An Uncommon Presentation of Renal Angiomyolipoma: A Case Report.

Renal angiomyolipoma (AML) is a rare benign tumor of the kidney that can occur as a sporadic lesion or a part of tuberous sclerosis. A 77-year-old female patient with a history of hypertension, hyperlipidemia, and an unclear history of left nephrectomy in 1999 presented with progressive shortness of breath and palpitations. Her vital signs showed elevated blood pressure, and the examination was benign and non-focal. A work-up showed multiple lesions in her lungs and right kidney, representing lymphangioleiomyomatosis. The patient was diagnosed with tuberous sclerosis and was followed up by pulmonology and nephrology. She underwent embolization of the renal AML, after which her blood pressure (BP) was more controlled, and she reported feeling well and symptom-free. Renal AML, as a part of tuberous sclerosis, is a rare cause of secondary hypertension. Embolization of AML is effective in controlling BP.

Effect of atorvastatin on lipogenic, inflammatory and thrombogenic markers in women with the metabolic syndrome.

BACKGROUND AND AIM: Specific drug therapy to target the underlying proinflammatory and prothrombotic state in patients with metabolic syndrome (MS) is lacking. We sought to study the effect of high-intensity atorvastatin on markers of lipogenesis, inflammation and thrombogenesis, in women with MS in the absence of cardiovascular disease or diabetes. METHODS AND RESULTS: This randomized double-blinded controlled trial included 88 women with MS (according to National Cholesterol Education Panel Adult Treatment Panel III criteria) and low atherosclerotic cardiovascular risk. Participants were randomized to receive atorvastatin 80 mg or matching placebo. Thrombogenic, lipogenic and inflammatory markers were collected at the time of enrollment, after a 6-week dietary run-in phase (time of randomization), and at 6- and 12-weeks after randomization. At 6 weeks post-randomization, there was significant reduction in total cholesterol, low density lipoprotein cholesterol, triglycerides, apolipoprotein-B (Apo-B) and Apo-B/Apo-A1 ratio in the atorvastatin arm compared to placebo. This difference persisted at 12-weeks post randomization. There was no significant difference in fasting blood glucose, high-density lipoprotein cholesterol, high sensitivity C-reactive protein, serum leptin, Apo-A1, intercellular adhesion molecule 1 and platelet activity. A significant increase in vascular adhesion molecule 1 at 6 and 12 weeks was seen within the atorvastatin arm. No difference was observed in blood pressure and waist circumference. CONCLUSIONS: In conclusion, high-intensity atorvastatin has an early and significant impact on lipoproteins and apolipoproteins but did not lower inflammatory, thrombogenic or biomarkers of platelet activity and aggregation in women with MS. The use of statins for primary prevention in these patients should be further explored.

Syncope in Young Women: Broadening the Differential Diagnosis.

Syncope is defined as a sudden transient loss of consciousness (TLOC) with concomitant loss of postural tone followed by spontaneous recovery. It is a subset of a broader class of medical conditions, including postural orthostatic tachycardia syndrome (POTS), orthostatic hypotension, and neurally mediated syncope (NMS), that may result in TLOC. The overlap of these clinical conditions leads to confusion regarding syncope classification that can hinder evaluation strategies, and pose challenges for diagnosis and treatment, particularly in young women. In this article, we review POTS, orthostatic hypotension, and NMS with an emphasis on NMS. These diverse orthostatic clinical entities may be associated with syncope and are frequently observed in young, healthy women. The importance of considering NMS as a diagnosis of exclusion cannot be overstated. We report a series of three young, otherwise healthy women, initially diagnosed with NMS, whose clinical course evolved over time into more sinister diagnoses that were overlooked and associated with devastating clinical outcomes. These cases highlight the importance of maintaining a broad differential diagnosis when considering the diagnosis of NMS. Each case synopsis provides key clinical features that must be considered to avoid overlooking more serious clinical conditions.

Clinical Presentation and Outcomes by Sex in Arrhythmogenic Right Ventricular Cardiomyopathy: Findings from the North American ARVC Registry.

BACKGROUND: Sex differences in clinical presentation and outcomes of hereditary arrhythmias are commonly reported. We aimed to compare clinical presentation and outcomes in men and women with arrhythmogenic right ventricular cardiomyopathy (ARVC) enrolled in the North American ARVC Registry. METHODS: A total of 125 ARVC probands (55 females, mean age 38 ± 12; 70 males, mean age 41 ± 15) diagnosed, as either "affected" or "borderline" were included. Baseline clinical characteristics and time-dependent outcomes including syncope, ventricular tachycardia (VT), fast VT (>240 bpm), ventricular fibrillation (VF), and death were compared between males and females. RESULTS: The percentage of ARVC subjects diagnosed as "affected" (84% vs. 89%; P = 0.424) or "borderline" (16% vs. 11%; P = 0.424) was similar between females and males. Among the baseline characteristics, inverted T-waves in V2 trended to be more common in women (P = 0.09), whereas abnormal signal-averaged ECGs (SAECGs; P < 0.001) and inducible VT/VF (P = 0.026) were more frequent in men. During a mean follow-up of 37 ± 20 months, the probability of ICD-recorded VT/VF or death was not significantly different between men and women (P = 0.456). However, there was a trend toward lower risk of fast VT/VF or death in women compared to men (hazard ratio 0.41, 95% CI 0.151-1.113, P = 0.066). Abnormal SAECG and evidence of intramyocardial fat by cardiac MRI was associated with adverse outcomes in men (P = 0.006 and 0.02 respectively). CONCLUSION: In the North American ARVC Registry, we found similar frequency of "affected" and "borderline" subjects between men and women. Sex-related differences were observed in baseline ECG, SAECG, Holter-recorded ventricular arrhythmias, and VT inducibility. Men showed a trend toward greater risk of fast VT than women.

Predictors of mortality in patients hospitalized for congestive heart failure with left ventricular ejection fraction ≥ 40.

BACKGROUND: There are limited data regarding the predictors of mortality in patients with acute congestive heart failure (CHF) and left ventricular ejection fraction (LVEF) ≥ 40%. METHODS: We evaluated clinical characteristics, mortality and prognostic factors in a sample of consecutive patients hospitalized for CHF with LVEF ≥ 40%. Multivariable Cox regression models were developed to predict mortality using baseline clinical characteristics and echocardiographic variables. RESULTS: The study population consisted of 191 patients, mean age 70 ± 14.6 years (60% female) with average follow-up of 4.0 ± 2.8 years. Cumulative 5-year mortality was 58% in the entire population and it was 59% in men and 57% in women (p = 0.411). In multivariable analyses, predictors of mortality were the following: blood urea nitrogen (BUN) > 25 mg/dL (HR = 1.77; p = 0.002); absence of hypertension (HR = 1.58; p = 0.032), left ventricular end diastolic dimension (LVEDD) ≤ 4.1 cm (HR = 1.73; p = 0.011) and LVEF ≤ 45% (HR = 1.69; p = 0.027). CONCLUSIONS: Patients hospitalized for heart failure with LVEF ≥ 40% have very high mortality. Absence of hypertension, elevated BUN and lower LVEF ≤ 45% indicate increased risk of short- and long-term mortality. Lower LVEDD is an independent predictor of mortality in heart failure patients with LVEF ≥ 40%.

Improvement in glycemic control after left ventricular assist device implantation in advanced heart failure patients with diabetes mellitus.

Left ventricular assist devices (LVADs) have been shown to improve outcomes in advanced heart failure (HF). We hypothesized that LVADs improve glycemic control in HF patients with diabetes mellitus (DM). During a 6 year time period, 202 patients underwent mechanical circulatory support. Of these, 50 patients with DM were included. Data were collected within 2 months before LVAD implantation and at 5.6 ± 1.1 months post-LVAD implant. There was no significant difference in body mass index, hemoglobin, hematocrit, and renal function pre-LVAD and post-LVAD. Fasting blood glucose improved from 136 ± 35 to 108 ± 29 mg/dl post-LVAD (p < 0.001). In 18 patients taking insulin only, daily insulin dose decreased from 43 ± 37 to 29 ± 24 units (p = 0.02). Of the 17 patients taking oral hypoglycemic agents, four did not require antidiabetic medications, six continued the same dose, two required higher doses, and five patients were switched to insulin post-LVAD. In a subset of 22 patients with available data, hemoglobin A1c improved significantly post-LVAD (p < 0.001). C-reactive protein in a subset of 18 patients decreased post-LVAD (p = 0.059). In conclusion, diabetic patients with advanced HF appear to have significant improvement in glycemic control and require less antidiabetic medications post-LVAD.

Corrected QT interval as a predictor of mortality in elderly patients with syncope.

BACKGROUND: Prolonged corrected QT interval (QTc) holds independent prognostic importance in predicting mortality in patients with coronary artery disease, diabetes mellitus and congestive heart failure. However, its association with all cause or cardiac mortality in the general population remains unclear. We evaluated the relationship between prolonged QTc and total mortality among patients with syncope. METHODS: This was a retrospective study of 348 patients presenting to the emergency department with syncope of any etiology over a period of one year. All patients with atrial fibrillation, left bundle branch block and cardiac devices (pacemaker/defibrillator) were excluded. Prolonged QTc interval was defined as QTc interval ≥ 440 ms. The primary end point for this study was total mortality in patients presenting with syncope. RESULTS: There were 58 (16%) deaths in this population during a mean follow-up of 30 months. Patients with prolonged QTc interval had significantly higher mortality when compared to those with normal QTc interval (22% vs 11%; p = 0.004). This significance was not retained after adjustment for covariates in the Cox regression model, where we found that age ≥ 65 years (hazard ratio [HR] 7.9; 95% confidence interval [CI] 1.9-32.9; p = 0.004) and QTc interval ≥ 500 ms (HR 3.5; 95% CI 1.56-8.12; p = 0.002) were predictors of increased mortality among patients with syncope. CONCLUSIONS: In elderly patients presenting to the emergency department with syncope, QTc interval ≥ 500 ms helps identify patients at higher risk of adverse outcomes.

Iodoxolone-based hypervalent iodine reagents.

The fast access to simple (Z)-3-iodo acrylic acid derivatives which can be easily oxidized to the corresponding hypervalent iodine(III) reagents is described. They can be used for various reactions with superior or similar reactivity as conventional hypervalent iodine(III) reagents.