116 ‘Joy to be hidden, disaster not to be found’- rare case of intra cardiac lipoma in a child

Abstract

<h3>Aims</h3> Primary cardiac tumors in children although rare, are reported to be increasing annually in recent years. Most of them are benign in nature with the most common benign tumors being rhabdomyosarcoma, fibroma, and myxomas. Intracardiac lipomas have been reported in a few pediatric patients but none in the left atrium. We report a unique case of left atrial lipoma. <h3>Methods</h3> A 9 years old boy previously fit and well was referred due to increasing frequency of chest pain and a ‘racing heartbeat’ soon after the chest pain. The events lasted for a few seconds on a scale of 7 out of 10 and were random both at school and home. A family history of congenital cardiac disease was mentioned in the first cousin. Clinical examination was unremarkable. Baseline 12 lead electrocardiogram (ECG) showed sinus arrhythmia with normal QTc and a left axis deviation of 96 degrees. An echocardiogram showed situs solitus with atrioventricular-ventriculo atrial concordance. A 1.6cm*3.6cm hyper-echogenic, non-pedunculated mass was noted in the left atrial free wall protruding into the left atrial cavity with fractional shortening of 30% and ejection fraction of 56%. Cardiac magnetic resonance imaging (MRI) showed well-defined posterior mediastinal mass abutting the posterior left atrial wall between the right and left-sided pulmonary veins typical for lipoma. He was managed conservatively with regular outpatient follow-up after discussion with the tertiary center. <h3>Results</h3> Cardiac lipomas are rare benign tumors of the heart. They are usually asymptomatic and are thus most often diagnosed on autopsies. The median age was 13 years. The locations were in right AV grove, right ventricle, and central fibrous body. They were located in different regions of the heart but none in the left atrium.¹ The diagnostic modality of choice is cardiac MRI. Treatment guidelines have not yet been established due to the very low prevalence of these tumors and no randomized clinical trials. The management is thus guided by the patient‘s symptomatology.² Although benign, these lipomas can be aggressive causing signs of cardiac compromise. A surgical approach is justified in symptomatic patients, to alleviate symptoms and to prevent the progression of the disease. However, treatment in asymptomatic patients poses a serious dilemma to the patient and the clinician as there is no consensus to date.³ <h3>Conclusion</h3> Intracardiac lipomas can present from being asymptomatic to aggressive cardiac failure or sudden cardiac arrest. The importance of a 12-lead ECG and an echo should be emphasized in children presenting with cardiac symptoCardiac MRI is diagnostic and management depends on a decision by a joint specialist team along with radiologists and cardiac surgeons. <h3>References</h3> Shi L. Identification and clinical course of 166 pediatric cardiac tumors. <i>European journal of Pediatrics</i>. 2017. Miyake CY. Cardiac tumors and associated arrhythmias in pediatric patients, with observations on surgical therapy for ventricular tachycardia. <i>Journal of the American College of Cardiology</i>. 2011. Moinuddeen K. Lipomatous interatrial septal hypertrophy: an unusual cause of intracardiac mass. <i>European Journal of Cardio-thoracic Surgery</i>. 2002.