56. Case Report: Arrhythmogenic Desmoplakin Cardiomyopathy Northwestern University Feinberg School of Medicine

Published: Sept. 14, 2020, 11:54 a.m.

CardioNerds\xa0(Amit Goyal\xa0&\xa0Daniel Ambinder) join Northwestern University\xa0cardiology fellows (Sarah Hale, Sarah\xa0Chuzi, and Graham Lohrmann) for burgers and a great case by the Chicago River! They discuss a fascinating case of\xa0arrhythmogenic desmoplakin cardiomyopathy. Dr.\xa0Lisa\xa0Wilsbacher\xa0provides the E-CPR and program director Dr.\xa0Benjamin\xa0Freed provides a message for applicants.\xa0\xa0Episode notes were developed by Johns Hopkins internal medicine resident\xa0Richard Ferraro\xa0with mentorship from University of Maryland cardiology fellow\xa0Karan Desai.\xa0\n\n\n\n\n\nJump to: Patient summary - Case figures & media - Case teaching - References - Production team\n\n\n\nEpisode graphic by Dr. Carine Hamo\n\n\n\n\n\n\n\nThe\xa0CardioNerds Cardiology Case Reports\xa0series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an\xa0\u201cExpert CardioNerd Perspectives & Review\u201d (E-CPR)\xa0for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.\n\n\n\nWe are teaming up with the\xa0ACC FIT Section\xa0to use the\xa0#CNCR episodes\xa0to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an\xa0E-CPR\xa0segment and a message from the program director.\n\n\n\nCardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza\n\n\n\n\n\n\n\n\n\n\n\nPatient Summary\n\n\n\nA male in his early 40s presented for second opinion regarding multiple ICD shocks. 10 years prior\xa0he\xa0was diagnosed with a "weak heart," thought secondary to a viral illness and a dual-chamber ICD was placed at that time.\xa0He\xa0noted shocks occurring for the first time 5 years prior, at which time amiodarone was started. They recurred two years prior,\xa0when he was diagnosed\xa0with paroxysmal atrial fibrillation.\xa0Finally,\xa0he\xa0was hospitalized one month before presentation with multiple ICD shocks and was found to have high defibrillation thresholds (DFTs) and\xa0amiodarone was stopped. He\xa0 then presented for a second opinion from the Northwestern\xa0CardioNerds!\xa0\n\n\n\nThe patient had been doing well on GDMT and had NYHA Class I symptoms (Enjoy Ep #13 - Approach to GDMT).\xa0He did note a family history of a cousin with "cardiac issues" and did not know his father's family history. Physical exam demonstrated bradycardia and ECG demonstrated an a-paced, v-sensed rhythm at 50 bpm.\xa0TTE demonstrated a moderately dilated LV with LVEF 30%, globally reduced LV function and multiple wall motion abnormalities without a vascular distribution.\xa0 PET-CT was performed which showed diffuse uptake and high-intensity signal at the inferolateral and basal anterior walls. Cardiac MRI showed diffuse circumferential epicardial delayed enhancement with associated diffuse, enhancing thickening of the pericardium favoring inflammatory versus fibrotic process. Patient was initially diagnosed with cardiac sarcoid and started on prednisone and weekly methotrexate.\xa0\xa0\n\n\n\nOn return of genetic testing, patient found to have a pathogenic variant for\xa0desmoplakin\xa0gene, and it was felt his cardiomyopathy was secondary to\xa0desmoplakin\xa0Left Dominant Arrhythmogenic Cardiomyopathy\xa0(LDAC, or left-dominant ARVC)\xa0presenting with inflammatory myocardial injury.\xa0On follow up the patient remained listed for transplant, and DFTs improved off amiodarone.\xa0\xa0\n\n\n\n\n\n\n\n\nCase Media\n\n\n\n\nCXRECGCardiac MRIPET CT (Cardiac Sarcoid Protocol)Click to Enlarge\n\n\n\n\n\n\n\n\n\n\n\n\n\nEpisode Schematics & Teaching\n\n\n\nCreated by Dr.