Ann Thorac Surg 1995;60:1108-1109
© 1995 The Society of Thoracic Surgeons
Case Reports
Modified Surgical Palliation for a Rare Type of L-Transposition With Aortic Atresia
J. Nilas Young, MD,
Eric J. Kuncir, MD,
William M. DeCampli, MD,
J. Gregg Helton, MD,
Edward N. Ahearn, MD
Division of Cardiothoracic Surgery, Children's Hospital Oakland, Oakland, California, and Department of Surgery, University of California, Davis-East Bay, San Francisco, California
Accepted for publication April 10, 1995.
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Abstract
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L-Transposition of the great arteries with aortic atresia without an outlet chamber is a rare congenital heart defect. We used a modified Norwood procedure incorporating a type of direct coronary transfer for successful palliation of this lesion in a neonate.
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Introduction
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In attempting to surgically address a patient with a rare and complex form of aortic atresia and single ventricular chamber, we were stimulated to attempt an operation incorporating aspects of both the arterial switch and the Norwood procedures with a very successful outcome.
The patient was an 8-day-old, 3.9-kg male infant who presented in extremis with initial arterial blood gas values as follows: pH, 6.9; carbon dioxide tension, 36 mm Hg; oxygen tension, 56 mm Hg; HCO3-, 8 mEq/L; and base excess, -24 mmol/L. On physical examination, there was a single second heart sound and a grade 3/6 harsh nonspecific ejection murmur at the upper left sternal border. Chest roentgenogram demonstrated massive cardiomegaly with increased pulmonary vascularity. The electrocardiogram revealed sinus rhythm with left ventricular hypertrophy and a QRS axis of +85 degrees. Echocardiography revealed a double-inlet single ventricle with left ventricular morphology, L-transposition of the great arteries, aortic atresia, and no evidence of an outlet chamber. Pulmonary arterial blood flow was unrestrictive, and systemic arterial flow was accomplished through a large patent ductus arteriosus. Because of the completeness of information derived by echocardiography, cardiac catheterization was not performed.
The administration of prostaglandin E1, dopamine, digoxin, and diuretics along with mechanical ventilation allowed initial stabilization of the patient, but congestive heart failure persisted. At 12 days of age, the patient underwent a palliative operation. Using single main pulmonary artery and right atrial cannulation, deep hypothermic circulatory arrest, and single-dose antegrade cold blood cardioplegic arrest, an atrial septectomy was performed. Main pulmonary artery atresia was created by transecting the distal main pulmonary artery and oversewing the pulmonary arterial bifurcation with a pulmonary allograft patch. The ascending aorta (diameter, 1.5 mm) was divided in its midportion, and its proximal end anastomosed end-to-side to the pulmonary (neoaortic) root in a fashion similar to coronary transfer in an arterial switch procedure (Fig 1A
, B). Neoaortic reconstruction was fashioned by opening a 22-mm-diameter nonvalved pulmonary allograft conduit and fashioning a tube on its cardiac end corresponding to the supravalve pulmonary arterial diameter, and on the aortic arch end tailored to the opening in the underside of the aortic arch and proximal descending thoracic aorta. While the patient was rewarmed on bypass, a 3.5-mm polytetrafluoroethylene shunt was constructed between the innominate artery and the right pulmonary artery, and then the patient was weaned from bypass without difficulty (Fig 1C).
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Fig 1. . (A) Operative findings: L-transposition of the great arteries, aortic atresia with hypoplasia of ascending aorta and arch. (B) Creation of main pulmonary artery atresia, ligation and division of distal ascending aorta and tailored proximal ascending aorta. (C) Completed palliation: reimplantation of proximal aorta into neoaortic root, interposition pulmonary allograft tube, and modified Blalock-Taussig shunt.
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The patient had an uneventful postoperative course and was released from the hospital 15 days after operation with a systemic arterial oxygen saturation of 80%. At 6 months of age the patient underwent elective cardiac catheterization. Good growth without angiographic or hemodynamic evidence of obstruction was noted in the neoaortic root, the aortic arch, the common coronary artery root (old aortic root), and the central pulmonary arteries. There was no gradient between left and right atria, and no gradient across the reconstructed aortic outflow tract, ascending aorta, aortic arch, or descending thoracic aorta. After cardiac catheterization, the patient underwent an uneventful hemi-Fontan procedure and was discharged home after a 6-day hospitalization with a systemic arterial oxygen saturation of 82%. He is currently awaiting definitive Fontan repair. He is in the fiftieth percentile for growth and height, and his only medication is aspirin for anticoagulation.
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Comment
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Double-inlet single ventricle with L-transposition of the great arteries in association with aortic atresia without an outlet chamber is a rare lesion [1–3]. Rychik and associates [4] reported palliation of 16 infants with L-transposition of the great arteries, univentricular heart and outlet chamber, and restrictive ventricular septal defect. They employed the classic Norwood palliation, and did not report that a technical modification was required. In our case, we thought that because of the presence of aortic atresia with an extremely small L-malposed ascending aorta, use of the classic Norwood technique might result in considerable proximal spiraling of the neoaortic root with distortion of the neoaorta and secondary coronary artery ischemia. For this reason we elected to transpose the proximal aorta directly to the pulmonary root (neoaorta).
These modifications might prove beneficial in other patients with either common or atypical forms of hypoplastic heart syndromes. Additionally, one might consider using an appropriately sized distal divided ascending aorta as a modified Blalock shunt to the right pulmonary artery, thereby further simplifying the procedure. The use of an allograft tube to reconstruct the ascending aorta and arch is problematic in regard to future growth and may require surgical correction, but we have noted in several of our patients with surgically palliated hypoplastic left heart syndrome that pulmonary allograft tissues demonstrate a tendency to expand with time and may obviate this potential problem.
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Footnotes
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Address reprint requests to Dr Young, Department of Surgery, Children's Hospital Oakland, 747 52nd St, Oakland, CA 94609.
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References
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- McGarry KM, Taylor JF, Macartney FJ. Aortic atresia occurring with complete transposition of the great arteries. Br Heart J 1980;44:711–3.[Abstract/Free Full Text]
- Deanfield JE, Anderson RH, Macartney FJ. Aortic atresia with ``corrected transposition of the great arteries'' (atrioventricular and ventriculoarterial discordance). Br Heart J 1981;46:683–6.[Free Full Text]
- Bullaboy CA, Harned HS. Aortic atresia with double inlet left ventricle, rudimentary left-sided right ventricle, and ventriculoarterial discordance. Br Heart J 1984;52:349–51.[Abstract/Free Full Text]
- Rychik J, Murdison KA, Chin AJ, et al. Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis. J Am Coll Cardiol 1991;18:809–16.[Abstract]
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Abstract
Introduction
