Rapid Progression of Cardiac Allograft Vasculopathy With Sudden Cardiac Death Within the First Year of Heart Transplantation: A Case Report.

Cardiac allograft vasculopathy (CAV) is a major cause of mortality and morbidity within the first 3 years of heart transplantation (HT). CAV results in progressive coronary artery stenosis and may lead to arrhythmias and sudden cardiac death. In some cases, sudden cardiac death represents the initial presentation of CAV, as cardiac denervation blunts anginal symptoms that otherwise could have preceded sudden cardiac death (SCD). Here, we describe the case of a 56-year-old man with non-ischemic cardiomyopathy. He underwent left ventricular assist device implantation followed by orthotopic HT. Five months after transplantation, he presented with atypical mid-sternal chest discomfort initially thought to be musculoskeletal in origin. Routine coronary angiography at 12 months revealed severe, diffuse 3-vessel disease consistent with International Society of Heart and Lung Transplantation (ISHLT) CAV-3, and cardiac MRI revealed full thickness delayed enhancement in the inferior wall consistent with infarction. Despite overall preserved left ventricular ejection fraction, he developed polymorphic ventricular tachycardia (VT) with an appropriate shock from a wearable cardioverter-defibrillator (WCD) and was transferred with an intra-aortic balloon pump to a larger transplant center and underwent another heart transplantation. This case highlights rapid CAV progression leading to infarction and arrhythmia despite preserved LVEF, emphasizing the need for enhanced screening with biomarkers and imaging as well as earlier consideration for device-based strategies to prevent sudden cardiac death in high-risk transplant recipients.