Hypoplastic left heart syndrome: palliation without cardiopulmonary bypass.

Our 100% mortality rate with first-stage palliation of hypoplastic left heart syndrome performed with cardiopulmonary bypass led us to a procedure not necessitating bypass. In nine neonates with this congenital heart defect, a woven Dacron graft was placed from the main pulmonary artery to the descending thoracic aorta. The patent ductus arteriosus was ligated and the main pulmonary artery banded distal to the graft and proximal to the bifurcation. Five patients were extubated within 4 days. Only low-dose inotropic support was required in eight of the nine. There were no bleeding problems. Four patients died in the hospital: one of Candida sepsis at 81 days, one of low cardiac output at 2 days, and two of restrictive atrial septal defect at 3 and 5 days. The five living patients were discharged 11 to 80 days postoperatively (mean 38 days). We now perform balloon septostomies preoperatively in all patients and believe that this will improve the survival rate. We believe this simpler approach to the treatment of hypoplastic left heart syndrome may allow survival for a cardiac transplant or a staged Fontan procedure at a later date for more definitive treatment.

Determination of the time of closure of the ductus arteriosus in severely ill premature infants.

In order to investigate the time of ductal closure in the premature infant, we performed multiple echocardiographic examination in each of 42 premature infants. Twenty eight of these infants had spontaneous closure of the ductus arteriosus (median date of closure--3 days). Twelve required medical or surgical closure of the ductus arteriosus and two died of severe lung disease. There was a significant relationship of decreasing birth weight to prolonged patency of the ductus arteriosus.

Intrapericardial teratoma in infancy.

Intrapericardial teratoma in the newborn is a rare potentially fatal neoplasm. Pre-operative diagnosis depends upon recognition of specific radiographic and echocardiographic findings in a newborn with a larg cardiothymic image and pericardial effusion. Surgical intervention is usually curative whereas undiagnosed intrapericardial teratoma is often fatal.

De subitaneis mortibus. X. Familial congential heart block.

Complete heart block was found shortly after birth in a brother and sister (not twins). Both were treated by electronic pacing because of symptoms attributable to inadequate cardiac output and electrical instability of heart. The boy has done well with his artificial pacemaker and is now six years old. His sister died of complications due in part to the large size of her pacemaker and small size of her body. At necropsy special studies of her heart included the centers for normal impulse formation and concuction. The primary abnormalities were at the junction of atrial septum with atrioventricular (A-V) node, and at the origin of the two bundle branches from the His bundle. The A-V node was isolated by collagen at all its margins except its junction with the His bundle. The proximal His bundle was essentially normal, but from that point on through the initial protions of both the left and right bundle branches there was extensive caseous degeneration which interrupted any possible conduction. These findings are discussed in relation to fetal and postnatal development of the human A-V node, and the His bundle and its branches; and in the context of a recently observed mathematical relationship between sinus rate and two forms of experimentally produced A-V junctional escape rhythms.