Right ventricular failure in congenital heart disease

Abstract

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression, and ventricular remodeling. Pressure-overload RV failure caused by RV outflow tract obstruction after total correction of tetralogy of Fallot, pulmonary stenosis, atrial switch operation for transposition of the great arteries, congenitally corrected transposition of the great arteries, and systemic RV failure after the Fontan operation. Volume-overload RV failure may be caused by atrial septal defect, pulmonary regurgitation, or tricuspid regurgitation. Although the measurement of RV function is difficult because of many reasons, the right ventricle can be evaluated using both imaging and functional modalities. In clinical practice, echocardiography is the primary mode for the evaluation of RV structure and function. Cardiac magnetic resonance imaging is increasingly used for evaluating RV structure and function. A comprehensive evaluation of RV function may lead to early and optimal management of RV failure in patients with CHD.

Keywords: Congenital heart disease; Right ventricle; Right-side heart failure.

Fig. 1

Three-dimensional computed tomography images of a normal heart showing the inlet, trabeculated apical myocardium, and infundibulum of the right ventricle.

Fig. 2

Measurements of the tricuspid annular plane systolic excursion (TAPSE) using M-mode echocardiography at the junction of the tricuspid valve plane with the free wall of the right ventricle.

Fig. 3

Measurement of isovolumic acceleration (IVA) during isovolumic contraction at the level of the tricuspid annulus using a tissue Doppler echocardiography spectral curve. IVV, peak isovolumic velocity; t, time from zero crossing to peak isovolumic velocity. IVA=IVV/t.

Fig. 4

Measurement of right ventricular (RV) myocardial performance index (MPI) using pulsed-wave Doppler at the tip of tricuspid leaflets in the apical 4-chamber view (A) and at the site of just below the pulmonary valve in the RV outflow tract view (B). E, rapid filling velocity; A, atrial filling velocity; a, sum of isovolumic contraction time (IVCT), isovolumic relaxation time (IVRT) and ejection time (ET). RV MPI=(IVRT+IVCT)/ET=(a-ET)/ET.