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
Select item 32266496
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)