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Eur J Cardiothorac Surg 2009;35:606-614. doi:10.1016/j.ejcts.2008.11.005
Copyright © 2009, European Association for Cardio-thoracic Surgery. Published by Elsevier. All rights reserved.
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Roberto Formigari
Guido Michielon
Adriano Carotti
Alessandro Giardini
Roberto M. Di Donato
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Review

Genetic syndromes and congenital heart defects: how is surgical management affected?

Roberto Formigaria, Guido Michielonb, Maria Cristina Digiliob, Gerardo Piacentinic, Adriano Carottib, Alessandro Giardinia, Roberto M. Di Donatob, Bruno Marinoc,*

a Pediatric Cardiology and Cardiac Surgery, Policlinico ‘S. Orsola-Malpighi’, Bologna, Italy
b Pediatric Cardiac Surgery and Genetics, Ospedale del Bambino Gesù, Rome, Italy
c Department of Pediatrics, Sapienza University of Rome, Viale Regina Elena 324, 00161 Rome, Italy

Received 27 June 2008; received in revised form 30 October 2008; accepted 10 November 2008.

* Corresponding author. Tel.: +39 0649979210; fax: +39 0649970356. (Email: bruno.marino{at}uniroma1.it).

The population of neonates and children with congenital heart defects presents about a 30% prevalence of associated genetic syndrome or additional extracardiac anomalies and may show an increased risk of death or major complication at cardiac surgery. Since a well-defined pattern of combined cardiac and extracardiac anomalies may be found in relation to specific genetic defects, correct understanding of the genetic issues may help improving diagnosis, surgical approach and final outcome of these patients. Hereby we review the medical and surgical issues correlated to the genetic asset in patients with congenital heart defects and genetic syndromes, including trisomy 21, deletion 22q11, Noonan/LEOPARD, Turner, Marfan and Williams syndromes. Recognition of specific surgical risk factors can lead to the preparation of specific diagnostic and perioperative protocols in order to reduce operative mortality and morbidity.

Abbreviations: CHD = congenital heart defect

Key Words: Congenital heart disease • Genetic syndromes • Genotype–phenotype correlation






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Copyright © 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier. All rights reserved.