Transcatheter occlusion of gigantic persistent ductus arteriosus (PDA) using a custom-made persistent ductus arteriosus occluder.

We reported transcatheter closure of gigantic persistent ductus arteriosus (PDA) complicated by severe pulmonary hypertension (PH) using a custom-made PDA occluder. A 19-year-old lady weighing 45 kg visited to our Heart Saving Project in Mongolia with a chief complaint of shortness of breath. Contrast CT scan showed ellipsoidal section of PDA whose long axis being 28 mm, and the short axis of 21 mm. A custom-made PDA occluder, whose retention skirt, the aortic side, and the pulmonic side diameter of the body were 54, 36, 34 mm, respectively, was successfully deployed using 14-Fr sheath. Pulmonary pressure decreased around a half compared to before closure. A custom-made duct occluder could be a reasonable and cost-effective choice for transcatheter closure of gigantic PDA complicated by severe PH. © 2015 Wiley Periodicals, Inc.

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.

Transcatheter coil occlusion of perimembranous ventricular septal defects.

OBJECTIVES: Our purpose was to determine the feasibility and safety of transcatheter coil occlusion of a perimembranous VSD. BACKGROUND: Transcatheter coil occlusion of a perimembranous ventricular septal defect (VSD) may be a useful treatment; however, there are few reports on its technical aspects and outcome. METHODS: We attempted coil closure of a perimembranous VSD in 41 patients. We approached the VSD via the right ventricle (RV) in 16, via the left ventricle (LV) in 16, simultaneously deployed two coils from the RV (RV/RV) in three, and simultaneously deployed two, three, or four coils from the RV and LV (RV/LV) in six. Coils (0.038'' flipper, 0.052'' Gianturco, or 0.035'' platinum coil) were placed into the defect through the delivery catheter until any flow through the VSD disappeared or became trivial on angiography. RESULTS: We achieved 100% complete closure in all patients in whom coils were deployed, although some patients required a second session. A flipper coil used alone was insufficient to completely occlude VSDs larger than 2.5 mm. CONCLUSIONS: Transcatheter coil occlusion of a VSD can be performed reliably and safely by selecting appropriate embolic coils. Small defects (<2.5 mm) can be closed with a flipper coil, but defects >or=2.5 mm require a 0.052'' coil.

[Cost-effectiveness analysis of routine mumps immunization in Japan].

OBJECTIVE: Mumps immunization is not included in routine immunization in Japan. We measured the cost-effectiveness of routine immunization. METHODS: We surveyed outpatients prospectively from June 15, 2004, for 19 months in an area with a population of 100,000. Almost all of the 11 pediatric clinics and hospitals in this area cooperated. In 2006, we retrospectively surveyed all inpatients hospitalized for more than 24 hours and dying of mumps. RESULTS: We collected data from 189 doctors who rated outpatients and 112 families. The disease burden for outpatients including family nursing was estimated to be 47.1 billion yen nationwide. We estimated the total number of inpatients as 4,596. The disease burden of inpatients including the cost of family nursing was estimated to be 1.35 billion yen. Adding cases of sequelae and death, the total disease burden was estimated to be 52.5 billion yen. The incremental benefit cost ratio for routine immunization is higher than 1 even in the lower bounds of the 95% confidence interval. DISCUSSION AND CONCLUSIONS: The incremental benefit cost ratio shows that the additional benefit due to routine immunization exceeds additional cost, emphasizing the benefits of routine mumps immunization.

[Cost-effectiveness analysis of routine immunization for varicella in Japan].

OBJECTIVE: Immunization for varicella is not currently included in routine immunizations in Japan. This study was conducted to assess test its cost-effectiveness when it becomes a routine. METHODS: We surveyed and collected information for the year beginning 15th June 2004 in one area. Almost all 11 pediatric clinics or hospitals in the area cooperated in this survey. There was a questionnaire form for families and for doctors. Absent days from routine tasks, i.e., job, housekeeping or study, for family nursing and others were asked on family form. Medical costs and other costs were asked on doctor form. Neither form included the patient's name or name of the medical institution. Doctors explained this survey to patients' family on their first visiting day and asked to cooperate. If they refused the doctor form was also discarded. Since family form was written after recovery and mailed, cooperation in this survey was voluntary. Opportunity cost for family nursing is estimated as opportunity cost based on the 2002 Basic Surveillance of Wage Structure. The burden of sequel cases was estimated assuming six million yen per Quality-adjusted Life Years (QALY). Total number of patients was estimated to be 0.84 million, which includes unvaccinated people in a birth cohort. We adopted incremental benefit cost ratios form societal viewpoint as a measure. RESULTS: We collected 402 data from the doctor form and 265 from the family form. Total disease burden in all of Japan is estimated to be 52.2 billion yen, but opportunity cost accounted for 80%. The incremental benefit cost rations averaged more than 4 when vaccination cost is assumed to be five to twelve thousand yen. The lower limit of the confidence intervals is about 1.5. DISCUSSION AND CONCLUSION: The disease burden of varicella was greater than measles in 2000, when there were measles 0.2 million patients. The incremental benefit cost ratio showed that there will almost surely be an additional benefit due to routine immunization is greater than the additional cost. Therefore, we found strong evidence for routine immunization for varicella.

Coil occlusion of PDA in patients younger than 1 year: risk factors for adverse events.

BACKGROUND: Transcatheter occlusion of infantile patent ductus arteriosus (PDA) challenges the interventionist. PURPOSE: To analyze the risk factors for adverse events from this procedure in patients younger than 12 months. SUBJECTS: We retrospectively analyzed data on 32 patients younger than 12 months in whom transcatheter coil occlusion of a PDA was attempted. Ages ranged from 1 to 11 (median, 7) months and body weight from 1.2 to 10 (6.0) kg. The minimum ductal diameter ranged from 1.0 to 4.6 (3.3) mm and pulmonary to systemic flow ratio from 0.7 to 12.5 (2.2). Major adverse events were defined as those requiring surgery, while minor adverse events included transient hemolysis not needing treatment, coil migration with successful transcatheter retrieval, and mild left pulmonary artery (PA) stenosis. We determined whether any factors such as age, body weight, minimum PDA diameter, angiographic type, operator, and use of a 0.052-in. Gianturco coil related to the occurrence of adverse events. RESULTS: In two patients, coils could not be deployed in the appropriate position. They underwent surgery following transcatheter retrieval of coils. Coils were successfully deployed in the other 30 patients, however, one patient developed persistent hemolysis that required surgical retrieval of the coils and closure. PDA was completely closed in the other 29 patients (clinical success rate, 91%). Thus, there were three major adverse events, while minor adverse events occurred in five patients. Univariate analysis did not identify any single factor that contributed to either major, minor, or total adverse events. However, all major adverse events occurred in patients under 6 months and less than 6 kg body weight with a minimum duct diameter of more than 3.5 mm. CONCLUSION: Transcatheter coil occlusion of PDA is feasible in selected patients younger than 1 year. A minimum diameter more than 3.5 mm in patients under 6 kg may be a risk factor for major adverse events.

Genetic analysis in patients with left ventricular noncompaction and evidence for genetic heterogeneity.

Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by numerous excessively trabeculations and deep intertrabecular recesses. This study was performed to investigate Japanese LVNC patients for disease-causing mutations in a series of selected candidate genes. DNA was isolated from the peripheral blood of 79 cases including 20 familial cases and 59 sporadic cases. DNA samples were screened for mutations in the genes encoding G4.5 (TAZ), alpha-dystrobrevin (DTNA), alpha1-syntrophin (SNTA1), FK506 Binding protein 1A (FKBP1A or FKPB12: FKBP1A), and LIM Domain Binding protein 3 (Cypher/ZASP: LDB3), using single-strand conformational polymorphism analysis and DNA sequencing. DNA variants were identified in 6 of the 79 cases, including four familial cases and two sporadic cases. A splice acceptor mutation of intron 8 in TAZ (IVS8-1G>C) was identified in one family with isolated LVNC, resulting in deletion of exon 9 from mRNA. In a sporadic case of isolated LVNC and Barth syndrome (BTHS), a 158insC in exon 2 of TAZ resulting in a frame-shift mutation was identified. A 1876G>A substitution changing an aspartic acid to asparagine (D626N) was identified in LDB3 in four members of two families with LVNC. A 163G>A polymorphism was identified in LDB3, which changed a valine to isoleucine (V55I) in one patient with isolated LVNC. In addition, in a family with nonisolated LVNC, a 362C>T mutation was identified in DTNA. LVNC, like other forms of inherited cardiomyopathy, is a genetically heterogeneous disease, associated with variable clinical symptoms and can be inherited as an autosomal or X-linked recessive disorder.

A girl with partial trisomy 5q35-->qter and partial trisomy 13pter-->q31 derived via a maternal balanced translocation.

A girl with partial trisomy 5q35qter and partial trisomy 13pterq31 shows a less severe clinical course.