Coronary aneurysm in Behcet''s disease. Report of a case

Polymyositis, dermatomyositis, and inclusion body myositis, although immunopathologically distinct, share 3 dominant histological features: inflammation, fibrosis, and loss of muscle fibers. Progress in molecular immunology and immunogenetics has enhanced our understanding of these cellular processes. Based on the T-cell receptor gene rearrangement, the autoinvasive CD8+ T cells in polymyositis and inclusion body myositis, but not dermatomyositis, are specifically selected and clonally expanded in situ by heretofore unknown muscle-specific autoantigens. The messenger RNA of cytokines is variably expressed, except for a persistent up-regulation of interleukin 1beta in inclusion body myositis and transforming growth factor beta in dermatomyositis. In inclusion body myositis, the interleukin 1, secreted by the chronically activated endomysial inflammatory cells, may participate in the formation of amyloid because it up-regulates beta-amyloid precursor protein (beta-APP) gene expression and beta-APP promoter and colocalizes with beta-APP within the vacuolated muscle fibers. In dermatomyositis, transforming growth factor beta is overexpressed in the perimysial connective tissue but is down-regulated after successful immunotherapy and reduction of inflammation and fibrosis. The degenerating muscle fibers express several antiapoptotic molecules, such as Bcl-2, and resist apoptosis-mediated cell death. In myositis, several of the identified molecules and adhesion receptors play a role in the process of inflammation, fibrosis, and muscle fiber loss, and could be targets for the design of semispecific therapeutic interventions.     

Association between antiphospholipid antibodies and arterial or venous thrombosis/thrombocytopenia

The relationship between thrombotic or thrombocytopenic complications and the existence of anticardiolipin antibodies (aCL) and/or lupus anticoagulant (LA) was studied in 146 patients with systemic lupus erythematosus (SLE). The prevalence of arterial thrombosis was obviously higher in patients who had both aCL and LA than in patients with either aCL or LA alone or in those with neither. Since a substantial fraction of the former group of patients with arterial thrombosis also had thrombocytopenia, there is a possibility that aCL and LA might enhance platelet activation and aggregation. To test this hypothesis, we studied the in vitro effects of aCL and LA on the enhancement of platelet activation by flow cytometric analysis using anti-CD62P and anti-CD41 monoclonal antibodies directed against platelet activation-dependent granule-external membrane (PADGEM) protein and platelet glycoprotein IIb (GPIIb). The IgG fraction purified from aCL+.LA+ plasma apparently enhanced platelet activation induced by adenosine diphosphate (ADP) at a low concentration, but IgG fractions from aCL+.LA- or aCL-.LA+ plasma did not cause enhancement of platelet activation. These results suggest that aCL and LA may cooperate to promote platelet activation, and may be involved, at least partially, in the pathogenesis of arterial thrombosis in patients with SLE.     

Ischemic heart disease with the anterior branches of the right coronal artery

The coronary arteries shunting was performed in four elderly men with severe coronary heart disease, complicated by stagnant heart insufficiency and right ventriculus insufficiency (RVI). One patient could not be disconnected from the artificial blood circulation (ABC) apparatus till the venous shunting (VS) to atherosclerotically strictured right ventriculus anterior branch of right coronary artery was not accomplished. Other three patients, to whom VS to this branch was already conducted, were disconnected from the ABC without complications. In three patients echocardiography trusted RV disorders existence before this branch shunting conduction with improvement after shunting. While coronary arteries shunting conduction the anastomosis ought to be done to anterior branches of RVI when they are affected.     

Bronchial atresia associated with epibronchial right pulmonary artery and aberrant right middle lobe artery

We report a case of bronchial atresia associated with an epibronchial right pulmonary artery (ERPA) and an aberrant right middle lobe artery (ARMLA). CT showed a branching opacity, which was hyperintense on MR images, in the anterior segment of the right upper lobe with segmental hyperinflation and the ERPA. At surgery, the ARMLA was found to originate from the ERPA, crossing the anterior aspect of the right upper lobe bronchus. It is postulated that the ARMLA might have interfered with the normal bronchial development, leading to the development of segmental bronchial atresia.     

Lung transplantation for primary pulmonary hypertension and giant pulmonary artery aneurysm

We report the case of an 18-year-old patient with a giant pulmonary artery aneurysm and primary pulmonary hypertension who was successfully treated with bilateral lung transplantation and complete reconstruction of the pulmonary artery.     

Anomalous right coronary artery from the pulmonary artery in Taussig-Bing anomaly

The presence of associated anomalies in patients with double-outlet right ventricle can significantly alter surgical intervention. Preoperative delineation of these anomalies can facilitate surgical planning and improve outcome. We describe a case in which the right coronary artery and anterior descending coronary artery arose from the pulmonary artery in a patient with double-outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). Recognition of this important anomaly prevented significant intraoperative myocardial damage by altering techniques of cardioplegia administration for myocardial preservation.     

Imaging spectrum of thrombo-occlusive vascular disease associated with antiphospholipid antibodies

The association of antiphospholipid antibodies with unexplained thrombo-occlusive vascular disease is well known but often remains unrecognized. The most well-studied clinical manifestation is venous thrombosis, but arterial occlusive disease involving multiple sites is also well documented. Twenty-six cases of thrombo-occlusive disease were observed in 22 patients over a 3-year period. Magnetic resonance imaging and angiography were used to make the diagnoses. None of the patients who underwent angiography or venography developed thrombolytic disease related to the puncture site. This group of patients with antiphospholipid antibody syndrome had a wide distribution of arterial and venous thrombotic disease. Radiologists should consider antiphospholipid antibody syndrome in the differential diagnosis when evaluating thrombo-occlusive vascular disease that is unexpected or occurs without risk factors. Knowledge of antiphospholipid antibody status has important implications for prognosis and therapy.     

Anomalous origin of the right coronary artery from the pulmonary artery

Anomalous origin of a coronary artery from the pulmonary artery is a relatively rarer congenital anomaly which almost exclusively involves the left coronary artery. A previously healthy 25-year-old woman had a cardiopulmonary arrest. Following resuscitation, investigations revealed an even rarer anomaly, an anomalous right coronary artery arising from the pulmonary artery. Successful surgical repair was accomplished by reimplanting the right coronary artery into the aortic root. The patient is currently asymptomatic 2 years postoperatively. The pertinent literature is reviewed.     

Superior mesenteric artery aneurysm in Beh?et's disease

Beh?et's disease is a multisystem disorder characterized by recurrent orogenital ulcerations and uveitis. Vascular involvement can include both arteries and veins, with a preponderance of venous lesions. Aneurysms of splanchnic arteries due to Beh?et's disease have been rarely reported. We present an unusual case of superior mesenteric artery aneurysm due to Beh?et's disease diagnosed by ultrasonography and angiography.     

Thromboembolic disease developing during oral contraceptive therapy in young females with antiphospholipid antibodies

The role of oral contraceptives as a triggering factor for thrombosis in patients with lupus anticoagulant (LA) and/or anticardiolipin antibodies (ACA) has not yet been established. We describe the cases of three women aged 19, 29 and 48 years who developed venous thrombosis after 16 +/- 3.4 (mean +/- SD) cycles of oral contraceptives. They were all asymptomatic before taking the pill. Two patients subsequently developed venous and/or arterial recurrence of thrombosis. Laboratory studies performed after the diagnosis of thrombosis, showed the presence of LA and elevated levels of ACA (IgG and IgM) in all three patients. None of these patients had autoimmune diseases and therefore appeared to have a primary antiphospholipid antibody syndrome. The three patients belonged to a group of 45 young females who experienced their first thrombotic event while taking the pill. This group had a similar prevalence (8%) for antithrombin deficiency and antiphospholipid antibodies. We surmise that some of the women who developed venous thrombosis while taking the pill might have an undetected primary antiphospholipid syndrome.     

Management of giant coronary artery aneurysm with fistulization into the right atrium

A giant right coronary artery aneurysm communicating with the right atrium is reported. Its diagnosis using echocardiography, computed tomography of the chest, and angiography is illustrated. The operative management of this rare cardiac pathology is described. The role of operation in such a large aneurysm is emphasized.     

Surgical treatment of coronary artery aneurysm with rupture into the right atrium

Considering the increasing number of patients with chest pain who undergo routine coronary artery arteriography, coronary artery aneurysm may be found more frequently. To know how to manage these aneurysms, we must understand their possible complications. The aneurysms can produce symptoms of angina or acute myocardial infarction by total thrombosis of the aneurysm and vessel, embolism to the distal vessel, or progressive enlargement and encroachment upon the distal vessel until it is occluded. Moreover, the aneurysm may enlarge and rupture into the free pericardium or produce a fistula by eroding into a chamber of the heart. The case described herein may represent the first reported case of a coronary artery aneurysm eroding into a cardiac chamber and causing an arteriovenous fistula. The treatment of choice is resection of the aneurysm, closure of the fistula, and re-establishment of continuity of the distal coronary artery with a saphenous vein bypass graft.     

Large right atrial aneurysm: rare cause of recurrent pulmonary embolism]

Case report on a 72 year old female patient presenting with collapse and progressive dyspnea. X-ray of the chest showed a marked enlargement of the heart extending to the right. Echocardiography revealed an impressive displacement of the heart chambers to the left caused by a large aneurysm (11 x 5 x 5 cm) located anteriorly and medially from the right heart communicating with the right atrium. Pronounced compression of the basal part of the right ventricle was present. Within the aneurysmatic cavity several highly mobile thrombi and extensive spontaneous echo contrast were demonstrated. On magnetic resonance imaging and right heart angiocardiography the aneurysm was visualized as well. Coronary angiography revealed a marked curved displacement of the right coronary artery to the left side. Perfusion lung scanning suggested bilateral segmental pulmonary embolism proven by digital substraction angiography. With long-term oral anticoagulation the patient remained symptom-free during follow-up of now more than four years.     

Case report: tuberculous pulmonary arteritis--an unusual cause of right pulmonary artery stenosis

Previously we reported that intracerebroventricular (i.c.v.) administration of nitric oxide (NO) donors, sodium nitroprusside (SNP) (1-10 microg), and 3-morpholino-sydnonimine (SIN-1) (10-100 microg), induced dose-dependent increases in plasma prolactin levels of freely moving male rats, suggesting a role of NO in the control of prolactin secretion. The present results show that i.c.v. pretreatment with methylene blue (MB) (30 microg), a guanylyl cyclase inhibitor, significantly reduced the effects of microinjections of SNP (3 and 5 microg), however, this did not modify the stimulatory action of SIN-1 (30 microg) on plasma prolactin levels of conscious male rats. Alone, MB did not modify basal prolactin levels. These results suggest different mechanisms of action of SNP and SIN-1 to stimulate prolactin secretion in vivo. Activation of soluble guanylyl cyclase seems to mediate the neuroendocrine action of NO released from SNP but not of SIN-1. Different cellular distribution of NO generating activity from these donors as well as the possible generation of other radicals simultaneously with NO from SIN-1 could explain these differences.     

Different anticoagulant and immunological properties of anti-prothrombin antibodies in patients with antiphospholipid antibodies

Lupus anticoagulant (LA) antibodies are acquired inhibitors of coagulation belonging-together with anticardiolipid (aCL) antibodies-to the family of antiphospholipid antibodies. Since LA antibodies affect coagulation reactions via recognition of the complex of lipid-bound prothrombin, they may be better named anti-prothrombin antibodies. We studied their immunological properties in the plasma of 59 patients with antiphospholipid antibodies by means of specific ELISA systems that allowed the characterization of the interaction of these antibodies with human prothrombin and anionic phospholipids. The mode of presentation of prothrombin was found to greatly influence the reactivity of anti-prothrombin antibodies. In fact, when plain polystyrene plates were used to immobilize prothrombin, virtually no binding was observed. Conversely, when prothrombin was coated on high-activated PVC ELISA plates, 34 samples (58%) contained antibodies that recognize human prothrombin in solid phase. In particular, IgG antibodies were found in 21 plasmas and IgM in 22; both IgG and IgM isotypes were present in 9 of these cases. A higher prevalence was observed in the ELISA for the detection of the antibodies directed at the calcium-mediated complex of phosphatidylserine (PS)-bound prothrombin: 53 samples (90%), preadsorbed with cardiolipin liposomes to remove aCL antibodies, showed the presence of IgG and/or IgM anti-prothrombin antibodies. When the results were analyzed according to the immunoglobulin isotypes, 44 (75%) and 39 (66%) samples were found to contain IgG and IgM anti-prothrombin antibodies, respectively. Both IgG and IgM were present in the plasma of 30 patients. Only half of these samples reacted also with PVC-bound prothrombin. Apparently, the higher rate of positivity of the ELISA for the detection of antibodies to the complex of PS-bound prothrombin was not due to differences in the amount of antigen available in the 2 systems, as judged by binding experiments performed with a rabbit polyclonal anti-human prothrombin antiserum. Finally, the anticoagulant properties of 14 total IgG preparations (12 of them contained anti-prothrombin antibodies positive in both ELISA systems, whereas the other 2 cases reacted either with PVC-bound prothrombin only or with PS-bound prothrombin only) were evaluated by diluted Russell's Viper Venom Time and by diluted activated Partial Thromboplastin Time. To rule out the beta 2-glycoprotein I (beta 2-GPI)-dependent anticoagulant effect of the aCL antibodies contained in the preparations, the coagulation tests were performed in beta 2-GPI deficient plasma. Six preparations failed to show anticoagulant activity in both assay systems, suggesting that 2 types of IgG anti-prothrombin antibodies exist, that differ with respect to their anticoagulant properties. These findings suggest that anti-prothrombin antibodies resemble aCL antibodies with respect to the behaviour in "in vitro" coagulation reactions and underline the wide heterogeneity of antiphospholipid antibodies.     

Variations in normal coronary vasodilatory reserve stratified by artery, gender, heart transplantation and coronary artery disease

OBJECTIVES: The purpose of the study was to assess the spectrum of coronary vasodilatory reserve values in patients with angiographically normal arteries who had atypical chest pain syndromes or remote coronary artery disease or were heart transplant recipients. BACKGROUND: The measurement of post-stenotic coronary vasodilatory reserve, now possible in a large number of patients in the cardiac catheterization laboratory, is increasingly used for decision making. Controversy exists regarding the range of normal values obtained in angiographically normal coronary arteries in patients with different clinical presentations. METHODS: Quantitative coronary arteriography was performed in 214 patients classified into three groups: 85 patients with chest pain syndromes and angiographically normal arteries (group 1); 21 patients with one normal vessel and at least one vessel with > 50% diameter lumen narrowing (group 2); and 108 heart transplant recipients (group 3). Coronary vasodilatory reserve (the ratio of maximal to basal average coronary flow velocity) was measured in 416 arteries using a 0.018-in. (0.04 cm) Doppler-tipped angioplasty guide wire. Intracoronary adenosine (8 to 18 micrograms) was used to produce maximal hyperemia. RESULTS: Coronary vasodilatory reserve was higher in angiographically normal arteries in patients with chest pain syndromes (group 1:2.80 +/- 0.6 [group mean +/- SD]) than in normal vessels in patients with remote coronary artery disease (group 2: 2.5 +/- 0.95, p = 0.04); both values were significantly higher than those in the post-stenotic segment of the diseased artery (1.8 +/- 0.6, p < 0.007). Coronary vasodilatory reserve in transplant recipients (group 3) was higher than that in the other groups (3.1 +/- 0.9, p < 0.05 vs. groups 1 and 2) as a group and for individual arteries. When stratified by vessel, coronary vasodilatory reserve was similar among the left anterior descending, left circumflex and right coronary arteries. There were no differences between coronary vasodilatory reserve values on the basis of gender for patients with coronary artery disease and transplant recipients. In group 1 (chest pain), there was a trend toward higher coronary vasodilatory reserve in men than in women (2.9 +/- 0.6 vs 2.7 +/- 0.6, p = 0.07). CONCLUSIONS: These findings identify a normal reference range for studies assessing the coronary circulation and post-stenotic coronary vasodilatory reserve in patients with and without coronary artery disease encountered in the cardiac catheterization laboratory.