Abstract

Abstract Background Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease (CHD), notably in patients with left-to-right shunts. Patients with severe PAH due to CHD, Eisenmenger syndrome (ES), represent a high-risk group of pulmonary artery (PA) thrombosis patients. We present serial adult cases of CHD with PAH complicated by pulmonary embolism (PE). Case Description We found 3 cases of pulmonary embolism on adult congenital heart disease in our center. All of the patients were female, with an age range of 36 to 61 years old. The presenting symptoms were shortness of breath, WHO classification class II-III. All patients were hemodynamically stable. CHD and estimated RV systolic pressure were evaluated with echocardiography. CT-PA was performed in all patients, which findings were supporting PE. All patients were treated with anticoagulation. After three months, right heart catheterization was conducted in two patients, and one patient was refused. Discussion The prevalence of PE among adult patient with CHD and PAH range from 17 to 21%. PE was frequently found in older patients, women, and patients with lower oxygen saturation. To date, no uniformity regarding routine prophylaxis anticoagulation therapy in ES due to the high bleeding risk in this population despite being at risk of PA thrombosis. We conclude that risk stratification for PA thrombosis in adult CHD-PAH patients is essential, and further research is needed to prevent PE in this population.

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