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.
Highlights
Pediatric patients with cardiomyopathies, presenting with acute heart failure, are known to have high mortalityHow to cite this paper: Kampel, L., et al (2014) The Outcome of Infants and Children with Heart Muscle Disease: Prognosis in Centers without Heart Transplantation Option
The American Heart Association (AHA) published practical guidelines for heart transplantation in pediatric patients with heart failure associated with cardiomyopathies [8]
The left ventricular shortening fraction ranged from 8% to 25%
Summary
Pediatric patients with cardiomyopathies, presenting with acute heart failure, are known to have high mortalityHow to cite this paper: Kampel, L., et al (2014) The Outcome of Infants and Children with Heart Muscle Disease: Prognosis in Centers without Heart Transplantation Option. Pediatric patients with cardiomyopathies, presenting with acute heart failure, are known to have high mortality. As there are no known factors that could accurately determine who is at risk for death [5] [6], the natural history of acute heart failure due to cardiomyopathy is still unpredictable. Therapy for this condition includes medications, mechanical support and heart transplantation. The decision to proceed to transplantation is complex, as at the time a donor heart becomes available, the risk for death is hard to determine, and some patients may undergo transplantation despite having a curable disease
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