BACKGROUND The prevalence of pulmonary arterial hypertension (PAH) in Saudi adults with sickle cell disease (SCD), the mechanism of its development, and its prospective prognostic significance are unknown. OBJECTIVE To assess the prevalence of PAH among sickle cell disease patients attended hematology outpatient clinic at King Fahad Hospital, Al Hassa, Saudi Arabia. METHODS Doppler echocard...

Pulmonary hypertension (PH) is defined by a mean pulmonary arterial pressure over 25 mmHg at rest and is diagnosed by right heart catheterization. Among the different groups of PH, pulmonary arterial hypertension (PAH) is characterized by a progressive obstruction of distal pulmonary arteries, related to endothelial cell dysfunction and vascular cell proliferation, which leads to an increased p...

Objective. The objective of this study was to determine the frequency of pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) with an isolated, large left-to-right shunt and to indicate the factors in the development of PAH. Methods. The pressure measurements in the cardiac chambers and the calculations based on the Fick's principle were compared among 3 separate groups of pa...

We evaluated the prevalence and prognostic value of CT-pulmonary angiographic (CTPA) measures in 292 treatment naive patients with pulmonary arterial hypertension (PAH). Pulmonary artery calcification (13%) and thrombus (10%) were exclusively seen in PAH-congenital heart disease. Oesophageal dilation (46%) was most frequent in PAH-systemic sclerosis. Ground glass opacification (GGO) (41%), peri...

Mutations in genes encoding members of the transforming growth factor (TGF)-beta superfamily have been identified in idiopathic forms of pulmonary arterial hypertension (PAH). The current study examined whether perturbations to the TGF-beta/Smad2,3 signalling axis occurred in a monocrotaline (MCT) rodent model of experimental PAH. Expression of the TGF-beta signalling machinery was assessed in ...

Despite the development of specific therapies for pulmonary arterial hypertension (PAH) some patients fail to respond to such treatment. One of the potential reasons for the unresponsiveness to targeted therapies may be the presence of fibrous occlusion of small pulmonary veins that accompanies pre-capillary arteriopathy. This type of pathologic change is called pulmonary veno-occlusive disease...

PURPOSE OF REVIEW Evidence-based therapies and guidelines for pulmonary arterial hypertension are critiqued. RECENT FINDINGS Morbidity and mortality in pulmonary arterial hypertension reflects failure of right ventricular compensation for increased afterload caused by obstructive pulmonary arterial remodeling. This predominantly reflects excessive proliferation/impaired apoptosis of smooth mu...

Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery (PA) pressure, which negatively affects the right ventricular (RV) function. This report shows a patient with severe PAH, on whom a comprehensive MRI exam was performed to evaluate both PA and RV. New imaging sequences were implemented for obtaining additional parameters about the patient's condition. The result...

conclusions the etiology of pah in thalassemia is multifactorial such as inflammatory mediators. also, the absence of the spleen plays an important role in developing a high trv and pah. results average age of the patients was 21.15 ± 6.68 years. no significant difference was observed in the pap between the 2 groups of thalassemia major and intermedia and also the 2 sex groups. indeed, 6.6% of ...

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...