Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a 32-year old female patient with primary antiphospholipid syndrome (PAPS) and several thromboembolic events despite stable doses of oral anticoagulation, good patient compliance and maintained INR values of >3. Over the preceding 3 years the patient had presented a wide spectrum of manifestations of APS, including recurrent venous and arterial thromboses, cardiac, gynecological (HELLP syndrome), neurological involvements, livedo reticularis, a mild thrombocytopenia and the most feared manifestation of the catastrophic antiphospholipid syndrome (CAPS). Life-threatening bilateral subdural bleeding occurred while she was anticoagulated. The clinical features appeared to be refractory to oral anticoagulation with phenprocoumon. They were life threatening on each occasion and she developed repetitive episodes of organ damage with cardiac insufficiency (NYHA III), pulmonary hypertension and other residual defects. Even during heparinization recurrent thromboembolism supervened as well as livedo reticularis of the extremities. Lupus anticoagulants (LAC), anticardiolipin (aCL) antibodies and anti-beta(2)-glycoprotein-1 (beta(2)GPI) titers were all markedly elevated. This case report shows that recurrent episodes of thrombosis can occur despite seemingly adequate anticoagulation in patients with CAPS.
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PMID:Recurrent life-threatening thromboembolism and catastrophic antiphospholipid syndrome in a patient despite sufficient oral anticoagulation. 1516 58

Cardiac involvement is a not uncommon complication in patients with antiphospholipid syndrome (APS). Herein, the case is reported of cardiac failure in a female patient with Libman-Sacks endocarditis and with primary APS diagnosed eight years previously. Aggressive anticoagulation therapy and medical treatment for the cardiac failure over a 12-month period resulted in a partial regression of the severe mitral regurgitation. Close clinical and echocardiographic surveillance during the follow up of patients with APS and heart valve disease is mandatory. Optimal treatment, including adequate aggressive anticoagulation therapy and specific treatment for heart failure, may play a pivotal role in reducing the severity of valve dysfunction in these patients.
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PMID:Libman-sacks endocarditis and primary antiphospholipid syndrome. 1624 13

Antiphospholipid syndrome is an autoimmune disease characterized by recurrent thrombosis and the presence of antiphospholipid antibodies. Clinical presentations are dependent on the affected vessels and organs. The most common presentation of antiphospholipid syndrome is arterial or venous thrombosis. An unusual presentation of the disease is characterized by microvascular thrombosis with multiorgan involvement, which is termed catastrophic antiphospholipid syndrome. The diagnosis of catastrophic antiphospholipid syndrome can be difficult because of the heterogeneity of the different clinical forms. Clinical manifestations of catastrophic antiphospholipid syndrome are complex with multiple organ involvement, resulting in renal insufficiency, heart failure, acute respiratory distress syndrome, and liver involvement. Early diagnosis and aggressive therapies are essential in this condition because of the extremely high mortality rate. Herein, the case of a 14-year-old girl with catastrophic antiphospholipid syndrome that was previously misdiagnosed as a vasculitis related to parvovirus B19 infection is presented.
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PMID:A case of catastrophic antiphospholipid syndrome in an adolescent girl with parvovirus B19 infection. 1856 54

The antiphospholipid syndrome is characterized by arterial and venous thrombosis and is associated with the presence of circulating antiphospholipid antibodies. Arterial thrombosis can result in myocardial infarction, which may potentially lead to end-stage heart failure. Here we report our anticoagulation protocol for patients with antiphospholipid syndrome that undergo axial-flow left ventricle assist devices (HeartMate II; Thoratec, Pleasanton, CA) implantation.
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PMID:Implantation of a left ventricular assist device in a patient with primary antiphospholipid syndrome. 1864 Mar 48

Nonbacterial thrombotic (noninfectious, pseudoinfectious--PIE) endocarditis is characterized by precipitation of thrombus, not containing bacteria, on the valve cusps. Mitral and aortal valves are affected most frequently. Vegetations, as a rule, do not exceed 6-7 mm and have a high inclination to embolism. Hypercoagulation plays a leading role in PIE pathogenesis. The most frequent acquired causes of sterile vegetation forming are malignant tumors and rheumatic diseases (especially systemic lupus erythematosus--SLE and antiphospholipid syndrome--APS). Valve pathology is most frequent lesion of heart in APS patients. It is supposed, that antibodies to phospholipids (aPL) have a special importance in valve lesion pathogenesis at APS, besides, changes in valve apparatus at SLE are associated exactly with aPL. Main problems of PIE patients are recurrent thromboembolism, development of valve dysfunction with clinical signs of heart failure (4-6% cases), difficulties in differential diagnostics: PIE is hard to diagnose if basic disease is accompanied by fever (diffuse diseases of connective tissue etc.). Transesophageal echocardiography is a leading method in PIE diagnostics. The main therapeutic option in PIE treatment is anticoagulant therapy: nonfractional or subcutaneous heparin in presence of systemic or pulmonary embolism, in patients with disseminated malignant tumors--complete doses of nonfractional heparin.
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PMID:[Pseudoinfectious endocarditis]. 1872 Jul 5

A case of a 29-year-old woman 18 days after delivery with catastrophic antiphospholipid syndrome secondary (CAPS) due to undiagnosed systemic lupus erythematosus, leading to cardiogenic shock is reported. Laboratory evaluation revealed increased anticardiolipin antibodies, lupus anticoagulant, antinuclear antibody and thrombocytopenia. Left ventricular ejection fraction was 20%, neurologic deficit and acute renal failure were also present. Cardiac involvement is common in CAPS, but cardiomyopathy due to microvascular thrombosis is rare. CAPS should be considered as a cause of acute heart failure in a women with systemic lupus erythematosus. In the presented case early therapy with anticoagulants, steroids, immunoglobulins and plasmaferesis was beneficial.
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PMID:[Catastrophic antiphospholipid syndrome complicated by cardiogenic shock - a case report]. 1965

A woman with isolated ACTH deficiency and empty sella in a background of primary antiphospholipid syndrome (APS) is described. A 24-year-old woman was admitted for premature delivery at 30 weeks of gestation and was found to have severe pulmonary hypertension and right sided heart failure. A few hours after delivery, without excessive blood loss, she suddenly lost consciousness, and was found to be hypotensive and hypoglycemic. The findings on hormonal evaluation were consistent with isolated secondary adrenal insufficiency as both ACTH and cortisol levels were very low. Magnetic resonance imaging of the pituitary fossa showed an empty sella. Her severe cor pulmonale was found to be due to primary APS. The patient was given replacement of glucocorticoid and treated with frusemide, spironolactone, warfarin, sildenafil and inhaled iloprost for her pulmonary hypertension, after which her clinical status improved substantially. Follow-up tests of the hypothalamic-pituitary axis found normal serum LH, FSH, TSH, FT4 and prolactin secretion; serum ACTH and plasma cortisol levels remained low.
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PMID:Secondary adrenal insufficiency and primary antiphospholipid syndrome. 1991 97

Pure red cell aplasia (PRCA) is a disease with important relationships to autoimmune mechanisms. Although some autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus, have been associated with PRCA, until this point no studies have described the association between PRCA and primary antiphospholipid syndrome (APS). This is the first case report of PRCA associated with primary APS in a 39-year-old man with acute heart failure secondary to an anaemic condition that was diagnosed as pure red cell aplasia. The patient was later diagnosed with retinal artery and vein thromboses and bilateral deep venous thromboses of the femoral and popliteal veins. The most common causes of PRCA and other thrombophilias were ruled out in this investigation through complementary tests. This association with APS adds a new possibility to the study of PRCA pathophysiology.
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PMID:Pure red cell aplasia and primary antiphospholipid syndrome: a unique association. 2023 20

Various autoimmune rheumatic diseases (ARDs), including rheumatoid arthritis, spondyloarthritis, vasculitis and systemic lupus erythematosus, are associated with premature atherosclerosis. However, premature atherosclerosis has not been uniformly observed in systemic sclerosis. Furthermore, although experimental models of atherosclerosis support the role of antiphospholipid antibodies in atherosclerosis, there is no clear evidence of premature atherosclerosis in antiphospholipid syndrome (APA). Ischemic events in APA are more likely to be caused by pro-thrombotic state than by enhanced atherosclerosis. Cardiovascular disease (CVD) in ARDs is caused by traditional and non-traditional risk factors. Besides other factors, inflammation and immunologic abnormalities, the quantity and quality of lipoproteins, hypertension, insulin resistance/hyperglycemia, obesity and underweight, presence of platelets bearing complement protein C4d, reduced number and function of endothelial progenitor cells, apoptosis of endothelial cells, epigenetic mechanisms, renal disease, periodontal disease, depression, hyperuricemia, hypothyroidism, sleep apnea and vitamin D deficiency may contribute to the premature CVD. Although most research has focused on systemic inflammation, vascular inflammation may play a crucial role in the premature CVD in ARDs. It may be involved in the development and destabilization of both atherosclerotic lesions and of aortic aneurysms (a known complication of ARDs). Inflammation in subintimal vascular and perivascular layers appears to frequently occur in CVD, with a higher frequency in ARD than in non-ARD patients. It is possible that this inflammation is caused by infections and/or autoimmunity, which might have consequences for treatment. Importantly, drugs targeting immunologic factors participating in the subintimal inflammation (e.g., T- and B-cells) might have a protective effect on CVD. Interestingly, vasa vasorum and cardiovascular adipose tissue may play an important role in atherogenesis. Inflammation and complement depositions in the vessel wall are likely to contribute to vascular stiffness. Based on biopsy findings, also inflammation in the myocardium and small vessels may contribute to premature CVD in ARDs (cardiac ischemia and heart failure). There is an enormous need for an improved CVD prevention in ARDs. Studies examining the effect of DMARDs/biologics on vascular inflammation and CV risk are warranted.
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PMID:Cardiovascular disease in autoimmune rheumatic diseases. 2354 82

A 53-year-old woman with systemic lupus erythematosus and antiphospholipid syndrome presented with central nervous system (CNS) lupus and vegetation of the mitral and aortic valves. Her CNS lupus was relieved with methylprednisolone pulse therapy; however, her mitral regurgitation worsened, and she developed acute decompensated heart failure. The mitral and aortic valves were replaced with mechanical heart valves. Microscopic examination of the excised valves showed no bacterial invasion, and Libman-Sacks (LS) endocarditis of both valves was confirmed. This was a case of LS endocarditis with clear vegetation that spread over the mitral and aortic valves.
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PMID:Double-valve replacement for mitral and aortic regurgitation in a Patient with Libman-Sacks endocarditis. 2513 Jan 8


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