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

Dinarti LK, Hartopo AB, Kusuma AD, Satwiko MG, Hadwiono MR, Pradana AD, Anggrahini DW.
BMC Cardiovasc Disord. 2020 Apr 7;20(1):163. doi: 10.1186/s12872-020-01434-z.
PMID: 32264836 Free PMC Article
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Backgrounds: The 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.

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 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).

Results: We 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.

Conclusions: The 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.


Fig. 1 The flowchart of patients enrollment of COHARD-PH registry


Fig. 2 The proportion of sex in COHARD-PH registry. The majority were female patients (78.5%)

Fig. 3 The percentage of symptoms of patients. Dyspnea on effort in 363 patients (35.9%), easily fatigued in 165 patients (16.3%), chest pain/discomfort in 109 patients (10.8%), palpitation in 94 patients (9.3%), cough in 63 patients (6.2%), headache in 15 patients (1.5%), leg swelling in 10 patients (1.0%), dizziness/syncope in 7 patients (0.7%), and other symptoms in 12 patients (1.2). Asymptomatic patients were 95 (9.4%) and indeterminate were 79 patients (7.8%)

Fig. 4 The PAH development associated with defect diameters in ASD. Minimum defect (2.3 ± 0.8 vs. 1.9 ± 1.5 cm, p < 0.001) and maximum defect (2.6 ± 0.9 vs. 2.2 ± 1.8 cm, p = 0.001) between ASD patients with PAH (n = 341) and those without PAH (n = 157)

Fig. 5 The PAH development did not associate with shunt flow in ASD. The Qp:Qs ratio by TTE (3.6 ± 2.2 vs. 3.3 ± 2.2, p = 0.118) between ASD patients with PAH (n = 306) and those without PAH (n = 138) and flow ratio by RHC (2.6 ± 1. 3vs. 2.8 ± 1.4, p = 0.080), between ASD patients with PAH (n = 326) and those without PAH (n = 137)