Aortic regurgitation in children
- David W Brown, MD
David W Brown, MD
- Associate Professor of Pediatrics
- Harvard Medical School
Aortic regurgitation (AR) is a common finding in children undergoing echocardiography. It rarely occurs as an isolated lesion, as it is usually associated with aortic stenosis or a ventricular septal defect. The latter disorders are discussed separately. (See "Valvar aortic stenosis in children" and "Pathophysiology and clinical features of isolated ventricular septal defects in infants and children".)
All forms of AR produce a similar hemodynamic abnormality. The inability of the aortic valve leaflets to remain closed during diastole results in a portion of the left ventricular stroke volume leaking back from the aorta into the left ventricle. The added volume of regurgitant blood produces an increase in left ventricular end-diastolic volume; according to Laplace's law, the increase in left ventricular end-diastolic volume causes an elevation in wall stress. The heart responds with compensatory myocardial hypertrophy, referred to as eccentric left ventricular hypertrophy. Eccentric left ventricular hypertrophy is characterized by elevated left ventricular mass, but normal mass to volume ratio, and returns wall stress towards a normal level .
The combination of hypertrophy and chamber enlargement raises the total stroke volume. The net effect is that forward stroke volume and therefore cardiac output are initially maintained despite the regurgitant lesion. Although the left ventricular volume is increased, end-diastolic pressure is normal due to an increase in ventricular compliance. Thus, the heart initially adapts well to chronic AR, functioning as a very efficient and compliant high output pump.
Severe AR results in increased end-diastolic and end-systolic volumes, and in the eventual failure of compensatory hypertrophy to maintain normal wall stress in the face of increasing volumes and decreased systolic function . Reduced aortic diastolic pressures from excessive runoff, especially during exercise, may impair coronary perfusion, while increased left atrial pressure may lead to pulmonary edema. (See "Clinical manifestations and diagnosis of chronic aortic regurgitation in adults".)
There are many etiologies of AR (AR), which rarely occurs as an isolated lesion . A bicuspid aortic valve is a common cause. AR also occurs in children with Marfan syndrome, but, as described below, is more common in adults in association with progressive aortic root dilation (see "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders"). Rheumatic heart disease was an important etiology in the past, but is now uncommon in developed countries. Other associations include Turner syndrome, osteogenesis imperfecta, subaortic stenosis, prolapse of an aortic leaflet through a ventricular septal defect (typically membranous or subpulmonary), tetralogy of Fallot, and truncus arteriosus.
- Latson LA. Aortic stenosis: valvar, supravalvar, and fibromuscular subvalvar. In: The Science and Practice of Pediatric Cardiology, Garson A, Bricker JT, Fisher DJ, Neish SR (Eds), Williams and Wilkins, Baltimore 1998. p.1257.
- Donofrio MT, Engle MA, O'Loughlin JE, et al. Congenital aortic regurgitation: natural history and management. J Am Coll Cardiol 1992; 20:366.
- Moore P, Egito E, Mowrey H, et al. Midterm results of balloon dilation of congenital aortic stenosis: predictors of success. J Am Coll Cardiol 1996; 27:1257.
- Choudhry NK, Etchells EE. The rational clinical examination. Does this patient have aortic regurgitation? JAMA 1999; 281:2231.
- Cheitlin MD. Finding 'just the right moment' for operative intervention in the asymptomatic patient with moderate to severe aortic regurgitation. Circulation 1998; 97:518.
- van Karnebeek CD, Naeff MS, Mulder BJ, et al. Natural history of cardiovascular manifestations in Marfan syndrome. Arch Dis Child 2001; 84:129.
- Bruno L, Tredici S, Mangiavacchi M, et al. Cardiac, skeletal, and ocular abnormalities in patients with Marfan's syndrome and in their relatives. Comparison with the cardiac abnormalities in patients with kyphoscoliosis. Br Heart J 1984; 51:220.
- Rhodes LA, Keane JF, Keane JP, et al. Long follow-up (to 43 years) of ventricular septal defect with audible aortic regurgitation. Am J Cardiol 1990; 66:340.
- Lin M, Chiang HT, Lin SL, et al. Vasodilator therapy in chronic asymptomatic aortic regurgitation: enalapril versus hydralazine therapy. J Am Coll Cardiol 1994; 24:1046.
- Schön HR, Dorn R, Barthel P, Schömig A. Effects of 12 months quinapril therapy in asymptomatic patients with chronic aortic regurgitation. J Heart Valve Dis 1994; 3:500.
- Wisenbaugh T, Sinovich V, Dullabh A, Sareli P. Six month pilot study of captopril for mildly symptomatic, severe isolated mitral and isolated aortic regurgitation. J Heart Valve Dis 1994; 3:197.
- Scognamiglio R, Fasoli G, Ponchia A, Dalla-Volta S. Long-term nifedipine unloading therapy in asymptomatic patients with chronic severe aortic regurgitation. J Am Coll Cardiol 1990; 16:424.
- Scognamiglio R, Rahimtoola SH, Fasoli G, et al. Nifedipine in asymptomatic patients with severe aortic regurgitation and normal left ventricular function. N Engl J Med 1994; 331:689.
- Greenberg B, Massie B, Bristow JD, et al. Long-term vasodilator therapy of chronic aortic insufficiency. A randomized double-blinded, placebo-controlled clinical trial. Circulation 1988; 78:92.
- Alehan D, Ozkutlu S. Beneficial effects of 1-year captopril therapy in children with chronic aortic regurgitation who have no symptoms. Am Heart J 1998; 135:598.
- Nishimura RA, Otto CM, Bonow RO, 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. Circulation 2014; 129:e521.
- Bacha EA, Satou GM, Moran AM, et al. Valve-sparing operation for balloon-induced aortic regurgitation in congenital aortic stenosis. J Thorac Cardiovasc Surg 2001; 122:162.
- Keane JF, Fyler DC. Aortic outflow abnormalities. In: Nadas' Pediatric Cardiology, Keane JF, Lock JE, Fyler DC (Eds), Saunders/Elsevier, Philadelphia 2006. p.598.
- Bacha EA, McElhinney DB, Guleserian KJ, et al. Surgical aortic valvuloplasty in children and adolescents with aortic regurgitation: acute and intermediate effects on aortic valve function and left ventricular dimensions. J Thorac Cardiovasc Surg 2008; 135:552.
- Walker WE, Duncan JM, Frazier OH Jr, et al. Early experience with the ionescu-shiley pericardial xenograft valve. Accelerated calcification in children. J Thorac Cardiovasc Surg 1983; 86:570.
- Kirklin JK, Smith D, Novick W, et al. Long-term function of cryopreserved aortic homografts. A ten-year study. J Thorac Cardiovasc Surg 1993; 106:154.
- Ohye RG, Gomez CA, Ohye BJ, et al. The Ross/Konno procedure in neonates and infants: intermediate-term survival and autograft function. Ann Thorac Surg 2001; 72:823.
- Laforest I, Dumesnil JG, Briand M, et al. Hemodynamic performance at rest and during exercise after aortic valve replacement: comparison of pulmonary autografts versus aortic homografts. Circulation 2002; 106:I57.
- Laudito A, Brook MM, Suleman S, et al. The Ross procedure in children and young adults: a word of caution. J Thorac Cardiovasc Surg 2001; 122:147.