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Cardiac surgery in patients with trisomy 18

Yukihiro Kaneko1, Jotaro Kobayashi, Ikuya Achiwa

  • 1Department of Cardiovascular Surgery, Japanese Red Cross Medical Center, Tokyo, Japan. yukihirokaneko@hotmail.com

Pediatric Cardiology
|April 3, 2009
PubMed

Insights

Cardiac surgery can effectively treat congenital heart defects in trisomy 18 patients, improving symptoms and survival. Palliative procedures showed better long-term outcomes than primary intracardiac repair for these complex cases.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Genetics

Background:

  • Trisomy 18 (Edwards syndrome) is a genetic disorder associated with severe congenital anomalies.
  • Congenital heart defects are common in trisomy 18, often leading to significant morbidity and mortality.
  • Cardiac surgery for trisomy 18 is infrequently performed but increasingly considered.

Purpose of the Study:

  • To report the clinical details and outcomes of cardiac surgery in patients with trisomy 18.
  • To evaluate the effectiveness of different surgical approaches (palliative vs. primary repair) in this population.
  • To assess postoperative survival and identify factors influencing outcomes.

Main Methods:

  • Retrospective review of 17 trisomy 18 patients who underwent cardiac surgery.
  • Analysis of patient demographics, cardiac defect types, surgical procedures (palliative or primary repair), and postoperative outcomes.
  • Comparison of survival rates between palliative surgery and primary intracardiac repair groups.

Main Results:

  • Seventeen patients underwent surgery at a median age of 66 days.
  • Common defects included ventricular septal defects, coarctation of the aorta, and patent ductus arteriosus.
  • 82% of patients were discharged with improved symptoms; median survival was 179 days.
  • Palliative surgery resulted in significantly better postoperative survival (median 257 days) compared to primary repair (median 48 days).

Conclusions:

  • Cardiac surgery can be effective in managing serious cardiac symptoms in trisomy 18 patients.
  • Palliative surgical strategies appear to offer better survival outcomes than primary intracardiac repair in this cohort.
  • Further research is needed to optimize surgical management for trisomy 18 patients with congenital heart disease.

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