Pulmonic regurgitation natural history, complications and prognosis: Difference between revisions

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{{Pulmonic regurgitation}}
{{Pulmonic regurgitation}}
{{CMG}}
{{CMG}},{{AE}}, {{AKI}}, {{AA}}, {{JA}}


==Overview==
==Overview==
The majority of [[patients]] with mild [[pulmonary regurgitation]] (PR) are asymptomatic and have a benign 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. The severity of [[PR]] after [[TOF]] repair can increase over time and [[patients]] may develop [[symptoms]] from an early [[age]]. Complications that may result from [[PR]]  include progressive [[right ventricle|right ventricular]] dilatation, [[heart failure]], [[tricuspid regurgitation]], [[ventricular arrhythmias]], and [[sudden cardiac death]]. The prognosis of [[pulmonic regurgitation]] depends on the severity of the condition, etiology, and associated complications. Symptomatic [[patients]] are treated with [[pulmonary valve]] replacement (PVR) and have a good prognosis.


==Natural history==
==Natural History, Complications, and Prognosis==
*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>
===Natural history===
*Majority of patients with mild PR are asymptomatic and have a beningn course, not progressing to chronic PR.
*'''Mild [[PR]]'''<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>:
*Patients tolerate severe chronic PR for a long period of time and begin to develop symptoms when the right ventricle function begins to decline.
**The majority of [[patients]] with mild PR are asymptomatic and have a benign course, not progressing to chronic [[PR]].  
*Chronic severe PR leads to progressive dilation and systolic dysfunction of the right ventricle resulting in symptoms.
**Mild [[PR]] is a common finding on [[2D echo]].
*Patients with acute worsening of PR should be evaluated for  associated conditions such as pulmonary hypertension which increase the pressure gradient.
*'''Acute worsening of [[PR]]''':
**[[Patients]] with [[acute]] worsening of [[PR]] should be evaluated for associated conditions such as [[pulmonary hypertension]] which increase the [[pressure gradient]].
*'''Chronic [[PR]]'''<ref name="pmid15028368">{{cite journal |vauthors=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. |volume=43 |issue=6 |pages=1068–74 |date=March 2004 |pmid=15028368 |doi=10.1016/j.jacc.2003.10.045 |url=}}</ref>:
**[[Patients]] tolerate severe chronic [[PR]] for a long period of time and begin to develop [[symptoms]] when the [[right ventricle|right ventricular]] systolic function begins to decline or marked dilatation occur. In a [[symptomatic]] patient [[RV]] dysfunction has usually become irreversible.
**Chronic severe [[PR]] leads to progressive dilation and [[systolic dysfunction]] of the [[RV]](right ventricle) resulting in [[symptoms]].
*'''Isolated congenital [[PR]]'''<ref name="pmid6207619">{{cite journal |vauthors=Shimazaki Y, Blackstone EH, Kirklin JW |title=The natural history of isolated congenital pulmonary valve incompetence: surgical implications |journal=Thorac Cardiovasc Surg |volume=32 |issue=4 |pages=257–9 |date=August 1984 |pmid=6207619 |doi=10.1055/s-2007-1023399 |url=}}</ref>:
**Among [[patients]] with isolated congenital [[PR]], it is uncommon to develop [[symptoms]] before 30 years of [[age]].
**After the age of 40 years, the development of [[symptoms]] such as [[right heart failure]] or even [[SCD]] demonstrates an increased risk.
*'''Post [[TOF]] repair'''<ref name="pmid1622697">{{cite journal |vauthors=Carvalho JS, Shinebourne EA, Busst C, Rigby ML, Redington AN |title=Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of residual pulmonary regurgitation |journal=Br Heart J |volume=67 |issue=6 |pages=470–3 |date=June 1992 |pmid=1622697 |pmc=1024889 |doi=10.1136/hrt.67.6.470 |url=}}</ref><ref name="BouzasKilner2005">{{cite journal|last1=Bouzas|first1=Beatriz|last2=Kilner|first2=Philip J.|last3=Gatzoulis|first3=Michael A.|title=Pulmonary regurgitation: not a benign lesion|journal=European Heart Journal|volume=26|issue=5|year=2005|pages=433–439|issn=0195-668X|doi=10.1093/eurheartj/ehi091}}</ref>:
**Among [[patients]] with [[TOF]] repair, [[symptoms]] may develop at an early [[age]]. Thie finding is partly attributed to the effect of associated [[lesions]] on [[myocardial]] function. Post complete [[TOF]] repair, impaired exercise capacity is directly related to the degree of residual [[PR]].
**The severity of [[PR]] after [[TOF]] repair can increase over time. The data is supported by experimental<ref name="pmid11696479">{{cite journal |vauthors=Kuehne T, Saeed M, Reddy G, Akbari H, Gleason K, Turner D, Teitel D, Moore P, Higgins CB |title=Sequential magnetic resonance monitoring of pulmonary flow with endovascular stents placed across the pulmonary valve in growing Swine |journal=Circulation |volume=104 |issue=19 |pages=2363–8 |date=November 2001 |pmid=11696479 |doi=10.1161/hc4401.098472 |url=}}</ref><ref name="pmid14557371">{{cite journal |vauthors=Kuehne T, Saeed M, Gleason K, Turner D, Teitel D, Higgins CB, Moore P |title=Effects of pulmonary insufficiency on biventricular function in the developing heart of growing swine |journal=Circulation |volume=108 |issue=16 |pages=2007–13 |date=October 2003 |pmid=14557371 |doi=10.1161/01.CIR.0000092887.84425.09 |url=}}</ref> and clinical<ref name="pmid4033177">{{cite journal |vauthors=Siwek LG, Applebaum RE, Jones M, Clark RE |title=Acute control of pulmonary regurgitation with a balloon "valve". An experimental investigation |journal=J. Thorac. Cardiovasc. Surg. |volume=90 |issue=3 |pages=404–9 |date=September 1985 |pmid=4033177 |doi= |url=}}</ref> evidence.
*'''Idiopathic Dilatation of [[Pulmonary artery|Pulmonary Artery]] (IDPA)'''<ref name="pmid24133364">{{cite journal |vauthors=Choi YJ, Kim U, Lee JS, Park WJ, Lee SH, Park JS, Shin DG, Kim YJ |title=A case of extrinsic compression of the left main coronary artery secondary to pulmonary artery dilatation |journal=J. Korean Med. Sci. |volume=28 |issue=10 |pages=1543–8 |date=October 2013 |pmid=24133364 |pmc=3792613 |doi=10.3346/jkms.2013.28.10.1543 |url=}}</ref><ref name="pmid28228295">{{cite journal |vauthors=Malviya A, Jha PK, Kalita JP, Saikia MK, Mishra A |title=Idiopathic dilatation of pulmonary artery: A review |journal=Indian Heart J |volume=69 |issue=1 |pages=119–124 |date=2017 |pmid=28228295 |pmc=5319124 |doi=10.1016/j.ihj.2016.07.009 |url=}}</ref>:
**The condition is usually asymptomatic but may demonstrate [[symptoms]] in case of development of complications. The duration of illness has been demonstrated to be more than 20 years among 10% [[patients]].
**Dilated [[pulmonary arteries]] may lead to the compression of [[left main coronary artery]].


==Complications==
===Complications===
Complications which may result from pulmonary regurgitation include:<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><ref name="pmid15364855">{{cite journal| author=Frigiola A, Redington AN, Cullen S, Vogel M| title=Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot. | journal=Circulation | year= 2004 | volume= 110 | issue= 11 Suppl 1 | pages= II153-7 | pmid=15364855 | doi=10.1161/01.CIR.0000138397.60956.c2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15364855  }} </ref><ref name="pmid20713900">{{cite journal| author=Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J et al.| title=Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. | journal=Circulation | year= 2010 | volume= 122 | issue= 9 | pages= 868-75 | pmid=20713900 | doi=10.1161/CIRCULATIONAHA.109.928481 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20713900  }} </ref>
*Common complications of [[pulmonary regurgitation]] (PR) include:<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><ref name="pmid15364855">{{cite journal| author=Frigiola A, Redington AN, Cullen S, Vogel M| title=Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot. | journal=Circulation | year= 2004 | volume= 110 | issue= 11 Suppl 1 | pages= II153-7 | pmid=15364855 | doi=10.1161/01.CIR.0000138397.60956.c2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15364855  }} </ref><ref name="pmid20713900">{{cite journal| author=Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J et al.| title=Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. | journal=Circulation | year= 2010 | volume= 122 | issue= 9 | pages= 868-75 | pmid=20713900 | doi=10.1161/CIRCULATIONAHA.109.928481 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20713900  }} </ref><ref name="pmid11041398">{{cite journal |vauthors=Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD, Redington AN |title=Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study |journal=Lancet |volume=356 |issue=9234 |pages=975–81 |date=September 2000 |pmid=11041398 |doi=10.1016/S0140-6736(00)02714-8 |url=}}</ref><ref name="BouzasKilner2005">{{cite journal|last1=Bouzas|first1=Beatriz|last2=Kilner|first2=Philip J.|last3=Gatzoulis|first3=Michael A.|title=Pulmonary regurgitation: not a benign lesion|journal=European Heart Journal|volume=26|issue=5|year=2005|pages=433–439|issn=0195-668X|doi=10.1093/eurheartj/ehi091}}</ref>
*Progressive right ventricular dilatation
*#Progressive [[right ventricular]](RV) dilatation
*Heart failure
*#[[RV]] dysfunction
*Tricuspid regurgitation
*#[[Heart failure]] (HF): [[Exercise intolerance]] is one of the features of [[Heart failure|HF]]. To read about the degree of [[Heart failure|HF]] and its manifestations according to NYHA classification [[New york heart association functional classification|click here]].
*Ventricular arrythmias
*#[[Tricuspid regurgitation]]
*Sudden cardiac death
*#Ventricular [[arrhythmias]] (such as [[ventricular tachycardia]])
*#[[Nutmeg liver|Hepatic congestion]] may develop secondary to [[right heart failure]]. [[Hepatic dysfunction]] may ensue [[thromboembolic]] events.
*#[[Sudden cardiac death]]:
**Among [[patients]] with repaired [[TOF]], [[pulmonic regurgitation]] is a major [[hemodynamic]] lesion associated with [[ventricular tachycardia]] and [[sudden cardiac death]].
**[[Sudden cardiac death]] among [[patients]] with IDPA is due to the compression of [[left main coronary artery]].<ref name="pmid24133364">{{cite journal |vauthors=Choi YJ, Kim U, Lee JS, Park WJ, Lee SH, Park JS, Shin DG, Kim YJ |title=A case of extrinsic compression of the left main coronary artery secondary to pulmonary artery dilatation |journal=J. Korean Med. Sci. |volume=28 |issue=10 |pages=1543–8 |date=October 2013 |pmid=24133364 |pmc=3792613 |doi=10.3346/jkms.2013.28.10.1543 |url=}}</ref><ref name="pmid8461230">{{cite journal |vauthors=Andrews R, Colloby P, Hubner PJ |title=Pulmonary artery dissection in a patient with idiopathic dilatation of the pulmonary artery: a rare cause of sudden cardiac death |journal=Br Heart J |volume=69 |issue=3 |pages=268–9 |date=March 1993 |pmid=8461230 |pmc=1024995 |doi=10.1136/hrt.69.3.268 |url=}}</ref>


==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>
===Prognosis===
*The prognosis of [[pulmonic regurgitation]] depends on the severity of the condition, etiology and associated complications.<ref name="pmid31985929">{{cite journal |vauthors=Pendela VS, Ayyad R |title= |journal= |volume= |issue= |pages= |date= |pmid=31985929 |doi= |url=}}</ref>
*Mild to moderate [[PR]] is not associated with shortened survival.<ref name="pmid31985929">{{cite journal |vauthors=Pendela VS, Ayyad R |title= |journal= |volume= |issue= |pages= |date= |pmid=31985929 |doi= |url=}}</ref>
*The [[prognosis]] of [[symptomatic]] patients of [[PR]] is good after pulmonary [[valve replacement]].<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> Surgical Pulmonic Valve Repair (PVR) improves [[right ventricle|right ventricular]] filling and increases [[left ventricle|left ventricular]] [[stroke volume]].<ref name="FrigiolaGiardini2012">{{cite journal|last1=Frigiola|first1=A.|last2=Giardini|first2=A.|last3=Taylor|first3=A.|last4=Tsang|first4=V.|last5=Derrick|first5=G.|last6=Khambadkone|first6=S.|last7=Walker|first7=F.|last8=Cullen|first8=S.|last9=Bonhoeffer|first9=P.|last10=Marek|first10=J.|title=Echocardiographic assessment of diastolic biventricular properties in patients operated for severe pulmonary regurgitation and association with exercise capacity|journal=European Heart Journal - Cardiovascular Imaging|volume=13|issue=8|year=2012|pages=697–702|issn=2047-2404|doi=10.1093/ehjci/jes002}}</ref> Ten year overall and event-free survival after PVR is 98% and 70%, respectively.<ref name="LeeKim2012">{{cite journal|last1=Lee|first1=Cheul|last2=Kim|first2=Yang Min|last3=Lee|first3=Chang-Ha|last4=Kwak|first4=Jae Gun|last5=Park|first5=Chun Soo|last6=Song|first6=Jin Young|last7=Shim|first7=Woo-Sup|last8=Choi|first8=Eun Young|last9=Lee|first9=Sang Yun|last10=Baek|first10=Jae Suk|title=Outcomes of Pulmonary Valve Replacement in 170 Patients With Chronic Pulmonary Regurgitation After Relief of Right Ventricular Outflow Tract Obstruction|journal=Journal of the American College of Cardiology|volume=60|issue=11|year=2012|pages=1005–1014|issn=07351097|doi=10.1016/j.jacc.2012.03.077}}</ref>
* Among patients with [[pulmonary hypertension]] (PAH), the severity and duration of [[pulmonary hypertension|PAH]] determines the ultimate [[prognosis]].
*The [[prognosis]] of [[PR]] due to [[Absent pulmonary valve|congenital absence of pulmonic valve]] is poor and may limit [[patient]]'s life expectancy in the absence of [[valve replacement]]. [[Absent pulmonary valve]] (APVS) is associated with severe [[regurgitation]] and complications secondary to [[respiratory distress]].<ref name="pmid24843213">{{cite journal |vauthors=Grewal DS, Chamoli SC, Saxena S |title=Absent pulmonary valve syndrome - Antenatal diagnosis |journal=Med J Armed Forces India |volume=70 |issue=2 |pages=198–200 |date=April 2014 |pmid=24843213 |pmc=4017172 |doi=10.1016/j.mjafi.2013.07.002 |url=}}</ref>
*Higher pre-operative [[RV]] [[end-systolic volume]] index (ESVI) is the only independent [[risk factor]] for suboptimal outcomes post pulmonary [[valve replacement]].<ref name="LeeKim2012">{{cite journal|last1=Lee|first1=Cheul|last2=Kim|first2=Yang Min|last3=Lee|first3=Chang-Ha|last4=Kwak|first4=Jae Gun|last5=Park|first5=Chun Soo|last6=Song|first6=Jin Young|last7=Shim|first7=Woo-Sup|last8=Choi|first8=Eun Young|last9=Lee|first9=Sang Yun|last10=Baek|first10=Jae Suk|title=Outcomes of Pulmonary Valve Replacement in 170 Patients With Chronic Pulmonary Regurgitation After Relief of Right Ventricular Outflow Tract Obstruction|journal=Journal of the American College of Cardiology|volume=60|issue=11|year=2012|pages=1005–1014|issn=07351097|doi=10.1016/j.jacc.2012.03.077}}</ref>
*[[QRS]] duration of ⩾180 ms on resting [[EKG]] has been demonstrated to be a strong predictor of [[ventricular arrhythmias]] and [[sudden death]] among patients post [[TOF]] repair.<ref name="pmid7600655">{{cite journal |vauthors=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 |volume=92 |issue=2 |pages=231–7 |date=July 1995 |pmid=7600655 |doi=10.1161/01.cir.92.2.231 |url=}}</ref>


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 12:16, 8 August 2020

Pulmonic regurgitation Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1],Associate Editor(s)-in-Chief: , Aravind Kuchkuntla, M.B.B.S[2], Aysha Anwar, M.B.B.S[3], Javaria Anwer M.D.[4]

Overview

The majority of patients with mild pulmonary regurgitation (PR) are asymptomatic and have a benign 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. The severity of PR after TOF repair can increase over time and patients may develop symptoms from an early age. Complications that may result from PR include progressive right ventricular dilatation, heart failure, tricuspid regurgitation, ventricular arrhythmias, and sudden cardiac death. The prognosis of pulmonic regurgitation depends on the severity of the condition, etiology, and associated complications. Symptomatic patients are treated with pulmonary valve replacement (PVR) and have a good prognosis.

Natural History, Complications, and Prognosis

Natural history

Complications


Prognosis

References

  1. ↑ Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "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". J Am Coll Cardiol. 52 (23): e143–263. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.
  2. ↑ Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ (March 2004). "Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging". J. Am. Coll. Cardiol. 43 (6): 1068–74. doi:10.1016/j.jacc.2003.10.045. PMID 15028368.
  3. ↑ Shimazaki Y, Blackstone EH, Kirklin JW (August 1984). "The natural history of isolated congenital pulmonary valve incompetence: surgical implications". Thorac Cardiovasc Surg. 32 (4): 257–9. doi:10.1055/s-2007-1023399. PMID 6207619.
  4. ↑ Carvalho JS, Shinebourne EA, Busst C, Rigby ML, Redington AN (June 1992). "Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of residual pulmonary regurgitation". Br Heart J. 67 (6): 470–3. doi:10.1136/hrt.67.6.470. PMC 1024889. PMID 1622697.
  5. ↑ 5.0 5.1 Bouzas, Beatriz; Kilner, Philip J.; Gatzoulis, Michael A. (2005). "Pulmonary regurgitation: not a benign lesion". European Heart Journal. 26 (5): 433–439. doi:10.1093/eurheartj/ehi091. ISSN 0195-668X.
  6. ↑ Kuehne T, Saeed M, Reddy G, Akbari H, Gleason K, Turner D, Teitel D, Moore P, Higgins CB (November 2001). "Sequential magnetic resonance monitoring of pulmonary flow with endovascular stents placed across the pulmonary valve in growing Swine". Circulation. 104 (19): 2363–8. doi:10.1161/hc4401.098472. PMID 11696479.
  7. ↑ Kuehne T, Saeed M, Gleason K, Turner D, Teitel D, Higgins CB, Moore P (October 2003). "Effects of pulmonary insufficiency on biventricular function in the developing heart of growing swine". Circulation. 108 (16): 2007–13. doi:10.1161/01.CIR.0000092887.84425.09. PMID 14557371.
  8. ↑ Siwek LG, Applebaum RE, Jones M, Clark RE (September 1985). "Acute control of pulmonary regurgitation with a balloon "valve". An experimental investigation". J. Thorac. Cardiovasc. Surg. 90 (3): 404–9. PMID 4033177.
  9. ↑ 9.0 9.1 Choi YJ, Kim U, Lee JS, Park WJ, Lee SH, Park JS, Shin DG, Kim YJ (October 2013). "A case of extrinsic compression of the left main coronary artery secondary to pulmonary artery dilatation". J. Korean Med. Sci. 28 (10): 1543–8. doi:10.3346/jkms.2013.28.10.1543. PMC 3792613. PMID 24133364.
  10. ↑ Malviya A, Jha PK, Kalita JP, Saikia MK, Mishra A (2017). "Idiopathic dilatation of pulmonary artery: A review". Indian Heart J. 69 (1): 119–124. doi:10.1016/j.ihj.2016.07.009. PMC 5319124. PMID 28228295.
  11. ↑ Gregg D, Foster E (2007). "Pulmonary insufficiency is the nexus of late complications in tetralogy of Fallot". Curr Cardiol Rep. 9 (4): 315–22. PMID 17601398.
  12. ↑ Helbing WA, Roest AA, Niezen RA, Vliegen HW, Hazekamp MG, Ottenkamp J; et al. (2002). "ECG predictors of ventricular arrhythmias and biventricular size and wall mass in tetralogy of Fallot with pulmonary regurgitation". Heart. 88 (5): 515–9. PMC 1767425. PMID 12381647.
  13. ↑ Frigiola A, Redington AN, Cullen S, Vogel M (2004). "Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot". Circulation. 110 (11 Suppl 1): II153–7. doi:10.1161/01.CIR.0000138397.60956.c2. PMID 15364855.
  14. ↑ Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J; et al. (2010). "Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study". Circulation. 122 (9): 868–75. doi:10.1161/CIRCULATIONAHA.109.928481. PMID 20713900.
  15. ↑ Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD, Redington AN (September 2000). "Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study". Lancet. 356 (9234): 975–81. doi:10.1016/S0140-6736(00)02714-8. PMID 11041398.
  16. ↑ Andrews R, Colloby P, Hubner PJ (March 1993). "Pulmonary artery dissection in a patient with idiopathic dilatation of the pulmonary artery: a rare cause of sudden cardiac death". Br Heart J. 69 (3): 268–9. doi:10.1136/hrt.69.3.268. PMC 1024995. PMID 8461230.
  17. ↑ 17.0 17.1 Pendela VS, Ayyad R. PMID 31985929. Missing or empty |title= (help)
  18. ↑ Lee C, Kim YM, Lee CH, Kwak JG, Park CS, Song JY; et al. (2012). "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". J Am Coll Cardiol. 60 (11): 1005–14. doi:10.1016/j.jacc.2012.03.077. PMID 22921969.
  19. ↑ Frigiola, A.; Giardini, A.; Taylor, A.; Tsang, V.; Derrick, G.; Khambadkone, S.; Walker, F.; Cullen, S.; Bonhoeffer, P.; Marek, J. (2012). "Echocardiographic assessment of diastolic biventricular properties in patients operated for severe pulmonary regurgitation and association with exercise capacity". European Heart Journal - Cardiovascular Imaging. 13 (8): 697–702. doi:10.1093/ehjci/jes002. ISSN 2047-2404.
  20. ↑ 20.0 20.1 Lee, Cheul; Kim, Yang Min; Lee, Chang-Ha; Kwak, Jae Gun; Park, Chun Soo; Song, Jin Young; Shim, Woo-Sup; Choi, Eun Young; Lee, Sang Yun; Baek, Jae Suk (2012). "Outcomes of Pulmonary Valve Replacement in 170 Patients With Chronic Pulmonary Regurgitation After Relief of Right Ventricular Outflow Tract Obstruction". Journal of the American College of Cardiology. 60 (11): 1005–1014. doi:10.1016/j.jacc.2012.03.077. ISSN 0735-1097.
  21. ↑ Grewal DS, Chamoli SC, Saxena S (April 2014). "Absent pulmonary valve syndrome - Antenatal diagnosis". Med J Armed Forces India. 70 (2): 198–200. doi:10.1016/j.mjafi.2013.07.002. PMC 4017172. PMID 24843213.
  22. ↑ Gatzoulis MA, Till JA, Somerville J, Redington AN (July 1995). "Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death". Circulation. 92 (2): 231–7. doi:10.1161/01.cir.92.2.231. PMID 7600655.
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