Long-term follow-up of patients with PAH associated with congenital heart disease at a university pulmonary arterial hypertension (PAH) center: Real-life data

Introduction: CHD-PAH is a disease that may develop at any period of CHD in the presence of shunt and includes a heterogeneous patient population characterized by a high morbidity and mortality rate. Anatomic-Physiological and Clinical classifications are used to describe these patients. Although there are several studies evaluating the survival of the anatomical and clinical classes, the survival difference between the classes has not been determined yet. In addition, the predictive parameters for mortality in patients with CHD-PAH have not yet been established. Objective: In our study, we aimed to determine the 5-year and 10-year survival and the prognostic factors of mortality in both the anatomic and clinical classes of CHD-PAH. At the same time, we aimed to evaluate the quality of life, mood-state disorders and cognitive functions of patients with CHD-PAH. Methods: Our study has 2 components: retrospective and cross-sectional. Survival analysis and determination of predictor factors of mortality were performed by retrospectively evaluating 82 patients with CHD-PAH who were followed in our clinic and. In the 40 patients with CHD-PAH, SF-36, MoCA and HAD scales were applied cross-sectionally. Results: A total of 82 patients (62.2% female, age: 39 ± 15 years) were included in the study. The 5-year survival rates for ES, Left-right shunt-induced PAH, Small defect-induced PAH and PAH after corrective cardiac surgery were 85%, 88%, 50% and 83%, respectively. The 10-year survival rate for ES, Left-right shunt induced PAH and PAH after corrective cardiac surgery was 78%, 70%, and 53%, respectively (p=0.022). There was no statistically significant difference between the 5-year survival of the anatomical classes (p = 0.422). In multivariate analyzes, high Nt-Pro BNP and high right atrial pressure were predictive of mortality. (p = 0.009 and p = 0.022). When compared SF-36 parameters of the CHD-PAH patients with the Turkey average all parameters except mental health were lower and there were a serious deterioration in the quality of life patients with CHD-PAH. It was determined that 5 (12.5%) patients had anxiety and 10 (25%) patients had a risk of depression and mild cognitive impairment was present in 26 (69%) patients. Conclusion: The best 5-year survival was found in the Left-right shunt induced PAH class, and the worst survival was in the PAH class with the small defect. The 10-year survival of the ES group was better than the Left-right shunt-induced PAH group. In the univariate analysis, 17 parameters were prognostic for mortality, but only 2 parameters (high Nt-ProBNP and SaAB) were found to be related to death in multivariate analyzes. There was a significant deterioration in the quality of life of patients with CHD-PAH, and mood-state disorders were also found to affect quality of life.