BACKGROUND Decline in oxygen uptake efficiency (OUE), especially during exercise, is found in patients with chronic heart failure. In this study we aimed to test the validity and usefulness of OUE in evaluating gas exchange abnormality of patients with idiopathic pulmonary arterial hypertension (IPAH). METHODS We retrospectively investigated the cardiopulmonary exercise test (CPET) with gas e...

INTRODUCTION Despite the prognostic importance of traditionally derived measurements, the significance of right heart catheterization (RHC) remains controversial. Thus, a continued search for hemodynamic markers that define better responsive patients is required. Since, right ventricular failure is the most fatal pathway, right (RVPO) and left (LVPO) ventricular power output are parameters that...

Background—Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH. Methods and Results—Two registries were used to retrospectively identify children (0–17 years) w...

Pulmonary vasoconstriction and vascular remodeling are two major causes for elevated pulmonary vascular resistance and pulmonary arterial pressure in patients with idiopathic pulmonary arterial hypertension (IPAH). An increase in cytosolic free Ca(2+) concentration ([Ca(2+)](cyt)) in pulmonary artery smooth muscle cells (PASMC) is a major trigger for pulmonary vasoconstriction and an important ...

AIMS Increased afterload in idiopathic pulmonary arterial hypertension (IPAH) causes right ventricular (RV) hypertrophy and failure. Since RV remodelling occurs with alterations in RV oxygen metabolism, increasing our understanding in the factors determining RV O(2) consumption in IPAH is necessary. In the left ventricle, it is known that heart rate and systolic blood pressure are the main dete...

A recent article by Lindsay and others reported that stool quantitative polymerase chain reaction (qPCR), with a 14,000 copy number cutoff, identified more cases of Shigella infection than conventional and widely used culture methods. The authors suggested that there may be a significant underestimation of the contribution of Shigella to diarrheal disease because of the limits of culture and ha...

Despite systematic screening and improved treatment strategies, the prognosis remains worse in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) compared to idiopathic/hereditary (IPAH). We aimed investigate differences clinical characteristics, outcome, performance of ESC/ERS risk stratification tool these patient groups. This retrospective analysis i...

ObjectivesDespite advances in treatment of idiopathic pulmonary arterial hypertension (IPAH), there remains no medical cure, and patients can experience disease progression leading to right heart failure, progressive exercise intolerance, death. The reversed Potts shunt (left artery descending aorta) was reintroduced for end-stage IPAH permit decompression the suprasystemic ventricle by left sh...

Several human diseases have been associated with mitochondrial voltage-dependent anion channel-1 (VDAC1) due to its role in calcium ion transportation and apoptosis. Recent studies suggest that VDAC1 may interact with endothelium-dependent nitric oxide synthase (eNOS). Decreased VDAC1 expression may limit the physical interaction between VDAC1 and eNOS and thus impair nitric oxide production, l...