Ann Thorac Surg 2005;79:1044-1047
© 2005 The Society of Thoracic Surgeons
Case report
One-Stage Unifocalization and Palliative Right Ventricular Outflow Tract Reconstruction
Takeshi Shinkawa, MDa,
Masaaki Yamagishi, MD*,a,
Keisuke Shuntoh, MDa,
Akiyuki Takahashi, MDa,
Kyoko Hayashida, MDa,
Nobuo Kitamura, MDa
a Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan
Accepted for publication September 22, 2003.
* Address reprint requests to Dr Yamagishi, Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi, Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
myama{at}koto.kpu-m.ac.jp
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Abstract
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We report a successful case of one-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction for pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries and central pulmonary arterial absence. Through a median sternotomy, one-stage unifocalization was accomplished using autologous pericardial conduit and no prosthetic material. To achieve an adequate pulmonary blood flow, the right ventricular outflow tract was reconstructed so that it would be 70% of the normal pulmonary annular diameter, while the ventricular septal defect was left open. One-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction is considered to be a good surgical option for selected patients with these diseases.
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Introduction
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Today there is still no consensus as to the surgical procedure for pulmonary atresia and ventricular septal defect (VSD) with major aortopulmonary collateral arteries (MAPCAs) and central pulmonary arterial absence. We describe a successful case of one-stage unifocalization (UF) concomitant with palliative right ventricular outflow tract (RVOT) reconstruction.
A 6-month-old boy weighing 8.2 kg was referred to our hospital after a diagnosis of pulmonary atresia with VSD and MAPCAs. Angiography showed three MAPCAs originating from the descending aorta, one on the right side and two on the left. The right MAPCA went to the right lung. Although the smaller left MAPCA went to the left lung, the other MAPCA also had a branch to the right lung. We referred to this branch as the smaller right MAPCA and we referred to the MAPCA originating on the right as the greater right MAPCA. The central pulmonary artery was completely absent. The three MAPCAs supplied pulmonary blood flow to all pulmonary segments (Fig 1). As measured by catheterization, the Qp/Qs was 0.8.
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Fig 1. Preoperative angiography showing (A) three major aortopulmonary collateral arteries (MAPCAs) from the descending aorta. (B) Schematic diagram of the MAPCAs.
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A median sternotomy was performed. Two autologous pericardial rolls, each 10 mm in diameter were created. All the MAPCAs were identified and mobilized free in the posterior mediastinum by dissecting between the superior vena cava, the ascending aorta, the descending aorta and the roof of the left atrium. The bilateral pleural spaces were opened widely to dissect bilateral hilar portions and to identify the MAPCAs. All the MAPCAs were divided after establishing a cardiopulmonary bypass. An inverted "C" shape was incised in the greater MAPCA and a wide opening was made by side-to-side anastomosis. The smaller right MAPCA was also incised longitudinally and unifocalized to the greater right MAPCA by side-to-side anastomosis. One pericardial roll was anastomosed end-to-end to the open site of UF. The greater left MAPCA was incised longitudinally and a wide opening was made in it. The other pericardial roll was anastomosed end-to-end to the open site in the greater left MAPCA, and smaller left MAPCA was unifocalized by oblique end-to-side anastomosis to the pericardial roll. The right pericardial roll was placed under the superior vena cava and the ascending aorta. Two pericardial rolls were anastomosed side-to-end to make a confluence of pulmonary arteries. One polytetrafluoroethylene valve was sewn onto the inside of the pericardial roll. After aortic cross-clamping, a right ventriculotomy was made at the RVOT. The ventriculotomy and the valved pericardial roll were anastomosed together. The diameter of the ventriculotomy and the valved conduit was 7 mm, which was 70% of the normal pulmonary annular diameter (calculated from the equation by Rowlatt and colleagues [1]). The VSD was left open (Fig 2).
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Fig 2. Operative procedures showing (A) incisions of the major aortopulmonary collateral arteries (MAPCAs), (B) unifocalization of the right MAPCAs by side-to-side anastomosis, (C) anastomosis of one pericardial roll and unifocalized right MAPCAs, anastomosis of the other pericardial roll and the greater left MAPCA, unifocalization of the smaller left MAPCA in an end-to-side fashion, connection of both pericardial rolls and reconstruction of the continuity between the right ventricle and the mono-valved pericardial roll.
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Postoperative angiography showed a widely patent autologous pericardial roll serving as the central pulmonary artery. The right pulmonary artery was stenotic where the ascending aorta may have been compressing it. All the peripheral pulmonary arteries were connected to the pericardial roll, except for one right MAPCA. The remaining MAPCA originated from the descending aorta, and it was not identified by the initial angiography (Fig 3). Three months after the operation, a reoperation was performed to enlarge the right pulmonary artery with azygos venous patch and to connect the remaining right MAPCA attached to the pericardial roll. Postoperative angiography at 11 months after the initial operation showed good growth of each pulmonary artery (Fig 4). As measured by catheterization, the Qp/Qs was 1.15 and the pulmonary arterial pressure was 23/7 mm Hg. Definitive repair, including VSD closure and re-RVOT reconstruction with a valved polytetrafluoroethylene patch, was carried out 20 months after the initial operation.
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Fig 3. Postoperative angiography showing (A) the widely patent autologous pericardial roll serving as the central pulmonary artery and the right pulmonary arterial stenosis (white arrow), and (B) the remaining right major aortopulmonary collateral arteries (MAPCA) originating from the descending aorta (black arrow).
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Fig 4. The follow-up angiography before the definitive repair showing the widely patent autologous pericardial roll as the central pulmonary artery.
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Comment
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Treating patients with pulmonary atresia, VSD, and MAPCAs is surgically challenging. There is yet no consensus as to which is the most appropriate surgical strategy. Recently many infants have been treated by single-stage UF and complete repair [2, 3] as opposed to conventional multi-stage UF through a lateral thoracotomy followed by VSD closure. Single-stage complete repair in infants has the advantages of greater potential for growth of the respiratory system, avoidance of the deleterious effects of chronic cyanosis and volume overload of the heart, and prevention of the pulmonary vascular obstruction developing in unprotected collaterals [3, 4], but it is also technically difficult and highly invasive. It was reported that the longer cardiopulmonary bypass time needed for single-stage complete repair increased perioperative mortality [4]. Moreover single-stage complete repair would have the risks of postoperative pulmonary hypertension and pulmonary hypertensive crisis, even if the intraoperative flow study was performed [5, 6]. Therefore, we prefer one-stage UF through a median sternotomy concomitant with palliative RVOT reconstruction in infants, because it is less invasive than single-stage complete repair, it has great potential for growth of the pulmonary artery, and it poses little risks of postoperative pulmonary hypertension and hypertensive crisis.
It is difficult to control the balance of the pulmonary and systemic blood flow during palliative operation, and generally more consideration is needed as to how to achieve an adequate pulmonary blood flow. We managed to achieve an adequate pulmonary blood flow with the palliative RVOT reconstruction in several patients by making the diameter of the RVOT 70% of that of the normal pulmonary annulus. Careful postoperative care and follow-up to avoid high pulmonary flow were needed.
We prefer direct native tissue-to-tissue anastomosis and autologous material such as the pericardium and the venous wall graft for UF. The autologous pericardium is very useful material because it has growth potential, but it is difficult to maintain its shape. In this patient, the pericardial rolls were placed behind the superior vena cava and the ascending aorta. The pericardial roll was anastomosed with the MPACAs at the bilateral hilar portion without excessive tension, but it was compressed by the ascending aorta and became stenotic. We have to be aware of the risk of compression and distortion of the autologous pericardium and, if necessary, alter the route of the pericardial roll or perform an additional procedure such as aortopexy.
In conclusion, we believe that one-stage UF concomitant with palliative RVOT reconstruction through a median sternotomy is a good surgical option for selected patients with pulmonary atresia, VSD, and MAPCAs.
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References
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- Rowlatt UF, Rimoldi HJ, Lev M. The quantitative anatomy of the normal child's heart. Pediatr Clin North Am. 1963;10:499–588
- Reddy VM, Liddicoat JR, Hanley FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 1995;109:832–845[Abstract]
- Tchervenkov CI, Salasidis G, Cecere R, et al. One-stage midline unifocalization, and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals. J Thorac Cardiovasc Surg. 1997;114:727–737[Abstract/Free Full Text]
- Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation. 2000;101:1826–1832[Abstract/Free Full Text]
- Reddy VM, Petrossian E, McElhinney DB, Moore P, Teitel DF, Hanley FL. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed? J Thorac Cardiovasc Surg. 1997;113:858–868[Abstract/Free Full Text]
- Carotti A, Di Donato RM, Squitieri C, Guccione P, Catena G, Castaneda AR. Total repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: an integrated approach. J Thorac Cardiovasc Surg. 1998;116:914–923[Abstract/Free Full Text]
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Abstract
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