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 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.

Association between serum soluble ST2 level and right ventricle systolic function on pulmonary hypertension due to atrial septal defect

“…In the COHARD-PH registry, most patients with ASD did not diagnosed properly in childhood and consequently develop complication of PH, Eisenmenger syndrome and right heart failure. 8 This study was the first to assess the association between serum sST2 level, taken from pulmonary artery, with S' parameter in ASD-associated PH. Previous research had shown a significant correlation between serum sST2 with RV systolic function parameters,i.e Tricuspid Annular Plane Systolic Excursion (TAPSE) and Right Ventricle Fractional Area Change (RVFAC), in the PH subjects population due to chronic obstructive pulmonary disease.…”

“…The subjects were patients registered in the Congenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry, Yogyakarta, Indonesia whom recruited between January 2017 and April 2018 period. 8 The inclusion criteria of this study were1) adult (≥ 18 years old) uncorrected secundum ASD; 2) patients had been undergone right heart catheterization (RHC) and confirmed PAH, and 3) patients had sinus rhythm in electrocardiogram. The exclusion criteria of this study were 1) patients with history of myocardial infarction; 2) patients with impaired left ventricular systolic function; 3) patients have significant valve diseases; 4) patients have lung diseases (asthma bronchial, chronic obstructive and restrictive lung diseases, and post lung tuberculosis); 5) patients with diabetes mellitus; 6) patients with chronic renal disease, and 7) patients with a history of malignancy.…”

Pulmonary artery hypertension patients and the coronavirus disease of 2019 (COVID-19): are they protected from severe disease?

“…In our registry of adult with congenital heart disease and pulmonary hypertension (COHARD-PH registry) and primary PAH, the majority of patients was at age 30-50 years old. 11 These populations have less risk to develop severe and complicated COVID-19. Older ages at > 65 years old are the most vulnerable populations to develop severe COVID-19.…”

“…3,5 The lower body mass index Lower body mass index is observed in patients with PAH, especially CHDassociated PAH. 7,11 The obesity is one of risk factors for developing severe COVID-19.…”

Exercise Program Improves Functional Capacity and Quality of Life in Uncorrected Atrial Septal Defect-Associated Pulmonary Arterial Hypertension: A Randomized-Control Pilot Study

“…The prevalence of ASD-PAH is 9%–35% worldwide [ 3 ]. In Indonesia, among adult ASD patients registered in the COngenital HeARt Disease in adults and Pulmonary Hypertension (COHARD-PH) registry in our hospital, 67% had developed PAH, and around 20% of them had severe PAH [ 4 ]. Based on correctability criteria for defect closure, only 59.1% of the patients were suitable for closure either by surgery or device [ 4 ].…”