Gotyo Naoki, Kida Michiya, Horiuchi Toshiyuki, Hirata Yukio
Department of Endocrinology and Metabolism, Tokyo Metropolitan Toshima Hospital, Tokyo, Japan.
Endocr J. 2009;56(6):807-15. doi: 10.1507/endocrj.k09e-080. Epub 2009 Jun 9.
We describe here a patient with torsade de pointes associated with recurrent ampulla cardiomyopathy, who was later proven to suffer from idiopathic AC TH deficiency. A 70-year-old man was admitted to our hospital for bacterial pneumonia. A cardiac examination performed on admission revealed ampulla cardiomyopathy, which improved spontaneously as the pneumonia was cured. Two months after discharge, he was transferred to our hospital for relapse of the pneumonia. After the second admission, the pneumonia subsided with antibiotic treatment and his general condition ameliorated gradually. However, on the 20(th) hospital day, he was found lying on the floor in a prone position in cardiopulmonary arrest. Cardiac telemetry monitoring showed torsade de pointes worsening to ventricular fibrillation, and immediate cardiac defibrillation was performed. The electrocardiogram after successful defibrillation showed inverted T waves in the chest leads with long QT intervals, and subsequent emergent coronary catherization revealed the recurrence of ampulla cardiomyopathy. Thereafter, endocrinological examinations for the diagnosis of sustained hyponatremia demonstrated secondary adrenal insufficiency caused by idiopathic AC TH deficiency. The cardiomyopathy resolved promptly after steroid hormone replacement without relapse as did the hyponatremia. Patients with ampulla cardiomyopathy or ventricular fibrillation without apparent etiology should be examined for adrenal function. If begun as soon as adrenal insufficiency is diagnosed, immediate steroid replacement therapy can prevent the deterioration and relapse of cardiac involvement.
我们在此描述一名患有与复发性壶腹性心肌病相关的尖端扭转型室速的患者，该患者后来被证实患有特发性促肾上腺皮质激素（ACTH）缺乏症。一名70岁男性因细菌性肺炎入住我院。入院时进行的心脏检查发现有壶腹性心肌病，随着肺炎治愈，该病症自行改善。出院两个月后，他因肺炎复发转至我院。第二次入院后，经抗生素治疗肺炎消退，他的总体状况逐渐改善。然而，在住院第20天时，他被发现俯卧在地板上，处于心肺骤停状态。心脏遥测监测显示尖端扭转型室速恶化为心室颤动，随即进行了心脏除颤。成功除颤后的心电图显示胸导联T波倒置，QT间期延长，随后的急诊冠状动脉造影显示壶腹性心肌病复发。此后，针对持续性低钠血症的内分泌检查表明，该患者患有由特发性ACTH缺乏引起的继发性肾上腺功能不全。使用类固醇激素替代治疗后，心肌病迅速缓解，未再复发，低钠血症情况也如此。对于患有壶腹性心肌病或无明显病因的心室颤动的患者，应检查其肾上腺功能。一旦诊断出肾上腺功能不全，立即开始类固醇替代治疗可预防心脏病变的恶化和复发。
