DRESS syndrome-associated acute necrotizing eosinophilic myocarditis with giant cells.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is an uncommon drug hypersensitivity reaction caused by a wide variety of agents. It has a characteristic latent period between 2 and 8 weeks from the onset of drug ingestion followed by a slow resolution with the potential for relapse. Despite being a potentially fatal disease, little is understood about its variable clinical presentation and why it can present long after removal of the offending drug. Visceral organ involvement typically occurs, but rarely results in clinically manifested cardiac injury. In its most aggressive form, acute necrotizing eosinophilic myocarditis (ANEM) can present with DRESS. We present an unusual case of DRESS syndrome due to lamotrigine with confirmed ANEM showing both eosinophils and rare giant cell infiltrates on endomyocardial biopsy. Although lamotrigine has been reported to cause DRESS, it has not been previously implicated as a cause of ANEM.

Is 30-Day Mortality after Admission for Heart Failure an Appropriate Metric for Quality?

BACKGROUND: The Centers for Medicare and Medicaid Services (CMS) model for publicly reporting national 30-day-risk-adjusted mortality rates for patients admitted with heart failure fails to include clinical variables known to impact total mortality or take into consideration the culture of end-of-life care. We sought to determine if those variables were related to the 30-day mortality of heart failure patients at Geisinger Medical Center. METHODS: Electronic records were searched for patients with a diagnosis of heart failure who died from any cause during hospitalization or within 30 days of admission. RESULTS: There were 646 heart-failure-related admissions among 530 patients (1.2 admissions/patient). Sixty-seven of the 530 (13%) patients died: 35 (52%) died during their hospitalization and 32 (48%) died after discharge but within 30 days of admission; of these, 27 (40%) had been transferred in for higher-acuity care. Fifty-one (76%) died from heart failure, and 16 (24%) from other causes. Fifty-five (82%) patients were classified as American Heart Association Stage D, 58 (87%) as New York Heart Association Class IV, and 30 (45%) had right-ventricular systolic dysfunction. None of the 32 patients who died after discharge met recommendations for beta-blockers. Criteria for prescribing angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and mineralocorticoid receptor blockers were not met by 33 of the 34 patients (97%) with heart failure with reduced ejection fraction not on one of those drugs. Fifty-seven patients (85%) had a do-not-resuscitate (DNR) status. CONCLUSION: A majority of heart failure-related mortality was among patients who opted for a DNR status with end-stage heart failure, limiting the appropriateness of administering evidence-based therapies. No care gaps were identified that contributed to mortality at our institution. The CMS 30-day model fails to take important variables into consideration.

Agreement between heart failure patients and providers in assessing New York Heart Association functional class.

BACKGROUND: Uncertainty persists regarding whether patient assessment of New York Heart Association (NYHA) functional classification should be preferred over provider assessment among patients with heart failure (HF). OBJECTIVES: To compare patient against provider NYHA assessments, and both to distance walked on a 6-minute walk test (6MWT) among patients with HF. METHODS: In this prospective study, we enrolled 101 HF patients who self-assessed NYHA classification. Health care providers who were blinded to patient ratings of NYHA also rated NYHA. Patients completed a 6MWT according to a standardized protocol. We used Spearman coefficients (rs) to evaluate the correlations between variables. RESULTS: Patient- and provider-determined NYHA class were poorly correlated, but the relationship was statistically significant (rs = 0.40, p < 0.001). Patients consistently reported better NYHA class (class I: 72% vs 15%) than providers. Provider-determined NYHA had a stronger correlation with 6MWT distance (rs = -0.36, p < 0.001 vs. rs = -0.22, p = 0.03). Providers assigned a worse class to older patients who had comorbidity; patients with dyspnea and longer HF duration assigned themselves a worse class. CONCLUSION: Patients and providers exhibited poor agreement in NYHA assignment.

Risk Stratification Model for 30-Day Heart Failure Readmission in a Multiethnic South East Asian Community.

There are limited accurate 30-day heart failure (HF) readmission risk scores using readily available clinical patient information on a well-defined HF cohort. We analyzed 1,475 admissions discharged from our hospital with a primary diagnosis of HF between 2010 and 2012. HF diagnostic criteria included satisfying clinical Framingham criteria, elevated serum N-terminal pro-natriuretic peptide, and evidence of cardiac dysfunction on transthoracic echocardiography. The patients were randomly divided into 2 groups; 60% were used as the derivation cohort and 40% as the validation cohort. Bivariate analysis and logistic regression were used to develop the model. Weighted risk scores were derived from the odds ratio of the logistic regression model. Total risk scores were computed by simple summation for each patient. The 7 significant independent predictors of 30-day HF readmission used to derive the risk scoring tool were the number of previous HF-related admission in the preceding 1 year, index admission length of stay, serum creatinine level, electrocardiograph QRS duration, serum N-terminal pro-natriuretic peptide level, number of Medical Social Service needs, and ß blocker prescription on discharge. The area under the curve was 0.76. Sensitivity and specificity were 78.3% and 60.7%, respectively. The positive predictive value and negative predictive value were 18.9% and 96%, respectively. The actual observed and predicted 30-day heart failure readmission rates matched. In conclusion, we have developed the first 30-day HF readmission risk score, with good discriminatory ability, for an urban multiethnic Asian heart failure cohort with stringent diagnostic criteria. It consists of 7 easily obtained variables.

Anomalous coronary circulation: left anterior descending and left circumflex coronary arteries arising from the right sinus of valsalva.

Approximately 1% of adults who undergo cardiac catheterization have coronary anomalies. Patients may present with chest pain, arrhythmias, presyncope, and sometimes sudden cardiac death. Multidetector computed tomography (MDCT) is an excellent tool for identifying coronary artery anomalies and defining their course and relationship to the great vessels and surrounding structures; its value is incremental to conventional angiography. We present a rare case of a coronary anomaly involving three separate ostia at the right sinus of Valsalva for the left and right coronary vessels.

Isolated subacute effusive and nonconstrictive epicarditis masquerading as a right atrial mass.

Epicarditis (visceral pericardial inflammation) is a very unique and rare diagnosis. It is almost always associated with parietal pericardial involvement and may occur in medical conditions such as viral, bacterial (mycobacterial) infections and uremia or postoperatively in the setting of cardiac surgery. Frequently, no etiology is found. Most cases are associated with constrictive physiology, and patients present with symptoms and signs of right-sided heart failure. Effusive epicarditis is often present, and the clinical features may easily be confused with those of pericardial effusion with tamponade. We report a unique case of isolated subacute effusive and nonconstrictive epicarditis mimicking a right atrial mass in a 72-year-old patient who was diagnosed with nonmetastatic gastric adenocarcinoma. Our case is unique for several reasons: inflammation was limited to the epicardium (very few cases have been described to date); the patient was asymptomatic, with no clinical or echocardiographic evidence of constriction (this represents a novel finding, explained in part by the more limited extent of inflammation, with no significant fibrotic component and no parietal pericardial involvement); and this is the first report of epicarditis occurring in association with a malignancy, which we hypothesize may represent an inflammatory paraneoplastic process.

CT angiography for evaluation of coronary artery disease in inner-city outpatients: an initial prospective comparison with stress myocardial perfusion imaging.

To evaluate the utility of CT coronary angiography (CTA) for demonstrating coronary artery disease in inner-city outpatients, we prospectively compared CTA with stress SPECT myocardial perfusion imaging in an ethnically diverse, gender balanced population. All patients gave written informed consent for this IRB approved, HIPAA compliant study. Sixty-one patients completed both CTA and SPECT. About 67% were ethnic minorities, 51% were women. A stenosis of >or=70% on CTA was considered positive. Results were compared with perfusion defects on SPECT and correlated with clinical endpoints (hospital admissions, cardiovascular events, coronary interventions and deaths). CTA and SPECT data were compared with results of coronary angiography, when performed. There was moderate global agreement of 79% (48/61) between CTA and SPECT, kappa = 0.483 (SE +/- 0.13, P = 0.0001). With SPECT as the reference standard, CTA had sensitivity of 73% (11/15), specificity of 80% (37/46), negative predictive value of 90% (37/41) and positive predictive value of 55% (11/20). Positive SPECT was associated with positive CTA, (P < 0.0001, OR = 22). Eleven (18%) underwent subsequent cardiac catheterization, which was positive in 91% (10/11). CTA and SPECT had positive predictive values of 90 and 83% compared with catheterization. This study lends preliminary evidence to support to the utility of CTA as an alternative modality for the evaluation of CAD in an ethnically diverse, gender balanced inner-city outpatient population. Similar to more homogenous groups, CTA had a high negative predictive value and demonstrated disease occult to SPECT. Further study is necessary to evaluate the impact of CTA on patient outcomes.

Demonstration of left ventricular outflow tract eccentricity by 64-slice multi-detector CT.

BACKGROUND: Newer three-dimensional imaging technologies provide insight into cardiac shape and geometry from views previously unobtainable. Standard formulae like the continuity equation (CE) that rely on inherent assumptions about left ventricular outflow tract (LVOT) shape may need to be revisited. In the CE, small changes in LVOT diameter may significantly change calculated aortic valve area (AVA). Using 64-slice Multi-detector CT (MDCT), we performed LVOT planimetry to obviate the need for any geometric assumptions. METHODS: 64-slice MDCT was performed in 30 consecutive patients. The diameter-derived LVOT area (ALVOTdiam) was calculated from a view analogous to the 2D echo parasternal long axis. Direct planimetry of the LVOT (ALVOTplan) was performed just beneath the aortic valve in a plane perpendicular to the LVOT long axis. Further, assuming an ellipsoid outflow tract shape, LVOT area (ALVOTellip) was calculated using piab from the long and short diameters of the planimetered LVOT view. Eccentricity index (EI) was estimated by subtracting the ratio of shortest and longest LVOT diameters from one. RESULTS: ALVOTdiam always measured smaller than ALVOTplan (mean 3.7 +/- 1.2 cm2 vs. 4.1 +/- 1.3 cm2, respectively). The median EI was 0.18 (95% CI = 0.16-0.2; P = 0.0001). ALVOTellip more closely agreed with ALVOTplan (correlation = 0.96; P < 0.0001) than did ALVOTdiam (correlation = 0.87; P < 0.0001). CONCLUSION: Using MDCT, the LVOT was shown to be elliptical in most patients. Applying the CE which assumes roundness of the LVOT consistently underestimated the LVOT area which may affect estimated AVA. Planimetry of the LVOT utilizing three-dimensional imaging modalities such as 3-D echocardiography, MRI, or MDCT may render a more precise AVA.

Ratio of left atrial to left ventricular size: an anatomical marker of the diastolic left ventricular pressure-volume relationship.

BACKGROUND: Classification of diastolic heart function is best defined by the degree of leftward and upward shift of the diastolic pressure-volume relationship (DPVR). Direct measurement of DPVR, however, requires invasive techniques. Increased left atrial (LA) size is a marker of left ventricular (LV) diastolic hypertension, and so, the LA/LV diameter ratio has the potential to mark the degree of upward and leftward shift in the LV-DPVR. We thus investigated the association of this novel marker with exposures known to induce diastolic dysfunction and with clinical evidence of diastolic dysfunction. METHODS AND RESULTS: Reports from 7,803 patients undergoing maximal exercise stress echocardiography were reviewed. Increased LA/LV diameter ratio predicted diminished exercise capacity (P < 0.001) in a multivariate regression analysis. Increased LA and decreased LV diameters were each independently associated with exercise capacity (P < 0.001, both). Increased LA/LV diameter ratio was associated with hypertension (P = 0.001), diabetes (P = 0.03) and with increased severity of LV hypertrophy (P< 0.001). Those with LA/LV diameter ratio > or = 1.0 were more likely to use loop diuretics, odds ratio = 2.5 [95% CI, 1.4, 4.5], compared to those with lower ratio values. CONCLUSIONS: Increased LA/LV diameter ratio was observed in subjects with hypertension, diabetes and LV hypertrophy. Increased ratio predicted worse exercise capacity and was associated with more frequent loop diuretic use. These data are consistent with the hypothesis that this ratio is a noninvasive marker of the LV-DPVR.

Demonstration of left ventricular outflow tract eccentricity by real time 3D echocardiography: implications for the determination of aortic valve area.

BACKGROUND: Determination of the left ventricular outflow tract cross-sectional area (ALVOT) is necessary for calculating aortic valve area (AVA) by echocardiography using the continuity equation (CE). In the commonly applied form of CE, pir(2) is used to estimate ALVOT utilizing the assumptions that LVOT is round and the parasternal long axis (PLAX) plane bisects LVOT. Imaging LVOT using real time 3D echocardiography (RT3DE) eliminates the need for these assumptions. We tested the hypothesis that LVOT is round based on a formula for eccentricity. METHODS AND RESULTS: In 53 patients, 2D echocardiography (2DE) and RT3DE were acquired. ALVOT was calculated by 2DE using pir(2) (ALVOT-2D). Using RT3DE, ALVOT planimetry was performed immediately beneath the aortic valve (ALVOT-3Dplan). Eccentricity Index (EI) was calculated using the shortest and longest LVOT diameters. The long axis was measured to be larger by 0.53 cm +/- 0.36 (P < 0.005). The median EI was 0.20 (0.00-0.54), indicating that half the subjects had at least a 20% difference between the major and minor diameters. ALVOT-3Dplan was larger than ALVOT-2D (3.73 +/- 0.95 cm(2) vs. 3.18 +/- 0.73 cm(2); P < 0.001) by paired analysis. Using the equation of an ellipse (piab), ALVOT-3Dellip was 3.57 +/- 0.95 resulting in improved agreement with ALVOT-3Dplan. CONCLUSIONS: In our small patient sample with normal aortic valves, we showed the LVOT shape is usually not round and frequently, elliptical. Incorrectly assuming a round LVOT underestimated the ALVOT-3Dplan and consequently the AVA by 15%. Investigating the LVOT in aortic stenosis is warranted to evaluate whether RT3DE may improve measurement of AVA.

Pseudodyskinesis--a novel mechanism for abnormal motion of the heart's diaphragmatic wall.

We report a case of pseudodyskinesis, where there is dyssynchronous contraction of the heart's diaphragmatic wall despite normal wall thickening. This finding has previously been reported in a small group of patients with liver disease, and has been attributed to elevation of the diaphragm as a result of hepatomegaly and ascites. Our case demonstrates similar findings in a patient without liver disease, in whom the diaphragm was elevated secondary to volume loss in the chest. Our case supports the assertion that diaphragmatic elevation, regardless of cause, is indeed responsible for this probably common echocardiographic finding.

Biventricular mural vegetations in a patient without valvular pathology.

We present the case of a 40 year-old man with biventricular nonvalvular vegetations presenting with acute onset of unilateral hearing loss and headache as a result of septic emboli. The medical literature involving the rare diagnosis of mural vegetation is reviewed and unusual features of this case are discussed.

Clonidine-induced delirium.

Clonidine-induced delirium has rarely been reported. To the best of our knowledge, there are six related case reports in the literature. We describe one such case here and review the six previously published cases. Clonidine may induce a variety of psychological side effects ranging from depression to acute hallucination and delirium. However, there are no clearly identifiable risk factors for the development of severe psychological side effects, including dose of medication, duration of treatment, and predisposing mental illness. Treatment for clonidine-induced delirium involves cessation of the medication and patient observation. Given the large clinical burden of hypertension and the not uncommon requirement for polypharmacy to achieve blood pressure goals, heightened clinical awareness of this potential side effect appears justified.

A case of Takotsubo cardiomyopathy mimicking an acute coronary syndrome.

BACKGROUND: A 71-year-old woman presented with severe chest pain after an episode of acute emotional distress. Her serum levels of cardiac enzymes were slightly elevated and electrocardiography revealed anterior ST-segment elevations. Significant coronary stenoses were excluded. A left ventriculogram revealed apical ballooning and a hypercontractile basal segment. INVESTIGATIONS: Serum cardiac enzyme measurements, echocardiography, coronary angiography and left ventriculography. DIAGNOSIS: Takotsubo cardiomyopathy. MANAGEMENT: Treatment with beta-blockers, aspirin, angiotensin-converting-enzyme inhibitors, and intravenous diuretics.

A triglyceride/high-density lipoprotein ratio > or = 3.5 is associated with an increased burden of coronary artery disease on cardiac catheterization.

An elevated triglyceride (TG)/high-density lipoprotein (HDL) ratio has been described as a predictor of insulin resistance and cardiovascular events. We evaluated whether a TG/HDL ratio > or = 3.5 was associated with the burden of coronary artery disease (CAD) on cardiac catheterization. A retrospective chart review of 156 consecutive adults presenting to the Montefiore Medical Center Emergency Department with symptoms of unstable angina and no known history of CAD who underwent cardiac catheterization as part of their index hospitalization was performed. TG and HDL data were available in 100 patients within 6 months prior to admission and no more than 24 hours after presentation. A priori, a burden of CAD score was developed. On multivariate analysis, a TG/HDL ratio > or = 3.5 was associated with the burden of CAD (odds ratio, 2.87; 95% confidence interval, 1.03-7.96; p = 0.04). Further study is warranted.

Three-dimensional echocardiography of post-myocardial infarction cardiac rupture.

Ventricular septal defects and pseudoaneurysms are two serious complications of acute myocardial infarction and are associated with a high mortality if not surgically treated. Two-dimensional echocardiography provides excellent diagnostic information in such cases, but three-dimensional echocardiography may provide superior anatomic data of these potentially fatal complications. We describe two cases in which three-dimensional echocardiography provided incremental morphological information.