Hiroyuki Nagao, Takamichi Uchiyama, Shintaro Nemoto
This retrospective study evaluated the impact of ventricular septal defect (VSD) closure on long-term survival and hospitalization status in infants with trisomy 18. Thirty-three infants with trisomy 18 and VSD (2002-2025) were categorized into three groups: staged VSD closure following pulmonary artery banding (PAB) (n = 7), PAB alone (n = 3), or non-surgical management (n = 23). Median follow-up durations (interquartile range) were 124.6 (75.8-153.1), 17.8 (11.4-18.1), and 17.1 (2.7-59.3) months, respectively. Kaplan-Meier analysis demonstrated significantly better survival in the VSD closure group (log-rank p = 0.009). However, these survivors required frequent unexpected hospitalizations for non-cardiac comorbidities. In univariate negative binomial regression, respiratory and digestive complications were significantly associated with higher unexpected admission rates (incidence rate ratio [IRR] = 1.07, 95% confidence interval [CI]: 1.02-1.12; and IRR = 1.24, 95% CI: 1.08-1.41, respectively; p < 0.01). After multivariable adjustment, only digestive complications showed a notable, though non-significant, trend (IRR = 1.15, 95% CI: 0.98-1.36, p = 0.09). Notably, VSD closure was associated with a higher admission rate in univariate analysis (IRR = 1.95, 95% CI: 0.95-4.00, p = 0.07), but this association disappeared after multivariable adjustment. VSD closure was associated with improved survival in infants with trisomy 18, but a substantial hospitalization burden persisted due to non-cardiac comorbidities. These findings highlight the importance of lifelong multidisciplinary care and detailed parental counseling regarding the expected long-term medical burden following VSD closure.