Edward K Kasper

Johns Hopkins Medicine | JHUSOM · Department of Medicine

Background: Though sex and race-based patterns have been described in extracardiac organ involvement of sarcoidosis, cardiac sarcoidosis (CS) specific studies are lacking. Methods: We studied CS presentation, treatment, and outcomes based on sex and race among a tertiary center cohort. Multivariable adjusted Cox proportional hazards and survival...

Cardiac sarcoidosis (CS) is an important cause of cardiomyopathy. The trajectory of left ventricular ejection fraction (LVEF) in patients with CS undergoing treatment remains unclear. Patients with CS who were treated with corticosteroids and who underwent transthoracic echocardiography were studied. Baseline characteristics, treatment, echocardiog...

Background Sarcoidosis is a systemic disease characterized by granulomatous inflammation. Cardiac involvement is associated with increased morbidity. However, differences in clinical characteristics and outcomes based on initial sarcoidosis organ manifestation in patients with cardiac sarcoidosis (CS) have not been described. Methods A retrospecti...

Background Sarcoidosis is an inflammatory granulomatous disease that can affect several organs. Cardiac involvement is increasingly prevalent. However, characteristics of patients who present de novo with cardiac sarcoidosis (CS) compared to those diagnosed with CS after prior extracardiac sarcoidosis (ECS) diagnosis have not been studied. Methods...

Background Sex differences exist in myocarditis, with male predominance. Sarcoidosis causes an inflammatory cardiomyopathy with proposed shared mechanisms to myocarditis. Sarcoidosis is typically female predominant, but most published cohorts of cardiac sarcoidosis (CS) are majority male. Sex differences in CS have not been reported. Methods A sin...

Aims: Heart failure is an increasingly recognized later stage manifestation of arrhythmogenic right ventricular cardiomyopathy (ARVC) that can require heart transplantation (HT) to appropriately treat. We aimed to study contemporary ARVC HT outcomes in a national registry. Methods and results: The United Network for Organ Sharing registry was qu...

The prevalence of sarcoidosis-related cardiomyopathy is increasing. Sarcoidosis impacts cardiac function through granulomatous infiltration of the heart, resulting in conduction disease, arrhythmia and/or heart failure. Diagnosis of cardiac sarcoidosis can be challenging and requires clinician awareness as well as differentiation from overlapping d...

Background Cardiac sarcoidosis (CS) is a major cause of morbidity and mortality in patients with systemic sarcoidosis. Steroid sparing agents are increasingly used, despite lack of randomized trials or published guidelines to direct treatment. Methods This retrospective study included 77 patients with CS treated with prednisone monotherapy (n=32)...

Background: People with autoimmune or inflammatory conditions taking immunomodulatory/suppressive medications may have higher risk of novel coronavirus disease 2019 (COVID-19). Chronic disease care has also changed for many patients, with uncertain downstream consequences. Methods: We included participants with autoimmune or inflammatory conditi...

A large study of people with autoimmune or inflammatory conditions finds that glucocorticoid exposure may increase risk of COVID-19. The study also suggests that in this population, those with pandemic-related reduced income or employment were most vulnerable to care disruptions. Downloaded from https ABSTRACT Background

Background: People with autoimmune or inflammatory conditions who take immunomodulatory/suppressive medications may have a higher risk of novel coronavirus disease 2019 (COVID-19). Chronic disease care has also changed for many patients, with uncertain downstream consequences. Objective: Assess whether COVID-19 risk is higher among those on immunom...

Introduction Cardiac sarcoidosis (CS) is an increasingly recognized cause of cardiomyopathy, but treatment data are limited. Tumor necrosis factor alpha inhibitor (TNFai) use in CS is not well described, and there may be safety concerns in heart failure (HF). Hypothesis TNFai is well tolerated and effective in treating CS. Methods Retrospective m...

Background: Cardiac sarcoidosis (CS) is an increasingly recognized cause of cardiomyopathy, however data on immunosuppressive strategies are limited. Treatment with tumor necrosis factor (TNF) alpha inhibitors is not well described; moreover, there may be heart failure (HF)-related safety concerns. Methods: Retrospective multicenter study of TNF...

Background Corticosteroids are the therapeutic mainstay in cardiac sarcoidosis (CS). However, optimal dose and duration of therapy and predictors of patient response are not known. We aimed to describe the change in left ventricular ejection fraction (LVEF) and impact of prednisone regimen on LVEF in patients with CS. Methods Patients with CS trea...

Introduction Cardiac sarcoidosis (CS) is characterized histologically by the presence of noncaseating granulomas and manifests as conduction abnormality, ventricular arrhythmia and progressive heart failure. With advances in imaging techniques, CS is being increasingly recognized as an underlying cause of cardiomyopathy. To date, there are no rando...

Background: Patients with combined post- and precapillary pulmonary hypertension due to left heart disease have a worse prognosis compared with isolated postcapillary. However, it remains unclear whether increased mortality in combined post- and precapillary pulmonary hypertension is simply a result of higher total right ventricular load. Pulmonar...

Background: Patients with end-stage cardiomyopathy due to cardiac sarcoidosis (CS) may be referred for mechanical circulatory support (MCS) and heart transplantation (HT). Here, we describe outcomes of patients with CS undergoing HT, focusing on the use of MCS as a bridge-to-transplant (BTT). Methods: Using the United Network for Organ Sharing (...

Over the last decades, beta-blockers have been a key component of heart failure therapy. However, currently there is no method to identify patients who will benefit from beta-blocking therapy versus those who will be unresponsive or worsen. Furthermore, there is an unmet need to better understand molecular mechanisms through which heart failure the...

This study sought to evaluate if diastolic pulmonary gradient (DPG) can predict survival in patients with pulmonary hypertension due to left heart disease (PH-LHD). Patients with combined post- and pre-capillary PH-LHD have worse prognosis than those with passive pulmonary hypertension. The transpulmonary gradient (TPG) and pulmonary vascular resis...

The medical profession should play a central role in evaluating the evidence related to drugs, devices, and procedures for the detection, management, and prevention of disease. When properly applied, expert analysis of available data on the benefits and risks of these therapies and procedures can

Determining the prognosis of patients with heart failure is essential for patient management and clinical trial conduct. The relative value of traditional prognostic criteria remains unclear and the assessment of long-term prognosis for individual patients is problematic. To determine the ability of clinical, hemodynamic and echocardiographic param...

Background: Heart failure (HF) remains a condition with high morbidity and mortality. We tested a telephone support strategy to reduce major events in rural and remote Australians with HF, who have limited healthcare access. Telephone support comprised an interactive telecommunication software tool (TeleWatch) with follow-up by trained cardiac nur...

It is often challenging to assess cardiac filling pressure clinically. An improved system for detecting or ruling out elevated cardiac filling pressure may help reduce hospitalizations for heart failure. The blood pressure response to the Valsalva maneuver reflects left heart filling pressure, but its underuse clinically may be due in part to lack...

Opinion statement: Investigation of the complex biochemical pathways that underlie heart failure (HF) has led to the recognition of multiple molecular markers that may help to characterize patients with this disease. Although a myriad of novel biomarkers are being studied, most attention continues to be focused on the natriuretic peptides, brain n...

Lymphocytic myocarditis is a clinically important condition that is difficult to diagnose and distinguish. We hypothesized that the transcriptome obtained from an endomyocardial biopsy would yield clinically relevant and accurate molecular signatures. Microarray analysis was performed on samples from patients with histologically proven lymphocytic...

We sought to test the hypothesis that inherent biological factors contribute to gender differences in disease pathophysiology of new-onset heart failure (HF), which can be detected from the transcriptome of a single endomyocardial biopsy (EMB). We analysed samples from male (n = 29) and female patients (n = 14) with idiopathic dilated cardiomyopath...

Beta blockers are empirically used in many patients with heart failure (HF) and preserved ejection fraction (HFpEF) because they allow more time for diastolic filling and because they improve outcomes in patients with systolic HF. However, recent data suggest that impaired chronotropic and vasodilator responses to exercise, which can worsen with be...

It is essential that the medical profession play a significant role in critically evaluating the use of diagnostic procedures and therapies as they are introduced and tested in the detection, management, or prevention of disease states. Rigorous and expert analysis of the available data documenting

A primary challenge in the development of clinical practice guidelines is keeping pace with the stream of new data on which recommendations are based. In an effort to respond more quickly to new evidence, the American College of Cardiology Foundation/American Heart Association (ACCF/AHA) Task Force on Practice Guidelines has created a “focused upda...

B-type natriuretic peptide (BNP) correlates with cardiac filling pressures and outcomes in patients with heart failure. In heart transplant recipients, we hypothesize that a within-individual change in BNP over time would be more helpful than absolute BNP in detecting International Society of Heart and Lung Transplantation (ISHLT) grade 2R or great...

This study was designed to analyze how patient preferences for survival versus quality-of-life change after hospitalization with advanced heart failure (HF). Although patient-centered care is a priority, little is known about preferences to trade length of life for quality among hospitalized patients with advanced HF, and it is not known how those...

Prediction of prognosis remains a major unmet need in new-onset heart failure (HF). Although several clinical tests are in use, none accurately distinguish between patients with poor versus excellent survival. We hypothesized that a transcriptomic signature, generated from a single endomyocardial biopsy, could serve as a novel prognostic biomarker...

Developed in Collaboration With the American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society for Vascular Medicine and Biology, and Society for Vascular Surgery; WRITING COMMITTEE MEMBERS; Fleishe...

INTRODUCTION: One of the clinical dilemmas in cardiology is to accurately diagnose the etiology of patients with new onset heart failure such as to differentiate between potentially reversible myocarditis from idiopathic dilated cardiomyopathy (IDCM). Furthermore, it is difficult to predict the clinical trajectory of patients with IDCM. We hypothes...

Connective tissue disease (CTD) encompasses several disease or abnormal states characterized by inflammatory or degenerative changes in connective tissue. These disorders affect many organs, including the heart. However, the long-term survival of patients with cardiomyopathy and CTD is not known. The prognostic implications of different categories...

Cardiac allograft vasculopathy (CAV) poses the greatest threat to the long-term survival of cardiac transplant recipients, and these individuals often exhibit elevated levels of uric acid (UA), a stimulator of T cells. We hypothesized that hyperuricemia is associated with CAV in cardiac transplant recipients. UA levels were measured in cardiac tran...

The American College of Cardiology/American Heart Association (ACC/AHA) Task Force on Practice Guidelines makes every effort to avoid any actual, potential, or perceived conflict of interest that might arise as a result of an industry relationship or personal interest of the writing committee. Specifically, all members of the writing committee, as...

Purpose of the Expedited Update: Since the publication of the previous guidelines on perioperative cardiovascular evaluation for noncardiac surgery in 2002, the issue of perioperative beta blockade for non-cardiac surgery has taken on increased importance. Specifically, the Physicians Consortium for Performance Improvement and the Surgical Care Imp...

Because of potential side effects and logistical difficulty of titrating medications, outpatients with congestive heart failure rarely receive appropriate doses of carvedilol or other beta-blockers. To address these obstacles, we studied if an automated telemedicine system named TeleWatch (TW) could facilitate carvedilol titration in outpatients wi...

Antibody-mediated rejection (AMR) in human heart transplantation is an immunopathologic process in which injury to the graft is in part the result of activation of complement and it is poorly responsive to conventional therapy. We evaluated by immunofluorescence (IF), 665 consecutive endomyocardial biopsies from 165 patients for deposits of immunog...

Sarcoidosis is a systemic granulomatous disorder of unknown etiology. In patients with cardiomyopathy, the diagnosis of sarcoidosis has important treatment implications. We studied the prognostic implications of a cardiac biopsy diagnosis of sarcoidosis in patients with unexplained cardiomyopathy. We evaluated 1235 patients with unexplained cardiom...

Pulmonary hypertension (PH) is common in severe heart failure, but the effect of mild PH on posttransplantation PH and survival after heart transplantation has not been well described. This cohort study examined preoperative and postoperative hemodynamics in 172 heart transplant recipients at Johns Hopkins Hospital followed for up to 15.1 years. PH...

Cardiac transplant recipients have been regarded as not medically fit to fly an airplane. Recently, the Federal Aviation Administration decided to re-examine this policy and, in response, this study was undertaken to determine the risk of death from any cause and sudden-onset death in heart transplant recipients during the 12 months after an annual...

A 56-year-old man with normal cardiac function received treatment with interferon alpha-2b for malignant melanoma. Eight months after the initiation of therapy he developed fatigue and dyspnea on exertion. Two months later, he was admitted to hospital with ORTHOPNEA, worsening dyspnea and cough. Physical examination findings were consistent with co...

The prevalence of congestive heart failure is increasing and the prognosis remains poor. Cardiomyopathy is one of the most frequent causes of congestive heart failure and is the most common etiology of patients submitted to heart transplant. Determination of the etiology of cardiomyopathy has both prognostic and therapeutic implications. History, p...

Polyclonal antithymocyte globulin preparations contain antibodies with reactivity to endothelial cells. Therefore, we investigated whether treatment with this reagent caused complement deposition in human cardiac transplants. Frozen tissue was available from endomyocardial biopsies of 75 patients, who were transplanted between April 1995 and April...

Of the 60,000 patients receiving heart transplants between 1982 and 2001, approximately 12,000 are currently alive. The high incidence of hyperlipidemia and coronary disease (also known as accelerated graft atherosclerosis, or AGA) in these patients warrants early prophylaxis soon after transplantation with 3-hydroxy-3-methylglutaryl (HMG) Co-A red...

The etiology of cardiomyopathy is usually inferred from clinical information and preliminary laboratory studies. Patients with unexplained cardiomyopathy may be referred for endomyocardial biopsy (EMBx). It is unknown whether pathological information obtained from EMBx is beneficial or alters the diagnosis established clinically. This study was und...

Lymphocytic myocarditis presents as a primary disorder or in association with a systemic disease. Whether primary and secondary myocarditis have the same prognosis is unknown. Patients (n = 171) referred to the Johns Hopkins Cardiomyopathy service from 1984 to 1998 with newly diagnosed cardiomyopathy were observed for an average of 5.9 years after...

Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) has major implications for the management of patients and their first-degree relatives. Diagnosis is based on a set of criteria proposed by the International Task Force for Cardiomyopathies. We report our experience in providing a re-evaluation for patients who previous...

Complement is a multifunctional system of receptors and regulators as well as effector molecules. Both the pathogenic and diagnostic power of complement is based on the capacity of the complement system to amplify innate and adaptive immunity. This amplification is accomplished through two strategies: (1) enzymatic reactions in the complement casca...

This study analyzed the utility of electrocardiographic (ECG) and echocardiographic findings in the diagnosis of amyloidosis proven by endomyocardial biopsy. Cardiac amyloidosis is associated with characteristic ECG and echocardiographic changes, yet each finding alone is relatively nonspecific. A combination of noninvasive prognostic parameters wo...

Treatment of arrhythmogenic right ventricular dysplasia (ARVD) is mostly based on the prevention of sudden cardiac death that results from arrhythmias. A clinical history suggestive of ARVD requires careful evaluation including electrophysiological study. The potential ability to identify those patients who will have inducible VT with electrophysio...

Controversy exists regarding the relation between diabetes and nonischemic idiopathic cardiomyopathy (ICM), and only limited data on the incidence of ICM in adults with diabetes are available. Therefore, we used the 1995 Nationwide Inpatient Sample (NIS) to determine discharge rates and test the hypothesis that diabetes is independently associated...

Repeated hospital readmissions are frequent and increasing over time in patients with heart failure (HF). The predictors for readmission in patients with HF are not completely understood. The study was undertaken to investigate the time course of readmission by specific cause in patients with HF, and to examine the independent effects of HF etiolog...

Peripartum cardiomyopathy is a rare and life-threatening disease of unknown etiology. This diagnosis should be limited to previously healthy women who present with congestive heart failure (CHF) and decreased left ventricular systolic function in the last month of pregnancy or within 5 months after delivery. The diagnosis is not made in the presenc...

T he classic metabolic risk factors for atherosclerosis such as hypercholesterolemia, hypertriglyceridemia, low high-density lipoprotein cholesterol, and diabetes mellitus are only weakly associated with the development of arteriosclerosis in coronary arteries of donor hearts after cardiac transplantation.(1) Similarly, multiple nonimmune factors i...

Pulmonary hypertension is a clinically useful predictor of death in patients with heart failure. Whether pulmonary hypertension has the same prognostic value among specific underlying causes of cardiomyopathy is unknown. Using a diverse cohort of cardiomyopathy patients, we tested the hypotheses that (1) elevated mean pulmonary arterial pressure is...

Chlamydia pneumoniae has been associated with atherosclerosis, although its role in the process is not clearly defined. Heart transplant recipients are known to have high titers of antibodies to C. pneumoniae, and the organism has been recovered from the coronary arteries of both transplant recipients and donors. This study evaluated association be...

We sought to determine whether a multidisciplinary outpatient management program decreases chronic heart failure (CHF) hospital readmissions and mortality over a six-month period. Hospital admission for CHF is an important problem amenable to improved outpatient management. Two hundred patients hospitalized with CHF at increased risk of hospital re...

Background. Acute cellular rejection in cardiac allografts is a major cause of graft loss, and is associated with activation of the coagulation system. We investigated whether plasma markers of coagulation predict the presence of allograft rejection. Methods. A total of 132 blood specimens and endomyocardial biopsies were collected from 35 patients...

The reported mortality rate of peripartum cardiomyopathy (PPCM) is high, although the potential for spontaneous recovery of ventricular function is well established. The prevalence of myocarditis in PPCM has varied widely between studies. The purposes of this study were to define the long-term prognosis in a referral population of patients with PPC...

S108 The management of patients with heart failure due to left ventricular systolic dys- function has markedly changed during the past 15 years. Since 1985, angiotensin-con- verting enzyme (ACE)-inhibitors and beta- blockers have been shown to reduce mor- tality. Several medications have also been shown to increase mortality. Combined, these two fi...

We sought to use echocardiography to assess the presentation and potential for recovery of left ventricular (LV) function of patients with fulminant myocarditis compared with those with acute myocarditis. The clinical course of patients with myocarditis remains poorly defined. We have previously proposed a classification that provides prognostic in...

Previous studies of the prognosis of patients with heart failure due to cardiomyopathy categorized patients according to whether they had ischemic or nonischemic disease. The prognostic value of identifying more specific underlying causes of cardiomyopathy is unknown. We evaluated the outcomes of 1230 patients with cardiomyopathy. The patients were...

Lymphocytic myocarditis causes left ventricular dysfunction that may be persistent or reversible. There are no clinical criteria that predict which patients will recover ventricular function and which cases will progress to dilated cardiomyopathy. We hypothesized that patients with fulminant myocarditis may have a better long-term prognosis than th...

To determine the short-term safety and tolerability of the addition of ecadotril to conventional therapy in patients with mild to moderate heart failure. Fifty ambulatory patients, 18 to 75 years of age, with mild to moderate heart failure, left ventricular ejection fraction </=35%, taking stable doses of angiotensin-converting enzyme inhibitor, di...

Prolonged warm or cold ischemia is associated with poor survival of cardiac transplants, and ischemic changes in early posttransplantation endomyocardial biopsies correlate with the later development of chronic rejection. In animal models, tissue ischemia has been shown to activate complement. To determine whether ischemic changes in endomyocardial...

This report describes the evaluation of 1,278 patients referred to The Johns Hopkins Hospital with dilated cardiomyopathy. After a careful history and physical examination, selected laboratory tests, and endomyocardial biopsy, a specific diagnosis was made in 49% of cases. In 16% of cases the biopsy demonstrated a specific histologic diagnosis. Myo...

Successful strategies for avoiding obesity and hypercholesterolemia are difficult to validate because of imprecise problem identification. The purpose of this study was to describe the incidence, severity, and onset of obesity and hypercholesterolemia among heart transplant recipients and identify relationships between demographic variables and wei...

Primary amyloidosis, systemic senile amyloidosis, isolated atrial amyloidosis, and transthyretin isoleucine 122 amyloidosis frequently involve the heart. Amyloid fibrils infiltrate the myocardium, impairing ventricular contraction and relaxation. The clinical manifestations of cardiac infiltration in these disorders are protean, though congestive h...

Quilty lesions, as first described by Billingham in 1981, or ‘Quilty Effect’ (QE) are distinct endomyocardial mononuclear cell infiltrates that have been observed in human heart transplant recipients, as well as in experimental models of heart transplantation. In the present investigations, the pattern and extent of apoptosis (programmed cell death...

Dilated cardiomyopathy (DCM) is associated with a high incidence of malignant ventricular arrhythmias and sudden death. Abnormalities in repolarization of ventricular myocardium have been implicated in the development of these arrhythmias. Spatial heterogeneity in repolarization has been studied in DCM, but temporal fluctuations in repolarization i...

Vesnarinone is a novel oral agent that improves survival and symptoms of patients with dilated cardiomyopathy. Although it is thought to have positive inotropic effects, clinical data supporting this mechanism in patients with severe heart failure remain scant. The present study tested whether 3 months of oral vesnarinone therapy increases the inot...

It has been suggested that the interaction of cytomegalovirus (CMV) with the p53 tumor suppressor gene product plays a role in the development of coronary artery restenosis after angioplasty. CMV nucleic acids have been observed in the coronary arteries of allografted hearts, suggesting a possible role for the interaction of CMV with p53 in the dev...

Bundle branch reentry and interfascicular reentry are potential mechanisms of ventricular tachycardia in the setting of a dilated cardiomyopathy. We report a patient with myotonic dystrophy who presented with near syncope, in whom both of these mechanisms were present, leading to two different wide complex tachycardias. Electrophysiologic study dem...