A case report of recurrent primary posterior mediastinal perivascular epithelioid cell tumour compressing the right inferior pulmonary vein, atria, and inferior vena cava.

Background: Perivascular epithelioid cell tumours (PEComas) are rare soft tissue neoplasms that commonly occur in the uterus, skin, and liver and less commonly in the retroperitoneum, colon, and mediastinum. Case summary: A 36-year-old male patient with a history of mediastinal PEComa status post resection, essential hypertension, and atrial fibrillation status post appendage ligation currently not on anticoagulation presented with a 1-week history of fevers, chills, productive cough, chest pain, dyspnoea on exertion, loss of appetite, and general weakness. Vital signs, physical exam, laboratory data, electrocardiogram, and chest radiograph were grossly unremarkable. A multimodality imaging approach utilizing transthoracic echocardiogram, transoesophageal echocardiogram (TEE), cardiac magnetic resonance imaging (cMRI), and computed tomography angiography of the chest, abdomen, and pelvis revealed a local 40 mm × 53 mm globular bilobed vascularized scar-free posterior mediastinal mass arising from the roof of the left and right atria and extending superiorly to the main pulmonary artery and inferiorly to the inferior vena cava. Based on the mass' size and proximity to vital structures and tumour recurrence, the case was presented during tumour board rounds, and the outcome was to surgically resect the mass and then have the patient follow up with medical oncology and radiation oncology for possible chemotherapy and radiation, respectively. Discussion: Perivascular epithelioid cell tumours are rare, and mediastinal PEComas are even rarer, warranting a multimodality imaging approach involving TEE and cMRI and a multidisciplinary approach involving anaesthesiologists, cardiologists, cardiothoracic surgeons, medical oncologists, pathologists, radiologists, and radiation oncologists.

A case report of successful transaneurysmal repair of a giant isolated membranous ventricular septal aneurysm.

Background: Isolated membranous ventricular septal aneurysms are infrequent in clinical practice. Furthermore, current guidelines do not dictate how to diagnose or manage such lesions. Case summary: A 54-year-old male patient with a history of essential hypertension and tobacco use presented with chest pain associated with dyspnoea and nausea. Electrocardiogram was unrevealing. Physical exam was significant for a diastolic murmur heard best in the apex. Computed tomography angiography of the chest revealed an aneurysm measuring 5 cm in diameter along the ascending aorta. Transoesophageal echocardiography showed that the aneurysm originated from the membranous ventricular septum, coursed along the ascending aorta, and ended anteriorly to the surface of the right ventricle and ascending aorta. Cardiac magnetic resonance imaging confirmed these findings and demonstrated that the aneurysm comprised of two loculations. Given the size of the aneurysm and its proximal location to major cardiovascular structures, percutaneous repair was considered unsafe. Following a multidisciplinary meeting, the lesion was successfully resected via a transaneurysmal approach. Discussion: Isolated membranous ventricular septal aneurysms are best imaged via a combination of transoesophageal echocardiogram and cardiac magnetic resonance imaging and best managed via a multidisciplinary approach for optimal outcomes.

Changing Incidence and Mechanism of Pregnancy-Associated Myocardial Infarction in the State of California.

Background The objective of this study was to evaluate the temporal trends in pregnancy-associated myocardial infarction (PAMI) in the State of California and explore potential risk factors and mechanisms. Methods and Results The California State Inpatient Database was analyzed from 2003 to 2011 for patients with International Classification of Diseases, Ninth Revision (ICD-9) codes for acute myocardial infarction and pregnancy or postpartum admissions; risk factors were analyzed and compared with pregnant patients without myocardial infarction. A total of 341 patients were identified with PAMI from a total of 5 266 380 pregnancies (incidence of 6.5 per 100 000 pregnancies). Inpatient maternal mortality rate was 7%, and infant mortality rate was 3.5% among patients with PAMI. There was a nonsignificant trend toward an increase in PAMI incidence from 2003 to 2011, possibly attributable to higher incidence of spontaneous coronary artery dissection, vasospasm, and Takotsubo syndrome. PAMI, when compared with pregnant patients without myocardial infarction, was significant for older age (aged >30 years in 72% versus 37%, P<0.0005), higher preponderance of Black race (12% versus 6%, P<0.00005), lower socioeconomic status (median household income in lowest quartile 26% versus 20%, P=0.04), higher prevalence of hypertension (26% versus 7%, P<0.0005), diabetes (7% versus 1%, P<0.0005), anemia (31% versus 7%, P<0.0001), amphetamine use (1% versus 0%, P<0.00005), cocaine use (2% versus 0.2%, P<0.0001), and smoking (6% versus 1%, P=0.0001). Conclusions There has been a trend toward an increase in PAMI incidence in California over the past decade, with an increasing trend in spontaneous coronary artery dissection, vasospasm, and Takotsubo syndrome as mechanisms. These findings warrant further investigation.

Safe Transcatheter Aortic Valve Replacement in a Patient with a Highly Mobile Aortic Valve Mass.

Some cardiac valve masses may have embolic potential with worrisome consequences. We describe the dilemmas of and solutions for a highly mobile papillary fibroelastoma on the aortic valve in a nonsurgical patient undergoing transcatheter aortic valve replacement. It was performed safely. The potential strategies to minimize the risk of embolization are discussed.

Usefulness of Statins for Prevention of Venous Thromboembolism.

Venous thromboembolism (VTE), including deep venous thrombosis and pulmonary embolism, is common with an annual incidence of 1 to 5 per 1,000, resulting in major morbidity, mortality, and increased health care costs. It is more common in the elderly, obese, those with cancer, those undergoing surgery, and those with previous VTE. Strategy to reduce its occurrence has important public health implications. Pleotropic effects of statins may have beneficial effects on a number of potential targets associated with VTE. Statins have excellent safety profile and seem to be associated with beneficial effects in VTE in case-control studies, large observational studies, meta-analyses, and a randomized trial. In conclusion, after critically reviewing the clinical data supporting statin use in the prevention of VTE, we presented clinical recommendations for the use of statins in reducing VTE occurrence, especially in high-risk situations.

Sex Difference in Patients With Ischemic Heart Failure Undergoing Surgical Revascularization: Results From the STICH Trial (Surgical Treatment for Ischemic Heart Failure).

BACKGROUND: Female sex is conventionally considered a risk factor for coronary artery bypass grafting (CABG) and has been included as a poor prognostic factor in multiple cardiac operative risk evaluation scores. We aimed to investigate the association of sex and the long-term benefit of CABG in patients with ischemic left ventricular dysfunction enrolled in the prospective STICH trial (Surgical Treatment for Ischemic Heart Failure Study). METHODS: The STICH trial randomized 1212 patients (148 [12%] women and 1064 [88%] men) with coronary artery disease and left ventricular ejection fraction ≤35% to CABG+medical therapy (MED) versus MED alone. Long-term (10-year) outcomes with each treatment were compared according to sex. RESULTS: At baseline, women were older (63.4 versus 59.3 years; P=0.016) with higher body mass index (27.9 versus 26.7 kg/m2; P=0.001). Women had more coronary artery disease risk factors (diabetes mellitus, 55.4% versus 37.2%; hypertension, 70.9% versus 58.6%; hyperlipidemia, 70.3% versus 58.9%) except for smoking (13.5% versus 21.8%) and had lower rates of prior CABG (0% versus 3.4%; all P<0.05) than men. Moreover, women had higher New York Heart Association class (class III/IV, 66.2% versus 57.0%), lower 6-minute walk capacity (300 versus 350 m), and lower Kansas City Cardiomyopathy Questionnaire overall summary scores (51 versus 63; all P<0.05). Over 10 years of follow-up, all-cause mortality (49.0% versus 65.8%; adjusted hazard ratio, 0.67; 95% confidence interval, 0.52-0.86; P=0.002) and cardiovascular mortality (34.3% versus 52.3%; adjusted hazard ratio, 0.65; 95% confidence interval, 0.48-0.89; P=0.006) were significantly lower in women compared with men. With randomization to CABG+MED versus MED treatment, there was no significant interaction between sex and treatment group in all-cause mortality, cardiovascular mortality, or the composite of all-cause mortality or cardiovascular hospitalization (all P>0.05). In addition, surgical deaths were not statistically different (1.5% versus 5.1%; P=0.187) between sexes among patients randomized to CABG per protocol as initial treatment. CONCLUSIONS: Sex is not associated with the effect of CABG+MED versus MED on all-cause mortality, cardiovascular mortality, the composite of death or cardiovascular hospitalization, or surgical deaths in patients with ischemic left ventricular dysfunction. Thus, sex should not influence treatment decisions about CABG in these patients. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov. Unique identifier: NCT00023595.

Survival Benefit of Statins in Hemodialysis Patients Awaiting Renal Transplantation.

End-stage renal disease (ESRD) patients have extraordinarily high cardiovascular risk and mortality, yet the benefit of statins in this population remains unclear based on the randomized trials. We investigated the prognostic value of statins in a large, pure cohort of prospectively recruited patients with ESRD awaiting renal transplantation, and being followed up in a dedicated cardiac clinic. We prospectively collected demographic, clinical, laboratory, and pharmacological data on 423 consecutive ESRD patients on hemodialysis awaiting renal transplantation. Survival analysis was performed as a function of statin therapy. The baseline characteristics were as follows: age 57 ± 11 years, males 64%, diabetes mellitus in 68%, known coronary artery disease in 30%, left ventricular (LV) ejection fraction 61 ± 11%. Over a mean follow-up of 2 years, there were 43 deaths. Adjusted for age, gender, hypertension, body mass index, diabetes mellitus, coronary artery disease, smoking, and treatment with angiotensin converting enzyme inhibitor, ß blocker, and antiplatelet medications, statin use was a predictor of lower mortality (hazard ratio 0.30, 95% confidence interval 0.11-0.79, p = 0.01). This beneficial effect of statin was supported by propensity score analysis (p = 0.02) and was consistent across all clinical subgroups. The benefit of statins seemed to be greater in those with LV hypertrophy and smoking. Statin therapy in hemodialysis patients awaiting renal transplant is independently associated with better survival supporting its use in this high-risk population.

Predictors and prognostic implications of major adverse cardiovascular events after renal transplant: 10 years outcomes in 321 patients.

Renal transplantation is the treatment of choice in patients with end-stage renal disease. Major adverse cardiac events (MACE) are common after renal transplant, especially in the perioperative period, leading to excess morbidity and mortality. The predictors and long-term prognostic implications of MACE are poorly understood. We analyzed predictors and implications of MACE in a cohort of 321 consecutive adult patients, who received renal allograft transplantation between 1995 and 2003 at our institution. The characteristics of 321 patients were: age at transplant 44 ± 13 years, 60% male, 36% diabetes mellitus (DM), left ventricular ejection fraction (LVEF) 60 ± 16%. MACE occurred in 21 patients with cumulative rate of 6.5% over 3 years after renal transplant, 57% occurring within 30 days, 67% within 90 days, and 86% within 180 days. MACE was not predicted by any clinical or pharmacological variables including age, gender, hypertension, DM, prior myocardial infarction, smoking, duration of dialysis, LVEF, or therapy with ß-blockers (BB), angiotensin converting enzyme inhibitors, or calcium channel blockers. However, a clinical decision to perform a stress test or a coronary angiogram was predictive of higher MACE rate. MACE, irrespective of type, was independently associated with higher mortality over a period up to 15 years and this seemed to be blunted by BB therapy. MACE rate after renal transplantation decreases over time, most occurring in the first 90 days and is not predicted by any of the traditional risk factors or drug therapies. It is associated with higher long-term mortality.

Progression of degenerative mitral stenosis: insights from a cohort of 254 patients.

BACKGROUND AND AIM OF THE STUDY: Degenerative mitral stenosis (DMS) is an increasingly common echocardiographic finding, yet the clinical and biological behavior and rate of progression of the condition are unknown. METHODS: A total of 254 patients was identified from the authors' echocardiographic database with DMS, defined as severe mitral annular calcification with extension into the mitral leaflets resulting in transmitral flow acceleration with a mean diastolic gradient of >2 mmHg in the absence of commissural fusion. Each patient required paired echocardiograms to have been recorded at least three months apart. Clinical, biochemical and pharmacological data were collected from each patient and related to the annualized rate of increase in mean diastolic mitral gradient and stenosis severity on a scale of 0 to 3. RESULTS: The characteristics of the patients were as follows: mean age 71 +/- 15 years; female gender 73%; and left ventricular ejection fraction 66 +/- 13%. Diabetes was present in 50% of patients, renal insufficiency in 40%, and coronary artery disease in 50%. Over a follow up period of 2.6 +/- 2.2 years, the mean gradient was increased by 0.8 +/- 2.4 mmHg (range: 0-15 mmHg) per year, while the stenosis grade was increased by 0.18 +/- 0.5 (range: 0-3) per year. The rate of progression was faster in patients with lesser degrees of stenosis (p = 0.01) and low serum albumen levels (p = 0.04), and slower in those receiving beta-blockers (p = 0.01). Milder stenosis, diabetes mellitus and lack of beta-blocker use were independent predictors of faster DMS progression. CONCLUSION: DMS progression is highly variable, but generally slow; its progression is accelerated in the presence of diabetes mellitus, but is retarded by beta-blocker use. DMS may be an active biological process offering potentially modifiable targets for intervention.

Cardiac rhythm device surgery with uninterrupted oral anticoagulation.

Current guidelines recommend interrupting anticoagulation and bridging therapy with heparin or low-molecular-weight heparin for cardiac rhythm device surgeries in patients with high thrombotic risk. However, there are some studies that suggest continuing warfarin may be safe. The study by Birnie et al. investigates this important clinical question in a randomized controlled trial setting. They randomly assigned 681 patients with high thrombotic risk (5% or more per year), in 18 centers, to receive either stopping warfarin combined with heparin bridging (standard of care) or continued uninterrupted warfarin therapy for cardiac rhythm device surgery. The trial was terminated after a second prespecified interim analysis by the data and safety monitoring board. Clinically significant device-pocket hematoma was noted in 12 out of 343 patients (3.5%) in the uninterrupted warfarin group, compared with 54 out of 338 (16.0%) in the heparin-bridging group (relative risk: 0.19; 95% CI: 0.10-0.36; p < 0.001). Uninterrupted warfarin was associated with better patient satisfaction, and there was no significant difference in thromboembolic or surgical complications between the two groups. These results demonstrate that device surgeries can be safely performed with continued warfarin, and bridging with heparin is associated with high risk of device-pocket hematoma.

Prognostic value of cardiac magnetic resonance imaging in patients with aortic regurgitation.

Guidelines on valve replacement recommend aortic valve replacement for patients with severe aortic regurgitation (AR) with symptoms or left ventricular (LV) dysfunction. However, the optimal timing of surgery for asymptomatic AR patients without LV dilation or dysfunction is not known. There are data to suggest that excess volume load imposed by AR may not only produce subclinical LV dysfunction, but produce neurohormonal activation similar to the heart failure syndrome resulting in reduced survival. The study by Myerson et al. is the first to investigate the predictive ability of cardiac MRI (CMR) for the outcome of asymptomatic patients with AR. They studied 113 asymptomatic patients with moderate-to-severe AR on echocardiography in four centers. A total of 39 (35%) patients developed symptoms or an indication for surgery over a mean follow-up period of 2.6 years. AR volume, AR regurgitant fraction, LV end-diastolic and end-systolic volumes had high discriminatory powers (area under curve of 0.96, 0.93, 0.88 and 0.78, respectively) to predict these events. Higher association with the outcome was observed when LV end-diastolic volume and regurgitant fraction were combined. A significantly higher number of patients with regurgitant fraction >33% were likely to progress to surgery compared with patients with a regurgitant fraction of <33% (85 vs 8%; p < 0.001). These results demonstrate a potential role for CMR for risk stratification of patients with asymptomatic moderate or severe AR, given the ability of CMR to accurately quantify AR and LV volumes. Based on the data presented, it is possible that we may be waiting too long to offer surgery in patients with severe AR.

Lack of relationship between microvascular and macrovascular disease in heart transplant recipients.

BACKGROUND: A key determinant of long-term survival in heart transplant recipients is the development of coronary vasculopathy. Both coronary macrovascular and microvascular disease are prognostically important. However, the relationship between these in transplant patients and the determinants of microvascular disease are not known. METHODS: We reviewed the simultaneously obtained endomyocardial biopsies and intravascular ultrasound (IVUS) images of coronary arteries in 33 heart transplant recipients. Coronary microvascular disease was classified by light microscopy into four grades based on thickening of endothelial cell layer and stenotic versus nonstenotic medial wall thickening. Macrovascular disease was evaluated from IVUS studies and assigned into one of five grades based on the Stanford classification. Coronary microvascular and macrovascular diseases were compared. RESULTS: Age at transplantation was 26 (18) years; 67% were men, and the average time to posttransplantation study was 4 years. Endomyocardial biopsy revealed more advanced grade C and D microvascular disease in 45% and 36% of the patients, respectively. However, IVUS analysis for macrovascular disease revealed mostly lesser changes with grade 1 in 12%, grade 2 in 61%, and grade 3 in 21%. There was no significant correlation between grades of microvascular and macrovascular disease (P=0.10). Microvascular disease correlated positively with donor age (P=0.06) and treatment with tacrolimus (0=0.02) and statins (P=0.05). CONCLUSIONS: There is a poor relationship between coronary microvascular and macrovascular disease in patients with cardiac transplants, likely indicating divergent pathogenetic mechanisms. Microvascular disease increases with donor age. There is an intriguing positive relationship between microvascular disease and treatment with statins and tacrolimus.

Slow rate of progression of grade 1 and 2+ aortic regurgitation.

BACKGROUND AND AIM OF THE STUDY: Although the progression of aortic stenosis has been well studied, the rate of progression of aortic regurgitation (AR) has not been definitively established. Further data would be valuable for clinical decision-making in patients with milder degrees of AR undergoing non-aortic valve cardiac surgery. Hence, this point was investigated in a large cohort of patients with grade 1 or 2+ AR. METHODS: The authors' echocardiographic database acquired between 1993 and 2007 was screened for patients with grade 1 or 2+ AR who had undergone follow up echocardiography at least one year later. The AR severity was graded as 1 to 4+, and any annual changes in AR grade were monitored. RESULTS: Among a total of 4,128 patients identified, 3,266 had grade 1+ AR and 862 had grade 2+ AR on the initial echocardiogram: the mean age was 67 +/- 15 years, and the duration of follow up was 4.2 +/- 2.7 years. Of those patients initially with grade 1+ AR, 95% showed no change in AR over a mean interval of 4.2 years, with an annual average increase in AR grade of 0.04. Of those patients initially with grade 2+ AR, 90% showed no change over this period, with an annual average increase in grade of 0.07. In the entire cohort, the AR progression correlated positively with age (p = 0.03), ventricular septal thickness (p < 0.0001), stroke distance (the time velocity integral of flow in the left ventricular outflow tract) (p = 0.0003), the presence of any degree of aortic stenosis (p = 0.01), and initial AR severity (p < 0.0001). CONCLUSION: The rate of AR progression is extremely slow, and prophylactic aortic valve replacement during non-aortic surgery may not be indicated in patients with grade 1 or 2+ AR in the absence of any higher risk for progression, such as grade 2+ AR combined with any degree of aortic stenosis and advanced age.

Prognostic implications of tricuspid regurgitation in patients with severe aortic regurgitation: results from a cohort of 756 patients.

Tricuspid regurgitation (TR) is common, but neglected. We evaluated the prognostic implications of TR in a cohort of 756 patients with severe aortic regurgitation (AR). A cohort of 756 patients with AR was identified from our echocardiographic database. Chart reviews were performed. Survival as a function of TR severity was analysed. Of the 756 patients with severe AR, 264 (35%) had ≥ 2+ TR. Univariate correlates of TR were older age (P < 0.0001), female gender (P < 0.0001), lower left ventricular ejection fraction (P < 0.0001), atrial fibrillation (P < 0.0001), presence of a pacemaker (P < 0.0001), higher PASP (P < 0.0001), presence of 3 or 4+ mitral regurgitation (P < 0.0001) and not being on a beta-blocker (P < 0.0001) or statins (P = 0.007). After adjusting for group differences, ≥ 2+ TR was an independent predictor of higher mortality (RR 1.47, P = 0.005). Aortic valve replacement (AVR) was independently associated with improved survival in patients with ≥ 2+ TR. (RR 0.46, 95% CI 0.36-0.60, P < 0.0001). In conclusion, in severe AR patients, ≥ 2+ TR is independently associated with a higher mortality. The performance of AVR in these patients with ≥ 2+ TR is associated with a survival benefit. Development of ≥ 2+ TR in these patients is a marker of decompensation and should serve as an indication for AVR.