Longitudinal results after first-stage palliation for hypoplastic left heart syndrome

To evaluate the results of palliative surgery for hypoplastic left heart syndrome, we reviewed the records of 57 infants who underwent first-stage reconstruction at our institution between July 1983 and April 1989. Of the 57 infants, 12 (21%) are long-term survivors and 45 (79%) have died. Thirty-one infants died within the first 30 days after surgery. Twenty-six of the 31 early deaths occurred within the first 24 hours after surgery. Causes of early mortality were low cardiac output (23), sepsis (two), sudden death (two), pulmonary vein atresia (three), and cardiac transplant (one). Late death occurred in 14 infants due to sepsis (three), sudden death (four), and death at reoperation (seven - three after Fontan procedure, three after shunt replacement, and one after transplant). Of the 31 patients who survived more than 24 hours, the complications noted by echocardiography and confirmed by catheterization when reoperation was indicated were significant arch obstruction (13%), branch pulmonary artery stenosis (23%), small atrial septal defect (16%), inadequate shunt (26%), neoaortic regurgitation (13%), tricuspid regurgitation (13%), ventricular dysfunction (29%), thrombus (6%), and superior vena cava obstruction (3%). Of the 31 patients who survived more than 24 hours, 16 additional palliative surgical procedures were performed in eight patients. These procedures included arch reconstruction (four), additional shunt (four), Glenn shunt (three), atrial septectomy (two), coarctation balloon angioplasty (two), and pulmonary artery reconstruction and reshunting (one). Of the 12 long-term survivors, four have had a successful Fontan procedure, one has had a transplant, and seven are awaiting a second-stage procedure. Thus, 69% of all deaths occurred within the first 30 days of surgery, and 58% of all deaths occurred within the first 24 hours due to cardiovascular collapse.