Riding the Waves of Novel Therapies in Hypertrophic Cardiomyopathy.

We discuss a case of a 60-year-old man with hypertrophic cardiomyopathy treated with the novel cardiac myosin inhibitor, mavacamten. Dynamic electrocardiogram patterns of left ventricular hypertrophy and left ventricular strain coincided with the patient starting mavacamten, discontinuing the drug, and then restarting mavacamten, highlighting electrocardiograms as accessible and inexpensive potential tools to monitor drug efficacy. This case also shows the ability of myosin inhibition to positively alter the adverse electrical changes associated with hypertrophic cardiomyopathy.

Tailoring the wrap: intraoperative functional lumen imaging probe (FLIP) during hiatal hernia repair.

Introduction: The introduction of the functional lumen imaging probe (FLIP) has provided objective, real-time feedback on the geometric variations with each component of a hiatal hernia repair (HHR). The utility of this technology in altering intraoperative decision-making has been scarcely reported. Herein, we report a single-center series of intraoperative FLIP during HHR.

Effectiveness of Standard Treatment for Stage 4 Colorectal Cancer: Traditional Management with Surgery, Radiation, and Chemotherapy.

Colorectal cancer (CRC) is the second most common cause of cancer-related death in the United States comprising 7.9% of all new cancer diagnoses and 8.6% of all cancer deaths.

Contemporary Therapies and Future Directions in the Management of Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a genetic disorder caused by pathogenic variants in sarcomeric genes, leading to left ventricular hypertrophy and complex phenotypic heterogeneity. While HCM is the most common inherited cardiomyopathy, pharmacological treatment options have previously been limited and were predominantly directed towards symptom control owing to left ventricular outflow obstruction. These therapies, including beta blockers, calcium channel blockers, and disopyramide, have not been shown to affect the natural history of the disease, which is of particular concern for younger patients who have an increased lifetime risk of experiencing arrhythmias, heart failure, and sudden cardiac death.

Cardiovascular Characteristics of Patients with Genetic Variation in Desmoplakin ().

Background: Variants in the desmoplakin () gene have been recognized in association with the pathogenesis of arrhythmogenic right ventricular cardiomyopathy (ARVC) for nearly 20 years. More recently, genetic variation in has also been associated with left-dominant arrhythmogenic cardiomyopathy. Data regarding the cardiac phenotypes associated with genetic variation in have been largely accumulated from phenotype-first studies of ARVC.

Left Ventricular Hypertrophy and Hypertrophic Cardiomyopathy in Adult Solid Organ Transplant Recipients.

Background: Hypertrophic cardiomyopathy (HCM) in pediatric solid organ transplant recipients has been reported in association with use of calcineurin inhibitors. However, data on the incidence and prevalence of HCM in adult posttransplant patients are limited. We sought to describe the clinical characteristics of solid organ transplant recipients who were diagnosed with HCM from 2011 to 2021 at a single center.

Applying Shared Decision Making to Sports Participation for a Patient With Hypertrophic Cardiomyopathy.

A patient with hypertrophic cardiomyopathy and a cardio-defibrillator implanted for primary prevention would like to compete on the ski team at his school. This case illustrates how a shared decision-making approach can be applied when counseling patients with hypertrophic cardiomyopathy about exercise and sports participation. ().

Recurrent Left Pleural Effusion Following Left Atrial Appendage Closure With the Watchman Device.

A 75-year-old woman with recent left atrial appendage closure with the Watchman device (Boston Scientific, Natick, Massachusetts) presented with recurrent left pleural effusion. The constellation of chest pain, pericardial effusion, and exudative pleural effusion were suggestive of an inflammatory process precipitated by microperforation of the fixation anchors during the Watchman placement. ().

Incremental prognostic value of visually estimated coronary artery calcium in patients undergoing positron emission tomography imaging.

Objective: Visually estimated coronary artery calcium (VECAC) from chest CT or attenuation correction (AC)/CT obtained during positron emission tomography (PET)-myocardial perfusion imaging (MPI) is feasible. Our aim was to determine the prognostic value of VECAC beyond conventional risk factors and PET imaging parameters, including coronary flow reserve (CFR).

Methods: We analysed 608 patients without known coronary artery disease who underwent PET-MPI between 2012 and 2016 and had AC/CT and/or chest CT images.

Long-Term Outcomes of the Electrically Unresponsive, Anatomically Intact Facial Nerve Following Vestibular Schwannoma Surgery.

 The study aimed to determine long-term outcomes in patients with intraoperative electrical conduction block in an anatomically intact facial nerve (FN).  Single center retrospective review of prospectively collected database of all vestibular schwannoma surgeries between January 1, 2008 and August 25, 2015. Operative notes were reviewed and patients with anatomically intact FNs, but complete conduction block at the end of surgery were included for analysis.

Sarcomere mutation negative hypertrophic cardiomyopathy is associated with ageing and obesity.

Background: Despite advances in our understanding of the genetic causes of hypertrophic cardiomyopathy (HCM), a large portion of this patient population do not carry sarcomere gene mutations when screened. It remains largely unknown why patients without sarcomere mutations develop asymmetric myocardial hypertrophy.

Methods: We performed a retrospective analysis of probands with HCM who underwent genetic testing to determine if clinical phenotypes were different depending on sarcomere mutation status.

Ruptured temporal horn aneurysm associated with Moyamoya collateral vessel coexisting with contralateral arteriovenous malformation - technical note with clinical vignette.

The co-occurrence of a brain arteriovenous malformation, Moyamoya Disease, and intracranial aneurysm is exceedingly rare. We report the third case of this disease constellation, and the first where the aneurysm arises from the Moyamoya collateral vessel. We review the relevant literature and discuss proposed pathophysiological mechanisms and clinical implications.

Outcomes and Complications of Endovascular Mechanical Thrombectomy in the Treatment of Acute Posterior Circulation Occlusions: A Systematic Review.

Background: The use of thrombectomy in the treatment for acute ischemic stroke (AIS) affecting the anterior circulation is well established. Comparatively, fewer data exist on the effectiveness of these techniques in treating posterior circulation occlusions. This review analyzes and reports on the usefulness and outcomes of emergent thrombectomy in large-vessel occlusions affecting the posterior circulation.

False Localizing Trigeminal V2 Sensory Loss in Vestibular Schwannoma.

False localizing signs involving cranial nerves are rare, even more so when involving the trigeminal nerve. Here we present the first case of trigeminal V2 sensory loss as a false localizing sign. The sensory dysfunction was caused by a large contralateral cystic vestibular schwannoma and subsequently improved after tumor resection.

Alterations in sarcomere function modify the hyperplastic to hypertrophic transition phase of mammalian cardiomyocyte development.

It remains unclear how perturbations in cardiomyocyte sarcomere function alter postnatal heart development. We utilized murine models that allowed manipulation of cardiac myosin-binding protein C (MYBPC3) expression at critical stages of cardiac ontogeny to study the response of the postnatal heart to disrupted sarcomere function. We discovered that the hyperplastic to hypertrophic transition phase of mammalian heart development was altered in mice lacking MYBPC3 and this was the critical period for subsequent development of cardiomyopathy.