Background Pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD) (CTD-PAH) remains a difficult challenge in clinical practice. We aimed to evaluate the effects of targeted vasodilators patients severe CTD-PAH. Methods The data 53 CTD-PAH hospitalized at Department Rheumatology and Immunology, Affiliated Drum Tower Hospital Nanjing University Medical School, were...

Atrial septal defect (ASD) with severe pulmonary arterial hypertension (PAH) is thought to preclude shunt closure. However, there are several reports that vasodilator treatment is associated with good clinical outcome in these patients, recently. We report a case of good clinical outcome in a patient with ASD and severe PAH successfully treated with operative closure of ASD and subsequent use o...

Pulmonary arterial hypertension (PAH) in patients with portal hypertension is also referred to as portopulmonary hypertension (PPHTN). Here, we report a case of PPHTN caused by alcoholic liver cirrhosis in a 43-year-old male who experienced repetitive syncope on exertion. The continuous monitoring of pulmonary artery pressure and radial artery pressure revealed that his PAH was aggravated with ...

OBJECTIVES This study evaluated the safety and efficacy of inhaled treprostinil as add-on therapy to oral bosentan in patients with pulmonary arterial hypertension (PAH). BACKGROUND The addition of a long-acting prostacyclin analogue via the inhaled route might be a safe and effective strategy to optimize therapy in PAH patients on bosentan. METHODS Twelve patients with symptomatic PAH desp...

Background: Two pivotal studies, RAPIDS-1 and RAPIDS-2 revealed that bosentan reduces the development of new digital ulcers (DUs) in patients with systemic sclerosis (SSc). However data regarding long-term use this dual endothelin antagonist receptor treatment DUs is scarce. Objectives: The aim present study was to evaluate long term efficacy safety profile related SSc. Methods: A prospective o...

microsomal epoxide hydrolase: genetic polymorphism and functional expression in vitro of amino acid variants. After great development in the knowledge of pulmonary arterial hypertension (PAH) during the last decades, there is now an increasing interest in the pathophysiology of other forms of pulmonary hypertension, such as chronic obstructive pulmonary disease (COPD). The results obtained in t...

Polycystic kidney disease (PKD) is characterized by interstitial fibrosis and formation of renal cysts. Interestingly, interstitial fibrosis and renal cyst formation were also seen in human endothelin-1 (ET-1) transgenic mice. This study, therefore, analyzes the tissue distribution of ET-1, the tissue concentrations of ET-1, as well as the expression of ET receptor subtypes in the kidneys of a ...

OBJECTIVES We sought to determine the optimal dose of the selective endothelin A (ET(A)) receptor antagonist sitaxsentan for the treatment of pulmonary arterial hypertension (PAH); for observation only, an open-label (OL) bosentan arm was included. BACKGROUND Endothelin is a mediator of PAH. In a preliminary PAH study, the selective ET(A) receptor antagonist sitaxsentan improved six-min walk ...

BACKGROUND In idiopathic pulmonary arterial hypertension (IPAH), peripheral airway obstruction is frequent. This is partially attributed to the mediator dysbalance, particularly an excess of endothelin-1 (ET-1), to increased pulmonary vascular and airway tonus and to local inflammation. Bosentan (ET-1 receptor antagonist) improves pulmonary hemodynamics, exercise limitation, and disease severit...