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Aortic regurgitation in children

David W Brown, MD
Section Editor
David R Fulton, MD
Deputy Editor
Carrie Armsby, MD, MPH


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 [1].

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 [1]. 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 [2]. 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.

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Literature review current through: Nov 2017. | This topic last updated: Sep 07, 2016.
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