1/44. Goldenhar's syndrome associated with cardiac malformations.A case of Goldenhar's syndrome associated with cardiac malformations such as single ventricle, atresia of pulmonary artery, and patent ductus arteriosus is described. The association of cardiac malformations with Goldenhar's syndrome is very rare and suggests that it is necessary to perform a careful clinical evaluation in this syndrome whether or not additional malformations may exist in visceral organs.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/44. Necrotizing tracheobronchitis in patent ductus arteriosus-dependent cyanotic congenital heart disease.We report 2 patients with necrotizing tracheobronchitis (NTB) associated with patent ductus arteriosus-dependent cyanotic congenital heart disease. The pathologic findings suggest that hypotension and decreased tracheo-bronchial perfusion were the major contributing factors in the development of NTB. Necrotizing tracheobronchitis developed in infants with pulmonary atresia and Ebstein's anomaly with pulmonary stenosis. Both infants required prostaglandin E1 infusion from early infancy, and presented with sudden onset of dyspnea and hypercapnea. In one infant, NTB developed prior to mechanical ventilation. In the other infant, NTB developed after 4 days of mechanical ventilation. Care of both infants involved minimal pressures and FiO(2), adequate humidification, and optimal temperature of inspired gases; these factors probably did not play a role in the development or worsening of NTB. Both infants had hypotension and hypoxemia. These factors could have contributed to the development of NTB because of decreased perfusion pressure and tissue hypoxia. As the area of necrosis and its severity correlated with the area of blood supply served by the specific feeding arteries, we speculate that tissue hypoperfusion was the major cause of NTB.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
3/44. Unilateral CHARGE association.A case with a predominantly unilateral CHARGE association is reported. The CHARGE association refers to a combination of congenital malformations. This boy had left-sided anomalies consisting of choanal atresia, coloboma and peripheral facial palsy. The infant had a frontal encephalocele, an anomaly not included in the definitions of CHARGE association. CONCLUSION: even when anomalies are predominantly unilateral, the CHARGE association should be considered in the differential diagnosis.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
4/44. A new use for the amplatzer duct occluder device.We report a case in which the Amplatzer device for percutaneous occlusion of ductus arteriosus was successfully used for occluding a large systemic-pulmonary collateral vessel in a patient who had previously undergone surgery for correction of pulmonary atresia and ventricular septal defect (Rastelli technique), and was awaiting the change of a cardiac tube. In the first attempt, the device embolized to the distal pulmonary bed and, after being rescued with a Bitome, it was appropriately repositioned with no complications and with total occlusion of the vessel.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
5/44. twins with oesophageal atresia and the CHARGE association.twins are rarely concordant for oesophageal atresia and tracheo-oesophageal fistula. Such a case is presented and their similar associated anomalies described. The CHARGE association is considered as a possible diagnosis and the aetiological implications considered.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
6/44. Lobar emphysema due to ductus arteriosus compressing right upper bronchus in an infant with congenital heart disease.A 1-month-old boy with tetralogy of fallot, pulmonary atresia, right aortic arch, and right ductus arteriosus, exhibited progressive right upper lobar emphysema since his birth. The emphysema was caused by the right ductus arteriosus compressing the right upper bronchus. After division of the ductus arteriosus the emphysema completely regressed. We should explore the cause of lobar emphysema thoroughly before lobectomy especially when it is extrinsic. The emphysema may regress by eliminating the extrinsic factor.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
7/44. Total cavopulmonary connection in a bedridden patient with sotos syndrome.sotos syndrome is an overgrowth disorder of unknown etiology associated with a high incidence of congenital heart defects. Of 60 patients with sotos syndrome treated in our hospital, 6 had congenital heart defects. We describe a case of successful total cavopulmonary connection at 30 months of age in a patient having pulmonary atresia with intact ventricular septum and a patent ductus arteriosus who had walking disability. The postoperative course was uneventful.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
8/44. Stent implantation in right-sided patent ductus arteriosus to relieve severe cyanosis in adult patient with pulmonary atresia and ventricular septal defect.patients with unrepaired pulmonary atresia and ventricular septal defect may develop stenosis of collaterals or shunts to the pulmonary arteries leading to hypoperfusion of lungs and systemic hypoxemia. A 25-year-old female with pulmonary atresia and ventricular septal defect presented with progressively increasing cyanosis and exercise intolerance. A restrictive right-sided patent ductus arteriosus was identified as the main source of pulmonary blood flow. We report transcatheter implantation of a balloon-expandable stent across the stenosed duct to augment the pulmonary blood flow as a palliative management option. Patient had immediate improvement in arterial oxygen saturation from 66% to 85% with excellent clinical improvement and stable oxygen saturation on 8 months of follow-up.- - - - - - - - - - ranking = 6keywords = atresia (Clic here for more details about this article) |
9/44. esophageal atresia, duodenal atresia, and unilateral lung agenesis: a case report.The association of pure esophageal atresia, duodenal atresia, and unilateral lung agenesis has not been reported previously. Here the authors present a case of a newborn with this constellation of anomalies that underwent staged repair. The primary principle guiding treatment was the avoidance of iatrogenic injury to the single lung. Therefore, the order of operations proceeded as follows: (1) placement of a decompressing gastrostomy tube, (2) repair of the duodenal atresia, and (3) repair of the esophageal atresia. The congenital closed loop obstruction caused by the esophageal and duodenal atresias was beneficial in that it resulted in growth by stretching of the distal esophagus, allowing a tension-free primary repair of the esophageal atresia.- - - - - - - - - - ranking = 14keywords = atresia (Clic here for more details about this article) |
10/44. Stent implantation into vertical arterial duct in adult patient with cyanotic congenital heart disease.A 26-year-old patient, who has double inlet left ventricle, pulmonary atresia, and a patent vertical arterial duct, presented with progressive cyanosis with an arterial oxygen saturation of 71%. He had had three shunt operations and only the last aortopulmonary shunt was patent. angiography revealed a stenotic aortopulmonary shunt, stenotic vertical arterial duct, and a branch stenosis of the left pulmonary artery. He underwent successful stent implantation into both the aortopulmonary shunt and vertical arterial duct and his arterial oxygen saturation increased to 88%. This case report illustrates that stent implantation into a vertical arterial duct is feasible and can be an alternative to shunt operation.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
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