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Clinical and genetic characteristics of pulmonary arterial hypertension in Lebanon

Clinical Trial | By Abou Hassan OK, Haidar W, BouAkl I et al.

BACKGROUND - Pulmonary arterial hypertension (PAH) is a rare disease with an incidence rate of 2-6 cases per million per year. Our knowledge of the disease in the Middle East and North Africa (MENA) region is limited by the small number of clinical studies and the complete absence of genetic studies. METHODS - Our aim was to shed light on the clinical and genetic characteristics of PAH in Lebanon an...

The association between body mass index and obesity with survival in pulmonary arterial hypertension

Clinical Trial | By Weatherald J, Huertas A, Boucly A et al.

BACKGROUND - An obesity paradox, wherein obese patients have lower mortality, has been described in cardiopulmonary diseases, including pulmonary arterial hypertension (PAH). Our objective was to determine whether obesity and body mass index (BMI) are associated with mortality in patients with PAH. METHODS - We assessed incident patients with idiopathic, drug-induced, and heritable PAH from the Fre...

Skeletal muscle mitochondrial oxidative phosphorylation function in idiopathic pulmonary arterial hypertension: in vivo and in vitro study

Clinical Trial | By Sithamparanathan S, Rocha MC, Parikh JD et al.

Mitochondrial dysfunction within the pulmonary vessels has been shown to contribute to the pathology of idiopathic pulmonary arterial hypertension (IPAH). We investigated the hypothesis of whether impaired exercise capacity observed in IPAH patients is in part due to primary mitochondrial oxidative phosphorylation (OXPHOS) dysfunction in skeletal muscle. This could lead to potentially new avenues of treatment beyond targeting the pulmona...

A Case of Pulmonary Hypertension Associated with Idiopathic Hypereosinophilic Syndrome

Clinical Case Study | By Ibe T, Wada H, Sakakura K et al.

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...

Right ventricular stroke work correlates with outcomes in pediatric pulmonary arterial hypertension

Original Research | By Yang W, Marsden AL, Ogawa MT et al.

Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). Optimizing treatment strategies and timing for transplant remains challenging. Thus, a quantitative measure to predict disease progression would be greatly beneficial in treatment planning. We devised a novel method to assess right ventricular (RV) stroke work (RVSW) as a potential biomarker of...

Genetic analyses in a cohort of 191 pulmonary arterial hypertension patients

Clinical Trial | By Yang H, Zeng Q, Zhou Z et al.

BACKGROUND - Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder associated with high pulmonary artery pressure. Genetic testing enables early diagnosis and offers an opportunity for family screening. To identify genetic mutations and help make a precise diagnosis, we performed genetic testing in 191 probands with PAH and tried to analyze the genotype-phenotype correlation. METHODS - Ini...

rhACE2 Therapy Modifies Bleomycin-Induced Pulmonary Hypertension via Rescue of Vascular Remodeling

Original Research | By Rathinasabapathy A, Bryant AJ, Suzuki T et al.

BACKGOURND - Pulmonary hypertension (PH) is a progressive cardiovascular disease, characterized by endothelial and smooth muscle dysfunction and vascular remodeling, followed by right heart failure. Group III PH develops secondarily to chronic lung disease such as idiopathic pulmonary fibrosis (IPF), and both hastens and predicts mortality despite of all known pharmacological interventions. Thus, there is urgent need for development of n...

Sildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: A Phase IIb, randomised, double-blind, placebo-controlled study - Rationale and study design

Clinical Trial | By Behr J, Nathan SD, Harari S et al.

BACKGROUND - Pulmonary hypertension (PH) is commonly observed in patients with advanced idiopathic pulmonary fibrosis (IPF). Despite the availability of therapies for both IPF and PH, none are approved for PH treatment in the context of significant pulmonary disease. This study will investigate the use of sildenafil added to pirfenidone in patients with advanced IPF and risk of PH, who represent a group with a high unmet me...

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)

Guideline | By Galiè N, Humbert M, ESC Scientific Document Group.

Progression of Device-Detected Subclinical Atrial Fibrillation and the Risk of Heart Failure

Clinical Trial | By Wong JA, Conen D, Van Gelder IC et al.

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