Ro/SSA antibody, complete atrioventricular block, pacemaker implantation, QT prolongation, torsade de pointes. Congenital complete atrioventricular block (CAVB) is a rare but life-threatening disease. Most CAVB cases are caused by the transfer of maternal anti-Ro/SS-A antibody to the fetus through the placenta. Anti-Ro/SS-A antibody associated CAVB generally occurs in the neonatal period; however, it may occasionally occur later. Recently, it has been reported that anti-Ro/SS-A antibody contributes not only to CAVB but also to the development of sinus bradycardia or QT prolongation. Here we report an infant case of refractory torsade de pointes (TdP), which was controlled by a pacemaker management. A 2-year-old boy visited our hospital because he suffered from bronchitis. Nebulized beta stimulant, expectorant, and leukotriene receptor antagonist were administered. During the physical examination, we noticed the patient's bradycardia. We performed an electrocardiogram (ECG) and significant bradycardia and QT prolongation were confirmed (Fig. 1a). A medical history taken from the mother revealed that she was found to be positive for the anti-Ro/SSA antibody and the patient had been noted as having severe bradycardia during fetal period in another institution. We therefore retrieved his previous ECG during his neonatal period and reconfirmed the findings (Fig. 1b). An ECG that was performed immediately after his birth showed no abnormal findings, so he did not receive clinical follow up after the neonatal period. We requested further investigation in the initial institution, but the recorded ECG revealed a normal corrected QT interval (Fig. 1c). Holter ECG was therefore scheduled. Six months after suffering from bronchitis, he was referred to our emergency room because of febrile seizure. An ECG detected TdP (Fig. 1d-1), and magnesium sulfate was infused immediately. Subsequent ECG after elimination of TdP

Introduction: Recent advances of CT technology have made it possible to obtain clear coronary artery lesion (CAL) image. However, high dose radiation exposure is inevitable. Also it becomes difficult to obtain adequate images when patient’s heart rate is high. Recently 320row area detector CT (ADCT) has been introduced. Using this device, clear coronary artery images are obtainable with low dose radiation exposure. Also it provides adequate image for high heart rate patients. However, there is few report which describe its utility for infantile patients. Aim: To evaluate the feasibility of 320row area detector CT for evaluation of CAL for infantile KD patients. Subjects and methods: Nine infants who have CAL due to KD have been evaluated. Mean age at examination was 30.5+/-22.0 (mean+/-SD) months, weight was 13.6+/-6.5 kg and mean time elapse from diagnosis of KD was 18.7+/-16.0 months. Patients were evaluated using ADCT and findings obtained were compared with either coronary angiography (CAG) or echocardiography. Radiation exposure dose were also explored. Results: All patients have accomplished examination and adequate images were obtained. Mean heart rate at examination was 105+/-27 bpm. Examinations were conducted to two patients with breath holding and others with spontaneous breathing. Compared with CAG and echocardiography, similar results were obtained. Mean effective radiation dose was 6.14+/-2.22 mSv. Conclusion: Infantile KD patient’s CALs were able to be evaluated by ADCT with relatively low dose radiation exposure, without breath holding. ADCT have contributed to reduce the need for repeated invasive CAG.

Background: Neonatal cardiomyopathy is a rare disease that ranges from being asymptomatic to abruptly lethal and is not well characterized [1]. We investigated the clinical features of five neonates with cardiomyopathy in our hospital to determine key clinical characteristics. Methods: We retrospectively reviewed the records of five newborns who were diagnosed with cardiomyopathy between January 2000 and December 2018. The primary evaluation included reasons for diagnosis, underlying diseases, therapy, and turning point. Results: Patients with hypertrophic cardiomyopathy (HCM) or left ventricular noncompaction (LVNC) were diagnosed on the basis of cardiac murmur, while the patient with dilated cardiomyopathy (DCM) was diagnosed on the basis of sucking failure. Underlying diseases included Noonan syndrome and LEOPARD syndrome. All patients had received β-blockers, and those with DCM and LVNC were also administered diuretics and angiotensin-converting enzyme inhibitors. The two patients with HCM underwent follow-up as out-patients. One patient with HCM died at 3 years old because of arrhythmia. The patient with DCM died due to heart failure 38 days after birth. The patient with LVNC exhibited severe heart failure after birth, requiring follow-up while considering heart transplantation. Conclusions: Noonan syndrome and LEOPARD syndrome, which is RAS/MAPK-related diseases, should be considered in patients diagnosed HCM. Because heart failure progresses rapidly in patients with neonatal DCM and those with LVNC, planned therapy should include consideration of heart transplantation. Case-1The baby was born by caesarean section at 38 weeks of gestation because of a breech presentation. The infant weighed was 2940 g at birth and her Apgar scores were normal. Her family histories were unremarkable. She was transferred to our hospital because of a cardiac murmur. Echocardiography on admission showed hypertrophy of the interventricular and posterior left ventricular walls (Fig-A). The pressure gradient at the left ventricular outflow tract