Diagnostic Considerations

As with any cardiovascular diagnosis, it is important to document the presence and extent of pulmonary or pulmonic regurgitation (PR), the treatment options considered and undertaken (including antibiotic prophylaxis for dental and other procedures), and the plans for follow-up care.

Other conditions to be considered in patients with suspected PR include the following:

Marfan syndrome
Rheumatic heart disease
Carcinoid heart disease
Aortic insufficiency: The Graham Steell murmur of pulmonary hypertension is identical in quality to that of the early diastolic murmur in aortic insufficiency. They must be differentiated.

Pregnancy

Pregnancy is usually well tolerated in patients with PR, including those who have had surgical repairs for tetralogy of Fallot, except when right-sided heart failure is present that cannot be medically controlled.

Infective endocarditis

Pulmonic valve endocarditis is almost always associated with immunosuppressed states, intravenous/injection drug abuse, and/or congenital heart disease. The risk of endocarditis is thought to chiefly depend on two factors: the presence of high-velocity flow that injures the endothelium by shear forces or the jet impact and exposure to infective organisms. In a series of 186 patients from a congenital heart disease registry with varying degrees of pulmonary and/or tricuspid regurgitation and normal pulmonic and tricuspid valves, investigators observed that the occurrence of pulmonic and tricuspid valve endocarditis was extremely low.^[15]The study, however, was not large enough to resolve the question of endocarditis risk in this group, and the authors still advised antibiotic prophylaxis against endocarditis.

The American Heart Association (AHA) recommendations on prevention of infective endocarditis do not support the necessity for antibiotic prophylaxis in pulmonic regurgitation for otherwise structurally normal pulmonic valves, especially if no diastolic murmur is audible (see Infective Endocarditis). However, PR in congenital heart malformations, acquired valvular dysfunction as in rheumatic heart disease, complex cyanotic heart disease, prosthetic valves, and prior bacterial endocarditis comprise moderate- to high–risk conditions that warrant antibiotic prophylaxis.^{[16, 17, 18]}

Differential Diagnoses

Infective Endocarditis
Idiopathic Pulmonary Arterial Hypertension
Pulmonary Arterial Hypertension
Syphilis
Tetralogy of Fallot

Workup

[Guideline] Nishimura RA, Otto CM, Bonow RO, et al, for the American College of Cardiology., American College of Cardiology/American Heart Association., et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Thorac Cardiovasc Surg. 2014 Jul. 148 (1):e1-e132. [QxMD MEDLINE Link].
Hamza N, Ortiz J, Bonomo RA. Isolated pulmonic valve infective endocarditis: a persistent challenge. Infection. 2004 Jun. 32 (3):170-5. [QxMD MEDLINE Link].
Renella P, Aboulhosn J, Lohan DG, et al. Two-dimensional and Doppler echocardiography reliably predict severe pulmonary regurgitation as quantified by cardiac magnetic resonance. J Am Soc Echocardiogr. 2010 Aug. 23 (8):880-6. [QxMD MEDLINE Link].
Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993 Aug 26. 329 (9):593-9. [QxMD MEDLINE Link].
Liu S, Xu X, Liu G, Ding X, Zhao X, Qin Y. Comparison of immediate and long-term results between the single balloon and inoue balloon techniques for percutaneous pulmonary valvuloplasty. Heart Lung Circ. 2015 Jan. 24 (1):40-5. [QxMD MEDLINE Link].
Eyskens B, Brown SC, Claus P, et al. The influence of pulmonary regurgitation on regional right ventricular function in children after surgical repair of tetralogy of Fallot. Eur J Echocardiogr. 2010 May. 11 (4):341-5. [QxMD MEDLINE Link].
Harrild DM, Powell AJ, Tran TX, et al. Long-term pulmonary regurgitation following balloon valvuloplasty for pulmonary stenosis risk factors and relationship to exercise capacity and ventricular volume and function. J Am Coll Cardiol. 2010 Mar 9. 55 (10):1041-7. [QxMD MEDLINE Link].
Pellikka PA, Tajik AJ, Khandheria BK, et al. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 1993 Apr. 87 (4):1188-96. [QxMD MEDLINE Link].
Nollert G, Fischlein T, Bouterwek S, Bohmer C, Klinner W, Reichart B. Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol. 1997 Nov 1. 30 (5):1374-83. [QxMD MEDLINE Link].
Hickey EJ, Veldtman G, Bradley TJ, et al. Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades. Eur J Cardiothorac Surg. 2009 Jan. 35 (1):156-64; discussion 164. [QxMD MEDLINE Link].
Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000 Sep 16. 356 (9234):975-81. [QxMD MEDLINE Link].
Nollert G, Fischlein T, Bouterwek S, et al. Long-term results of total repair of tetralogy of Fallot in adulthood: 35 years follow-up in 104 patients corrected at the age of 18 or older. Thorac Cardiovasc Surg. 1997 Aug. 45 (4):178-81. [QxMD MEDLINE Link].
Khairy P, Aboulhosn J, Gurvitz MZ, et al, for the Alliance for Adult Research in Congenital Cardiology (AARCC). Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010 Aug 31. 122 (9):868-75. [QxMD MEDLINE Link].
Lee C, Kim YM, Lee CH, et al. 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. 2012 Sep 11. 60 (11):1005-14. [QxMD MEDLINE Link].
Dodo H, Perloff JK, Child JS, et al. Are high-velocity tricuspid and pulmonary regurgitation endocarditis risk substrates?. Am Heart J. 1998 Jul. 136(1):109-14. [QxMD MEDLINE Link].
[Guideline] Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2017 Jun 20. 135 (25):e1159-95. [QxMD MEDLINE Link].
[Guideline] Warnes CA, Williams RG, Bashore TM, et al. 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). Circulation. 2008 Dec 2. 118 (23):e714-833. [QxMD MEDLINE Link].
[Guideline] American Heart Association. Infective endocarditis. Available at http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/TheImpactofCongenitalHeartDefects/Infective-Endocarditis_UCM_307108_Article.jsp#.WzFNJVVKiHs. Updated: March 29, 2018; Accessed: June 25, 2018.
Kitagawa A, Oka N, Kimura S, et al. Clinical utility of the plasma brain natriuretic peptide level in monitoring tetralogy of Fallot patients over the long term after initial intracardiac repair: considerations for pulmonary valve replacement. Pediatr Cardiol. 2014 Dec 12. [QxMD MEDLINE Link].
Ristow B, Ahmed S, Wang L, Liu H, Angeja BG, Whooley MA. Pulmonary regurgitation end-diastolic gradient is a Doppler marker of cardiac status: data from the Heart and Soul Study. J Am Soc Echocardiogr. 2005 Sep. 18(9):885-91. [QxMD MEDLINE Link].
Ristow B, Ali S, Ren X, Whooley MA, Schiller NB. Elevated pulmonary artery pressure by Doppler echocardiography predicts hospitalization for heart failure and mortality in ambulatory stable coronary artery disease: the Heart and Soul Study. J Am Coll Cardiol. 2007 Jan 2. 49(1):43-9. [QxMD MEDLINE Link].
Kutty S, Valente AM, White MT, et al. Usefulness of pulmonary arterial end-diastolic forward flow late after tetralogy of Fallot repair to predict a "restrictive" right ventricle. Am J Cardiol. 2018 Jun 1. 121 (11):1380-6. [QxMD MEDLINE Link].
[Guideline] El-Harasis MA, Connolly HM, Miranda WR, et al. Progressive right ventricular enlargement due to pulmonary regurgitation: Clinical characteristics of a "low-risk" group. Am Heart J. 2018 Jul. 201:136-40. [QxMD MEDLINE Link].
Lancellotti P, Tribouilloy C, Hagendorff A, et al for the European Association of Echocardiography. European Association of Echocardiography recommendations for the assessment of valvular regurgitation. Part 1: aortic and pulmonary regurgitation (native valve disease). Eur J Echocardiogr. 2010 Apr. 11 (3):223-44. [QxMD MEDLINE Link].
Jones MI, Qureshi SA. Recent advances in transcatheter management of pulmonary regurgitation after surgical repair of tetralogy of Fallot. F1000Res. 2018. 7:[QxMD MEDLINE Link]. [Full Text].
[Guideline] American Heart Association Task Force on Practice Guidelines. 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 Interve... J Am Coll Cardiol. 2008 Dec 2. 52 (23):e143-263. [QxMD MEDLINE Link].
McElhinney DB, Hellenbrand WE, Zahn EM, et al. Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial. Circulation. 2010 Aug 3. 122 (5):507-16. [QxMD MEDLINE Link].
Egbe AC, Connolly HM, Pellikka PA, et al. Outcomes of warfarin therapy for bioprosthetic valve thrombosis of surgically implanted valves: a prospective study. JACC Cardiovasc Interv. 2017 Feb 27. 10 (4):379-87. [QxMD MEDLINE Link].
[Guideline] Jamieson WR, Cartier PC, Allard M, et al. Surgical management of valvular heart disease 2004. Can J Cardiol. 2004 Oct. 20 suppl E:7E-120E. [QxMD MEDLINE Link].
[Guideline] Baumgartner H, Falk V, Bax JJ, et al, for the ESC Scientific Document Group. 2017 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2017 Sep 21. 38 (36):2739-91. [QxMD MEDLINE Link].

Media Gallery

Continuous-wave signal of transpulmonary flow in severe pulmonary regurgitation. Courtesy of Wikimedia Commons [AMC Echolab, AMC, The Netherlands].

of 1

Author

Tarek Ajam, MD, MS Fellow in Cardiovascular Medicine, Aurora Health Care

Tarek Ajam, MD, MS is a member of the following medical societies: American College of Cardiology, American College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Tarek Helmy, MD, FACC, FSCAI Professor of Medicine, Division of Cardiology, Director of Cardiac Catheterization Laboratory, University of Cincinnati School of Medicine

Tarek Helmy, MD, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Marschall S Runge, MD, PhD Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Texas Medical Association, Southern Society for Clinical Investigation, American Federation for Clinical Research, Association of Professors of Medicine, Association of Professors of Cardiology, American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Medical Research, American Heart Association

Disclosure: Received honoraria from Pfizer for speaking and teaching; Received honoraria from Merck for speaking and teaching; Received consulting fee from Orthoclinica Diagnostica for consulting.

Chief Editor

Richard A Lange, MD, MBA President, Texas Tech University Health Sciences Center, Dean, Paul L Foster School of Medicine

Richard A Lange, MD, MBA is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, Association of Subspecialty Professors

Disclosure: Nothing to disclose.

Additional Contributors

Lauralyn B Cannistra, MD, FACC Director of Echocardiography Lab and Cardiac Rehabilitation, Assistant Professor, Department of Medicine, Memorial Hospital of Rhode Island, Brown University School of Medicine

Lauralyn B Cannistra, MD, FACC is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Anesthesiologists, American Society of Echocardiography, Rhode Island Medical Society

Disclosure: Nothing to disclose.

Xiushui (Mike) Ren, MD Cardiologist, The Permanente Medical Group; Associate Director of Research, Cardiovascular Diseases Fellowship, California Pacific Medical Center

Xiushui (Mike) Ren, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Society of Echocardiography

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous author Pablo J Saavedra, MD, to the development and writing of this article.

Sections Pulmonary Regurgitation (Pulmonic Regurgitation)
Overview
Presentation
DDx
Workup
Treatment
Medication
References

Pulmonary Regurgitation (Pulmonic Regurgitation) Differential Diagnoses

Diagnostic Considerations

Pregnancy

Infective endocarditis

Differential Diagnoses

References

Tables

Contributor Information and Disclosures

What would you like to print?