Ann Thorac Surg 1996;62:1199-1202
© 1996 The Society of Thoracic Surgeons
Case Report
Direct Anastomosis of Pulmonary Artery-to-Right Ventricular Outflow for Correction of Tetralogy of Fallot With Pulmonary Atresia
Fumikazu Nomura, MD,
Keishi Kadoba, MD,
Hajime Ichikawa, MD,
Hiroshi Akedo, MD,
Hikaru Matsuda, MD
First Department of Surgery, Osaka University Medical School, Osaka, Japan
Accepted for publication April 20, 1996.
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Abstract
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A case of tetralogy of Fallot with well-developed infundibulum and pulmonary atresia was successfully repaired with direct anastomosis to reconstruct the pulmonary arterial trunk without using an extracardiac conduit. This technique may give the maximum chance for growth of the new pulmonary trunk and vasculature.
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Introduction
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One-stage neonatal repair for congenital heart disease is the current trend and has several advantages. Di Donato and associates [1] have reported that symptomatic tetralogy of Fallot with and without pulmonary atresia in a neonate was repaired primarily instead of by shunt operation in case of adequate continuity between the right ventricular outflow tract and the pulmonary arterial trunk. A long discontinuity between both segments is considered to be a contraindication to primary repair of tetralogy of Fallot. However, a few methods to reconstruct the new pulmonary arterial trunk with viable autologous tissue have been reported [2–5]. Recently, we successfully performed direct anastomosis to construct a new pulmonary arterial trunk with aggressive mobilization of right and left pulmonary arteries, without using an extracardiac conduit or the left atrial appendage.
A 45-day-old female baby had suffered from cyanosis since birth and was medically treated with prostaglandin E1. She was followed up by serial echocardiography with the diagnosis of tetralogy of Fallot with pulmonary atresia. Catheterization at the age of 18 days showed reasonable right and left pulmonary artery size (right, 4.7 mm; left, 4.7 mm; pulmonary artery index, 173 mm/m2) with confluence (Fig 1
). Pulmonary blood flow was totally dependent on the patent ductus arteriosus. The pulmonary arterial trunk was absent, and only a fibrous connection could be found between the right ventricular outflow tract and the pulmonary artery and a well-developed right ventricular infundibulum. There was a gap, about 10 mm long, between the right ventricle and the pulmonary artery by angiography. Late-phase right ventriculography revealed a small left appendage.
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Fig 1. . (A) Preoperative right ventriculography showed confluent pulmonary artery with small stump of pulmonary artery trunk (arrows). (B) Preoperative patent ductus angiography revealed confluent pulmonary artery with small stump of pulmonary artery trunk (arrows). (C) Lateral view of preoperative right ventriculography showed short pulmonary artery trunk stump and left atrial appendage (arrows).
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A piece of autologous pericardium was treated with 0.6% glutaraldehyde for 15 minutes to be subsequently used as a pulmonary monocusp valve. The distance between the pulmonary artery and the right ventricle was fairly long (13 mm), with a fibrous string connecting them, and a very small stump of pulmonary arterial trunk was present. Using moderately hypothermic (25°C) cardiopulmonary bypass with blood cardioplegia, we cut off this fibrous connection from the right ventricle, opened it to the central pulmonary artery, and then enlarged it toward the left pulmonary artery. Right atriotomy and standard right ventriculotomy were performed to close the malaligned ventricular septal defect and resect some muscle bands of the infundibulum. The right ventricular outflow tract reconstruction was performed without external conduit or left atrial appendage interposition, but with direct anastomosis at the posterior side augmented with equine pericardium combined with a glutaraldehyde-treated autologous pericardial monocusp at the anterior wall (Fig 2). Both pulmonary arteries were well mobilized to both hili, which could be the key technique to make the direct anastomosis possible. The patent foramen ovale was left open.
This patient could be easily weaned off cardiopulmonary bypass. Data from cardiac catheterization 5 months after the corrective operation showed satisfactory postoperative hemodynamics. The right ventricular/left ventricular ratio was 0.45, with a maximum pressure gradient from right ventricle to peripheral pulmonary artery of 13 mm Hg. The degree of pulmonary regurgitation was mild, and a trivial left-to-right shunt was present via the foramen ovale by echocardiography. Angiography of the right ventricular outflow tract showed a smooth and wide passage from the right ventricle to both right and left pulmonary arteries without stenosis (Fig 3).
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Fig 3. . Postoperative right ventriculography showed lack of stenosis of the right ventricular outflow tract (arrows) and mild pulmonary regurgitation.
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Comment
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As a general rule, confluent pulmonary arteries without major systemic-to-pulmonary collaterals receiving retrograde blood flow through a patent ductus are likely to be of adequate size. As Di Donato and associates [1] suggested, our proposal of one-stage repair for tetralogy of Fallot with pulmonary atresia (ductus dependent) is feasible in some neonates and early infants. This repair facilitates early physiologic repair and avoids pulmonary arterial distortion and stenosis; moreover, it could help to induce normal growth of the pulmonary vasculature. The main purpose of our procedure was to give a maximum chance for growth of the right ventricle-to-pulmonary artery connection, using as viable tissue her own pulmonary artery.
On the other hand, it can be extremely difficult, in performing a palliative operation on very small neonates, to avoid pulmonary artery distortion and achieve a satisfactory balance of pulmonary-to-systemic blood flow. In addition, the long-standing volume load of a systemic-to-pulmonary shunt has been demonstrated to compromise left ventricular function.
Conceptually this procedure is the same as the réparation à l'étage ventriculaire procedure [2, 3] in Taussig-Bing anomaly or the Barbero-Marcial procedure in truncus arteriosus [4]. It has the potential for future growth of the pulmonary trunk and arteries. Of course, this speculation needs further evaluation. However, we believe that this direct anastomosis method on the posterior side is possible for some cases of tetralogy of Fallot with pulmonary atresia if the infundibulum of the right ventricle is well developed and both pulmonary arteries are well mobilized up to both hili of the lung to pull the confluent pulmonary artery down to the right ventricular incision without extreme tension. The other issue is the benefit of the presence of the pulmonary valve when an extracardiac conduit is used. In our case, mild pulmonary regurgitation at 5 months after operation showed that the monocusp composite pulmonary valve had been effectively functioning for at least 5 months. Thus, this technique may give the maximum chance for growth of the pulmonary vasculature, the pulmonary arterial trunk, and the right ventricular outflow tract with minimal or mild pulmonary regurgitation.
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Footnotes
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Address reprint requests to Dr Matsuda, First Department of Surgery, Osaka University Medical School, 2-2, Yamadaoka, Suita, Osaka 565, Japan.
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References
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- Di Donato RM, Jonas RA, Lang P, Rome JJ, Mayer JE, Castañeda AR. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg 1991;101:126–37.[Abstract]
- Lecompte Y, Neveau JY, Leca F. Reconstruction of the pulmonary outflow tract without prosthetic conduit. J Thorac Cardiovasc Surg 1982;84:727–33.[Abstract]
- Rubay J, Lecompte Y, Batisse A. Anatomic repair of anomalies of ventriculo-arterial connection (REV). Results of a new technique in cases associated with pulmonary outflow obstruction. Eur J Cardiothorac Surg 1988;2:305–50.[Abstract/Free Full Text]
- Barbero-Marcial M, Riso A, Atik E, Jatene A. A technique for correction of truncus arteriosus type I and II without extracardiac conduits. J Thorac Cardiovasc Surg 1990;99:364–9.[Abstract]
- Kitagawa T, Katoh I, Chikugo F, et al. Technique for constructing the pulmonary trunk for tetralogy of Fallot with pulmonary atresia. Ann Thorac Surg 1995;59:1245–8.[Abstract/Free Full Text]
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Abstract
Introduction
