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CardioNerds\xa0(Amit Goyal\xa0&\xa0Daniel Ambinder) join Summa Health cardiology fellows (Jack Hornick, Phoo Pwint Nandar, and Sideris Facaros) for a hike on the Towpath Trail at Cuyahoga Valley National Park in Akron, Ohio! They discuss an informative case of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) complicated by ventricular tachycardia & cardiogenic shock. Dr. Kenneth Varian provides the E-CPR and program director, Dr. Marc Penn provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident,\xa0Eunice Dugan,\xa0with mentorship from University of Maryland cardiology fellow\xa0Karan Desai.\xa0\xa0\n\n\n\n\n\nJump to: Patient summary - Case media - Case teaching - References \n\n\n\nEpisode graphic by Dr. Carine Hamo\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\nPatient Summary\n\n\n\nA female in her 40s with\xa0no past medical history presented\xa06 years prior\xa0with\xa0acute onset dizziness, palpitations and fatigue without chest pain. She had no family history of arrythmias, SCD, or prior syncope. Her heart rate was 170 bpm and EKG showed wide complex, regular tachycardia felt to be VT.\xa0She underwent synchronized cardioversion to sinus rhythm. Her baseline EKG showed sinus bradycardia with low voltage, incomplete RBBB, and ventricular ectopy. Labs were unrevealing, and social history was negative for toxic insults or illicit substance abuse. TTE showed preserved LVEF and normal valves, but RV was dilated with decreased systolic function.\xa0LHC was without obstructive coronary disease. She was diagnosed with ARVC and received an ICD for secondary prevention. She was discharged on sotalol for arrythmia management. Her genetic testing later returned positive for uncertain significance in the DSP gene and JUP gene, both commonly implicated in ARVC. She was followed in the outpatient setting for 5 years with no apparent shocks.\xa0Six years later, she presented\xa0with acute onset dizziness and palpitations similar to\xa0her initial presentation. EKG showed a wide complex tachycardia at 170 bpm treated with amiodarone and cardioversion. On ICD interrogation,\xa0she was found to have had several episodes of VT,\xa0but at\xa0a\xa0rates\xa0below the\xa0VT\xa0detection zone programmed in the ICD.\xa0Subsequent RHC showed significantly depressed cardiac index and RV dysfunction.\xa0She underwent successful inpatient VT ablation. She was then discharged home with plans for close follow up; however, 2 days later, she started feeling nauseous with fatigue and abdominal pain. She was sent straight to the nearest transplant-capable hospital where she was found to be in cardiogenic shock. She was admitted to ICU and started on inotropes. Due to refractory shock, she was cannulated for VA ECMO and successfully underwent cardiac transplantation two days later.\xa0\xa0\n\n\n\n\n\n\n\nCase Media\n\n\n\n\n123Click to Enlarge\n\n\n\nA. Post cardioversion ECG: NSR, low voltage, incomplete RBBB, PVCB. TTE: RV enlargement C. TTE: Tissue Doppler velocity (S') low\n\n\n\n\n\nTEE\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\nEpisode Schematics & Teaching\n\n\n\n\n\n\n\nThe CardioNerds 5! \u2013 5 major takeaways from the #CNCR case\n\n\n\nWhat is ARVC?\xa0\n\n\n\nArrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is a heritable cardiac muscle disorder that classically involves the RV (though LV involvement is increasingly being recognized) marked by loss of healthy myocardium and replacement with fibrofatty tissue predominantly due to genetic defects in both\xa0desmosomal\xa0and non-desmosomal\xa0proteins. Clinical manifestations include RV dysfunction, ventricular arrhythmias, and sudden cardiac death (SCD).\xa0This is a progressive disease\xa0that can affect the epicardium and/or mid-myocardium first and then move towards the sub-endocardium.\xa0\xa0It affects approximately 1 in 5000 individuals and\xa0is an important cause of (SCD)\xa0in young patients.