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Prognostic Effect and Longitudinal Hemodynamic Assessment of Borderline Pulmonary Hypertension Skeletal muscle mitochondrial oxidative phosphorylation function in idiopathic pulmonary arterial hypertension: in vivo and in vitro study 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) Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study The association between body mass index and obesity with survival in pulmonary arterial hypertension Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension Clinical and genetic characteristics of pulmonary arterial hypertension in Lebanon Right ventricular stroke work correlates with outcomes in pediatric pulmonary arterial hypertension Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension: a multicenter, double-blind, randomized clinical trial 中国肺高血压诊断和治疗指南2018
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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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