Arrhythmogenic Right Ventricular Cardiomyopathy: The Role of Genetics in Diagnosis, Management, and Screening.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a predominantly autosomal dominant genetic condition in which fibrous and fatty tissue infiltrate and replace healthy myocardial tissue. This uncommon yet debilitating condition can cause ventricular arrhythmias, cardiac failure, and sudden cardiac death. Management focuses primarily on prevention of syndrome sequelae in order to prevent morbidity and mortality. Genetic testing and screening in affected families, although utilized clinically, has not yet been incorporated in guidelines due to lack of larger studies and data. We aim herein to identify causative gene mutations, present advancements in diagnosis and management, and describe the role of genetic screening and counseling in patients with ARVC. With the advancement of genetic testing and therapy, diseases such as ARVC may become more accurately diagnosed and more effectively managed, ultimately significantly reducing morbidity and mortality.

Cardiac Sarcoidosis: A Unique Presentation.

Isolated cardiac sarcoidosis is a rare subset of sarcoidosis, a systemic autoimmune condition primarily found in African American females. The manifestations of cardiac sarcoidosis include atrioventricular and bundle branch blocks, arrhythmias, heart failure, and pericardial effusions, although these complications occur at varying prevalence. The diagnosis of cardiac sarcoidosis requires several different criteria; however, recent literature has focused heavily on imaging modalities such as cardiac magnetic resonance imaging. We present a case of a 42-year-old Caucasian male who was found to have unexplained cardiac arrhythmias and ultimately diagnosed with cardiac sarcoidosis by imaging modalities.

Acute Liver Failure due to Altered Fontan Circulation in a Patient With Tricuspid Atresia: A Case Report.

Tricuspid atresia is a congenital malformation of the tricuspid valve resulting in a lack of blood flow between the right atrium and the right ventricle. Management generally involves staged surgical intervention enabling affected individuals to survive into adulthood. Although surgical intervention greatly improves morbidity and mortality in this patient population, there are many long-term complications associated with the creation of a surgical shunt. We report a case of a 33-year-old male with tricuspid atresia who underwent Fontan surgery as a child and presented to our hospital with acute liver failure.

Obesity and the Other Independent Predictors in Elective Endotracheal Tube Intubations: A Narrative Review.

Obesity is one of the challenging elements in health care. Studies have shown that as the body mass index (BMI) increases, the risk of chronic conditions tends to increase due to altered physiologic and metabolic demands. In addition to underlying physiological changes, anatomical changes can lead to common procedural challenges, such as difficult intravenous (IV) cannulation, difficult airway, and difficult intubation, which makes their preoperative and postoperative care challenging for the anesthesiologists. According to previous studies, there is no single best predictor for difficult airway or intubations and no designed protocol for choosing an intubation technique in obese patients. Some of the preoperative risk factors and techniques such as the modified Mallampati class, sternomental distance, thyromental distance, neck circumference, indirect mirror laryngoscopy, BMI, and intraoperative risk factors such as inappropriate positioning of the patient, suboptimal medication dosing, inappropriate laryngoscopy device acted as independent predictors for difficult airway and difficult intubation. Analyzing each element's importance and making suitable decisions for the individual will reduce the complications and prepare for unplanned emergencies in the operating room. This review is convincing with previous studies that obesity itself is not an independent predictor. Instead, as a preoperative risk factor, and till date, sternomental distance and the number of intubation attempts were demonstrated as significant independent predictors for adverse events. All the other independent factors and considerations were discussed, which can help with further research.

Neuromyelitis Optica Spectrum Disorder: A Rare Cause of Spinal Cord and Optic Nerve Involvement.

Neuromyelitis optica spectrum disorder (NMOSD) is defined as a rare central nervous system, demyelinating, autoimmune disorder with autoantibodies against aquaporin-4. Commonly affecting females, NMOSD is known to also be a relapsing disease and can increase in severity during each episode. Diagnostic criteria include ruling out multiple sclerosis, spinal magnetic resonance imaging, and autoantibody detection. While management focuses on relapse treatment and prevention, high-dose steroids and plasma exchange have proven to be useful options. We present a case of a young female presenting with NMOSD relapse appropriately managed with plasma exchange.

A Fatal Case of Pembrolizumab-Induced Myocarditis in Non-Small Cell Lung Cancer.

Immune channel inhibitor-induced myocarditis is rare, and its management is challenging. Recently, guidelines were established for all ICIs, yet they do not take into account individual drug toxicities or screening protocols for prevention. We present a rare case of rapidly progressive pembrolizumab-induced fatal myocarditis in an initially asymptomatic patient. (Level of Difficulty: Beginner.).