Intracardiac mass as initial cardiac manifestation of Behçet's disease: think before you cut.

Behçet's disease (BD) is a chronic multisystemic inflammatory disorder. Cardiac abnormalities including intracardiac thrombi have been described in up to 16% of cases. The clinical presentation of cardiac complications in BD may include fever, dyspnea, chest pain, hemoptysis, and edema. We present 2 cases of patients who underwent surgical excision of intracardiac masses thought to be intracardiac malignancies. Further pathological and clinical evaluation established intracardiac inflammatory masses due to BD as the final diagnosis. As intracardiac masses may be the presenting manifestation of BD, it is crucial for echocardiographers to consider BD in the differential diagnosis. A careful history and physical exam looking for signs and symptoms of BD is critical before considering surgical excision of unexplained intracardiac masses. If the final diagnosis is BD anti-inflammatory therapy should be considered the basis of treatment.

Efficacy of adalimumab therapy for life-threatening pulmonary vasculitis in Behçet's disease.

Inflammatory large-vessel vasculitis in Behçet's disease may cause life-threatening arterial aneurysms that are prone to rupture. We report a patient with Behçet's disease with right ventricular thrombus and large aneurysms of the pulmonary arteries that led to recurrent episodes of hemoptysis. Following relapses and only partial response to repeated courses of cyclophosphamide and steroids, the patient was treated with adalimumab (Humira) and is now in clinical remission for over 30 months, with regression of her pulmonary lesions. Anti-TNFα treatment is a potential therapeutic option in patients with life-threatening complications due to large-vessel vasculitis.

[Prevention and treatment of atherosclerosis and cardiovascular diseases].

Atherosclerosis is one of the main causes of morbidity and mortality world-wide and specifically in Israel. These guidelines update the previous guidelines of the Israeli Society for Research, Prevention and Treatment of Atherosclerosis, published in 2005. The need for an update is based on new scientific data published in recent years necessitating changes in the recommendations for preventing and treating atherosclerosis. These guidelines were written in collaboration between all the societies outlined here and the content of this statement was approved by the delegates of these societies. The recommendations were written taking into consideration guidelines published by other international medical societies and also the specific needs of the Israeli medical system. Due to limitations of space, in the current paper we present: assessment of cardiovascular risk, smoking cessation and the treatment of dyslipidemia. Other sections including: recommendations to the general population, nutritional and physical activity recommendations, treatment of hypertension, prevention of ischemic stroke and the metabolic syndrome are available at http://www.ima.org.il/harefuah.

Role of heparanase on hepatic uptake of intestinal derived lipoprotein and fatty streak formation in mice.

BACKGROUND: Heparanase modulates the level of heparan sulfate proteoglycans (HSPGs) which have an important role in multiple cellular processes. Recent studies indicate that HSPGs have an important function in hepatic lipoprotein handling and processes involving removal of lipoprotein particles. PRINCIPAL FINDINGS: To determine the effects of decreased HSPGs chain length on lipoprotein metabolism and atherosclerosis, transgenic mice over-expressing the human heparanase gene were studied. Hepatic lipid uptake in hpa-Tg mice were evaluated by giving transgenic mice oral fat loads and labeled retinol. Sections of aorta from mice over-expressing heparanase (hpa-Tg) and controls (C57/BL6) fed an atherogenic diet were examined for evidence of atherosclerosis. Heparanase over-expression results in reduced hepatic clearance of postprandial lipoproteins and higher levels of fasting and postprandial serum triglycerides. Heparanase over-expression also induces formation of fatty streaks in the aorta. The mean lesion cross-sectional area in heparanase over-expressing mice was almost 6 times higher when compared to control mice (23,984 µm(2)±5,922 vs. 4,189 µm(2)±1,130, p<0.001). CONCLUSIONS: Over-expression of heparanase demonstrates the importance of HSPGs for the uptake of intestinal derived lipoproteins and its role in the formation of fatty streaks.

CMV-related thrombocytopenia treated with foscarnet: a case series and review of the literature.

Severe thrombocytopenia as a complication of CMV infection in immune competent adults is uncommon with only a few cases reported in the literature. The mechanism of CMV-related thrombocytopenia is unclear, resulting in a wide range of treatments used. The use of the antiviral agent ganciclovir was reported in five cases, with variable results. The use of foscarnet, which does not share the myelosuppressive effects of ganciclovir, in adult immune competent patients has not been reported. We review the literature and report two cases of CMV-related thrombocytopenia that were treated with foscarnet. In both cases a recent acute infection with CMV was well established and viral eradication following treatment with foscarnet was demonstrated. In one patient thrombocytopenia resolved following treatment with foscarnet and viral eradication. In the other patient, thrombocytopenia resolved following splenectomy, which was performed after viral eradication. Due to the therapeutic consequences of this diagnosis, it may be worthwhile to perform CMV screening in selected patients with thrombocytopenia. In case CMV viremia is demonstrated, viral eradication seems to have a positive influence on the resolution of thrombocytopenia. Foscarnet is a reasonable first line anti CMV agent in this setting.

ST deviation pattern and infarct related artery in acute myocardial infarction.

BACKGROUND: Myocardial infarction (MI) may be classified as ST-elevation MI (STEMI) or non-ST-elevation MI (NSTEMI). There is little data regarding the relationship between the infarct related artery (IRA), clinical characteristics of the patients, and the ST deviation pattern (ie, STEMI or NSTEMI). HYPOTHESIS: There is a predilection of any coronary artery to a particular ST deviation pattern of acute MI. METHODS: We reviewed our institutional database and selected patients who presented with an acute MI and underwent coronary angiography within 7 days of admission. The analysis included 830 patients of whom 563 had STEMI and 267 had NSTEMI. The culprit lesion was defined by reviewing each patient's angiographic report, electrocardiogram, and echocardiogram. TIMI flow rate was determined. RESULTS: The IRA in STEMI was most frequently the left anterior descending coronary artery (LAD) followed by the right coronary artery (RCA) and then the left circumflex coronary artery (LCX), a statistically significant difference. In patients with NSTEMI there were no significant differences in IRA. Patients with STEMI and LCX as the IRA were significantly younger and had a higher percentage of TIMI grade 3 flow then patients with STEMI and LAD or RCA as IRAs. These differences were not noted in patients with NSTEMI regardless of IRA. CONCLUSIONS: In STEMI there were significant differences in age and TIMI flow depending on the IRA. These findings were not demonstrated in patients with NSTEMI.

ST deviation pattern in acute myocardial infarction is not related to lesion location.

BACKGROUND: In ST-elevation MI (STEMI) the culprit artery is usually occluded, whereas non-STEMI (NSTEMI) it is usually patent. The location of the ruptured plaque may influence MI type. We examine whether the distance from the coronary ostium to the culprit lesion is different in STEMI as compared to NSTEMI. METHODS: We selected patients who presented with an acute MI and underwent coronary angiography during hospitalization. The analysis included 754 patients of whom 514 had STEMI and 240 had NSTEMI. The distance from the coronary ostium to the site of thrombosis was measured. RESULTS: For both STEMI and NSTEMI patients the first 60 mm of the coronary artery contained 75% of the culprit lesions. There were no significant differences in median distances from the vessel ostium to the site of thrombosis as well. CONCLUSIONS: The distance from coronary ostium to culprit lesion is similar in STEMI and NSTEMI. Culprit lesion location does not appear to influence the development of STEMI as opposed to NSTEMI.

The relationship of plasminogen activator inhibitor-1 levels to the ST deviation pattern of acute myocardial infarction.

OBJECTIVES: Myocardial infarction (MI) may be classified as ST elevation MI (STEMI) or non-ST elevation MI (NSTEMI). Procoagulants such as plasminogen activator inhibitor-1 (PAI-1) as well as markers of inflammation such as C-reactive protein (CRP), serum amyloid A (SAA) and interleukin-6 (IL-6) are elevated in acute coronary syndromes. However, no study has examined whether levels of these markers differ in patients with STEMI as opposed to NSTEMI. We sought to determine whether there are differences in plasma levels of PAI-1, CRP, SAA or IL-6 in patients with STEMI compared to patients with NSTEMI. METHODS: Seventy-six consecutive patients presenting with acute MI (37 with STEMI and 39 with NSTEMI) were prospectively enrolled. Blood samples were obtained from patients within 6 h from presentation and plasma PAI-1, CRP, IL-6 and SAA concentrations were measured. RESULTS: Plasma levels of PAI-1 were significantly higher in patients with STEMI compared to NSTEMI: 85.7 +/- 5 vs. 61.3 +/- 5 ng/ml (p < 0.001), while CRP, SAA and IL-6 levels were not significantly different between STEMI and NSTEMI patients. CONCLUSIONS: Higher plasma PAI-1 levels in STEMI patients may contribute to the predilection of these patients to occlusive thrombi and STEMI.

Ockham's razor or Hickam's dictum: a right atrial mass following excision of left atrial myxoma.

We report a case of a 54-year-old man presenting with a right atrial mass 7 months after undergoing a left atrial myxoma excision surgery. The differential diagnosis included recurrent myxoma or thrombus. The patient underwent repeat open sternotomy on cardiopulmonary bypass. Histopathological evaluation of the mass revealed an organizing thrombus. This report is, to our knowledge, the first that demonstrates right atrial thrombus shortly following excision of left atrial myxoma.

Ethnic gap in coronary artery disease: comparison of the extent, severity, and risk factors in Arab and Jewish middle-aged women.

The authors examined risk factors and extent of coronary artery disease (CAD) among Jewish and Arab women in Jerusalem, where Arab women were found to have worse outcome. All angiographically confirmed cases of CAD among women aged 45 to 65 years who were hospitalized during 1990 to 1995 consisted of 40 Arab and 179 Jewish patients. Arab women had more atypical clinical presentations (P<.0001) and more extensive CAD (P=.0016) despite younger age (53+/-3 vs 55+/-5 years; P<.0003) and lesser smoking (P<.0006). The Arab women, however, were more likely to be obese (80% vs 46%; P=.0002), be physically inactive (100% vs 89%; P=.0285), and have diabetes mellitus (73% vs 40%; P=.0004). Moreover, they were more likely to have 3 or more risk factors (45% vs 23%; P=.036). Thus, a combination of an atypical presentation and higher risk (ie, diabetes mellitus combined with hypertension and other risk factors) and much more extensive disease readily explains their worse outcome.

Brain natriuretic peptide levels predict perioperative events in cardiac patients undergoing noncardiac surgery: a prospective study.

OBJECTIVES: Brain natriuretic peptide (BNP) levels correlate with prognosis in patients with cardiac disease and may be useful in the risk stratification of cardiac patients undergoing noncardiac surgery (NCS). The objective of this study was to examine whether BNP levels predict perioperative events in cardiac patients undergoing NCS. METHODS: Patients undergoing NCS with at least 1 of the following criteria were included: a clinical history of congestive heart failure (CHF), ejection fraction <40%, or severe aortic stenosis. All patients underwent echocardiography and measurement of BNP performed using the ADVIA-Centaur BNP assay (Bayer HealthCare). Clinical endpoints were death, myocardial infarction or pulmonary congestion requiring intravenous diuretics at 30 days of follow-up. RESULTS: Forty-four patients were entered into the study; 15 patients (34%) developed cardiac postoperative complications. The mean BNP level was 1,366 +/- 1,420 pg/ml in patients with events and 167 +/- 194 pg/ml in patients without events, indicating a highly significant difference (p < 0.001). The ROC area under the curve was 0.91 (95% CI 0.83-0.99) with an optimal cutoff of >165 pg/ml (100% sensitivity, 70% specificity). CONCLUSIONS: BNP levels may predict perioperative complications in cardiac patients undergoing NCS, and the measurement of BNP should be considered to assess the preoperative cardiac risk.

Smoking cessation does not alter ST deviation pattern of recurrent myocardial infarctions.

BACKGROUND: Myocardial infarction (MI) may be classified as ST elevation MI (STEMI) or non ST elevation MI (NSTEMI). We used the term recurrent MI (RMI's) to denote repeated MI episodes, in a particular patient, in which a different coronary site is responsible for each episode. Recently we reported that most patients with recurrent MI episodes will have either STEMI's or NSTEMI's but not both. A history of smoking was associated with recurrent STEMI's. OBJECTIVE: To determine whether smoking cessation will alter the type of RMI in patients with an index MI of STE type. METHODS: The analysis included 128 patients who underwent at least 2 MI episodes. We attempted to include only MI's of native vessels, without the presence of extra cardiac conditions that intensify myocardial ischemia. All 128 patients were active smokers who presented with an index MI of the STE type. Of these patients 94 had recurrent STEMI and 34 had recurrent NSTEMI (STE/NSTE group). RESULTS: We identified all patients who were no longer active smokers at the time of the recurrent MI: there were 31 (33%) such patients in the STEMI group and 13 (38%) in the STE/NSTE group (p=NS). CONCLUSION: Smoking cessation did not influence the type of recurrent MI in these patients.

The diagnosis of primary cardiac lymphoma by right heart catheterization and biopsy using fluoroscopic and transthoracic echocardiographic guidance.

We describe a case of primary cardiac lymphoma diagnosed by transvenous biopsy under fluoroscopic and transthoracic echocardiographic guidance. A 38-year-old man was admitted because of exertional dyspnea. ECG revealed complete atrioventricular block. Transthoracic echocardiography revealed a large mass attached to the interventricular septum and protruding into the right atrium. The patient underwent a right heart catheterization and a biopsy was taken from the mass using fluoroscopic and transthoracic echocardiographic guidance. Diagnosis of malignant lymphoma was established by the biopsy specimen. The use of transthoracic echo in conjunction with fluoroscopy may be useful for the diagnosis of intracardiac mass transvenously.

Role of naturally occurring CD4+ CD25+ regulatory T cells in experimental atherosclerosis.

OBJECTIVE: Naturally occurring CD4+ CD25+ regulatory T cells (Tregs) exert suppressive effects on effector CD4 cells and downregulate experimental autoimmune disorders. We investigated the importance and potential role of Tregs in murine atherogenesis. METHODS AND RESULTS: Tregs were investigated comparatively between aged and young apolipoprotein E-knockout (ApoE-KO) mice and age-matched C57BL/6 littermates. The effect of oxidized LDL (oxLDL) was tested on the functional suppressive properties of Tregs from ApoE-KO and C57BL/6 mice. Tregs, CD4+ CD25- cells, and saline were infused into ApoE-KO mice to study their effects on atherogenesis. Treg numbers were reduced in atherosclerotic compared with nonatherosclerotic ApoE-KO mice. The functional suppressive properties of Tregs from ApoE-KO mice were compromised in comparison with those from their C57BL/6 littermates. Thus, oxLDL attenuated the suppressive properties of Tregs from C57BL/6 mice and more so in ApoE-KO mice. Transfer of Tregs from age-matched ApoE-KO mice resulted in significant attenuation of atherosclerosis compared with that after delivery of CD4+ CD25+/- T cells or phosphate-buffered saline. CONCLUSIONS: CD4+ CD25+ Tregs may play a protective role in the progression of atherosclerosis and could be considered a therapeutic tool if results from human studies can solidify observations in murine models.

Chronicle of a death foretold: a case of catastrophic vascular Behcet's disease.

A 20-year-old man with Behcet's disease characterized by recurrent arterial aneurysms presented with a new aortic root aneurysm. This patient previously had aneurysms of the coronary arteries and vein, as well as ruptured renal artery aneurysm. Chronic maintenance immunosuppressive therapy was recommended due to the catastrophic nature of the disease, which the patient refused to take. The patient died shortly after admission. This case demonstrates the unique catastrophic natural history of vascular Behcet's disease with recurrent life-threatening arterial events, and this case stresses the therapeutic dilemma of maintenance immunosuppressive therapy in selected patients.

Echocardiographic manifestations of Adamantiades-Behcet's disease.

Adamantiades-Behcet disease (ABD) is a multisystemic, chronic inflammatory disorder of unknown etiology with diffuse clinical manifestations including those involving the cardiovascular system. While the disease is most prevalent in the Mediterranean region, the Middle East and the Far East, its prevalence is increasing in Western countries due to migration patterns. Cardiovascular involvement in ABD may include myocardial disease, venous disease and disease of the aorta and great vessels. Use of echocardiography in these patients is crucial to assess their pathology and in this article we review the spectrum of echocardiographic findings in patients with ABD.

Spleen rupture secondary to anticonvulsant hypersensitivity syndrome.

Anticonvulsant hypersensitivity syndrome (AHS) is a rare complication associated with the use of anti-epileptic medications. The syndrome's main symptoms are cutaneous eruptions, fever, hepatitis, and lymphadenopathy. We describe a 23-year-old woman who developed AHS 2 months after starting phenytoin therapy. She presented with fever, orofacial edema, skin rash, and lymphadenopathy. Four days after admission, she developed agonizing epigastric pain with diffuse epigastric tenderness. An abdominal CT scan demonstrated splenomegaly with a large, hypodense area involving the upper half of the spleen, consistent with splenic rupture. She was managed medically in an effort to avoid splenectomy. There are no other documented cases of spleen rupture linked to AHS. A possible mechanism is the alteration of lymphocytic function due to the accumulation of cytotoxic metabolites and infiltration of the spleen with lymphocytic cells, distending the capsule and predisposing it for rupture. We believe that the case presented here should increase awareness of the potentially life-threatening complications associated with AHS.

Physicians underdiagnose and undertreat obesity in ishemic heart disease patients: data from the HOLEM Study Group.

BACKGROUND: Obesity is an independent risk factor for ischemic heart disease and affects the status of other risk factors for cardiovascular disease. OBJECTIVE: To study the attitude of physicians to obesity by examining discharge letters of overweight patients with ischemic heart disease. METHODS: We used the HOLEM database for this analysis. The HOLEM project was designed to study the NCEP (National Cholesterol Education Program) guideline implementation among patients with IHD at hospital discharge. We documented the recording of risk factors and treatment recommendations for IHD by reviewing the discharge letters of 2994 IHD patients admitted to four central hospitals in Israel between 1998 and 2000. A follow-up visit was held 6-8 weeks after discharge, at which time the diagnosis of IHD was verified, risk factor status was checked, height and weight were measured and drug treatment was reviewed. RESULTS: Mean body mass index was 28.3 kg/m2 and 32% were obese (BMI > or = 30 kg/m2). Only 39.6% of the obese patients and 65.8% of the morbidly obese patients (BMI > or = 40 kg/m2) had "obesity" noted in their discharge letters, and weight loss recommendation was written in only 15% of the obese patients' discharge letters. Acute episodes like acute myocardial infarction and unstable angina did not influence the notation of obesity, and only BMI and the number of additional risk factors were positively correlated with the notation of this risk factor. CONCLUSIONS: Despite the importance of obesity, weight status was not noted and weight loss was not recommended in most of the discharge letters of obese IHD patients.