Anatomical features and clinical correlations in Caucasian patients with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy.

AIM: Arrhythmogenic right ventrticular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by fibrofatty replacement and a high risk of ventricular arrhythmias (VA) and sudden cardiac death (SCD). The aim of the present investigation is to examine the pathological profile and the clinical correlations in a group of ARVD/C patients. METHODS: We conducted a multicenter study evaluating 47 patients (31 men; mean age 37±14 years) with definite ARVD/C. Diagnosis was established according to the actual clinicomorphologic criteria at autopsy or clinically. We divided the study population in 2 different groups. First group included 28 alive patients and the second 19 patients dead suddenly. RESULTS: Age at presentation was different in the two groups (P=0.0015). We observed an important association regarding the risk of sudden death and the history of physical exercise (P=0.0017). Moreover patients with negative outcome (i.e., SCD, cardiac transplantation, congestive heart failure) had a significantly association with biventricular form of ARVD/C (P=0.0034) and age presentation (P=0.003). Left ventricular (LV) involvement was frequently observed in the two groups (17% and 32% respectively). Post-mortem examination revealed frequent inflammatory infiltrates (26%) indicating active myocarditis, which probably justify the fatal arrhythmic events occurred in these patients. CONCLUSION: Frequent LV involvement justifies the recent adoption of the broad term Arrhythmogenic Cardiomyopathy. Early age presentation, sport activity and the biventricular form of ARVD/C represent important predictors of adverse outcome that can be useful to early identify patients at high risk.

ST segment elevations: always a marker of acute myocardial infarction?

Chest pain is one of the chief presenting complaints among patients attending Emergency department. The diagnosis of acute myocardial infarction may be a challenge. Various tools such as anamnesis, blood sample (with evaluation of markers of myocardial necrosis), ultrasound techniques and coronary computed tomography could be useful. However, the interpretation of electrocardiograms of these patients may be a real concern. The earliest manifestations of myocardial ischemia typically interest T waves and ST segment. Despite the high sensitivity, ST segment deviation has however poor specificity since it may be observed in many other cardiac and non-cardiac conditions. Therefore, when ST-T abnormalities are detected the physicians should take into account many other parameters (such as risk factors, symptoms and anamnesis) and all the other differential diagnoses. The aim of our review is to overview of the main conditions that may mimic a ST segment Elevation Myocardial Infarction (STEMI).

WITHDRAWN: Proteinase 3 (PR3) gene is highly expressed in CBF leukemias and codes for a protein with abnormal nuclear localization that confers drug sensitivity.

Core-binding factor (CBF) leukemias are characterized by a high degree of sensitivity to high-dose cytarabine (ARA-C) treatment and by a relatively favorable prognosis compared with most other forms of adult acute myeloid leukemia (AML). The molecular basis of the response to chemotherapy is still being analyzed. The proteinase 3 (PR3) gene codes for a serine protease with a broad spectrum of proteolytic activity. PR3 is involved in the control of proliferation of myeloid leukemia cells, and when it is abnormally expressed, it confers factor-independent growth to hematopoietic cells. In this study, we analyzed the expression levels of PR3 in 113 AML patients. PR3 is highly expressed in AML, mainly in CBF leukemias in which PR3 is not only expressed, but also abnormally localized within the nuclear compartment. Nuclear PR3 results in cleavage of nuclear factor (NF)-kappaB p65 into an inactive p56 subunit lacking any transcriptional activity. The nuclear localization of PR3 is responsible for increased proliferation, apoptosis arrest and increased sensitivity to high-dose ARA-C. This study provides a new molecular mechanism that is responsible for NF-kappaB inactivation and increased sensitivity to chemotherapy in CBF leukemias.Leukemia advance online publication, 14 January 2010; doi:10.1038/leu.2009.207.

Carotid intimal-media thickness and endothelial function in young patients with history of myocardial infarction.

AIM: The aim of the study was to evaluate the prevalence of carotid atherosclerosis and endothelial dysfunction in 45 young patients (38 mens and 7 females) with myocardial infarction (MI), age 29-45, mean age 42+/-3 years, to verify its possible role as a marker of coronary atherosclerosis. METHODS: Vascular echography was performed to verify the presence of carotid atherosclerosis and/or endothelial dysfunction in 45 young patients with MI and in 45 healthy control subjects well matched for age and sex. RESULTS: We observed a normal intima media thickness (IMT) only in 30% of patients with juvenile myocardial infarction (JMI) compared with 66% in the control group (P<0.0001) and 34% of patients showed an increased IMT compared with 24% of healthy subjects (P<0.0001). Compared with control subjects, patients with JMI had lower flow-mediated reactivity of the brachial arteries (P<0.05). There was a negative linear relationship between flow-mediated dilation and IMT (P<0.001). The severity of coronary artery disease (CAD) was correlated with increased IMT and with a lower flow-mediated dilation. Finally, multiple regression analysis, demonstrated that both brachial-artery reactivity and carotid IMT were significantly and independently correlated with severity of CAD. CONCLUSIONS: Structural (carotid atherosclerosis) and functional changes (endothelial dysfunction) were present at an early age in the arteries of persons with history of JMI.

In-hospital stay of patient with acute coronary syndrome with or without diabetes mellitus.

AIM: Type 2 diabetes is associated with an increase in the risk of coronary heart disease, by a factor of two to four. The scientific community has suggested that all patients with diabetes could be treated as if they had a prior coronary heart disease. METHODS: A computer review of 11-year prevalence (from 1991 to 2002) of type 2 diabetes among 3242 patients admitted to the Intensive Coronary Care Unit of the Division of Cardiology of the University Hospital ''Paolo Giaccone'', Palermo, was carried out, with the diagnosis of ST elevation myocardial infarction (STEMI) (51%), non ST elevation myocardial infarction (NSTEMI) (6%) or unstable angina (UA) (43%). RESULTS: Prevalence of type 2 diabetes was 31.5% on the overall population; in particular, a higher prevalence was found among STEMI affected patients (37% of the diabetic patients). The average number of days of an in-hospital stay was of 10.4+/-3.1 for diabetic patients without complications (N.=602) and of 15.9+/-4.4 for diabetic patients with clinical complications (N.=421) as compared with non diabetic patients non complicated (N.=1821) or complicated (N.=398), with an average in-hospital stay of 7.4+/-1 and 12.8+/-3.2 days respectively; P<0.005. CONCLUSIONS: Diabetic patients with acute coronary syndrome had more clinical complications (41.1% vs 17.9%, P=0.0001) and a longer in-hospital stay period, resulting in an increased management costs, in comparison with non diabetic patients.

Clinical significance of macrophage colony stimulating factor levels in acute coronary syndrome.

AIM: The aim of this paper was to ascertain whether macrophage colony stimulating factor (MCSF) serum levels, measured during the acute phase of coronary syndromes (ACS), are useful to predict short term outcomes. METHODS: Seventy-four consecutive patients (mean age: 66+/-12), admitted to the Intensive Coronary Care Unit of Palermo University Hospital (Italy) affected by ACS were observed; 39 patients showed a non ST elevation (NSTEMI) and 35 showed a ST elevation myocardial infarction (STEMI). During the hospital stay, all patients underwent echocardiography and 84% of patients received coronary angiography. Peripheral venous blood samples were collected for the determination of serum levels of MCSF, C-reactive protein (CRP), fibrinogen, I troponin and complete lipid pattern. RESULTS: There was no significant difference in MCSF concentrations for STEMI versus NSTEMI patients (326.65+/-143.87 vs 297.15+/-110.43 pg/mL, P=NS). Higher levels of MCSF (363.00+/-147.61 vs 251.00+/-186.69, P=0.03) and CRP (1.04+/-0.40 vs 0.97+/-0.50 mg/L, P=0.03) were found in patients with a worst in hospital stay (recurrence of angina, re-infarction, death) and with a more severe coronary artery disease (330.03+/-241.51 vs 223.61+/-128.29 pg/mL, P=0.04 and 1.14+/-0.50 vs 0.60+/-0.22 mg/L, P=0.05). CONCLUSIONS: MCSF levels are useful in the prediction of short term prognosis in ACS patients.

Short term prognostic role of NT-proBNP in patients after myocardial infarction.

AIM: The clinical and prognostic role of cardiac natriuretic peptides (CNP) in patients with heart failure is well known; recently, several studies have evaluated the possibility of using CNP to evaluate their potential prognostic role in patients with acute coronary syndromes (ACS). The aim of this study was to evaluate the short term prognostic value of NT-proBNP in 70 patients admitted for ACS. METHODS: The authors studied 70 patients with ACS, evaluating, at admission, clinical-anamnestic, instrumental and laboratory characteristics including NT-proBNP plasma levels. Patients were monitored in a 6-month-follow-up to record adverse fatal events and their possible correlation with baseline characteristics. RESULTS: The incidence of adverse events during the follow-up period was 28% (10 patients). In patients with adverse events, the authors observed lower left ventricle ejection fraction (P=0.01), higher prevalence of ST elevation myocardial infarction (P=0.03) and higher NT-proBNP levels (P=0.03), compared to those without adverse events. Moreover, the logistic regression analysis underlined how ST elevation myocardial infarction (P=0.05) and higher NT-proBNP levels (P=0.05) were the only predictive variables for adverse events during the follow up period. CONCLUSIONS: This study demonstrates the short term prognostic role of NT-pro BNP in patients admitted for ACS.

Prognostic role of endothelial dysfunction and carotid intima-media thickness in patients undergoing coronary stent implantation.

AIM: Impaired endothelial function and increased carotid intima-media thickness are key events in the atherosclerotic process and predict future cardiovascular events in subjects with and without coronary artery disease. The purpose of this study was to investigate whether the vasodilator response to increased flow in the brachial artery and the presence of carotid lesions may have a prognostic significance for in-stent restenosis in patients undergoing coronary angioplasty. METHODS: The study population included 58 patients undergoing percutaneous coronary intervention (PCI) with stenting and at least 10 months of follow-up. All patients underwent ultrasound detection of brachial artery reactivity 30 days after PCI. Flow mediated dilatation (FMD) was investigated after 5 minutes of occlusion of the artery and nitroglycerin mediated dilation (NMD) was investigated after administration of sublingual nitrates. Vascular echography was performed to measure intima media thickness (IMT) of carotid arteries. At baseline we evaluated all the established traditional cardiovascular risk factors. We also subdivided our study cohort according to values of FMD in patients with FMD above and patients below the median value. RESULTS: Patients with FMD above the median value showed higher prevalence of hypertension (P=0.002), diabetes (P=0.02) and carotid IMT (P=0.006) than those below the median. Brachial FMD was inversely correlated (P=0.001) to carotid IMT. At the end of follow-up clinical events occurred in nine patients. In a multivariate analysis, including all the variables evaluated at baseline, carotid IMT (P=0.02), level of glycemia (P=0.001), a lower FMD (P=0.005) and presence of carotid plaque remained the only variables predictive of restenosis. CONCLUSIONS: Evaluation of FMD and carotid IMT may provide important prognostic information in patients undergoing PCI.

Carotid and peripheral atherosclerosis in patients who underwent primary percutaneous coronary intervention and outcome associated with multifocal atherosclerosis.

AIM: The aim of our study was to determine if patients with multifocal atherosclerosis have a worse prognosis than patients with atherosclerosis only in the coronary bed. METHODS: We studied 45 subjects admitted to intensive coronary care unit of the Division of Cardiology with the diagnosis of acute myocardial infarction (AMI). Traditional cardiovascular risk factors were investigated and laboratory analysis included measurement of plasma lipids, glycemia, fibrinogen and high-sensitivity-C-reactive protein (hs-CRP). Each patient underwent coronary-angiography as well as carotid and peripheral arterial ultrasound examination. A follow-up of 13+/-2 months was performed. RESULTS: We found that the severity of coronary atherosclerosis is significantly associated with the presence of carotid (P<0.05) and peripheral atherosclerosis (P<0.005). Markers of inflammation, hs-CRP (P<0.005) and fibrinogen (P<0.05), were significantly associated with multifocal atherosclerosis. We have shown that an increased number of coronary vessels with atherosclerotic stenosis is associated with a higher value of carotid (P<0.0001) and peripheral intima media thickness (P<0.0001). During 13 months of follow-up the incidence of fatal or non fatal events was 18%. The multivariate analysis showed that the variables independently associated with fatal and non fatal events were: male sex (P<0.001), family history of cardiovascular disease (P<0.005), hypertension (P<0.01), diabetes mellitus (P<0.05), higher levels of total cholesterol (P<0.05), smoking habit (P<0.05), and multifocal atherosclerosis (P<0.05). CONCLUSIONS: The ultrasound examination of carotid and peripheral atherosclerotic lesions may be useful in placing patients with AMI in a category of higher risk of cerebrovascular and cardiovascular events. Moreover, the precocious identification of patients at risk can suggest a more aggressive pharmacological treatment and a more accurate follow-up in order to avoid future events.

[Gastrointestinal stromal tumor (GIST): a rare case of severe gastric haemorrhage]. / Tumore stromale gastrointestinale: un raro caso di emorragia gastrica severa.

INTRODUCTION: A rare case of gastrointestinal stromal tumor (GIST) provoking a severe gastric bleeding is reported. Case report and results. The Authors report on the case of a 53-year-old male patient who presented with hematemesis, melena and severe acute anemia (Hb: 6 g/dl). EGDS disclosed a protruding lesion centrally ulcerated, of 7 cm in size, localized in the upper half of gastric corpus. Multiple endoscopic biopsies were negative for neoplastic changes. Because of further gastric bleeding, a total gastrectomy with "Roux-en-Y" reconstruction was performed in urgency GIST of smooth muscle was diagnosed by histological and immunohistochemical postoperative examination. DISCUSSION: Gist are neoplasms arising from connective tissue elements of gastrointestinal wall, which represent about 2% of GI-tract malignant tumor. Tumor size of 5 cm or greater, elevated mitotic count, lack of histological differentiation are significantly associated with a shorter recurrence-free survival. GIST-s are rarely cause of an inarrestable gastric bleeding. For gastroenterological surgeons it is critical to select the most suitable surgical procedure. In our case, 9 months after gastrectomy the patient is well, in spite the severe clinical background and the malignancy degree. CONCLUSION: The GIST-s have to be taken into account in the differential diagnosis of GI-tract tumors if endoscopic biopsies are negative for malignancy. In our opinion, total or partial gastrectomy fro gastric should be preferred. Moreover, a close follow up is recommended.

[Klippel-Trenaunay syndrome: clinical and surgical aspects]. / La sindrome di Klippel-Trenaunay. Aspetti clinici e chirurgici.

The Klippel-Trenaunay syndrome is a rare syndrome of uncertain etiology. The characteristic elements are flat angiomatosis, hypertrophy of soft tissue and bone tissue and alterations of the venous system, with the exclusion of hemodynamically significant arteriovenous fistulae. The authors report a clinical case and review the international literature. Treatment is conservative in the majority of cases; surgery is reserved for patients with disabling morphological and functional alterations.

[Leiomyosarcoma of the mesentery. A case report and clinico-diagnostic considerations]. / Il leiomiosarcoma del mesentere. Descrizione di un caso e considerazioni clinico-diagnostiche.

Leiomyosarcoma of the mesentery is a rare neoplasm. The clinical manifestation is non-specific and preoperative diagnosis is often late. Mitotic activity shows no significant correlation with the biological behavior of neoplasm. The surgical excision is the therapy of choice but local recurrence is high. The authors report a case of leiomyosarcoma which was treated by surgical removal and underline the diagnostic difficulties.

[Colon volvulus]. / Volvolo del colon.

The authors report a clinical case and review the international literature. After an analysis of the incidence and the predisposing factors causing this disorder, they focus attention on the question of therapy. In forms with vital loop non-surgical derotation must be attempted. In the event of the failure of non-invasive treatment and to prevent recidivation these forms are treated surgically, also using videolaparoscopy. In forms with non-vital loop, surgery consists of the section of the necrotic segment and preparation of anastomosis which may be immediate or deferred depending on general and local conditions.

[Biliary ileus. Case report and therapeutic considerations]. / Ileo biliare. Presentazione di un caso e considerazioni clinico-terapeutiche.

The authors, after having described, a case of biliary ileus, analyse the principal pathogenetic aspects of the disease, and underline the diagnostic and therapeutic difficulties. They believe that the simple enterolithotomy represents, initially, the best therapy, in particular with patients in poor clinical conditions.

Left ventricular filling rate at rest and during exercise in patients with previous myocardial infarction.

The purpose of this investigation was to define, with radionuclide technique, the variation on left ventricular filling rate in patients with coronary artery disease, and to determine the effects of dynamic exercise on this variation. The study was carried out on 91 subjects, 46 patients with anterior and 30 with inferior previous transmural myocardial infarction; 15 healthy subjects were studied as control group. All the patients underwent coronary angiography and left ventriculography. From the left ventricular time activity curve we considered the diastolic parameters of the peak filling rate (PFR). We considered also the relative end-diastolic volume (rEDV) and the relative end-systolic volume (rESV). These parameters were determined at rest and at the fifth minute of a symptom limited dynamic exercise taken in the supine position, on an ergometric bicycle. In normal subjects rest mean PFR values is 3.08 +/- 0.51 edv/s, during exercise occurs a physiological increase and mean PFR values becomes 5.48 +/- 1 edv/s. The patients with previous myocardial infarction show a PFR significantly smaller than in normal subjects. Abnormal PFR indices during exercise are present in a large number of these patients and the higher anomalies of PFR during exercise were found among patients with anterior myocardial infarction. In these patients we found an increase of rESV during exercise. In conclusion myocardial infarction induces significant alterations of the PFR; physical exercise reveals PFR alterations not exhibited at rest and rESV increase during exercise could be responsible for the PFR alteration observed.

Prognostic significance of the evolution of left ventricular ejection fraction in patients with acute myocardial infarction not treated with thrombolytic therapy.

Several controlled trials on the thrombolytic treatment of acute myocardial infarction (AMI) have failed to demonstrate that thrombolysis has a simultaneous positive effect on left ventricular function and survival. One explanation may be that spontaneous changes in left ventricular function occurred during the progression of AMI in control patients. The aim of this study was to evaluate the spontaneous evolution of left ventricular ejection fraction (LVEF) and its prognostic influence on early (1 month) and late (1 year) mortality in patients with AMI. We studied 216 patients admitted to our CCU within 24 h of the onset of symptoms. LVEF was determined by radionuclide ventriculography on admission (RNV1) and at the end of the necrotic phase (RNV2). Fourteen patients died before RNV2. On the basis of LVEF values at RNV1, the remaining 202 patients were divided into two groups: those with a normal LVEF (> or = 55%), and those with an abnormal LVEF (< 55%). Among patients with a normal LVEF at RNV1 (64 patients), a significant increase (> 12%) in LVEF at RNV2 was observed in 12.5%, a significant decrease (> 12%) in 12.5% and no change at all in 75%. All of these patients survived, regardless of the evolution of LVEF. In patients with an abnormal LVEF at RNV1 (138) a significant increase (> 5%) in LVEF at RNV2 was observed in 72.5%, a significant decrease (> 5%) in 6.5% and no change at all in 21%.(ABSTRACT TRUNCATED AT 250 WORDS)

[The development of a computer model in the quantitative assessment of thallium-201 myocardial scintigraphy]. / Sviluppo di un modello informatico nella valutazione quantitativa della scintigrafia miocardica al tallio-201.

Thallium-201 scintigraphy is a widely used noninvasive procedure for the detection and prognostic assessment of patients with suspected or proven coronary artery disease. Thallium uptake can be evaluated by a visual analysis or by a quantitative interpretation. Quantitative scintigraphy enhances disease detection in individual coronary arteries, provides a more precise estimate of the amount of ischemic myocardium, distinguishing scar from hypoperfused tissue. Due to the great deal of data, analysis, interpretation and comparison of thallium uptake can be very complex. We designed a computer-based system for the interpretation of quantitative thallium-201 scintigraphy data uptake. We used a database (DataEase 4.2-DataEase Italia). Our software has the following functions: data storage; calculation; conversion of numerical data into different definitions classifying myocardial perfusion; uptake data comparison; automatic conclusion; comparison of different scintigrams for the same patient. Our software is made up by 4 sections: numeric analysis, descriptive analysis, automatic conclusion, clinical remarks. We introduced in the computer system appropriate information, "logical paths", that use the "IF ... THEN" rules. The software executes these rules in order to analyze the myocardial regions in the 3 phases of scintigraphic analysis (stress, redistribution, re-injection), in the 3 projections (LAO 45 degrees, LAT,ANT), considering our uptake cutoff, obtaining, finally, the automatic conclusions. For these reasons, our computer-based system could be considered a real "expert system".