Successful Management in an Infant Patient of PHACE Syndrome with a Complicated Aortic Arch Anomaly.

PHACE syndrome is a congenital disorder often associated with a cervicofacial infantile hemangioma and complicated cardiovascular malformations. Patients with PHACE syndrome often have complex aortic arch anomalies, longer aortic stenosis or agenesis segments, and increased vascular tortuosity; therefore, perioperative management and surgical repair are challenging. We report a case of a female infant with PHACE syndrome and complex cardiovascular anomalies such as a double aortic arch associated with interruption of the left aortic arch, coarctation of the right aortic arch, patent ductus arteriosus, ventricular septal defect, and atrial septal defect. She was born at 36 weeks of gestation (birth weight, 2,150 g) and the diagnosis was confirmed by three-dimensional computed tomography. Because her patent ductus arteriosus did not close at first, her heart failure was managed preoperatively without prostaglandin E 1. We initially attempted to promote weight gain. Surgical planning and simulation were performed using the patient-specific three-dimensional cardiovascular model created from computed tomography data. She underwent a successful aortic arch reconstruction by an end-to-side anastomosis with anterior patch augmentation at the age of 56 days. Detailed planning and simulation before surgery were vital in achieving favorable outcomes. Careful management and surgical planning using a patient-specific three-dimensional model are vital, especially in patients with complex malformations, such as in our case.

Percutaneous Atrial Septal Defect Closure in Adult Ebstein's Anomaly with Exertional Hypoxemia.

In Ebstein's anomaly, percutaneous atrial septal defect (ASD) closure for the treatment of hypoxemia due to a right-to-left interatrial shunt remains controversial. We report the case of a 40-year-old woman with Ebstein's anomaly who developed cyanosis and shortness of breath on exercise. Her symptoms improved after percutaneous ASD closure and her clinical course has been good during follow-up. The balloon ASD occlusion test, combined with dobutamine stimulation before the procedure, is useful to confirm treatment indication. A prior electrophysiological evaluation is also important because Ebstein's anomaly is often complicated by atrioventricular recurrent tachycardia.

Improvement of blood lipid profiles by Goishi tea polyphenols in a randomised, double-blind, placebo-controlled clinical study.

Dyslipidaemia is a risk factor for arteriosclerosis. Recent studies have shown that dyslipidaemia is effectively prevented by various polyphenols. In this clinical study (UMIN trial: 000024028), we evaluated the beneficial effects of polyphenols contained in Goishi tea on blood lipid profiles. Seventy-seven subjects with LDL cholesterol (CHO) ≧120 mg/mL were randomly divided into two groups for 12 weeks of polyphenol intake as follows: the Goishi tea group for daily consumption of Goishi tea containing 122 mg of polyphenols and the placebo group for the corresponding consumption of a placebo drink containing 12.2 mg of polyphenols. Intake of Goishi tea polyphenols tended to increase HDL CHO and suppress the elevation of triglycerides. These effects were particularly notable among the subjects with a body mass index <25 kg/m2. These findings suggest that Goishi tea polyphenols may suppress arteriosclerosis and reduce cardiovascular event risk by improving blood lipid profiles and thereby preventing dyslipidaemia.

[Tetralogy of Fallow with Total Anomalous Pulmonary Vein Return and Atrial Septal Defect;Successful Two-staged Surgical Management;Report of a Case].

A combination of tetralogy of Fallot( TOF) and total anomalous pulmonary venous return(TAPVR) is rare and results in chronic volume and pressure load of the right side of the heart and underfilling of the left heart. We report a successful 2-staged surgical correction of TOF associated with TAPVR and atrial septal defect. The patient was unsuitable for total primary intracardiac correction because the volume of the left ventricle was considered to be small. First, repair of anomalous pulmonary venous return and palliative right ventricle outflow tract reconstruction were simultaneously performed in 2 months of birth. One year after 1st operation, cardiac catheterization revealed that normalization of left ventricle volume, so 2nd operation was planned. Total correction of ventricular septal defect and right ventricle outflow reconstruction was performed and the patient was discharged on the 21st postoperative day with good hemodynamic status.

Perineurioma in the sigmoid colon diagnosed and treated by endoscopic resection.

Perineuriomas are rare benign peripheral nerve sheath tumors in the gastrointestinal tract. We recently encountered a submucosal lesion in the sigmoid colon that was resected by endoscopic mucosal resection and was then diagnosed as perineurioma by immunohistochemistry. A 51-year-old female with a positive test for fecal occult blood was referred to our hospital for screening colonoscopy. Colonoscopy identified a submucosal lesion, approximately 15 mm in diameter, in the sigmoid colon. Endoscopic ultrasound showed a 15-mm tumor with a strong acoustic shadow. Endoscopic mucosal resection was performed in order to make a precise diagnosis as well as removal. The specimen revealed spindle cell proliferation without atypia, and immunostaining revealed that the spindle cells were positive for collagen type IV and glut-1, and the lesion was diagnosed as a colonic perineurioma with no malignancy. Gastroenterologists as well as pathologists should be aware of this type of submucosal lesion, and immunohistochemical evaluation is highly recommended when an unusual mesenchymal tumor is found.

Successful introduction of interventional catheterisation and other paediatric cardiology services in a developing country.

BACKGROUND: Providing care for children with cardiac disease remains a challenge in developing countries. MATERIALS AND METHODS: Since 2001, a team of paediatric cardiologists has been visiting Mongolia to perform children's cardiac catheterisation, as well as screening children for cardiac disease. Considering the limited medical resources, as well as cost, risk, and benefit, we focused our activities on diagnostic consultation by echocardiography, transcatheter closure of persistent arterial duct, balloon dilation of valvar pulmonary stenosis and aortic coarctation, and diagnostic catheterisation. RESULTS: Up to 2011, we have completed echocardiography in 1200 patients; diagnostic catheterisations in 59 patients; and catheter interventions in 255 patients, including 224 for persistent arterial duct, 23 for valvar pulmonary stenosis, 6 for aortic coarctation, and a few others. We have visited 14 rural areas to screen for children's cardiac diseases. A total of 131 persistent ducts were closed with coils and a further 93 with a duct occluder. Migration of the coil or occluder to the pulmonary artery occurred once for each device. Pulmonary valvuloplasty was successful in 23 patients and a coarctation was effectively dilated in six patients. CONCLUSIONS: The limited healthcare resources in developing countries such as Mongolia make catheter interventional procedures an attractive alternative to surgery in treating children with simple but critical congenital heart diseases. Introduction of the duct occluder extended the application of transcatheter occlusion of persistent arterial duct in Mongolia to larger vessels and avoided the expense of coil occlusion using multiple coils.

Usefulness of balloon angioplasty for the right ventricle-pulmonary artery shunt with the modified Norwood procedure.

OBJECTIVE: We sought to evaluate the efficacy of balloon angioplasty (BA) for severely desaturated patients due to a stenotic right ventricle (RV) to pulmonary artery (PA) shunt following modified Norwood procedure. METHODS: Of 87 patients who underwent a Norwood procedure with the RV-PA shunt between February 1998 through March 2010, 22 (25%) patients underwent BA. The efficacy of BA was assessed by angiographic measurement of the changes in the internal diameters of the stenotic portions of the shunt, changes in arterial saturation and clinical outcomes. RESULTS: BA was performed for stenotic RV-PA shunts following stage I palliation (n = 17, 77%), or those placed as an additional blood source (n = 5, 23%, 3 patients awaiting biventricular repair, 2 patients following stage II palliation). The location of the BA was at the distal anastomosis in 12 (54.5%), proximal anastomosis in 21 (95.4%) and in the mid-portion of the shunt in 11 (50%) cases. The diameters of these three shunt portions were measured from the anterior-posterior and lateral angiographic images, increasing significantly after BA (p < 0.0001) in all. Arterial saturation significantly improved after BA in all cases (66.5 ± 4.3% to 79.4 ± 3.4%, p < 0.0001). Freedom from reintervention was 100%. All patients underwent subsequent elective planned surgery at an appropriate age with no mortality. CONCLUSIONS: A BA-alone strategy for a stenotic RV-PA shunt was effective for all three shunt portions, minimizing shunt-related premature surgical intervention.

Rudimentary right ventricle to pulmonary artery shunt in the Norwood procedure.

We describe here successful palliative repair of tricuspid atresia, hypoplastic right ventricle, transposition of the great arteries, and hypoplastic aortic arch in a neonate. The repair consisted of the Norwood procedure with a rudimentary right ventricle to pulmonary artery shunt, which was located on the right side of a neo-aorta. This procedure could be a useful adjunct to avoid left ventriculotomy and its subsequent dysfunction.

[A case of toxic epidermal necrolysis with severe intestinal manifestation].

TEN is a severe dermatological disorder characterized by extended epidermal necrosis. Disseminated mucosal erosions have been occasionally reported to occur in the gastrointestinal tract. We report a case of toxic epidermal necrolysis (TEN) with severe intestinal manifestation. A 52-year-old woman was admitted with high fever, skin eruption and severe diarrhea. She was diagnosed as toxic epidermal necrolysis (TEN), which was most likely due to nonsteroidal anti-inflammatory drugs (NSAIDs). After skin lesion recovered, fever and bloody diarrhea went on. Colonoscopy and X-ray revealed lead-pipe like stenotic long loops which was caused by erosion and sublation of whole mucosa of large intestinum, and severe stenosis of ileum end. The ileo-cecal region was resected on the 216(th) hospital day. Pathological examination showed sublation of mucoepithelium and inflammatory change in the muco-submucosal layer, but muscular layers of mucosa remained intact. Pathologically, the intestinal lesion resemble the changes in the early skin lesions and seems to be part of the systemic lesion of TEN.