Interact CardioVasc Thorac Surg 2007;6:416-417. doi:10.1510/icvts.2006.148270
© 2007 European Association of Cardio-Thoracic Surgery
One-stage unifocalization followed by staged Fontan operation
Takeshi Shinkawaa,
Masaaki Yamagishia,*,
Keisuke Shuntoha and
Hitoshi Yakub
a Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
b Division of Cardiovascular Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan
Received 10 November 2006;
received in revised form 15 February 2007;
accepted 16 February 2007
*Corresponding author. Tel.: +81-75-251-5752; fax: +81-75-257-5910.
E-mail address: myama{at}koto.kpu-m.ac.jp (M. Yamagishi).
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Abstract
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We report a successful case of staged Fontan operation for single ventricle, pulmonary atresia, major aortopulmonary collaterals and absent central pulmonary artery. The one-stage unifocalization, concomitant with central pulmonary artery creation by autologous pericardium and modified Blalock-Taussig shunt at 1 month of age as the first palliation, and the Glenn operation and pulmonary arterial augmentation with superior vena caval patch at 9 months of age as the second palliation were performed. Extracardiac conduit Fontan operation was completed at 22 months of age. The pulmonary artery continued developing with increasing pulmonary arterial index and stable pulmonary arterial pressure throughout the clinical course. The one-stage unifocalization in early infancy and the repeated surgical intervention to the pulmonary artery without prosthetic material provided excellent pulmonary arterial growth and Fontan completion.
Key Words: Unifocalization; Pulmonary atresia; Single ventricle; Collateral
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1. Introduction
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There is still no consensus as to which is the best surgical procedure for functional single ventricle and major aortopulmonary collateral arteries (MAPCAs). We describe a successful case of staged Fontan operation for asplenia, dextrocardia, single ventricle, pulmonary atresia, MAPCAs and absent central pulmonary artery (PA).
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2. Clinical summary
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A one-month-old boy with asplenia, dextrocardia, single ventricle, pulmonary atresia and bilateral superior vena cavae was referred to our hospital. An aortogram at 1 month of age showed three MAPCAs originating from the aortic arch and descending aorta and connected to all the pulmonary segments. The central PA was absent (Fig. 1a, b).
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Fig. 1. (a), (b) Preoperative aortogram showing a small MAPCA originating from the aortic arch and two large MAPCAs from the descending aorta with absent central PA. (c) Post-unifocalization angiogram showing the created central PA. (d) Post-Fontan angiogram showing excellent development of the PA.
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At 1 month of age and with a body weight of 4.4 kg, he underwent a one-stage unifocalization concomitant with central PA creation and modified Blalock-Taussig shunt. Through a median sternotomy, a cardiopulmonary bypass was established and all MAPCAs were divided. The floor of the central PA was created by side-to-side anastomosis with two large MAPCAs, and the roof with a small MAPCA and a large pericardial patch. A modified Blalock-Taussig shunt was placed on the new central PA (Fig. 2). An angiogram at 7 months of age showed an adequate development of the PA with a pulmonary arterial index (PAI) of 143, and peripheral pulmonary stenosis in a few locations (Fig. 1c). The mean PA pressure measured by a catheter was 11 mmHg. He underwent a bilateral bidirectional Glenn operation concomitant with bilateral PA augmentation by bilateral superior vena caval patches at 9 months of age [1]. The Blalock-Taussig shunt was kept open for an additional flow. An angiogram at 20 months of age showed a sufficient development of the PA with a PAI of 181 and no peripheral pulmonary stenosis. The mean PA pressure measured by a catheter was 14 mmHg. Fontan operation with expanded polytetrafluoroethylene extracardiac conduit was completed at 22 months of age. An angiogram at 36 months of age showed excellent development of the PA with a PAI of 228 and smooth venous flow in the pulmonary trees (Fig. 1d). The central venous pressure measured by a catheter was 11 mmHg.
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Fig. 2. Operative scheme of one-stage unifocalization. (a) Dividing and incising all MAPCAs, (b) creating the floor of central pulmonary artery, (c) creating the roof of central pulmonary artery and placing a Blalock-Taussig shunt.
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3. Discussion
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Functional single ventricle and MAPCAs used to be considered contraindications for the Fontan operation, and surgical treatment is still challenging. For the patients with pulmonary atresia, ventricular septal defect and MAPCAs, however, some authors reported sufficient pulmonary arterial growth after one-stage unifocalization in early infancy [2]. Moreover, a successful case of Fontan operation for a patient with MAPCA has been reported recently [3]. We believe that one-stage unifocalization can also afford sufficient and balanced pulmonary arterial growth for Fontan candidates, even when lacking a central PA, and that it is important for Fontan candidates to solve morphological problems in the PA as earlier as possible without using prosthetic material [4]. In the present case, the PA continued developing with increasing PAI and stable PA pressure throughout the clinical course, even after the Fontan operation. We think that the one-stage unifocalization with autologous pericardium in early infancy and the repeated surgical intervention to the pulmonary artery without prosthetic material resulted in this excellent pulmonary arterial growth. The number of pulmonary segments which are required for the Fontan operation is not clear yet, however, unifocalization of more pulmonary segments without pulmonary hypertension may improve the patient's prognosis. In the present case, the connections between MAPCAs and all pulmonary segments at the outset enabled the one-stage unifocalization of all pulmonary segments and contributed to the good result.
In conclusion, one-stage unifocalization with autologous material in early infancy, followed by staged Fontan operation, is a good surgical option for selected patients with functional single ventricle and MAPCAs.
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References
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- Shinkawa T, Yamagishi M, Shuntoh K, Koushi K, Ogawa M, Yaku H. Pulmonary artery augmentation using autologous vena cava in right heart bypass operations. Ann Thorac Surg 2006; 81:1143–1145.[Abstract/Free Full Text]
- Reddy VM, Liddicoat JR, Hanley FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1995; 109:832–845.[Abstract]
- Reinhartz Olaf, Mohan Reddy V, Petrossian Edwin, Sam Suleman Edwin, Mainwaring Richard D, Rosenthal David N, Feinstein Jeffrey A, Gulati Raj, Hanley Frank L. Unifocalization of major aortopulmonary collaterals in single-ventricle patients. Ann Thorac Surg 2006; 82:934–939.[Abstract/Free Full Text]
- Hibino N, Shin'oka T, Kurosawa H. Long-term histologic findings in pulmonary arteries reconstructed with autologous pericardium. N Engl J Med 2003; 348:865–867.[Free Full Text]
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Abstract
1. Introduction