Shabanian Reza, Ahani Manizheh, Zandiyeh Shima, Nikdoost Aliyeh, Dadkhah Minoo, Asbagh Parvin Akbari, Badv Reza Shervin
Department of Pediatric Cardiology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Department of Pediatric Cardiac Surgery, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Ann Pediatr Cardiol. 2020 Apr-Jun;13(2):141-143. doi: 10.4103/apc.APC_73_19. Epub 2020 Apr 2.
A 5-year-old boy with the history of intractable seizure for the past 2 years was transferred to the emergency room for cardiopulmonary resuscitation because of the prolonged seizure and profound cyanosis. He was intubated and resuscitated by cardioversion for a bizarre shape ventricular tachycardia (VT). After noxious stimulation, he showed multiple polymorphic ventricular premature beats that were followed by a bidirectional VT in favor of catecholaminergic polymorphic VT. The genetic assessment was positive for CASQ2 mutation. In the follow-up, the arrhythmia was controlled by nadolol, however with a prominent neurological sequela.
一名5岁男孩,过去2年有难治性癫痫病史,因癫痫持续发作且伴有严重发绀被转至急诊室进行心肺复苏。他因怪异形态的室性心动过速(VT)接受了气管插管并通过心脏复律进行复苏。在有害刺激后,他出现了多个多形性室性早搏,随后是双向VT,提示儿茶酚胺能多形性VT。基因评估显示CASQ2突变呈阳性。在随访中,心律失常通过纳多洛尔得到控制,但出现了明显的神经后遗症。