ObjectiveCurrent data on intensive care unit (ICU) admissions in patients with adult congenital heart disease (ACHD) are limited and focus on admissions after elective cardiac surgery. This study describes non-elective ICU admissions in patients with ACHD.MethodsA retrospective matched cohort study was performed from January 2000 until December 2015 in a tertiary care centre ICU (there was no cardiac care unit). Primary outcomes were short-term (during hospital stay or <30 days after discharge) and long-term (>30 days after discharge until end of follow-up) mortality. Outcomes were compared with non-ACHD non-elective ICU admissions, matched 1:1 on age, sex and admission diagnosis.ResultsA total of 138 admissions in 104 patients with ACHD (65.9% male, median age 30 years) were included, during 8.6 years of follow-up. The majority had a moderate-to-severe heart defect. Arrhythmia was the most common admission diagnosis (44.2%), followed by haemorrhage (10.9%), heart failure (8.7%) and pulmonary disease (8.7%). Short-term mortality and total mortality were lower in the ACHD admissions than in the non-ACHD admissions (4.8% vs 16.3%, p=0.005 and 17.3% vs 28.9%, p=0.030), whereas long-term (12.5% vs 12.6%, p=0.700) did not differ. Severe CHD (HR 3.1, 95% CI 1.1 to 8.6) at baseline, and mechanical circulatory support device use (8.3, 1.4 to 47.4) and emergency intervention (0.2, 0.1 to 0.7) during the ICU stay were independently associated with mortality in the ACHD group.ConclusionsNon-elective ICU admissions in patients with ACHD are most often for arrhythmia and in patients with moderate-to-severe CHD. Reassuringly, short-term and total mortality are lower compared with patients without ACHD, however, long-term mortality is higher than expected for patients with ACHD.