PubMed · 4673533
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A Lellouch. [Aortic kinking syndrome. (Pseudocoarctation)].. https://pubmed.ncbi.nlm.nih.gov/4673533/
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This paper describes a 12-year-old Japanese boy with coarctation of the aorta, renal hypoplasia, Turner/Noonan surface anomalies, and a 46,XY karyotype. Although the patient might represent an exceptional case of Noonan syndrome, the combination of the somatic stigmata appears to be consistent with a mutation of the putative lymphogenic gene(s) for Turner somatic stigmata.
BACKGROUND: Current corrective surgical approaches for the Taussig-Bing heart include arterial switch with ventricular septal defect (VSD) closure and intraventricular repair as described by Kawashima. METHODS: Between 1983 and 1994, 20 children underwent intracardiac repair of Taussig-Bing anomaly. Mean age at operation was 17 months (range, 1 week to 9 years). Prior palliation included pulmonary artery band (15) with coarctation repair (8) and atrial septectomy (1). Arterial switch with VSD closure was performed in 16 patients, 10 with anteroposterior great arteries. Kawashima repair was performed in 4 patients, all with side-by-side great arteries. RESULTS: After arterial switch, there was one operative death (6.2%) due to myocardial ischemia and three late deaths (18.7%) due to pulmonary hypertension, gastrointestinal bleeding, and acute lymphocytic leukemia. In the Kawashima repair group there have been no deaths. After arterial switch, 9 patients underwent 11 reoperations for residual coarctation (3), residual pulmonary artery stenosis (2), aortic valve replacement, aortic valvuloplasty, unrecognized VSD, mitral valvuloplasty, mediastinitis, and pacemaker insertion. After Kawashima repair, 1 patient underwent reoperation for baffle stenosis and 1 for an unrecognized VSD. CONCLUSIONS: For children with Taussig-Bing anomaly, the Kawashima intraventricular repair (for side-by-side great arteries) preserves the native aortic valve and avoids coronary dissection. The arterial switch operation with VSD closure can be applied without ventriculotomy to all great artery relationships and allows neonatal repair with or without concomitant coarctation repair. Both techniques yield excellent early and intermediate-term results despite the high rates of prerepair palliation and postrepair reoperation for both groups.
BACKGROUND: An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure. METHODS: From June 1982 to April 1995, of 128 patients with partial atrioventricular canal, 35 patients (27%) underwent surgical treatment at less than 1 year of life. Associated cardiac anomalies were present in 22 patients. Only 7 patients (20%) had Down's syndrome. Five patients with left ventricular hypoplasia underwent aortic coarctectomy (3 patients) or Norwood operation (2 patients). The other 30 patients underwent anatomic repair in 24 cases and aortic coarctectomy in 6. The surgical results of patients submitted for anatomic repair were retrospectively correlated with the echocardiographic mitral valve diameter. RESULTS: There were 7 deaths (29%) after anatomic repair, 2 (22%) after aortic coarctectomy, and 2 (100%) after Norwood operation. Infants with a mitral valve diameter less than 2.5 x 10-2 m/m2 died at repair. In a mean follow-up of 73.5 months there were five secondary mitral valve plasties and three repairs after aortic coarctectomy. CONCLUSIONS: Among patients with partial atrioventricular canal, there is an important subgroup with clinical signs of heart failure in the first year of life. Left-sided obstructive lesions and complex mitral valve anomalies seem to play a fundamental role in the clinical evolution and prognosis of these patients. The echocardiographic mitral valve diameter may be useful for determining the correct surgical indication.