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.

A Patient-Specific Hollow Three-Dimensional Model for Simulating Percutaneous Occlusion of Patent Ductus Arteriosus.

Percutaneous catheter closure of patent ductus arteriosus (PDA) is difficult when the ductus is large and long or shows calcification. We created a patient-specific 3-dimensional (3D) model for PDA, with which we simulated device deployment, thereby selecting the device/size in a patient-by-patient manner. We assessed whether this 3D model is effective for catheter PDA closure.The 3D model was created in this institute, requiring 3 days and 90 US dollars. After its introduction, 7 consecutive patients (the study group) with severe PDA underwent closure with the aid of the 3D model. The control group consisted of 4 patients before 3D-model introduction, with all having severe PDA: the requirement of computed tomography was considered a criterion of severe or difficult-procedure-requiring PDA.In all study group patients, the devices/sizes could be pre-selected based on the simulation, whereas devices were changed during the procedure in 2 of 4 in the control group. In the study group, compared with the control group, the fluoroscopic (median 31 [interquartile range of 16-42] versus 39 [19-71] minutes, respectively) and total procedural times (median 107 [interquartile range 67-114] versus 124 [78-184] minutes, respectively) were shorter. A questionnaire confirmed the doctors' understanding of the procedure.This 3D model may be effective for percutaneous catheter closure of PDA. This may be especially true in cases of severe or difficult-procedure-requiring PDA.

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.

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.

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.

A novel anti-peroxiredoxin autoantibody in patients with Kawasaki disease.

Antibodies to the anti-oxidative peroxiredoxin (Prx) enzymes occur in both systemic autoimmune disease and vasculitis in adulthood. Because increased oxidative stress induces vasculitis in Kawasaki disease (KD), autoimmunity to Prxs in patients with KD was investigated. The presence of antibodies to Prx 1, 2 and 4 was analyzed by ELISA and Western blot. Of 30 patients with KD, 13 (43.3%) possessed antibodies to Prx 2, whereas these antibodies were present in only 1 of 10 patients (10.0%) with sepsis (4 with purulent meningitis and 6 with septicemia). In contrast, antibodies to Prx 1 and 4 were not detected in either group. There was no significant correlation among the titers of the three antibodies. Clinical parameters were compared between anti-Prx 2-positive and -negative patients. The presence of anti-Prx 2 antibodies correlated with a longer period of fever and poor response to high-dose γ-globulin therapy in patients with KD. Anti-Prx 2-positive patients had significantly greater excretion of urinary 8-isoprostaglandin than did anti-Prx 2-negative patients. These results provide the first evidence for an antibody to Prx 2 in patients with KD. They also suggest that this antibody might serve as a marker of disease severity and be involved in the pathophysiology of vasculitis in some patients with KD.

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.

Long-term survival without surgical intervention in a patient with a natural history of a single right ventricle: A case report.

Single ventricle (SV), a complex cardiac anomaly, if left untreated, is thought to lead to a poor prognosis. Herein, we report the case of a long-term survivor with an unrepaired SV with right ventricle (RV) morphology. A 55-year-old man presented with a SV with RV morphology, with a double outlet, large atrial septal defect, common atrioventricular valve, pulmonary valve stenosis (PS), and dextrocardia. Because the native PS provided adequate restriction of the pulmonary blood flow, he did not develop pulmonary hypertension; however, he had severe cyanosis. In patients with SV and moderate PS, even if the SV has RV morphology, long-term survival may be possible without surgical intervention. .

[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.

Absent Ductus Venosus Associated with Partial Liver Defect.

Absent ductus venosus (ADV) is a rare vascular anomaly. We describe a fetus/neonate with ADV with a partial liver defect. A 41-year-old woman was referred to our institute because of fetal cardiomegaly detected by routine prenatal ultrasound, which revealed absence of ductus venosus with an umbilical vein directly draining into the right atrium, consistent with extrahepatic drainage type of ADV. She vaginally gave birth to a 3,096-gram male infant at 38 weeks of gestation. Detailed ultrasound examination revealed a defect of the hepatic rectangular leaf at half a month postnatally. He showed normal development at 1.5 years of age with the liver abnormality and a Morgagni hernia. Liver morphological abnormality should also be considered as a complication of ADV.

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.

Radial incision for ventricular septal defect with membranous septal aneurysm.

Surgical exposure and accurate closure of a ventricular septal defect with a membranous septal aneurysm beneath the septal tricuspid leaflet carries a risk of tricuspid valve dehiscence and conduction disturbances when the septal leaflet is detached along the tricuspid annulus. To avoid these problems, we use a radial incision to expose and close perimembranous ventricular septal defects. We reviewed recent cases to determine the risks and benefits of this technique. From January 2005 through September 2008, 30 patients underwent closure of a perimembranous ventricular septal defect through a right atrial approach at our institution. The operation included radial incision of the membranous septal aneurysm to improve visualization of the perimembranous ventricular septal defect in 9 patients. There was no perioperative or late death. The operative and postoperative courses were uneventful in all cases. A residual leak was detected in only one patient. No patient had more than mild postoperative tricuspid valve insufficiency, none underwent reoperation, and no new arrhythmia or conduction disturbance was detected during follow-up. The radial incision for closure of a ventricular septal defect with a membranous septal aneurysm provides satisfactory exposure of the defect through the right atriotomy, for safe and accurate closure.

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.

Transcatheter repair of native coarctation in children with Turner syndrome: three case reports and literature review.

Transcatheter management of native coarctation of the aorta in 3 children with Turner syndrome is reported. Two children, 12 and 17 years of age, underwent stent implantation and 1 child, 3 years old, underwent balloon angioplasty alone. In all children the procedures were successful, both hemodynamically and angiographically. Three- to 4-year follow-up after the procedures has revealed no signs of complications, including restenosis or aneurysm formation.