The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry: a descriptive study from single-center hospital registry of adult congenital heart disease and pulmonary hypertension in Indonesia

Abstract

Backgrounds: The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first congenital heart disease (CHD) and CHD-related pulmonary hypertension (PH) in adults registry in Indonesia. The study aims to describe prevalence, characteristics, and hemodynamic the demographics, clinical presentation, and hemodynamics data of adult CHD and CHD-related PH in Indonesia. Methods: The COHARD-PH registry is a hospital-based, single-center, and prospective registry which includes adult patients with CHD and CHD-related PH. The patients were enrolled consecutively. For this study, we evaluated the registry patients from July 2012 until July 2018 2019. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6 minute walking test, laboratory measurement, and transthoracic and transoesofageal echocardiography. Right heart catheterisation was performed to measure hemodynamics and confirmed the diagnosis of pulmonary artery hypertension (PAH). Results: We registered 803 1012 patients during the study. The majority were young-adult females. The majority of CHD was secundum ASD (79.0%) (73.4%). The main symptom was dyspneu on effort. The majority of patients (78.1%) (77.1%) had already developed signs of PH assessed by echocardiography. The Eisenmenger syndrome was encountered in 17.3% 18.7% patients. Based on the right heart catheterisation, 67.8% 66.9% patients had developed PAH. Patients with PAH were significantly older, had lower peripheral oxygen saturation, had lower 6 minute walking distance, and had higher NTproBNP. There was an increased of proportion of PAH prevalence according to age range, with the highest prevalence between 51 and 60 years old. Conclusions: The COHARD-PH registry is the first Indonesian adult-CHD and CHD-related PH registry. The prevalence, characteristics, and hemodynamics data demographics, clinical presentation, and hemodynamics data of this registry reflects situation in developing countries which need to be compared with similar registries from developed countries.