1/187. Prenatal detection of a tetralogy of fallot with origin of the left pulmonary artery from the ascending aorta in a familial 22q11 microdeletion.Here we report a case of prenatal diagnosis of anomalous origin of the left pulmonary artery from the ascending aorta associated with a tetralogy of fallot in a familial form of 22q11 deletion. The mother, who had a normal heart and a velo-cardio-facial syndrome, had a first child with a pulmonary atresia plus ventricular septal defect associated with a 22q11 deletion. prenatal diagnosis during the second pregnancy identified the above-described cono-truncal anomaly and FISH study showed a recurrent 22q11 deletion. This case illustrates the intrafamilial variability of cardiac involvement in 22q11 deletion as well as the possibility of diagnosing complex cono-truncal malformations during fetal life.- - - - - - - - - - ranking = 1keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
2/187. Left main coronary artery compression by aneurysmal pulmonary artery in a patient with tetralogy of fallot with absent pulmonary valve.We describe an 11-year-old girl with tetralogy of fallot and absent pulmonary valve, who on selective coronary angiography was found to have extrinsic compression of the left main coronary artery by the aneurysmally dilated pulmonary artery. This abnormality has not been reported previously.- - - - - - - - - - ranking = 4.7363632659968keywords = pulmonary valve, valve (Clic here for more details about this article) |
3/187. Esophageal lung with multiple congenital anomalies: conundrums in diagnosis and management.BACKGROUND/PURPOSE: Communicating bronchopulmonary foregut malformations (CBPFM) are a diverse group of potentially devastating congenital anomalies with anatomy that may be difficult to delineate. The authors present a case that illustrates conundrums in the diagnosis and management of these complex disorders. methods: A term baby had esophageal atresia (EA), tracheoesophageal fistula (TEF), and tetralogy of fallot. Initially, a gastrostomy was performed, and a balloon catheter was inserted through the endotracheal tube to occlude the fistula until the patient was hemodynamically stable. Subsequently, the fistula was ligated. Postoperatively, the left lung collapsed, and bronchoscopy showed an atretic left mainstem bronchus. Repeat thoracotomy showed that the fistula ligation was intact. air was introduced through the gastrostomy tube, and, surprisingly, the left lung inflated, indicating the left mainstem bronchus arose from the esophagus distal to the ligated TEF. RESULTS: Despite reopening this fistula, ventilation remained poor, and support was withdrawn. autopsy findings confirmed a unilobed left lung arising from the esophagus, EA, TEF, an atretic left mainstem bronchus, tetralogy of fallot, and digeorge syndrome. CONCLUSIONS: This is the first report of a combination of EA and distal TEF with a second CBPFM involving the esophagus and the entire left lung. Successful correction of these anomalies will require extensive delineation of the anatomy to plan an operative strategy.- - - - - - - - - - ranking = 0.029536595618571keywords = atresia (Clic here for more details about this article) |
4/187. Laparoscopic cholecystectomy for the adult with unrepaired tetralogy of fallot: a case report.A 24-year-old woman with a history of unrepaired tetralogy of fallot was scheduled to undergo laparoscopic cholecystectomy. Her significant history included tetralogy of fallot with pulmonary atresia, hypoplastic left pulmonary artery, pulmonary vascular obstructive disease, a functioning right subclavian artery to right pulmonary artery shunt (modified Blalock-Taussig palliative procedure) with a similar shunt on the left side that is occluded. The patient underwent general endotracheal anesthesia for laparoscopic cholecystectomy for cholelithiasis and pancreatitis. Anesthetic induction, intraoperative course, and the postoperative period proceeded uneventfully, and the patient quickly progressed to the preoperative level of functioning. The careful application of pharmacological and physiological principles guided the anesthetic plan and produced a successful outcome. Principles for the anesthetic management of the patient with cyanotic congenital heart disease undergoing noncardiac surgery are reviewed.- - - - - - - - - - ranking = 1keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
5/187. cyanosis due to diastolic right-to-left shunting across a ventricular septal defect in a patient with repaired tetralogy of fallot and pulmonary atresia.cyanosis as a result of right-to-left shunting across a ventricular septal defect is commonly encountered in patients with congenital heart disease when systolic pressure in the right ventricle exceeds that in the left ventricle. Reported is the case of a child who remained cyanosed after surgical correction of pulmonary atresia despite right ventricular systolic pressure being lower than left ventricular pressure. Colour-flow Doppler showed a residual ventricular septal defect, with right-to-left shunting in diastole alone.- - - - - - - - - - ranking = 4.0295365956186keywords = pulmonary atresia, atresia (Clic here for more details about this article) |
6/187. Pathologic finding of restenosis in stent-implemented right ventricle-pulmonary artery extracardiac conduit.We describe an excised specimen of a stent-implanted valved equine pericardial extracardiac conduit in the right heart. It appears from careful pathologic examination that the stent acted as a nidus for thrombus formation followed by thick neo-intimal development over the stent, which caused restenosis. Restenosis occurred despite anticoagulation.- - - - - - - - - - ranking = 0.0026258730355296keywords = valve (Clic here for more details about this article) |
7/187. Anomalous origin of the left coronary artery in tetralogy of fallot associated with abnormal mitral valve pathology.The association of anomalous left coronary artery with congenital heart disease is a rare occurrence. Seven cases of anomalous left coronary artery associated with tetralogy of fallot have been reported in the literature. We report a unique case with severe mitral valve abnormality that precluded standard surgical repair.- - - - - - - - - - ranking = 0.013129365177648keywords = valve (Clic here for more details about this article) |
8/187. A new approach to correction of tetralogy of fallot with absent pulmonary valve.A new technique for primary repair of tetralogy of fallot with absent pulmonary valve syndrome is suggested. In addition to the standard intracardiac portion of tetralogy of fallot repair, this approach includes translocation of the pulmonary artery anterior to the aorta, shortening of the ascending aorta, and shortening and plication of the pulmonary artery. This new technique should eliminate compression of the tracheobronchial tree caused by impingement by the pulmonary artery.- - - - - - - - - - ranking = 4.7363632659968keywords = pulmonary valve, valve (Clic here for more details about this article) |
9/187. biliary atresia splenic malformation syndrome--is it a result of embryonically midline rotational defects? A case report.The authors present a case of biliary atresia splenic malformation (BASM) syndrome in a 34-week gestational age boy who died shortly after birth. autopsy results showed polysplenia, short pancreas, extrahepatic biliary atresia, heterotaxy of liver, tracheoesophageal and duodenal atresia, right lung hypoplasia, cardiac defects with tetralogy of fallot, axial skeletal defects, and minor genitourinary disorders. Additional histopathologic findings were congenital hepatic fibrosis and bronchopulmonary dysplasia. The authors consider that the abnormalities of this case are possibly a unique combination. Midline defects, which are the most notable features of the presented case, may be closely related to pathogenesis of BASM syndrome.- - - - - - - - - - ranking = 0.20675616933keywords = atresia (Clic here for more details about this article) |
10/187. Familial absent pulmonary valve syndrome without deletions of chromosome 22q11.Deletions of chromosome 22q11 are common in patients with tetralogy of fallot, and in those with absent pulmonary valve syndrome. In this report, we describe a pair of siblings with absent pulmonary valve syndrome, neither of whom had deletions of chromosome 22q11. The finding of familial absent pulmonary valve syndrome without deletion of 22q11 in our patients suggests an alternative genetic basis for this rare condition.- - - - - - - - - - ranking = 6.6309085723956keywords = pulmonary valve, valve (Clic here for more details about this article) |
| | Next -> |