Abstract
Abstract Background and Aims Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). There were several variables predictor of mortality in PAH-CHD from previous studies, but study predictor of mortality in PAH-CHD with shunts has never been done, especially in Indonesia. This study aims to identify independent predictors of mortality among adult PAH-CHD with shunt in Dr. Sardjito Hospital, Yogyakarta, Indonesia. Method and Results We conducted a retrospective cohort study that included adult PAH-CHD patients with shunts from COHARD-PH (The Congenital HeARt Disease in adult and Pulmonary Hypertension) registry who were recruited from July 2012 - October 2020. Twelve variables were selected as potential predictors of mortality. Predictor variables were obtained from clinical (WHO-functional class, SaO2, 6-min walking distance), laboratory (haemoglobin, NT-pro BNP), echocardiographic (pericardial effusion, defect size, TAPSE), and hemodynamic (RA pressure, cardiac index, SvO2, flow ratio) data at the time of diagnosis of PAH. Baseline characteristics showed significantly lower systolic blood pressure, higher incidence of Eisenmenger syndrome, higher NT-pro BNP, and lower cardiac output in subjects who died as compared to those survived. Multivariate analysis showed cardiac index as an independent predictor of mortality (OR 7.69; p = 0.011). Systolic blood pressure was a significant confounder of cardiac index in affecting mortality (OR 10.56; p = 0.001). Conclusion Cardiac index was an independent predictor of mortality of PAH-CHD with shunts. Systolic blood pressure was a significant confounder of cardiac index on mortality.
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