Electrophysiologic and Cardiovascular Manifestations of Duchenne and Becker Muscular Dystrophies.

BACKGROUND: There have been significant advances in the diagnosis and management of the hereditary muscular disorders Duchenne and Becker Muscular Dystrophy (DMD and BMD). Cardiac electrophysiologic and Cardiovascular involvement has long been important in the surveillance, care, and prognosis of patients with both BMD and DMD, and is the leading cause of mortality in patients with DMD. With improved long-term prognosis, rhythm disorders and progressive cardiomyopathy with resultant heart failure are increasingly common. OBJECTIVES: This review aims to provide an overview to electrophysiologists and cardiologists of the cardiac electrophysiologic phenotypes and genetics of the BMD and DMD and highlight the recent discoveries that have advanced clinical course and management. METHODS: A systematic review was performed in the diagnosis and management of Duchenne and Becker muscular dystrophies. The Cochrane Library, PubMed, MEDLINE, the Europe PubMed Central, AMED, and EMBASE database were accessed for available evidence. The research reported in this paper adhered to PRISMA guidelines. RESULTS: Evidence from randomized control trials and studies cited in expert consensus and practice guidelines are examined. Advanced imaging techniques and a spectrum of rhythm disorders associated with the progressive cardiomyopathy are presented. Early initiation of heart failure therapies, the role of cardiac implantable devices, and novel gene therapies approved for use with the potential to alter the disease course are discussed. CONCLUSION: DMD and BMD patients may have profound cardiac and cardiac electrophysiologic involvement, which when diagnosed and treated earlier, could lead to improved outcomes.

Transcatheter Treatment of Mitral Valve Regurgitation in the Setting of Concomitant Coronary or Multivalvular Heart Disease: A Focused Review.

Treatment for mixed valve disease has historically been limited, often surgery being the only option. With the recent advancement of transcatheter therapies, percutaneous approaches are quickly becoming viable therapeutic considerations in inoperable or high-risk patients, also offering the option for a staged or same-session treatment. Guidelines are primarily focused on single-valve disease. However, patients often present with multiple pathologies. This review summarizes the data and literature on transcatheter treatment of patients with mitral regurgitation who concomitantly have aortic stenosis or regurgitation, tricuspid regurgitation, or ischemic cardiomyopathy. Pathophysiology, hemodynamics, available therapies as well as order and timing of interventions are discussed.

A Case of Severe Metabolic Acidosis in the Setting of a Strict Ketogenic Diet.

Patients with metabolic acidosis often present with obscure, multifactorial etiologies, making efficient diagnosis and treatment key to preventing poor clinical outcomes. This case report describes a patient with severe metabolic acidosis in which the underlying cause was not immediately apparent. After a thorough work-up and history taking, the patient's strict ketogenic diet was identified as the most likely source of his illness. Over multiple days, the patient improved as he resumed a normal diet and was treated for refeeding syndrome. This case highlights the importance of taking a comprehensive social and diet history when assessing a patient with metabolic acidosis. It also highlights the need for physicians to understand and be ready to counsel on the possible effects of fad diets, such as the ketogenic diet.

Comparison of BMI on operative time and complications of robotic inguinal hernia repair at a VA medical center.

BACKGROUND: BMI is a risk factor for recurrence and post-operative complications in both open and laparoscopic totally extraperitoneal approach (TEP) repair. Robotic surgery using the transabdominal preperitoneal approach (TAPP) is a safe and viable option for inguinal hernia repair (IHR). The objective of this study is to determine how difference in BMI influences rate of operative time, complications, and rate of recurrence in a robotic TAPP IHR. METHODS: We performed a retrospective review of patients who underwent robotic inguinal hernia repair between 2012 and 2019 at a Veterans Health Administration facility (N = 304). The operating time, outcomes, and overall morbidity and mortality for robotic IHR were compared between three different BMI Groups. These groups were divided into: "Underweight/Normal Weight" (BMI < 25) n = 102, "Pre-Obese" (BMI 25-29.9) n = 120, and "Obese" (BMI 30 +) n = 82. RESULTS: The average operating time of a bilateral IHR by BMI group was 83.5, 98.4, and 97.8 min for BMIs < 25, 25-29.9, and 30 +, respectively. Operating time was lower in the Underweight/Normal BMI group compared to the Pre-Obese group (p = 0.006) as well as the Obese group (p = 0.001). For unilateral repair, the average operation length by group was 65.2, 70.9, and 85.6 min for BMIs < 25, 25-29.9, and 30 +, respectively, demonstrating an increased time for Obese compared to Underweight/Normal BMI (p = 0.001) and for Obese compared to Pre-Obese (p = 0.01). Demographic/comorbidity variables were not significantly different, except for a higher percentage of white patients in the Underweight/Normal BMI group compared to the Pre-Obese and Obese groups (p = 0.02 and p = 0.0003). There was no significant difference in complications or recurrence. CONCLUSION: BMI has a significant impact on the operating time of both unilateral and bilateral robotic hernia repair. Despite this increased operative time, BMI group did not differ significantly in postoperative outcomes or in recurrence rates.