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Updated: Jul 13, 2026
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart disease
Christina L Greene1, Antonia Schulz1, Mariana Chávez1
1Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Mass.
Insights
Cardiac repair in patients with trisomy 13 and trisomy 18 offers improved long-term survival compared to nonoperative management. This study found significantly higher one-year survival rates for operated patients, suggesting surgical intervention is beneficial.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Genetic Syndromes
Background:
- Trisomy 13 (Patau syndrome) and trisomy 18 (Edwards syndrome) are severe chromosomal abnormalities often associated with congenital heart disease (CHD).
- The optimal management strategy for CHD in these complex patient populations remains a subject of debate.
Purpose of the Study:
- To compare the short- and long-term outcomes of surgical cardiac repair versus nonoperative management for patients diagnosed with trisomy 13 or trisomy 18 and CHD.
- To evaluate the safety and efficacy of primary cardiac repair in this high-risk group.
Main Methods:
- A retrospective review of patients with trisomy 13/18 and CHD admitted between 1985 and 2023.
- Patients were stratified into two cohorts: those who underwent cardiac surgery (operated) and those who received nonoperative management (nonoperated).
- Outcomes including mortality, length of stay, and long-term survival were compared between the cohorts.
Main Results:
- The study identified 62 patients (34 operated, 28 nonoperated) with trisomy 13 (n=9) and trisomy 18 (n=53).
- The operated cohort demonstrated a 30-day postoperative survival of 94% and a one-year survival of 79%, significantly higher than the 51.5% one-year survival in the nonoperated group (P < .003).
- Nonoperative management was associated with an increased risk of mortality (HR, 3.28; P = .004).
Conclusions:
- Primary cardiac repair in patients with trisomy 13/18 and CHD can be performed with low early mortality.
- Surgical intervention is associated with significantly improved long-term survival compared to nonoperative management in this cohort.
- These findings support the consideration of cardiac repair as a viable treatment option for selected patients with these genetic syndromes and CHD.
Objective:
To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease.
Methods:
An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared.
Results:
Between 1985 and 2023, 62 patients (34 operated and 28 nonoperated) with trisomy 13 (n = 9) and trisomy 18 (n = 53) were identified. The operated cohort was 74% girls, underwent mainly The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures (n = 24 [71%]) at a median age of 2.5 months (interquartile range [IQR], 1.3-4.5 months). This compares with the nonoperative cohort where 64% (n = 18) would have undergone The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures if surgery would have been elected. The most common diagnosis was ventricular septal defect. Postoperative median intensive care unit stay was 6.5 days (IQR, 3.7-15 days) with a total hospital length of stay of 15 days (IQR, 11-49 days). Thirty-day postoperative survival was 94%. There were 5 in-hospital deaths in the operated and 7 in the nonoperated cohort. Median follow-up was 15.4 months (IQR, 4.3-48.7 months) for the operated and 11.2 months (IQR, 1.2-48.3 months) for the nonoperated cohorts. One-year survival was 79% operated versus 51.5% nonoperated (P < .003). Nonoperative treatment had an increased risk of mortality (hazard ratio, 3.28; 95% CI, 1.46-7.4; P = .004).
Conclusions:
Controversy exists regarding the role of primary cardiac repair in patients with trisomy 13/18 and congenital heart disease. Cardiac repair can be performed safely with low early mortality and operated patients had higher long-term survival compared with nonoperated in our cohort.
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