Long-Term Safety and Efficacy of Mavacamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Interim Results of the PIONEER-OLE Study.

Background: The phase 2 PIONEER-HCM (Phase 2 Open-label Pilot Study Evaluating Mavacamten in Subjects With Symptomatic Hypertrophic Cardiomyopathy and Left Ventricular Outflow Tract Obstruction) study showed that mavacamten improved left ventricular outflow tract gradients, exercise capacity, and symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM), but the results of longer-term treatment are less well described. We report interim results from the PIONEER-OLE (PIONEER Open-Label Extension) study, the longest-term study of mavacamten in patients with symptomatic obstructive HCM.

Methods And Results: Patients who previously completed PIONEER-HCM (n=20) were eligible to enroll in PIONEER-OLE.

Response to letter to editor, re-predictors of morbidity and in-hospital mortality following procedure-related cardiac tamponade.

We had included only the patients with cardiac tamponade, excluded those coded for pericardial effusion. The feasibility of comparison of the databases of two regions needs to be evaluated. There are some inherent limitations for the studies carried out from the National In-patient Samples (NIS) database.

Chronic kidney disease and left ventricular diastolic dysfunction (CKD-LVDD) alter cardiac expression of mitochondria-related genes in swine.

Cardiovascular disease and heart failure doubles in patients with chronic kidney disease (CKD), but the underlying mechanisms remain obscure. Mitochondria are central to maintaining cellular respiration and modulating cardiomyocyte function. We took advantage of our novel swine model of CKD and left ventricular diastolic dysfunction (CKD-LVDD) to investigate the expression of mitochondria-related genes and potential mechanisms regulating their expression.

Mitral Annulus Calcium Score in Patients With Calcific Mitral Stenosis Undergoing Invasive Hemodynamic Assessment.

Background: Aortic valve calcium score is associated with hemodynamic severity of aortic stenosis. Whether this association is present in calcific mitral stenosis remains unknown.

Methods And Results: This study was a retrospective analysis of consecutive patients with mitral stenosis secondary to mitral annular calcification (MAC) undergoing transseptal catheterization.

Predictors of morbidity and in-hospital mortality following procedure-related cardiac tamponade.

Background: Cardiac tamponade (CT) can be a complication following invasive cardiac procedures. We assessed CT following common cardiac electrophysiology (EP) procedures to facilitate risk prediction of associated morbidity and in-hospital mortality.

Methods: Patients who underwent various EP procedures in the cardiac catheterization lab (ablations and device implantations) were identified using the International Classification of Diseases, Ninth and Tenth Edition, Clinical Modification (ICD-9-CM and ICD-10-CM, respectively) from the Nationwide Inpatient Sample (NIS) database.

Heart failure is associated with increased risk of all-cause mortality after transvenous lead extraction: A systematic review and meta-analysis.

Background: Transvenous lead extraction (TLE) is increasingly considered in cardiac implantable electronic device management. Heart failure (HF) might be associated with mortality risks after the TLE procedure. This study aims to assess mortality risk in HF patients undergoing TLE.

Intrahepatic transcriptomics differentiate advanced fibrosis and clinical outcomes in adults with the Fontan circulation.

Background: The molecular mechanisms underlying Fontan associated liver disease (FALD) remain largely unknown. We aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes.

Methods: This retrospective cohort study included adults with the Fontan circulation at the Ahmanson/UCLA Adult Congenital Heart Disease Center.

Erratum: Health disparities and treatment approaches in portopulmonary hypertension and idiopathic pulmonary arterial hypertension: An analysis of the Pulmonary Hypertension Association Registry.

[This corrects the article DOI: 10.1177/20458940211020913.].

Cardiac Resynchronization Therapy for Adult Patients With a Failing Systemic Right Ventricle: A Multicenter Study.

Background The objective of this international multicenter study was to investigate both early and late outcomes of cardiac resynchronization therapy (CRT) in patients with a systemic right ventricle (SRV) and to identify predictors for congestive heart failure readmissions and mortality. Methods and Results This retrospective international multicenter study included 13 centers. The study population comprised 80 adult patients with SRV (48.

Frequency of Arrhythmias and Postural Orthostatic Tachycardia Syndrome in Patients With Marfan Syndrome: A Nationwide Inpatient Study.

Background Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder affecting multiple systems, particularly the cardiovascular system. The leading causes of death in MFS are aortopathies and valvular disease. We wanted to identify the frequency of arrhythmia and postural orthostatic tachycardia syndrome, length of hospital stay, health care-associated costs (HAC), and in-hospital mortality in patients with MFS.

Incidence of Preclinical Heart Failure in a Community Population.

Background A high prevalence of preclinical heart failure (HF) (Stages A and B) has previously been shown. The aim of this study was to explore factors associated with the incidence of preclinical HF in a community population. Methods and Results Retrospective review of 393 healthy community individuals aged ≥45 years from the Olmsted County Heart Function Study that returned for 2 visits, 4 years apart.

Invasive Hemodynamic Predictors of Survival in Patients With Mitral Stenosis Secondary to Mitral Annular Calcification.

Background The aim of this study was to establish prognostic hemodynamic parameters in patients with mitral stenosis secondary to mitral annular calcification. Methods and Results A retrospective cohort of 105 patients undergoing transseptal catheterization for hemodynamic evaluation of mitral annular calcification-related mitral stenosis between 2004 and 2020 was studied. Mitral valve gradient (MVG) and mitral valve area (MVA; calculated by the Gorlin formula) were measured using direct left atrial and left ventricular pressures.

Effect of Shared Decision-Making for Stroke Prevention on Treatment Adherence and Safety Outcomes in Patients With Atrial Fibrillation: A Randomized Clinical Trial.

Background Guidelines promote shared decision-making (SDM) for anticoagulation in patients with atrial fibrillation. We recently showed that adding a within-encounter SDM tool to usual care (UC) increases patient involvement in decision-making and clinician satisfaction, without affecting encounter length. We aimed to estimate the extent to which use of an SDM tool changed adherence to the decided care plan and clinical safety end points.

Outcomes of Elderly Patients Undergoing Left Atrial Appendage Closure.

Background Elderly patients have a higher burden of comorbidities that influence clinical outcomes. We aimed to compare in-hospital outcomes in patients ≥80 years old to younger patients, and to determine the factors associated with increased risk of major adverse events (MAE) after left atrial appendage closure. Methods and Results The National Inpatient Sample was used to identify discharges after left atrial appendage closure between October 2015 and December 2018.

The Doppler shock index measured by a wearable ultrasound patch accurately detects moderate-to-severe central hypovolemia during lower body negative pressure.

Objective: Moderate-to-severe hemorrhage is a life-threatening condition, which is challenging to detect in a timely fashion using traditional vital signs because of the human body's robust physiologic compensatory mechanisms. Measuring and trending blood flow could improve diagnosis of clinically significant exsanguination. A lightweight, wireless, wearable Doppler ultrasound patch that captures and trends blood flow velocity could enhance hemorrhage detection.

Simultaneous Measurement of Lung Diffusing Capacity and Pulmonary Hemodynamics Reveals Exertional Alveolar-Capillary Dysfunction in Heart Failure With Preserved Ejection Fraction.

Background Hemodynamic perturbations in heart failure with preserved ejection fraction (HFpEF) may alter the distribution of blood in the lungs, impair gas transfer from the alveoli into the pulmonary capillaries, and reduce lung diffusing capacity. We hypothesized that impairments in lung diffusing capacity for carbon monoxide (DL) in HFpEF would be associated with high mean pulmonary capillary wedge pressures during exercise. Methods and Results Rebreathe DL and invasive hemodynamics were measured simultaneously during exercise in patients with exertional dyspnea.

Impact of Atrial Fibrillation on Outcome in Takotsubo Syndrome: Data From the International Takotsubo Registry.

Background Atrial fibrillation (AF) is a major risk factor for mortality. The prevalence, clinical correlates, and prognostic impact of AF in Takotsubo syndrome (TTS) have not yet been investigated in a large patient cohort. This study aimed to investigate the prevalence, clinical correlates, and prognostic impact of AF in patients with TTS.

Predictors of Use and Outcomes of Mechanical Valve Replacement in the United States (2008-2017).

Background Contemporary nationwide data on the use, predictors, and outcomes of mechanical valve replacement in patients less than 70 years of age are limited. Methods and Results We identified hospitalizations for aortic valve replacement (AVR) or mitral valve replacement (MVR) in the Nationwide Inpatient Sample between January 1, 2008, and December 31, 2017. The study's end points included predictors of mechanical valve replacement and risk-adjusted in-hospital mortality.

Long-Term Survival of Patients With Left Ventricular Noncompaction.

Background The prognosis of left ventricular noncompaction (LVNC) remains elusive despite its recognition as a clinical entity for >30 years. We sought to identify clinical and imaging characteristics and risk factors for mortality in patients with LVNC. Methods and Results 339 adults with LVNC seen between 2000 and 2016 were identified.

Determinants of Morbidity and Mortality Associated With Isolated Tricuspid Valve Surgery.

Background Whether the poor outcomes of isolated tricuspid valve surgery are related to the operation itself or to certain patient characteristics including late referral is unknown. Methods and Results Adult patients who underwent isolated tricuspid valve surgery were identified in the Nationwide Readmissions Database (2016-2017). Patients who had redo tricuspid valve surgery, endocarditis, or congenital heart disease were excluded.

Impact of Sirolimus as a Primary Immunosuppressant on Myocardial Fibrosis and Diastolic Function Following Heart Transplantation.

Background Myocardial fibrosis is an important contributor for development of diastolic dysfunction. We investigated the impact of sirolimus as primary immunosuppression on diastolic dysfunction and fibrosis progression among heart transplantation recipients. Methods and Results In 100 heart transplantation recipients who were either treated with a calcineurin inhibitor (CNI) (n=51) or converted from CNI to sirolimus (n=49), diastolic function parameters were assessed using serial echocardiograms and right heart catheterizations.

Ceramides and Ceramide Scores: Clinical Applications for Cardiometabolic Risk Stratification.

Ceramides are bioactive lipids that have an important role in many cellular functions such as apoptosis and inflammation. During the past decade emerging clinical data have shown that ceramides are not only of great biochemical interest but may also have diagnostic utility. Ceramides have shown independent predictive value for negative cardiovascular outcomes as well as for the onset of type 2 diabetes.

Temporal Trends in the Incidence and Outcomes of Pacemaker Implantation After Transcatheter Aortic Valve Replacement in the United States (2012-2017).

Background Nationwide studies documenting temporal trends in permanent pacemaker implantation (PPMI) following transcatheter aortic valve replacement (TAVR) are limited. Methods and Results We selected patients who underwent TAVR between 2012 and 2017 in the National Readmission Database. The primary end point was the 6-year trend in post-TAVR PPMI at index hospitalization and at 30, 90, and 180 days after discharge.