TITLE:
The Outcome of Infants and Children with Heart Muscle Disease: Prognosis in Centers without Heart Transplantation Option
AUTHORS:
L. Kampel, Z. Perles, A. J. J. T. Rein, N. El-Lahham, J. Braun, S. Gavri, J. Golender, A. Nir
KEYWORDS:
Pediatrics; Cardiomyopathy; Heart Failure; Survival; Transplantation
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.4 No.1,
March
6,
2014
ABSTRACT: Objective: Acute
heart failure due to heart muscle disease is potentially fatal in pediatric
patients. Heart transplantation (Tx) is utilized to improve survival. However,
spontaneous recovery may occur. The outcome of patients treated in centers with
no Tx option was studied. Patients
and Methods: A retrospective
review of infants and children (≤16 years) who presented with heart muscle
disease (left ventricular shortening fraction, LVSF ≤ 25%) between 1992
and2007, intwo medical centers in Jerusalem, was performed.Results: There were 62 patients, mean age 2.5
years, mean LVSF 16.3%. The 5-year survival was 73%. Lower LVSF at presentation
(p = 0.006) was independently associated
with mortality. Recovery (normalization of LVSF) occurred in 56% of survivors.
Of the 38 patients who were eligible for Tx at their presentation, according to
the AHA guidelines, 22 (58%) survived and 13 (34%) recovered.Conclusions: This study shows the long term outcome
of pediatric patients with acute heart muscle disease treated with no Tx
option. These findings may reflect the impact of improved medical management of
these patients in recent years.