scholarly journals 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

2020 ◽  
Author(s):  
Lucia Kris Dinarti ◽  
Anggoro Budi Hartopo ◽  
Arditya Damar Kusuma ◽  
Muhammad Gahan Satwiko ◽  
Muhammad Reyhan Hadwiono ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Clinical Presentation ◽  
Right Heart Catheterization ◽  
Walking Distance ◽  
Heart Catheterization ◽  
Single Center ◽  
Right Heart

Abstract Backgrounds: The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first registry for congenital heart disease (CHD) andCHD-related pulmonary hypertension (PH) in adults in Indonesia.The study aims to describethe 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 evaluatedthe registry patients from July 2012 until July 2019. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6-minute walking test, laboratory measurement, and transthoracic and transesophageal echocardiography. Right heart catheterization was performed to measure hemodynamics and confirmthe 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-minute 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.

scholarly journals 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

2020 ◽  
Author(s):  
Lucia Kris Dinarti ◽  
Anggoro Budi Hartopo ◽  
Arditya Damar Kusuma ◽  
Muhammad Gahan Satwiko ◽  
Muhammad Reyhan Hadwiono ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Clinical Presentation ◽  
Right Heart Catheterization ◽  
Walking Distance ◽  
Heart Catheterization ◽  
Single Center ◽  
Right Heart

Abstract Backgrounds: The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first registry for congenital heart disease (CHD) andCHD-related pulmonary hypertension (PH) in adults in Indonesia.The study aims to describethe 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 evaluatedthe registry patients from July 2012 until July 2019. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6-minute walking test, laboratory measurement, and transthoracic and transesophageal echocardiography. Right heart catheterization was performed to measure hemodynamics and confirmthe 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-minute 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.

scholarly journals 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

2019 ◽  
Author(s):  
Lucia Kris Dinarti ◽  
Anggoro Budi Hartopo ◽  
Arditya Damar Kusuma ◽  
Muhammad Gahan Satwiko ◽  
Muhammad Reyhan Hadwiono ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Clinical Presentation ◽  
Walking Distance ◽  
Single Center ◽  
Right Heart ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation

Abstract Backgrounds: The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first congenital heart disease (CHD) and CHD-related pulmonary hypertension (PH) in adults registry in Indonesia. The study aims to describe prevalence, characteristics, and hemodynamic 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 2018 2019. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6 minute walking test, laboratory measurement, and transthoracic and transoesofageal echocardiography. Right heart catheterisation was performed to measure hemodynamics and confirmed the diagnosis of pulmonary artery hypertension (PAH). Results: We registered 803 1012 patients during the study. The majority were young-adult females. The majority of CHD was secundum ASD (79.0%) (73.4%). The main symptom was dyspneu on effort. The majority of patients (78.1%) (77.1%) had already developed signs of PH assessed by echocardiography. The Eisenmenger syndrome was encountered in 17.3% 18.7% patients. Based on the right heart catheterisation, 67.8% 66.9% patients had developed PAH. Patients with PAH were significantly older, had lower peripheral oxygen saturation, had lower 6 minute walking distance, and had higher NTproBNP. There was an increased of proportion of PAH prevalence according to age range, with the highest prevalence between 51 and 60 years old. Conclusions: The COHARD-PH registry is the first Indonesian adult-CHD and CHD-related PH registry. The prevalence, characteristics, and hemodynamics data demographics, clinical presentation, and hemodynamics data of this registry reflects situation in developing countries which need to be compared with similar registries from developed countries.

scholarly journals 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

2019 ◽  
Author(s):  
Lucia Kris Dinarti ◽  
Anggoro Budi Hartopo ◽  
Arditya Damar Kusuma ◽  
Vera Christina Dewanto ◽  
Monika Setiawan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Developed Countries ◽  
Walking Distance ◽  
Heart Catheterization ◽  
Single Center ◽  
Peripheral Oxygen Saturation

Abstract Backgrounds The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first congenital heart disease (CHD) and PH registry in Indonesia. The study aims to describe prevalence, demographics, and hemodynamics data of adult CHD and PH in Indonesia.Methods The COHARD-PH registry is a hospital-based, single-center, and prospective cohort registry which includes adult patients with CHD and CHD-related PH. The patients were enrolled consecutively. We evaluate the registry patients from July 2012 until July 2018. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6 minute walking test, laboratory measurement, and transthoracic and transoesofageal echocardiography. Right Heart Catheterization (RHC) was performed to measure hemodynamics and confirmed the diagnosis of pulmonary artery hypertension (PAH).Results We registered 803 patients during the study. The majority were young-adult females. The majority of CHD was secundum ASD (79.0%). The main symptom was dyspneu on effort. The majority (78.1%) already develops signs of PH assessed by echocardiography. The Eisenmenger syndrome was encountered in 17.3% subjects. Based on the RHC, 67.8% subjects had developed PAH. Patients with PAH were significantly older, lower peripheral oxygen saturation, lower 6 minute walking distance, and higher NTproBNP. There was an increased of PAH prevalence according to age range, with the highest prevalence between 51 and 60 years old.Conclusions The COHARD-PH registry is the first Indonesian adult-CHD and PH registry. The incidence, demographics data, and hemodynamics data of this registry reflects situation in developing countries which need to be compared with similar registries from developed countries.

scholarly journals Exercise right heart catheterization before and after balloon pulmonary angioplasty in inoperable patients with chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402091788
Author(s):  
Christoph B. Wiedenroth, MD ◽  
Andreas J. Rieth, MD ◽  
Steffen Kriechbaum, MD ◽  
H.-Ardeschir Ghofrani, MD ◽  
Andreas Breithecker, MD ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Exercise Capacity ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Walking Distance ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Hemodynamics ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

Background * These authors contributed equally as last authors. Balloon pulmonary angioplasty is an evolving, interventional treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary hypertension at rest as well as exercise capacity is considered to be relevant outcome parameters. The aim of the present study was to determine whether measurement of pulmonary hemodynamics during exercise before and six months after balloon pulmonary angioplasty have an added value. Methods From March 2014 to July 2018, 172 consecutive patients underwent balloon pulmonary angioplasty. Of these, 64 consecutive patients with inoperable CTEPH underwent a comprehensive diagnostic workup that included right heart catheterization at rest and during exercise before balloon pulmonary angioplasty treatments and six months after the last intervention. Results Improvements in pulmonary hemodynamics at rest and during exercise, in quality of life, and in exercise capacity were observed six months after balloon pulmonary angioplasty: WHO functional class improved in 78% of patients. The mean pulmonary arterial pressure (mPAP) at rest was reduced from 41 ± 9 to 31 ± 9 mmHg (p < 0.0001). The mPAP/cardiac output slope decreased after balloon pulmonary angioplasty (11.2 ± 25.6 WU to 7.7 ± 4.1 WU; p < 0.0001), and correlated with N-terminal fragment of pro-brain natriuretic peptide (p = 0.035) and 6-minute walking distance (p = 0.01). Conclusions Exercise right heart catheterization provides valuable information on the changes of pulmonary hemodynamics after balloon pulmonary angioplasty in inoperable CTEPH patients that are not obtainable by measuring resting hemodynamics.

scholarly journals Adult congenital heart disease and the COVID-19 pandemic

Heart ◽  
2020 ◽  
Vol 106 (17) ◽  
pp. 1302-1309 ◽  
Author(s):  
Robert M Radke ◽  
Tim Frenzel ◽  
Helmut Baumgartner ◽  
Gerhard-Paul Diller
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
High Risk ◽  
Congenital Heart ◽  
Pragmatic Approach ◽  
Right Heart ◽  
High Risk Patients ◽  
Risk Patients

Adults with congenital heart disease (ACHD) may be at high risk in the case of COVID-19. Due to the heterogeneity of ACHD and secondary complications, risk profiles are, however, not uniform. This document aims to give an overview of relevant data and outline our pragmatic approach to disease prevention and management. Based on anatomy and additional physiological factors including symptoms, exercise capacity, heart failure, pulmonary hypertension and cyanosis, we propose a pragmatic approach to categorising patients into low-risk, intermediate-risk and high-risk groups. We regard especially patients with complex cyanotic conditions, those with palliated univentricular hearts, heart failure, severe valvular disease or pulmonary hypertension as high-risk patients. To avoid infection, we recommend self-isolation and exemption from work for these cohorts. Infected ACHD patients with low or moderate risk and without signs of deterioration may be remotely followed and cared for at home while in self isolation. High-risk patients or those with signs of respiratory or cardiovascular impairment require admission ideally at a tertiary ACHD centre. Especially patients with complex, cyanotic disease, heart failure and arrhythmias require particular attention. Treatment in patients with cyanotic heart disease should be guided by the relative degree of desaturation compared with baseline and lactate levels rather than absolute oxygen saturation levels. Patients with right heart dilatation or dysfunction are potentially at increased risk of right heart failure as mechanical ventilation and acute respiratory distress syndrome can lead to increase in pulmonary arterial pressures.

scholarly journals Pulmonary Hypertension in Children across Africa: The Silent Threat

2021 ◽  
Vol 2021 ◽  
pp. 1-13
Author(s):  
Judith Namuyonga ◽  
Ana Olga Mocumbi
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Rheumatic Heart Disease ◽  
Congenital Heart ◽  
Premature Mortality ◽  
Right Heart Catheterization ◽  
Sub Saharan Africa ◽  
Heart Catheterization ◽  
Sub Saharan ◽  
Rheumatic Heart

Pulmonary hypertension (PH) is a complex puzzle in Africa, especially among children who present with a cocktail of issues including recurrent pulmonary infections, unoperated congenital heart disease, and advanced rheumatic heart disease. Sickle cell anemia and neonatal complications of transiting from fetal circulation also contribute to the burden of pulmonary hypertension. Mortality from pulmonary arterial hypertension (PAH) remains high in Africa (18-21%), claiming sufferers in the first 6 months after diagnosis. Unfortunately, PH remains underreported in sub-Saharan Africa since many centers lack the capacity to diagnose and confirm it by the recommended gold standard, right heart catheterization. The unresolved burden of unoperated congenital heart lesions and rheumatic heart disease, among other preventable causes, stand out as major causes of PH in African children. This paper highlights pediatric PAH as a result of major gaps in care and illustrates the need for its prevention as well as for the promotion of research into the most important drivers, to prevent premature mortality in the continent.

scholarly journals The Essential Role of Imaging in the Evaluation of Patients With Pulmonary Arterial Hypertension in Association With Congenital Heart Disease

2013 ◽  
Vol 11 (4) ◽  
pp. 171-182
Author(s):  
Giancarlo Scognamiglio ◽  
Sonya V. Babu-Narayan ◽  
Michael B. Rubens ◽  
Michael A. Gatzoulis ◽  
Wei Li
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Pulmonary Arterial ◽  
The Individual

Pulmonary arterial hypertension (PAH) is a hemodynamic and pathophysiologic condition defined as an increase in mean pulmonary artery pressure (MPAP) of ≥25 mm Hg at rest measured at right heart catheterization (RHC).12 Patients with PAH associated with congenital heart disease (PAH-CHD) are a growing population consisting of an anatomically and phenotypically heterogeneous group, where differences among specific cardiac defects, along with their varied clinical course and prognosis, influence treatment choices for the individual patient.

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