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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In order to test the hypothesis that HLA-linked developmental determinants are involved in the pathogenesis of congenital heart disease, we performed tissue typing on 10 families in which two or more siblings had cardiac malformations. No association between the occurrence of congenital heart disease and the HLA complex was found.
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PMID:Studies of the HLA complex in families of children with congenital heart disease. 8 Aug 36

Over the last few years the study of idiopathic haemochromatosis has not brought to light any basic change in the overall pattern of organic and metabolic damage produced by the disease and comprising altered skin pigmentation, liver disease, diabete mellitus, heart disease, endocrine dysfunction, bone and joint disease. Nevertheless, certain facets of the clinical picture have been described and progress has been made in understanding the signs of the disease. Although the desferrioxamine test is no without merit, especially if performed after vitamin C administration, for measuring the extent of iron overload, two methods seem better equipped: serum ferritin radioimmunoassay and measurement of iron concentration in a liver biopsy specimen. The HLA antigen A3 and, more especially, haplotype A3, B14, are markers for the genetic basis of the disease. Repeated phlebotomy therapy generally brings about symptomatic improvement and a significant increase in survival.
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PMID:[Idiopathic haemochromatosis. I. Clinical, biological and therapeutic aspects (author's transl)]. 37 16

Familial prevalence of some congenital cardiopathies leads to the conclusion that genetic factors might be involved. The case histories are presented of three families with atrial septal defect, Type II (ASD II) in which autosomal dominant inheritance was assumed on account of the pedigree analysis. Each family member was examined serologically, morphologically and morphometrically. One family was also tested for cytogenetic abnormalities. It is highly probable that the gene responsible for the defect is linked to the HLA system (Lod score = + 3.612) and is, therefore, located on the short arm of chromosome 6. The morphological examinations demonstrated uniformity of individual ear traits in related patients; moreover, the palmar dermatoglyphics showed a tendency to shortening of main line C, to ulnar and distal shifting of the carpal triradius and to an increase in hypothenar patterns. In addition a study was carried out of patients with apparently sporadic cardiopathy. A similar trend as to palmar configuration was observed. An attempt was made to connect known factors causing malformations with the results of this investigations.
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PMID:[Mechanisms common to the development of malformation in congenital and sporadic forms of atrial septal defect (type II) (author's transl)]. 45 15

Heart disease is the main cause of death in patients with Takayasu arteritis. It has been reported that this disease is closely related to the presence of HLA Bw52 antigen. To assess the correlation between this antigen and left ventricular involvement, we studied 40 patients with Takayasu arteritis, 21 with and 19 without Bw52, using Tl-201 stress myocardial scintigraphy and echocardiography. Those with Bw52 had a significantly higher incidence of abnormal electrocardiographic findings (67% vs 26%; P < 0.05) and of aortic regurgitation (52% vs 11%; P < 0.05). The echocardiographically determined interventricular septal wall thickness plus left ventricular posterior wall thickness (25 +/- 8 vs 17 +/- 3 mm; P < 0.01) and the left ventricular mass (257 +/- 132 vs 142 +/- 51 g; P < 0.01) were significantly increased in the patients with Bw52. Scintigraphically determined perfusion abnormalities were significantly more frequent in those with Bw52 (76% vs 32%; P < 0.05). These observations indicate that patients with Takayasu arteritis and Bw52 antigen have a more severe left ventricular involvement than the patients without that antigen. The left ventricular impairment may account for the poor prognosis of Takayasu patients with Bw52.
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PMID:Left ventricular dysfunction and HLA Bw52 antigen in Takayasu arteritis. 136 Sep 56

Research of the relevant international literature on HLA studies in patients with hypertrophic cardiomyopathy yielded controversial results. There are no studies, conducted in sufficiently large groups of patients, that would consider the different functional and morphological forms of the disease. Therefore, the authors carried out detailed typing of 60 Class I and II antigens in 117 patients known to suffer from hypertrophic cardiomyopathy. Values of the relative risk and chi-square test showed a number of possible associations. However, after correction for the number of antigens tested, only HLA-B21 was shown to have a significantly high frequency (in patients with the obstructive form and in those with advanced myocardial hypertrophy, defined as a wall thickness greater than 30 mm). An association with this antigen has previously been demonstrated in a number of cases of ischaemic heart disease, myocardial infarction of young people, and in hypertensive subjects. HLA typing may be helpful in recognizing forms which are not fully typical. In Czechoslovakia, HLA-B21 carriers are at increased risk of developing a serious heart disease manifesting already in young age.
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PMID:An immunogenetic study in hypertrophic cardiomyopathy. 181 Jul 2

We studied the distribution of HLA, B and C antigens in 73 Chagasic subjects with and without heart disease. Both groups were matched for age, sex, birth place and history of family residence. Thirty two subjects without evidence of Chagasic infection, with and without heart disease and matched for the same variables were also studied. Compared to all other groups, a significant increase in the level of the B40 Cw3 antigen combination was found in Chagasic subjects without evidence of heart disease.
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PMID:[HLA antigens in Chagas cardiomyopathy: new evidence based on a case-control study]. 184 66

It has been hypothesized that dilated cardiomyopathy (DCM) is of dysimmune origin. Conventional immunological studies have provided no evidence that a primary disregulation of immune mechanisms is involved. In the present study, the possibility of an individual predisposition to DCM resting on a preferential distribution of HLA system antigens has been investigated. Typing of the HLA system antigens A and B was performed in a group of 38 DCM patients who were heavy drinkers. The results were compared with those obtained in: (a) 57 alcoholic patients without cardiopathy, and (b) a population of 306 healthy subjects. All subjects were caucasians. Compared with alcoholic patients without cardiac disease, DCM patients had a prevalence of B8 allele. The relative risk of developing DCM was 2.83 in the presence of the B8 antigen. This result suggests a genetic predisposition to DCM: the B8 allele, prevalent among our patients, is associated with the phenotype of numerous autoimmune diseases. This study therefore supports the theory that DCM is of dysimmune origin, but this must be confirmed by further investigations conducted on a larger number of cases.
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PMID:[Antigens A and B of the HLA system in dilated cardiomyopathies related to alcohol]. 244 17

The correlation analysis of ratios between six polymorphic genetic systems (ABO, MNSs, Rh, Hp, Gm, HLA) and mortality from ishemic heart disease, brain vascular lesions, and hypertensive disease in 17 European populations has been made. A statistically significant correlation has been established between the populational frequency of most of the 50 phenotypes and genes under study, and mortality. The qualitative structure of correlations and their quantitative expression depend on the cause of death, age and sex. The possible mechanisms of relationship between the genetic populational differences and mortality from cardiovascular diseases are discussed.
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PMID:[Balanced hereditary polymorphism and the mortality from cardiovascular diseases in the populations of 17 countries of Europe. I. A correlation analysis]. 653 62

Heart disease characterized by endomyocardial fibrosis is one of the major causes of morbidity and mortality in the idiopathic hypereosinophilic syndrome. From our series of 50 patients with idiopathic hypereosinophilia, we defined the noncardiovascular characteristics that distinguish patients at risk of developing endomyocardial fibrosis from those who remain free of heart disease. These groups did not differ with respect to the extent of eosinophilia or the duration of disease. Patients with clinically overt heart disease were more likely (p less than 0.05) to be male and HLA-Bw44 positive and have splenomegaly, thrombocytopenia, elevated serum levels of vitamin B12, and hypogranular or vacuolated eosinophils and abnormal early myeloid precursors in the peripheral blood. These idiopathic hypereosinophilic patients with heart disease were also more likely to have fibrosis and decreased megakaryocytes in the bone marrow. In contrast, those who remained free of heart disease tended to be female and have angioedema, hypergammaglobulinemia, elevated serum levels of immunoglobulin E (IgE), and circulating immune complexes. Therefore, in the idiopathic hypereosinophilic syndrome, male patients with a myeloproliferative type disorder and the HLA-Bw44 haplotype were at a much increased risk for the development of endomyocardial fibrosis. However, those patients with a hypersensitivity-like illness and angioedema who were female did not develop heart disease. Appreciation of this relative degree of risk for the major complication of the idiopathic hypereosinophilic syndrome should prove useful in the early identification and appropriate treatment of patients in whom endomyocardial fibrosis might develop.
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PMID:Noncardiovascular findings associated with heart disease in the idiopathic hypereosinophilic syndrome. 686 80

The finding at autopsy of typical pathologic features of hypertrophic heart disease (idiopathic hypertrophic subaortic stenosis, IHSS) and mitral valve prolapse (MVP) in a single patient prompted study of a number of close relatives of this patient. Several additional cases of IHSS or MVP were found. The HLA typing of this kindred revealed that four out of seven members tested had the Bw 35 antigen. Although the association might be due to chance, this kindred, together with prior reports of similar bizarre myocardial cellular disarray in HISS and MVP, suggest the hypothesis that in some instances, IHSS and MVP may represent a continuum of hereditary cardiac disorders.
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PMID:Coexisting hypertrophic heart disease and mitral valve prolapse. A continuum of hereditary cardiac disease? 719 56


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