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Ann Thorac Surg 2007;83:2262-2263
© 2007 The Society of Thoracic Surgeons
Department of Surgery, Oregon Health & Science University, Mail Code L223, 3181 Sam Jackson Park Rd, Portland, OR 97201
(Email: karamlou{at}ohsu.edu).
The recent letter by Carotti and colleagues [1] addresses the association between a rare variant of truncus arteriosus and ductal origin of the distal pulmonary artery, which was not mentioned in our recent review [2]. The modified Van Praagh classification [3] describes a single origin of the pulmonary artery from the common arterial trunk (usually the right pulmonary artery), with either the ductus or collateral vessels supplying the contralateral side, classified as type A3. Others have described this morphologic type as hemitruncus [4, 5] to emphasize the discontinuity of the pulmonary arteries.
Although we are aware of this variant of truncus arteriosus, it is worth mentioning that overall, Van Praagh types A1 and A2 represent the majority of the morphologic spectrum of truncus arteriosus, comprising 86% of patients with truncus arteriosus in the GLH autopsy series and 89% in the Toronto series [6, 7]. Those patients with a true ductal origin of the distal pulmonary artery, as opposed to those with multiple collateral supply, represent an even more discrete subgroup, with only 2% of all patients with type A3 truncus arteriosus. Accordingly, we had no patients with hemitruncus in our series. This rarity notwithstanding, the morphology of hemitruncus is highly variable, and thus experience with each type is limited, making useful inferences problematic.
We chose instead to concentrate on the morphologic spectrum present in our series of patients, although certainly the association mentioned by Carotti and colleagues [1] is relevant. Regarding surgical management, it is pragmatic to consider general principles used in the management of patients with ductal origin of the distal pulmonary artery that are described in our report. Specifically, those with pulmonary atresia and bilateral ducti in which unifocalization to create an intrapericardial pulmonary confluence followed by right ventricular outflow tract reconstruction was required. Or, our description of the importance of pulmonary overcirculation as a presenting sign in patients with type A3 truncus arteriosus. The association with deletion of chromosome 22q11 with truncus arteriosus (ie, roughly 33%) is an important point [8, 9]. However this genetic abnormality is not specific to ductal origin of the distal pulmonary artery, and therefore it was not mentioned in our report.
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