Unique variant of Adams-Oliver syndrome with dilated cardiomyopathy and heart block

Pediatr Int. 2013 Aug;55(4):508-12. doi: 10.1111/ped.12011.

Abstract

Reported herein is the case of a 2-year-old boy with Adams-Oliver syndrome who presented with dilated cardiomyopathy and complete atrioventricular block. The patient had aplasia cutis congenita with partial aplasia of the skull bones, and terminal transverse limb malformations characteristic of the disease. Although congenital cardiac malformations may be associated with the syndrome, dilated cardiomyopathy has not been previously reported to be associated with the syndrome.

Keywords: Adams-Oliver syndrome; atrioventricular block; dilated cardiomyopathy; smoking; thrombophilia.

Publication types

  • Case Reports
  • Video-Audio Media

MeSH terms

  • Cardiomyopathy, Dilated / complications*
  • Cardiomyopathy, Dilated / diagnosis
  • Child, Preschool
  • Diagnosis, Differential
  • Echocardiography
  • Ectodermal Dysplasia / complications*
  • Ectodermal Dysplasia / diagnosis
  • Electrocardiography*
  • Heart Block / complications*
  • Heart Block / diagnosis
  • Humans
  • Limb Deformities, Congenital / complications*
  • Limb Deformities, Congenital / diagnosis
  • Male
  • Scalp Dermatoses / complications
  • Scalp Dermatoses / congenital*
  • Scalp Dermatoses / diagnosis

Supplementary concepts

  • Adams Oliver syndrome