Transitioning disopyramide to mavacamten in obstructive hypertrophic cardiomyopathy: A case series and clinical guide.

Background: Hypertrophic cardiomyopathy (HCM) is a genetic disorder for which first-line treatments for obstructive HCM (oHCM) include beta-blockers, non-dihydropyridine calcium channel blockers, and disopyramide for refractory cases. Mavacamten, a selective cardiac myosin inhibitor, is indicated for symptomatic oHCM to improve functional capacity and symptoms. Use of disopyramide and mavacamten together is not recommended due to concerns of additive negative inotropic effects.

Heart-liver-kidney transplantation for AL amyloidosis using normothermic recovery and storage from a donor following circulatory death: Short-term outcome in a first-in-world experience.

AL amyloidosis is a rare condition characterized by the overproduction of an unstable free light chain, protein misfolding and aggregation, and extracellular deposition that can progress to multiorgan involvement and failure. To our knowledge, this is the first worldwide report to describe triple organ transplantation for AL amyloidosis and triple organ transplantation using thoracoabdominal normothermic regional perfusion recovery with a donation from a circulatory death (DCD) donor. The recipient was a 40-year-old man with multiorgan AL amyloidosis with a terminal prognosis without multiorgan transplantation.

Thin filament cardiomyopathies: A review of genetics, disease mechanisms, and emerging therapeutics.

All muscle contraction occurs due to the cyclical interaction between sarcomeric thin and thick filament proteins within the myocyte. The thin filament consists of the proteins actin, tropomyosin, Troponin C, Troponin I, and Troponin T. Mutations in these proteins can result in various forms of cardiomyopathy, including hypertrophic, restrictive, and dilated phenotypes and account for as many as 30% of all cases of inherited cardiomyopathy.

The Impact of Health Care Disparities on Patients With Heart Failure.

Heart failure (HF) remains a condition associated with high morbidity, mortality, and associated costs. Although the number of medical and device-based therapies available to treat HF are expanding at a remarkable rate, disparities in the risk for incident HF and treatments delivered to patients are also of growing concern. These disparities span across racial and ethnic groups, socioeconomic status, and apply across the spectrum of HF from stage A to stage D.

Factors influencing transfusion-associated HLA sensitization in patients bridged to heart transplantation using ventricular assist device.

Background: Bridging heart failure patients with mechanical ventricular assist devices (VAD) enables access to transplantation. However, VAD is associated with increased risk for anti-HLA antibodies associated with rejection of subsequent allografts. Factors determining alloantibody formation in these patients remain undefined.

Heart transplantation outcomes for rheumatic heart disease: Analysis of international registry data.

Background: Rheumatic heart disease (RHD), an autoimmune sequela of Group A streptococcal infection, is a chronic valvular disease affecting 32 million people worldwide, predominantly in developing nations. As the predisposition to autoimmune sequela still remains post transplantation, our primary objective was to assess if there were differences in mortality and rejection rates.

Methods And Results: Using the International Society for Heart and Lung Transplantation (ISHLT) adult heart transplant registry, we identified 42 RHD patients who had undergone heart transplantation between 1988 and 2014.

Management of RVAD Thrombosis in Biventricular HVAD Supported Patients: Case Series.

Patients with biventricular assist devices (BIVAD) are severely critically ill compared with patients who receive left ventricular assist device (LVAD) support alone and portend a significant risk for greater adverse events including pump thrombosis (PT). Current approaches to treat PT are limited to LVAD with variable outcomes depending on the management strategy implemented. Moreover the management of PT in patients with BIVAD support, specifically to the right ventricular assist device (RVAD), is unknown.

Which advanced heart failure therapy strategy is optimal for patients over 60 years old?

Background: The optimal advanced heart failure (HF) therapy strategy for patients aged 60 or older with end-stage HF refractory to optimal medical therapy remains uncertain. This study compares outcomes of three advanced HF therapy strategies in this patient population.

Methods: A single-center retrospective study was conducted in 95 patients aged 60-73 years who had undergone isolated heart transplantation (HTx) or continuous flow left ventricular assist device (LVAD) implantation from 2010 to 2017.

Durable Biventricular Support Using Right Atrial Placement of the HeartWare HVAD.

Patients with Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) levels 1-2 who either have or are at risk for right ventricular failure face significant morbidity and mortality after continuous flow left ventricular assist device (CF-LVAD) implantation. Currently, the options for biventricular support are limited the Total Artificial Heart (TAH; CardioWest, Syncardia, Tuscon, AZ) or biventricular assist device (BiVAD), which uses bulky extracorporeal or implantable displacement pumps. We describe a successful series based on an innovative approach for biventricular support in consecutive INTERMACS levels 1-2 patients utilizing a HeartWare Ventricular Assist Device (HVAD; HeartWare, Framingham, MA) in a left ventricular (LV-HVAD) and a right atrial (RA-HVAD) configuration.

Detection and Management of Geographic Disparities in the TOPCAT Trial: Lessons Learned and Derivative Recommendations.

TOPCAT was a multinational clinical trial of 3,445 heart failure with preserved ejection fraction (HFpEF) patients that enrolled in 233 sites in six countries in North America, Eastern Europe and South America. Patients with a heart failure hospitalization in the last 12 months or an elevated B-type natriuretic peptide (BNP) were randomized to the mineralocorticoid receptor antagonist spironolactone vs. placebo.

Mechanical Circulatory Support: Current Status and Future Directions.

Advance heart failure (AHF) is a growing epidemic with high morbidity and mortality. Left ventricular assist device (LVAD) has come to offer an opportunity to improve survival and quality of life. This form of therapy however, is not free of complications and poses a challenge to apply to a broader population.

Effect of peripheral vascular disease on mortality in cardiac transplant recipients (from the United Network of Organ Sharing Database).

Peripheral vascular disease (PVD) portends increased morbidity and mortality in patients with heart failure. In those with advanced heart failure, heart transplantation (HT) is the only causative therapy to increase survival. However, little is known about the impact of symptomatic PVD on survival of HT recipients in large multicenter cohorts.

Inotropic contractile reserve can risk-stratify patients with HIV cardiomyopathy: a dobutamine stress echocardiography study.

Objectives: The purpose of this study was to assess whether inotropic contractile reserve (ICR) during dobutamine stress echocardiography (DSE) could risk-stratify patients with human immunodeficiency virus (HIV) cardiomyopathy and predict improvement of left ventricular ejection fraction (LVEF).

Background: HIV cardiomyopathy is an important cause of heart failure and death. ICR is associated with better survival and improvement of LVEF in patients with ischemic and nonischemic cardiomyopathies.

Risk stratification and prognosis of human immunodeficiency virus-infected patients with known or suspected coronary artery disease referred for stress echocardiography.

Background: Patients with human immunodeficiency virus (HIV) infection are at increased risk of accelerated coronary artery disease (CAD) and cardiovascular events. Stress echocardiography (SE) is routinely used for risk stratification and prognosis of patients with known or suspected CAD. The prognostic value of SE in this high-risk group is unknown.

Right ventricular dysfunction is a strong predictor of developing atrial fibrillation in acutely decompensated heart failure patients, ACAP-HF data analysis.

Background: Heart failure and atrial fibrillation (AFib) are the twin epidemics of modern cardiovascular disease. The incidence of new-onset AFib in acute decompensated heart failure (ADHF) patients is difficult to predict and the short- and long-term outcomes of AFib in a cohort of patients admitted with ADHF are unknown.

Methods And Results: A total of 904 patients admitted with ADHF were studied.

Selective and specific regulation of ectodomain shedding of angiotensin-converting enzyme 2 by tumor necrosis factor alpha-converting enzyme.

Angiotensin-converting enzyme 2 (ACE2) is a newly identified regulator of the renin-angiotensin system. This type I membrane-anchored protein has a catalytically active ectodomain that undergoes shedding. Tumor necrosis factor alpha-converting enzyme (TACE) has been shown to be involved in ACE2 shedding.