Pulmonary arterial hypertension in a multi‐ethnic Asian population: Characteristics, survival and mortality predictors from a 14‐year follow‐up study

Lim, Yinghao; Low, T.S.; Chan, Siew‐Pang; Teo, Ting Wei; Jang, Jin-Hao Justin; Yip, Nicole; Kuntjoro, Ivandito; Tay, Edgar; Yip, James

doi:10.1111/resp.13392

Cited by 36 publications

(39 citation statements)

References 27 publications

(34 reference statements)

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“…The main symptoms of patients were associated with the development of PAH, which was later confirmed by RHC procedure. We excluded patients with complex adult CHDs from the analysis because most of them were already diagnosed in childhood and the proportion The early finding of CHD-related PAH is often not easy to recognize due to the unknown precise period of PAH [11]. The chronic systemic-to-pulmonary shunt is a congenital malformation causing blood overflow in the pulmonary vasculature from infancy, and if left untreated may give rise to PAH in adult life.…”

Section: Discussionmentioning

confidence: 99%

“…The prevalence and incidence of CHD-related PH are still unknown; nevertheless in clinical practice, adult patients with undetected and delayed diagnosis of CHD are frequent [10]. Compared with registries from developed countries, the situation regarding the adult CHDrelated PH in developing countries are very different [11][12][13]. The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry was initiated in 2012 to be the first registry done in Indonesia to describe adult CHD and CHD-related PH populations.…”

Section: Introductionmentioning

confidence: 99%

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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

Hartopo

Kusuma

et al. 2020

BMC Cardiovasc Disord

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Hartopo

Kusuma

et al. 2020

BMC Cardiovasc Disord

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“…Despite of the predictive value, the REVEAL score is too complicated to apply in routine practice. Additionally, it remains uncertain whether the existed risk assessment would be effective for PAH‐CHD patients who consisted of most PAH patients in Asia …”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary arterial hypertension (PAH) is characterised by progressively increased pulmonary arterial pressure, which results from several different diseases, such as congenital heart diseases, connective tissue disease, chronic thrombotic pulmonary disease and others. Different from the etiology in Western countries, PAH associated with congenital heart disease (PAH‐CHD) accounts for most incident of PAH in Asia . Similar to idiopathic PAH (iPAH), part of PAH‐CHD patients develops right heart failure because of the irreversible PAH regardless the targeting medicine therapy …”

Section: Introductionmentioning

confidence: 99%

Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study

Deng

Jin

et al. 2019

Clinical Respiratory J

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IntroductionCurrent guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been specified for PAH associated with congenital heart disease (PAH‐CHD).ObjectivesThe aim of this study was to propose an accurate and simple system based on current guidelines for risk stratification in PAH‐CHD patients during 12‐month follow‐up.MethodsWe reviewed 288 Chinese PAH‐CHD patients between January 2014 and December 2016 in this retrospective cohort study. The low‐risk criteria according to 2015 European Society of Cardiology guidelines and the adverse events (AEs) during follow‐up were collected. The association between low‐risk criteria and AEs was assessed with Cox regression, and a simplified risk stratification system was proposed.ResultsThere were 105 PAH‐CHD patients included in the final analysis. Twenty‐nine patients had AEs defined as death, initiation of new or combined medication treatment, or re‐hospitalisation because of the PAH worsening. Among the low‐risk criteria, WHO/NYHA functional class, 6‐minute walking distance (6MWD), NT‐proBNP and SvO2 were significantly different between AE and AE‐free groups. However, 6MWD (HR = 0.08, 95% CI: 0.03‐0.19, P < 0.001) and NT‐proBNP (HR = 0.35, 95% CI: 0.16‐0.78, P = 0.01) were the only independent predictors of AEs in multivariable model. When taking them into a simplified system for risk stratification, the number of low‐risk criteria at diagnosis discriminated the risk of AEs (P < 0.001).ConclusionsAmong the low‐risk criteria proposed by current guidelines, 6MWD and NT‐proBNP predicted AEs independently for PAH‐CHD patients. Simplified risk stratification system by taking these two parameters numerically provides accurate prognostic information in PAH‐CHD patients.

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“…Such registries include the REVEAL registry in the USA, PHSANZ in Australia and New Zealand and several European registries . A recent publication in Respirology by Lim et al . is an important addition to our understanding of the epidemiology and outcomes in PAH.…”

mentioning

confidence: 99%

Pulmonary arterial hypertension: In Asia, as elsewhere, still a lethal disease despite modern treatment

Reynolds

2018

Respirology

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Pulmonary arterial hypertension in a multi‐ethnic Asian population: Characteristics, survival and mortality predictors from a 14‐year follow‐up study

Abstract: In this first registry study from a South-East Asian population, our survival rates are comparable with other national registries. The RS is validated in our population to be a good predictor of mortality.

Cited by 36 publications

References 27 publications

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

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

Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study

Pulmonary arterial hypertension: In Asia, as elsewhere, still a lethal disease despite modern treatment

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