Outcome of children with end-stage congenital heart disease waiting for cardiac transplantation

Background: End-stage congenital heart disease (CHD) is a major indication for pediatric cardiac transplantation. The objective of the study was to evaluate pretransplant outcome of children with CHD. Methods: The clinical profile and outcome of patients with CHD <20 years of age listed for transplantation (1993 to 1999) were reviewed and patients who died waiting (Group I) were compared with survivors to transplant (Group II). Results: Mean age of the patients (n = 46) was 8.3 +/- 8 years. Primary indications for transplant were ventricular dysfunction in 36 (78%), failed Fontan in 8 (18%) and severe hypoxemia in 2 (4%) patients. Thirty-two patients were Status 1 (70%), 14 were Status 2 and 5 patients were de-listed. Twenty-nine of the 41 patients that remained listed survived to transplant, 12 (29%) died waiting. Causes of death were sepsis in 2 and severe heart failure (HF) in 10 patients. Eight patients died with multi-system organ failure, including 3 on mechanical circulatory support. Mean time to death was 29 +/- 28 days and time to transplant was 94 +/- 176 days. Mean age at listing was younger in Group I (2.6 +/- 4 years) compared with Group II (9.1 +/- 7 years, p < 0.05). Mean HF duration was shorter in Group I (3.6 +/- 3.9 months) compared with Group II (25 +/- 33 months, p < 0.05). Fifty-day actuarial survival on the waiting list was lower in infants (38%) compared with older children (91%, p < 0.05). In contrast to the high mortality (71%) in infants with CHD, all infants with cardiomyopathy survived to transplant. Conclusions: Seventy-one percent of patients listed with CHD survived to transplant. Younger age at listing and rapid onset of HF were significant risk factors for pretransplant mortality.