Abstract
BackgroundsThe COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first registry for congenital heart disease (CHD) and CHD-related pulmonary hypertension (PH) in adults in Indonesia. The study aims to describe the demographics, clinical presentation, and hemodynamics data of adult CHD and CHD-related PH in Indonesia.MethodsThe 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 2019. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6-min walking test, laboratory measurement, and transthoracic and transesophageal echocardiography. Right heart catheterization was performed to measure hemodynamics and confirm the diagnosis of pulmonary artery hypertension (PAH).ResultsWe registered 1012 patients during the study. The majority were young, adult females. The majority of CHD was secundum ASD (73.4%). The main symptom was dyspnea on effort. The majority of patients (77.1%) had already developed signs of PH assessed by echocardiography. The Eisenmenger syndrome was encountered in 18.7% of the patients. Based on the right heart catheterization, 66.9% of patients had developed PAH. Patients with PAH were significantly older, had lower peripheral oxygen saturation, had lower 6-min walking distance, and higher NTproBNP. The NTproBNP level independently predicted the development of PAH among CHD.ConclusionsThe COHARD-PH registry is the first Indonesian adult-CHD and CHD-related PH registry. The demographics, clinical presentation, and hemodynamics dataof this registry reflect the situation in developing countries which needs to be compared with similar registries from developed countries.
Highlights
The prevalence of adult congenital heart disease (CHD) in developed countries continues to rise due to improved survival attributed to successful surgical and medical management in childhood [1]
The demographics, clinical presentation, and hemodynamics dataof this registry reflect the situation in developing countries which needs to be compared with similar registries from developed countries
The pulmonary artery hypertension (PAH) or pulmonary hypertension (PH) group 1 is a clinical group which is characterised by hemodynamic parameters as pre-capillary PH (mPAP ≥25 mmHg with pulmonary artery wedge pressure ≤15 mmHg) and pulmonary vascular resistance (PVR) > 3 Wood units (WU) [4]
Summary
The prevalence of adult congenital heart disease (CHD) in developed countries continues to rise due to improved survival attributed to successful surgical and medical management in childhood [1]. PH is defined as an increase in mean pulmonary artery pressures (mPAP) ≥25 mmHg at rest [4]. Based on the current clinical classification of PH, CHD may cause pulmonary artery hypertension (PAH) which is defined as a group 1 in this classification [4]. The PAH or PH group 1 is a clinical group which is characterised by hemodynamic parameters as pre-capillary PH (mPAP ≥25 mmHg with pulmonary artery wedge pressure (mPAWP) ≤15 mmHg) and pulmonary vascular resistance (PVR) > 3 Wood units (WU) [4]. The implications of CHD-related PH are limited functional capacity, increased risk of arrhythmias, right heart failure, and increased mortality [5]
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