Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In primary pulmonary hypertension of recent clinical onset, pulmonary endothelial cells show injury. To characterize this phenomenon, we measured plasma von Willebrand factor (vWF) by immunologic and ristocetin cofactor assays in 6 patients with primary pulmonary hypertension, 17 patients with secondary pulmonary artery hypertension associated with congenital heart disease or cystic fibrosis, and 13 patients with congenital heart disease and normal pulmonary artery pressure. In selected cases, we also determined the vWF multimer pattern. In all 6 cases of primary pulmonary hypertension, the ristocetin cofactor activity was increased relative to the vWF antigen (vWF:Ag) concentration (a ratio of 2.55 +/- 0.36; normal range, 0.8 to 1.4); 4 of the 6 also had a similar and abnormal vWF multimer pattern--an increased proportion of the fastest moving bands. In the other 2, the multimer pattern was normal. Of the other 30 patients, a mild increase in ristocetin cofactor/vWF:Ag was seen in only 2 with secondary pulmonary hypertension and 1 with normal pulmonary artery pressure: these also had an abnormal vWF multimer pattern that was different from that observed in patients with primary pulmonary hypertension. The vWF abnormalities we describe in primary pulmonary hypertension offer a marker of the disease and could be helpful in understanding its pathogenesis.
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PMID:von Willebrand factor abnormalities in primary pulmonary hypertension. 310 58

Fourteen patients, 2 to 20 years old were investigated. Two had primary pulmonary hypertension, 11 had congenital heart disease and post-tricuspid shunts, and 1, a 20-year-old patient, was investigated after he had undergone surgical correction of truncus arteriosus I. Pulmonary arterial pressure, pulmonary flow index, peripheral systolic blood pressure and heart rate were measured before, and several times after intrapulmonary injection into the pulmonary artery of 0.5 microgram nifedipine/kg. Six patients were given an additional dose of 1 microgram nifedipine per kilogram into the pulmonary artery and hemodynamic measurements were repeated. In eight children, receiving 100% oxygen via a breathing mask, nifedipine effects were compared with oxygen effects. After 10 minutes under oxygen, the same hemodynamics were determined as after nifedipine. In addition, in four of these children aortic pressure and arterial oxygen saturation were also measured. Maximal effects occurred within 4 minutes. 0.5 micrograms nifedipine per kilogram caused a slight reduction in mean pulmonary arterial pressure (p less than 0.05), as well as increase in pulmonary flow index (p less than 0.005). However, no significant change in heart rate or in systolic blood pressure was observed. 1 microgram nifedipine per kilogram IP had almost the same effects. No adverse side effects occurred, besides mild headaches in one child. A comparison of nifedipine injected into the pulmonary artery with oxygen breathing in congenital heart disease combined with pulmonary hypertension, is reported for the first time. Nifedipine had a more pronounced and beneficial effect with a selective action on the pulmonary vascular bed.
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PMID:Hemodynamic effects of nifedipine and oxygen in children with pulmonary hypertension. 315 41

The outcome of 240 pregnancies in 202 patients with cardiac disease, who delivered between Jan. 1982 and Dec. 1987 at the National Cardiovascular Center was analyzed. Ninety-six patients had congenital heart disease. Forty-two had acquired valvular heart disease, 83 had cardiac arrhythmia, 7 had aortitis syndrome, 7 had cardiomyopathy and 2 had secondary angina pectoris. There was one case of each of the following cardiac diseases: Primary pulmonary hypertension, Marfan's syndrome and pericarditis. Nineteen of the above lapsed into congestive heart failure and there were two cases of maternal death. One was a case of primary pulmonary hypertension and the other had had an aortic valve replaced with a Bjork-Shiley prosthesis because of rheumatic valve disease. Another 16 mothers had artificial preterm delivery at a gestational age of 30 weeks to 36 weeks to reduce the risk of congestive heart failure. There were 5 cases of IUFD, three of which were attributed to maternal cardiac disease and another to fetal failure. Neonatal congenital heart disease was found in 8 cases. The percentage of IUGR, excepting twin pregnancies, was 14.8%.
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PMID:[The course of pregnancy in patients with cardiac disease]. 317 Dec 68

Right ventricular anterior wall thickness measured by M-mode echocardiography and right ventricular systolic pressure obtained by right heart catheterization were correlated in 62 patients with chronic right ventricular overload including congenital heart disease and primary pulmonary hypertension. The patients were divided into two groups; one, with right ventricular systolic pressures of 39 mmHg or less; the other, 40 mmHg or more. The following results were obtained. 1. The correlation coefficient for right ventricular anterior wall thickness and right ventricular systolic pressure was r = 0.90 (p less than 0.001), and the regression equation was y = 13.2x-1.3. 2. Right ventricular end-diastolic dimension increased significantly in both groups, but no statistically significant differences were detected between the two. Right ventricular anterior wall thickness increased significantly in the group with higher right ventricular pressures (7.1 +/- 0.5 mm vs 3.1 +/- 0.5 mm). 3. When right ventricular anterior wall thickness was more than 4.0 mm, pulmonary hypertension was detected, with a sensitivity of 97.5% and a specificity of 90.9%. In conclusion, measurements of right ventricular anterior wall thickness by M-mode echocardiography via the anterior chest wall proved to be potentially useful in predicting right ventricular systolic pressures in patients with chronic right ventricular overloads.
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PMID:[Usefulness of measuring right ventricular anterior wall thickness in patients with right ventricular overload using M-mode echocardiography]. 350 8

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.
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PMID:Automated morphologic evaluation of pulmonary arteries in primary pulmonary hypertension. 380 57

In children with congenital heart disease serial noninvasive assessment of the pulmonary vascular bed is desirable in order to determine the appropriate timing of cardiac catheterization and corrective surgery. To assess the value of pulmonary Doppler echocardiography for the estimation of pulmonary arterial pressure we correlated the rightsided systolic time intervals (preejection period, acceleration period, ejection period) derived from pulmonary artery Doppler traces with catheterization data (systolic, mean and diastolic pulmonary arterial pressure, total and vascular pulmonary resistances). 62 children aged from 1 month to 15 years suffering from congenital heart disease (n = 52), from rheumatic heart disease (n = 4), from cardiomyopathy (n = 5) or from primary pulmonary hypertension (n = 1) were investigated. The major finding was a highly significant correlation between the acceleration period and the systolic pulmonary arterial pressure (r = 0.79; p less than or equal to 0.0001). However the acceleration period could not be used for prediction of pulmonary arterial pressure in children with poor myocardial contractility.
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PMID:[Doppler echocardiographic evaluation of pressure in the pulmonary artery in children with congenital heart defects]. 381 67

A thorough cardiovascular evaluation is necessary to detect potentially lethal abnormalities that may predispose children and adolescents to sports-related sudden death. Hypertrophic cardiomyopathy and anomalous origin of the left coronary artery from the right sinus of Valsalva are the two most common cardiac abnormalities associated with sudden death during childhood. Other conditions potentially associated with sudden death are Marfan's syndrome, aortic valve stenosis, primary pulmonary hypertension, and arrhythmias. A detailed medical evaluation is indicated for subjects who have a history of nonvasodepressor syncope, exercise-related syncope or presyncope, angina pectoris, or known congenital or acquired heart disease. In addition, a family history of premature sudden unexpected syncope or death, hypertrophic cardiomyopathy, premature atherosclerotic heart disease, severe arrhythmias, or Marfan's syndrome or premature aortic aneurysms is an indication for a thorough cardiovascular evaluation before participation in sports.
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PMID:Cardiovascular evaluation of the child and adolescent before participation in sports. 406 61

Combined heart and lung transplantation was carried out in ten patients at Stanford University Medical Center between March, 1981, and December, 1982. All patients had end-stage pulmonary hypertension. 7 of them had Eisenmenger's syndrome and 3 primary pulmonary hypertension. 3 patients died within a month of operation, but the remaining recipients are well 2 months to 2 years after transplantation. The hospital stay of the survivors ranged from 38 to 85 days. All survivors have returned to normal activity. The results of heart and lung transplantation have thus been considerably superior to those reported previously for lung transplantation. It is suggested that cardiopulmonary replacement is suitable treatment for end-stage pulmonary hypertension with or without associated congenital heart disease and that its application to other forms of advanced pulmonary failure may be warranted.
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PMID:Combined heart and lung transplantation. 613 56

Six patients received heart-lung transplants between March, 1981, and January, 1982. There were four women and two men between 26 and 45 years of age, three with primary pulmonary hypertension and three with congenital heart disease and pulmonary hypertension (Eisenmenger's syndrome). Immunosuppression was primarily with cyclosporin-A, with additional corticosteroid, azathioprine, and rabbit antihuman thymocyte globulin. Six episodes of allograft rejection in four patients (10, 11, 21, 24, 53, and 86 days after transplantation) were detected by means of transvenous endomyocardial biopsy. All patients experienced pulmonary edema early after transplantation (reimplantation response), and two patients required mechanical ventilatory support for allograft rejection at 10 and 11 days. Treatment of rejection consisted of intravenous methylprednisolone (four episodes) or augmented oral prednisone (two episodes), with resolution. No episode thought to be pulmonary rejection has occurred in the absence of cardiac findings. Four patients are alive from 6 to 15 months after transplantation and are functionally normal. Early experience with heart-lung transplantation suggests (1) that allograft rejection can be detected by cardiac findings and successfully treated by augmented corticosteroids, (2) that lung rejection does not occur in the absence of cardiac findings, (3) that the frequency and severity of rejection episodes are not greater than with standard cardiac transplantation, and (4) that the frequency of rejection episodes is highest within the first 60 days after transplantation.
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PMID:Diagnosis and treatment of allograft rejection in heart-lung transplant recipients. 640 22

This report is a brief summary on current events related to lung and heart-lung transplantation. Eleven patients have undergone transplantation of the heart and both lungs at Stanford University. The ages ranged from 22-45, the average age being about 36 years, and included were four females and seven males. The diagnosis was primary pulmonary hypertension in three and Eisenmenger syndrome--congenital heart disease with pulmonary hypertension--in eight. Eight patients are living and well, two to more than 24 months after transplantation of the heart and both lungs. All these patients were discharged and are fully rehabilitated, which is an important consideration. There have been three operative deaths, one was secondary to two previous operations that made our operation much too long, another was secondary to the use of intravenous cyclosporine, and the third was related to the poor maintenance of the donor lung. Three of the eleven patients were catheterized following the transplant from six months to a year after transplantation, and the pulmonary artery pressure and pulmonary vascular resistance were absolutely normal in all three of these individuals. Of course the plan is to go ahead with further catheter studies at yearly intervals in all of the patients. The last patient underwent transplantation in January, 1983. I think no matter how effective or how ingenious the medical staff is with artificial organs, it will be a long time before these early results of transplantation of the heart and both lungs can be matched by any types of artificial organ implants.
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PMID:Lung and heart-lung transplantation. 643 26


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