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Update on chronic thromboembolic pulmonary hypertension Pulmonary hypertension related to congenital heart disease: a call for action 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT) Active and Passive Vaccination for Pulmonary Arterial Hypertension: A Novel Therapeutic Paradigm Diagnosis and management of acute deep vein thrombosis: a joint consensus document from the European Society of Cardiology working groups of aorta and peripheral vascular diseases and pulmonary circulation and right ventricular function Haemodynamic definitions and updated clinical classification of pulmonary hypertension Stress Echocardiography and PH: What Do the Findings Mean? Genetic analyses in a cohort of 191 pulmonary arterial hypertension patients Circulating Plasma microRNAs In Systemic Sclerosis-Associated Pulmonary Arterial Hypertension Will Pulmonary Artery Denervation Really Have a Place in the Armamentarium of the Pulmonary Hypertension Specialist?
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Clinical Case Study2018 May 23. [Epub ahead of print]

JOURNAL：Int Heart J. Article Link

A Case of Pulmonary Hypertension Associated with Idiopathic Hypereosinophilic Syndrome

Ibe T, Wada H, Sakakura K et al. Keywords: Corticosteroid; Hypereosinophilia; Pulmonary arterial hypertension; Steroid therapy

ABSTRACT

Hypereosinophilic syndrome (HES) is characterized by multi-organ damage that is associated with tissue hypereosinophilia. A persistently elevated eosinophilic count is also required for the diagnosis of HES. Although HES affects various organs, damage to pulmonary artery is rarely reported. We present a case of a 39-year-old man who was diagnosed with pulmonary hypertension (PH) associated with idiopathic HES. Although the pulmonary arterial hypertension specific drugs including intravenous epoprostenol could not control his PH, corticosteroid was effective for both hypereosinophilia and PH. Our case suggests the importance of steroid therapy as well as specific drugs for pulmonary arterial hypertension in the treatment of PH associated with HES.
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