Cyanotic adults with congenital heart disease (ACHD) rely on life-sustaining adaptations to their chronic cyanosis and are at risk of adverse consequences if such adaptations are out of balance. They may benefit from surgical treatment options that were not available during their childhood. The “GUCH morbidity and mortality scores” were specifically designed to predict risks after surgery in ACHD and included comorbidities and patient age. We aim to assess their performance in cyanotic compared to non-cyanotic ACHD.

Data of all consecutive adults who underwent CHD surgery in 2005–2016, were collected. Mortality was defined as hospital mortality or mortality within 30 days following surgery. Morbidity was defined as occurrence of one or more of the following complications: renal failure requiring dialysis, neurological deficit persisting at discharge, atrioventricular block requiring pacemaker implantation, mechanical circulatory support, phrenic nerve injury and unplanned reoperation. The performance of the GUCH scores was assessed using the area under the receiver operating characteristics curve (C-index, 95% CI).

We evaluated 824 operations including 99 performed in cyanotic ACHD. The mean age at operation was 34±13 years (18–72 years). Cyanotic patients had higher hospital mortality and morbidity than non-cyanotic patients (11.1% vs. 2.4%, P<0.0001; and 22.2% vs. 8.4%, P<0.0001 respectively). C-index for GUCH mortality score in cyanotic and non-cyanotic ACHD were not different: 0.722 (0.536–0.907) and 0.800 (0.712-0.887) (P=0.44), respectively. C-index for GUCH morbidity score was lower in cyanotic than in non-cyanotic ACHD: 0.483 (0.335–0.632) vs. 0.671 (0.601–0.741) (P=0.027).

GUCH mortality score is efficient in cyanotic ACHD patients. However, the GUCH morbidity score should be refined in these patients.