Pulmonic regurgitation causes

Jump to navigation Jump to search

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mugilan Poongkunran M.B.B.S [2] Aravind Kuchkuntla, M.B.B.S[3], Aysha Anwar, M.B.B.S[4], Javaria Anwer M.D.[5]


Pulmonic regurgitation may be normal (physiologic) and occasionally a murmur can be heard among thin subjects. Life-threatening cause of PR include chest trauma. The most common causes of pulmonary regurgitation are following the repair of tetralogy of Fallot and pulmonary stenosis. PR is also common among patients with pulmonary hypertension (PAH). Rheumatic heart disease is also an uncommon cause and is more commonly observed in developing countries. The primary causes of PR include an intrinsic pathology in the pulmonic valve and secondary causes include extrinsic causes. The causes of pulmonic regurgitation may also be reckoned on the basis of the organ systems involved.


Life-threatening causes

  • Life-threatening causes of PR include chest trauma.

Common causes

Less common causes

Causes by etiology

  • PR may be physiologic or pathologic. Pathologic causes may be divided among primary and secondary. Isolated PR is very rare and is most commonly associated with other congenital heart diseases.[10] The following flow chart demonstrates the causes of PR based upon the etiology.

Causes of Pulmonic regurgitation (PR)
Mostly found among healthy young people on doppler echo
Pulmonary artery dilation/
right ventricular outlet aneurysm
(congenital, tertiary syphilis,
Behçet disease, or Marfan syndrome}
Pulmonary hypertension
(SLE can be a cause)
•Quadricuspid or Bicuspid valves
Valvular hypoplasia
pulmonary valve prolapse
Absent pulmonary valve
•Isolated congenital PR
Ebstein’s anomaly (acute PR)
Peripheral pulmonary artery stenosis
Trauma (usually life-threatening)[14]
Blunt or penetrating chest trauma
Myocardial rupture
Infective endocarditis (can be life-threatening)
Rheumatic heart disease
Tumor/Genetic causes[15][18]
Carconoid heart disease
Myxomatous degeneration of the pulmonary valve
•Post pulmonary valve stenosis repair
• Post TOF transannular patch repair
•Post Blalock-Taussig shunt TOF repair
•Post balloon or surgical valvulotomy or valvuloplasty for pulmonary stenosis (acute PR)
•Perforation of valvular pulmonary atresia (acute PR)
Medications acting via serotonergic pathways such as ergot derivatives (Pergolide)[22][23]

Causes by Organ System[14][18][19][20][21][24][4][17][9]

Cardiovascular Congenital heart disease, dilated cardiomyopathy, Eisenmenger syndrome, endocarditis, infective endocarditis, myocardial rupture, patent ductus arteriosus, rheumatic heart disease, right ventricular tumors, myxomatous degeneration of the pulmonary valve, and Takayasu arteritis
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic No underlying causes
Drug Side Effect Cabergoline, ergotamine, fenfluramine, fluoxetine, methysergide, paroxetine, pergolide, phentermine, sertraline
Ear Nose Throat No underlying causes
Endocrine Carcinoid syndrome
Environmental Pneumoconiosis
Gastroenterologic No underlying causes
Genetic Mucopolysaccharidoses, patent ductus arteriosus, polycystic kidney disease, X-linked dilated cardiomyopathy, Marfan syndrome
Hematologic No underlying causes
Iatrogenic Balloon valvuloplasty of the pulmonary valve, cardiac catheterization, cardiopulmonary resuscitation, heart surgery, pacemaker syndrome, percutaneous coronary intervention, prosthetic valve dysfunction, Tetralogy of Fallot repair
Infectious Disease Infective endocarditis, myocarditis, rheumatic fever, tertiary syphilis
Musculoskeletal/Orthopedic No underlying causes
Neurologic No underlying causes
Nutritional/Metabolic Gouty valvular tophi
Obstetric/Gynecologic No underlying causes
Oncologic Carcinoid syndrome
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary Cor pulmonale, idiopathic pulmonary fibrosis, Langerhans cell histiocytosis, pneumoconiosis, pulmonary hypertension, sarcoidosis
Renal/Electrolyte End stage renal disease, polycystic kidney disease
Rheumatology/Immunology/Allergy Rheumatic fever, sarcoidosis, Behçet disease
Sexual No underlying causes
Trauma Blunt or penetrating chest trauma
Urologic No underlying causes
Miscellaneous SLE

Causes in alphabetical order


  1. Fauci, Anthony (2008). Harrison's principles of internal medicine. New York: McGraw-Hill Medical. ISBN 978-0071466332.
  2. Warnes, Carole A.; Williams, Roberta G.; Bashore, Thomas M.; Child, John S.; Connolly, Heidi M.; Dearani, Joseph A.; del Nido, Pedro; Fasules, James W.; Graham, Thomas P.; Hijazi, Ziyad M.; Hunt, Sharon A.; King, Mary Etta; Landzberg, Michael J.; Miner, Pamela D.; Radford, Martha J.; Walsh, Edward P.; Webb, Gary D. (2008). "ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary". Circulation. 118 (23): 2395–2451. doi:10.1161/CIRCULATIONAHA.108.190811. ISSN 0009-7322.
  3. Weinberg, Catherine R.; McElhinney, Doff B. (2014). "Pulmonary Valve Replacement in Tetralogy of Fallot". Circulation. 130 (9): 795–798. doi:10.1161/CIRCULATIONAHA.114.005551. ISSN 0009-7322.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Saremi, Farhood; Gera, Atul; Yen Ho, S.; Hijazi, Ziyad M.; Sánchez-Quintana, Damián (2014). "CT and MR Imaging of the Pulmonary Valve". RadioGraphics. 34 (1): 51–71. doi:10.1148/rg.341135026. ISSN 0271-5333.
  5. Sharma RK, Talwar D, Gupta SK, Bansal S (2016). "Idiopathic dilatation of pulmonary artery". Lung India. 33 (6): 675–677. doi:10.4103/0970-2113.192869. PMC 5112830. PMID 27891002.
  6. 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.
  7. 7.0 7.1 Deb SJ, Zehr KJ, Shields RC (October 2005). "Idiopathic pulmonary artery aneurysm". Ann. Thorac. Surg. 80 (4): 1500–2. doi:10.1016/j.athoracsur.2004.04.011. PMID 16181901.
  8. 8.0 8.1 Xie D, Chen C, Wang H, Xu Z, Jiang G (September 2015). "Refractory pulmonary artery aneurysm in Behçet's disease". Ann Transl Med. 3 (16): 239. doi:10.3978/j.issn.2305-5839.2015.09.27. PMC 4598455. PMID 26539456.
  9. 9.0 9.1 Yang, Jing; Peng, Min; Shi, Juhong; Zheng, Wenjie; Yu, Xuezhong (2019). "Pulmonary artery involvement in Takayasu's arteritis: diagnosis before pulmonary hypertension". BMC Pulmonary Medicine. 19 (1). doi:10.1186/s12890-019-0983-7. ISSN 1471-2466.
  10. 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.
  11. Macchi C, Orlandini SZ, Orlandini GE (January 1994). "An anatomical study of the healthy human heart by echocardiography with special reference to physiological valvular regurgitation". Ann. Anat. 176 (1): 81–6. doi:10.1016/s0940-9602(11)80421-8. PMID 8304596.
  12. Král J, Hradec J, Petrásek J (1989). "Valvular regurgitations in healthy young people". Cor Vasa. 31 (6): 485–94. PMID 2637102.
  13. Maciel BC, Simpson IA, Valdes-Cruz LM, Recusani F, Hoit B, Dalton N, Weintraub R, Sahn DJ (1991). "Color flow Doppler mapping studies of "physiologic" pulmonary and tricuspid regurgitation: evidence for true regurgitation as opposed to a valve closing volume". J Am Soc Echocardiogr. 4 (6): 589–97. doi:10.1016/s0894-7317(14)80218-6. PMID 1760180.
  14. 14.0 14.1 14.2 14.3 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: 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)". Circulation. 118 (23): 2395–451. doi:10.1161/CIRCULATIONAHA.108.190811. PMID 18997168.
  15. 15.0 15.1 15.2 15.3 15.4 Lancellotti, P.; Tribouilloy, C.; Hagendorff, A.; Moura, L.; Popescu, B. A.; Agricola, E.; Monin, J. L.; Pierard, L. A.; Badano, L.; Zamorano, J. L.; Sicari, R.; Vahanian, A.; Roelandt, J. R. T. C. (2010). "European Association of Echocardiography recommendations for the assessment of valvular regurgitation. Part 1: aortic and pulmonary regurgitation (native valve disease)". European Journal of Echocardiography. 11 (3): 223–244. doi:10.1093/ejechocard/jeq030. ISSN 1525-2167.
  16. Tselios K, Gladman DD, Urowitz MB (2017). "Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies". Open Access Rheumatol. 9: 1–9. doi:10.2147/OARRR.S123549. PMC 5191623. PMID 28053559.
  17. 17.0 17.1 Nollen GJ, van Schijndel KE, Timmermans J, Groenink M, Barentsz JO, van der Wall EE, Stoker J, Mulder BJ (May 2002). "Pulmonary artery root dilatation in Marfan syndrome: quantitative assessment of an unknown criterion". Heart. 87 (5): 470–1. doi:10.1136/heart.87.5.470. PMC 1767105. PMID 11997425.
  18. 18.0 18.1 Fox DJ, Khattar RS (October 2004). "Carcinoid heart disease: presentation, diagnosis, and management". Heart. 90 (10): 1224–8. doi:10.1136/hrt.2004.040329. PMC 1768473. PMID 15367531.
  19. 19.0 19.1 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.
  20. 20.0 20.1 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.
  21. 21.0 21.1 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.
  22. Corvol, Jean-Christophe; Anzouan-Kacou, Jean-Baptiste; Fauveau, Elodie; Bonnet, Anne-Marie; Lebrun-Vignes, Bénédicte; Girault, Camille; Agid, Yves; Lechat, Philippe; Isnard, Richard; Lacomblez, Lucette (2007). "Heart Valve Regurgitation, Pergolide Use, and Parkinson Disease". Archives of Neurology. 64 (12): 1721. doi:10.1001/archneur.64.12.1721. ISSN 0003-9942.
  23. Smith SA, Waggoner AD, de las Fuentes L, Davila-Roman VG (August 2009). "Role of serotoninergic pathways in drug-induced valvular heart disease and diagnostic features by echocardiography". J Am Soc Echocardiogr. 22 (8): 883–9. doi:10.1016/j.echo.2009.05.002. PMC 3808845. PMID 19553085.
  24. Curtiss, E I; Miller, T R; Shapiro, L S (1983). "Pulmonic regurgitation due to valvular tophi". Circulation. 67 (3): 699–701. doi:10.1161/01.CIR.67.3.699. ISSN 0009-7322.
  25. Bhattacharyya S, Schapira AH, Mikhailidis DP, Davar J (2009). "Drug-induced fibrotic valvular heart disease". Lancet. 374 (9689): 577–85. doi:10.1016/S0140-6736(09)60252-X. PMID 19683643.
  26. Sharma S, Kirpalani AL, Kulkarni A (2010). "Severe pulmonary hypertension in a young patient with end-stage renal disease on chronic hemodialysis". Ann Pediatr Cardiol. 3 (2): 184–6. doi:10.4103/0974-2069.74055. PMC 3017928. PMID 21234203.
  27. Hendrikx M, Van Dorpe J, Flameng W, Daenen W (1996). "Aortic and mitral valve disease induced by ergotamine therapy for migraine: a case report and review of the literature". J Heart Valve Dis. 5 (2): 235–7. PMID 8665020.
  28. Wilke A, Hesse H, Hufnagel G, Maisch B (1997). "Mitral, aortic and tricuspid valvular heart disease associated with ergotamine therapy for migraine". Eur Heart J. 18 (4): 701. PMID 9129909.
  29. Nadrous HF, Pellikka PA, Krowka MJ, Swanson KL, Chaowalit N, Decker PA; et al. (2005). "Pulmonary hypertension in patients with idiopathic pulmonary fibrosis". Chest. 128 (4): 2393–9. doi:10.1378/chest.128.4.2393. PMID 16236900.
  30. Chaowalit N, Pellikka PA, Decker PA, Aubry MC, Krowka MJ, Ryu JH; et al. (2004). "Echocardiographic and clinical characteristics of pulmonary hypertension complicating pulmonary Langerhans cell histiocytosis". Mayo Clin Proc. 79 (10): 1269–75. doi:10.4065/79.10.1269. PMID 15473409.
  31. Bana DS, MacNeal PS, LeCompte PM, Shah Y, Graham JR (1974). "Cardiac murmurs and endocardial fibrosis associated with methysergide therapy". Am Heart J. 88 (5): 640–55. PMID 4420941.
  32. Nelson J, Shields MD, Mulholland HC (1990). "Cardiovascular studies in the mucopolysaccharidoses". J Med Genet. 27 (2): 94–100. PMC 1016928. PMID 2108248.
  33. Pritchett AM, Morrison JF, Edwards WD, Schaff HV, Connolly HM, Espinosa RE (2002). "Valvular heart disease in patients taking pergolide". Mayo Clin Proc. 77 (12): 1280–6. doi:10.4065/77.12.1280. PMID 12479512.
  34. Adeva M, El-Youssef M, Rossetti S, Kamath PS, Kubly V, Consugar MB; et al. (2006). "Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD)". Medicine (Baltimore). 85 (1): 1–21. doi:10.1097/01.md.0000200165.90373.9a. PMID 16523049.
  35. Arkles JS, Opotowsky AR, Ojeda J, Rogers F, Liu T, Prassana V; et al. (2011). "Shape of the right ventricular Doppler envelope predicts hemodynamics and right heart function in pulmonary hypertension". Am J Respir Crit Care Med. 183 (2): 268–76. doi:10.1164/rccm.201004-0601OC. PMID 20709819.
  36. Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ (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.