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Ventricular septal defect closure in a patient with VACTERL syndrome: anticipating sequelae in a rare genetic

Ali Can Hatemi1, Mete Gursoy, Kadir Ceviker

  • 1Department of Cardiovascular Surgery, Institute of Cardiology, Istanbul University, Haseki, Istanbul 34304, Turkey. hatemi@superonline.com

Texas Heart Institute Journal
|July 10, 2008
PubMed

Insights

Genetic disorders complicate pediatric cardiac surgery. Closing a ventricular septal defect in a VACTERL syndrome patient improved outcomes, highlighting the importance of addressing noncardiac defects.

Area of Science:

  • Pediatric Cardiac Surgery
  • Medical Genetics
  • Congenital Heart Defects

Background:

  • Noncardiac anomalies in genetic disorders pose significant risks for pediatric cardiac surgery patients.
  • These patients face higher rates of prolonged hospitalization, increased costs, morbidity, and mortality.
  • Ventricular septal defect (VSD) is a common cardiac anomaly associated with various genetic syndromes.

Observation:

  • VACTERL syndrome, a rare pattern of birth defects, has a notable incidence of VSD (22.3%).
  • This case study focuses on a 4-month-old female infant diagnosed with VACTERL syndrome postnatally.
  • The patient underwent surgical closure of a ventricular septal defect.

Findings:

  • Surgical closure of the VSD was performed in the infant with VACTERL syndrome.
  • The impact of VSD closure on the patient's operative and postoperative course is discussed.
  • Addressing the VSD is crucial for managing complex pediatric cardiac surgery cases associated with genetic disorders.

Implications:

  • Effective management of noncardiac components, such as VSD in VACTERL syndrome, is vital for improving outcomes in pediatric cardiac surgery.
  • Early identification and surgical intervention for VSD in patients with genetic syndromes can mitigate complications.
  • This case underscores the need for a multidisciplinary approach to care for children with complex congenital conditions.

Keywords:
Abnormalities, multiple/epidemiologychromosome breakageesophageal atresia/complications/epidemiology/etiology/genetics/surgeryfetal growth retardation/epidemiologyfetal heartheart defects, congenital/chemically induced/classification/epidemiologyinfant, newbornprogesterone/adverse effectsrespiration disorders/etiologytracheoesophageal fistula/complications/surgery

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