Radical surgery for a ventricular septal defect associated with trisomy 18

Gen Thorac Cardiovasc Surg. 2010 May;58(5):223-7. doi: 10.1007/s11748-009-0431-3. Epub 2010 May 7.


Purpose: Congestive heart failure is one of the major causes of early death of patients with trisomy 18. Ventricular septal defect (VSD) is the most common heart defect in patients with trisomy 18, and closure of the VSD may elongate the lifespan of the patient. Morphological characteristics of these patients, such as thoracic deformity, prominent right ventricular hypertrophy, and dysplastic tricuspid valve may complicate closure of the VSD. We report our initial experience of VSD closure in patients with trisomy 18 and estimate the feasibility of the surgical procedure.

Methods: Between June 2005 and September 2007, five female patients with trisomy 18 and VSD underwent radical operations. Four of them had undergone previous palliative surgery entailing pulmonary artery banding.

Results: All patients survived surgery. The average cardiac arrest time during surgery was 74 +/- 22 min. No early death occurred within 30 days of surgery. One patient with hydrocephalus treated by a previous ventriculoperitoneal shunt died in hospital owing to postoperative intracranial hypertension. Four of five patients were discharged from the hospital. Of the four discharged patients, a girl who underwent primary radical operation died of pneumonia. Another patient died of sudden cardiopulmonary arrest. The average survival of the patients in this study was 815 +/- 389 days at data acquisition, with two patients still alive.

Conclusion: Closure of VSDs in patients with trisomy 18 was found feasible and was associated with extended survival.

MeSH terms

  • Abnormalities, Multiple*
  • Cardiac Catheterization
  • Cardiac Surgical Procedures* / adverse effects
  • Cardiac Surgical Procedures* / mortality
  • Child, Preschool
  • Chromosomes, Human, Pair 18*
  • Coronary Angiography
  • Echocardiography
  • Feasibility Studies
  • Female
  • Genetic Predisposition to Disease
  • Heart Septal Defects, Ventricular / diagnosis
  • Heart Septal Defects, Ventricular / genetics
  • Heart Septal Defects, Ventricular / mortality
  • Heart Septal Defects, Ventricular / surgery*
  • Hospital Mortality
  • Humans
  • Infant
  • Palliative Care
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Trisomy*