Schistosomiasis: A neglected cause of pulmonary arterial hypertension in Brazil

• Published in: Current problems in cardiology 2024-03, Vol.49 (3), p.102340-102340, Article 102340
• Main Authors: Bobrovski, Vinícius G., Pinheiro, Alisson L., de-Castro, João A.L., Rech, Eduardo B., Zacarkim, Ezequiel V., dos-S-Reis, Elise S., Eger, Iriane
• Format: Article
• Language: English
• Subjects: Brazil - epidemiology; Complications of schistosomiasis; Familial Primary Pulmonary Hypertension - complications; Humans; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - epidemiology; Hypertension, Pulmonary - etiology; Pulmonary Arterial Hypertension; Pulmonary hypertension; Schistosomiasis; Schistosomiasis - complications; Schistosomiasis - diagnosis; Schistosomiasis - drug therapy
• ISSN: 0146-2806; 1535-6280
• DOI: 10.1016/j.cpcardiol.2023.102340

•S. mansoni, endemic in Brazil, is the main cause of PAH-Sch.•Periportal fibrosis associated with PAH is highly suggestive of PAH-Sch.•Exclusive illustration of the pathophysiology of PAH-Sch and the parasite life cycle.•Proposal for an algorithm from diagnosis to treatment of PAH-Sch in Brazil.•The impact of anthelmintic therapy on the evolution of PAH. Schistosomiasis is a prevalent disease in Brazil whose etiological agent is Schistosoma mansoni, the main species associated with pulmonary arterial hypertension (PAH), a serious complication. It is estimated that this complication affects up to 15% of patients with the hepatosplenic form of the disease. Despite being an endemic country, Brazil does not have a screening scheme for cases of PAH associated with schistosomiasis (PAH-Sch), nor protocols for notification and treatment of this vascular complication. The objectives of this literature review are to gather knowledge about the pathophysiology, clinical manifestations, diagnosis and treatment of PAH-Sch and to highlight relevant aspects for the Brazilian reality. The pathophysiology, although lacking information, has proliferative vasculopathy as a central element. The clinical presentation of this disease can be asymptomatic or with nonspecific manifestations. Thus, complementary exams are essential for a confirmatory diagnosis, the gold standard being right heart catheterization, a scarce resource in endemic regions of the country. The treatment of PAH-Sch is similar to that performed for other causes of PAH, but the impact of anthelmintic therapy on the evolution of the vascular pathology is unknown. Therefore, Brazil needs to develop a screening plan for early diagnosis of PAH-Sch and new studies should be carried out to determine a more specific treatment. [Display omitted]