Pulmonary hypertension in adults with congenital heart disease. Clinical phenotypes and outcomes in the advanced pulmonary vasodilator era

[Background] Mortality of pulmonary hypertension associated with congenital heart disease (PAH-CHD) in adults remains high.[Objectives] To identify predictors of death and to assess the impact of treatment on outcome.[Methods] Retrospective, multicenter cohort study of 103 adults with PAH-CHD followed-up for 8.6 ± 4.6 years. Patients were grouped according to underlying shunt type into pre-tricuspid, post-tricuspid and complex. Survival rates were analyzed and predictors of death were investigated with Cox regression models.[Results] In the post-tricuspid and complex groups (38 and 37 patients, respectively), the most common clinical PAH-CHD subgroup was Eisenmenger syndrome (76.3% and 59.5%, respectively) whereas, in the pre-tricuspid group (28 patients), 46.5% of patients had small or corrected defects. Overall, 88 patients received vasodilators; 39% required combination-therapy. Overall survival at 10 years was 65%. Mortality was highest in the pre-tricuspid group, FC-III-IV and amongst patients receiving monotherapy (p < 0.050). On multivariate analysis, predictors of poor outcome were pericardial effusion (HR: 4,520 [1,470–13,890]; p = 0,008), oxygen saturation(HR: 0.940 [0,900 - 0,990]; p = 0,018) and genetic syndromes(HR: 3,280 [1,098–9,780]; p = 0,033).[Conclusions] Patients in advanced stages at initiation of treatment were at high risk of death and strong consideration should be given for more aggressive therapy.This study was supported by Clinical Research Grant 2017 from the Spanish Society of Cardiology. The regional Registry of Adult Congenital Heart Disease in Andalusia (RACCA) is supported by the Spanish Society of Cardiology and was launched through a research grant from Actelion Pharmaceuticals.Peer reviewe