TY - JOUR
T1 - Evolution of a rare ECG pattern in an aggressive case of neonatal tuberous sclerosis complex
AU - Iezzi, Federica
AU - Quarti, Andrea
AU - Capestro, Alessandro
AU - Surace, Francesca Chiara
AU - Pozzi, Marco
N1 - Publisher Copyright:
© 2018 The Authors
PY - 2018
Y1 - 2018
N2 - 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.
AB - 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.
KW - Diffuse rhadbomyomatosis
KW - Rhabdomyoma-like cells
KW - ST-elevation
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U2 - 10.1016/j.ijscr.2018.02.030
DO - 10.1016/j.ijscr.2018.02.030
M3 - Article
AN - SCOPUS:85043356856
SN - 2210-2612
VL - 44
SP - 197
EP - 201
JO - International Journal of Surgery Case Reports
JF - International Journal of Surgery Case Reports
ER -