Outcome of Infants and Children with Acute Heart Muscle Disease: Results from Centers with no Pediatric Heart Transplantation

  • Liyona Kampel, The Hebrew University Medical School, Jerusalem, Israel
  • Dr Zeev Perles, Hadassah - Hebrew University Medical Center, Jerusalem, Israel
  • Prof Azaria Rein, Hadassah - Hebrew University Medical Center, Jerusalem, Israel
  • Dr Nail El-Lahham, Hadassah - Hebrew University Medical Center, Jerusalem, Israel
  • Dr Jacques Braun, Hadassah - Hebrew University Medical Center, Jerusalem, Israel
  • Dr Benjamin Farber, Shaare Zedek Medical Center, Jerusalem, Israel
  • Dr Amiram Nir, Shaare Zedek Medical Center, Jerusalem, Israel

    • Objectives
    Acute heart failure (AHF) due to myocarditis or dilated cardiomyopathy in infants and children is potentially fatal. Heart transplantation (Tx) is utilized to improve survival. However, complete recovery may occur with medical treatment alone. The prognosis of patients treated medically and the proportion of patients who undergo Tx but would have survived and even recovered without Tx, is unclear.
    Methods
    Retrospective review of infants and children (<18 years) who presented with cardiomyopathy (left ventricular shortening fraction, LVSF < 25%), between 1992 and 2007 in Hadassah and Shaare Zedek Medical Centers, where Tx is not an option, was performed.
    Results
    There were 62 patients, 42% males, mean age 2.5 years, mean LVSF 16.3% (LVSF<20% in 47 patients). Overall mortality was 27%, which is not different from that reported in centers utilizing Tx (p=0.35). Higher LVSF at presentation (p=0.006) and the use of intravenous immunoglobulin (IVIG, p=0.018) were independently associated with better survival. There was no correlation between survival and gender, preceding viral illness or left ventricular diastolic dimensions. Recovery (normalization of LVSF) occurred in 57% of survivors. Of the 38 patients who were eligible for Tx according to the AHA guidelines, 22 (58%) survived and 12 (32%) recovered.
    Conclusions
    While there is no doubt that some patients are saved by heart transplantation, this study shows that of patients who may have been Tx recipients in other centers, 58% survived and 32% recovered. Furthermore, our results suggest that the use of IVIG improves survival in non-selective pediatric AHF population.