1/619. Development of rib notching after a cava-pulmonary anastomosis. A patient with tetralogy of fallot and mitral regurgitation developed unilateral rib notching on the right side secondary to a palliative cava-pulmonary anastomosis. After the operation, decreased perfusion of the right upper and middle lobes stimulated the development of collateral vessels from the systemic circulation to the right pulmonary artery. To our knowledge, this is the first time that this has been described. ( info) |
2/619. Decreased left ventricular filling pressure 8 months after corrective surgery in a 55-year-old man with tetralogy of fallot: adaptation for increased preload. A 55-year-old man with tetralogy of fallot underwent corrective surgery. Left ventricular filling pressure increased markedly with increased left ventricular volume one month after surgery, then decreased over the next 7 months, presumably due to increased left ventricular compliance. ( info) |
| We report a case of infective endocarditis secondary to enterococcus avium in a 1-year-old infant with tetralogy of fallot and a Blalock-Taussig shunt. To our knowledge, this is the first case of E. avium endocarditis to be reported. ( info) |
4/619. 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. ( info) |
5/619. Jagged1 mutations in patients ascertained with isolated congenital heart defects. Mutations in Jagged1 cause alagille syndrome (AGS), a pleiotropic disorder with involvement of the liver, heart, skeleton, eyes, and facial structures. Cardiac defects are seen in more than 95% of AGS patients. Most commonly these are right-sided defects ranging from mild peripheral pulmonic stenosis to severe forms of tetralogy of fallot. AGS demonstrates highly variable expressivity with respect to all of the involved systems. This leads us to hypothesize that defects in Jagged1 can be found in patients with presumably isolated heart defects, such as tetralogy of fallot or pulmonic stenosis. Two patients with heart defects of the type seen in AGS and their relatives were investigated for alterations in the Jagged1 gene. Jagged1 was screened by a combination of cytogenetic and molecular techniques. Patient 1 was studied because of a four-generation history of pulmonic stenosis. Molecular analysis showed a point mutation in Jagged1 in the patient and her mother. Patient 2 was investigated owing to the finding of tetralogy of fallot and a "butterfly" vertebra on chest radiograph first noted at age 5 years. She was found to have a deletion of chromosome region 20p12 that encompassed the entire Jagged1 gene. The identification of these two patients suggests that other patients with right-sided heart defects may have subtle findings of AGS and Jagged1 mutations. ( info) |
| 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. ( info) |
7/619. 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. ( info) |
8/619. Sevoflurane for dental extraction in children with tetralogy of fallot. Two children with tetralogy of fallot presented for dental extraction. Anaesthesia was induced rapidly and smoothly by inhalation of sevoflurane. We discussed the advantages of sevoflurane as an induction agent as compared to halothane in these children. ( info) |
9/619. amiodarone-induced pulmonary toxicity in an adolescent. We report amiodarone-induced pulmonary toxicity in an 18-year-old boy who had undergone corrective surgery for tetralogy of fallot 4 years earlier, and was treated with amiodarone because of recurrent malignant postoperative ventricular tachyarrhythmias. Toxicity was recognized on the basis of clinical features, chest X-ray, high-resolution contrast enhanced computerized tomography, and resolution of the findings subsequent to withdrawal of amiodarone and treatment with steroids. Pulmonary toxicity due to amiodarone, as far as we know, has not been reported in children and young adults, and its occurence even in young adults requires wider appreciation. ( info) |
| aortopulmonary septal defect and tetralogy of fallot is a rare combination. We report a case of tetralogy of fallot, non-confluent pulmonary arteries with the left arising from the arterial duct, and a large aortopulmonary septal defect diagnosed by echocardiogram and confirmed by cardiac catheterization. ( info) |