Functionally Aberrant Mutant KCNQ1 With Intermediate Heterozygous and Homozygous Phenotypes

Background

Deleterious mutations in KCNQ1 may lead to an autosomal dominant form of long QT syndrome (LQTS) (Romano-Ward) or autosomal recessive form (Jervell and Lange-Nielsen). Both are associated with severe ventricular tachyarrhythmias due to the reduction of the slowly activating delayed rectifier K⁺ current (I_Ks). Our objective was to investigate the functional consequences of KCNQ1-R562S mutation in an atypical form of KCNQ1-linked LQTS.

Contrary to Expectations: Off-Label Transcatheter Aortic Valve Replacement in the Case of Left Ventricular Outflow Tract Obstruction

We present the case of a 71-year-old woman who showed recurrent signs of congestive heart failure with the need of rehospitalization after double valve (mitral and aortic) replacement. Extensive diagnostic workup revealed a moderate aortic stenosis and additionally a significant left ventricular outflow tract obstruction. The tissue overgrowth might be attributed to an inflammatory reaction with extensive pannus deposit after aortic valve surgery. With no-option for re-do surgery we performed the first-in-man off-label valve-in-left ventricular outflow tract procedure with an Edwards Sapien III 23 mm in deep orientation.     

Left Ventricular Systolic Dysfunction and Cardiovascular Outcomes in Tetralogy of Fallot: Systematic Review and Meta-analysis

BackgroundAlthough there are robust data about the pathophysiology and prognostic implications of left ventricular (LV) systolic dysfunction in patients with acquired heart disease, similar prognostic data about LV systolic dysfunction are sparse in the tetralogy of Fallot (TOF) population. The purpose of this study was to perform a meta-analysis of all studies that assessed the relationship between LV ejection fraction (LVEF) and cardiovascular adverse events (CAEs) defined as death, aborted sudden death, or sustained ventricular tachycardia.MethodsWe used random-effects models to calculate hazard ratios (HRs) and 95% confidence intervals (CIs).ResultsOf the 1,809 citations, 7 studies with 2,854 patients (age 28 ± 4 years) were included. During 5.6 ± 3.4 years' follow-up, there were 82 deaths, 17 aborted sudden cardiac deaths, and 56 sustained ventricular tachycardia events. Overall, CAEs occurred in 5.1% (144 patients). As a continuous variable, LVEF was a predictor of CAE (HR 1.29, 95% CI, 1.09-1.53, P = 0.001) per 5% decrease in LVEF. Similarly, LVEF < 40% was also a predictor of CAE (HR 3.22, 95% CI, 2.16-4.80, P < 0.001).ConclusionsLV systolic dysfunction was an independent predictor of CAE, and we observed a 30% increase in the risk of CAE for every 5% decrease in LVEF, and a 3-fold increase in the risk of CAE in patients with LVEF <40% compared with other patients. These findings underscore the importance of incorporating LV systolic function in clinical risk stratification of patients with TOF and the need to explore new treatment options to address this problem.     

Right Ventricular Diastolic Function and Right Atrial Function and Their Relation With Exercise Capacity in Ebstein Anomaly

BackgroundRight ventricular (RV) diastolic function and right atrial (RA) function are poorly characterized in patients with Ebstein anomaly (EA) but may influence functional capacity. We aimed to evaluate RV diastolic function and RA function in EA and study their relationship with biventricular systolic function and exercise capacity.MethodsSeventy-two patients with EA and 69 controls prospectively underwent echocardiography, cardiovascular magnetic resonance imaging, and cardiopulmonary exercise testing to investigate RV systolic and diastolic function, RA function, and exercise capacity.ResultsAltered RV diastolic function was indicated by the reduced tricuspid valve E/A ratio, percentage RV filling time, and early and late diastolic strain rate; and by the increased tricuspid valve E/E′, isovolumic relaxation time, and RV myocardial performance index. The average of 6-RV-segment early diastolic strain rate correlated modestly with peak VO₂ (r = 0.38, P < 0.01), RV ejection fraction (r = 0.41, P < 0.01), and left ventricular ejection fraction (r = 0.33, P < 0.05). Patients with EA had impaired RA reservoir, conduit, and pump function, which were associated with peak VO₂ (r = 0.54, P < 0.001 for reservoir function).ConclusionsAltered RV diastolic function and RA function in patients with EA are associated with impaired biventricular systolic function and exercise capacity. The stronger correlation of RA vs RV function with exercise capacity suggests that it may be important to evaluate RA function in this population.     

Assessment of Novel Peguero-Lo Presti Electrocardiographic Left Ventricular Hypertrophy Criteria in a Large Asian Population: Newer May Not Be Better

Background

Recently, the novel Peguero-Lo Presti electrocardiographic criteria to diagnose left ventricular hypertrophy (LVH) were developed from Caucasian American population with a relatively high sensitivity. However, further validation on a large Asian population has never been conducted. Thus, this study was to test and validate the overall performance of this index in a general population from China.

Cardiomyopathy and Heart Failure in Patients With HIV Infection

With the advent and widespread use of antiretroviral therapy (ART), the epidemiology of cardiomyopathy and heart failure (HF) associated with HIV infection is changing. Near-normal life expectancy in contemporary HIV-infected populations has been associated with prolonged exposure to increased cardiometabolic burden and chronic immune activation and systemic inflammation. Therefore, the pre-ART phenotype of HIV-associated cardiomyopathy with overt left ventricular systolic dysfunction and poor prognosis has been replaced over time by cardiomyopathy with a more insidious course, more frequent ischemic background, and highly prevalent left ventricular diastolic dysfunction. Patients with HIV are more prone to development of coronary artery disease and development of HF after myocardial infarction. The role of ongoing immune activation and systemic inflammation, despite highly active ART (HAART), appears to be central in this process. The role of HAART toxicity is controversial, as HAART itself appears to be protective for the development of HF, but recent data suggest that protease inhibitors might adversely affect the course of HIV-associated HF. Because of these unique features, the optimal therapeutic approach for HIV-associated cardiomyopathy remains unknown. The current therapeutic approaches are an extrapolation from noninfected populations. Importantly, the significance of the highly prevalent diastolic abnormalities among HIV-infected patients is not known. Therefore, further research is needed to identify its prognostic implications. Considering the prevalence of structural and functional cardiac abnormalities in HIV-infected persons and the lack of evidence on how to best screen and treat these patients, systematic research on this topic is a public health priority.     

Abnormal Myocardial Dietary Fatty Acid Metabolism and Diabetic Cardiomyopathy

Patients with diabetes are at very high risk of hospitalization and death from heart failure. Increased prevalence of coronary heart disease, hypertension, autonomic neuropathy, and kidney failure all play a role in this increased risk. However, cardiac metabolic abnormalities are now recognized to play a role in this increased risk. Increased reliance on fatty acids to produce energy might predispose the diabetic heart to oxidative stress and ischemic damage. Intramyocellular accumulation of toxic lipid metabolites leads to a number of cellular abnormalities that might also contribute to cardiac remodelling and cardiac dysfunction. However, fatty acid availability from circulation and from intracellular lipid droplets to fuel the heart is critical to maintain its function. Fatty acids delivery to the heart is very complex and includes plasma nonesterified fatty acid flux as well as triglyceride-rich lipoprotein-mediated transport. Although many studies have shown a cross-sectional association between enhanced fatty acid delivery to the heart and reduction in left ventricular function in subjects with prediabetes and diabetes, these mechanisms change very rapidly during type 2 diabetes treatment. The present review focuses on the role of fatty acids in cardiac function, with particular emphasis on the possible role of early abnormalities of dietary fatty acid metabolism in the development of diabetic cardiomyopathy.     

Right Ventricular Function and Beneficial Effects of Cardiac Rehabilitation in Patients With Systolic Chronic Heart Failure

Background

It has been recognized that a comprehensive cardiac rehabilitation (CR) program improves mortality in patients with chronic heart failure. On the other hand, the magnitude of the improvement in exercise capacity after CR differs among individuals. The aim of this study was to assess the echocardiographic determinants of responders to CR using preload stress echocardiography.

The Potential Effects of New Stent Platforms for Coronary Revascularization in Patients With Diabetes

Coronary artery disease in patients with diabetes mellitus (DM) is characterized by extensive atherosclerosis, longer lesions, and diffuse distal disease. Consequently, these patients have worse outcomes after coronary revascularization, regardless of the modality used. Traditionally, coronary artery bypass grafting (CABG) has been regarded as more effective than percutaneous coronary intervention (PCI) in patients with DM, likely because of more complete revascularization and protection against disease progression in the bypass segment. Revascularization with balloon angioplasty, bare-metal stents, and first-generation drug-eluting stents have all been shown to be inferior to CABG in patients with DM. Current professional society guidelines reflect these findings, strongly recommending CABG over PCI in this setting. Newer stent platforms, however, have challenged this notion. The use of thinner struts, biocompatible polymer coating, and newer antiproliferative agents have improved the rates of cardiovascular events in patients with DM revascularized percutaneously. Since the publication of current guidelines, new studies suggested acceptable outcomes in patients with DM revascularized with second-generation drug-eluting stents, even though these conclusions are drawn from small subgroup analyses or nonrandomized studies. Robust registry data suggest similar mortality with lower rates of stroke after PCI compared with surgery, at the expense of increased rates of repeat revascularization. If complete revascularization can be achieved, similar rates of myocardial infarction are also observed. Therefore, contemporary revascularization in patients with DM with multivessel coronary artery disease should involve a multidisciplinary approach, in which interventional cardiologists and cardiac surgeons involve their patients to individualize treatment choices, and balance the risks and effectiveness of each modality.     

Coronary Vasospasm Following Heart Transplantation: Rapid Progression to Aggressive Cardiac Allograft Vasculopathy

Coronary artery vasospasm (CVS) has been described in orthotopic heart transplant patients but is rare in the post-transplanted, denervated heart. Severe CVS has been associated with accelerated cardiac allograft vasculopathy (CAV) and allograft rejection. Allograft vasculopathy is the leading cause of decreased long-term survival in orthotopic heart transplant. The prognostic significance and relationship of the presence and severity of CVS with CAV are not well understood. We present a case of severe symptomatic CVS with rapid development of severe CAV. Our case emphasizes the need for close angiographic surveillance and intracoronary imaging for early detection of CAV in the presence of vasospasm.     

Invasive Hemodynamics and Rejection Rates in Patients With Cardiac Sarcoidosis After Heart Transplantation

Background

Orthotopic heart transplant (OHT) is increasingly used for end-stage heart failure due to cardiac sarcoidosis (CS). However, concern regarding long-term outcomes in patients with CS after OHT persists because of multiorgan involvement.

Successful Localization and Removal of an Aberrant Sewing Needle in the Posterior Mediastinum: Usefulness of Multidetector Computed Tomography

Foreign metallic bodies in the mediastinum are relatively rare and may cause life-threatening damages to multiple organs in the mediastinum; therefore, the foreign body warrants urgent or elective removal. When the foreign body remains in the posterior mediastinum surrounded by important organs, for example, the heart or the great vessels, it is critical to determine the right approach for its safe removal. Here, we present the successful removal of a sewing needle from the posterior mediastinum using multidetector computed tomography that helped identify the foreign body’s location and its relationship with the surrounding organs.     

Early vs Late Surgery for Patients With Endocarditis and Neurological Injury: A Systematic Review and Meta-analysis

Background

Surgical timing in infective endocarditis (IE) with preoperative neurological events remains controversial. The relevant society guidelines are each on the basis of a small number of observational studies. This meta-analysis was designed to search the available literature broadly and assess the weight of available evidence as comprehensively as possible.

Resource Use and Burden of Hospitalization,Outpatient, Physician,and Drug Costs in Short- and Long-term Care After Acute Myocardial Infarction

Background

Little is known about the resource use and cost burden of acute myocardial infarction (AMI) beyond the index event. We examined resource use and care costs during the first and each subsequent year, among patients with incident AMI.

Cardiac Surgery in HIV Patients: State of the Art

The clinical status of HIV infection has changed dramatically with the introduction of combined antiretroviral therapy. Patients with HIV are now living long enough to be susceptible to chronic illnesses, such as coronary disease and nonischemic cardiomyopathy, which can be consequences of the combined antiretroviral therapy treatment itself. Cardiovascular diseases are a major source of morbidity and mortality in HIV-positive patients. Increasingly, such patients might be candidates for the full range of cardiac surgical interventions, including coronary bypass, valve surgery, and heart transplantation. There has been a shift from offering palliative procedures such as pericardial window and balloon valvuloplasty, to more conventional and durable surgical therapies in HIV-positive patients. We herein provide an overview of the contemporary outcomes of cardiac surgery in this complex and unique patient population. We review some of the ethical issues around the selection and surgical care of HIV-positive patients. We also discuss strategies to best protect the surgical treatment team from the risks of HIV transmission. Finally, we highlight the need for involvement of dedicated infectious disease professionals in a multidisciplinary heart team approach, aiming at the comprehensive care of these unique and complex patients.     

Coronary Artery Disease Manifestations in HIV: What,How, and Why

Understanding why persons with human immunodeficiency virus (HIV) have accelerated atherosclerosis and its sequelae, including coronary artery disease (CAD) and myocardial infarction, is necessary to provide appropriate care to a large and aging population with HIV. In this review, we delineate the diverse pathophysiologies underlying HIV-associated CAD and discuss how these are implicated in the clinical manifestations of CAD among persons with HIV. Several factors contribute to HIV-associated CAD, with chronic inflammation and immune activation likely representing the primary drivers. Increased monocyte activation, inflammation, and hyperlipidemia present in chronic HIV infection also mirror the pathophysiology of plaque rupture. Furthermore, mechanisms central to plaque erosion, such as activation of toll-like receptor 2 and formation of neutrophil extracellular traps, are also abundant in HIV. In addition to inflammation and immune activation in general, persons with HIV have a higher prevalence than uninfected persons of traditional cardiovascular risk factors, including dyslipidemia, hypertension, insulin resistance, and tobacco use. Antiretroviral therapies, although clearly necessary for HIV treatment and survival, have had varied effects on CAD, but newer generation regimens have reduced cardiovascular toxicities. From a clinical standpoint, this mix of risk factors is implicated in earlier CAD among persons with HIV than uninfected persons; whether the distribution and underlying plaque content of CAD for persons with HIV differs considerably from uninfected persons has not been definitively studied. Furthermore, the role of cardiovascular risk estimators in HIV remains unclear, as does the role of traditional and emerging therapies; no trials of CAD therapies powered to detect clinical events have been completed among persons with HIV.     

Women and Peripheral Arterial Disease: A Review of Sex Differences in Epidemiology,Clinical Manifestations,and Outcomes

Cardiovascular disease (CVD) is a leading cause of morbidity and mortality in women. Peripheral arterial disease (PAD), a manifestation of CVD and a marker for other adverse CVD outcomes such as ischemic heart disease and stroke, remains underrecognized and undertreated in women. Contrary to the previous belief that PAD is mainly a disease of white men, contemporary data suggest equal, if not higher, prevalence rates in nonwhite women. Women often present with asymptomatic or atypical disease and seek medical attention with more advanced disease. Cardiovascular morbidity and mortality rates, as well as procedural mortality rates, remain elevated in women compared with men. There are sex-specific markers and comorbidities with a higher female prevalence that are associated with PAD. Greater focus on PAD in cardiovascular trials, equivalent enrollment of women in large trials, and focused prevention strategies may help reduce the economic burden and adverse outcomes associated with PAD in women.