1/14. Complete surgical repair of aortic atresia with a normal left ventricle.Aortic atresia with a normal left ventricle and normal mitral valve is an uncommon congenital lesion. We present two such cases and describe two different approaches of achieving biventricular surgical repair.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/14. pulmonary atresia with intact ventricular septum, right-sided aortic arch and ventriculocoronary connection--prenatal echocardiographic diagnosis.The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
3/14. Mitral atresia with premature closure of foramen ovale. A rare hemodynamic cause for failure of Blalock-Taussig anastomosis to relieve inadequate pulmonary blood flow.The clinical, hemodynamic, and surgical findings encountered in the management of a hypoxic male infant with a rare and complex variety of cyanotic congenital heart disease associated with inadequate pulmonary blood flow are described. A poor clinical response to creation of a Blalock-Taussig anastomosis led to the discovery of mitral atresia complicated by premature closure of the foramen ovale and partially relieved by the presence of a levoatriocardinal vein. The subsequent creation of an atrial septal defect enhanced the function of the subclavian artery to pulmonary artery anastomosis and provided palliative relief of hypoxia. Some of the clinical and laboratory findings indicating the presence of additional lesions complicating the picture of a tetralogy of fallot and requiring additional surgical considerations are discussed. The experience indicates that hemodynamic as well as surgical causes may explain the failure of a systemic artery to pulmonary artery anastomosis to function adequately and should be sought.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
4/14. Invasive intrauterine treatment of pulmonary atresia/intact ventricular septum with heart failure.The mortality and morbidity of children with pulmonary atresia/intact ventricular septum (PA/IVS) are linked to the degree of right ventricular (RV) hypoplasia. Opening up the pulmonary valve (PV) in fetal life could result in improved growth of the RV making it amenable to biventricular repair postnatally. Successful valvulotomy of the PV was performed in a fetus with heart failure at 28 weeks. Following the procedure there was significant growth of the tricuspid valve and RV. The neonate was delivered at 38 weeks with a RV suitable for biventricular repair. In utero pulmonary valvulotomy is feasible and may change the natural history of the condition in affected fetuses with PA/IVS.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
5/14. Complete atrioventricular canal and tetralogy of fallot with pulmonary atresia.Our patient was diagnosed with complete atrioventricular canal and tetralogy of fallot with pulmonary atresia at the age of 1 month. Then he underwent right and left Blalock-Taussig shunts at the ages of 2 months and 5 years, respectively. His cyanosis had increased at 20 years of age. cardiac catheterization showed occlusion of the left Blalock-Taussig shunt and absence of the left pulmonary artery. lung perfusion scintigram showed late phase perfusion in the left lung. Chest computed tomographic scan demonstrated the left pulmonary artery. We describe the operative technique of total correction.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
6/14. Aortopulmonary window with ventricular septal defect and pulmonary atresia: prenatal diagnosis and successful early surgical correction.We report a case in which aortopulmonary window (APW) in combination with pulmonary atresia was diagnosed correctly in a neonate by echocardiography. Prenatal echocardiography showed progression of tetralogy of fallot to pulmonary atresia with retrograde pulmonary perfusion, concealing the concomitant APW in fetal life. Due to intractable heart failure, primary correction was successfully performed at the age of 4 weeks (weight 2280 g).- - - - - - - - - - ranking = 1.2keywords = atresia (Clic here for more details about this article) |
7/14. Pulmonary agenesis as part of the VACTERL sequence.Pulmonary agenesis is an uncommon anomaly that has been reported in isolation and in association with other congenital defects. Such defects include oesophageal atresia, cardiac malformation, horseshoe kidney, and anal atresia. Over a period of three years we have seen five neonates or fetuses with unilateral agenesis of the lung. All the cases had three or more anomalies seen in the VACTERL sequence in addition to the pulmonary atresia. None had a tracheo-oesophageal fistula. None were the products of consanguineous marriages. There had been no recurrence of this range of defects in any of the families at the time of writing. We suggest that pulmonary agenesis may occur as an alternative to tracheo-oesophageal fistula in the VACTERL sequence.- - - - - - - - - - ranking = 0.6keywords = atresia (Clic here for more details about this article) |
8/14. A problem in Fontan's operation: sinus septal defect complicating tricuspid atresia.In a case with tricuspid atresia, a coronary sinus septal defect was found and closed following Fontan's operation. This defect should be found, at latest, during surgery, otherwise Fontan's operation cannot produce functional repair.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
9/14. Protein-losing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve).Protein-losing enteropathy occurred in a 7-year-old girl with tricuspid atresia, concordant ventriculo-arterial connexions and a relatively large hypoplastic right ventricle, one year after an atrio-ventricular type of Fontan operation by means of a valveless woven Dacron conduit. Severe conduit regurgitation and a marked enlargement of the hypoplastic right ventricle were demonstrated at recatheterization. Insertion of a bioprosthetic valve at the base of the right atrial appendage led to a dramatic clinical recovery of the patient. The use of a valved conduit is recommended when an atrioventricular type of Fontan repair is planned in patients with tricuspid atresia, concordant ventriculo-arterial connexions and relatively large hypoplastic right ventricle.- - - - - - - - - - ranking = 1.2keywords = atresia (Clic here for more details about this article) |
10/14. Conduit repair for complex congenital heart disease with pulmonary atresia or right ventricular outflow tract obstruction. part I: surgical results.From 1972 to 1981, 40 valved extracardiac conduits have been implanted to bridge the right ventricular outflow tract (RVOT) in 37 patients for different complex congenital cardiac anomalies. The patients ranged in age from 2 to 23 years (mean 8.5 years). Two Dacron prostheses with incorporated Lillehei-Kaster valves, 1 valveless Dacron conduit, 27 Hancock conduits, and 9 lonescu-Shiley conduits were chosen according to availability and the progress in conduit technology. Thirty-three patients had had up to 4 previous palliative procedure before conduit repair. Although the complication rate appears considerable, the mortality rate seemed to be unaffected by the high percentage of prior surgery. Exchange of the conduit became necessary in 2 children, because of conduit stenosis with calcification of the Hancock valve 5 and 7 years respectively after implantation, and in another patient an outgrown Lillehei-Kaster valve Dacron conduit had to be replaced 9 years after repair. Five children died in the early postoperative course: one in pulmonary failure and kinking of the conduit; one with right heart failure and pulmonary hypertension stage IV; 2 in myocardial failure, and one due to severe bleeding from myocardial failure, and one due to severe bleeding from the prosthesis. Three patients died in the late postoperative course 11 weeks, 4 years and 4 years respectively after conduit repair. Conduit surgery offers new possibilities for repair of complex cyanotic cardiac malformations. At present, however, none of the commercially available conduits is an ideal substitute. Our limited experience with the hemodynamic results of lonescu Shiley conduits is promising although longer observation periods are needed for a definitive judgement. At present, the largest possible conduit should be used whenever possible; problems of compression or kinking can be avoided with proper positioning of the conduit.- - - - - - - - - - ranking = 0.8keywords = atresia (Clic here for more details about this article) |
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