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

Seema Mital, Linda J. Addonizio, Jacqueline M. Lamour, Daphne T. Hsu

Research output: Contribution to journalArticle

36 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)147-153
Number of pages7
JournalJournal of Heart and Lung Transplantation
Volume22
Issue number2
DOIs
StatePublished - Feb 1 2003
Externally publishedYes

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Heart Transplantation
Heart Diseases
Transplants
Heart Failure
Ventricular Dysfunction
Waiting Lists
Mortality
Cardiomyopathies
Survivors
Cause of Death
Sepsis
Transplantation
Pediatrics
Survival

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Transplantation

Cite this

Outcome of children with end-stage congenital heart disease waiting for cardiac transplantation. / Mital, Seema; Addonizio, Linda J.; Lamour, Jacqueline M.; Hsu, Daphne T.

In: Journal of Heart and Lung Transplantation, Vol. 22, No. 2, 01.02.2003, p. 147-153.

Research output: Contribution to journalArticle

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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.",
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