Reports on the incidence of persistent left superior vena cava (PLSVC) in the normal population are limited to studies involving pacemaker implantation candidates and cadavers. The incidence in patients with congenital heart diseases (CHDs) is estimated to be higher than that in the normal population; however, the details are unclear. To investigate the incidence of PLSVC in the normal population and in patients with CHDs, subjects were examined prospectively using echocardiography. Normal subjects consisted of 2841 successive neonates without intra-cardiac or congenital anomalies born in Gifu Prefectural General Medical Center. Additionally, 1920 patients with CHDs were evaluated. The incidence of PLSVC in normal neonates was 0.21% (95% confidence interval 0.042-0.38%). A high incidence (more than 7.0 times the incidence in normal subjects) was observed in all CHD patients. The high incidence group included coarctation of the aorta (CoA) (23.7%) and double outlet right ventricle (DORV) patients (24.6%). The second group consisted of CHD patients with ventricular septal defect (VSD), with an incidence ranging from 5.1 to 6.1%. The low incidence group comprised patients with other CHDs, with an incidence between 1.5 and 3.1%. The incidence of PLSVC in trisomy 21 and atrial septal defect patients was significantly higher than that in normal neonates. The incidence of PLSVC in the normal population and in patients with CHDs was systematically evaluated for the first time. The incidence in CHD patients appeared to be positively influenced by the type of CHD, particularly by DORV, CoA, and VSD.
IntroductionThere are several types of congenital heart disease which may be complicated by pulmonary hypertension (PH) following excessive pulmonary blood flow, such as large ventricular septal defect (VSD), double outlet right ventricle, and single ventricle.Primary repair is generally accepted as the best option for such diseases, although pulmonary artery banding (PAB) is occasionally indicated as a palliative procedure for situations in which primary repair has a considerable risk. We report on the case of a patient with coarctation of the aorta (CoA) complicated by multiple muscular VSDs (Swiss cheese type) for whom we performed arch repair and PAB as the first-stage surgery. His VSDs spontaneously became smaller following PAB, consequently, we chose to perform percutaneous pulmonary debanding rather than surgical debanding. We also created a model of the PAB and studied the anatomic development.
A B S T R A C TPulmonary artery banding (PAB) is a standard operation for various congenital heart defects complicated by pulmonary hypertension (PH) and judged unsuitable for primary intracardiac repair. We report successful percutaneous pulmonary artery debanding in a baby complicated by muscular ventricular septal defect (VSD), that was initially large and multiple, but closed spontaneously later. The 5-monthold boy was referred to our hospital on day 3, diagnosed as having aortic coarctation (CoA), with multiple muscular VSDs and severe PH. On day 6, he underwent CoA repair and PAB using expanded polytetrafluoroethylene (ePTFE), while the muscular VSDs were left open. We planned percutaneous pulmonary debanding at the age of 5 months, as the muscular VSDs had become small. After dilation with a Mustang 1 (Boston Scientific, Marlborough, Massachusetts, United State) balloon (12 mm diameter) there was a persistent waist indicating a residual narrowing. Use of an extra-high pressure balloon, Conquest 1 (Medicon, Osaka, Japan) balloon of the same size, completely eliminated the waist. In in vitro experiments, the Mustang 1 partially tore the ePTFE, while a Conquest 1 of the same diameter completely opened the band. The mechanism of debanding was tearing of the ePTFE by the knot of the suture thread. Percutaneous pulmonary debanding to avoid unnecessary surgery is feasible in such a patient if the VSD becomes small.
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