Abstract
Complex unroofed coronary sinus with a persistent left superior vena cava has as its
commonest major associated intracardiac anomaly a partial or complete atrioventricular
canal defect. In this clinical setting, biventricular repair with construction of
a complex intra-atrial baffle from the pulmonary veins to the mitral valve has a reported
mortality rate of as high as 50%. Looking for an improvement, we have carried out
an extracardiac repair of the anomalous systemic venous component with atrial septation.
In 2 infants (aged 7 and 12 weeks) with unroofed coronary sinus, bilateral superior
venae cavae, right isomerism, and complete atrioventricular canal, in addition to
patch closure of the ventricular component of the atrioventricular septal defect,
a baffle was constructed between the pulmonary veins and the mitral valve. In four
subsequent infants (aged 7,10,16, and 20 weeks) with unroofed coronary sinus, bilateral
superior venae cavae, complete atrioventricular canal, right isomerism (n = 2), and
mild infundibular stenosis (n = 1), repair consisted of end-to-side anastomosis of
the left superior vena cava to the right superior vena cava and complete repair of
the atrioventricular canal and associated conditions. There was no mortality. The
early postoperative course in the two patients with intra-atrial baffle was characterized
by increased left-atrial pressure (18 and 20mmHg), with varying degrees of pulmonary
venous congestion, supraventricular tachycardias, and systemic hypotension. The pulmonary
venous congestion increased, so that one patient was successfully converted 10 weeks
postoperatively to an extracardiac repair with septation of the atria and the other
will probably follow. In the 4 patients with a primary extracardiac repair, the hemodynamic
result was excellent, with a median leftatrial pressure of 11 mmHg on the first postoperative
day. At a median follow-up of 12 months, all 5 patients with an extracardiac repair
are clinically well with widely patent anastomoses between the left and right superior
venae cavae. The extracardiac repair technique for complex unroofed coronary sinus,
as opposed to the intra-atrial baffle repair, avoids creation of a small and low-compliance
left-atrial compartment with the potential for development of pulmonary venous congestion.
Key words
Unroofed coronary sinus - Left superior vena cava - Atrioventricular canal
