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

Sneddon's syndrome consists of livedo reticularis and cerebral vascular accidents with no evidence of systemic disease responsible for the livedo. The syndrome has been assimilated to a subgroup of systemic lupus erythematosus (SLE) with presence of antibodies directed against phospholipids. Recently, a significant increase in the frequency of cardiac valve diseases has been demonstrated in some SLE patients with livedo reticularis, cerebral vascular accidents and antiphospholipid antibodies. We report the case of a 26-year old woman who had been presenting for 6 years with idiopathic livedo reticularis. Her history was remarkable for the occurrence of 2 cerebral ischaemic accidents at the ages of 23 and 26 years, generalized convulsive seizures at 22 years, and hypertension of pregnancy with 2 miscarriages. Biopsy of the livedo showed normal histological patterns, but electron microscopy detected an obliterating endothelial proliferation and endothelial cells with numerous Weibel-Palade bodies. Laboratory signs of SLE, as well as antiphospholipid antibodies were absent. At the age of 26 years, cardiac abnormalities were heard at auscultation for the first time, and echocardiography showed that they were due to a fairly loose mitral stenosis. According to Burton's criteria our patient had all the typical features of Sneddon's syndrome. The finding of mitral stenosis--an emboligenic cardiopathy that is potentially responsible for cerebral vascular accidents--raises the problem of its relationship with Sneddon's syndrome. The association does not seem to be fortuitous, since our case is very similar to the cases of SLE or antiphospholipid antibody syndrome associated with cardiac valve lesions. However, this case is particular in that 6 years after the onset of the disease there was still no sign of SLE and of antiphospholipid antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Livedo reticularis, cerebrovascular disorders and mitral disease: a new cause of Sneddon's syndrome?]. 208 86

Patients with cyanotic congenital heart disease exhibit an increased incidence of thrombotic episodes and are frequently thrombocytopenic. We studied the sera of 15 patients with this type of heart malformation, searching for anticardiolipin antibodies. 3/15 had positive results. The three of them were adult females; two had thrombotic episodes and a false positive VDRL. Thus, cyanotic congenital heart disease may be another disease entity associated with the antiphospholipid syndrome.
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PMID:Antiphospholipid syndrome in patients with cyanotic congenital heart disease. 758 82

Two cases of primary antiphospholipid antibody syndrome are reported. One patient presented multiple abortions and epilepsy. The second patient was affected by a brain vascular accident, with a residual hemiparesis. Both cases showed livedo reticularis in arms, NMR evidence of diffuse lesions of the white matter, high serum levels of anticardiolipin antibodies and cardiopathy. Lupus anticoagulant was also found in the serum of the first patient, and cortisone and antiaggregants enabled her to reach term in a fifth pregnancy after four miscarriages. In the other case histological examination of specimens of skin, peripheral nerve and skeletal muscle revealed occlusive, non arteriosclerotic vasculopathy and an absence of inflammatory lesions. Histological study has rarely been performed in primary antiphospholipid syndrome but suggests that the mechanism of thrombosis is not vascular; in our subjects it revealed findings similar to those in Sneddon syndrome.
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PMID:Primary antiphospholipid syndrome: two case reports, one with histological examination of skin, peripheral nerve and muscle. 799 67

Current advances in the study of hypertrophic osteoarthropathy are discussed. An update of the classification of hypertrophic osteoarthropathy is given in which the POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is included among the associated diseases. Cyanotic heart disease is the internal illness most closely linked to hypertrophic osteoarthropathy. The bony alterations at the distal phalanxes and on the periosteum of the tubular bones leave a characteristic and indelible mark that can be diagnosed centuries after the death of the individual. Current thinking suggests that localized activation of endothelial cells by an abnormal platelet population, with the ensuing release of fibroblast growth factors, plays a central role in the pathogenesis of the acropachy. Antiphospholipid syndrome may be a feature of cardiogenic hypertrophic osteoarthropathy. Unraveling the mechanisms of hypertrophic osteoarthropathy may help in understanding the pathogenesis of the associated diseases.
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PMID:Hypertrophic osteoarthropathy. 911 Jan 40

This is a unique report of systemic-to-pulmonary artery shunt thromboses secondary to primary antiphospholipid syndrome and antithrombin III deficiency in a neonate with cyanotic congenital heart disease. This infant with tricuspid atresia experienced thromboses of two modified Blalock-Taussig shunts en route to a bidirectional cavo-pulmonary shunt and potential future Fontan operation. Chronic warfarin anticoagulation has prevented additional thrombo-embolic events.
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PMID:Primary antiphospholipid syndrome: a cause of catastrophic shunt thrombosis in the newborn. 1077 32

Thromboses are infrequent but serious complications of patients in the NICU. Thromboses tend to occur in very sick neonates, particularly preterm neonates, and the majority of such thromboses are related to central vascular catheters. Other risk factors for neonatal thromboses include infants of diabetic mothers, sepsis, small for gestational age, congenital heart disease, maternal antiphospholipid syndrome, and possibly inherited prothrombotic disorders. Appropriate treatment, dosage, and duration of therapy for neonatal thromboses has not been studied in clinical trials. Treatment options include observation, anticoagulation, thrombolysis, and surgical thrombectomy. Regardless of the treatment chosen, all neonates with thromboses require frequent reassessment of the thromboses by angiography, echocardiography, or ultrasound until thrombus resolution occurs.
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PMID:Evaluation and treatment of thrombosis in the neonatal intensive care unit. 1098 32

Antiphospholipid syndrome (APS) is defined by the presence of arterial and venous thromboses, recurrent fetal death, cerebrovascular accidents, hemolytic anaemia, thrombocytopenia and various other manifestations in different organs. APS is a clinical entity that can appear commonly alongside systemic lupus erithemathous on it can occur as a primary disease. The syndrome is defined by the presence of antiphospholipid antibodies in serum, a group of immunoglobulins (IgG, IgM, IgA or an mixture of them) that adopt an hexagonal configuration when they are incubated at 37grades C. In APS, it is rather common to find cardiac lesions such as non-verrucous endocarditis, valvular lesions (especially of the mitral valve), microvascular cardiac disease and more risk of thrombosis at this level, myxomas that could be the cause of a systemic inflammation in relation to the production of antiphospholipid antibodies, intracardiac thrombii and congenital heart disease. We present the case of a woman with APS associated with interauricular communication whose initial diagnosis were pulmonary thromboembolism and cerebrovascular stroke. We were able to diagnose the cardiac abnormality by the use of transesophagic echocardiography. We propose the use of this imaging technique for patients with APS even though the transthoracic Doppler echocardiography was found to be normal. In this way we will be able to rule out cardiac lesions which could also be the cause of embolic manifestations.
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PMID:[Antiphospholipid syndrome. The use of transesophageal echocardiography]. 1458 41

We describe two patients who developed stroke and ischemia of the left lower limb, related to intracardiac thrombi, as the first manifestation of primary antiphospholipid syndrome (PAPS). Transesophageal echocardiography (TEE) revealed intracardiac thrombi as abnormal hyperechogenic, nonmobile masses, firmly attached to the left atrial appendage and the anterior part of the mitral annulus, respectively. Our patients received high-intensity oral anticoagulant therapy (INR 3.0-4.0), which resulted in the rapid disappearance of clinical symptoms, without subsequent recurrence of thromboembolic manifestations, and the disappearance of intracardiac thrombi on TEE. Our findings underscore that PAPS should be suspected in patients presenting with intracardiac thrombi, even if they have no previous history of thromboembolic disorders. Moreover, because intracardiac thrombi may precede other manifestations of PAPS, this finding in young patients without underlying heart disease should invoke a search for both antiphospholipid and anticardiolipin antibodies.
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PMID:Intracardiac thrombi in primary antiphospholipid syndrome: two case reports. 1496 6

The etiology of valvular heart diseases (VHD) has changed in the last 50 years in the industrialized countries. A significant reduction in the incidence of rheumatic fever and its sequelae, increase in life expectancy, recognition of new causes of VHD and advancement in technology are responsible for the metamorphosis of the etiology of VHD. Heritable disorders of connective tissue (marfan syndrome, Ehlers-Danlos syndrome, adult polycystic kidney disease, floppy mitral valve/mitral valve prolapse); congenital heart disease (bicuspid aortic valve); inflammatory/immunologic disorders (rheumatic fever, AIDS, Kawasaki disease, syphilis, seronegative spondyloarthropathies, systemic lupus erythematosus, antiphospholipid syndrome); endocardial disorders (nonbacteremic thrombotic endocarditis, infective endocarditis, endomyocardial fibroelastosis); myocardial dysfunction (ischemic heart disease, dilated cardiomyopathy, hypertrophic cardiomyopathy); diseases and disorders of other organs (chronic renal failure, carcinoid heart disease); aging (calcific aortic stenosis, mitral annular calcification); postinterventional valvular disease; drugs and physical agents are all clinical entities associated with VHD. It should be emphasized that VHDs still constitute a major health problem which will increase with the aging population.
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PMID:Etiology of valvular heart disease. 1503 Feb 51

Cerebral venous thrombosis in various clinical conditions, such as congenital heart disease (CHD) and hypercoagulable states, have been recognized previously. However, dural sinus thrombosis in a pediatric patient with Antiphospholipid Syndrome (APLS) after the repair of a ventricular septal defect (VSD) has not been reported yet. A child who underwent an operation for the surgical repair of VSD under cardiopulmonary bypass (CPB) developed a stroke after the procedure. A cranial computer tomography (CT) scan of the patient showed dural sinus thrombosis and severe cerebral edema. APLS and CPB were thought to be the only risk factors of dural sinus thrombosis. This observation warrants attention and screening should be taken into consideration in patients with APLS who carry a higher risk for dural sinus thrombosis after CPB.
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PMID:Dural sinus thrombosis after cardiopulmonary bypass. 1516 29


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