Cardiac Transplantation in Danon Disease.

BACKGROUND: Danon disease (DD) is a rare X-linked dominant cardioskeletal myopathy caused by mutations in the lysosome-associated membrane protein-2 (LAMP-2) gene that is usually lethal without cardiac transplantation. The purpose of this study was to characterize post-transplant outcomes in a large cohort of patients with DD who underwent cardiac transplantation. METHODS: The clinical phenotype and outcome data of patients with DD who underwent cardiac transplantation (n = 38; 19 males and 19 females) were obtained from 8 centers. Study outcomes included graft survival, defined as death or retransplantation, and episodes of acute cellular and antibody-mediated rejection and cardiac allograft vasculopathy at 1 year. RESULTS: Median follow-up time after transplantation for the entire cohort was 4.4 years (IQR: 1.5-12.8 years). The median age at transplant for the cohort was 20.2 years (15.8-27.9 years), with no difference in age between sexes. Median pretransplant left-ventricular ejection fraction for the entire cohort was 30% (range 11%-84%). Males had higher pretransplant aspartate aminotransferase, alanine aminotransferase and creatine phosphokinase levels than females (P < 0.001). There were 2 deaths in the entire cohort and 2 retransplants. There was no difference in actuarial graft survival between males and females (P = 0.8965); the estimated graft survival was 87.1% (95%CI: 63.6%-95.9%) at 5 years. One episode (2.7%) of antibody-mediated rejection, grade 2, and 7 episodes (19%) of acute cellular rejection, grade 2 or 3, were reported in patients who survived to discharge (6 females and 1 male; P = 0.172). CONCLUSIONS: Heart transplantation outcomes are acceptable in DD with high probabilities of 5-year graft survival for males and females suggesting that cardiac transplantation is an effective treatment option for DD patients.

Myocarditis in the Athlete: A Focus on COVID-19 Sequelae.

Myocarditis is a leading cause of sudden death in athletes. Early data demonstrating increased prevalence of cardiac injury in hospitalized patients with COVID-19 raised concerns for athletes recovered from COVID-19 and the possibility of underlying myocarditis. However, subsequent large registries have provided reassuring data affirming low prevalence of myocarditis in athletes convalesced from COVID-19. Although the clinical significance of subclinical myocarditis detected by cardiac MRI remains uncertain, clinical outcomes have not demonstrated an increase in acute cardiac events in athletes throughout the pandemic. Future directions include defining mechanisms underlying "long-haul" COVID-19 and the potential impact of new viral variants.

Mitigating clinical waste in the trauma intensive care unit: Limited clinical utility of cardiac troponin testing for trauma patients with atrial fibrillation.

BACKGROUND: The clinical significance of obtaining cardiac troponin (cTn) levels among trauma patients with new onset arrhythmias is unknown. We aimed to assess whether cTn levels actually influence clinical decision making or represent an inappropriate use of resources. METHODS: Trauma patients admitted from 2013 to 2014 diagnosed with atrial fibrillation (AF) were retrospectively reviewed using the institutional trauma database. Demographics, cTn levels, and myocardial infarction (MI) diagnosis data were recorded. Standard univariate tests were used to compare data between patients with and without cTn. RESULTS: There were 258 patients included of which 126 patients had cTn levels obtained (48.8%, TEST group). The remaining 132 patients (51.2%) were untested (noTEST group). Among TEST patients, use of echocardiography nearly doubled and cardiology consultations increased (all p < 0.05). No TEST patients suffered MI or PE. CONCLUSIONS: Obtaining cTn values in trauma patients with new-onset AF resulted in increased resource utilization without clinical utility.

Improvement in the assessment of diastolic function in a clinical echocardiography laboratory following implementation of a quality improvement initiative.

BACKGROUND: The aim of this study was to demonstrate improvement in the characterization of diastolic function in the routine practice of a clinical echocardiography laboratory after the implementation of a quality improvement initiative. The echocardiographic analysis of left ventricular (LV) diastolic dysfunction is an inherently complex process involving the integration of multiple indices for accurate assessment. METHODS: A baseline survey of 50 randomly chosen echocardiographic studies was reviewed for the accuracy of diastolic function assessment. A four-step quality improvement protocol was then initiated: (1) sonographer and physician education; (2) the implementation of data acquisition protocol changes using LV inflow, tissue Doppler velocity of the mitral annulus in early diastole (e'), flow propagation velocity of LV inflow (Vp), and left atrial volume index (LAVI), along with the establishment of uniform criteria for diagnostic interpretation; (3) peer review of performance; and (4) focused interactive case review sessions. RESULTS: At baseline, measurements of LV inflow were most often correct (100% accurate), while measurements of e' (82% accurate), Vp (12% accurate), and LAVI (12% accurate) and the proper classification of diastolic function (44% accurate) were significantly limited. After the quality improvement initiative, there were significant increases in the accuracy of all recorded measurements, with e' 92% accurate (a 10% improvement; P < .10), Vp 67% accurate (a 55% improvement; P < .001), LAVI 80% accurate (a 68% improvement, P < .001), and proper characterization of diastolic function 76% accurate (a 32% improvement, P < .001). CONCLUSIONS: A multifaceted quality improvement protocol including staff education, systematic support with enhanced infrastructure, and peer review with feedback can be effective for improving the clinical performance of a nonacademic echocardiography laboratory in the characterization of diastolic function.

Recurrent, severe, and rapidly reversible apical ballooning syndrome in status asthmaticus.

Apical ballooning syndrome, or Tako-tsubo cardiomyopathy, is a peculiar form of transient left-ventricular dysfunction originally described as triggered by emotional stress. Subsequent reports indicated that physical stressors can also induce this clinical syndrome. We describe for the first time, to the best of our knowledge, a case of recurrent, severe, and quickly reversible apical ballooning syndrome provoked by the use of high-dose inhaled ß-adrenergic agonists in status asthmaticus.