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Cardiovascular Disease |

An Uncommon Presentation of Broad Complex Tachycardia With Aberrant Conduction in Patient With Ventricular Noncompaction Cardiomyopathy

Urooj Fatima, MD; Muhammad Ebrahim Khan, MD; Gunjan Choudhary, MD; Abhishek Kulkarni, MD; Maninder Singh, MD; Dwight Stapleton, MD
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Guthrie/Robert Packer Hospital, Sayre, PA


Chest. 2014;146(4_MeetingAbstracts):100A. doi:10.1378/chest.1993960
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Abstract

SESSION TITLE: Cardiovascular Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Left ventricular Non-compaction cardiomyopathy is a rare entity with the prevalence of about 0.014% . It occurs due to arrest of normal embryogenesis of the endocardium and myocardium that results in spongy appearance of the myocardium. Unlike other forms of cardiomyopathy it can manifest as systemic thromboemboli , arrhythmias or congestive heart failure ,with echocardiography being the diagnostic procedure of choice.

CASE PRESENTATION: 47 Years old male with the established daignosis of left ventricular non-compaction cardiomyopathy diagnosed at the age of 45 years through echocardiography when he was found to have extensive trabeculations of the left ventricle cavity in the mid to apical region and along the inferior wall , was admitted with multiple syncopal episodes . On review of EKG from the transferring facility he was found to have broad complex tachycardia with aberrant left bundle branch block pattern . For further evaluation patient underwent electrophysiology study that showed inducible ectopic atrial tachycardia post ablation that required ablation around the coronary sinus however no inducible ventricular arrhythmias were seen. On the echocardiography patient was found to have dilated cardiomyopathy with biventricular systolic dysfunction ,ejection frcation of 15% and severely enlarged left atrium with mildly dilated right atrium and dilated coronary sinus of around 15mm. To rule out coronary artery disease patient underwent cardiac catheterization that showed normal coronary arteries. He was continued with aggressive medical management along with the AICD placement for the primary prevention of the sudden cardiac death.

DISCUSSION: Left ventricular Non-compaction cardiomyopathy can result in both systolic and daistolic dysfunction .Arrhythmias are common finding in left ventricular non-compaction cardiomyopathy with the percentage of ventricular arrhythmia being around 47% and atrial fibrillation around 25%, however rest of the arrhythmias are rare. Daignosis is usually made by echocardiography that shows prominent ventricular trabeculations and intratrabecular recesses.These patients benefit from aggressive medical management and AICD placement as usually the prognosis of this entity of cardiomyopathy is poor leading to cardiac transplantation or death as early as within 6 years of diagnosis.

CONCLUSIONS: This case is unique as patient with the established daignosis of left ventricular non-compaction cardiomyopathy presented with broad complex tachycardia with aberrant conduction rather then commonly encountered atrial fibrillation or ventricular arrhythmias .The possible etiology of this seemed to be dilated atrium and coronary sinus ,with the focus of arrhythmia being the OS of coronary sinus.

Reference #1: 2004; 109: 2965-2971 doi: 10.1161 circulations.

Reference #2: Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997;72: 26-31

DISCLOSURE: The following authors have nothing to disclose: Urooj Fatima, Muhammad Ebrahim Khan, Gunjan Choudhary, Abhishek Kulkarni, Maninder Singh, Dwight Stapleton

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