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      Morphologic Substrates for First-Branch Pulmonary Arterial Hypoplasia in Transposition of the Great Arteries

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          Abstract

          Background: Distal right-sided outflow obstruction remains a problem after arterial switch operation. We studied the anatomical features of the pulmonary trunk (PT) and its branches that are susceptible to right and left pulmonary arterial (RPA and LPA) hypoplasia in transposition of the great arteries (TGA). Methods: One hundred and one angiograms of TGA performed between 1981 and 1996 were viewed, and Polaroid photos were taken at end-systole. The diameters of RPA, LPA, PT, duct, ascending aorta, and angles between PA and PT were measured, and the ductal flow direction was recorded. Results: Forty-eight cases (47.5%) had a PA/PT diameter ratio (both PAs had same size) below 0.49. A smaller PA/PT was significantly related to posterior inclination of the proximal PT [narrower right (r = 0.50, p < 0.00001) and left (r = 0.48, p < 0.00001) PA-PT angle in lateral view] and a larger duct (r = 0.37, p < 0.0001). Eighteen patients had a follow-up angiogram after a mean period of 8.5 months. Those with a closed duct had evident PA growth (n = 12, 0.51 ± 0.09 to 0.74 ± 0.17, p < 0.0001), but four patients with an attenuated duct had no significant change (0.58 ± 0.06 to 0.68 ± 0.08, p = NS), and one with a persistent large duct had even regression of PA/PT (0.36–0.19). The direction of ductal flow was toward the aorta during early systole on cineangiogram. Conclusions: First-branch PA hypoplasia, which is frequently seen in TGA, was related to the right-to-left shunt through a duct resulting in hemodynamic starvation, and to posterior inclination of the proximal PT in this setting. Natural regression of the duct facilitated PA growth.

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          Most cited references17

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          Results of the arterial switch operation in neonates with transposed great arteries.

          The arterial switch operation is judged the best palliative operation for neonates with transposed great arteries. We aimed to assess the value of analysing a large series of unselected cases by this technically demanding operation and formulate a realistic prognosis. We reviewed all 432 neonates (mean age at operation 7 days, mean weight 3.25 kg) who underwent an arterial switch operation between 1987 and 1999. Follow-up (mean time: 4.9 years) was complete in 412 patients. Survival probability and freedom from reoperation was 94% and 78% at 10 years, respectively. 26 patients died, 16 because of myocardial ischaemia. Risk factors for death included early experience, low weight, associated cardiovascular malformations (especially hypoplasia of the right ventricle or aortic arch), and difficult patterns of coronary arteries. The risk of the coronary artery pattern was greatly reduced in those who had recent operations. At last follow-up, 90% of patients had normal life without treatment, and 94% a normal heart function on echocardiography. The arterial switch operation in neonates achieves excellent results mid-term. Obstruction of the translocated coronary arteries is responsible for most deaths and a substantial number of reoperations. Although confirmation is needed, these results allow anticipation of a favourable long-term prognosis.
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            Usefulness of magnetic resonance imaging for evaluating great-vessel anatomy after arterial switch operation for D-transposition of the great arteries.

            Magnetic resonance imaging (MRI) produces high-resolution images of great-vessel anatomy in pediatric patients. In this study seven patients, aged 6 to 27 months were evaluated by using gated MRI and two-dimensional echocardiography 4 to 26 months after arterial switch operation for D-transposition of the great arteries. Measurements were taken at the right and left ventricular outflow tracts, beneath the semilunar valves, at the midaortic sinuses, at the anastomotic sites of the main pulmonary artery and the aorta, at the origin of the branch pulmonary arteries, and at the distal pulmonary arteries 1 cm beyond the bifurcation. Concordant results were obtained with both imaging techniques from all sides with the exception of the left pulmonary artery and the right pulmonary artery. With MRI, four patients had significant narrowing at the right pulmonary artery origin and six patients had narrowing at the left pulmonary artery origin. With two-dimensional echocardiogram, two patients had narrowing at the right pulmonary artery origin and four patients had narrowing at the left pulmonary artery origin. The measured pulmonary artery intraluminal diameters in these patients were consistently smaller when assessed by MRI versus two-dimensional echocardiography. To verify these results, five of seven patients underwent cardiac catheterization to provide physiologic correlation before reoperation; the MRI results were found to be significantly closer to the actual catheterization measurements. We conclude that MRI is a sensitive imaging technique for evaluation of great-vessel anatomy in patients after arterial switch operation for D-transposition of the great arteries. It is particularly useful in the evaluation of the branch pulmonary artery anatomy.
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              Modified arterial switch operation by spiral reconstruction of the great arteries in transposition

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                Author and article information

                Journal
                CRD
                Cardiology
                10.1159/issn.0008-6312
                Cardiology
                S. Karger AG
                0008-6312
                1421-9751
                2007
                May 2007
                02 February 2007
                : 107
                : 4
                : 362-369
                Affiliations
                Departments of aPediatrics and cSurgery, Mackay Memorial Hospital, Mackay Medicine, bNursing and Management College, Departments of dSurgery and ePediatrics, National Taiwan University Hospital, and National Taiwan University College of Medicine, Taipei, Taiwan
                Article
                99052 Cardiology 2007;107:362–369
                10.1159/000099052
                17283427
                afde31f0-7ba8-48a6-aba1-3cbe75eac1ea
                © 2007 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 30 March 2006
                : 02 September 2006
                Page count
                Figures: 3, Tables: 3, References: 30, Pages: 8
                Categories
                Original Research

                General medicine,Neurology,Cardiovascular Medicine,Internal medicine,Nephrology
                First-branch pulmonary arterial hypoplasia,Patent ductus arteriosus,Right-to-left shunt,Posterior inclination of proximal pulmonary trunk,Transposition of great vessels

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