Multimodal assessment of arrhythmogenic right ventricular cardiomyopathy
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Department of Nuclear Cardiology, National Institute of Cardiology Ignacio Chavez, Mexico City, Mexico Academic Unit of Medicine, Autonomous University of Nayarit, Tepic-Nayarit, Mexico Mexican Faculty of Medicine, University La Salle, Mexico City, Mexico Institute of Medical Sciences, Autonomous University of Ciudad Juarez, Mexico City, Mexico Department of Cardiac Magnetic Resonance, National Institute of Cardiology Ignacio Chavez, Mexico City, Mexico Department of Echocardiography, ABC Medical Center, I.A.P, Mexico City, Mexico Department of Physiology, Faculty of Medicine, National Autonomous University of Mexico, Mexico City, Mexico
Received Oct 19, 2019; accepted Oct 22, 2019 doi:10.1007/s12350-019-01944-0
INTRODUCTION The arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic cardiomyopathy. The prevalence is estimated to be 1/5000 cases of total population and may manifest as ventricular arrhythmia, sudden death, or heart failure.1 CASE PRESENTATION A 51-year-old man came to the emergency department with chest pain, dyspnea, nausea, and syncope. Physical examination revealed heart rate 180 bpm, blood pressure 90/70 mmHg, respiratory rate 21/min, oxygen saturation 90% on room air. The cardiac examination revealed apex in the fifth intercostal space, midclavicular line, no murmurs, and a third sound. Laboratory data showed elevated cardiac enzymes CK-MB of 3.36 ng/mL (normal range 0.6 to 6.3), highErick Alexanderson-Rosas, and Jesus A. Garcia-Diaz have contributed equally in the drafting of this paper. Funding No funding was available or needed. Reprint requests: Nilda Espinola-Zavaleta, MD, PhD, Department of Nuclear Cardiology, National Institute of Cardiology Ignacio Chavez, Juan Badiano N8 1, Colonia Seccio´n XVI, Tlalpan, P.C. 14080 Mexico City, Mexico; [email protected] J Nucl Cardiol 1071-3581/$34.00 Copyright Ó 2019 American Society of Nuclear Cardiology.
sensitivity troponin of 112.8 ng/L (nr 14 to 42.9), and NT-ProBNP of 461 pg/mL (nr 15 to 125). The electrocardiogram (ECG) showed ventricular tachycardia (VT) at a rate of 200 bpm (Figure 1A). He was managed successfully with electric cardioversion (Figure 1B). Transthoracic echocardiogram demonstrated right ventricular (RV) dilation and systolic dysfunction (Basal diastolic diameter: 50 mm, fractional shortening: 26.6%, S wave: 7.9 cm/s, 3D RVEF: 24%, Figure 2) but normal left ventricular systolic function (LVEF: 60%). Cardiac magnetic resonance corroborated the echocardiographic findings and showed late gadolinium enhancement in the RV free wall with extension to the LV distal apex (Figure 3). Radionuclide ventriculography-SPECT was performed, using a GE-Millenium MPS gamma-camera. A 30 mCi of 99mTc was used and planar images were acquired. The study was processed with EF-Analysis software. RV dilation, global hypokinesia, systolic dysfunction (RVEF: 31%) and dyssynchrony (histogram bandwidth: 45°, phase standard deviation: 50°) with mild LV systolic dysfunction (LVEF: 50%), and dyssynchrony were found (Figures 4
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