title: Outcome of children with end-stage congenital heart disease waiting for cardiac transplantation.
abstract: BACKGROUND
  End-stage congenital heart disease (CHD) is a major indication for pediatric cardiac transplantation. The objective of the study was to evaluate pre-transplant 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 pre-transplant mortality.
authors.affiliation: Division of Pediatric Cardiology, College of Physicians and Surgeons, Columbia University, New York, New York 10032, USA. sm364@columbia.edu
authors.forename: Seema
authors.initials: S
authors.lastname: Mital
authors.forename: Linda J
authors.initials: LJ
authors.lastname: Addonizio
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Lamour
authors.forename: Daphne T
authors.initials: DT
authors.lastname: Hsu
mesh_terms: D000206:Actuarial Analysis; D009202:Cardiomyopathies / Q000401:mortality / Q000601:surgery; D016022:Case-Control Studies; D002423:Cause of Death; D002648:Child; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality* / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D012307:Risk Factors; D015996:Survival Rate; D014850:Waiting Lists