Ductal origin of the distal pulmonary artery in truncus arteriosus.

The Annals of thoracic surgery (Impact Factor: 3.65). 07/2007; 83(6):2262; author reply 2262-3. DOI: 10.1016/j.athoracsur.2006.10.010
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    ABSTRACT: There are no large series describing the morphologic spectrum and the clinical outcomes of children with ductal origin of the distal pulmonary artery (PA). Medical records were reviewed for all children presenting between 1970 and 2001. Angiograms were reviewed at presentation and at last available follow-up. Forty-five patients were identified with median presenting age of 14 days (range, birth to 6.5 years). Ductal origin of the distal PA occurred as an isolated finding in 16 patients (36%), with tetralogy of Fallot in 12 (27%), with pulmonary atresia-ventricular septal defect in 13 (29%), and with heterotaxy in 4 (9%). Diagnostic pulmonary venous wedge angiography was performed in 21 patients (47%). Surgical procedures were undertaken in 31 patients, and were initial systemic-ductal PA shunt in 13 patients, interposition graft in 6, direct anastomosis to the main PA in 2, ductal PA banding in 2, unifocalization of the ductal PA with complete or staged pulmonary atresia-ventricular septal defect repair in 7, and heart transplantation in 1 patient. Surgical revision was required in 3 patients and catheter interventions in 12 patients. Overall 20-year survival was 70% and was improved among patients without congestive heart failure at presentation (p = 0.08, hazard ratio: 2.81). Reconstruction of the ductal PA decreased the prevalence of pulmonary parenchymal hypoplasia (p < 0.001) and scoliosis at last available follow-up. Ductal origin of the distal PA is associated with important multisystem morbidity and mortality. Early diagnosis and repair of the ductal PA, especially in children presenting with pulmonary overcirculation, may improve outcomes.
    The Annals of thoracic surgery 03/2006; 81(3):950-7. DOI:10.1016/j.athoracsur.2005.08.065 · 3.65 Impact Factor
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    ABSTRACT: Evaluation of a consistent policy of elective repair of truncus arteriosus at 2-3 months of age for the patients who are independent of hospital ward care, and long-term outcome. Retrospective study of 82 patients with truncus arteriosus who underwent total repair at the Victorian Paediatric Cardiac Surgical Unit between 1979 and December 1995. The timing was based on a consistent policy of elective repair at 2-3 months of age for patients who were independent of hospital ward care. Earlier repair was performed when the patients were in uncontrolled congestive heart failure. Follow-up was complete for all patients with a mean of 76 months (1-183). There were 11 hospital deaths (13.4% CL 9-18.5), and five late deaths, actuarial survival at 80 months was 81% (CL 70-88%) with 39 patients uncensored at that point. For the purpose of this presentation, patients have been grouped according to their age at repair; 1, neonates n = 17 (hospital mortality = 5); 2, infants 1-6 months of age n = 48 (hospital mortality = 4); 3, patients beyond 6 months n = 17 (hospital mortality = 2). This series includes 10 patients with interrupted aortic arch with no mortality, and 10 patients with discontinuous pulmonary artery (hospital mortality = 2). Thirty-seven patients have had 54 conduits replaced to date. Using multiple regression, body weight < 3 kg was the only significant independent risk factor for hospital mortality. Our management policy tended to gather patients with risk factors described elsewhere into presentation group 1 and low risk truncus patients into presentation group 2. Deferral of surgery to 2-3 months of age is possible and lowers the surgical risk.
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    ABSTRACT: DiGeorge anomaly and related syndromes are frequent genetic conditions (1/4000 per live birth) characterized by conotruncal heart disease, anomalies of the face, thymic dysfunction, hypoparathyoidism and developmental delay, due to microdeletion of chromosome 22q11. Common arterial trunk (CAT) is one of the classic conotruncal defect of this syndrome; it ranges between 3 and 10% of patients and the prevalence of deletion 22q11 in a population of children with CAT is 30%. In patients with CAT and deletion 22q11 there is a prevalence of additional anomalies of the aortic arch (interruption, right sided or double aortic arch) and/or of the pulmonary arteries (discontinuity, stenosis or crossed pulmonary arteries). Severe dysplasia of the truncal valve (insufficiency and/or stenosis) can be present as well as the truncal origin from the right ventricle. These additional cardiovascular defects could complicate surgical repair. A multispecialistic approach is indicated for these patients in order to reduce mortality and mobidity.
    Progress in Pediatric Cardiology 06/2002; DOI:10.1016/S1058-9813(02)00004-8