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2021년 제72차 대한내과학회 추계학술대회
￭S-188 ￭ A case report on middle-aged woman diagnosed
with arrhythmogenic right ventricular dysplasia (ARVD)
가톨릭중앙의료원 여의도성모병원 내과학교실
조용은, 조은주
Introduction: Arrhythmogenic right ventricular dysplasia (ARVD) is a progressive cardiomyopathy with fibro-fatty replacement of myocardium, mainly in the right ventricle. The pathology predispose to ventricular arrhythmias, heart failure and sudden cardiac death.
We hereby report a case of middle-aged woman diagnosed with ARVD.
Case: A 44-year old woman walked into the emergency room presenting with abdominal distension and dyspnea. She had history of cerebral infarction, and liver cirrhosis. She had no family history of cardiovascular disease. On auscultation, holosystolic murmur at the left middle sternal border was present. She showed pretibial pitting edema in both legs, and abdominal distension with shifting dullness.
Chest radiogram showed cardiomyopathy (Figure A) and electrocardiogram showed low voltage with fragmented R wave (Figure B).
Blood tests showed elevated NT pro-BNP, increased r-GTP and ALP. Echocardiogram showed huge RA and RV, with non-coapted TV due to annular dilatation (Figure C, D). Significant TR without increase of RV systolic pressure was noted due to dilated RV. She also had LV systolic dysfunction with hypokinesia of LV, especially akinesia in the entire apex. Cardiac MRI showed marked dilatation of RV with hypokinesia which was compatible with echocardiogram. Suspicious microaneurysms in RV lateral wall also suggests ARVD (Figure E). On holter monitoring, frequent premature atrial and ventricular complexes of RV origin and episodes of non-sustained ventricular tachycardia with inferior axis were documented (Figure F). The diagnosis was made based on ‘Revised Task Force Criteria 2010’, and she was informed the need of implantable cardiac defibrillator (ICD).
Conclusion: The diagnosis of ARVD is quite challenging due to its non-specific clinical presentation and therapeutic options remain limited because of its progressive nature. We prescribed diuretics to reduce preload, and beta-blocker for arrhythmia. After achieving negative intake and output balance, the patient’s ascites and pretibial pitting edema resolved and she discharged in tolerable state. The patient is attending the outpatient clinic for follow up and planning for ICD.
