ABSTRACT
The clinical presentation of right ventricular (RV) dysfunction due to congenital
heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads,
and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart
function. RV volume load may arise from pretricuspid left to right shunts (e.g., atrial
septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly
of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot).
RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary
arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary
to congenital and postoperative left to right shunts or from defects that cause pulmonary
venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis).
The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities
of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis,
or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale.
Congenital heart disease, therefore, must be included in the differential diagnosis
of patients who present with right ventricular dysfunction.
KEYWORDS
Congenital heart disease - right ventricular function - pulmonary hypertension
