Pulmonic regurgitation overview

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Pulmonic regurgitation Microchapters

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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: Aravind Kuchkuntla, M.B.B.S[2], Aysha Anwar, M.B.B.S[3]

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.

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.[1]

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.[2][3][4]

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.[5]

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.[6][7][8][9]

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.[10][11][12]

Epidemiology and demograpics

The prevalence of mild pulmonary regurgutation is present in 40% to 78% of patients with normal pulmonary valve anatomy.[13][14] There is increased prevalence of pulmonary regurgitation with increasing age.

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.[15][16][2][17]

Screening

There are no specific screening recommendations for patients with pulmonary regurgitation.[18]

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.[19] 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.[20]

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

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.[19][21]

Physical examination

Electrocardiogram

Chest x ray

Echocardiography

Cardiac MRI

Pulmonary angiography

Treatment

Medical therapy

Surgical therapy

Follow up

References

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  2. 2.0 2.1 Chaturvedi RR, Redington AN (2007). "Pulmonary regurgitation in congenital heart disease". Heart. 93 (7): 880–9. doi:10.1136/hrt.2005.075234. PMC 1994453. PMID 17569817.
  3. Di Lullo L, Floccari F, Rivera R, Barbera V, Granata A, Otranto G; et al. (2013). "Pulmonary Hypertension and Right Heart Failure in Chronic Kidney Disease: New Challenge for 21st-Century Cardionephrologists". Cardiorenal Med. 3 (2): 96–103. doi:10.1159/000350952. PMC 3721135. PMID 23922549.
  4. Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K; et al. (2010). "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". J Am Soc Echocardiogr. 23 (7): 685–713, quiz 786-8. doi:10.1016/j.echo.2010.05.010. PMID 20620859.
  5. Bigdelian H, Mardani D, Sedighi M (2015). "The Effect of Pulmonary Valve Replacement (PVR) Surgery on Hemodynamics of Patients Who Underwent Repair of Tetralogy of Fallot (TOF)". J Cardiovasc Thorac Res. 7 (3): 122–5. doi:10.15171/jcvtr.2015.26. PMC 4586599. PMID 26430501.
  6. Bacha EA, Scheule AM, Zurakowski D, Erickson LC, Hung J, Lang P; et al. (2001). "Long-term results after early primary repair of tetralogy of Fallot". J Thorac Cardiovasc Surg. 122 (1): 154–61. doi:10.1067/mtc.2001.115156. PMID 11436049.
  7. Jonas SN, Kligerman SJ, Burke AP, Frazier AA, White CS (2016). "Pulmonary Valve Anatomy and Abnormalities: A Pictorial Essay of Radiography, Computed Tomography (CT), and Magnetic Resonance Imaging (MRI)". J Thorac Imaging. 31 (1): W4–12. doi:10.1097/RTI.0000000000000182. PMID 26656195.
  8. Ansari MM, Cardoso R, Garcia D, Sandhu S, Horlick E, Brinster D; et al. (2015). "Percutaneous Pulmonary Valve Implantation: Present Status and Evolving Future". J Am Coll Cardiol. 66 (20): 2246–55. doi:10.1016/j.jacc.2015.09.055. PMID 26564602.
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  11. Sepulveda, G.; Lukas, D. S. (1955). "The Diagnosis of Tricuspid Insufficiency: Clinical Features in 60 Cases with Associated Mitral Valve Disease". Circulation. 11 (4): 552–563. doi:10.1161/01.CIR.11.4.552. ISSN 0009-7322.
  12. Graziosi M, Rapezzi C (2016). "Right ventricular arrhythmogenic cardiomyopathy: genetic and MR for modern clinical diagnosis". J Cardiovasc Med (Hagerstown). doi:10.2459/JCM.0000000000000470. PMID 27828830.
  13. Choong CY, Abascal VM, Weyman J, Levine RA, Gentile F, Thomas JD; et al. (1989). "Prevalence of valvular regurgitation by Doppler echocardiography in patients with structurally normal hearts by two-dimensional echocardiography". Am Heart J. 117 (3): 636–42. PMID 2784023.
  14. Takao S, Miyatake K, Izumi S, Okamoto M, Kinoshita N, Nakagawa H; et al. (1988). "Clinical implications of pulmonary regurgitation in healthy individuals: detection by cross sectional pulsed Doppler echocardiography". Br Heart J. 59 (5): 542–50. PMC 1276894. PMID 3382565.
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  17. Rebergen SA, Chin JG, Ottenkamp J, van der Wall EE, de Roos A (1993). "Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot. Volumetric quantitation by nuclear magnetic resonance velocity mapping". Circulation. 88 (5 Pt 1): 2257–66. PMID 8222120.
  18. Mercer-Rosa L, Yang W, Kutty S, Rychik J, Fogel M, Goldmuntz E (2012). "Quantifying pulmonary regurgitation and right ventricular function in surgically repaired tetralogy of Fallot: a comparative analysis of echocardiography and magnetic resonance imaging". Circ Cardiovasc Imaging. 5 (5): 637–43. doi:10.1161/CIRCIMAGING.112.972588. PMC 3476467. PMID 22869820.
  19. 19.0 19.1 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.
  20. 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.
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