A 17-year-old male elite athlete presented for evaluation after an abnormal pre-competitive college screening electrocardiogram. Subsequent evaluation revealed the presence of hypertrophic cardiomyopathy. He remained asymptomatic throughout four years of follow-up. Through shared decision making, he continued to play competitively and is now a professional athlete. (Level of Difficulty: Advanced.).

UNASSIGNED: To investigate the in-hospital outcomes of acute ischemic stroke in patients with hypertrophic cardiomyopathy (HCM).
UNASSIGNED: Using weighted discharge data from the National Inpatient Sample, we identified 5804 nonelective hospitalizations for ischemic stroke in adult patients with HCM between 2011 and 2017. For comparison, 58,179 hospitalizations for ischemic stroke in adult patients without HCM were selected as controls using the simple random sampling method.
UNASSIGNED: Compared with the patients without HCM, those with HCM had a higher prevalence of hyperlipidemia (62.4% vs 57.5%, respectively, P<.001) and chronic heart failure (25.4% vs 13.6%, respectively, P<.001) but a lower prevalence of diabetes (28.2% vs 34.9%, respectively, P<.001) and hypertension (42.9% vs 53.4%, respectively, P<.001). Atrial fibrillation was documented in 45.1% (n=2617) of the patients with HCM. However, only 28.0% (n=733) of these patients had long-term use of anticoagulants. The in-hospital death rate among the patients with HCM was 6.3% (n=368), which was significantly higher than that in the patients without HCM (4.1%, P<.001). Having HCM (odds ratio [OR], 1.35; P<.001), atrial fibrillation (OR, 2.08; P<.001), and chronic heart failure (OR, 1.65; P<.001) were significant predictors of in-hospital death. In patients with HCM who were discharged alive, 50.0% were transferred to skilled nursing facilities compared with 45.3% of those without HCM (P<.001).
UNASSIGNED: The prognosis of acute ischemic stroke is worse in patients with HCM than in those without HCM. These findings emphasize the importance of aggressive treatment of predisposing factors for stroke in patients with HCM, especially atrial fibrillation.

An accelerated idioventricular rhythm was seen on a routine preparticipation electrocardiogram of a 19-year-old healthy and symptom-free athlete. Family history was negative for cardiac disease. Additional investigations revealed a hypertrophic cardiomyopathy, confirmed with cardiac magnetic resonance imaging and genetic analysis. Accelerated idioventricular rhythm in young athletes warrants careful clinical evaluation. (Level of Difficulty: Advanced.).

Danon disease (DD) is a rare, X-linked genetic disorder caused by LAMP2 deficiency. Clinical phenotype involves early cardiomyopathy development along with pre-excitation, skeletal myopathy, retinopathy, and cognitive impairment. We highlight how a noninvasive diagnostic approach based on clinical and imaging red flags for DD can be employed to raise high clinical suspicion for DD, which was confirmed by genetic testing results. (Level of Difficulty: Intermediate.).

Patients with hypertrophic cardiomyopathy (HCM) typically have septal hypertrophy and left ventricular outflow tract gradient, usually present at rest with increase under certain hemodynamic conditions. We report 2 cases of HCM in which there was subtle septal hypertrophy; the gradient was detected only postprandially, highlighting the importance of considering postprandial imaging in patients with suspected HCM. (Level of Difficulty: Intermediate.).

Eosinophilic myocarditis (EM) is a cardiac manifestation of hypereosinophilic syndrome with a high mortality rate. EM shares imaging features similar to other restrictive cardiopathies, and include patchy intramural late gadolinium enhancement on cardiac magnetic resonance with or without presence of biventricular thrombus. Diagnosis is confirmed on histopathology, and is the current gold standard. Here we report clinical presentation and imaging findings of EM in a 70-year-old woman who presented with fever and chills.

UNASSIGNED: The purpose of this study is to determine whether or not left ventricular remodeling can be induced after septal myectomy in patients with obstructive hypertrophic cardiomyopathy, and if so, how it occurs, using gated cardiac computed tomography.
UNASSIGNED: Fifty patients with hypertrophic obstructive cardiomyopathy who underwent septal myectomy along the septal band between March 2016 and July 2020 were retrospectively reviewed. Recent consecutive 19 patients underwent postoperative cardiac computed tomography. In these patients, volumes of the septal band and thickness of 17 left ventricular myocardial segments were measured to determine the changes after surgery.
UNASSIGNED: The resection volume predicted by preoperative computed tomography and the actual resection volume were 6.7 ± 3.3 mL and 6.4 ± 2.7 mL. In-hospital mortality was 0%. Moderate or greater mitral valve regurgitation and systolic anterior motion decreased from 56% to 6% and 86% to 6%, respectively. Median preoperative ventricular septal thickness and left ventricular outflow tract pressure gradient at rest decreased from 20.0 mm (interquartile range, 17.0-24.0 mm) and 74.0 mm Hg (interquartile range, 42.5-92.5 mm Hg) to 14.0 mm (interquartile range, 11.5-16.0 mm) and 15.5 mm Hg (interquartile range, 12.1-21.5 mm Hg), respectively. Postoperative computed tomography confirmed a reduction in septal band volume of 5.7 ± 2.8 mL. Total left ventricular myocardial volume was reduced by 12.9 ± 8.8 mL, which exceeded the volume reduction of the resected septal band. All segments except the basal inferior and basal inferolateral regions showed a significant decrease in wall thickness by a median of 6.4%.
UNASSIGNED: Properly performed septal myectomy may induce remodeling of the entire left ventricle, not just the resected area.

We describe the case of a 5-year-old girl with genetically confirmed LEOPARD syndrome (LS) who presented with multiple lentigines, ocular hypertelorism, retardation of growth, myocardial hypertrophy, and diffuse coronary artery dilatation. This case highlights the importance of multimodality imaging for the assessment of LS-associated cardiovascular alterations and follow-up. (Level of Difficulty: Intermediate.).

This phase 1, randomized, double-blind, placebo-controlled study of aficamten (formerly CK-3773274) in healthy adults identified a pharmacologically active range of doses and exposures. At doses that were pharmacologically active (single doses of ≤50 mg or daily dosing of ≤10 mg for 14 or 17 days), aficamten appeared to be safe and well tolerated. Adverse events were generally mild and no more frequent than with placebo. Pharmacokinetic assessments showed dose proportionality over the range of single doses administered, and pharmacokinetics were not affected by administration with food or in otherwise healthy individuals with a cytochrome P450 2D6 poor metabolizer phenotype. (A Single and Multiple Ascending Dose Study of CK-3773274 in Health Adult Subjects; NCT03767855).

UNASSIGNED: The pedicled greater omentum, when applied onto stressed hearts using omentopexy, has been shown to be protective in humans and animals. The mechanisms underlying cardioprotection using omentopexy remain elusive. This study examined whether macrophage-mediated angiogenesis accounts for the cardioprotective effect of omentopexy in mice.
UNASSIGNED: C57BL/6 mice were subjected to minimally invasive transverse aortic constriction for 6 weeks and subsequent cardio-omentopexy for 8 weeks. Control mice underwent the same surgical procedures without aortic constriction or cardio-omentopexy.
UNASSIGNED: Transverse aortic constriction led to left ventricular concentric hypertrophy, reduced mitral E/A ratio, increased cardiomyocyte size, and myocardial fibrosis in the mice that underwent sham cardio-omentopexy surgery. The negative effects of transverse aortic constriction were prevented by cardio-omentopexy. Myocardial microvessel density was elevated in the mice that underwent aortic constriction and sham cardio-omentopexy surgery, and cardio-omentopexy further enhanced angiogenesis. Nanostring gene array analysis uncovered the activation of angiogenesis gene networks by cardio-omentopexy. Flow cytometric analysis revealed that cardio-omentopexy triggered the accumulation of cardiac MHCIIloLyve1+TimD4+ (Major histocompatibility complex class IIlow lymphatic vessel endothelial hyaluronan receptor 1+ T cell immunoglobulin and mucin domain conataining 4+) resident macrophages at the omental-cardiac interface. Intriguingly, the depletion of macrophages with clodronate-liposome resulted in the failure of cardio-omentopexy to protect the heart and promote angiogenesis.
UNASSIGNED: Cardio-omentopexy protects the heart from pressure overload-elicited left ventricular hypertrophy and dysfunction by promoting myocardial angiogenesis. Cardiac MHCIIloLyve1+TimD4+ resident macrophages play a critical role in the cardioprotective effect and angiogenesis of cardio-omentopexy.
UNASSIGNED: