The Evaluation of Fetal Cardiac Remote Screening in the Second Trimester of Pregnancy Using the Spatio-Temporal Image Correlation Method.

We prospectively performed remote fetal cardiac screening using the spatio-temporal image correlation (STIC), and examined the usefulness and problems of remote screening. We performed heart screening for all pregnant women at four obstetrics clinics over the three years from 2009 to 2014. The STIC data from 15,404 examinations in normal pregnancies (16-27 weeks, median 25 weeks) were analyzed. Obstetricians and sonographer collected STIC data from four-chamber view images. Eight pediatric cardiologists analyzed the images offline. A normal heart was diagnosed in 14,002 cases (90.9%), an abnormal heart was diagnosed in 457 cases (3.0%), and poor images were obtained in 945 cases (6.1%). 138 cases had congenital heart disease (CHD) after birth, and severe CHD necessitating hospitalization occurred in 36 cases. We were not able to detect CHD by screening in 12 cases. The sensitivity and specificity of STIC in CHD screening was 50% and 99.5%, respectively. The sensitivity and specificity of STIC in screening for severe CHD was 82% and 99.9%, respectively. The STIC method was useful in fetal remote screening for CHD. However, the fact that > 10% of images that could not be analyzed by this method was a problem.

Prenatal diagnosis of aortopulmonary window with interrupted aortic arch by fetal echocardiography.

We report the first case of prenatally diagnosed aortopulmonary window (APW) with interrupted aortic arch. The mother was referred for fetal echocardiography at 29 weeks' gestation because of suspected congenital heart disease. On the short-axis view, we clearly imaged the aortopulmonary septal defect at the proximal pulmonary artery and type A interrupted aortic arch. The window was 4 mm and the shunt flow detected by color flow Doppler was right-to-left in systole and early diastole. All aortic arch branches originated from the aorta proximal to the interruption. The well-developed ascending aorta was not continuous with the descending aorta in the sagittal view of the aortic arch. Visualization of the direction of the shunt flow across the APW and each origin of subclavian arteries was useful for the diagnosis of APW with interrupted aortic arch.

[Pharmaceutical analysis and clinical efficacy of Kampo medicine, maoto, extract suppository against pediatric febrile symptoms].

A traditional Chinese herbal medicine, Kampo medicine, maoto, has been widely used in the treatment of febrile symptoms caused by viral infection. This herbal extract granule for oral use, however, is not well accepted by infants or young children due to its unpleasant taste and odor. Therefore, we prepared Kampo medicine, maoto, suppository and investigated the pharmaceutical and clinical efficacy of the suppository. Kampo medicine, maoto, granules were micro-pulverized and homogeneously dispersed into Hosco-H15 to prepare suppositories containing 0.25 to 1.0 g herbal extract by the conventional fusion method. Content of l-ephedrine, an index compound of Kampo medicine, maoto, in the extract granules and suppositories was determined by using a high performance liquid chromatographic method. Physicochemical experiments revealed that the suppository containing 0.5 g herbal extract had the most suitable melting point of 34 degrees C. Contents of l-ephedrine in the suppository were constant, 93-96% of those in the same amount of the extract granules in different three lots. Upper and lower portions of the suppository had the same content of l-ephedrine. The suppository maintained more than 95% of l-ephedrine content through 6 months at 4 degrees C, room temperature and 40 degrees C, although maldistribution of the extract constituent was observed after storage at 40 degrees C. The suppository was administered to 21 pediatric febrile patients at a dose of 1/3 to 2 full pieces depending on their body weight and physical status. Significant reduction (p<0.001) of body temperature from 39.5 to 37.5 degrees C without serious adverse effects was observed in 17 patients who were monitored the clinical effects on the febrile symptoms. In conclusion, Kampo medicine, maoto, suppository was found to satisfy the physicochemical quality and quantity standards as well as to be clinically applicable to neonates, infants and children with viral febrile symptoms without any adverse effects.

Clinical and electrophysiological features of Japanese pediatric long QT syndrome patients with KCNQ1 mutations.

BACKGROUND: The purpose of the present paper was to determine the clinical and electrophysiological features of Japanese pediatric long QT syndrome (LQTS) patients with KCNQ1 mutations (LQT1). METHODS: KCNQ1 mutations were analyzed in 13 Japanese pediatric patients with prolonged QT interval on electrocardiogram (ECG). These LQT1 patients were reviewed, retrospectively, for the presence of past and family histories of LQTS-related cardiac events, other complications, and their ECG findings evaluated at rest and during exercise). RESULTS: KCNQ1 mutations were identified in eight patients (61.5%) from six unrelated families. Four missense mutations were identified in seven patients and an insertion/deletion in one. The mutations were located in the transmembrane domain in four patients and the C-terminal domain in four. Syncope did not occur in patients with the C-terminal domain mutations up to the age of 6-9 years, but family members of patients with the C-terminal domain mutations had a history of syncope in their elementary school days. Compared with a non-LQTS control group, peak heart rate (HR) on exercise and the HR increase during exercise were significantly lower in the LQT1 group (LQT1 vs control, 155 +/- 16 beats/min vs 182 +/- 13 beats/min, P < 0.01, 66 +/- 16 beats/min vs 99 +/- 24 beats/min, P < 0.01, respectively). CONCLUSIONS: The risk of LQTS-related cardiac events may not be different in pediatric LQT1 patients with C-terminal domain mutations than in patients with transmembrane domain mutations. Possible sinus node dysfunction or a poor HR response to sympathetic stimulation has been suggested in pediatric LQT1 patients.

Prevalence of arrhythmias and their risk factors mid- and long-term after the arterial switch operation.

Early results of the arterial switch operation (ASO) for transposition of the great arteries (TGA) are good, but there are few mid- and long-term data on postoperative arrhythmias, especially in Japan. In this study, clinical data on 624 1-year survivors who had an ASO between 1976 and 1995 were collected from six institutes in Japan up to October 2002. Sixty (9.6%) 1-year survivors had significant arrhythmias. Bradycardia occurred in 22 patients, including complete atrioventricular block (CAVB) in 12, sick sinus syndrome (SSS) in 6, and second-degree atrioventricular block in 4. Syncope developed in 2 with CAVB and 2 with SSS. Ten patients with bradycardia underwent permanent pacemaker implantation. Supraveutricular tachycardia (SVT) was seen in 25 patients, including paroxysmal supraventricular tachycardia in 16, atrial flutter in 7, and atrial fibrillation in 2. Six patients with SVT received antiarrhythmic medication. SVT was transient in 20 and persistent in 5. Ventricular arrhythmias occurred in 13 patients, including nonsustained ventricular tachycardia in 5, paroxysmal ventricular contractions with couplets in 5, ventricular flutter in 2, and sustained ventricular tachycardia in 1. Four patients with ventricular arrhythmias received antiarrhythmic medication. Of the study patients, 8 died 1 year or more after ASO. Death was directly related to arrhythmia in 1 patient and was due to nonsustained ventricular tachycardia with severe congestive heart failure. The presence of a ventricular septal defect (VSD) was a risk factor for postoperative arrhythmia. Patients with TGA and VSD had more arrhythmias than those with TGA and an intact ventricular septum (13.7 vs 8.7%, p < 0.05), and this was especially true for CAVB (3.9% vs 1.0%, p < 0.05). In 36 patients clearly documented time onset of postoperative arrhythmia arrhythmia developed in 18 (50%) after less than 1 year and in 15 (42%) after more than 5 years. In summary serious arrhythmias after ASO were uncommon, but postoperative arrhythmias, such as unpaced CAVB, SSS, and VT, were related to morbidity and mortality. VSD was a risk factor for postoperative arrhythmia, especially CAVB. Approximately half of the arrhythmias developed late. Lifelong monitoring with respect to arrhythmia is needed for patients after ASO.

Dilated cardiomyopathy after pacemaker implantation in complete heart block.

BACKGROUND: The aim of this study was to evaluate the clinical features of patients with congenital complete heart block (CCHB) who developed dilated cardiomyopathy (DCM) after pacemaker implantation (PMI) and to determine factors predicting DCM development. METHOD: A total of 15 patients were reviewed retrospectively. They were classified into two groups, one consisted of four patients who were diagnosed as having CCHB in utero or at birth and who developed DCM after PMI (DCM group) and the other consisted of 11 patients who did not (non-DCM group). RESULTS: Maternal autoantibodies were found in two of the DCM group and in five of the non-DCM group. Perfusion defects in myocardial imaging were detected in all DCM patients and in five non-DCM patients. DCM developed 2 to 43 months after PMI and three DCM patients died of heart failure 7 to 48 months after PMI. In pathological studies, endocardial or interstitial fibrosis was present in all DCM patients and in one of two in the non-DCM group. No significant differences between the two groups were found in age at PMI, atrial or ventricular rate, end-diastolic dimension and ejection fraction of the left ventricle before PMI, and width of QRS after PMI. CONCLUSION: Although it was suspected that the patients with CCHB had myocardial involvement before PMI, there was no significant factor predicting the risk of DCM after PMI. In addition to cardiac rhythm abnormalities, careful attention should be paid to cardiac function in CCHB patients after PMI.

"Bail-out" stenting for acute obstruction of a modified Blalock-Taussig shunt following selective angiography.

A 3-year-old boy developed acute obstruction of a left modified Blalock-Taussig shunt following selective power injection of contrast in the shunt. Balloon dilation was ineffective due to rapid recoil of the narrowed segment, but implantation of a stent effectively abolished the obstruction. The obstruction itself may have been produced by an intimal flap caused by the power injection.

Balloon angioplasty of postoperative coarctation in the transverse arch in infants: protecting the common carotid artery.

We performed balloon angioplasty in three patients with postoperative coarctation in the transverse arch adjacent to the left common carotid artery. The age at arch reconstruction was 5, 6, and 2 days, while the interval between operation and balloon dilatation was 59 days, 87 days, and 12 months, respectively. Two balloons, one in the stenosis and the other in the left common carotid artery, were introduced over a wire sequentially and inflated simultaneously until the waist of the balloon in the arch disappeared. After balloon dilatation, a significant reduction in the peak-to-peak pressure gradient and an increase in vessel diameters were observed in all patients. Further growth of the transverse arch was documented at follow-up in two patients. No aneurysm has been detected in any patients. We believe that placing a protective balloon in the neck vessel increases safety during balloon dilatation of coarctation in the transverse arch.