| author=Feltes TF, Bacha E, Beekman RH, Cheatham JP, Feinstein JA, Gomes AS et al.| title=Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. | journal=Circulation | year= 2011 | volume= 123 | issue= 22 | pages= 2607-52 | pmid=21536996 | doi=10.1161/CIR.0b013e31821b1f10 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21536996  }}</ref>
Recommendations for Percutaneous Pulmonary Valve Replacement
{| class="wikitable"
Class IIa
|-
 
| colspan="1" style="text-align:center; background:LightCoral"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
    It is reasonable to consider percutaneous pulmonary valve replacement in a patient with an RV-to–pulmonary artery conduit with associated moderate to severe pulmonary regurgitation or stenosis provided the patient meets inclusion/exclusion criteria for the available valve (Level of Evidence: B
|-
| bgcolor="LightCoral"|<nowiki>"</nowiki>'''1.'''It is reasonable to consider percutaneous pulmonary valve replacement in a patient with an RV-to–pulmonary artery conduit with associated moderate to severe pulmonary regurgitation or stenosis provided the patient meets inclusion/exclusion criteria for the available valve .''([[ACC AHA guidelines classification scheme#|Level of Evidence: A]])'' <nowiki>"</nowiki>
|}


==References==
==References==
{{Reflist|2}}
{{WH}}{{WS}}

Latest revision as of 20:19, 29 December 2016

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 related terms: Pulmonary Insufficiency, Pulmonary Incompetence, Pulmonary Valve Regurgitation, Pulmonary Regurgitation, Pulmonary Valve Incompetence.

Overview

Isolated pulmonary valve reguritation is a rare disease, it is usually associated with other congenital heart disease. PR usually occurs in patients who have underwent corrective surgery for tetralogy of Fallot or pulmonary valve stenosis. Mild PR is identified in 40 to 70% of patients with normal pulmonary valve and is clinically insignificant. Chronic severe PR usally presents with exercise intolerance and features of right heart failure with progression of the disease. If left untreated patients develop RV failure, arrythmias and sudden cardiac death. Echocardiography is the initial imaging modality to detect and quantify the severity of PR. CMR is the gold standard to study the morphology of the pulmonary valve, RVOT and the RV systolic function. Chronic significant PR is treated with pulmonary valve replacement. Timing of the surgery is an important prognostic factor determining the prognosis.

Historical Perspective

  • The pulmonary valve and its function of allowing blood to the lungs for nourishment was first described by Hippocrates.[1]
  • Erasistratus, mentioned the involvement of the pulmonary valve in the unidirectional flow.[1]
  • Galen described the membranes of the valves and named them as "semilunar".[1]
  • Mondino de Luzzi designed the sketch of the pulmonary valves in the anatomical position for the first time.[1]
  • Realdo Colombo described the pulmonary circulation for the first time.[1]

Epidemiology and Demographics

  • The prevalence of mild pulmonary regurgutation is present in 40% to 78% of patients with normal pulmonary valve anatomy.[2][3][4]
  • In patients born with congenital heart disease, 20% of patients have associated abnormalities of the pulmonary valve or the right ventricular outlet obstruction.[2]
  • There is increased prevalence of pulmonary regurgitation with increasing age.[2][4]
  • There is no racial or ethnic predilection for pulmonary regurgitation.

Classification

Pulmonary valve is classified into the following types based on the morphology of the valve and severity of the disease:

Based on the Pulmonary Valve Morphology

Pulmonary Regurgitation is classified into primary and secondary types based on the involvement of the pulmonary Valve.

  • Primary Pulmonary Regurgitation:The pulmonary valve morphology is affected. Isolated PR is very rare and is most commonly associated with other congenital heart diseases.[5]
  • Secondary or Functional Pulmonary Regurgitation: The pulmonary valve function is normal. Conditions such as pulmonary hypertension and PA aneursym cause dilation of the valve annulus leading to regurgitation.[6]

Based on the Severity

Pulmonary regurgitation is classified into three categories based on the severity of the regurgitant jet demonstrated on the 2D echo.[7]

  • Mild: Normal valve morphology and usually asymptomatic
  • Moderate: Normal to abnormal valve morphology with mild symptoms
  • Severe: Abnormal valve morphology with significant symptomatology

Natural History, Prognosis, Complications

Natural History

  • Mild PR is a very common finding on 2D echo.[8]
  • Majority of patients with mild PR are asymptomatic and have a beningn course, not progressing to chronic PR.
  • Patients tolerate severe chronic PR for a long period of time and begin to develop symptoms when the right ventricle function begins to decline.
  • Chronic severe PR leads to progressive dilation and systolic dysfunction of the right ventricle resulting in symptoms.
  • Patients with acute worsening of PR should be evaluated for associated conditions such as pulmonary hypertension which increase the pressure gradient.

Prognosis

  • Symptomatic patients are treated with pulmonary valve replacement and have a good prognosis.[9]

Complications

  • Progressive right ventricular dilation increases the risk of ventricular arrhythmias and sudden cardiac death. Patients with tetralogy of Fallot are at increased risk of developing these complications compared to patients with isolated PR.[10][11]

Pathophysiology

  • Patients with PR develop chronic right ventricular overload resulting in right ventricular remodelling and progressive decline in function.[12]
  • Progressive dilation of the right ventricle results in functional tricuspid regurgitation and increases the risk of developing arrhythmias.
  • The rate of decline in right ventricular systolic function is affected by associated conditions such as peripheral pulmonary artery stenosis and pulmonary hypertension which further increase the severity of pulmonary regurgitation.
  • In patients with increased pulmonary artery pressure from dysfunction of LV or residual pulmonary artery stenosis increases the severity of PR.
  • The severity of regurgitant jet is dependent on:[12]
    • Size of the regurgitant orifice
    • Afterload of the RV
    • RV diastolic complaince
    • Duration of RV diastole

According to 2014, ACC/AHA valvular heart disease guidelines the stages of severe pulmonary regurgitation are described as follows:[13]

Stage Definition Pulmonary Valve

Anatomy

Valve Hemodynamics Hemodynamic Consequences Symptoms
C,D Severe PR
  • Distorted or absent leaflets
  • Annular dilation
  • Color jet fills RVOT
  • CW jet density and contour: dense laminar flow with steep deceleration slope; may terminate abruptly
  • Paradoxical septal motion (volume overload pattern)
  • RV enlargement
None or variable and dependent on cause of PR and RV function

Causes

The most common causes of pulmonary regurgitation are following repair of tetralogy of Fallot and pulmonary stenosis. Other common causes include as follows:[14][15][16][17]

Congenital

Causes

Acquired

Causes

Chronic PR Acute PR
  • Quadricuspid or Bicuspid valves
  • Hypoplasia of the valves
  • Prolapse of the pulmonary valve
  • Infective endocarditis
  • Following transannular patching for tetralogy of Fallot
  • Post repair of pulmonary valve stenosis
  • Rheumatic heart disease
  • Carinoid Syndrome
  • Myxomatous Degeneration of the pulmonary valve.
  • Following transannular patching for tetralogy of Fallot
  • Following balloon or surgical valvulotomy or valvuloplasty for pulmonary stenosis
  • Absent pulmonary valve syndrome
  • Isolated congenital PR
  • Peripheral pulmonary artery stenosis
  • Pulmonary hypertension
  • Right ventricular outlet aneurysm
  • Neonatal Ebstein’s anomaly
  • Following balloon dilation of critical pulmonary stenosis
  • Perforation of valvar pulmonary atresia

Differentiating Pulmonary Regurgitation From Other Diseases

Disease Findings
Pulmonary Valve Regurgitation
  • PR is best heard over the left second and third interspaces and increases with inspiration
  • Usually secondary to repair of tetralogy of Fallot or pulmonic Valve stenosis.
Aortic Regurgitation
  • Patients present with dyspnea and fatigability as a consequence of reduced cardiac reserve in the fourth or fifth decade[18]
  • AR is heard over left sternal border or over the right second interspace and radiates to the neck
Tricuspid Regurgitation causing RV enlargement
  • Pansystolic murmur accentuating with inspiration[19]
  • RV heave
  • Gaint "V" wave seen on JVP examination
  • Hepatomegaly is seen in 90% of patients
  • Quantification of severity of TR is done by colour flow doppler imaging[20]
Left to Right Shunt causing RV enlargement
  • Usually seen in children with acyanotic congenital disease such as ASD
  • Fixed splitting of S2 is present
Arrthmogenic Right Ventricular Cardiomyopathy
  • Gradual replacement of normal functional myocardium with adipose or fibroadipose tissue[21]
  • Age of onset is 7 to 40years
  • Patients are usually asymptomatic, present with occasional palpitations
  • EKG shows negative "T" waves and epsilon waves with selective "S" wave delay in V1 to V3
  • RV is dilated and hypokinetic on echocardiography
  • Holter is the diagnostic test to diagnose hyperkinetic ventricular arrythmias

Diagnosis

History and Symptoms

Clinical presentation of pulmonary regurgitation varies on the severity of the regurgitation and the right ventricular function.[8][22]

Physical Examination

The physical examination findings in significant pulmonary regurgitation include:

Neck

  • Increased JVP
  • Prominent "a wave" may be present.
  • Prominent "v wave" may be present in presence of tricuspid regurgitation.
  • A palpable impulse (lift or heave) is usually present at the left lower sternal border because of right ventricular dilation.

Auscultation

  • Pulmonic regurgitation is associated with wide splitting of S2.
  • P2 is accentuated because of presence of pulmonary regurgitation. In case of, absence of pulmonic valves (congenital or secondary to surgical resection), P2 is inaudible.
  • A right-sided S3 may be audible and may also be accentuated with inspiration.
  • Likewise, a right-sided S4 may also be audible and accentuated with inspiration.
  • Murmur of residual pulmonic regurgitation after Tetralogy of Fallot repair:[23]
    • It is a low-pitched and soft murmur.
    • Best heard along the second or third intercostal spaces adjacent to the left sternal border.
    • It is accentuated by squatting and inspiration.
    • It is made softer by Valsalva maneuvers or expiration.
  • Murmur of pulmonic regurgitation associated with Pulmonic hypertension:[24]
    • When the pulmonary artery systolic pressure exceeds 60 mm Hg, dilatation of the pulmonary artery ring may then result in Graham-Steell's murmur.
    • It is a high-pitched, "blowing", early diastolic decrescendo murmur like that of aortic regurgitation.
    • Best heard along the left parasternal region.
    • It is accentuated by inspiration.

Echocardiography

It is the initial imaging diagnostic test to study the pulmonary valve, RVOT anatomy, to identify the presence and quantify the severity of PR. The findings include:[25]

  • In adults, visualization of the pulmonary valve is obtained from the parasternal short-axis view at the level of the aortic valve or from a subcostal approach.[7][26]
  • Pulmonary valve morphology anomalies such as bicuspid or quadricuspid valves, hypoplasia, dysplasia, absence of pulmonary valve and motion abnormalities can be detected.[27]
  • PR is diagnosed my demonstrating a diastolic jet in the RV outflow tract towards the RV.

Colour Flow Doppler

The findings suggestive of significant PR include: [28][29][30][31][32]

  • A narrow small central and spindle shaped regurgitant jet is seen in mild PR.
  • In severe PR a wide diastolic jet at the origin which occupies 65% of the the RVOT width is seen on colour doppler imaging. The duration of the jet increases with increasing severity of PR.
  • In severe PR, a rapid equalization of diastolic pressures between the pulmonary artery and RV occurs, resulting in a short-lived regurgitant jet which can mislead in diagnosis of the severity of PR.
  • In patients with chronic significant PR, dilation of the RV can be demonstrated. In patients with physiologic PR and acute PR RV dimensions are normal.

Vena Contracta Width

It is a more accurate method to assess the severity of PR, but it lacks validation studies.[33]

Flow convergence method

It can be assessed in few patients but lacks validation studies.[34][35]

Pulsed Doppler

It is useful to assess the forward and regurgitant flows at the pulmonary annulus and the pulmonary artery, which can be used to calculate the regurgitant volume and regurgitant fraction.[36]

Spectral Doppler

The density of the CW signal provides a qualitative measure of regurgitation. [37]

  • Pressure half-time (PHT) of less than 100 ms has a high sensitivity and specificity for identifying hemodynamically significant PR in congenital heart disease.[35]
  • PR Index: It is a ratio expressed between the duration of PR and total diastole which is measured from the end of forward pulmonary flow to the beginning of the next forward pulmonary flow curve. It has shown to have equal sensitivity to determine the severity of PR when compared to CMR.[38]
  • Myocardial performance index Tei index determined by tissue doppler imaging is a sensitive indicator of RV function in patients with chronic PR.[39]

Exercise Echocardiography

It is used to unmask latent RV dysfunction and is a helpful investigation to assess the RV function in patients who have underwent an intervention for significant PR.

Determination of severity of PR based on the findings on echocardiography:[40]

Parameters Mild Moderate Severe
Pulmonic valve morphology Normal Normal or abnormal Abnormal
Colour flow PR jet width Small, usually 10 mm in length with a narrow origin Intermediate
  • Large, with a wide origin
  • Can be brief in duration due to the equalization right ventricular and pulmonary diastolic pressures
Continous wave signal of PR jet Faint/slow deceleration Dense/variable Dense/steep deceleration, early termination of diastolic flow
Pulmonic vs. Aortic flow by pulse wave Normal or slightly increased Intermediate Greatly increased

EKG

  • EKG findings in chronic PR are non specific.
  • In patients with tetralogy of Fallot increased QRS duration with widened QRS complex reflects the severity of PR and right ventricular dilation predisposing the patients to develop malignant arrythmias. [41][42]
  • In patients with RV volume overload and isolated PR, QRS prolongation with rSr morphology can be seen in right precordial leads.
  • RBB is common in majority of patients who have tetralogy of Fallot repair with right ventriculotomy.

Chest X-Ray

Chest X-Ray in chronic PR the following findings can be demonstrated:

  • Cardiomegaly when there is dilation of the RV
  • Pulmonary artery dilation

Cardiac MRI

Cardiac magnetic resonance(CMR) is a gold standard for assessment of morphology of the pulmonary valve, for quantification of the severity of the regurgitation and the RV systolic function.

  • CMR is useful in quantification of the regurgitant volume and regurgitant fraction of PR by using sequences called “velocity- encoded phase-contrast images”.[43]
  • CMR is useful for evaluating pulmonary regurgitant fraction, RV end-diastolic and end- systolic volumes, and RV ejection fraction
  • CMR is the diagnostic modality preffered to determine the requirement of reintervention in patients with repaired tetralogy of Fallot and to assess the ventricular function and dimensions.

Cardiac Catheterization

Echo and CMR the investigations of choice in patients to detect and diagnose the severity of PR. Cardiac Catheterization is not a recommended for diagnosis of PR. In patients with poor echocardiographic windows and with pacemakers, CMR cannot be used, catheterization in such patients is a good alternative.[44]
Findings of significant PR include:

  • Low pulmonary artery end-diastolic pressure, resulting in wide pulse pressure
  • Increased right ventricular end-diastolic pressure
  • Equalization of RV and PA diastolic pressures

Treatment

Medical Therapy

  • There are no specific medical measures for management of PR.
  • Diuretics are recommended in patients with RV dysfunction for maintenance of fluid balance.
  • In patients with repaired tetralogy of Fallot, ACE inhibitors or beta-blockers are used to reverse the neuroharmonal activation and improve the symptoms.[45][46]

Surgical Therapy

Indications for Surgery

Indications for Pulmonary Valve Replacement include:[47]

  • Symptomatic patients with arrythmias or NYHA class higher than II
  • Ejection Fraction of less than 40% when assessed with CMR
  • Patients with progressive right ventricular regurgitation(right ventricular end- diastolic volume ≥160 mL/m2 or end-systolic volume ≥82 mL/m2 on CMR)
  • Moderate to severe tricuspid valve regurgitation, resulting from annular dilation
  • Patients at risk of developing arrythmias and with prolonged QRS duration.(total QRS duration ≥180 msec, or QRS duration increase >3.5 msec per year)
  • Severe pulmonic regurgitation in a patient with another cardiac lesion that requires operative intervention

Timing Of Surgery

  • Timing of pulmonary valve replacement is not well defined as in aortic and mitral regurgitation. However timely intervention is advised before the onset of RV dysfunction.[48]
  • Delayed intervention has shown to have poor outcomes and higher rate of re-intervention.
  • The prime goals of pulmonary valve replacement include improved functional class and quality of life, maintenance of right (and left) ventricular function, risk modification of arrhythmia and sudden cardiac death.[49]

Surgical Options

Pulmonary Valve Replacement(PVR) by surgical and percutaneous approach is the definitive treatment for the management of chronic PR and has proven to improve RV function, New York Heart Association Functional Class status, quality of life, and reduce risk for development of RV tachyarrhythmias and sudden cardiac death.[50]

Surgical Valve Implantation

  • Various valved conduits are placed to replace the pulmonic valve which include Homografts from cadavers, valved conduits, and the Contegra bovine jugular vein graft or a bioprosthetic valve implanted directly in the RV outflow tract.[51]
  • Bioprosthetic valves are usually preffered over mechanical valve prosthesis and have a longevity of around 15years.[9][52][53][54]
  • Mechanical valves are preffered in patients who are at high risk of reoperation such as patients with RV dysfunction.[55]
  • Stenosis of the conduit is the major limitation and 25% of patients have to undergo a repeat intervention.
Transcatheter Pulmonary Valve Replacement
  • The Melody transcatheter pulmonary valve(Medtronic) is approved by FDA in 2010.[56]
  • The current transcatheter valves are designed to treat conduit and bioprosthetic valve failure only.[57][58][59][60]
  • They are not useful to treat patients who had a RVOT reconstruction by transannular patching.
Complications
  • Stent fracture:It leads to an increase in RV outflow tract gradient and RV pressure and its incidence is around 21% in 1 series that used the Melody valve and was the major reason for a repeat intervention.[61][62]
  • Device instability and dislodgement[63]
  • Coronary compression due to stent placement[64]
  • Pulmonary Artery obstruction

Outcomes

  • Patients with percutaneous pulmonary valve replacement have good outcome and are free of reintervention at 1year.[65]
  • Patients with CMR derived pre operative right ventricular end diastolic volume index of less than 160ml/m² and end systolic volume index of less than 80ml/m² showed better outcomes. [9][66][67]

Follow Up

  • All the patients should undergo a baseline transthoracic echocardiogram after PVR.[8][68]
  • Anticoagulation is recommended in patients with mechanical valves and aspirin for patients with bioprosthetic valves.[52]
  • Oral anticoagulation in patients with bioprosthetic valves is recommended only when other indications such as atrial arrhythmia or prior thromoembolic event are present.
  • All patients are adviced for a lifelong follow up to assess the valve morphology and RV systolic function.

Prevention

There are no primary preventive measures but all the patients with mechanical valve prosthesis are recommended for infective endocarditis prophylaxis.[69]

ACC / AHA Guidelines - Recommendations for Surgery for Adults With Previous Repair of Tetralogy of Fallot(DO NOT EDIT)

According to 2008 ACC/AHA guidelines, ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease[70]

Class I
"1. Pulmonary valve replacement is indicated for severe pulmonary regurgitation and symptoms or decreased exercise tolerance.(Level of Evidence: B) "
Class IIa
"1. Pulmonary valve replacement is reasonable in adults with previous tetralogy of Fallot, severe pulmonary regurgitation, and any of the following:

Recommendations for Percutaneous Pulmonary Valve Replacement

According to 2011, Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease, A Scientific Statement From the American Heart Association.[64]

Class IIa
"1.It is reasonable to consider percutaneous pulmonary valve replacement in a patient with an RV-to–pulmonary artery conduit with associated moderate to severe pulmonary regurgitation or stenosis provided the patient meets inclusion/exclusion criteria for the available valve .(Level of Evidence: A) "

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