BACKGROUND There is an increasing recognition of the potential value of cardiopulmonary exercise testing (CPX) in patients with pulmonary hypertension (PH). Key CPX characteristics in these patients include: (1) a diminished aerobic capacity; (2) an abnormally elevated minute ventilation-carbon dioxide production relationship; and (3) an abnormally diminished partial pressure of end-tidal carbo...

Background: Vitamin D (Vit D) is linked to various conditions including musculoskeletal, metabolic and   cardiopulmonary diseases. However, it is not clear whether correction of vit D deficiency exerts any beneficial effect in patients with pulmonary hypertension. Methods: This study was a prospective uncontrolled longitudinal study. Patients with pulmonary hypertension and vit D d...

M ore than 50 yrs ago, DRESDALE et al. [1] reported a series of 39 patients with unexplained pulmonary hypertension and coined the term ‘‘primary pulmonary hypertension’’ to describe the condition, a term that has been revised to ‘‘idiopathic pulmonary arterial hypertension’’ in the most recent classification [2]. Idiopathic pulmonary arterial hypertension is a disease of the small pulmonary ar...

To assess the validity of ultrasound criteria for pulmonary arterial hypertension, we studied pulmonary valve motion in 28 patients and 20 normal subjects. In the latter group, we categorised normal movement of the posterior leaflet of the pulmonary valve in a fashion not previously described. Of the 28 patients, 19 had pulmonary arterial hypertension (pulmonary artery mean pressure more than 2...

Background: Right Ventricular (RV) hypertrophy is an adaptive response to chronic RV pressure overload in patients with pulmonary hypertension. We investigated the relationships between RV hypertrophy indicators, including electrocardiography, the percentage oxygen saturation (SaO2%), body mass index (BMI), and blood uric acid levels in patients with...

Pulmonary arterial hypertension is an obstructive pulmonary vasculopathy and the RV considered a major determinant of survival in patients with precapillary hypertension. The transition from adaptive to maladaptive phenotype remains poorly understood clinically unpredictable. Several mechanisms having been suggested right heart disease but causes cardiac remodeling remain unknown require furthe...

We report a very rare case of severe pulmonary arterial hypertension in a patient with neurofibromatosis type 1, and discuss the pathology, pathogenesis, current pulmonary hypertension classification system, and outcomes of pulmonary arterial hypertension in patients with neurofibromatosis type 1.