An Unusual Cause of Functional Mitral Stenosis: A Left Atrial Intimal Sarcoma.

Primary cardiac tumors are rare, with an incidence of <0.1% in postmortem series; sarcomas comprise 75% of these. Cardiac sarcomas may be life-threatening at the time of presentation. We describe a left atrial intimal sarcoma presenting with constitutional symptoms, obstructive shock, and systemic emboli, and treated with proton beam therapy. (Level of Difficulty: Intermediate.).

Relation of Transthoracic Echocardiographic Aortic Regurgitation to Pressure Half-time and All-Cause Mortality.

To evaluate the relation of aortic regurgitation (AR) pressure half-time (PHT) on transthoracic echocardiography (TTE) and all-cause mortality, we screened 118,647 baseline TTE reports from 2000 to 2017, to identify patients with any AR and PHT data. Patients with infective endocarditis or previous aortic valve replacement were excluded. The relation of baseline PHT on time to all-cause mortality was evaluated using Cox regression. A total of 2,653 patients were included (73.1 ± 14.3 years; 53.8% female; PHT, 530 ± 162 ms). Patients with shorter PHTs more frequently had 3-4+ AR (PHT ≤ 200 ms vs > 500 ms, 17.9% vs 0.6%, p < 0.0001). Diastolic parameters (E/e', E/A ratio, mitral valve deceleration time, and pulmonary artery systolic pressure) all significantly correlated with PHT (all p < 0.05). Over a median (IQR) follow-up of 8 (4 to 11 years), there were 799 (30.1%) deaths at a median (IQR) of 1.9 (0.4 to 4.3) years. On a univariate basis, a PHT ≤ 320 ms or > 750 ms was significantly related to increased mortality, even amongst those with nonsevere AR. After multivariable adjustment (in particular for E/e'), PHT was no longer significantly related to death. In conclusion, in this large, single center, retrospective study, AR PHT was not independently related to mortality. While a PHT ≤ 320 ms was associated with increased mortality in patients without severe AR, this relation was no longer significant after adjusting for diastolic functional variables. Thus, a PHT ≤ 320 ms in patients without significant AR may indicate prognostically-relevant diastolic dysfunction.

Comparison of intracardiac echocardiography and transesophageal echocardiography for imaging of the right and left atrial appendages.

BACKGROUND: Transesophageal echocardiography (TEE) is the standard for diagnosis of atrial thrombi and is performed before ablation of atrial arrhythmias. Intracardiac echocardiography (ICE) is routinely used during these procedures and may provide an alternative imaging modality. OBJECTIVE: The purpose of this study was to compare TEE and ICE for right atrial appendage (RAA) and left atrial appendage (LAA) anatomy and thrombus. METHODS: This prospective blinded study enrolled 71 patients with atrial arrhythmias who presented for ablation. TEE and ICE were performed simultaneously to assess the RAA and LAA for thrombi, spontaneous echo contrast, and dimensions. ICE images were acquired sequentially from the right atrium, right ventricular outflow tract, and the pulmonary artery. RESULTS: Imaging of the RAA and LAA was achieved in all 71 patients using ICE but in only in 69 patients using TEE because of inability to intubate the esophagus. A total of 4 thrombi were diagnosed (3 LAA, 1 RAA). All were detected by ICE but only 1 by TEE. Diagnostic imaging of the LAA was achieved in 71 patients (100%) with ICE and in 62 patients (87.3%) with TEE (P < .002). Spontaneous echo contrast was more commonly diagnosed with ICE (P < .01). There was strong correlation between TEE and ICE for length (r = 0.71), width (r = 0.94), and area (r = 0.88) of the LAA. Image quality with ICE was highest from the pulmonary artery and lowest from the right atrium. CONCLUSION: ICE imaging is a viable alternative to TEE for visualization of the LAA and RAA during catheter ablation procedures.

Valvular heart disease: classic teaching and emerging paradigms.

Valvular heart disease is both prevalent and increases with age. The final pathway of valvular disease is heart failure and sometimes sudden death, so clinicians must identify and treat it before these endpoints occur. Noninvasive diagnostic modalities such as echocardiography, exercise tolerance testing, and cardiac magnetic resonance provide additional quantitative, qualitative, and prognostic data. Studies have elucidated predictors of disease progression and potential medical therapies, but the niche of valvular disease has benefited relatively less from randomized controlled clinical trials than other cardiovascular disease fields. New invasive techniques like transcatheter valve replacement offer hope for high-risk operative candidates. We review classic teaching with current guidelines and emphasize recent advances in disease management.

Care of patients with apparently asymptomatic severe aortic valve stenosis.

Although most asymptomatic patients with aortic stenosis (AS) have an excellent prognosis with observation only, some patients with severely stenotic aortic valves in the absence of symptoms may benefit from early intervention. In this article, we will review the literature on the use of varied imaging modalities, clinical factors, and biomarker testing to risk stratify such patients. Additionally, we will review the role of medical therapy to modify the course in AS.

Predictors of preserved left ventricular systolic function after surgery for chronic organic mitral regurgitation: a prospective study.

BACKGROUND AND AIM OF THE STUDY: For patients with chronic severe mitral regurgitation (MR), one consideration for the timing of mitral valve surgery is the desire to have a preserved postoperative left ventricular ejection fraction (LVEF). It was hypothesized that the preoperative left ventricular (LV) volume and effective forward LVEF (defined as the ratio of forward aortic stroke volume and LV end-diastolic volume (LVEDV)) would accurately predict the intermediate (three-month) LVEF following surgery. It was also hypothesized that at years after successful surgical correction, there would be ongoing positive ventricular remodeling with improvements in systolic function. A quantitative volumetric cardiovascular magnetic resonance (CMR) system was used prospectively to study these relationships, and to define predictors of postoperative LV systolic function after surgery for chronic severe MR. METHODS: Twenty patients (eight females, 12 males; mean age 52 +/-12 years) with chronic severe MR, who had been referred for elective mitral valve repair or replacement with chordal preservation, were prospectively enrolled. All patients underwent quantitative CMR with assessment of LV volume, LVEF, and effective forward LVEF prior to, and at three months after, surgery. Fourteen of the patients returned at a median of 27 months after valve surgery for repeat CMR. RESULTS: The preoperative LVEF was normal in 90% of patients (0.62 +/- 0.09), but the effective forward LVEF was depressed in all subjects (0.36 +/- 0.09). Compared to preoperative values, at three months after surgery the LVEF had declined by 0.16 +/- 0.08 (p < 0.001), whereas the effective forward LVEF was increased by 0.09 +/- 0.10 (p < 0.001) and the LV end-diastolic volume index (LVEDVI) had declined by 32% (p < 0.001). Multivariate analysis demonstrated a significant correlation between postoperative LVEF and both preoperative LV end-systolic volume index (LVESVI) (p = 0.003) and the preoperative effective forward LVEF (p = 0.05). A depressed postoperative LVEF (< 0.50) during the early period was best predicted by a preoperative effective forward LVEF < 0.40 (sensitivity 100%, specificity 75%). At the 27-month follow up, 12 of the 14 patients (86%) had a normal LVEF, and there was a continuing normalization of other LV parameters, including the LVEDVI and LV mass index. CONCLUSION: Among patients with chronic severe MR, the preoperative LVESVI best correlated with the three-month postoperative LVEF. A preoperative effective forward LVEF < 0.40 accurately predicted a depressed postoperative LVEE. Over the succeeding two years, there will be ongoing positive LV remodeling. If confirmed in a larger series, the effective forward LVEF may serve as a useful clinical guide for the timing of corrective surgery in this population.

Cardiovascular magnetic resonance imaging for valvular heart disease.

Valvular heart disease (VHD) is a clinically important diagnosis, with significant associated morbidity and mortality. Multiple imaging modalities exist to characterize valvular and associated cardiac anatomy. Cardiovascular magnetic resonance (CMR) has emerged as a comprehensive noninvasive imaging modality for VHD. With use of well-established, standardized imaging sequences, CMR can accurately and precisely diagnose valvular structural abnormalities, assess severity of regurgitant and stenotic lesions, and potentially define patient prognosis. This article reviews the clinical applications of CMR in assessment of VHD.

Association of TIMI myocardial perfusion grade and ST-segment resolution with cardiovascular magnetic resonance measures of microvascular obstruction and infarct size following ST-segment elevation myocardial infarction.

BACKGROUND: Impairment of coronary microvascular perfusion is common among patients with ST-segment elevation myocardial infarction (STEMI) treated with primary percutaneous coronary intervention (PCI). Cardiovascular magnetic resonance imaging (CMR) can identify microvascular obstruction (MO) following reperfusion of STEMI. We hypothesized that myocardial perfusion, as assessed by the Thrombolysis in Myocardial Infarction (TIMI) Myocardial Perfusion Grade (TMPG), would be associated with a CMR metric of MO in this population. METHODS: Twenty-one STEMI patients who underwent successful primary PCI were evaluated. Contrast-enhanced CMR was performed within 7 days of presentation and repeated at three months. TIMI Flow Grade (TFG), corrected TIMI Frame Count (cTFC), TMPG, MO, infarct size, and left ventricular ejection fraction (EF) were assessed. RESULTS: The median peak creatine phosphokinase (CPK) was 1,775 IU/l (interquartile range 838-3,321). TFG 3 was present following PCI in 19 (90%) patients. CMR evidence of MO was present in 52% following PCI. Abnormal post-PCI TMPG (0/1/2) was present in 48% of subjects and was associated with MO on CMR (90% MO with TMPG 0/1/2 vs. 18% MO with TMPG 3, P < 0.01). Abnormal post-PCI TMPG was also associated with a greater peak CK (median 3,623 IU/l vs. 838 IU/l, P < 0.001) and greater relative infarct size (17.3% vs. 5.2%, P < 0.01). CONCLUSION: Among STEMI patients undergoing primary PCI, post-PCI TMPG correlates with CMR measures of MO and infarct size. The combined use of both metrics in a comprehensive assessment of microvascular integrity and infarct size following STEMI may aid in the evaluation of future therapeutic strategies.

Effects of N-acetyl-cysteine on endothelial function and inflammation in patients with type 2 diabetes mellitus.

Endothelial dysfunction has been associated with premature vascular disease. There is increasing data that N-acetyl-cysteine (NAC) may prevent or improve endothelial dysfunction. The aim of this study was to assess the effects of NAC on endothelial function in patients with type 2 diabetes mellitus, a population at high risk for endothelial dysfunction. Twenty-four patients with diabetes mellitus were assigned randomly to initial therapy with either 900 mg NAC or placebo twice daily in a double-blind, cross-over study design. Flowmediated vasodilation (FMD) of the brachial artery was assessed at baseline, after four weeks of therapy, after a four-week wash-out period, and after another four weeks on the opposite treatment. Plasma and red blood cell glutathione levels and high-sensitivity C-reactive protein (CRP) were measured at all four visits. At baseline, FMD was moderately impaired (3.7±2.9%). There was no significant change in FMD after four weeks of NAC therapy as compared to placebo (0.1±3.6% vs. 1.2±4.2%). Similarly, there was no significant change in glutathione levels. However, median CRP decreased from 2.35 to 2.14 mg/L during NAC therapy (p=0.04), while it increased from 2.24 to 2.65 mg/L with placebo. No side effects were noted during the treatment period. In this double-blind, randomized cross-over study, four weeks of oral NAC therapy failed to improve endothelial dysfunction in patients with diabetes mellitus. However, NAC therapy decreased CRP levels, suggesting that this compound may have some efficacy in reducing systemic inflammation.

Rimonabant: the role of endocannabinoid type 1 receptor antagonism in modulating the weight and lipid profile of obese patients.

Rimonabant is a selective blocker of the CB(1) cannabinoid receptor that has been developed for treatment of abdominal obesity, dyslipidemia, and control of diabetes. Four randomized clinical trials have demonstrated that following 1 year of treatment, 20 mg/d of rimonabant is associated with greater weight loss and reduction in waist circumference compared with placebo. Therapy with rimonabant is associated with favorable changes in serum high-density lipoprotein and triglycerides, as well as with an improvement in glycemic control among obese type 2 diabetic patients. Rimonabant appears to be well tolerated, with the most common side effects being nausea, anxiety, and depressive symptoms. Rimonabant is a novel agent that has the potential to be a useful adjunct to lifestyle and behavior modification in treatment of abdominal obesity, dyslipidemia, and dysglycemia.

Severity of mitral and aortic regurgitation as assessed by cardiovascular magnetic resonance: optimizing correlation with Doppler echocardiography.

BACKGROUND: Cardiovascular magnetic resonance (CMR) is widely recognized as a non-invasive gold standard for quantification of ventricular volumes. In addition, it is an emerging diagnostic modality for clinical evaluation of mitral regurgitation (MR) and aortic regurgitation (AR). CMR facilitates accurate quantitation of regurgitation volumes and regurgitant fraction, but referring physicians are often more comfortable with qualitative measures, and few data exist for correlation of qualitative CMR regurgitation severity with that obtained by more conventional qualitative Doppler echocardiography. Because patients with AR and MR may commonly be assessed by both echocardiography and CMR modalities, consistency between qualitative gradient of regurgitation severity is important for follow-up. Therefore, we sought to define the CMR regurgitant fractions that best correlate with qualitative mild, moderate, and severe regurgitation by color Doppler echocardiography. METHODS AND RESULTS: Data from 141 consecutive patients (age 53 +/- 15 yr; 43% female) with contemporary (median, 31 days) CMR and echocardiographic data, including 107 regurgitant valves and 70 normal valves, were compared. Thresholds were developed on an initial cohort of patients with 55 regurgitant valves, and subsequently tested on a later cohort of patients with 52 regurgitant valves. Regurgitation fraction (RF) limits that optimized concordance of CMR and echo severity grades were similar for MR and AR and were: mild < or = 15%, moderate 16-25%, moderate-severe 26-48%, severe > 48%. CONCLUSIONS: The current study provides simple qualititative threshold grades for MR and AR severity that allows for standardized reporting of regurgitation severity by CMR and excellent correlation with clinical echocardiography.

Rimonabant: a cannabinoid receptor type 1 blocker for management of multiple cardiometabolic risk factors.

Rimonabant is a first selective blocker of the cannabinoid receptor type 1 (CB1) being developed for the treatment of multiple cardiometabolic risk factors, including abdominal obesity and smoking. In four large trials, after one year of treatment, rimonabant 20 mg led to greater weight loss and reduction in waist circumference compared with placebo. Therapy with rimonabant is also associated with favorable changes in serum lipid levels and an improvement in glycemic control in prediabetes patients and in type 2 diabetic patients. At the same dose, rimonabant significantly increased cigarette smoking quit rates as compared with placebo. Rimonabant seems to be well tolerated, with a primary side effect of mild nausea. As an agent with a novel mechanism of action, rimonabant has a potential to be a useful adjunct to lifestyle and behavior modification in treatment of multiple cardiometabolic risk factors, including abdominal obesity and smoking.