Evolution of a rare ECG pattern in an aggressive case of neonatal tuberous sclerosis complex. (2018)

• Record Type: Journal Article
• Authors: Iezzi, Federica; Quarti, Andrea; Capestro, Alessandro; Surace, Francesca Chiara; Pozzi, Marco
• Is Part Of: International journal of surgery case reports. Volume 44(2018)
• Page Start: 197
• Page End: 201
• Publication Date: 2018
• Subjects: Diffuse rhadbomyomatosis -- ST-elevation -- Rhabdomyoma-like cells; Surgery -- Periodicals; Surgical Procedures, Operative -- Periodicals; Surgery; Electronic journals; Periodicals; 617.005
• DOI: 10.1016/j.ijscr.2018.02.030
• Languages: English
• ISSNs: 2210-2612

Abstract

Highlights: Cardiac rhabdomyomas may alter intracardiac electrical conduction. Rhabdomyoma arrhythmias are caused by isolated atrial depolarization disturbances. Destruction of conduction system has been described in diffuse rhabdomyomatosis. Abstract: Introduction: Rhabdomyomas are the most frequent cardiac tumors in children. Furthermore, they are often associated to tuberous sclerosis complex, an autosomal dominant neurocutaneous disorder characterized by tumor-like malformations that involve many organ systems. Presentation of the case: We describe a rare ECG pattern in a severe case of neonatal tuberous sclerosis complex. Discussion: In the presence of significant rhabdomyomatosis related to tuberous sclerosis, multiple clusters of rhabdomyoma-like cells can infiltrate the myocardium, with increased fibrosis areas. Conclusion: Considering the fact that rhabdomyomas often show spontaneous regression, close follow-up is sufficient in hemodynamically stable cases. Destruction of the conduction system, with arrhythmias as consequence, can be the presenting feature of diffuse rhabdomyomatosis.