Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Qualitative and quantitative studies were performed on pulmonary blood vessels in lung tissue obtained by biopsy, pneumonectomy, or autopsy from 58 patients in the Registry of Primary Pulmonary Hypertension sponsored by the Heart, Lung, and Blood Institute of the National Institutes of Health. In 49 patients (84%), the hypertensive vascular disease involved predominantly or exclusively muscular pulmonary arteries and arterioles. In each of these 49 patients, pulmonary artery medial hypertrophy was observed, and in 48 patients, it was also associated with intimal or luminal lesions. On the basis of the predominant histopathologic features, 25 of the 48 patients were classified as having pulmonary arteriopathy with plexiform lesions characterized by a combination of concentric laminar intimal fibrosis, eccentric intimal fibrosis, and plexiform lesions; in nine of these 25, recanalized thrombi were also present. Pulmonary arteriopathy with thrombotic lesions, defined by the presence of both eccentric intimal fibrosis and recanalized thrombi but without plexiform lesions, was observed in 19 patients. Intimal fibrosis, either concentric or eccentric, without plexiform or thrombotic lesions was found in four patients. Among the remaining nine patients in the Registry, pulmonary veno-occlusive disease was present in seven and chronic pulmonary venous hypertension in one. Pulmonary blood vessels were microscopically normal in a lung biopsy specimen from another patient. In general, patients with plexiform lesions and those with veno-occlusive disease had a much poorer prognosis than patients with thrombotic lesions. The present study shows the existence of several distinct histopathologic patterns of pulmonary vascular disease in individuals with primary pulmonary hypertension diagnosed by standardized clinical and laboratory criteria.
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PMID:Histopathology of primary pulmonary hypertension. A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry. 280 80

Pulmonary arteriopathy (PA) is the pathologic hallmark in human medicine of diffuse constrictive (medial and intimal remodeling) or multifocal complex (plexiform and dilatative lesions) arterial lesions, or both, that lead to irreversible obliteration of the arterial lumen. Clinically, PA leads to pulmonary arterial hypertension (PAH), of which idiopathic (IPAH) is one of the 5 subsets, and ultimately, to right-sided heart failure (RHF). Clinical and pathologic findings from 6 dogs with diagnosis of IPAH and PA were reviewed. These dogs were of various pure (5/6, 83%) and mixed (1/6, 17%) breeding, 5 months to 9 years (mean 5.2 years) old, and predominantly female (4/6, 67%) and reproductively intact (4/6, 67%). Doppler echocardiography (n = 5) indicated increased pulmonary arterial pressures during systole (70-135 mm Hg, mean 98 mm Hg) and diastole (35-80 mm Hg, mean 58 mm Hg). All 6 dogs had right ventricular pressure overload, right ventricular eccentric hypertrophy, and RHF. Histologic examination confirmed the clinical diagnosis of IPAH in all dogs, revealing PA characterized by 1 of the 4 main human histologic subsets: 1) isolated medial hypertrophy (1/6, 17%); 2) medial hypertrophy-intimal thickening without the plexiform lesion (1/6, 17%); 3) medial hypertrophy-intimal thickening concurrent with the plexiform lesion, which often was regionally clustered and situated near branching points of the respiratory artery, the poststenotic dilatation lesion, and vasculitis (4/6, 66%); and 4) isolated arteritis (1/6, 17%). Ancillary lesions similar to those in humans also complicated the PA (5/6, 83%). The complex lesions and ancillary exudative alveolitis seemed to be important indicators of severe, likely rapidly progressive and fatal, IPAH.
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PMID:Pulmonary arteriopathy and idiopathic pulmonary arterial hypertension in six dogs. 1684 93