Pulmonic regurgitation overview: Difference between revisions

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{{Pulmonic regurgitation}}
{{Pulmonic regurgitation}}
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==Overview==
==Overview==
Pulmonary valve regurgitation is a condition where the [[pulmonary valve]] is not strong enough to prevent backflow into the [[right ventricle]].  Nearly all individuals have physiologic (trace-to-mild) pulmonic regurgitation, and the incidence increases with advancing age.  Hence, there is a backward flow of blood from the [[pulmonary artery]], through the [[pulmonary valve]], and into the [[right ventricle]] of the heart during [[diastole]].  
[[Pulmonic regurgitation]] (PR) is a condition where the [[pulmonary valve]] is not strong enough to prevent backflow into the [[right ventricle]].  Nearly all individuals have physiologic (trace-to-mild) pulmonic [[regurgitation]] and the [[incidence]] increases with advancing [[age]].  Hence, there is a backward flow of blood from the [[pulmonary artery]], through the [[pulmonary valve]], and into the [[right ventricle]] of the heart during [[diastole]].  
 
[[PVR]] may be classified according to the [[pulmonary valve]] morphology and severity of the [[disease]]. However, in a small percentage of [[patients]], it is a normal finding. [[PVR]] may share overlapping [[symptoms]] with certain other conditions such as [[aortic regurgitation]], [[Tricuspid regurgitation|tricuspid re gurgitation]], left to right shunting, right ventricular [[cardiomyopathy]], [[pulmonary hypertension]], [[infective endocarditis]], carcinoid heart disease, [[syphilis]] and [[Marfan's syndrome|Marfan syndrome]].
 
The main pathophysiologic mechanism for pulmonary regurgitation includes the backflow of blood into the right ventricle resulting in ventricular overload and ventricular remodeling. Complications that may result from pulmonary regurgitation include progressive right ventricular dilatation, [[heart failure]], [[tricuspid regurgitation]], [[ventricular arrhythmias]], and [[sudden cardiac death]].
 
The [[diagnosis]] of [[pulmonic regugitation]] may include detailed [[history]], and [[physical examination]]. Certain diagnostic tests such as [[echocardiography]] and [[cardiac MRI]] may help confirm the diagnosis. The mainstay of treatment for [[PR]] may include medical therapy for mild to moderate cases and pulmonary valve replacement in severe cases. Medical therapy may include use of [[Diuretic|diuretics]] and [[ACE inhibitor|ACE inhibitors]] in patients with right ventricular dysfunction.
 
Lifelong follow up may be required among [[patients]] with [[PR]] to monitor pulmonary valve [[morphology]] and to assess right ventricular function.
 
==Historical perspective==
==Historical perspective==
The pulmonary valve and its function of allowing blood to the lungs for nourishment was first described by Hippocrates. Erasistratus, mentioned the involvement of the pulmonary valve in the unidirectional flow. 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>
[[Pulmonic regurgitation]] murmur was first described as [[Graham-Steell murmur]] by Dr. Graham Steel in 1888. Before that  The [[pulmonary valve]] and its function of allowing [[blood]] to the [[lungs]] for nourishment was first described by Hippocrates. Ibn Nafis then described the [[pulmonary circulation]]. Mondino drew a sketch of the [[pulmonary valves|pulmonic valve]]. In 1888 an early blowing [[diastolic murmur]] due to [[pulmonary hypertension]] was described by Graham-steel, known as Graham-steel murmur. Techniques were then developed to diagnose [[pulmonary valve]] regurgitation via the dye method and [[cardiac catheterization]].


==Classification==
==Classification==
Pulmonary valve regurgitation may be classified according to pulmonary valve morphology and severity of the disease. According to the pulmonary valve morphology, it may be classified into primary and secondary or functional regurgitation. Severity of disease may classify into mild, moderate and severe disease.<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><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><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>
[[Pulmonary valve]] [[regurgitation]] ([[PR]]) may be classified according to [[etiology]] of the [[regurgitation|regurgitant flow]], severity of the [[disease]] and chronicity. Based on the [[etiology]] of the [[regurgitation|regurgitant flow]], [[PR]] may be classified into primary and secondary/ functional types. The severity of the [[disease]] may classify into mild, moderate, and severe [[disease]]. New York Heart Association's (NYHA) functional classification helps to gauge the severity of the [[heart failure]] which is a complication of [[PR]]. The severity of [[PR]] can be assessed by utilizing the American Heart Association and American College of Cardiology (AHA/ACC) staging of [[valvular heart disease]] (VHD).


==Pathophysiology==
==Pathophysiology==
Pathophysiologic mechanism of pulmonic regurgitation include right ventricular overload resulting in right ventricular remodelling and progressive decline in function. 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.<ref name="pmid26430501">{{cite journal| author=Bigdelian H, Mardani D, Sedighi M| title=The Effect of Pulmonary Valve Replacement (PVR) Surgery on Hemodynamics of Patients Who Underwent Repair of Tetralogy of Fallot (TOF). | journal=J Cardiovasc Thorac Res | year= 2015 | volume= 7 | issue= 3 | pages= 122-5 | pmid=26430501 | doi=10.15171/jcvtr.2015.26 | pmc=4586599 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26430501  }} </ref>
[[Pulmonary valve]] is located at the junction of the [[Ventricular outflow tract|right ventricular outflow tract]] and [[pulmonary artery]]. Three equal-sized, semilunar cusps or leaflets make up the [[pulmonary valve]]. [[Pulmonary valve]] opens during right ventricular [[systole]] and closes during right ventricular [[diastole]]. [[PR|Pulmonary regurgitation]] (PR) may be caused by an acquired alteration in the valvular [[morphology]], idiopathic dilatation of the [[pulmonary artery]] (IDPA), [[pulmonic valve]] ring dilatation,  congenital absence or malformation of the [[pulmonary valve|valve]], and increasing [[regurgitation]] causing [[right ventricle|right ventricular]] volume overload. The pathophysiologic mechanism of [[pulmonic regurgitation]] includes [[right ventricular overload]] resulting in the right [[ventricular remodeling]] and progressive decline in function. The rate of decline in right ventricular [[systole|systolic function]] is affected by associated conditions such as peripheral [[pulmonary artery stenosis]] and [[pulmonary hypertension]] which further increase the severity of [[pulmonary regurgitation]]. Among [[patients]] with severe [[PR]], the gene expression pattern of GRK2 and β2-[[Adrenergic receptor|adrenoceptor]] (molecular markers of [[cardiac dysfunction]]) has been reported to be altered. Isolated [[PR]] is uncommon and is usually demonstrated with other [[valve|valvular]] abnormalities or in certain conditions. Conditions associated include [[TOF]], [[rheumatic heart disease]] and [[syphilis]]. On gross pathology [[vegetation|vegetative lesions]] on the [[pulmonic valve]] leaflets may be observed among [[patient|patients]] with [[acquired]] alteration in the [[valve|valvular]] leaflet [[morphology]].


==Causes==
==Causes==
A small percentage of pulmonic regurgitation is normal and occasionally can be heard in thin subjects. The most common causes of pulmonary regurgitation are following repair of tetralogy of Fallot and pulmonary stenosis.<ref name="pmid11436049">{{cite journal| author=Bacha EA, Scheule AM, Zurakowski D, Erickson LC, Hung J, Lang P et al.| title=Long-term results after early primary repair of tetralogy of Fallot. | journal=J Thorac Cardiovasc Surg | year= 2001 | volume= 122 | issue= 1 | pages= 154-61 | pmid=11436049 | doi=10.1067/mtc.2001.115156 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11436049  }}</ref><ref name="pmid26656195">{{cite journal| author=Jonas SN, Kligerman SJ, Burke AP, Frazier AA, White CS| title=Pulmonary Valve Anatomy and Abnormalities: A Pictorial Essay of Radiography, Computed Tomography (CT), and Magnetic Resonance Imaging (MRI). | journal=J Thorac Imaging | year= 2016 | volume= 31 | issue= 1 | pages= W4-12 | pmid=26656195 | doi=10.1097/RTI.0000000000000182 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26656195  }} </ref><ref name="pmid26564602">{{cite journal| author=Ansari MM, Cardoso R, Garcia D, Sandhu S, Horlick E, Brinster D et al.| title=Percutaneous Pulmonary Valve Implantation: Present Status and Evolving Future. | journal=J Am Coll Cardiol | year= 2015 | volume= 66 | issue= 20 | pages= 2246-55 | pmid=26564602 | doi=10.1016/j.jacc.2015.09.055 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26564602  }} </ref><ref name="pmid18997168">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). | journal=Circulation | year= 2008 | volume= 118 | issue= 23 | pages= 2395-451 | pmid=18997168 | doi=10.1161/CIRCULATIONAHA.108.190811 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18997168  }} </ref>
A small percentage of [[pulmonic regurgitation]] is normal and occasionally can be heard in thin subjects. The most common causes of [[PR]] are following repair of [[tetralogy of Fallot]] and [[pulmonary stenosis]].


==Differential Diagnosis==
==Differential Diagnosis==
The diseases which may present with overlapping symptoms as pulmonic regurgitation may include aortic regurgitation, tricuspid regurgitation, left to right shunting, right ventricular cardiomyopathy, pulmonary hypertension, infective endocarditis, carcinoid heart disease, syphilis and marfan syndrome.<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><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><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>
[[PR|Pulmonic regurgitation]] (PR) must be differentiated from other [[diseases]] that cause blowing [[Diastolic heart murmur|decrescendo murmur]] such as [[aortic regurgitation]]. The diseases which may present with overlapping symptoms as [[pulmonic regurgitation]] may include [[aortic regurgitation]], [[tricuspid regurgitation]], left to right shunting, right ventricular [[cardiomyopathy]], [[pulmonary hypertension]], [[infective endocarditis]], [[carcinoid]] heart disease, [[syphilis]] and [[marfan syndrome]].


==Epidemiology and demograpics==
==Epidemiology and demograpics==
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> There is increased prevalence of pulmonary regurgitation with increasing age.
The [[prevalence]] of mild [[PR]] among [[patients]] with normal [[anatomy]] of the [[pulmonary valve]] is 40% to 78%. Among [[patients]] born with [[congenital heart disease]], 20% of [[patients]] have associated abnormalities of the [[pulmonary valve]] or the [[Right ventricular outflow tract obstruction|right ventricular outlet obstruction]]. The [[incidence]] and [[prevalence]] of [[PR]] increases with age. 24% of the [[deaths]] due to [[valvular heart disease]] are attributed to [[tricuspid valve]] and [[pulmonic valve]] abnormalities combined. There is one study supporting the increased [[prevalence]] of [[PR]] among women. In developing countries [[PAH|Pulmonary hypertension]] (PAH) is primarily due to [[rheumatic heart disease]] (RHD) which is rare in developed countries. [[PAH]] is a major cause of secondary [[PR]].


==Risk factors==
==Risk factors==
The risk factors of pulmonic regurgitation may include pulmonary hypertension, surgical repair of teratology of fallot, endocarditis, left sided heart disease, previous ross procedure, collagen vascular disease, and involving the main pulmonary artery.<ref name="pmid20202522">{{cite journal| author=Harrild DM, Powell AJ, Tran TX, Trang TX, Geva T, Lock JE et al.| title=Long-term pulmonary regurgitation following balloon valvuloplasty for pulmonary stenosis risk factors and relationship to exercise capacity and ventricular volume and function. | journal=J Am Coll Cardiol | year= 2010 | volume= 55 | issue= 10 | pages= 1041-7 | pmid=20202522 | doi=10.1016/j.jacc.2010.01.016 | pmc=4235281 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20202522  }} </ref><ref name="pmid18377431">{{cite journal| author=Ammash NM, Dearani JA, Burkhart HM, Connolly HM| title=Pulmonary regurgitation after tetralogy of Fallot repair: clinical features, sequelae, and timing of pulmonary valve replacement. | journal=Congenit Heart Dis | year= 2007 | volume= 2 | issue= 6 | pages= 386-403 | pmid=18377431 | doi=10.1111/j.1747-0803.2007.00131.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18377431  }} </ref><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><ref name="pmid8222120">{{cite journal| author=Rebergen SA, Chin JG, Ottenkamp J, van der Wall EE, de Roos A| title=Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot. Volumetric quantitation by nuclear magnetic resonance velocity mapping. | journal=Circulation | year= 1993 | volume= 88 | issue= 5 Pt 1 | pages= 2257-66 | pmid=8222120 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8222120  }} </ref>
MOst potent risk factors for the development of [[pulmonic regurgitation]] may include [[pulmonary hypertension]], surgical repair of [[teratology of Fallot]], and [[congenital heart diseases]]. Less common but important risk factors include [[endocarditis]], left sided heart disease, previous [[Ross procedure]], [[collagen vascular disease]], and [[malignancies]] involving the main [[pulmonary artery]].


==Screening==
==Screening==
There are no specific screening recommendations for patients with pulmonary regurgitation.<ref name="pmid22869820">{{cite journal| author=Mercer-Rosa L, Yang W, Kutty S, Rychik J, Fogel M, Goldmuntz E| title=Quantifying pulmonary regurgitation and right ventricular function in surgically repaired tetralogy of Fallot: a comparative analysis of echocardiography and magnetic resonance imaging. | journal=Circ Cardiovasc Imaging | year= 2012 | volume= 5 | issue= 5 | pages= 637-43 | pmid=22869820 | doi=10.1161/CIRCIMAGING.112.972588 | pmc=3476467 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22869820  }} </ref>
There are no specific screening recommendations for patients with [[pulmonary regurgitation]] (PR). However, [[patients]] on an increased risk of developing [[PR]] secondary to conditions such as repair of [[Tetralogy of Fallot]] (TOF), [[pulmonary atresia]] or [[truncus arteriosus]] may be evaluated by routine [[echocardiography]], [[ECG]] or [[MRI]] to assess [[right ventricle|right ventricular]] size and status of [[pulmonary valve]]. A study recommends considering ADAMTS19 genetic testing among all [[patients]] with multiple semilunar valve abnormalities. The key [[diagnosis|diagnostic]] tests that may be used for screening of [[PAH]] (a major risk factor for [[PR]]) may include doppler [[transthoracic echocardiography]], DLCO, [[Brain natriuretic peptide|BNP]], [[N-terminal prohormone of brain natriuretic peptide|NT-pro-BNP]], [[serum urate]] levels, and ECG.


==Natural history, complications and prognosis==
==Natural history, complications and prognosis==
Majority of patients with mild 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.<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> Complications which may result from pulmonary regurgitation include progressive right ventricular dilatation, heart failure, tricuspid regurgitation, ventricular arrythmias, and sudden cardiac death. 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>
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.


==Diagnosis==
==Diagnosis==
The diagnosis of pulmonic regurgitation may include detailed history, physical examination and diagnostic tests such as EKG, echocardiography, chest x ray and cardiac MRI.
The diagnosis of pulmonic regurgitation may include detailed history, physical examination and diagnostic tests such as EKG, echocardiography, chest x ray and cardiac MRI.


==History and symptoms==
===History and symptoms===
Clinical presentation of pulmonary regurgitation varies on the severity of the regurgitation and right ventricular dysfunction. Isolated pulmonary regurgitation is usually asymptomatic. However, patients with chronic PR may present with ankle edema, swelling of feet or legs, dyspnea on exertion, fatigue, hemoptysis, nocturnal cough and palpitations.<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>
The history and clinical presentation of [[pulmonary regurgitation]] (PR) vary with the cause of the [[regurgitation]] and [[right ventricular dysfunction]]. The [[patient]] may present with a history related to the primary cause of [[PR]]. Isolated [[pulmonary regurgitation]] is usually asymptomatic. However, patients with chronic [[PR]] may present with [[symptoms]] of [[heart failure]] such as [[dyspnea on exertion]], [[fatigue]], [[ankle edema]], [[hemoptysis]], nocturnal [[cough]] and [[palpitations]]. [[Smoking]] or [[intravenous drug use (recreational)]] history are important to assess the cause fo [[PR]].


==Physical examination==
===Physical examination===
Physical examination findings of pulmonary regurgitation includes increased JVP, prominent "a" wave, "v" wave in the neck. A palpable impulse (lift or heave) is usually present at the left lower sternal border because of right ventricular dilation. On auscultation, it may be associated with wide splitting of S2 with right sided S3 accentuated with respiration. Murmer of pulmonic regurgitation may vary depending on the underlying cause.<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><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>
Physical examination findings of [[pulmonary regurgitation]] (PR) includes a well-appearing [[patient]]. On neck exam, increased [[JVP]], prominent [["a" wave]], [["v" wave]] in the neck may be observed. Precordial (cardiac) exam may reveal a palpable [[apical impulse]] (lift or heave) is usually present at the left lower sternal border because of [[right ventricular dilation]]. On [[auscultation]], it may be associated with wide splitting of [[S2]] with right sided [[S3]] accentuated with respiration. [[Murmur]] of [[pulmonic regurgitation]] may vary depending on the underlying cause. [[Pedal edema]] and [[hepatomegaly]] demonstrate [[right heart failure]].


==Electrocardiogram==
===Electrocardiogram===
EKG findings in patients wit chronic PR may be non specific. However, in patients with tetrology of fallot may show increased QRS duration with widened QRS complex. It may reflect the severity of PR and right ventricular dilation predisposing the patient to develop 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>
[[EKG]] findings among patients wit chronic [[PR|Pulmonic regurgitation]] (PR) may be non-specific. [[Ventricular tachycardia]] is demonstrated on EKG among [[patients]] with [[PR]] and [[RV]] dilatation. [[Patients]] may develop [[atrial flutter]]/[[atrial fibrillation|fibrillation]] after years of [[PR]] development. Among patients with [[tetralogy of Fallot]] (TOF), increased [[QRS]] duration with widened [[QRS]] complex reflects the severity of [[PR]] and [[right ventricular dilation]] predisposes the [[patients]] to develop malignant [[arrythmias]].


==Chest x ray==
===Chest X-ray===
Chest x ray may not be required for the diagnosis of pulmonic regurgitation. However, lateral and PA view of chest radiograph may help determining the right ventricular enlargement. Right atrial enlargement may also be seen in patients with concomitant tricuspid regurgitation.<ref name=abc>https://radiopaedia.org/articles/right-ventricular-enlargement Accessed on 3rd January, 2017</ref>
[[Chest x ray]] may not be required for the diagnosis of [[pulmonic regurgitation]] (PR). However, lateral and [[PA view]] of [[chest radiograph]] may help determining the [[right ventricle|right ventricular]] enlargement. [[Right atrial enlargement]] may also be seen among [[patients]] with concomitant [[tricuspid regurgitation]]. On plain chest Xray [[PR]] may be characterized by [[Right ventricle|right ventricular]] enlargement, prominent [[pulmonary trunk]], features of [[tricuspid regurgitation]] (TR), and of [[congestive heart failure]] (CHF).


==Echocardiography==
===Echocardiography===
Echocardiography is the initial test which may be used to assess pulmonary valve morphology, RVOT anatomy, and to identify the presence and quantify the severity of PR. Different modes of echocardiography may be used to improve the accuracy of findings and assess the severity of the disease which include colour flow doppler, flow convergence method, pulsed doppler, spectral doppler and exercise echocardiography.<ref name="pmid24468055">{{cite journal| author=Valente AM, Cook S, Festa P, Ko HH, Krishnamurthy R, Taylor AM et al.| title=Multimodality imaging guidelines for patients with repaired tetralogy of fallot: a report from the AmericanSsociety of Echocardiography: developed in collaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology. | journal=J Am Soc Echocardiogr | year= 2014 | volume= 27 | issue= 2 | pages= 111-41 | pmid=24468055 | doi=10.1016/j.echo.2013.11.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24468055  }} </ref><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><ref name="pmid12835667">{{cite journal| author=Zoghbi WA, Enriquez-Sarano M, Foster E, Grayburn PA, Kraft CD, Levine RA et al.| title=Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography. | journal=J Am Soc Echocardiogr | year= 2003 | volume= 16 | issue= 7 | pages= 777-802 | pmid=12835667 | doi=10.1016/S0894-7317(03)00335-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12835667  }} </ref>
[[Echocardiography]] is the initial test that may be used to assess [[pulmonary valve]] morphology, [[RVOT|RVOT anatomy]], and to identify the presence and quantify the severity of [[PR|pulmonary regurgitation]] (PR). Different modes of [[echocardiography]] may be used to improve the accuracy of findings and assess the severity of the [[disease]] which include doppler|color flow [[doppler]], continuous wave [[doppler]], pulsed [[doppler]], spectral [[doppler]] and [[Exercise stress testing#Exercise/Pharmacologic Stress Echocardiography|exercise echocardiography]]. The severity of [[PR]] can be assessed by observing color [[pulmonic valve]] [[morphology]], flow [[PR]] jet size and density, and regurgitant Fraction (RF) via doppler [[echocardiography]].


==Cardiac MRI==
===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”.<ref name="pmid19164336">{{cite journal| author=Wald RM, Redington AN, Pereira A, Provost YL, Paul NS, Oechslin EN et al.| title=Refining the assessment of pulmonary regurgitation in adults after tetralogy of Fallot repair: should we be measuring regurgitant fraction or regurgitant volume? | journal=Eur Heart J | year= 2009 | volume= 30 | issue= 3 | pages= 356-61 | pmid=19164336 | doi=10.1093/eurheartj/ehn595 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19164336  }} </ref> 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 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 [[regurgitation|regurgitant]] volume and the regurgitant fraction of [[PR]] by using sequences called “velocity- encoded phase-contrast images”. CMR is useful for evaluating pulmonary regurgitant fraction, [[RV]] [[End-diastolic volume|end-diastolic]] and [[end-systolic volume]] and RV [[ejection fraction]]. CMR is the [[diagnosis|diagnostic]] modality preferred to determine the requirement of re-intervention among [[patients]] with repaired [[tetralogy of Fallot]] and to assess the [[ventricle|ventricular]] function and dimensions.


==Pulmonary angiography==
===Pulmonary angiography===
Pulmonary angiography may play a role in patients with tetrology of fallot repair having pulmonary regurgitation.<ref name="pmid20693132">{{cite journal| author=Erdoğan I, Celiker A, Hazirolan T, Haliloğlu M, Karagöz T| title=Angiocardiography and magnetic resonance imaging to assess pulmonary regurgitation in repaired tetralogy of Fallot. | journal=Anadolu Kardiyol Derg | year= 2010 | volume= 10 | issue= 4 | pages= 353-7 | pmid=20693132 | doi=10.5152/akd.2010.095 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20693132  }} </ref>
Pulmonary [[angiography]] may play a role among [[patients]] with [[TOF]] repair having [[pulmonary regurgitation]].


==Treatment==
==Treatment==
Treatment of pulmonic regurgitation may include medical therapy, surgical therapy and regular follow up.
Treatment of pulmonic regurgitation may include medical therapy, surgical therapy and regular follow up.


==Medical therapy==
===Medical therapy===
Medical management of pulmonic regurgitation may include use of diuretics in patients with RV dysfunction. ACE inhibitors and B blockers may be used to reverse neurohormonal activation and improve 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> Antibiotic prophylaxis may be indicated in certain conditions such as patients with cyanotic heart disease, prosthetic heart valves, rheumatic heart disease, and patients previously having sustained bacterial endocarditis. Surgical management of pulmonic regurgitation may include replacement of pulmonary valve.
Treatment of [[pulmonic regurgitation]] may be divided into medical and surgical treatment. Medical management of [[pulmonic regurgitation]] may include use of [[diuretics]] in patients with [[RV dysfunction]]. [[ACE inhibitors]] and [[beta blockers]] may be used to reverse neurohormonal activation and improve symptoms. [[Antibiotic]] [[prophylaxis]] may be indicated in certain conditions such as patients with [[cyanotic heart disease]], [[prosthetic heart valves]], [[rheumatic heart disease]], and patients previously having sustained [[bacterial endocarditis]]. Among [[patients]] with [[carcinoid syndrome|carcinoid heart disease]] [[subcutaneously]] administered [[octreotide]] in 2–4 divided doses (50–1500 μg/day) provides symptomatic and biochemical benefit.


==Surgical therapy==
===Surgical therapy===
Surgical management of pulmonic regurgitation may include replacement of pulmonary valve. The major indications for pulmonic valve replacement may include 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 and severe pulmonic regurgitation in a patient with another cardiac lesion that requires operative intervention.<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>  Follow up of patients with pulmonic regurgitation requires regular echocardiographic monitoring after PVR, oral anticoagulation in patients with mechanical or bioprosthetic valves and lifelong follow up to monitor pulmonary valve morphology and RV 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>
Surgical management of [[pulmonic regurgitation]] may include [[pulmonary valve]] replacement (PVR). The major indications for PVR may include symptomatic [[patients]] with [[arrythmias]] or [[NYHA]] class higher than II, an [[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 dilatation, [[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 and severe [[pulmonic regurgitation]] among [[patients]] with another cardiac lesion that requires operative intervention. Timing of pulmonary [[valve replacement]] is not well defined. However timely intervention is advised before the onset of [[RV dysfunction]]. 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]]. Among [[patients]] with [[arrhythmias]], intraoperative electrophysiological mapping with [[cryoablation]] during [[pulmonary valve]] replacement has demonstrated promising results.


==Follow up==
===Follow up===
Follow up of the [[patients]] with [[pulmonic regurgitation]] requires regular [[echocardiographic]] monitoring after [[PVR]]. [[Oral anticoagulation therapy|Oral anticoagulation]] among patients with [[Bioprosthetic valves|bioprosthetic valve]]<nowiki/>s is recommended only when other indications such as [[atrial arrhythmia]] or prior [[thromboembolic]] event are present. Upon surveillance among [[patients]] with [[PR]] monitoring [[right ventricle|right ventricular]] dilatation and its sequelae holds more significance than the [[PR|regurgitation]] itself.


==References==
==References==

Latest revision as of 14:52, 8 August 2020

Pulmonic regurgitation Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differential diagnosis

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Electrocardiogram

Chest X-Ray

Echocardiography

Cardiac MRI

Severity Assessment

Treatment

Medical Therapy

Surgical therapy

Follow up

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

Overview

Pulmonic regurgitation (PR) is a condition where the pulmonary valve is not strong enough to prevent backflow into the right ventricle. Nearly all individuals have physiologic (trace-to-mild) pulmonic regurgitation and the incidence increases with advancing age. Hence, there is a backward flow of blood from the pulmonary artery, through the pulmonary valve, and into the right ventricle of the heart during diastole.

PVR may be classified according to the pulmonary valve morphology and severity of the disease. However, in a small percentage of patients, it is a normal finding. PVR may share overlapping symptoms with certain other conditions such as aortic regurgitation, tricuspid re gurgitation, left to right shunting, right ventricular cardiomyopathy, pulmonary hypertension, infective endocarditis, carcinoid heart disease, syphilis and Marfan syndrome.

The main pathophysiologic mechanism for pulmonary regurgitation includes the backflow of blood into the right ventricle resulting in ventricular overload and ventricular remodeling. Complications that may result from pulmonary regurgitation include progressive right ventricular dilatation, heart failure, tricuspid regurgitation, ventricular arrhythmias, and sudden cardiac death.

The diagnosis of pulmonic regugitation may include detailed history, and physical examination. Certain diagnostic tests such as echocardiography and cardiac MRI may help confirm the diagnosis. The mainstay of treatment for PR may include medical therapy for mild to moderate cases and pulmonary valve replacement in severe cases. Medical therapy may include use of diuretics and ACE inhibitors in patients with right ventricular dysfunction.

Lifelong follow up may be required among patients with PR to monitor pulmonary valve morphology and to assess right ventricular function.

Historical perspective

Pulmonic regurgitation murmur was first described as Graham-Steell murmur by Dr. Graham Steel in 1888. Before that The pulmonary valve and its function of allowing blood to the lungs for nourishment was first described by Hippocrates. Ibn Nafis then described the pulmonary circulation. Mondino drew a sketch of the pulmonic valve. In 1888 an early blowing diastolic murmur due to pulmonary hypertension was described by Graham-steel, known as Graham-steel murmur. Techniques were then developed to diagnose pulmonary valve regurgitation via the dye method and cardiac catheterization.

Classification

Pulmonary valve regurgitation (PR) may be classified according to etiology of the regurgitant flow, severity of the disease and chronicity. Based on the etiology of the regurgitant flow, PR may be classified into primary and secondary/ functional types. The severity of the disease may classify into mild, moderate, and severe disease. New York Heart Association's (NYHA) functional classification helps to gauge the severity of the heart failure which is a complication of PR. The severity of PR can be assessed by utilizing the American Heart Association and American College of Cardiology (AHA/ACC) staging of valvular heart disease (VHD).

Pathophysiology

Pulmonary valve is located at the junction of the right ventricular outflow tract and pulmonary artery. Three equal-sized, semilunar cusps or leaflets make up the pulmonary valve. Pulmonary valve opens during right ventricular systole and closes during right ventricular diastole. Pulmonary regurgitation (PR) may be caused by an acquired alteration in the valvular morphology, idiopathic dilatation of the pulmonary artery (IDPA), pulmonic valve ring dilatation, congenital absence or malformation of the valve, and increasing regurgitation causing right ventricular volume overload. The pathophysiologic mechanism of pulmonic regurgitation includes right ventricular overload resulting in the right ventricular remodeling and progressive decline in function. 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. Among patients with severe PR, the gene expression pattern of GRK2 and β2-adrenoceptor (molecular markers of cardiac dysfunction) has been reported to be altered. Isolated PR is uncommon and is usually demonstrated with other valvular abnormalities or in certain conditions. Conditions associated include TOF, rheumatic heart disease and syphilis. On gross pathology vegetative lesions on the pulmonic valve leaflets may be observed among patients with acquired alteration in the valvular leaflet morphology.

Causes

A small percentage of pulmonic regurgitation is normal and occasionally can be heard in thin subjects. The most common causes of PR are following repair of tetralogy of Fallot and pulmonary stenosis.

Differential Diagnosis

Pulmonic regurgitation (PR) must be differentiated from other diseases that cause blowing decrescendo murmur such as aortic regurgitation. The diseases which may present with overlapping symptoms as pulmonic regurgitation may include aortic regurgitation, tricuspid regurgitation, left to right shunting, right ventricular cardiomyopathy, pulmonary hypertension, infective endocarditis, carcinoid heart disease, syphilis and marfan syndrome.

Epidemiology and demograpics

The prevalence of mild PR among patients with normal anatomy of the pulmonary valve is 40% to 78%. Among patients born with congenital heart disease, 20% of patients have associated abnormalities of the pulmonary valve or the right ventricular outlet obstruction. The incidence and prevalence of PR increases with age. 24% of the deaths due to valvular heart disease are attributed to tricuspid valve and pulmonic valve abnormalities combined. There is one study supporting the increased prevalence of PR among women. In developing countries Pulmonary hypertension (PAH) is primarily due to rheumatic heart disease (RHD) which is rare in developed countries. PAH is a major cause of secondary PR.

Risk factors

MOst potent risk factors for the development of pulmonic regurgitation may include pulmonary hypertension, surgical repair of teratology of Fallot, and congenital heart diseases. Less common but important risk factors include endocarditis, left sided heart disease, previous Ross procedure, collagen vascular disease, and malignancies involving the main pulmonary artery.

Screening

There are no specific screening recommendations for patients with pulmonary regurgitation (PR). However, patients on an increased risk of developing PR secondary to conditions such as repair of Tetralogy of Fallot (TOF), pulmonary atresia or truncus arteriosus may be evaluated by routine echocardiography, ECG or MRI to assess right ventricular size and status of pulmonary valve. A study recommends considering ADAMTS19 genetic testing among all patients with multiple semilunar valve abnormalities. The key diagnostic tests that may be used for screening of PAH (a major risk factor for PR) may include doppler transthoracic echocardiography, DLCO, BNP, NT-pro-BNP, serum urate levels, and ECG.

Natural history, complications and prognosis

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.

Diagnosis

The diagnosis of pulmonic regurgitation may include detailed history, physical examination and diagnostic tests such as EKG, echocardiography, chest x ray and cardiac MRI.

History and symptoms

The history and clinical presentation of pulmonary regurgitation (PR) vary with the cause of the regurgitation and right ventricular dysfunction. The patient may present with a history related to the primary cause of PR. Isolated pulmonary regurgitation is usually asymptomatic. However, patients with chronic PR may present with symptoms of heart failure such as dyspnea on exertion, fatigue, ankle edema, hemoptysis, nocturnal cough and palpitations. Smoking or intravenous drug use (recreational) history are important to assess the cause fo PR.

Physical examination

Physical examination findings of pulmonary regurgitation (PR) includes a well-appearing patient. On neck exam, increased JVP, prominent "a" wave, "v" wave in the neck may be observed. Precordial (cardiac) exam may reveal a palpable apical impulse (lift or heave) is usually present at the left lower sternal border because of right ventricular dilation. On auscultation, it may be associated with wide splitting of S2 with right sided S3 accentuated with respiration. Murmur of pulmonic regurgitation may vary depending on the underlying cause. Pedal edema and hepatomegaly demonstrate right heart failure.

Electrocardiogram

EKG findings among patients wit chronic Pulmonic regurgitation (PR) may be non-specific. Ventricular tachycardia is demonstrated on EKG among patients with PR and RV dilatation. Patients may develop atrial flutter/fibrillation after years of PR development. Among patients with tetralogy of Fallot (TOF), increased QRS duration with widened QRS complex reflects the severity of PR and right ventricular dilation predisposes the patients to develop malignant arrythmias.

Chest X-ray

Chest x ray may not be required for the diagnosis of pulmonic regurgitation (PR). However, lateral and PA view of chest radiograph may help determining the right ventricular enlargement. Right atrial enlargement may also be seen among patients with concomitant tricuspid regurgitation. On plain chest Xray PR may be characterized by right ventricular enlargement, prominent pulmonary trunk, features of tricuspid regurgitation (TR), and of congestive heart failure (CHF).

Echocardiography

Echocardiography is the initial test that may be used to assess pulmonary valve morphology, RVOT anatomy, and to identify the presence and quantify the severity of pulmonary regurgitation (PR). Different modes of echocardiography may be used to improve the accuracy of findings and assess the severity of the disease which include doppler|color flow doppler, continuous wave doppler, pulsed doppler, spectral doppler and exercise echocardiography. The severity of PR can be assessed by observing color pulmonic valve morphology, flow PR jet size and density, and regurgitant Fraction (RF) via doppler echocardiography.

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 the regurgitant fraction of PR by using sequences called “velocity- encoded phase-contrast images”. CMR is useful for evaluating pulmonary regurgitant fraction, RV end-diastolic and end-systolic volume and RV ejection fraction. CMR is the diagnostic modality preferred to determine the requirement of re-intervention among patients with repaired tetralogy of Fallot and to assess the ventricular function and dimensions.

Pulmonary angiography

Pulmonary angiography may play a role among patients with TOF repair having pulmonary regurgitation.

Treatment

Treatment of pulmonic regurgitation may include medical therapy, surgical therapy and regular follow up.

Medical therapy

Treatment of pulmonic regurgitation may be divided into medical and surgical treatment. Medical management of pulmonic regurgitation may include use of diuretics in patients with RV dysfunction. ACE inhibitors and beta blockers may be used to reverse neurohormonal activation and improve symptoms. Antibiotic prophylaxis may be indicated in certain conditions such as patients with cyanotic heart disease, prosthetic heart valves, rheumatic heart disease, and patients previously having sustained bacterial endocarditis. Among patients with carcinoid heart disease subcutaneously administered octreotide in 2–4 divided doses (50–1500 μg/day) provides symptomatic and biochemical benefit.

Surgical therapy

Surgical management of pulmonic regurgitation may include pulmonary valve replacement (PVR). The major indications for PVR may include symptomatic patients with arrythmias or NYHA class higher than II, an 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 dilatation, 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 and severe pulmonic regurgitation among patients with another cardiac lesion that requires operative intervention. Timing of pulmonary valve replacement is not well defined. However timely intervention is advised before the onset of RV dysfunction. 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. Among patients with arrhythmias, intraoperative electrophysiological mapping with cryoablation during pulmonary valve replacement has demonstrated promising results.

Follow up

Follow up of the patients with pulmonic regurgitation requires regular echocardiographic monitoring after PVR. Oral anticoagulation among patients with bioprosthetic valves is recommended only when other indications such as atrial arrhythmia or prior thromboembolic event are present. Upon surveillance among patients with PR monitoring right ventricular dilatation and its sequelae holds more significance than the regurgitation itself.

References

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