A 68-year-old man underwent percutaneous transluminal coronary angioplasty (PTCA) to left anterior descending artery (LAD) seg 7 after acute anteroseptal myocardial infarction 8 years previously. He was admitted because of syncope attack due to sustained ventricular tachycardia and subsequent fibrillation. He was treated medically in the ICU after cardiopulmonary resuscitation. Medical treatment with amiodarone and lidocaine was not successful and he was transferred to our hospital for surgical treatment of malignant ventricular tachycardia (VT) associated with left ventricular aneurysm and acute cholecystitis that occurred during admission. Left ventriculogram showed left ventricular aneurysm (ejection fraction: 35%) without any significant coronary lesions. The patient successfully underwent a Dor operation (left ventriculoplasty), double encircling endocardial cryoablation without endocardial resection, and preoperative and intraoperative endocardial mapping. Cholecystectomy was simultaneously performed after complete closure of the median chest incision. The recurrence of VT was never recognized clinically or electrophysiologically. The extended encircling endocardial cryoablation without endocardial resection and preoperative and intraoperative electrophysiological study, was a simple and effective method for ventricular tachycardia.

Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating anti-enterocyte autoantibodies. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, and mutations in FOXP3, which is a master gene of regulatory T cells (Tregs), are major causes of AIE. Recent studies have demonstrated that mutations in other Treg-associated genes, such as CD25 and CTLA4, show an IPEX-like phenotype. We present the case of a 13-year-old girl with CTLA4 haploinsufficiency, suffering from recurrent immune thrombocytopenic purpura and intractable diarrhea. We detected an autoantibody to the AIE-related 75 kDa antigen (AIE-75), a hallmark of the IPEX syndrome, in her serum. She responded well to a medium dose of prednisolone and a controlled dose of 6-mercaptopurine (6-MP), even after the cessation of prednisolone administration. Serum levels of the soluble interleukin-2 receptor and immunoglobulin G (IgG) were useful in monitoring disease activity during 6-MP therapy. In conclusion, autoimmune-mediated mechanisms, similar to the IPEX syndrome, may be involved in the development of enteropathy in CTLA4 haploinsufficiency. Treatment with 6-MP and monitoring of disease activity using serum levels of soluble interleukin-2 receptor and IgG is suggested for such cases.