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Originally published by 2 Minute Medicine® (view original article). Reused on AccessMedicine with permission.

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1. Congenital heart surgery (CHS) in patients with trisomy 13 (T13) and trisomy 18 (T18) was significantly associated with a reduction in in-hospital mortality compared to patients who did not undergo CHS. There was no significant difference in in-patient mortality for T13 and T18 patients without congenital heart disease (CHD) versus those with CHD who did not undergo CHS.

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2. Greater age, older age at admission, and female sex were significantly associated with improved survival in both T13 and T18 undergoing CHS.

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Evidence Rating: 2 (Good)

Study Rundown:

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Patients with T13 and T18 have low survival rates, with most children dying shortly after birth. There is limited data to suggest that some children may live longer. Both syndromes are associated with neurodevelopmental disability, specific physical features and multiple congenital anomalies, with CHD occurring in up to 80% and 90% of patients with T13 and T18, respectively. As CHS is not offered at most centers, there is limited data on outcomes. The purpose of this retrospective cohort was to evaluate the impact of CHS on in-hospital mortality for T18 and T13 patients. Researchers found a significant reduction in-hospital mortality for both T13 and T18 patients who underwent CHS, compared to those who did not. However, there was no in-hospital mortality difference between T13 and T18 with CHD who did not undergo CHS versus those without a CHD diagnosis. This study also demonstrated that for T13 and T18 infants undergoing CHS, greater age, older age at admission, and female sex were linked with improved survival. While this study is the largest study of T13 and T18 CHS outcomes to date, limitations include potential confounders, such as mosaicism or extra-cardiac diagnoses, that may influence the clinical course of survival. For clinicians, this study provides data that may help to risk stratify patients for CHS.

In-Depth [retrospective cohort]:

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Participants in this multi-center retrospective study included 1668 patients (T13=648; T18=1020), with data extracted from the Pediatric Health Information System (PHIS). Inclusion criteria included an ICD-9 code for either T13 or T18 and admission age of ≤14 days. Patients were excluded if they were coded with a diagnosis of both T13 and T18 or an additional diagnosis of T12 or a second admission within 14 days of life. CHD was coded in 86% and 91% of patients with T13 and T18, respectively, and CHS was performed in 7% of each group. The most common defects were atrial and ventricular defects and patent ductus arteriosus. In-hospital mortality was significantly lower for patients that received CHS versus no CHS in the T13 (10 [16%] versus 383 [44%], P≤0.003) and T18 groups (11 [30%] versus 285 [55%], P=0.003). For patients with T13 and T18 who underwent CHS, greater weight (OR=0.62, 95%CI=0.40-0.69, p<0.001 for T13; OR=0.28, 95%CI=0.20-0.35, P<0.001 for T1) and older age at admission (OR=0.8, 95%CI=0.80-0.93, p<0.001 for T18) were associated with improved survival. Male sex in CHS was associated with poorer survival (OR=1.50, 95%CI=1.04-2.17, p=0.028 for T13; OR=1.15, 95%CI=1.11-2.06, p=0.009 for T18). There was no significant difference in in-hospital mortality between T13 and T18 patients without CHD and those with CHD who did not have CHS.

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