Paroxysmal Supraventricular Tachycardia in Neonatal Tuberous Sclerosis Complex and Cardiac Rhabdomyoma: Report of One Case

心臟橫紋肌瘤是結節性硬化症常見的症狀之一，也是兒童最常見心臟腫瘤。其表現可能以毫無症狀、心律不整、血循障礙及猝死來表現。雖然結節性硬化症常可以見到心臟橫肉紋肌瘤，但在文獻報告中，新生兒結節硬化症合併心臟橫肉紋肌瘤以上心室頻脈爲初始症狀表現者極爲少見。我們報告一例女性新生兒因在子宮發現上心室頻脈而於34週早産産出，並自外院轉入本院。在本院心臟超音波發現有數個心臟腫瘤、開放性動脈導管及心房缺損，經由adenosine及digoxin注射，其上心室頻脈雖能暫時消失，但是仍會斷續復發。第十六天大復發時，經藥物給予仍無效，因此在同步化電擊緩解上心室頻脈後，我們給予digoxin長期治療，此後無心律不整復發及其他合併症出現。電腦斷層及核磁共振發現腦部有結節性硬化病竈，皮膚上有低色素沈著斑塊，經確診爲結節性硬化症。九個月後的追蹤雖然此一病患沒有進一步的心律不整復發，但心臟超音波仍然發現心臟橫紋肌留尚未變小而且有一腫瘤位於左心室出口，我們建議病人仍應定期追蹤觀察。

關鍵字

結節性硬化症；心臟橫紋肌瘤；上心室頻脈

並列摘要

Cardiac rhabdomyoma, the most common cardiac tumor of infant and child, has been known to be associated with tuberous sclerosis complex. However, tuberous sclerosis complex with fetal supraventricular tachycardia is rarely reported. Herein, we report a female neonate diagnosed as tuberous sclerosis complex with cardiac rhabdomyoma presenting as supraventricular tachycardia. The newborn infant was initially treated with vagal maneuver, adenosine push, and digoxin. Although the tachycardia converted into normal sinus rhythm, frequent recurrence of PSVT was noted. At 16 days of age, the recurrent supraventricular tachycardia was refractory to vagal maneuver and drug therapy. Direct-current synchronized cardioversion converted the arrhythmia to normal sinus rhythm and oral digoxin effectively controlled the condition of the patient without further PSVT attacks. Subsequent 9-month follow-up revealed no further attack of arrhythmia. Regression of cardiac rhabdomyoma has not been visualized on echocardiogram at 9 months old.

並列關鍵字

Tuberous sclerosis complex ； cardiac rhabdomyoma ； supraventricular tachycardia

國際替代計量

Paroxysmal Supraventricular Tachycardia in Neonatal Tuberous Sclerosis Complex and Cardiac Rhabdomyoma: Report of One Case

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