Unusual case of cerebral embolism after initiation of selexipag for sarcoidosis-related pulmonary hypertension: a case report

Background: Pulmonary hypertension is a rare complication of sarcoidosis. The pathogenesis of sarcoidosis-related pulmonary hypertension is multifactorial, and patients with sarcoidosis-related pulmonary hypertension can have variable treatment responses and prognoses. While selexipag (Nippon Shinyaku / Kyoto / Japan) was recently approved in Japan for the treatment of pulmonary hypertension, the risk of cerebral infarction has not been clearly reported. Case report: A 63-year-old Asian female with a diagnosis of ocular and cutaneous sarcoidosis developed shortness of breath and was referred to our department to rule out cardiac sarcoidosis. Swan–Ganz catheterization was performed, and she was diagnosed with pulmonary arterial hypertension and started on selexipag. A few days after starting treatment, she presented with hemiplegia and was diagnosed with cardiogenic cerebral embolism by using magnetic resonance imaging. As there was no evidence of pre-existing intracardiac thrombosis, we suspected unusual cerebral embolism. Echocardiography revealed a deep venous thrombus and a bubble study revealed a right-left shunt through a patent foramen ovale. Conclusions: The initiation of selexipag improved pulmonary blood flow and caused cerebral embolism, which was an unusual and unexpected event. This report highlights the importance of confirming the presence of patent foramen ovale and a deep venous thrombus before starting treatment for pulmonary hypertension.