Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registry

Background: Pulmonary arterial hypertension (PAH) is a complication of left-to-right intracardiac shunt congenital heart disease (LtR-shunt CHD). There are several known predictors of mortality in PAH patients, however predictors of mortality in LtR-shunt CHD-associated PAH need to be validated....

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Main Authors: Muflihatul Baroroh Rochmat, Budi Yuli Setianto, Dyah Wulan Anggrahini, Lucia Kris Dinarti, Anggoro Budi Hartopo
Format: Article
Language:English
Published: Indonesian Heart Association 2025-01-01
Series:Majalah Kardiologi Indonesia
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Online Access:https://ijconline.id/index.php/ijc/article/view/1623
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author Muflihatul Baroroh Rochmat
Budi Yuli Setianto
Dyah Wulan Anggrahini
Lucia Kris Dinarti
Anggoro Budi Hartopo
author_facet Muflihatul Baroroh Rochmat
Budi Yuli Setianto
Dyah Wulan Anggrahini
Lucia Kris Dinarti
Anggoro Budi Hartopo
author_sort Muflihatul Baroroh Rochmat
collection DOAJ
description Background: Pulmonary arterial hypertension (PAH) is a complication of left-to-right intracardiac shunt congenital heart disease (LtR-shunt CHD). There are several known predictors of mortality in PAH patients, however predictors of mortality in LtR-shunt CHD-associated PAH need to be validated. Objectives: We aimed to investigate the predictors of mortality among adult LtR-shunt CHD-associated PAH patients.  Methods: This research was a retrospective cohort study that included adult patients with LtR-shunt CHD-associated PAH retrieved from the COHARD-PH registry. Several baseline variables were selected as potential predictors of mortality, namely (1) clinical data: WHO-functional class, SaO2, 6-min walking distance, systolic blood pressure, and Eisenmenger syndrome; (2) laboratory data: hemoglobin and NT-pro BNP levels; (3) echocardiography data: pericardial effusion, defect size, and TAPSE; and (4) hemodynamic data: right atrial pressure, cardiac output and index, SvO2, and flow ratio. The mortality outcome was assessed from the cohort registry. Results: A total of 124 subjects with LtR-shunt CHD-associated PAH were included. Sixteen subjects (12.9%) died during the follow-up period. The baseline variables which showed significant association with mortality were lower systolic blood pressure, Eisenmenger syndrome, higher NT-pro BNP level, and lower cardiac output. The multivariable analysis showed that systolic blood pressure <100 mmHg (OR 10.99; 95% CI 2.54-47.51, p=0.001), cardiac index <2.5 L/min/m2 (OR 8.13; 95% CI: 1.59-42.28, p=0.011) and Eisenmenger syndrome (OR 3.87; 95%CI: 1.06-14.07) were the independent predictors for mortality. Conclusions: The systolic blood pressure <100 mmHg, cardiac index <2.5 L/min/m2, and Eisenmenger syndrome were independent predictors of mortality among adults with LtR-shunt CHD-associated PAH.
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spelling doaj-art-7add81047cf74ec4bdab1a7d571d59b02025-01-10T16:38:16ZengIndonesian Heart AssociationMajalah Kardiologi Indonesia0126-37732620-47622025-01-0146110.30701/ijc.1623Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registryMuflihatul Baroroh RochmatBudi Yuli SetiantoDyah Wulan AnggrahiniLucia Kris DinartiAnggoro Budi Hartopo Background: Pulmonary arterial hypertension (PAH) is a complication of left-to-right intracardiac shunt congenital heart disease (LtR-shunt CHD). There are several known predictors of mortality in PAH patients, however predictors of mortality in LtR-shunt CHD-associated PAH need to be validated. Objectives: We aimed to investigate the predictors of mortality among adult LtR-shunt CHD-associated PAH patients.  Methods: This research was a retrospective cohort study that included adult patients with LtR-shunt CHD-associated PAH retrieved from the COHARD-PH registry. Several baseline variables were selected as potential predictors of mortality, namely (1) clinical data: WHO-functional class, SaO2, 6-min walking distance, systolic blood pressure, and Eisenmenger syndrome; (2) laboratory data: hemoglobin and NT-pro BNP levels; (3) echocardiography data: pericardial effusion, defect size, and TAPSE; and (4) hemodynamic data: right atrial pressure, cardiac output and index, SvO2, and flow ratio. The mortality outcome was assessed from the cohort registry. Results: A total of 124 subjects with LtR-shunt CHD-associated PAH were included. Sixteen subjects (12.9%) died during the follow-up period. The baseline variables which showed significant association with mortality were lower systolic blood pressure, Eisenmenger syndrome, higher NT-pro BNP level, and lower cardiac output. The multivariable analysis showed that systolic blood pressure <100 mmHg (OR 10.99; 95% CI 2.54-47.51, p=0.001), cardiac index <2.5 L/min/m2 (OR 8.13; 95% CI: 1.59-42.28, p=0.011) and Eisenmenger syndrome (OR 3.87; 95%CI: 1.06-14.07) were the independent predictors for mortality. Conclusions: The systolic blood pressure <100 mmHg, cardiac index <2.5 L/min/m2, and Eisenmenger syndrome were independent predictors of mortality among adults with LtR-shunt CHD-associated PAH. https://ijconline.id/index.php/ijc/article/view/1623congenital heart defectspulmonary hypertensionmortalityEisenmenger syndrome
spellingShingle Muflihatul Baroroh Rochmat
Budi Yuli Setianto
Dyah Wulan Anggrahini
Lucia Kris Dinarti
Anggoro Budi Hartopo
Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registry
Majalah Kardiologi Indonesia
congenital heart defects
pulmonary hypertension
mortality
Eisenmenger syndrome
title Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registry
title_full Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registry
title_fullStr Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registry
title_full_unstemmed Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registry
title_short Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registry
title_sort systolic blood pressure cardiac index and eisenmenger syndrome are predictors of mortality in pulmonary arterial hypertension associated with congenital heart disease an analysis from the cohard ph registry
topic congenital heart defects
pulmonary hypertension
mortality
Eisenmenger syndrome
url https://ijconline.id/index.php/ijc/article/view/1623
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