An 81-year-old woman with arrhythmogenic right ventricular cardiomyopathy underwent catheter ablation for atrial fibrillation and atrial flutter. Hypoxemia refractory to the administration of oxygen was seen after transseptal puncture. Transthoracic echocardiography revealed right to left shunt via an iatrogenic atrial septal defect (IASD) that was increased by tricuspid regurgitation flow. Her hypoxemia improved after IASD occlusion with the inflation of a venogram balloon catheter. Emergent surgical IASD closure was successfully performed. IASD after transseptal puncture for atrial fibrillation ablation infrequently causes severe complications that require emergent repair.
UNASSIGNED: Some cases requiring iatrogenic atrial septal defect (IASD) closure after atrial fibrillation (AF) ablation have been reported. We describe the case of an arrhythmogenic right ventricular cardiomyopathy patient with right to left shunt via an IASD which required emergent surgical repair after AF ablation. Right to left shunt after trans-septal puncture is rare, however it can be an emergent life-threatening complication. IASD occlusion with venogram balloon catheter is helpful for the diagnosis and the short-term solution.

Secondary prevention of sudden cardiac death in the young patient with arrhythmogenic right ventricular cardiomyopathy and hemodynamically tolerated ventricular tachycardia is still a challenging field. We present a combined approach, including subcutaneous implantable cardioverter-defibrillator (ICD) and catheter ablation, as a promising treatment to prevent both ventricular tachycardia recurrences and ICD shocks. (Level of Difficulty: Intermediate.).

The primary etiology of a diverse range of cardiomyopathies is now understood to be genetic, creating a new paradigm for targeting treatments on the basis of the underlying molecular cause. This review provides a genetic and etiologic context for the traditional clinical classifications of cardiomyopathy, including molecular subtypes that may exhibit differential responses to existing or emerging treatments. The authors describe several emerging cardiomyopathy treatments, including gene therapy, direct targeting of myofilament function, protein quality control, metabolism, and others. The authors discuss advantages and disadvantages of these approaches and indicate areas of high potential for short- and longer term efficacy.

A 27-year-old woman presented with palpitations and was found to have episodes of a non-sustained wide complex tachycardia. In this report, we discuss a differential diagnosis for the patient\'s wide complex tachycardia and the important ECG findings which lead to her diagnosis. (Level of Difficulty: Advanced.).

Cardiac sarcoidosis (CS) overlaps in clinical presentation with arrhythmogenic right ventricular cardiomyopathy and shares phenotypic classification, including the presence of epsilon waves. The presence of conduction disease is seen exclusively in CS, as an important phenotypic difference. We present a case of ventricular tachycardia and epsilon waves due to CS, without conduction disease. (Level of Difficulty: Intermediate.).

Two middle-aged women had evidence suggesting right ventricular hypertrophy on routine electrocardiograms. Their echocardiograms showed right ventricular thickening and cardiac magnetic resonance imaging revealed right ventricular fatty infiltration. Neither patient fulfilled the criteria for arrhythmogenic right ventricular cardiomyopathy, and both had a benign clinical course. (Level of Difficulty: Intermediate.).

Catecholaminergic polymorphic ventricular tachycardia is a genetic disorder that causes ventricular tachyarrhythmias via increased release of intracellular calcium. The standard diagnostic measure is an exercise stress test that reveals ventricular ectopy. We present an extraordinary case marked by a normal stress test and no relation to exertion. (Level of Difficulty: Intermediate.).

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. (Level of Difficulty: Beginner.).

Epsilon waves are the surface manifestation of myocardial regions with delayed activation and are considered the hallmark of arrhythmogenic right ventricular cardiomyopathy. However, other conditions can also result in epsilon waves and simulate arrhythmogenic right ventricular cardiomyopathy. In this case, a patient presents with recurrent ventricular tachycardia and large epsilon waves due to cardiac sarcoidosis. (Level of Difficulty: Intermediate.).

We report a case of a woman with arrhythmogenic right ventricular cardiomyopathy (ARVC) who presented with severe right-sided heart failure and cardiac cirrhosis that mandated heart-liver transplantation. This case highlights an emerging sex-based difference in ARVC where female sex is associated with a higher risk of heart failure than in male patients with ARVC. (Level of Difficulty: Advanced.).