Sick Sinus Syndrome in a Patient with Extensive Cardiac Lipomatosis (Sinus Node Dysfunction in Lipomatosis)

Abstract

We present a case of a 45-year-old man with an incidental and longstanding diagnosis of extensive mediastinal and cardiac lipomatosis. Along the years, he had experienced various arrhythmias, mainly bradyarrhythmias, mostly asymptomatic. Recently after documenting a sinus pause of 6 seconds and runs of nonsustained ventricular tachycardias, he underwent an implantation of a cardioverter-defibrillator. There are many reports of cardiac lipomatosis in the literature, including reports of related ventricular arrhythmias, some of which are fatal. (PACE 2010; 513-515).

Pathological and Comprehensive Genetic Investigation of Autopsy Cases of Idiopathic Bradyarrhythmia

“…Additionally, a previous study showed that a few cases of SND had extensive cardiac lipomatosis as a disease-causing lesion. 7 Our Case 1 showed that reduction in functional nodal cells may be a more common pathological substrate of advanced SND rather than fibrosis and/or fatty infiltration in many cases.…”

“…fatty infiltration or neoplastic change, have been reported as pathological substrates for bradyarrhythmia, and advanced fibrosis was not observed in such cases. 7 Therefore, objective morphological investigation into the amount of conduction fibers may be important for considering the etiology of idiopathic bradyarrhythmia. Moreover, the etiology of degeneration and/or fibrosis in the conduction system in idiopathic bradyarrhythmia cases has not yet been explored.…”

Massive Cardiac Lipomatosis of the Right Heart as Cause of Sudden Cardiac Death

“…In the reported case, the absence of significant fibrosis and/or degenerative myocytic changes were in favour of fatty infiltration of both right atrium and ventricle. Various arrhythmias and conduction disturbances have been reported in association with this condition [1]. In the most frequent type of cardiac lipomatosis, the infiltration of the interatrial septum, commonly cause premature atrial contractions, supraventricular tachycardia and atrial fibrillation [4], conversely, the involvement of the atrio-ventricular node can lead to a conduction block [5].…”

“…In most cases, since the patients are typically asymptomatic, this finding is incidental, found either by imaging studies or during autopsies [1]. Over 200 cases have been described [1] in the literature.…”

“…In most cases, since the patients are typically asymptomatic, this finding is incidental, found either by imaging studies or during autopsies [1]. Over 200 cases have been described [1] in the literature. We describe the autopsy case of a patient with sudden cardiac death caused by massive cardiac lipomatosis of the right heart with dysfunction of the atrioventricular node.…”