Suspect Moschcowitz syndrome: how, when, why
1) Department of Advanced Medical and Surgical Sciences, University of Campania “L. Vanvitelli”, Italy
2) MD, PhD , Associate Professor of Internal Medicine , Chief of Postgraduate School of Emergency Medicine . Department of Advanced Medical and Surgical Sciences, University of Campania “L. Vanvitelli”, Naples, Italy
This case report aim to underline the importance of a clinical suspicion of arrhythmogenic right ventricular dysplasia (ARVD) in individuals admitted to ED for syncope or for malignant ventricular arrhythmias even when the patient is not a young one or is not an athlete. ARVD is a myocardial disease, often familial, that is characterized by a progressive fibro fatty replacement of the right ventricular myocardium and clinically by ventricular arrhythmias of right ventricular origin, which may lead to sudden death, mostly in young people and athletes. Diagnosis id often difficult because there is no a single test used for. Diagnosis is based on the presence of structural, histological data, EKG and genetic factors according to the 1994 Task Force and successive modifications.
A 72 years old female patient was admitted to ED for referred syncope. Her main complain at the time of admission was a diffuse pain, identified as Numeric Rating Scale (NRS) 7. On physical exam: post-traumatic left cheekbone ecchymosis, negative cardiopulmonary examination. Neurological exam revealed hypertonia, hypereflexia, hyperesthesia, hypotonic involving both superior and inferior limbs. On EKG we observed sinusal tachycardia (hearth rate 112 bpm); cervical south compuretized tomography (CT) was negative. Patient was suffering but stable during the night. Suddenly we assisted to a worsening of the patient clinical condition with drop of the arterial blood pressure treated with noradrenaline and a worsening of the respiratory condition, which required airway intubation. Cervical magnetic resonance (CMR) was performed as an emergency diagnostic exam for suspected neurological shock. CMR showed a cervical spinal cord injury (C2-C3 level). EKG was performed again and it shower a sinusal rhythm with 70 bpm and Epsilon wave which is included in the major criteria for the diagnosis of ARVD. Echocardiogram showed right ventricular dilatation, met the major criteria for ARVD (local, apical RV dyskinesia), which could be identified as a possible cause of the syncope.
This case report aim to underline the importance of a clinical suspicion of ARDV in individuals admitted to ED for syncope or for malignant ventricular arrhythmias even when the patient is not a young one or is not an athlete. Suspicion of ARDV/C should be considered even in elderly patients considering the possible late onset of the disease. Despite the old age, all the diagnostic criteria for ARDV were met so AQRDV was identified as the first cause for syncope and for the subsequent SCIWORA syndrome.
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