Hyperpolarized-MRI in Hypertrophic Cardiomyopathy: A Narrative Review.

Hypertrophic cardiomyopathy is a genetically inherited cardiac disorder that presents with diverse clinical phenotypes. It is associated with significant adverse outcomes, including arrhythmias and sudden cardiac death. Current gold-standard diagnostic methods include echocardiography and cardiac magnetic resonance imaging.

The Winding Road to Dyspnea: A Case Report of an Unusual Presentation of Anomalous Left Coronary Artery from the Pulmonary Artery.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. Such patients are unlikely to survive adulthood without a major surgical correction. We report a 30-year-old female with a lifelong murmur who presented to the sports cardiology clinic with progressively reduced exercise tolerance.

A Case Report: The Utility of Multimodality Imaging in the Diagnosis of Cardiac Sarcoidosis-Has It Surpassed the Need for a Biopsy?

Cardiac sarcoidosis (CS) is a rare but life-threatening disorder, occurring in 2-5% of sarcoidosis cases, though post-mortem studies suggest a higher prevalence. It presents diagnostic challenges due to nonspecific symptoms and the low sensitivity of an endomyocardial biopsy. Recent guidelines emphasize multimodal imaging, such as cardiac magnetic resonance imaging (MRI) and positron emission tomography (PET).

Unmasking an Intracardiac Shunt in a Case of Persistent Unexplained Hypoxia: A Case Report.

: Pulmonary hypertension (PH) is characterized by an increase in mean pulmonary arterial pressure and pulmonary vascular resistance. It is frequently encountered in patients with significant intracardiac shunts, often necessitating the implementation of a closure device or surgical correction. Nevertheless, the occurrence of a concomitant atrial septal defect (ASD) with a right-to-left shunt inducing left ventricular dysfunction is a rare phenomenon.

Unraveling Mitral Annular Disjunction: A Case Report of Ventricular Arrhythmia Detected via Smartwatch.

Mitral valve prolapse (MVP) is commonly benign, but may result in life-threatening arrhythmias and sudden cardiac death (SCD). Mitral annular disjunction (MAD) often coexists with mitral valve prolapse (MVP) and has been implicated in the development of ventricular arrhythmias through myocardial stretch and fibrosis. Here, we present a case that highlights the diagnostic value of multimodal imaging in evaluating ventricular ectopy in the context of MVP and MAD.

The Utility of Nuclear Imaging in Hypertrophic Cardiomyopathy: A Narrative Review.

Hypertrophic cardiomyopathy (HCM) is the most common genetically inherited cardiac condition, characterized by clinical heterogeneity and a significantly increased risk of adverse cardiovascular outcomes, including sudden cardiac death. Current diagnostic methods primarily use echocardiography, often supplemented by cardiovascular magnetic resonance imaging (MRI), to assess the clinical profile of the disorder. Effective risk stratification protocols are essential for managing patients with HCM.

Role of Multimodality Imaging in Cardiac Sarcoidosis: A Retrospective Single-Center Experience.

Cardiac sarcoidosis (CS) is a rare entity characterized by granulomatous infiltration of the myocardium, which can lead to myocardial fibrosis, conduction abnormalities, and the development of heart failure, thereby elevating the risk of sudden cardiac death (SCD). While endomyocardial biopsy (EMBx) is regarded as the gold standard for diagnosis, its low sensitivity and inherent procedural risks may limit its practical application. This study retrospectively explored the role of advanced imaging modalities, specifically cardiovascular magnetic resonance imaging (CMR) and fluorodeoxyglucose positron emission tomography (FDG-PET), in the diagnosis and management of CS within a single center.

QRS prolongation is associated with associated with adverse cardiac remodeling in hypertrophic cardiomyopathy.

Background: Signal-averaged electrocardiogram (SAECG) records myocardial depolarization, and can detect inhomogeneous/slow conduction in fibrotic myocardium, which promotes reentrant ventricular arrhythmias (VAs). Hypertrophic cardiomyopathy (HCM) is associated with a high prevalence of cardiac fibrosis and VAs, but abnormal SAECG has low predictive power for VAs. We hypothesized that HCM-specific structural/electrical remodeling underlies this result.

Is it a thrombus or a tumor? An imaging dilemma for clinicians.

Atrial fibrillation, the most common cardiac arrhythmia in the Western world, confers a 5-fold increase in stroke, mainly due to thrombus formation in the left atrial appendage. Early rhythm control is often beneficial in reducing adverse cardiovascular events in higher-risk populations. Here, we present a patient who was found to have a 1 cm stalk-like lesion in the left atrial appendage on transesophageal echocardiogram prior to electrical cardioversion.

Regional quantification of cardiac metabolism with hyperpolarized [1-C]-pyruvate CMR evaluated in an oral glucose challenge.

Background: The heart has metabolic flexibility, which is influenced by fed/fasting states, and pathologies such as myocardial ischemia and hypertrophic cardiomyopathy (HCM). Hyperpolarized (HP) C-pyruvate MRI is a promising new tool for non-invasive quantification of myocardial glycolytic and Krebs cycle flux. However, human studies of HP C-MRI have yet to demonstrate regional quantification of metabolism, which is important in regional ischemia and HCM patients with asymmetric septal/apical hypertrophy.

Probing human heart TCA cycle metabolism and response to glucose load using hyperpolarized [2- C]pyruvate MRS.

Introduction: The healthy heart has remarkable metabolic flexibility that permits rapid switching between mitochondrial glucose oxidation and fatty acid oxidation to generate ATP. Loss of metabolic flexibility has been implicated in the genesis of contractile dysfunction seen in cardiomyopathy. Metabolic flexibility has been imaged in experimental models, using hyperpolarized (HP) [2- C]pyruvate MRI, which enables interrogation of metabolites that reflect tricarboxylic acid (TCA) cycle flux in cardiac myocytes.

Probing Human Heart TCA Cycle Metabolism and Response to Glucose Load using Hyperpolarized [2-C]Pyruvate MR Spectroscopy.

Introduction: The normal heart has remarkable metabolic flexibility that permits rapid switching between mitochondrial glucose oxidation and fatty acid (FA) oxidation to generate ATP. Loss of metabolic flexibility has been implicated in the genesis of contractile dysfunction seen in cardiomyopathy. Metabolic flexibility has been imaged in experimental models, using hyperpolarized (HP) [2-C]pyruvate MRI, which enables interrogation of metabolites that reflect tricarboxylic acid (TCA) cycle flux in cardiac myocytes.

Regional quantification of cardiac metabolism with hyperpolarized [1-C]-pyruvate MRI evaluated in an oral glucose challenge.

Background: The heart has metabolic flexibility, which is influenced by fed/fasting states, and pathologies such as myocardial ischemia and hypertrophic cardiomyopathy (HCM). Hyperpolarized (HP) C-pyruvate MRI is a promising new tool for non-invasive quantification of myocardial glycolytic and Krebs cycle flux. However, human studies of HP C-MRI have yet to demonstrate regional quantification of metabolism, which is important in regional ischemia and HCM patients with asymmetric septal/apical hypertrophy.

Systolic blood pressure ≤110 mm Hg is associated with severe coronary microvascular ischemia and higher risk for ventricular arrhythmias in hypertrophic cardiomyopathy.

Background: Coronary microvascular dysfunction (CMD) and hypertension (HTN) occur frequently in hypertrophic cardiomyopathy (HCM), but whether blood pressure (BP) influences CMD and outcomes is unknown.

Objective: The purpose of this study was to test the hypothesis that HTN is associated with worse CMD and outcomes.

Methods: This retrospective study included 690 HCM patients.

Collegiate Wrestler With a Bicuspid Aortic Valve and Aortic Dilation.

Bicuspid aortic valve and aortopathy are generally considered contraindications to isometric exercise. For athletes with mild disease at low risk of adverse events, a shared decision-making approach for continued sports participation is reasonable. We present a case of a collegiate wrestler with bicuspid aortic valve and aortopathy to illustrate shared decision making.

Right ventricular function declines prior to left ventricular ejection fraction in hypertrophic cardiomyopathy.

Background: The right ventricle (RV) in hypertrophic cardiomyopathy (HCM) tends to be neglected, as previous efforts have predominantly focused on examining the prognostic value of left ventricular (LV) abnormalities. The objectives of this study were to assess RV function in HCM, changes over time, and association with clinical outcomes.

Methods: Two hundred and ninety HCM patients with preserved LV ejection fraction (LVEF ≥ 55%) and 30 age- and sex-matched controls underwent cardiovascular magnetic resonance (CMR).

The Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder, affecting 1 out of 500 adults globally. It is a widely heterogeneous disorder characterized by a range of phenotypic expressions, and is most often identified by non-invasive imaging that includes echocardiography and cardiovascular magnetic resonance imaging (CMR). Within the last two decades, cardiac magnetic resonance imaging (MRI) has emerged as the defining tool for the characterization and prognostication of cardiomyopathies.

Gastroenteritis and cardiogenic shock in a healthcare worker: a case report of COVID-19 myocarditis confirmed with serology.

Background: Coronavirus disease 2019 (COVID-19) myocarditis is emerging as a component of the hyperactive inflammatory response secondary to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Isolated gastrointestinal symptoms are uncommon presenting features in adults with COVID-19 myocarditis. The availability of antibody testing is a valuable addition to the confirmation of COVID-19, when repeated reverse transcriptase-polymerase chain reaction of nasopharyngeal swabs are negative.

The Cardiac Effects of Performance-Enhancing Medications: Caffeine vs. Anabolic Androgenic Steroids.

Several performance-enhancing or ergogenic drugs have been linked to both significant adverse cardiovascular effects and increased cardiovascular risk. Even with increased scrutiny on the governance of performance-enhancing drugs (PEDs) in professional sport and heightened awareness of the associated cardiovascular risk, there are some who are prepared to risk their use to gain competitive advantage. Caffeine is the most commonly consumed drug in the world and its ergogenic properties have been reported for decades.

Hypertrophic Cardiomyopathy Patients With Paroxysmal Atrial Fibrillation Have a High Burden of Left Atrial Fibrosis by Cardiac Magnetic Resonance Imaging.

Objectives: This study hypothesized that paroxysmal atrial fibrillation (PAF) reflects the presence of a more severe cardiac hypertrophic cardiomyopathy (HCM) phenotype.

Background: HCM is characterized by myocyte hypertrophy, fibrosis, and a high prevalence of PAF. It is currently unresolved whether atrial fibrillation (AF) is a marker or a mediator of adverse outcomes in HCM.

Higher incidence of vasodilator-induced left ventricular cavity dilation by PET when compared to treadmill exercise-ECHO in hypertrophic cardiomyopathy.

Background: Vasodilator-induced transient left ventricular cavity dilation (LVCD) by positron emission tomography (PET) is associated with microvascular dysfunction in hypertrophic cardiomyopathy (HCM). Here we assessed whether HCM patients who develop LVCD by PET during vasodilator stress also develop LV cavity dilation by echocardiography (ECHO-LVCD) following exercise stress.

Methods: A retrospective analysis of cardiac function and myocardial blood flow (MBF) was conducted in 108 HCM patients who underwent perfusion-PET and exercise-ECHO as part of their clinical evaluation.

Progression of myocardial fibrosis in hypertrophic cardiomyopathy: mechanisms and clinical implications.

Aims: Myocardial fibrosis as detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) is a powerful prognostic marker in hypertrophic cardiomyopathy (HCM) and may be progressive. The precise mechanisms underlying fibrosis progression are unclear. We sought to assess the extent of LGE progression in HCM and explore potential causal mechanisms and clinical implications.