Effect of cardiac surgery on survival in patients with trisomy 18: A single-center experience
Mehmet Çelik1, Mahmut Gökdemir2, Nimet Cındık2
1Department of Cardiovascular Surgery, Başkent University Hospital, Konya, Türkiye.
Insights
Cardiac surgery may improve survival for infants with trisomy 18, but high mortality persists due to other organ failures. A multidisciplinary approach is recommended for better outcomes in these complex cases.
Area of Science:
- Pediatric Cardiology
- Genetics
- Critical Care Medicine
Background:
- Trisomy 18 (Edwards syndrome) is a severe genetic disorder often associated with complex congenital heart defects.
- Management strategies for trisomy 18 patients, particularly regarding surgical intervention, remain a subject of debate due to high morbidity and mortality.
- This study investigates the outcomes of surgical versus medical management in infants diagnosed with trisomy 18.
Purpose of the Study:
- To compare the survival rates and outcomes of patients with trisomy 18 who underwent cardiac surgery versus those managed non-surgically.
- To identify factors contributing to mortality in trisomy 18 patients.
- To evaluate the potential benefits of a multidisciplinary approach in managing trisomy 18.
Main Methods:
- Retrospective analysis of 18 patients diagnosed with trisomy 18 between May 2014 and January 2022.
- Patients were divided into two groups: surgical intervention (n=10) and medical management (n=8).
- Data collected included cardiac pathology, survival duration, and causes of death.
Main Results:
- All patients had cardiac pathology; 30% in the surgical group and 25% in the medical group were discharged.
- Median survival was significantly longer in the surgical group (150 days) compared to the medical group (8 days) (p=0.009).
- Mortality in the medical group was due to multi-organ failure, while the surgical group experienced deaths from sepsis, heart failure, and respiratory failure.
Conclusions:
- Cardiac surgery appears to offer a survival benefit for trisomy 18 patients, despite high overall mortality.
- Non-cardiac complications, such as multi-organ failure, remain significant challenges.
- A comprehensive, multidisciplinary approach is crucial for improving long-term survival and quality of life in trisomy 18 patients.
Background:
In this study, we aimed to compare the results of operated patients diagnosed with trisomy 18 with those who were followed with medical treatment alone.
Methods:
Between May 2014 and January 2022, a total of 18 patients (6 males, 12 females; median age: 39 days; range, 32 to 79 days) diagnosed with trisomy 18 were retrospectively analyzed. Patient data were obtained from the pediatric cardiovascular surgery digital database. The patients were divided into two groups: those who underwent surgery (n=10) and those who were followed with medical treatment (n=8).
Results:
Cardiac pathology was detected in all 18 patients included in the study. Three (30%) patients in the surgical group and two (25%) patients in the medical treatment group were discharged and followed with medical treatment. One of the three patients discharged after surgery died during follow-up. The median survival in surgical and medical treatment groups was 150 (range, 75 to 308) days and eight (range, 3 to 51) days, respectively (p=0.009). While patients in the medical treatment group died due to multi-organ failure, those in surgical group died due to sepsis, heart failure, and respiratory failure.
Conclusion:
Although cardiac surgery contributed positively to survival in patients with trisomy 18, the mortality rate was still high due to non-cardiac causes. We believe that a multidisciplinary approach would contribute positively to the treatment of this patient group with multi-organ failure and would aid in prolonging their life span.
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