title: Diversity of congenital cardiac defects and skeletal deformities associated with the Holt-Oram syndrome.
abstract: BACKGROUND
  The Holt-Oram syndrome is a rare congenital disorder involving the skeletal and cardiovascular systems. It is characterized by upper limb deformities and cardiac malformations, atrial septal defects in particular.
  
  
  METHODS
  Four consecutive patients 1-15 years old with the Holt-Oram syndrome presented over a 10 year span for surgical treatment of their cardiac maladies. The spectrum of the heart defects and skeletal deformities encountered in these patients are described and discussed.
  
  
  CONCLUSIONS
  The Holt-Oram syndrome is an autosomal dominant condition; however absence of the morphological features of the trait in close family members is not rare. Although patients are known to predominately present with atrial septal defects, other cardiovascular anomalies, including rhythm abnormalities, are not uncommon. Skeletal disorders vary as well.
  
  
  CONCLUSIONS
  Cardiovascular disorders, skeletal malformations and familial expression of the Holt-Oram syndrome, vary widely.
authors.affiliation: Department of Paediatric and Congenital Cardiac Surgery, Onassis Cardiac Surgery Centre, Athens, Greece.
authors.forename: Gregory
authors.initials: G
authors.lastname: Chryssostomidis
authors.affiliation: Department of Paediatric and Congenital Cardiac Surgery, Onassis Cardiac Surgery Centre, Athens, Greece.
authors.forename: Meletios
authors.initials: M
authors.lastname: Kanakis
authors.affiliation: Athinaiki Mediclinic, Athens, Greece.
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Fotiadou
authors.affiliation: Department of Paediatric Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece.
authors.forename: Cleo
authors.initials: C
authors.lastname: Laskari
authors.affiliation: Department of Anaesthesiology, Onassis Cardiac Surgery Centre, Athens, Greece.
authors.forename: Theofili
authors.initials: T
authors.lastname: Kousi
authors.affiliation: Department of Anaesthesiology, Onassis Cardiac Surgery Centre, Athens, Greece.
authors.forename: Christos
authors.initials: C
authors.lastname: Apostolidis
authors.affiliation: Department of Paediatric and Congenital Cardiac Surgery, Onassis Cardiac Surgery Centre, Athens, Greece.
authors.forename: Prodromos
authors.initials: P
authors.lastname: Azariadis
authors.affiliation: Department of Paediatric and Congenital Cardiac Surgery, Onassis Cardiac Surgery Centre, Athens, Greece. Electronic address: achatzis@ath.forthnet.gr.
authors.forename: Andrew
authors.initials: A
authors.lastname: Chatzis
keywords: Congenital; Heart disease; Heart-limb; Holt–Oram; Skeletal disorders; Syndrome