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[Familial dilated cardiomyopathy in patients transplanted for idiopathic dilated cardiomyopathy]

Lorenzo Monserrat1, Manuel Hermida, Beatriz Bouzas

  • 1Servicio de Cardiología del Hospital Juan Canalejo, A Coruña, Spain. Lorenzo_Monserrat@cannalejo.org

Revista Espanola De Cardiologia
|July 13, 2002
PubMed

Insights

Familial dilated cardiomyopathy (DCM) is common in heart transplant recipients. Early signs like left ventricular enlargement and conduction defects may indicate familial disease.

Area of Science:

  • Cardiology
  • Genetics
  • Transplantation Medicine

Background:

  • Dilated cardiomyopathy (DCM) is a significant cause of heart failure requiring transplantation.
  • Understanding the genetic basis of DCM is crucial for risk stratification and family screening.

Purpose of the Study:

  • To determine the prevalence of familial DCM in heart transplant patients.
  • To characterize the clinical features and inheritance patterns of familial DCM.
  • To identify potential early markers for familial DCM.

Main Methods:

  • Studied 43 families with heart transplant recipients diagnosed with idiopathic DCM.
  • Excluded patients with a history of alcohol abuse.
  • Conducted clinical evaluations, ECGs, echocardiograms, blood tests, and DNA analysis in patients and relatives.

Main Results:

  • Familial DCM was identified in 25.6% of probands, and possible familial DCM in another 25.6%.
  • Fifteen relatives had DCM, 26 had left ventricular enlargement (LVE), and 5 had hypertrophic cardiomyopathy.
  • Autosomal dominant inheritance was most common, with some cases suggesting recessive inheritance and others showing conduction system defects.

Conclusions:

  • Familial DCM has a high prevalence in heart transplant candidates.
  • LVE, conduction abnormalities, and elevated creatine kinase may serve as early indicators of familial DCM.
  • Hypertrophic cardiomyopathy and familial DCM can be present even in patients with a prior diagnosis of alcoholic DCM.
Abstract

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