Ductal Origin of the Distal Pulmonary Artery in Truncus Arteriosus

The Annals of thoracic surgery (Impact Factor: 3.85). 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 · 3.85 Impact Factor
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    ABSTRACT: Salient clinical, hemodynamic, angiocardiographic, and pathologic findings are presented in 100 patients with truncus arteriosus communis, 79 of whom were studied at autopsy. In this study of typical truncus, all had a ventricular septal defect (type A). Truncus with a partially formed aorticopulmonary septum (type A1) was much the commonest form (50%). Cases with no remnant of aorticopulmonary septum (type A2) were second in frequency (21%). The distinction between types A1 and A2 could not be made with certainty in 9%, because these types merge into one another. Cases with absence of either pulmonary artery branch (type A3) were the least frequent form (8%). Truncus with interruption, atresia, preductal coarctation, or severe hypoplasia of the aortic arch (type A4) constituted 12%. The diagnosis of truncus is primarily angiocardiographic. The plane of the truncal valve in the lateral projection is distinctive. It tilts anteriorly, facing the patient's toes, which can be of assistance in differential diagnosis. The angiocardiographic features of type A4, although unfamiliar, are pathognomonic. Since the median age at death was only 5 weeks, and in view of the difficulties associated with pulmonary artery banding, our goal should be the surgical correction of truncus during the first and second months of life.
    American Heart Journal 08/1976; 92(1):23-38. · 4.46 Impact Factor
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    ABSTRACT: Although the early mortality for repair of truncus arteriosus has decreased in the modern era, routine correction in the neonate has not been widely adopted. To assess the results of our protocol of early repair, we reviewed 46 neonates and infants undergoing repair of truncus arteriosus at the University of Michigan Medical Center from January 1986 to January 1992. Their ages ranged from 1 day to 7 months (median 13 days) and weights from 1.8 kg to 5.4 kg (mean 3.1 kg). Repair was performed beyond the first month of life in only 8 patients, because of late referral in 7 and severe noncardiac problems in 1. Associated cardiac anomalies were frequently encountered, the most common being interrupted aortic arch (n = 5), nonconfluent pulmonary arteries (n = 4), hypoplastic pulmonary arteries (n = 4), and major coronary artery anomalies (n = 3). Truncal valve replacement was performed in 5 patients with severe regurgitation, 3 of whom also had truncal valve systolic pressure gradients of 30 mm Hg or more. The truncal valve was replaced with a mechanical prosthesis in 2 patients and with a cryopreserved homograft in 3 patients. Right ventricle-pulmonary artery continuity was established with a homograft in 41 patients (range 8 mm to 15 mm), a valved heterograft conduit in 4 (range 12 mm to 14 mm), and a nonvalved polytetrafluoroethylene tube in the remaining patient (8 mm). There were 5 hospital deaths (11%, 70% confidence limits 7% to 17%). Multivariate and univariate analyses failed to demonstrate a relationship between hospital mortality and age, weight, or associated cardiac anomalies. Only 1 death occurred among 9 patients with interrupted aortic arch or nonconfluent pulmonary arteries. Hospital survivors were followed-up from 3 months to 6.3 years (mean 3 +/- 0.4 years). Late noncardiac deaths occurred in 3 patients, all within 4 months after the operation. Actuarial survival was 81% +/- 6% at 90 days and beyond. Despite the prevalence of major associated conditions, early repair has resulted in excellent survival. We continue to recommend repair promptly after presentation, optimally within the first month of life.
    Journal of Thoracic and Cardiovascular Surgery 07/1993; 105(6):1057-65; discussion 1065-6. · 4.17 Impact Factor