Yoichiro Ishii

External validation of a risk score to predict intravenous immunoglobulin resistance in patients with kawasaki disease.

Background: We previously developed a new risk score to predict intravenous immunoglobulin (IVIG) resistance in Kawasaki disease. However, the IVIG dosage used in that study (1 g/kg/d for 2 consecutive days) differs from the single infusion of 2 g/kg recommended in the United States and elsewhere. Our aim was to assess the validity and applicability of our risk score in patients treated with a single infusion. Methods: We used a database of 1626 patients with Kawasaki disease given initial IVIG treatment at a dose of 1 g/kg/d for 2 consecutive days (n = 990; IVIG- 1 g/kg × 2) or 2 g/kg/d for 1 day (n = 636; IVIG- 2 g/kg × 1) across 17 hospitals in Japan. Patients received the total IVIG dose within 36 hours in IVIG- 1 g/kg × 2 and 24 hours in IVIG- 2 g/kg × 1. We stratified the patients according to a risk scoring system developed to predict IVIG unresponsiveness, based on scores of ≥5 points. We compared the accuracy of prediction between the 2 groups using receiver operating characteristic analysis. Results: Baseline characteristics and clinical outcomes were similar between both groups. The areas under the receiver operating characteristic curve in IVIG- 2 g/kg × 1 were similar to those of IVIG- 1 g/kg × 2. Using a cut-off risk score of ≥5 points, we could identify IVIG resistance in terms of coronary artery abnormalities within 1 month and coronary artery abnormalities at 1 month with equivalent sensitivity and specificity in both groups. Conclusion: Our risk score can be used to predict IVIG unresponsiveness to a regimen based on a single infusion of 2 g/kg IVIG.

Congenital aneurysm of the right atrial appendage in a fetus.

A 43-year-old pregnant woman was referred to our maternity department at the 38th week of gestation because of fetal dilation of the atrium. She was a primipara at an advanced age and had no other prenatal data of interest. Initially, fetal echocardiography showed a large intrapericardial structure, anterior and to the right of the right atrium (RA), communicating with the RA. The right appendage, measuring 34 9 17 mm, was very dilated and positioned together with the RA as if it were a third atrium (Fig. 1). There was no tricuspid regurgitation or other structural abnormalities. A normal vaginal delivery was performed in the 41st week. The neonate was a female and weighed 3,038 g. The neonate had no significant findings at cardiorespiratory examination after birth. A chest X-ray showed mild cardiomegaly. An electrocardiogram showed slight RA loading and no evidence of arrhythmia. Echocardiography in the subcostal view showed dilation of the RA similar to that of the prenatal period. The lesion was confirmed by computed tomography in the axial view, which depicted a giant aneurysm of the right appendage measuring 28 9 17 mm and causing compression of the right ventricular inlet (Fig. 2a). We ensured that there was no thrombus formation in the aneurysm (Fig. 2b, c). We did not administer medication, including antiplatelet therapy. Careful observation is required for thromboembolic complications until the patent foramen ovale is closed.

Single Coronary Artery with Right Ventricular Fistula: Case Report and Literature Review

We report a rare case of a 6-year-old boy who was diagnosed with coronary artery fistulae communicating with the right ventricle and a left single coronary artery. Preoperative angiography showed a dilated and tortuous single coronary artery draining into the right ventricle. Two coronary artery fistulae draining into the right ventricle were detected at operation and both of these were ligated. Postoperative angiography showed that the single coronary artery diameter was almost normalized, although the vessel was still slightly tortuous. His clinical course was uneventful. In this report, we summarize cases of coronary artery fistula with single coronary artery that have been reported in the literature as well as our case.

Abnormal cardiac axis as a prenatal marker of left pulmonary artery sling

Left pulmonary artery sling (LPAS) is a rare vascular anomaly. The left pulmonary artery arises distally from the right pulmonary artery on the right side of the trachea and passes between the trachea and esophagus towards the left lung, compressing the lower trachea. LPAS is associated with congenital tracheal stenosis, which frequently requires early surgical intervention and has a poor prognosis due to severe airway obstruction after birth. Therefore, LPAS should be prenatally diagnosed to prepare for surgical intervention for tracheal stenosis. To the best of our knowledge, there are few reports on prenatal echocardiographic findings in LPAS. We report three prenatal cases of LPAS, which resulted in respiratory symptoms. We discuss fetal ultrasound findings and highlight the abnormal rotation of the fetal cardiac axis to the right as a useful sign in the prenatal screening of LPAS.

Usefulness of Percutaneous Transluminal Pulmonary Artery Balloon Angioplasty after Bilateral PulmonaryArtery Banding: Prevention against Additional Surgical Interventions

Objectives: We evaluated the efficacy of balloon dilatation after bilateral pulmonary artery banding for the treatment of severe congenital heart disease that depends on patent ductus arteriosus for systemic circulation. Background: Neonatal cardiopulmonary bypass can cause brain injury, which may result in a severely impaired neurodevelopmental outcome. Hence, we perform bilateral pulmonary artery banding combined with postoperative percutaneous trans catheter angioplasty with balloon dilatation as first-stage palliation. Methods: From October 2007 to December 2013, 27 consecutive patients underwent bilateral pulmonary artery banding at our institution. We retrospectively obtained their diagnostic, clinical, and catheter examination data from the medical records. Results: Bilateral pulmonary artery banding was performed at a median of 7 days. Among the 27 patients, 16 underwent expansive angioplasty using a balloon catheter at each banding site to increase pulmonary blood flow. The mean age at dilatation was 53.4 ± 25.9 days, and the mean pulmonary artery index significantly increased from 130.6 mm2/m2 ± 51.1 mm2/m2 before dilatation to 243.6 mm2/ m2 ± 93.5 mm2/m2 after dilatation (p<0.01). All patients underwent a secondstage operation at a mean age of 126 ± 14.8 days. Of the 11 patients who did not undergo balloon dilatation, five required additional surgical interventions. However, of the 16 patients who underwent balloon dilatation, only one required an additional surgical intervention (p<0.01). Conclusions: Our strategy of balloon dilatation after bilateral pulmonary artery banding can improve the outcome of clinical treatment for severe congenital heart disease in infants. However, further studies are warranted.

Isolated unilateral agenesis of the pulmonary artery: surgical repair with an artificial graft.

This report describes a 3-month-old Japanese boy with a diagnosis of isolated unilateral agenesis of the proximal right pulmonary artery with severe pulmonary hypertension. One-stage reconstruction of the right pulmonary artery was performed without cardiopulmonary bypass. The hilar right pulmonary artery and the distal main pulmonary artery were joined by anastomosis to an artificial ring graft. The boy’s postoperative course was uneventful, and the pressure in the pulmonary artery was within the normal range. Although mild right pulmonary artery stenosis remained, the authors’ therapeutic strategy may provide a clinically important option for isolated unilateral agenesis of the pulmonary artery.