P4695Pulmonary hypertension in grown-up congenital heart disease, 2018 Nice classification. Impact on mortality. GUCH Registry data

Abstract

Abstract Background Pulmonary hypertension (PH) is a serious complication of grown-up congenital heart (GUCH) diseases. Its onset is associated with a marked increase in mortality in these patients. Purpose To classify GUCH patients with pulmonary hypertension (PH) according to PH subgroups, and explore association between PH and mortality during follow up. Methods An observational retrospective study was conducted on patients on the GUCH database. Overall data were analyzed. Patients diagnosed with PH were selected and classified according to the 2018 Nice HT guidelines on pulmonary arterial hypertension secondary to congenital heart disease: Group 1 (PAH1) and its 4 subgroups (SG1: Eisenmenger's syndrome; SG2: left to right shunts; SG3: PAH associated with congenital heart disease; SG4: post-operative PAH); Group 2 (PH2), Group 3 (PH3), Group 4 (PH4), Group 5 (PH5). Statistical analysis: Kaplan Meier Log Rank test using SPSS 20 software. Results The database included 2073 patients (P), mean age 31.8±12.9 years, 54.2% (1123 P) were women. Overall mortality: 4.2% (88 P). Follow up: 44.1±37 months. Results showed 5.1% of patients (105 P) had PH, 3.8% (79 P) of whom corresponded to group PAH1, and 1.3% (26 P) to the remaining groups. Analysis of P with PH showed that 67.6% (71 P) were women, mean age was 41.3±14.7 years, mortality during follow up was 25.7% (27 P). Classification according to group showed 75.2% (79) corresponded to PAHG1, 9.5% (10) to PH2, 0% to PH3, 1% (1) to PH4, and 14.3 (15) to PH5. Analysis of P in PAHG1 showed a 43% (34 P) prevalence of SG1, 27.8% (22 P) of SG2, 8.9% (7 P) of SG3, and 20.3% (16 P) of SG4. Mortality per group was 22.8% (18 P) in PAH1, 20% (2 P)in PH2, 0% in PH 3, 0% in PH 4, and 46.7% (7 P) in PH 5. Kaplan Meier survival curves showed significant association between PH and mortality, Log Rank=0.000, and worst survival in groups PH2, PAH1 and PH5. No differences in mortality were observed among PAH1 subgroups, Log Rank= 0.458. Survival curves Conclusions PH negatively affected survival of GUCHpatients. Analysis of mortality during follow up showed patients in group PH5 to have the worst outcomes, possible because patients with a single ventricle were included in this group. Group PAH1 comprised 75% of PH patients, with a greater predominance of patients with Eisenmenger's syndrome, but no significant differences in mortality during follow-up among the 4 subgroups.