Pulmonary hypertension, a common manifestation of advanced sarcoidosis, is thought to result from fibrosis with chronic hypoxia and destruction of small vessels, extrinsic compression of pulmonary arteries, or granulomatous vasculitis. We report a case of sarcoidosis-associated pulmonary hypertension due to fibrosing mediastinitis. Our patient presented with cough and dyspnea on exertion and wa...

ABBREVIATIONS : AHI 5 apnea-hypopnea index ; BNP 5 B-type natriuretic peptide ; CRP 5 C-reactive protein ; CSA 5 central sleep apnea ; CSR 5 Cheyne-Stokes respiration ; CTEPH 5 chronic thromboembolic pulmonary hypertension ; IPAH 5 idiopathic pulmonary arterial hypertension ; LV 5 left ventricular ; mPAP 5 mean pulmonary artery pressure ; NPV 5 negative predictive value ; OHS 5 obesity hypovent...

Left-sided pulmonary artery agenesis is a rare malformation that commonly requires childhood intervention secondary to associated congenital cardiovascular anomalies. We present an uncommon case of left-sided agenesis with an associated right-sided aortic arch and significant hypoplasia of the ipsilateral lung. Additionally, there is radiographic evidence of emphysema and pulmonary artery hyper...

Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hyp...

Objective: Rare disease Background: Pulmonary Langerhans cell histiocytosis (PLCH) can be associated with pulmonary hypertension, although this association is more prevalent with other interstitial lung diseases. However, the diagnosis and effective treatment strategies for PLCH-associated pulmonary hypertension remain controversial. Case Report: A 27-year-old woman, who was an ex-smoker, was d...

The appearance of the right ventricular myocardium on thallium 201 myocardial perfusion images was evaluated in patients with chronic pulmonary hypertension and compared to patients without pulmonary hypertension. Four groups of patients were studied: 1) eight normals, 2) five patients with angiographically documented coronary artery disease and normal pulmonary artery pressures, 3) ten patient...

Pulmonary arterial hypertension affects the distal pulmonary vasculature causing intimal, medial, adventitial fibrosis, thickening and periadventitial changes leading to progressive increase in pulmonary vascular resistance and pulmonary arterial pressure [1]. Pulmonary hypertension (PH) is defined as mean pulmonary artery pressure equal to or greater than 25 mm Hg at rest. PH is classified int...

conclusions this study indicated that the rv systolic strain and strain rate can be used to detect early rv systolic dysfunction in ssc patients without pulmonary hypertension. these parameters may be useful for the provision of a more adequate management of ssc patients. results in the ssc patients, the rv strain (- 19 ± 10 vs. - 25 ± 4 %; p = 0.004) and the systolic strain rate (- 1.3 ± 0.5 v...

Pulmonary angiogram of chronic pulmonary embolism. A 31-year-old man with progressive dyspnea and clinical evidence of pulmonary hypertension had right heart pressures as follows: right atrium, 12; right ventricle, = 98/18; pulmonary artery, 90/40; mean, 65; and pulmonary capillary wedge, 8 mm Hg. The contrast pulmonary angiogram shows occlusion of the descending left pulmonary artery and 80% s...