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Objective: The aim of this case report was to highlight the importance of ruling out pheochromocytoma in a patient with Von Hippel-Lindau disease (VHL) and cardiovascular manifestations. Clinical Presentation and Intervention: A 22-year-old woman with type IIb VHL presented with signs and symptoms of acute decompensated heart failure. Transthoracic echocardiography showed a dilated left ventricle with severely depressed ejection fraction, confirmed by MRI. Urinary catecholamine and metanephrine tests had elevated levels and an abdominal MRI showed the presence of two cystic masses at the left hypochondrium. Surgical resection of both masses was performed, confirming the diagnosis of pheochromocytoma and clear cell renal carcinoma on histology. Six-month echocardiography showed a left ventricle with normal diameters and preserved ejection fraction. Genetic analysis revealed a germline mutation (exon 3 deletion of VHL). As there was no family history of VHL, it was determined to be a de novo mutation. Conclusion: This case report showed an atypical manifestation in a patient with VHL and underlines the importance of screening for pheochromocytoma in such patients. [ABSTRACT FROM AUTHOR]

Abstract: Aims: We analyzed the usefulness of quantitative intravenous myocardial contrast echocardiography to study microvasculature perfusion after infarction in comparison with intracoronary myocardial contrast echocardiography. Methods and results: Thirty-two patients with a first ST elevation myocardial infarction, single-vessel disease and an open artery (TIMI 3) were studied before discharge. Myocardial perfusion in the risk area was quantified with intracoronary and intravenous myocardial contrast echocardiography. Perfusion was normal (intracoronary contrast echocardiography normalized videointensity >0.75) in 78 out of 97 dysfunctional segments (80%). Sensitivity and specificity of intravenous contrast echocardiography to predict normal perfusion were 87% and 63% for ‘first-pass myocardial blood flow’ (upslope of contrast arrival×peak intensity after intravenous bolus injection of contrast) and 91% and 89% for end-systolic single-triggered images captured every 6 cycles, respectively. In an analysis per patients, normal perfusion (0 or 1 hypoperfused segments with intracoronary contrast echocardiography) was observed in 22 cases (69%). End-systolic single-triggered images showed a strong correlation with intracoronary contrast echocardiography (R 2 =0.82, p =0.0001). Conclusions: Intravenous contrast echocardiography is a useful technique to analyze microvasculature perfusion soon after infarction. A quantitative analysis of single-triggered images is an easy-to-obtain and reliable method to define perfusion when compared with intracoronary contrast echocardiography. [Copyright &y& Elsevier]

Introduction: In acute coronary syndromes, myocardial damage markers and acute-phase reactants predict adverse cardiac events. The aim of this study was to define the fitted prognostic value of the most widely used variables of necrosis and inflammation as well as of homocysteine. Methods and results: Troponin I, high-sensitivity C-reactive protein, fibrinogen and homocysteine were measured in 515 consecutive patients admitted to our institution for non-ST elevation acute coronary syndrome. The risk for major events (death or nonfatal myocardial infarction) through 6 months of follow-up was analysed. In the univariate analysis, all markers were related to major events (p<0.01 in all cases). In a multivariate model fitting for baseline characteristics and electrocardiographic changes, the only biomarkers related to major events were C-reactive protein >11 mg/l (2.1 [1.2–3.8] p=0.007) and troponin I >3 ng/ml (1.9 [1.1–3.4] p=0.03). Moreover, the rate of major events was significantly higher (p<0.0001) only when both C-reactive protein and troponin I were increased (31.4% vs. 9.3% if any or both markers were normal). Conclusion: In non-ST elevation acute coronary syndromes elevated levels of troponin I, C-reactive protein, fibrinogen and homocysteine are strongly related to the risk of major events. The prognostic value of troponin I and C-reactive protein is independent and additive with respect to each other. [Copyright &y& Elsevier]

Background and Objective: We aimed to delineate the sex differences in short-term (one-month) and long-term (one-year) cardiac death after an acute coronary syndrome., Patients and Method: After the publication of the new myocardial infarction definition, we prospectively analyzed 1,324 consecutive patients admitted with a diagnosis of acute coronary syndrome to a tertiary hospital. 483 (37%) of these patients had myocardial infarction with ST-elevation, 439 (33%) had myocardial infarction without ST elevation (troponin I > 1 ng/ml) and 402 (30%) had an unstable angina (troponin I < 1 ng/ml)., Results: Within 1-year of follow-up, 177 deaths (13.4%) were detected. There was a similar rate of cardiac death in female and male patients with 'non-ST elevation myocardial infarction' (one-month: 9.7% vs 7.1%, p = NS; one-year: 16.7% vs 13.2%, p = NS) and with unstable angina (one-month: 3% vs 1.9%, p = NS; one-year: 3% vs 5.6%, p = NS). Among patients with 'ST-elevation myocardial infarction' women showed a higher rate of cardiac death at one-month (21.5% vs 9.8%; p < 0.0001) and at one-year (28.9% vs 14.1%, p < 0.0001) than men. In the multivariate analysis, independent predictors of cardiac death in 'ST-elevation myocardial infarction' at one-year were age > 70 years (p < 0.0001), Killip class > 1 (p < 0.0001) and lack of reperfusion (p = 0.003) but not having a female sex., Conclusions: Patients with 'non-ST elevation acute coronary syndromes' did not display sex differences with regard to mortality. Women with 'ST-elevation myocardial infarction' had a higher mortality; however, these differences were not independently related to a female sex but to a worse baseline clinical profile and a lesser rate of reperfusion.

Background and Objective: We intended to determine whether C-reactive protein (CRP) provides independent prognostic information after a non-ST elevation acute coronary syndrome., Patients and Method: We prospectively studied 630 consecutive patients admitted with a diagnosis of non-ST elevation acute coronary syndrome. Cut-off values were: troponin I > 1 ng/ml (n = 354; 56%) and CRP > 11 mg/l (n = 273; 43%)., Results: Within a one-year follow-up period, 56 (9%) cardiac deaths, 85 (13%) myocardial infarctions (MI) and 127 (20%) first major events were detected. Patients with increased CRP showed higher rates of death at one-month (8% vs 1%), death at one-year (15% vs 4%), myocardial infarction at one-month (8% vs 4%), myocardial infarction at one-year (19% vs 9%), major events at one-month (15% vs 5%) and major events at one-year (30% vs 13%). In the multivariate analysis, once adjusted for baseline and electrocardiogram data and for myocardial damage markers, CRP was an independent predictor of death at one-month (odds ratio [OR] 4.6) and death at one-year (OR = 2.7), major events at one-month (OR = 1.8) and major events at one-year (OR = 1.8). Troponin I predicted MI at one-month (OR = 2.5) and MI at one-year (OR = 2.2)., Conclusions: CRP provided independent information to predict major events in non-ST elevation acute coronary syndromes. Troponin I was a more powerful predictor of MI than PCR. The analysis of CRP and myocardial damage markers in the short-term and long-term risk stratification seems worthy.

Objectives: We analyzed whether the study of systolic function by echocardiography adds independent information to that afforded by biochemical markers in predicting six-month major events after non-ST elevation acute coronary syndrome., Patients and Method: Baseline clinical and electrocardiographic data as well as serum concentrations of troponin, myoglobin, C-reactive protein, fibrinogen and homocysteine were recorded prospectively in 515 consecutive patients admitted because of non-ST elevation acute coronary syndrome. Ejection fraction (echocardiogram) was determined in 248 cases (48%). Predictors of cardiac death or infarction within the following six months were analyzed., Results: In the 248 patients in whom ejection fraction was analyzed, 38 major events were recorded. Increased biochemical markers were related to major events (p < 0.05 for all markers). In the final multivariate model, which included clinical, electrocardiographic, serological and systolic function data, ejection fraction was the most powerful predictor of six-month major events: age > 70 years (p = 0,04), insulin-dependent diabetes (p = 0.03), C-reactive protein > 11 mg/l (p = 0.004) and ejection fraction < 50% (p < 0.0001); C-statistic = 0.80., Conclusions: Apart from the clinical and biochemical profile, analysis of systolic function is advisable for correct risk stratification of patients with non-ST elevation acute coronary syndrome.