C Lofiego

C Stöllberger

D Aras

EN Oechslin

F Ichida

LE Groot-de Laat De

PP Sengupta

R Engberding

RT Murphy

TK Chin

A. Yaksh

D. Haitsma

T. Ramdjan

K. Caliskan

T. Szili-Torok

N. M. S. de Groot

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CASE REPORTUnexpected finding in an adult with ventricular fibrillationand an accessory pathway: non-compaction cardiomyopathyA. Yaksh & D. Haitsma & T. Ramdjan & K. Caliskan &T. Szili-Torok & N. M. S. de GrootPublished online: 8 January 2014# The Author(s) 2014. This article is published with open access at Springerlink.comIntroductionIn this report, we demonstrate a patient presenting with anout-of-hospital cardiac arrest due to ventricular fibrillation(VF). At the hospital the presence of an accessory path-way could be seen on the surface electrocardiogram(ECG). Surprisingly, cardiac imaging also showed thepresence of isolated left ventricular non-compaction car-diomyopathy (INVM).INVM was first described in 1984 by Engberding et al.as an unclassified cardiomyopathy [1]. It is assumed to bethe result of an arrest of the compaction process duringthe normal development of the heart (week 5–8). InINVM, the spaces within the intertrabeculated meshworkpersist with deep recesses and no other cardiac abnormal-ities [1, 2]. Clinical presentation of INVM includesheart failure, thromboembolic events and arrhythmias[1, 3, 5, 7]. Conduction abnormalities and arrhythmiasobserved in INVM patients are left or right bundlebranch block, supraventricular tachycardia and ventric-ular tachycardia [1–3, 5–10].However, the presence of an accessory pathway and INVMin one patient with VF has never been described before.Case reportA 19-year-old female presented to the emergency depart-ment after an out-of-hospital cardiac arrest due to VF.After alcohol consumption she jumped off a 1 m highpier into the water. While dressing she complained ofdizziness, palpitations and breathlessness. She collapsednear her car and lost consciousness. The paramedics ar-rived within 7 min and provided cardiopulmonary resus-citation. VF was documented on arrival (Fig. 1). Afterthree DC shocks sinus rhythm resumed and due to a lowGlasgow Coma Score she was intubated. At the intensivecardiac care unit therapeutic hypothermia was induced for24 h. She regained consciousness without any signs ofpersistent neurological injury. Anamnestic there were noprevious palpitations or (near) collapses. The patient hadnoted that she was relatively quickly exhausted duringphysical exercise. Despite this, she played field hockeywithout any restraints. Her family history was negative forcardiovascular diseases, arrhythmias or sudden cardiacdeath. The 12-lead ECG after defibrillation showed pre-A. Yaksh :D. Haitsma : T. Ramdjan :N. M. S. de Groot (*)Department of Cardiology, Thoraxcentrum - Ba 579,Erasmus MC, ‘s Gravendijkwal 230,3015 CE Rotterdam, the Netherlandse-mail: nmsdegroot@yahoo.comK. Caliskan : T. Szili-TorokDepartment of Cardiology, Thoraxcentrum - Ba 577,Erasmus MC, ‘s Gravendijkwal 230,3015 CE Rotterdam, the NetherlandsA. Yaksh :D. Haitsma : T. Ramdjan :K. Caliskan : T. Szili-Torok :N. M. S. de GrootDepartment of Cardiology, Erasmus Medical Center,Rotterdam, the NetherlandsNeth Heart J (2014) 22:182–185DOI 10.1007/s12471-013-0513-9excitation with delta waves (positive in I, aVL, V1-6;negative in II, III, aVF) suggestive of a right-sidedposteroseptal accessory pathway (Fig. 1). Therefore, dur-ing hospitalisation, the patient underwent an electrophys-iology study. Figure 2 shows a Kent potential recorded ata right-sided posteroseptal bypass tract. The accessorypathway was successfully ablated at this site. The surfaceECG after the ablation procedure showed no pre-excitation (PR 122 ms) and no delta waves (Fig. 1).Hence, VF was most likely due to the presence of anFig. 1 Electrocardiograms. aDocumentation of ventricularfibrillation by ambulanceparamedics. b ECG recorded onarrival to the emergencydepartment, demonstrating sinusrhythm 99 beats/min, left axis, PRinterval 115 ms (pre-excitation),QRS duration 144 ms, QTc457 ms, delta waves (positive in I,aVL, V1-6; negative in II, III,aVF). c ECG after ablation of theaccessory pathway,demonstrating sinus rhythm 70beats/min, PR interval 122 ms,QRS duration 96 ms, QTc391 ms, no delta waves, T-waveinversion in the inferior leads, leftventricular hypertrophy withassociated depolarisationdisordersNeth Heart J (2014) 22:182–185 183accessory pathway. The only abnormality on the surfaceECG was left ventricular hypertrophy associated withdepolarisation disorders. Transthoracic echocardiographyshowed normal left ventricular function, no significantvalvular dysfunction and normal atrial dimensions.However, the left ventricle was hypertrabeculated anddilated (diastole 56 mm, systole 38 mm), suspicious fornon-compaction cardiomyopathy. The diagnosis of non-compaction cardiomyopathy was confirmed by magneticresonance imaging (MRI) (Fig. 3). Based on this finding,a subcutaneous ICD was implanted for secondary preven-tion in this 19-year-old patient.DiscussionThe prevalence of INVM differs between 0.014 % (adultseries) and 0.14 % in paediatric series [3]. Reported differ-ences in the prevalence of INVM are probably caused byincreased awareness of the existence of the disease over time.The true prevalence may be even higher, because only symp-tomatic patients are screened for INVM [2].Echocardiography is useful for diagnosing INVM but recentlyMRI has proven to be more accurate for diagnosing INVM [1,4–6]. The time course of development of ventricular tachyar-rhythmia in INVM patients is at present unknown but an ICDis recommended for primary prevention of ventricular tachy-arrhythmia [1–3, 6–10]So far, an accessory pathway has only been described intwo adult and four paediatric INVM patients. However, anadolescent patient with a Wolff-Parkinson-White syndromeand INVM presenting with VF has to our knowledge neverbeen described before. Based on clinical data, it is impossibleto determine whether VF was the result of either the INVM oratrial fibrillation with fast conduction over the accessorypathway.In conclusion, we describe a 19-year-old patient who pre-sented with an out-of-hospital cardiac arrest due to VF in theFig. 2 Catheter Ablation. Right panel: fluoroscopic image in LAOduring the electrophysiology procedure. The ablation catheter(AC) located at the right-sided posteroseptal accessory pathway,a multipolar catheter in the coronary sinus (CS) and a quadripolarcatheter on the His bundle (H). Left panel: the bipolar electrogramrecorded from the ablation catheter located at the right-sidedposteroseptal accessory pathway.Fig. 3 Cardiac imaging.Echocardiogram (a) and amagnetic resonance image (b)showing hypertrabeculation of theleft ventricle (arrow)184 Neth Heart J (2014) 22:182–185presence of a right-sided posteroseptal located accessory path-way. Surprisingly, we also found an INVM.Funding NoneConflict of interests None declaredOpen AccessThis article is distributed under the terms of the CreativeCommons Attribution License which permits any use, distribution, andreproduction in any medium, provided the original author(s) and thesource are credited.References1. Engberding R, Yelbuz T, Breithardt G. Isolated noncompaction of theleft ventricular myocardium – a review of the literature two decadesafter the initial case description. Clin Res Cardiol. 2007;96:481–8.2. Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction ofleft ventricular myocardium. A study of eight cases. Circulation.1990;82:507–13.3. Aras D, Tufekcioglu O, Ergun K, et al. Clinical features of isolatedventricular noncompaction in adults long-term clinical course,echocardiographic properties, and predictors of left ventricular fail-ure. J Card Fail. 2006;12:726–33.4. De Groot-de Laat LE, Krenning BJ, ten Cate FJ, et al. Usefulness ofcontrast echocardiography for diagnosis of left ventricularnoncompaction. Am J Cardiol. 2005;95:1131–4.5. Lofiego C, Biagini E, Pasquale F, et al. Wide spectrum of presenta-tion and variable outcomes of isolated left ventricular non-compaction. Heart. 2007;93:65–71.6. Sengupta PP, Mohan JC, Mehta V, et al. Comparison of echocardio-graphic features of noncompaction of the left ventricle in adultsversus idiopathic dilated cardiomyopathy in adults. Am J Cardiol.2004;94:389–91.7. Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: adistinct cardiomyopathy with poor prognosis. J Am Coll Cardiol.2000;36:493–500.8. Ichida F, Hamamichi Y, Miyawaki T, et al. Clinical features ofisolated noncompaction of the ventricular myocardium: long-termclinical course, hemodynamic properties, and genetic background. JAm Coll Cardiol. 1999;34:233–40.9. Murphy RT, Thaman R, Blanes JG, et al. Natural history and familialcharacteristics of isolated left ventricular non-compaction. Eur HeartJ. 2005;26:187–92.10. Stöllberger C, Finsterer J, Blazek G. Left ventricularhypertrabeculation/noncompaction and association with additionalcardiac abnormalities and neuromuscular disorders. Am J Cardiol.2002;90:899–902.Neth Heart J (2014) 22:182–185 185

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Unexpected finding in an adult with ventricular fibrillation and an accessory pathway: non-compaction cardiomyopathy

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Unexpected finding in an adult with ventricular fibrillation and an accessory pathway: non-compaction cardiomyopathy

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