Bidirectional cavopulmonary anastomosis with intracardiac repair

Patients with hypoplasia of the right ventricle and tricuspid valve have historically undergone a total cavopulmonary connection or a two-ventricle repair with atrial fenestration.

We reviewed our experience with 9 patients with hypoplasia of the right ventricle and tricuspid valve who underwent a bidirectional cavopulmonary anastomosis with intracardiac repair. Patient diagnoses included pulmonary atresia with intact ventricular septum (n = 3); hypoplastic right ventricle and tricuspid valve with atrial septal defect, ventricular septal defect, and right ventricular outflow tract obstruction (n = 3); unbalanced atrioventricular canal defect (n = 1); inlet ventricular septal defect with coarctation (n = 1); and tricuspid stenosis with atrial septal defect (n = 1).

The median age at operation was 36 months. There was hypoplasia of the right ventricle and tricuspid valve in all patients. The tricuspid valve measured 56.5% of normal (range, 43.6% to 70.4%) by echocardiography, and the median ratio of the tricuspid valve to the mitral valve was 0.67 (range, 0.54 to 0.82). At operation, the median tricuspid valve annulus diameter was 65.6% of published autopsy normals (range, 57.8% to 78.5%) with a median Z value of -3.8 (range, -6.6 to -2.1). All patients survived operation. At a median follow-up of 16 months, 5 patients are asymptomatic, and 2 have occasional early-morning periorbital edema. Two patients are on a regimen of diuretics, 1 of whom is also taking an unloading agent. The patient with unbalanced atrioventricular canal died suddenly at home 6 months postoperatively.

Bidirectional cavopulmonary anastomosis with intracardiac repair may avoid the long-term complications associated with the Fontan modifications and eliminates the need of atrial fenestration in most instances. This operation should be considered for select patients with hypoplasia of the right ventricle and tricuspid valve.