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)

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

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 first case of Q fever endocarditis that has been diagnosed in Mexico is presented. A 10-year-old girl with discrete subaortic stenosis (SAS) and patent ductus arteriosus (PDA) was seen in December of 1996 with fever, hepatomegaly and splenomegaly. She presented also anemia, leukopenia, hypergammaglobulinemia, positive rheumatoid factor, cryoglobulinemia, antinuclear and anticytoplasmic antibodies (anti-RNA-proteins and anti-DNA). An aortic valve vegetation was seen by echocardiogram. Blood-cultures were negative. Antibody test for Coxiella burnetii was positive. Treatment with doxicyclin was initiated as soon the diagnosis was done. PDA was closed, SAS was liberated and two aortic vegetations were resected. Endocarditis in Q fever occurs when there is predisposing heart disease and/or immunodeficiency. Effective therapy has not yet been established. The diagnosis of Q fever endocarditis is difficult; it should be considered, in case of clinical suspicion of endocarditis with negative blood-cultures.
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PMID:[Coxiella burnetii endocarditis. A report of the first case diagnosed in Mexico]. 981 Mar 69

We report an unusual case in which infectious endocarditis presented systemic vasculitis and glomerulonephritis as the initial manifestation of the disease. The patient was a 16-year-old girl with congenital cyanotic heart disease who presented with skin purpura, proteinuria, and hematuria. She had hypergammaglobulinemia, cryoglobulinemia, and positive circulating immune complexes. Renal biopsy revealed crescentic glomerulonephritis. Her serum C3 level, which was initially normal, became decreased, and prednisolone and azathioprine were administered with a tentative diagnosis of systemic lupus erythematosus (SLE). Soon after, she developed fever and renal failure. Blood culture grew Streptococcus pyogenes, and the diagnosis of infectious endocarditis was made. Eight cases of systemic vasculitis and glomerulonephritis associated with infectious endocarditis have been described in the literature. Infectious endocarditis should be included in the differential diagnosis of systemic vasculitis and glomerulonephritis.
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PMID:Vasculitis associated with septicemia: case report and review of the literature. 1179 7