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Heart transplantation in infants with idiopathic hypertrophic cardiomyopathy

Luca Ragni1, Elena Biagini, Fernando M Picchio

  • 1Pediatric Cardiology and Adult Congenital Cardiology Unit, University of Bologna, Bologna, Italy.

Pediatric Transplantation
|November 11, 2008
PubMed

Insights

Idiopathic hypertrophic cardiomyopathy (HCM) in infants is rare. Heart transplantation (HT) may be the only option for neonates with severe heart failure and reduced heart cavity size.

Area of Science:

  • Pediatric Cardiology
  • Neonatology
  • Cardiovascular Surgery

Background:

  • Idiopathic hypertrophic cardiomyopathy (HCM) is a known condition in infants.
  • Limited data exists on heart transplantation (HT) for infants with HCM.
  • Severe heart failure (HF) can occur in neonates with HCM.

Purpose of the Study:

  • To describe the clinical course and outcomes of infants with idiopathic HCM requiring heart transplantation.
  • To highlight the potential need for HT in neonates with rapidly progressive HF due to HCM.

Main Methods:

  • Retrospective case series of 17 infants diagnosed with idiopathic HCM.
  • Detailed analysis of clinical presentation, echocardiographic findings, and management strategies.
  • Focus on two neonates who underwent heart transplantation for severe, progressive HF.

Main Results:

  • Idiopathic HCM was identified in 17 infants.
  • Two neonates presented with rapidly progressive severe HF and required heart transplantation.
  • Extreme reduction in cardiac cavity size was a significant finding in these cases.

Conclusions:

  • Rapidly progressive HF in infants with idiopathic HCM and extreme cavity reduction necessitates anticipation of HT.
  • Heart transplantation may be the sole therapeutic option for select neonates with severe, progressive HCM-related HF.

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