Interact CardioVasc Thorac Surg 2008;7:278-279. doi:10.1510/icvts.2007.171959
© 2008 European Association of Cardio-Thoracic Surgery
Proposal for bail-out procedures - Congenital |
Fontan operation through a right lateral thoracotomy to treat Cantrell syndrome with severe ectopia cordis
Yuki Okamoto*,
Yorikazu Harada and
Shunji Uchita
Nagano Children's Hospital, 3100 Toyoshina, Azumino city, Nagano, 399-8288 Japan
Received 19 November 2007;
received in revised form 23 December 2007;
accepted 24 December 2007
*Corresponding author. Tel.: +81-263-73-6700; fax: +81-263-73-5432.
E-mail address: yamanashimedical{at}yahoo.co.jp (Y. Okamoto).
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Abstract
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A median sternotomy would be very difficult for Cantrell syndrome with severe ectopia cordis. For Cantrell syndrome and tricuspid atresia after left modified Blalock-Taussig shunt with severe ectopia cordis, defect in the middle and inferior portion of the sternum, and the closing of ventriculo-peritoneal shunt, we performed extracardiac total cavopulmonary connection through a right lateral thoracotomy after establishing right modified Blalock-Taussig shunt and performing coil embolization of left modified Blalock-Taussig shunt by cardiologists.
Key Words: Congenital heart disease; Interventional catheterization; Univentricular heart
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1. Introduction
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Cantrell syndrome with severe ectopia cordis is rare, and in association with tricuspid atresia, is extremely rare. The cardiac anomaly and omphalocele present in Cantrell syndrome increases mortality. Permanent cure of the syndrome is very difficult [1]. We report an extremely rare case of Cantrell syndrome with severe ectopia cordis and tricuspid atresia (Classification of Keith-Edwards: Ib) after a left modified Blalock-Taussig shunt that was treated with a Fontan operation through a right lateral thoracotomy combined with a catheter intervention [2].
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2. Case report
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A 10-year-old girl underwent skin grafting with a musculocutaneous flap for ectopia cordis and an operation for omphalocele soon after birth. A left modified Blalock-Taussig shunt was created at two months and a ventriculo-peritoneal shunt for hydrocephalus at ten months. The timing of the Fontan type operation was considered during follow-up based on her mental retardation and severe ectopia cordis. However, desaturation increased gradually and cardiac catheterization was performed to evaluate the indication for a Fontan type operation. The cardiac catheter study showed a main pulmonary artery pressure of 8 mmHg (mean), pulmonary vascular resistance of 2.1 Wood units/m2 and Nakata index of 510 mm2/m2 without pulmonary artery deformity [3]. A Fontan type operation was decided after much discussion regarding the clinical indication. Before the extracardiac total cavopulmonary connection operation, the following problems were pointed out. (1) A median approach was impossible because of severe ectopia cordis (Fig. 1), (2) there was a ventriculo-peritoneal shunt across the median line, and (3) if we approach the heart through a right lateral thoracotomy, is it possible to close the left modified Blalock-Taussig shunt together with the main pulmonary artery which is located on the left side of the ascending aorta? Before the total cavopulmonary connection operation, the anatomical relation of the great arteries was confirmed by 3-dimensional computed tomography (Fig. 2). Considering these problems, we planned catheter intervention for shunt occlusion to the left modified Blalock-Taussig shunt and decided to perform an extracardiac total cavopulmonary connection procedure through a right lateral thoracotomy.
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Fig. 2. 3DCT of right lateral view without right ribs.
VP shunt, ventricular-peritoneal shunt; mainPA: main pulmonary artery; AAo, ascendig aorta; SVC, superior vena cava; IVC, inferior vena cava; EC, ectopia cordis.
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The patient was placed in the left lateral position and a right lateral thoracotomy was performed in the third intercostal space. The visceral pleura and pericardium was incised and retracted to expose the heart. From a preoperative 3-dimensional computed tomography, the main pulmonary artery was located in the left anterior position of the ascending aorta, and we found it possible to approach the main pulmonary artery. Before occluding the left modified Blalock-Taussig shunt, a right modified Blalock-Taussig shunt using 5 mm expanded polytetrafluoroethylene was established. Then, the chest was closed. Soon after completion of the right modified Blalock-Taussig shunt operation, the patient was transferred to the cardiac cath lab adjacent to the surgical suite. Coil embolization of the left modified Blalock-Taussig shunt was performed by the cardiologists. On the following day, the total cavopulmonary connection operation was performed through a second right lateral thoracotomy. The main pulmonary artery was doubly ligated under extracorporeal circulation with an ascending aortic and bicaval cannulation. The superior vena cava was resected and the proximal stump was sutured closed, then the right modified Blalock-Taussig shunt was removed and the superior vena cava was anastomosed to the right pulmonary artery branch. Extracardiac total cavopulmonary connection was completed with an expanded polytetrafluoroethylene graft (18 mm diameter) that was anastomosed between the right pulmonary artery branch and the inferior vena cava. Her postoperative clinical course was uneventful, and she was discharged 14 days after the operation. She is doing well and doesn't have any cardiac events at 25 months after surgery.
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3. Discussion
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Cantrell syndrome was first reported in 1958 by Cantrell and his colleague and is characterized by the following anomalies: 1) a midline, supraumbilical abdominal wall defect (omphalocele), 2) a defect of the lower sternum, 3) a deficiency of the anterior diaphragm, 4) a defect in the diaphragmatic pericardium (ectopia cordis), and 5) congenital intracardiac defects [1]. The prognosis of this syndrome is dependent on the severity of omphalocele, cardiac anomaly, and ectopia cordis [1, 5]. As intracardiac defects, ventricular septal defect, atrial septal defect, tetralogy of Fallot and ventricular diverticulum are common in Cantrell syndrome, but tricuspid atresia is extremely rare [4]. Our patient had a rare cardiac anomaly of tricuspid atresia with severe ectopia cordis and omphalocele. Based on the previously reported severity grade, our patient fell into the high risk group. To our knowledge, this is the first report of a Fontan operation for tricuspid atresia with Cantrell syndrome. In this case, residual left modified Blalock-Taussig shunt flow seemed to cause additional flow in the pulmonary artery and this might disturb the Fontan circulation. We decided to take down the left modified Blalock-Taussig shunt and make a temporary shunt on the opposite side. To close the left modified Blalock-Taussig shunt, we selected hybrid therapy of coil embolization in collaboration with cardiologists to avoid the bilateral thoracotomy. For this very rare case, we found that preoperative 3-dimensional computed tomography was useful to confirm the anatomical relationship between ascending aorta and main pulmonary artery. In addition, hybrid therapy combining a surgical and catheter intervention was less invasive than surgery alone. Furthermore, we found that extracardiac total cavopulmonary connection through a right lateral thoracotomy was useful in our case since it was not possible to perform a median sternotomy [6].
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References
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- Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602–614.[Medline]
- Vlad P. Heart disease in infancy and childhood. Macmillan Publishing Co. 1980;421–487.
- Nakata S, Imai Y, Takanashi Y, Kurosawa H, Tezuka K, Nakazawa M, Ando M, Takao A. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart disease with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984;88:610–619.[Abstract]
- Crittenden IH, Adams FH, Mulder DG. A syndrome featuring defects of the heart, sternum, diaphragm, and anterior abdominal wall. Circulation 1959;20:396–404.[Medline]
- Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart. Pediatrics 1972;50:778–792.[Abstract/Free Full Text]
- Nanaumi M, Asou T, Takeda Y, Lin Z-B, Ohara K, Yoshimura H. Total cavopulmonary connection via a thoracotomy. Ann Thorac Surg 2002;74:917–919.[Abstract/Free Full Text]
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Abstract
1. Introduction