Case Illustration of the Natural History of Left Dominant Arrhythmogenic Cardiomyopathy.

Background: Arrhythmogenic left ventricular cardiomyopathy is an increasingly recognized cause of recurrent myocarditis, a mimicker of acute coronary syndrome, and an important cause of malignant ventricular arrythmias and heart failure. Desmoplakin is a protein that is critical to maintaining the structural integrity of the myocardium. Disruption of desmoplakin leads to fibrofatty infiltration of the myocardium which leads to congestive heart failure, cardiac arrhythmias, and sudden cardiac death. However, desmoplakin cardiomyopathy is often misdiagnosed, resulting in significant morbidity and mortality. We report 2 contrasting cases illustrating the natural history-hot and cold phases-of arrhythmogenic left ventricular cardiomyopathy. Case Series: The first case demonstrates a common phenotypic presentation of desmoplakin cardiomyopathy manifested as recurrent myocarditis and myocardial injury representing the hot phase. The second case is an undulating course of chronic systolic heart failure and ventricular arrhythmias representing the cold phase. Conclusion: Arrhythmogenic cardiomyopathy manifests as a spectrum of disease processes that involve the right, left, or both ventricles. Mutations in the desmoplakin gene are often associated with a left dominant ventricular cardiomyopathy. Diagnosis remains difficult as the condition has no signature clinical presentation, and imaging findings are variable.

Long-term patency of rescue stenting of an anomalous left circumflex coronary artery after transcatheter aortic valve replacement.

Transcatheter aortic valve replacement (TAVR) in the setting of an anomalous left circumflex coronary artery (LCX) has had a variety of outcomes. Most commonly an anomalous LCX originates as a separate ostium arising from the right coronary sinus or is found branching off of the proximal right coronary artery. The artery courses around the aortic annulus before taking the course seen in typical anatomy. Given this deviation from typical anatomy and increased aortic annulus pressure by the replacement valve, there is an increased risk of a complication such as acute coronary artery occlusion. Special consideration and preparation are needed to prevent adverse outcomes, including death. We report a case in which intraprocedural anomalous LCX rescue stenting proved to be effective for treatment of acute coronary occlusion. Follow-up angiography provided an opportunity to demonstrate long-term patency in rescue stenting during TAVR.