Pulmonic regurgitation natural history, complications and prognosis
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Pulmonic regurgitation Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1],Associate Editor(s)-in-Chief: , Aravind Kuchkuntla, M.B.B.S[2], Aysha Anwar, M.B.B.S[3], Javaria Anwer M.D.[4]
Overview
The majority of patients with mild pulmonary regurgitation (PR) are asymptomatic and have a benign course, not progressing to chronic PR. Patients tolerate severe chronic PR for a long period of time and begin to develop symptoms when the right ventricle function begins to decline. Chronic severe PR leads to progressive dilation and systolic dysfunction of the right ventricle resulting in symptoms. The severity of PR after TOF repair can increase over time and patients may develop symptoms from an early age. Complications that may result from PR include progressive right ventricular dilatation, heart failure, tricuspid regurgitation, ventricular arrhythmias, and sudden cardiac death. The prognosis of pulmonic regurgitation depends on the severity of the condition, etiology, and associated complications. Symptomatic patients are treated with pulmonary valve replacement (PVR) and have a good prognosis.
Natural History, Complications, and Prognosis
Natural history
- Mild PR[1]:
- Acute worsening of PR:
- Patients with acute worsening of PR should be evaluated for associated conditions such as pulmonary hypertension which increase the pressure gradient.
- Chronic PR[2]:
- Patients tolerate severe chronic PR for a long period of time and begin to develop symptoms when the right ventricular systolic function begins to decline or marked dilatation occur. In a symptomatic patient RV dysfunction has usually become irreversible.
- Chronic severe PR leads to progressive dilation and systolic dysfunction of the RV(right ventricle) resulting in symptoms.
- Isolated congenital PR[3]:
- Post TOF repair[4][5]:
- Among patients with TOF repair, symptoms may develop at an early age. Thie finding is partly attributed to the effect of associated lesions on myocardial function. Post complete TOF repair, impaired exercise capacity is directly related to the degree of residual PR.
- The severity of PR after TOF repair can increase over time. The data is supported by experimental[6][7] and clinical[8] evidence.
- Idiopathic Dilatation of Pulmonary Artery (IDPA)[9][10]:
- The condition is usually asymptomatic but may demonstrate symptoms in case of development of complications. The duration of illness has been demonstrated to be more than 20 years among 10% patients.
- Dilated pulmonary arteries may lead to the compression of left main coronary artery.
Complications
- Common complications of pulmonary regurgitation (PR) include:[11][12][13][14][15][5]
- Progressive right ventricular(RV) dilatation
- RV dysfunction
- Heart failure (HF): Exercise intolerance is one of the features of HF. To read about the degree of HF and its manifestations according to NYHA classification click here.
- Tricuspid regurgitation
- Ventricular arrhythmias (such as ventricular tachycardia)
- Hepatic congestion may develop secondary to right heart failure. Hepatic dysfunction may ensue thromboembolic events.
- Sudden cardiac death:
- Among patients with repaired TOF, pulmonic regurgitation is a major hemodynamic lesion associated with ventricular tachycardia and sudden cardiac death.
- Sudden cardiac death among patients with IDPA is due to the compression of left main coronary artery.[9][16]
Prognosis
- The prognosis of pulmonic regurgitation depends on the severity of the condition, etiology and associated complications.[17]
- Mild to moderate PR is not associated with shortened survival.[17]
- The prognosis of symptomatic patients of PR is good after pulmonary valve replacement.[18] Surgical Pulmonic Valve Repair (PVR) improves right ventricular filling and increases left ventricular stroke volume.[19] Ten year overall and event-free survival after PVR is 98% and 70%, respectively.[20]
- Among patients with pulmonary hypertension (PAH), the severity and duration of PAH determines the ultimate prognosis.
- The prognosis of PR due to congenital absence of pulmonic valve is poor and may limit patient's life expectancy in the absence of valve replacement. Absent pulmonary valve (APVS) is associated with severe regurgitation and complications secondary to respiratory distress.[21]
- Higher pre-operative RV end-systolic volume index (ESVI) is the only independent risk factor for suboptimal outcomes post pulmonary valve replacement.[20]
- QRS duration of ⩾180 ms on resting EKG has been demonstrated to be a strong predictor of ventricular arrhythmias and sudden death among patients post TOF repair.[22]
References
- ↑ 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.
- ↑ Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ (March 2004). "Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging". J. Am. Coll. Cardiol. 43 (6): 1068–74. doi:10.1016/j.jacc.2003.10.045. PMID 15028368.
- ↑ Shimazaki Y, Blackstone EH, Kirklin JW (August 1984). "The natural history of isolated congenital pulmonary valve incompetence: surgical implications". Thorac Cardiovasc Surg. 32 (4): 257–9. doi:10.1055/s-2007-1023399. PMID 6207619.
- ↑ Carvalho JS, Shinebourne EA, Busst C, Rigby ML, Redington AN (June 1992). "Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of residual pulmonary regurgitation". Br Heart J. 67 (6): 470–3. doi:10.1136/hrt.67.6.470. PMC 1024889. PMID 1622697.
- ↑ 5.0 5.1 Bouzas, Beatriz; Kilner, Philip J.; Gatzoulis, Michael A. (2005). "Pulmonary regurgitation: not a benign lesion". European Heart Journal. 26 (5): 433–439. doi:10.1093/eurheartj/ehi091. ISSN 0195-668X.
- ↑ Kuehne T, Saeed M, Reddy G, Akbari H, Gleason K, Turner D, Teitel D, Moore P, Higgins CB (November 2001). "Sequential magnetic resonance monitoring of pulmonary flow with endovascular stents placed across the pulmonary valve in growing Swine". Circulation. 104 (19): 2363–8. doi:10.1161/hc4401.098472. PMID 11696479.
- ↑ Kuehne T, Saeed M, Gleason K, Turner D, Teitel D, Higgins CB, Moore P (October 2003). "Effects of pulmonary insufficiency on biventricular function in the developing heart of growing swine". Circulation. 108 (16): 2007–13. doi:10.1161/01.CIR.0000092887.84425.09. PMID 14557371.
- ↑ Siwek LG, Applebaum RE, Jones M, Clark RE (September 1985). "Acute control of pulmonary regurgitation with a balloon "valve". An experimental investigation". J. Thorac. Cardiovasc. Surg. 90 (3): 404–9. PMID 4033177.
- ↑ 9.0 9.1 Choi YJ, Kim U, Lee JS, Park WJ, Lee SH, Park JS, Shin DG, Kim YJ (October 2013). "A case of extrinsic compression of the left main coronary artery secondary to pulmonary artery dilatation". J. Korean Med. Sci. 28 (10): 1543–8. doi:10.3346/jkms.2013.28.10.1543. PMC 3792613. PMID 24133364.
- ↑ 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.
- ↑ Gregg D, Foster E (2007). "Pulmonary insufficiency is the nexus of late complications in tetralogy of Fallot". Curr Cardiol Rep. 9 (4): 315–22. PMID 17601398.
- ↑ Helbing WA, Roest AA, Niezen RA, Vliegen HW, Hazekamp MG, Ottenkamp J; et al. (2002). "ECG predictors of ventricular arrhythmias and biventricular size and wall mass in tetralogy of Fallot with pulmonary regurgitation". Heart. 88 (5): 515–9. PMC 1767425. PMID 12381647.
- ↑ Frigiola A, Redington AN, Cullen S, Vogel M (2004). "Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot". Circulation. 110 (11 Suppl 1): II153–7. doi:10.1161/01.CIR.0000138397.60956.c2. PMID 15364855.
- ↑ Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J; et al. (2010). "Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study". Circulation. 122 (9): 868–75. doi:10.1161/CIRCULATIONAHA.109.928481. PMID 20713900.
- ↑ Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD, Redington AN (September 2000). "Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study". Lancet. 356 (9234): 975–81. doi:10.1016/S0140-6736(00)02714-8. PMID 11041398.
- ↑ Andrews R, Colloby P, Hubner PJ (March 1993). "Pulmonary artery dissection in a patient with idiopathic dilatation of the pulmonary artery: a rare cause of sudden cardiac death". Br Heart J. 69 (3): 268–9. doi:10.1136/hrt.69.3.268. PMC 1024995. PMID 8461230.
- ↑ 17.0 17.1 Pendela VS, Ayyad R. PMID 31985929. Missing or empty
|title=(help) - ↑ 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.
- ↑ Frigiola, A.; Giardini, A.; Taylor, A.; Tsang, V.; Derrick, G.; Khambadkone, S.; Walker, F.; Cullen, S.; Bonhoeffer, P.; Marek, J. (2012). "Echocardiographic assessment of diastolic biventricular properties in patients operated for severe pulmonary regurgitation and association with exercise capacity". European Heart Journal - Cardiovascular Imaging. 13 (8): 697–702. doi:10.1093/ehjci/jes002. ISSN 2047-2404.
- ↑ 20.0 20.1 Lee, Cheul; Kim, Yang Min; Lee, Chang-Ha; Kwak, Jae Gun; Park, Chun Soo; Song, Jin Young; Shim, Woo-Sup; Choi, Eun Young; Lee, Sang Yun; Baek, Jae Suk (2012). "Outcomes of Pulmonary Valve Replacement in 170 Patients With Chronic Pulmonary Regurgitation After Relief of Right Ventricular Outflow Tract Obstruction". Journal of the American College of Cardiology. 60 (11): 1005–1014. doi:10.1016/j.jacc.2012.03.077. ISSN 0735-1097.
- ↑ Grewal DS, Chamoli SC, Saxena S (April 2014). "Absent pulmonary valve syndrome - Antenatal diagnosis". Med J Armed Forces India. 70 (2): 198–200. doi:10.1016/j.mjafi.2013.07.002. PMC 4017172. PMID 24843213.
- ↑ Gatzoulis MA, Till JA, Somerville J, Redington AN (July 1995). "Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death". Circulation. 92 (2): 231–7. doi:10.1161/01.cir.92.2.231. PMID 7600655.