Effect of cardiac surgery on survival in patients with trisomy 18: A single-center experience

心脏手术对18三体综合征患者生存率的影响:单中心经验

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Abstract

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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