We describe a case report of a patient with cardiac arrest and Ebstein’s anomaly. This case report shows us necessity for arrhythmia evaluation and sudden death risk stratification even in asymptomatic patients. Prophylactic ICD im- plantation in this patient population is limited to observational studies and the selection of patients is impeded by the absence of randomized trials and weak predictors

Borka Pezo Nikolić

Boško Skorić

Bruno Buljević

Daniel Lovrić

Davor Miličić

Davor Puljević

Jadranka Šeparović Hanževački

English

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Coll. Antropol. 37 (2013) 4: 1357–1359Case reportCardiac Arrest in a Patient with Ebstein’sAnomaly without Accessory PathwaysBorka Pezo Nikoli}, Daniel Lovri}, Davor Puljevi}, Davor Mili~i}, Bruno Buljevi}, Bo{ko Skori}and Jadranka [eparovi} Han`eva~kiUniversity of Zagreb, School of Medicine, University Hospital Centre Zagreb, Department for Cardiovascular Diseases, Zagreb,CroatiaA B S T R A C TWe describe a case report of a patient with cardiac arrest and Ebstein’s anomaly. This case report shows us necessityfor arrhythmia evaluation and sudden death risk stratification even in asymptomatic patients. Prophylactic ICD im-plantation in this patient population is limited to observational studies and the selection of patients is impeded by the ab-sence of randomized trials and weak predictors.Key words: Ebstein anomaly, ventricular fibrillation, cardiac arrest, implantable cardiac defibrillatorIntroductionEbstein’s anomaly (EA) is a rare congenital heart dis-order accounting for <1% of all congenital heart defects.It is characterized by apical displacement of the septaland posterior tricuspid valve leaflets, leading to atria-lization of the right ventricle with a variable degree ofmalformation and displacement of the anterior leaflet.The abnormal development of the tricuspid valve in pa-tients with Ebstein’s anomaly results in several activa-tion abnormalities including delayed intraatrial conduc-tion, right bundle branch block (RBBB), and ventricularpreexcitation1.Case Report26 years old woman was brought to the emergency de-partment (ED) in a state of extreme cyanosis withoutmeasurable blood pressure or signs of spontaneousbreathing. Upon arrival in the ED ventricular fibrillationwas registered and the patient was defibrillated to sinusrhythm at the first attempt. After restoring sinusrhythm ECG revealed right bundle branch block (RBBB)without other pathological findings. The patient wasintubated and endovenous access was established. TheED staff was informed that she has had pulseless arrestfor more than 10 minutes before admittance and she wasaddmitted to the coronary care unit.Sixty minutes upon resuscitation, moderate hypo-thermia was initiated. The goal temperature of 33 °C wasachieved within 4 hours and hypothermia was main-tained for 18 hours. Rewarming of the patient occurredsubsequently during 24-hour period, without adverseevents. After rewarming she was breathing spontane-ously without overt signs of neurological damage. Thebrain CT scan was normal. Her sister explained that shewas diagnosed with EA in her childhood, but there wasno history of syncope or palpitations. She was clinicalyasymptomatic, in New York Heart Association (NYHA)class I, with regular follow up performed by her cardiolo-gist. Echocardiography exams were performed every twoyears.Transthoracic echocardiography exam was performedand showed neither pericardial effusion, nor left ventric-ular (LV) hypertrophy or left ventricular segmental wallmotion abnormalities. The LV ejection fraction was nor-mal. A typical echocardiographic finding for EA waspresent comprising of massively dilated right heartchambers, malposition of the anterior and septal tri-cuspid valve leaflets, »atrialization« of the right ventricleand mild tricuspid regurgitation (Figure 1). Cardiac MRIwith a Magnetom 3T (Siemens AG, Munich, Germany)was performed without any pathological findings outsideof the presence of EA.1357Received for publication May 1, 2012The patient underwent electrophysiology study withcatheters placed in the high right atrium, the bundle ofHis, right ventricular apex, and in the left atrium via pat-ent foramen ovale. The baseline intervals were normal:AH 90 ms and HV 50 ms. A dual AV-physiology was pres-ent. The electrophysiology study showed no evidence ofaccessory pathway. Also, no ventricular tachycardia wasinduced by programmed ventricular stimulation usingWellens protocol before and after isoproterenol adminis-tration.Implantation of implantable cardioverter defibrillatorwas performed to provide secondary prevention and pa-tient was released from the hospital.DiscussionWe are reporting a case of cardiac arrest with a docu-mented ventricular fibrillation in a female patient withEA. There is an emerging question about the etiologyand the nature of the arrest in our patient. The assess-ment and diagnostic workup did not reveal any abnor-mality that could be brought in connection with theevent. Theoretically, the arrest could be the consequenceof 1) a ventricular tachycardia that degenerated to ven-tricular fibrillation, 2) primary ventricular fibrillation, 3)a bradycardia that elicited secondary tachyarythmia, or4) some non-cardiac cause. All possible reasons havebeen described in the literature in a variety of patients.In most cases an arrhytmic death was assumed. Evenmore, in one cohort of patients that underwent surgicalcorrection of the anomaly the majority suffered suddencardiac death2.Presumably, tachyarrhythmias are the most frequentcause of death in such patients. Due to the developmen-tal changes that displace the leaflets, the fibrous tissue intrigonum fibrosum can also be changed. It is well knownthat patients with Ebstein’s anomaly commonly evenhave multiple accessory pathways. One third of patientswith Ebstein’s anomaly and symptomatic tachyarrhyth-mias have minimal or absent ECG features of ventricularpreexcitation. In these patients, the absence of RBBBpattern is a strong predictor of an AP. It is also knownthat most of them having RBBB do not have accessorypathway. In accordance with that, our reported patienthad RBBB and no evidence of accessory pathway inelectrophysiological study. In a small number of patientsventricular tachycardia was reported3.There are also case reports in the literature with pa-tients in whom bradyarrhythmias were detected, espe-cially atrial standstill. Altough ventricular tachycardia /ventricular fibrillation is not often in EA patient compar-ing with other congenital heart defects, this case reportshows the necessity for arrhythmia evaluation and sud-den death risk stratification even in asymptomatic pa-tients4. Current experience with implantable cardiover-ter defibrillators in this patient population is limited toobservational studies and the selection of patients forprophylactic implantable cardioverter defibrillator im-plantation is impeded both by the absence of randomizedtrials and weak predictors. Catheter ablation of ventricu-lar tachycardia has emerged as a promising therapy forabolishing or reducing the burden of arrhythmia but ex-perience is still limited and the impact on long-term out-come uncertain. Future studies will have to focus on im-proving risk stratification of patients with congenitalheart disease5.R E F E R E N C E S1. ITURRALDE P, NAVA S, SÁLICA G, MEDEIROS A, MARQUEZMF, COLIN L, VICTORIA D, DE MICHELI A, GONZALES MD. J Car-diovasc Electrophysiol, 17 (2006) 1332. DOI: 10.1111/j.1540-8167.2006.00617.x. — 2. HUANG CJ, CHIU IS, LIN FY, CHEN WJ, LIN JL, LO HM,WU MH, CHU SH, Thorac Cardiovasc Surg, 48 (2000) 347. DOI: 10.1055/s-2000-8348. — 3. OBIOHA-NGWU O, MILLIEZ P, RICHARDSONA, PITTARO M, JOSEPHSON ME, Circulation, 30 (2001) 104. DOI: 10.1161/hc4301.098011. — 4. WALSH EP, Circulation, 115 (2007) 3224.DOI: 10.1161/. — 5. YAP SC, HARRIS L, Expert Rev Cardiovasc Ther, 7(2009)1605. DOI: 10.1586/erc.09.153.B. Pezo Nikoli}University of Zagreb, School of Medicine, University Hospital Centre Zagreb, Department for Cardiovascular Diseases,Ki{pati}eva 12, Zagreb 10000, Croatiae-mail: borkapezo@yahoo.comB. Pezo Nikoli} et al.: Cardiac Arrest in Ebstein’s Anomaly, Coll. Antropol. 37 (2013) 4: 1357–13591358Fig. 1. Example of an echocardiogram (apical 4-chamber view) ofa patient with Ebstein’s anomaly. The functional RV is small, re-placed by the large RVA. RV – right ventricle; RVA – atrializedright ventricle; TV – tricuspid valve, LA – left atrium; LV – leftventricle; RA – right atrium.SR^ANI AREST KOD BOLESNICE S EBSTEINOVOM ANOMALIJOMS A @ E T A KOpisali smo slu~aj sr~anog aresta kod bolesnice s Ebsteinovom anomalijom. Ovaj slu~aj nam ukazuje na nu`nostevaluacije aritmija te stratifikacije rizika iznenadne sr~ane smrti ~ak i kod asimptomatskih pacijenata. U skupini ovak-vih bolesnika profilakti~ka ugradnja ICD-a ograni~ena je samo na opservacijske studije dok je izbor bolesnika ote`anzbog nedostatka randomiziranih istra`ivanja te nedovoljno definiranih predskazatelja rizika.B. Pezo Nikoli} et al.: Cardiac Arrest in Ebstein’s Anomaly, Coll. Antropol. 37 (2013) 4: 1357–13591359

Cardiac Arrest in a Patient with Ebstein’s Anomaly without Accessory Pathways

Cardiac Arrest in a Patient with Ebstein’s Anomaly without Accessory Pathways

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