Left Ventricular Noncompaction and Pre-Excitation: An Unusual Finding in Adults

Abstract

Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy characterized by prominent left ventricular (LV) trabeculae, deep intertrabecular recesses, and the thin compacted layer. The disease is potentially associated with sudden cardiac death due to LV dysfunction and ventricular arrhythmias. The presence of accessory pathway and Wolff-Parkinson-White syndrome is particularly rare in adults. Here we describe the rare association of LVNC and ventricular pre-excitation in an 18-year-old female with neonatal hypoxic brain injury. Keywords: Wolff-Parkinson-White syndrome; pre-excitation syndromes; isolated noncompaction of the ventricular myocardiumLeft ventricular noncompaction (LVNC) is a rare form of cardiomyopathy characterized by prominent left ventricular (LV) trabeculae, deep intertrabecular recesses, and the thin compacted (C) layer 1 . The diagnosis is usually performed by echocardiography or magnetic resonance imaging (MRI). The disease is potentially associated with sudden cardiac death (SCD) due to LV dysfunction and ventricular arrhythmias. The presence of accessory pathway and Wolff-Parkinson-White (WPW) syndrome is particularly rare in adults 2,3 . Here we describe the rare association of LVNC and ventricular pre-excitation in an 18-year-old female with neonatal hypoxic brain injury. There were no arrhythmias or WPW syndrome. Clinical follow-up was performed without complications. CASE PRESENTATIONAn 18-year-old female with severe neonatal hypoxic brain injury and chronic cognitive and motor disabilities was referred for outpatient cardiac consultation for preoperative evaluation. She presented severe scoliosis and motor disabilities requiring orthopedic surgery for spine stabilization. Her cardiac medical history was uneventful. Despite limited functional status, her parents denied any warning symptoms like chest pain, shortness of breath, palpitations, or syncope. Physical examination revealed good peripheral pulses, no peripheral edema, blood pressure 102/60 mmHg, and heart rate of 96 bpm. Cardiac auscultation showed regular rhythm with no murmurs or gallops. There were no signs of cardiomegaly.A 12-lead electrocardiography (ECG) revealed ventricular pre-excitation with right-bundle branch block morphology compatible with a left wall accessory-pathway (Figure 1). A 24-hour Holter did not record arrhythmias. A transthoracic echocardiogram was also performed. Despite limited acoustic window, the left ventricular ejection fraction (LVEF) was normal. However, there were signs of prominent trabeculation and deep recesses that communicate with the left ventricular cavity (Figure 2). These signs were compatible with isolated LVNC.An electrophysiological study (EPS) was considered, but deferred by patient's family. The patient underwent surgical procedure under cardiac monitoring. No arrhythmias or cardiovascular complications were reported. After 1 year of clinical follow-up, LVEF remained unaltered and the patient