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...
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...
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...
Guideline | By Galiè N, Humbert M, ESC Scientific Document Group.
Progression of Device-Detected Subclinical Atrial Fibrillation and the Risk of Heart Failure
Review Article | By Knuuti J, Ballo H, Juarez-Orozco LE et al.
AIMS- To determine the ranges of pre-test probability (PTP) of coronary artery disease (CAD) in which stress electrocardiogram (ECG), stress echocardiography, coronary computed tomography angiography (CCTA), single-photon emission computed tomography (SPECT), positron emission tomography (PET), and cardiac magnetic resonance (CMR) can reclassify patients into a post-testprobability that defines (>85%) or excludes (<...