Details

Autor(en) / Beteiligte

• Imon, Hikaru
• Kagajo-Tezuka, Mari
• Iwata, Haruka
• Watanabe, Ryusuke
• Ohta, Masaaki
• Matsubara, Yuko
• Higaki, Takashi
• Eguchi, Mariko

Titel

A neonatal case of cardiac rhabdomyoma successfully treated with everolimus

Ist Teil von

• Journal of Japan Society of Perinatal and Neonatal Medicine, 2020, Vol.56(1), pp.148-153

Ort / Verlag

Japan Society of Perinatal and Neonatal Medicine

Erscheinungsjahr

2020

Link zum Volltext

Quelle

Free E-Journal (出版社公開部分のみ）

Beschreibungen/Notizen

• Cardiac tumors are rare during childhood. The most common cardiac tumor in infants and in children is rhabdomyoma accounting for up to 64％ of all pediatric cardiac tumors. Cardiac rhabdomyoma is often asymptomatic and spontaneous regression is frequently observed. On the other hand, cardiac dysfunction, outflow obstruction and arrhythmia has been reported, resulting in sudden death in some cases. The principal therapy of cardiac rhabdomyoma is symptomatic treatment, although surgical intervention could be a choice for cases with severe symptoms. Recently mTOR inhibitor such as everolimus became available for cardiac rhabdomyoma and is reported to be effective especially in the treatment during fetal to neonatal periods. We report a case diagnosed as cardiac rhabdomyoma by fetal echography at 34-week of gestation. The tumor size was around 3.0cm2 showing a left ventricular obstruction. As the risk of sudden death is considered to be high because ventricular premature contraction rapidly increased, treatment with everolimus was introduced from the day of birth. As a result, the tumor size reduced from 3.0cm2 to 1.4cm2 at 14th day following administration, resulting in disappearance of ventricular premature contraction. Administration of everolimus was ceased at 32-day old. Thereafter, the tumor has increased slightly but no symptoms of obstruction of left ventricular out flow tract have been observed. To provide early and appropriate treatment intervention, careful follow up of cardiac function and arrhythmia from pre-natal period is essential.