Clinical characteristics and outcomes of cardiovascular surgery and transcatheter procedures in adults with Down syndrome and congenital heart disease.

Background: Advances in medical care have increased the number of adults with Down syndrome and congenital heart disease (CHD), including those with residual or unrepaired lesions. However, clinical outcomes following invasive cardiovascular procedures in this population remain poorly understood. Methods: We extracted data on patients with Down syndrome aged ≥16 years and diagnosed with CHD from the Japanese Registry of All Cardiac and Vascular Diseases Diagnosis Procedure Combination (JROAD-DPC), covering the period from April 1, 2013, to March 31, 2023. Patients who underwent cardiovascular surgery or transcatheter procedures were included in the analysis. Results: The study included 115 patients (median age: 21 [17-33] years; 47.8 % female), of whom 72 underwent surgery and 43 underwent transcatheter intervention. Procedures were most common among those aged 16-29 years. The main CHD subtypes were tetralogy of Fallot, atrial septal defects, and atrioventricular septal defects. Catheter-based interventions were associated with shorter hospital stay and no in-hospital mortality. In contrast, all three deaths (4.2 %) occurred in the surgical group, with pulmonary hypertension medications used in two of these patients. Among surgical patients receiving pulmonary hypertension medications, the in-hospital mortality rate reached 25 %. In multivariate analysis, emergency admission and the use of pulmonary hypertension medications were independently associated with longer hospital stays after surgery. Conclusions: In adults with Down syndrome and CHD, invasive cardiovascular procedures had relatively acceptable in-hospital outcomes. Careful patient selection and perioperative management are crucial when planning cardiac surgery, especially in patients taking medication for pulmonary hypertension.