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

Clinical studies have suggested that patients with Down syndrome have precocious development of pulmonary hypertension, even in the absence of congenital heart disease. To examine the pathologic basis of this impression, we studied 82 patients with Down syndrome autopsied at The Johns Hopkins Hospital from 1913 to present. The patients ranged in age from 26 hours to 25 years; 41 (50%) were female. Atrioventricular canal defect was present in 40 patients, nine of whom also had pulmonary stenosis. Eight had isolated ventricular septal defect and seven had other malformations. Histologic sections of the lung from each patient were studied and the degree of hypertensive pulmonary vascular disease graded. Age and sex-matched controls without cardiovascular manifestations, all other autopsied patients with atrioventricular canal defect not associated with Down syndrome, and age-matched patients with ventricular septal defect or other malformations were similarly studied for pulmonary vascular changes. Comparison of the 27 patients with Down syndrome and no cardiovascular malformations with normal controls showed no differences, with one notable exception: an 8-month-old child with Down syndrome had severe HPVD secondary to idiopathic pulmonary hypertension. HPVD in patients with the various cardiovascular malformations was similar for those groups with Down syndrome and those without, when HPVD was considered as a function of the patients' age and the type of malformation. We conclude that, in general, patients with Down syndrome have no predisposition to develop severe or precocious hypertensive pulmonary vascular disease.
 ...
PMID:Hypertensive pulmonary vascular disease in Down syndrome. 15 39

Most cardiovascular problems in pregnant women arise from the complications of preexisting chronic conditions (e.g., rheumatic and congenital heart disease) and hypertensive vascular disease. Regular supervision of these patients is essential to detect incipient pulmonary congestion or disturbances of cardiac rhythm. Even if the pregnancy has been uncomplicated, hospital admission 1-4 weeks before the due date is recommended to ensure optimal conditions for labor. Vaginal delivery at term with adequate sedation and use of forceps to shorten the 2nd stage of labor is the perferred mode. Induction of labor may be indicated in hypertensive vascular disease or in cases where adjusting or discontinuing drug therapy calls for precise timing of delivery. Eisenmenger's disease and primary hypertension are potential medical indications for pregnancy termination. The distribution pattern of organic heart disease encountered in pregnant women has changed in the past 20 years, with a decrease in rheumatic and an increase in congenital heart disease. The incidence of chronic rheumatic heart disease in pregnant women fell from 3.5% of all deliveries at Newcastle General Hospital in 1942-51 to 1.1% in 1962-71. Acute pulmonary edema in mitral stenosis is currently a major risk during pregnancy. There is no optimal stage of pregnancy for valvotomy, nor evidence that this procedure induces miscarriage in the early weeks. Pregnancy has become less hazardous in severe forms of congenital heart disease as more patients with these disorders have undergone cardiac surgery prior to pregnancy. Pregnancy is not believed to have any effect on the longterm course of rheumatic heart disease. Patients with aortic stenosis, coarctation of the aorta, primary pulmonary hypertension, Fallot's tetralogy, Eisenmenger's syndrome, and surgically untreated cyanotic lesions require special attention during pregnancy. The outlook for women who become pregnant after an acute cardiac infarction episode depends on the functional state of the heart at the time of pregnancy and the presence or absence of angina pain. There has been a gradual decline in perinatal mortality, especially in cases complicated by rheumatic heart disease.
 ...
PMID:Cardiac disorders. 34 Jan 1

Heart-lung transplantation was, for many years, conceptualized as a possible treatment for patients with combined end-stage cardiac and pulmonary disease. As experience grew with heart transplantation, particularly in the 1970s, the difficulties of performing the orthotopic operation in patients with fixed pulmonary hypertension became apparent. This further impetus for combined heart-lung transplantation led to successful animal experiments in the late 1970s, and the first successful heart-lung transplant operation was performed in 1981. There has been significant evolution in the operative technique for the recipient operation, with emphasis on preservation of the phrenic, vagal, and recurrent laryngeal nerves and on meticulous hemostasis, with particular attention to the bronchial vessels of the posterior mediastinum. Donor procurement is of critical importance to the success of the operation, and criteria for donor selection have been well established. Lung preservation remained, for many years, a significant limitation, but current techniques involving the use of prostaglandin E1 have led to safe distant procurement with ischemic times up to 6 hours. The heart-lung transplant operation remains an effective modality for the treatment of patients with congenital heart disease, primary pulmonary hypertension, chronic obstructive pulmonary disease, and cystic fibrosis.
 ...
PMID:Technique of combined heart-lung transplantation. 148 24

A postulated association between primary pulmonary hypertension (PPH) and autoimmune diseases prompted this study. To see if autoantibodies and an association with the major histocompatibility locus (MHC) accompany the familial form of pulmonary hypertension (PHT), we determined human leukocyte antigen (HLA)-class I (A,B,C) and HLA-class II (DR,DQ) typing serologically, serum immunoglobulin (Ig) isotypes, and antinuclear (ANA)-autoantibodies in three families in which more than one member had PPH (FPPH) and in a fourth family with PPH and PHT associated with congenital heart disease. The three FPPH families had 15 PPH + members, eight (in whom the alleles were determined or could be inferred) typed for HLA-DRw52 and seven for HLA-DR3,DRw52,DQw2. These three families each had one PPH+,DR3+ member, with an immunoglobulin isotype deficiency, one with IgA and two with mild IgG. The fourth family differed in that both the patient and asymptomatic relatives had varying autoantibodies and different HLA associations. This family had an anti-centromere+ child with PHT and a congenital heart lesion, a mother who died of PPH, and asymptomatic ANA+ relatives (father anti-Sm+/anti-RNP+; autoantibodies unknown in fraternal twin sister and in maternal grandmother). HLA typing showed that the proband and her fraternal twin sister received DR5,DRw52,DQw3 from their father whereas the proband received DR4,DRw53,DQw3 from the mother and her sister received DR2,DQw1. In summary, three FPPH families had multiple PPH+ members who lacked autoantibodies and typed for HLA-DR3,DRw52,DQw2, whereas a fourth family with familial PHT had PHT+ and PHT- members with autoantibodies and different HLA associations.
 ...
PMID:Familial pulmonary hypertension: immunogenetic findings in four Caucasian kindreds. 155 3

We have performed heart-lung transplantation in 10 children for the preoperative diagnoses of primary pulmonary hypertension (4), complex congenital heart disease with pulmonary hypertension (4), pulmonary atresia (1), and cystic fibrosis (1). Ages ranged from 4 months to 18 years. There were 15 episodes of pulmonary rejection, with an occurrence rate of 1.67 episodes per patient. Pulmonary infections occurred frequently, with an occurrence rate of 3.3 episodes per patient. The actuarial survival rate at 1 and 2 years was 78% and 47%, respectively. Patient attrition between 1 and 2 years was attributable to the complications of obliterative bronchiolitis, which has effected 71% (5/7) of the long-term survivors. Four of the 5 surviving children have minimal physical limitation and are in functional class I. These data support continued investigation into heart-lung transplantation in children and set the stage for further program development into single-lung transplantation in children.
 ...
PMID:Heart-lung transplantation in infants, children, and adolescents. 205 4

The purpose of this study is to assess the right atrial and ventricular function by radionuclide ventriculography using continuous infusion of 81mKr in normal subjects and in patients with heart disease. 1) Reproducibility of RVEF measurement by continuous infusion of 81mKr was good (interobserver; gamma = 0.97, p less than 0.001, n = 20: intraobserver; gamma = 0.97, p less than 0.001, n = 20). This method had a excellent correlation with RVEF of 99mTc first-pass technique (gamma = 0.92, p less than 0.001, n = 20). 2) RVEF was measured in 10 normal volunteers, 76 patients with myocardial infarction (OMI), 20 patients with dilated cardiomyopathy (DCM), 5 patients with arrhythmogenic right ventricular dysplasia (ARVD) and 5 patients with primary pulmonary hypertension (PPH). In OMI and DCM, their RVEF was lower than that of normal volunteers and, in ARVD and PPH, lower than that of OMI and DCM. There was a significant inverse correlation between RVEF, mean pulmonary artery pressure and right ventricular end-diastolic volume index. 3) The effect of the location of right coronary artery (RCA) lesions on RVEF during exercise was also evaluated. Ten normal volunteers and 27 patients with OMI were studied at rest and during exercise. The patients with OMI were divided into two groups: those without proximal RCA lesions (non RCA group, n = 12) and those with proximal RCA lesions (RCA group, n = 15). Although there were no significant increases of RVEF during exercise in both group, the percent change in RVEF was less in RCA group than in non RCA group. These findings suggested that proximal RCA stenosis is a major determinant of exercise RVEF. 4) To assess the right atrial function, right atrial volume curve was measured in 10 normal volunteers, 32 patients with OMI and 4 patients with PPH. The curve was clearly divided into 4 phase; filling phase (312 +/- 40 msec), early ejection phase (276 +/- 53 msec), plateau an index of right atrial reservoir function, was 0.41 +/- 0.05 and Contractile Volume/Stroke Volume (Contr. V/SV), as an index of right atrial pump function, was 0.23 +/- 0.05 in normal volunteers. In OMI and PPH, atrial reservoir function decreased and atrial pump function increased. It was concluded that radionuclide ventriculography using continuous infusion of 81mKr was useful to assess the right heart function.
 ...
PMID:[Assessment of right atrial and ventricular function by radionuclide ventriculography using continuous infusion of 81mKr]. 206 Sep 2

Combined heart-lung transplantation is now being carried out in many centers around the world. Single-lung and double-lung transplantation are being done in only a few centers and are still considered to be experimental procedures. Combined heart-lung transplantation has been performed successfully in adults and in small children and offers a definite therapeutic alternative to such diseases as severe congenital heart disease with pulmonary hypertension, cystic fibrosis, and primary pulmonary hypertension. At present, the survival statistics for this procedure are similar to those for heart transplantation 15 years ago; it is hoped that, as with heart transplantation, survival rates will improve with better management and more reliable diagnosis of acute rejection, in order to prevent the insidious and relentless onset of obliterative bronchiolitis, which is now the most significant limiting factor to the success of long-term survival in heart-lung transplantation.
 ...
PMID:Pathology of the transplanted heart and lung. 240 6

Among 87 pregnancies complicated by heart disease, delivered during the decade 1977-86, 70 (81%) had a congenital heart malformation, 7 (8%) an acquired heart disease and 10 (11%) arrhythmias or conduction disturbances. The incidence was 0.3%. The corresponding data from a report from Rigshospitalet during the 1950s were: 42%, 49%, and 9%, respectively, and an incidence of 0.9%. Ventricular septal defect (VSD) and atrial septal defect (ASD) were the most frequent malformations. The women were classified according to the NYHA before, during and after the pregnancy. All women except 4 re-entered their original functional class. In 51 cases, ECG showed completely normal sinus rhythm, while in 36, various degrees of arrhythmia or conduction disturbance were found as well as left or right ventricular hypertrophy and/or strains. Nine infants had congenital defects, 4 of which were a heart malformation (4.6%). One infant died. Gestational duration, weight and perinatal mortality did not differ significantly from that of the general population. Two women died, one of primary pulmonary hypertension and one with a rupture of the thoracic aorta. Rheumatic heart disease is no longer a significant factor in relation to pregnancy in Denmark, but congenital heart disease is still of great importance, because more survive and reach the age of fertility. Today most women can be brought safely through pregnancy, but obstetric, cardiologic and anesthesiologic expertise is still mandatory for a successful course and outcome of pregnancies complicated by heart disease.
 ...
PMID:Maternal heart disease. A survey of a decade in a Danish university hospital. 258 39

A girl aged 2 years and 10 months repeatedly suffered viral (thrice) and bacterial (colitis, salmonellosis, pneumonia 6 times) infections. At an age of 2 years primary pulmonary hypertension was diagnosed. Diagnostic catheterization was performed to exclude a congenital heart disease. The death occurred during the catheterization. An increase of beta-lymphocytes and plasma cells, a reduction of the T-lymphocyte zone were detected in the immunocompetent system during postmortem histological examination. The pathology diagnosis: primary immunodeficiency with a predominant suppression of cell-mediated immunity; hyperplasia of the lymph nodes with their plasmacytization; pneumosclerosis of all lobes of both lungs (a syndrome of primary pulmonary hypertension clinically); hypertrophy of the muscles of both atria and right ventricle, dilation of the heart cavities; acute heart insufficiency.
 ...
PMID:[Primary immunodeficiency state in a child with the pulmonary hypertension syndrome]. 293 90

Under long-term oral treatment with nifedipine (N) 2 of 3 patients (2 children with VSD and Eisenmenger, 1 patient with Truncus I after corrective surgery) showed a remarkable improvement in exercise tolerance and reduction in the total pulmonary resistance. Therefore a single-dose testing with N was performed in 9 patients aged 2 to 20 years with primary pulmonary hypertension (n = 2), secondary pulmonary hypertension because of congenital heart disease with intracardiac shunts (n = 6) and after correction of Truncus Type I (n = 1). Before and after the injection of 0.5 mcg/kg of N into the main pulmonary artery, pulmonary artery pressure, cardiac output and systolic blood pressure were measured at 1 minute intervals during right heart catheterization. The maximal reaction occurred 3 to 4 minutes after the injection. The total pulmonary vascular resistance decreased significantly from 2684.2 +/- 1829.2 to 1300.2 +/- 1117.0 dyn.sec.cm-5.m2 (p less than 0.025), the cardiac index increased significantly from 3.1 +/- 1.65 to 5.73 +/- 1.95 l/min.m2 (p less than 0.01) concomitantly with a mild reduction in the mean pulmonary artery pressure. No significant change in heart rate and systolic blood pressure was seen; no side effects were observed. 4 of the 5 patients with the highest pulmonary vascular resistance showed the best positive reactions. Therefore these 9 patients may benefit from a long-term oral treatment with nifedipine.
 ...
PMID:[Hemodynamic studies of nifedipine (Adalat) in pulmonary hypertension in childhood]. 310 Dec 92


1 2 3 4 5 6 7 8 9 10 Next >>