Finsterer Josef, Stöllberger Claudia, Demirtas Dita, Gencik Martin, Ohnutek Irene, Hornykewycz Anette
Krankenanstalt Rudolfstiftung , Vienna , Austria.
Acute Card Care. 2014 Dec;16(4):115-7. doi: 10.3109/17482941.2014.944538. Epub 2014 Aug 7.
Stress-induced cardiomyopathy (takotsubo-syndrome, TTS) and its recurrence have not been described in myotonic dystrophy-1.
The patient was a 47-year-old female who was suspected to suffer from myotonic dystrophy-1 at 20 years of age, upon the typical clinical presentation and the electrophysiological findings. During weaning from general anesthesia for resectioning of a pelvic tumour she developed ventricular fibrillation, but was successfully resuscitated. During coronary angiography two days later she experienced recurrent QT-prolongation, torsades de pointes, and ventricular fibrillation, but was successfully resuscitated again each time. Echocardiography and electrocardiography were indicative of TTS, which was confirmed by normal findings on echocardiography and electrocardiography two months later. Ten months after the first TTS she developed dyspnea, leg edema, and anginal chest pain. Recurrence of TTS was diagnosed upon a typical electrocardiography and echocardiography findings. Shortly after onset of the second TTS, she unexpectedly died from sepsis.
TTS may also occur in patients with myotonic dystrophy-1 induced by stress from surgery, respiratory insufficiency, or infection. In patients with myotonic dystrophy-1, takotsubo-syndrome may recur and may represent a previously unreported feature of cardiac involvement in myotonic dystrophy-1.
1型强直性肌营养不良患者中尚未见应激性心肌病(Takotsubo综合征,TTS)及其复发的相关报道。
该患者为47岁女性,20岁时因典型临床表现及电生理检查结果被怀疑患有1型强直性肌营养不良。在盆腔肿瘤切除术后全身麻醉撤机过程中,她发生了室颤,但成功复苏。两天后冠状动脉造影时,她再次出现QT间期延长、尖端扭转型室速和室颤,但每次均成功复苏。超声心动图和心电图提示TTS,两个月后超声心动图和心电图结果正常得以证实。首次发生TTS后10个月,她出现呼吸困难、腿部水肿和心绞痛。根据典型的心电图和超声心动图表现诊断为TTS复发。第二次TTS发作后不久,她意外死于败血症。
TTS也可能发生在因手术、呼吸功能不全或感染等应激因素诱发的1型强直性肌营养不良患者中。在1型强直性肌营养不良患者中,Takotsubo综合征可能复发,可能是1型强直性肌营养不良心脏受累的一种此前未报道的特征。