1/71. 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 = atresia (Clic here for more details about this article) |
2/71. 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 RESULTS: Despite reopening this fistula, ventilation remained poor, and support was withdrawn.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 = 1keywords = atresia (Clic here for more details about this article) |
3/71. 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 = atresia (Clic here for more details about this article) |
4/71. 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 = 5keywords = atresia (Clic here for more details about this article) |
5/71. 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 = 7keywords = atresia (Clic here for more details about this article) |
6/71. Coarctation co-existing with tetralogy of fallot and pulmonary atresia.We describe the finding of unsuspected aortic coarctation, discovered by catheterisation at 4 months, in a neonate first daignosed on the fourth day of life as having tetralogy of fallot with pulmonary atresia and major aorto-to-pulmonary collateral arteries. This previously unreported combination contravenes the established theory of patterns of flow during fetal life, and presented difficult issues in clinical management.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
7/71. Aortopulmonary window coexisting with tetralogy of fallot.Aortopulmonary window is a rare cardiac malformation presenting either in the isolated form, or in association with other cardiac anomalies. The isolated form usually presents an increased pulmonary blood flow as the main clinical feature, but if associated with other cardiac anomalies the clinical findings change according to the additional defect. Only 19 cases of aortopulmonary window associated with tetralogy of fallot have been reported. Five of them have associated pulmonary atresia. We report two neonatal cases of aortopulmonary window and tetralogy of fallot, one of them presenting an associated pulmonary atresia.- - - - - - - - - - ranking = 2keywords = atresia (Clic here for more details about this article) |
8/71. Rapid onset of intrapulmonary arteriovenous shunting after surgical repair of tetralogy of fallot with pulmonary atresia.We describe a 2-year-old girl with tetralogy of fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
9/71. tetralogy of fallot associated with chromosome 22q11.2 deletion in adolescents and young adults.PURPOSE: To clarify the clinical profiles of adolescents and young adults with tetralogy and 22q11.2 deletion, which has recently been identified as a cause of tetralogy of fallot in about 15% of patients. methods: Thirty-four patients with 22q11.2 deletion and tetralogy of fallot, with or without pulmonary atresia, including 15 males and 19 females, with their age ranging from 16 to 35 years (mean = 25) were studied. Main outcome measurements include chromosome deletion identified by fluorescence in situ hybridization (FISH) of peripheral blood lymphocytes, medical states assessed with new york heart association classification, social activity assessed with Warnes index, IQ assessed by Wechsler test. RESULTS: Eighteen of 20 patients with tetralogy and pulmonary stenosis had cardiac repair, and their cardiac conditions were good except one. Of 14 patients with tetralogy with pulmonary atresia, 7 had Rastelli type cardiac repair and were doing well, although 4 of them needed re-operation for conduit stenosis. No cardiac repair was done in the other 7 patients with tetralogy, pulmonary atresia and major collateral arteries because their peripheral pulmonary arteries were too small. In 28 of the 34 patients (82%), overall social activity was limited because of extracardiac diseases, including deafness, club feet, mental retardation, and schizophrenia. The IQ in 17 patients was 59 /- 13 (mean /- SD): range 41 to 79. In two patients, repeated IQ study showed a decrease. Four patients developed schizophrenia. CONCLUSION: Tetralogy with 22q11 deletion can be repaired surgically except in those patients with pulmonary atresia, major collateral arteries, and small peripheral pulmonary arteries. However, most of the adult patients show an inability to function in social life in contrast to most patients with tetralogy but without the deletion, who have a normal social life. Extracardiac diseases, including deafness, club feet, mental retardation, and schizophrenia were major handicaps limiting full social activities in postoperative adolescents and young adults with 22q11.2 deletion and tetralogy.- - - - - - - - - - ranking = 4keywords = atresia (Clic here for more details about this article) |
10/71. Monozygotic twins with chromosome 22q11 microdeletion and discordant phenotypes in cardiovascular patterning.Monozygotic twins with chromosome 22q11 microdeletions offer an ideal situation to observe the association of microdeletion and disrupted cardiovascular patterning. We report monozygotic twins concordant for 22q11.2 microdeletion but discordant for cardiovascular patterning. Both twins showed identical intracardiac defects including tetralogy of fallot with pulmonary atresia. Nevertheless, their great vessel patternings were variable. These twins show that the mispatterning of the great vessels may not correlate with intracardiac morphogenesis. The discordant development of the great vessels, especially in the pulmonary vascular system, has clinical significance for prognosis. The phenotypic variability of cardiovascular anomalies seen in 22q11 microdeletion cannot be explained on the basis of genotypic difference.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
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