Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Reduction of pulmonary vascular resistance by a high inspired oxygen concentration is a common, but not universal phenomenon in patients with pulmonary vascular disease of varying etiology that may determine their response to long-term domiciliary oxygen therapy. We therefore determined changes in PVR during oxygen therapy in two patient populations not previously studied: systemic sclerosis (n = 8, mean age +/- SEM, 44.5 +/- 5.4 years) and primary pulmonary hypertension (n = 7, mean age +/- SEM 38 +/- 7.8 years). All patients were hypoxemic (arterial oxygen tension, on air 9.5 +/- 1.2 kPa for SSc and 8.3 +/- 0.6 kPa for PPH, p greater than 0.05). Right atrial pressure, pulmonary artery pressure, pulmonary artery occlusion pressure, systemic arterial pressure, PaO2 and cardiac output by thermodilution were measured at three, 20-min intervals while inspiring air and again after inspiring 60 percent oxygen for 30 min. The PVR fell significantly with oxygen in patients with SSc from 797.6 +/- 179.2 to 610 +/- 151.6 dynes/s/cm-5 (p less than 0.01), and this fall correlated with baseline PAP and PaO2 prior to oxygen therapy (r = 0.86, p less than 0.025; r = 0.77, p less than 0.05, respectively). In patients with PPH, there was no significant fall in PVR with oxygen (from 969 +/- 80.2 to 851.9 +/- 91.2 dynes/s/cm-5, p greater than 0.05) and no predictor of a vasodilator response in individual patients. In SSc, hypoxic pulmonary vasoconstriction contributes more consistently to elevated PVR than in patients with PPH.
 ...
PMID:Hypoxic pulmonary vasoconstriction in systemic sclerosis and primary pulmonary hypertension. 189 17

Pulmonary wedge angiograms have been shown to reflect the severity of pulmonary vascular disease in congenital heart disease. Thirteen pulmonary wedge angiograms with a balloon occlusion catheter were performed in 11 adult patients (five normals and six with primary pulmonary hypertension [PPH]) and their features related to the resting pulmonary artery pressure (PAP). Individual cine frames from each study were selected and digitized with a computer-assisted operator-interactive program. By fitting densitometric profiles from the vessel segments, serial arterial cross-sectional diameters were calculated from mathematically derived points. There was a strong correlation between arterial taper (T, change in vessel caliber per unit axial length) and a power function of mean PAP with T = 0.304 X PAP-0.59, R = .91, P less than .001. These results demonstrate a correlation between an angiographically derived morphologic characteristic of the pulmonary vasculature (taper) and a hemodynamic parameter (PAP) in PPH. This offers a method to follow the course of the disease and the effects of drug therapy by assessing anatomic changes in the vessels.
 ...
PMID:Automated morphologic evaluation of pulmonary arteries in primary pulmonary hypertension. 380 57

Right ventricular cardiac function is altered by abnormalities affecting primarily the left-sided cardiac structures, the lungs, or the right-sided cardiac structures themselves. The most common cardiac causes for right ventricular dysfunction are chronic left ventricular ischemia and rheumatic mitral valvular disease. Pulmonary diseases that result in right ventricular dysfunction include pulmonary air-space disease, including emphysema, and pulmonary interstitial and parenchymal diseases, including idiopathic pulmonary fibrosis and cystic fibrosis. Chronic pulmonary vascular disease, including chronic thromboembolism and PPH have a significant effect on right ventricular performance. Common to all of these diseases is elevation of pulmonary vascular resistance with a commensurate increase in right ventricular pressure, resulting in right ventricular hypertrophy. The limited ability of right ventricular myocardium to function in the face of increased pulmonary resistance results in right ventricular dilatation, tricuspid regurgitation, and ultimately right ventricular failure. MR imaging provides direct, noninvasive visualization of the right ventricular chamber as well as the myocardium itself, allowing reliable demonstration of morphologic changes in the size and shape of the ventricle, thickness of the myocardium, and presence of abnormal infiltration by fat or edema. Furthermore, because MR imaging techniques do not depend upon geometric assumptions about the complex shape of the right ventricle, they may be used for accurate and reproducible quantitation of right ventricular volume and myocardial mass.
 ...
PMID:MR imaging of pulmonary hypertension and right ventricular dysfunction. 872 68

Atrial septostomy represents an additional, promising strategy in the treatment of severe PPH. Experience with this procedure still is limited; however, based on analyses of the worldwide experience, several general conclusions and recommendations can be made. 1. Atrial septostomy can be performed successfully in selected patients with advanced pulmonary vascular disease. 2. Patients with primary pulmonary hypertension who have undergone successful AS have shown: a significant clinical improvement beneficial and long-lasting hemodynamic effects at rest a trend toward improved survival 3. The procedure-related mortality of the collective experience is high (16%). Several recommendations can be made to minimize the risk: [figure: see text] Atrial septostomy should be attempted only in institutions with an established track record in the treatment of advanced pulmonary hypertension, where septostomy is performed with low morbidity. Atrial septostomy should not be performed in patients in whom death is impending or who have severe right ventricular failure and are on maximal cardiorespiratory support. An mRAP greater than 20 mm Hg, PVR index greater than 55 u/m2, and a predicted 1-year survival less than 40% are significant predictors of procedure-related death. Before cardiac catheterization, patients should have an acceptable baseline systemic oxygen saturation (> 90% in room air) and optimized cardiac function (adequate right heart filling pressure, additional inotropic support if necessary). During cardiac catheterization, the following are mandatory: Supplemental oxygen Mild sedation to prevent anxiety Careful monitoring of variables (left atrial pressure, SaO2, and mRAP) Step by step procedure After AS, it is important to optimize oxygen delivery. Transfusion of packed red blood cells or erythropoietin (before and following the procedure, if needed) may be necessary to increase oxygen content. 4. Because the disease process in PPH is unaffected by the procedure (late deaths), the long-term effects of an AS must be considered to be palliative. 5. Despite its risk, AS may represent a viable alternative for selected patients with severe PPH. Indications for the procedure may include: Recurrent syncope or right ventricular failure, despite maximal medical therapy, including oral calcium-channel blockers or continuous intravenous prostacyclin (Fig. 11) As a bridge to transplantation When no other option exists.
 ...
PMID:Atrial septostomy for pulmonary hypertension. 1159 Aug 48

The heart is a muscular mechanical pump with an ability to generate both flow [cardiac output] and pressure in the systemic circulation as well in the pulmonary vascular bed. The product of flow output and systemic arterial pressure is the rate of useful work done, or cardiac power output [CP], therefore for the right ventricle and the pulmonary arterial vascular bed it will be: the product of flow output and mean pulmonary arterial pressure will be the rate of useful work done, or cardiac pulmonary power output [CPP]. Cardiac pulmonary pumping reserve capability can be defined as the maximal cardiac pulmonary power output [CPP] achieved by the right heart during maximal stimulation. Therefore, CPP reserve is the increase in power output as the cardiac performance is increased from the resting to the maximally stimulated state. Recently, several studies have shown that CP is a direct indicator of overall cardiac function, as well is an indicator of exercise capacity and a powerful predictor of prognosis for patients with severe heart failure and ischemic cardiogenic shock. On this basis: 1. we decided to investigate the possible previously mentioned roles of the CP and CPP in patients with different degrees of pulmonary arterial hypertension [n = 137], but mainly in those with primary pulmonary arterial hypertension [PPH]. PPH patients [n = 50], as well as those with Eisenmenger's syndrome [n = 10] were found to have most abnormal resting CPP Indexes [I], [0.431 +/- 0.171, 0.607 +/- 0.124 watts/m2, respectively]. During exercise in PPH patients [n = 14], both CPI and CPPI reserves although they increase, were also found to be significantly diminished [CPI: from 0.546 +/- 170 to 1,116 +/- 0.275 watts/m2, p < 0.05], [CPPI: from 0.373 +/- 0.156 to 0.837 +/- 0.226 watts/m2, p < 0.05]. 2. We also found, significant differences in resting CPPI between PPH responders and no responders patients to hydralazine [0.273 +/- 0.04, 0.507 +/- 0.142 watts/m2, respectively, p < 0.01]. During exercise and under the influence of hydralazine, CPPI "reserve" was significantly diminished in those patients considered as not responders [0.507 +/- 0.142 to 0.723 +/- 0.232 watts/m2, p = ns] when compared with responders to the vasodilator therapy [0.273 +/- 0.04 to 0.903 +/- 0.057 watts/m2, p < 0.01]. On the basis of these initial findings on CPPI in PPH patients, we think that the values of this parameter should be investigated as an index for classifying the severity of this arterial vascular disease, for selecting patients as responders or not to vasodilator therapy, and in the future, to explore the possibility of is usefulness as a prognostic predictor index of mortality in a large population of patients with PPH.
 ...
PMID:[The possible clinical role of the cardiac pulmonary power in patients with primary pulmonary hypertension. Initial observations]. 1685 10