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J Thorac Cardiovasc Surg 2001;121:1205-1207
© 2001 The American Association for Thoracic Surgery
Brief Communications |
From the Departments of Pediatric Cardiovascular Surgerya and Pediatrics,b Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Received for publication Oct 10, 2000. Accepted for publication Oct 20, 2000. Address for reprints: Masaaki Yamagishi, MD, Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi, Hirokoji, Kamikyo-ku, Kyoto, 602-8566 Japan (E-mail: myama{at}koto.kpu-m.ac.jp).
Visceroatrial heterotaxia frequently involves cardiac anomalies.
1 We report the case histories of 2 neonates with the rare combination of left isomerism and aortic atresia who underwent a successful Norwood operation.
Clinical summaries
PATIENT 1
A female neonate weighing 3020 g was referred to us with respiratory distress. Echocardiography showed a common atrium, dominant right ventricle with posterior rudimentary left ventricle, common atrioventricular valve, aortic atresia, and patent ductus arteriosus (PDA). The helical computed tomographic (CT) angiography (X Vigor Laudator; Toshiba Corporation, Tokyo, Japan) was safely performed under a short-period respiratory hold (approximately 20 seconds) because the patient had been already intubated. Three-dimensional (3-D) differential color images
2 were reconstructed by using an image analyzer (X Tension, Toshiba). Video movies of rotating 3-D helical CT images were also provided for the better recognition of the anomalies. The 3-D helical CT image showed hypoplasia of the ascending aorta and aortic arch, coarctation of the aorta, PDA, deficiency of the inferior vena cava (IVC), and dilated azygos vein draining to the superior vena cava (SVC;Fig 1).
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PATIENT 2
A male neonate weighing 3100 g was referred to our hospital for surgical repair. Echocardiography disclosed a common atrium, dominant right ventricle with posterior rudimentary left ventricle, common atrioventricular valve, severe atrioventricular valve regurgitation, aortic atresia, right aortic arch, and PDA. Helical CT showed an anterior main pulmonary artery, posterior hypoplastic ascending aorta, hypoplasia of a right-sided aortic arch, coarctation of the aorta, bilateral PDA, right-sided descending aorta, stenosis at the origin of the right subclavian artery, deficiency of the IVC, bilateral SVC, and drainage of the azygos vein to the right SVC(Fig 2).
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Discussion
In the left isomeric heart, a single right ventricle with aortic atresia, unobstructed pulmonary artery, and a ductus-dependent systemic circulation is extremely uncommon.
3 Moreover, a successful Norwood operation for left isomeric heart with aortic atresia has not been reported previously. Among the peculiar morphologic features of the left isomeric heart, pulmonary venous distortion and atrioventricular valve regurgitation increase the risk of the Norwood operation.
4 On the other hand, because most cases of left isomeric heart have a common atrium or a large atrial septal defect, there is little chance of pulmonary obstructive disease caused by a narrow interatrial communication. Systemic venous anomalies, such as azygos connection, bilateral SVC, or deficiency of IVC, probably do not pose a major risk during the Norwood operation. As shown in the case of patient 2, the shape of the aortic arch of the left isomeric heart is substantially different from that of hypoplastic left heart syndrome with normal situs.
During the Norwood operation for the isomeric heart with peculiar arterial anomalies, the helical CT images are extremely useful for optimal 3-D orientation of the reconstructed neoaorta. Compared with the images obtained by means of conventional echocardiography or angiography, 3-D helical CT images allow precise determination of the shape and spatial relation of the great arteries from optional angles. This innovative technique can be adapted, even for neonates and infants with various serious arterial or venous anomalies.
2,5
Footnotes
*Gore-Tex; registered trade name of W. L. Gore & Associates, Inc, Flagstaff, Ariz. ![]()
References
This article has been cited by other articles:
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I. Shiraishi, Y. Yamamoto, S. Ozawa, A. Kawakita, K. Toiyama, T. Tanaka, K. Sakata, T. Hayano, T. Itoi, M. Yamagishi, et al. Application of helical computed tomographic angiography with differential color imaging three-dimensional reconstruction in the diagnosis of complicated congenital heart diseases J. Thorac. Cardiovasc. Surg., January 1, 2003; 125(1): 36 - 39. [Full Text] [PDF] |
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