Clinical and hemodynamic evaluation of schistosomiasis-associated pulmonary arterial hypertension from Egyptian pulmonary hypertension centers: epidemiology, risk factors, and survival determinants

TL;DR

This study compares Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) with idiopathic PAH in Egypt, highlighting differences in demographics, comorbidities, and survival factors.

Contribution

The study identifies ejection fraction as a key predictor of survival in Sch-PAH patients, offering insights for early risk assessment in developing regions.

Findings

Sch-PAH patients were significantly older and predominantly male compared to IPAH patients.

Lower ejection fraction was found to be an independent predictor of improved survival in Sch-PAH patients.

Sch-PAH is associated with higher comorbidity rates and more severe functional class and hemodynamic features.

Abstract

Schistosomiasis-associated PAH (Sch-PAH) falls under group I pulmonary hypertension and it affects 230 million people, mainly in sub-Saharan Africa. This significant burden has prompted studies on the clinical characteristics and outcomes of Sch-PAH to improve awareness and management, particularly in developing regions. This study investigates the characteristics of Sch-PAH, including demographics, clinical and hemodynamic features, and survival outcomes compared to idiopathic Pulmonary arterial hypertension (IPAH), while evaluating the prognosis and current clinical practices. A cohort of 83 patients, including 41 with Sch-PAH and 42 with IPAH, were studied retrospectively over five years (2019–2024) from 3 pulmonary hypertension centres in Egypt. Data collection focused on demographic details, comorbidities, echocardiographic findings, and survival rates. Sch-PAH patients were significantly older (50.4 ± 12 years) and predominantly male (P < 0.001) compared to IPAH patients (34.5 ± 9.7 years). Higher comorbidity rates, chiefly chest and hepatic disorders, were observed in Sch-PAH. The Sch-PAH group exhibited more WHO functional class IV cases and greater left atrium (LA) and pulmonary artery (PA) dilation (P = 0.007, P = 0.006, P = 0.01, respectively). Lower ejection fraction (EF%) and absence of PA dilation and portal hypertension were linked to improved survival, with EF% emerging as an independent survival predictor (OR = 0.72 and 0.55; P = 0.002 and < 0.001). The EF% cutoff below 66% showed high predictive accuracy for survival (AUC = 0.85, P = 0.008). Sch-PAH affects primarily older males with significant comorbidities. Its prevalence poses a public health challenge, making the identification of mortality predictors crucial for early patient risk assessment and improved prognosis management.

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