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Case Study
Extracardiac Fontan in apicocaval juxtaposition
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314553613 aan.sagepub.com
Juan-Miguel Gil-Jaurena, Ramo´n Pe´rez-Caballero, Ana Pita and Mayte Gonza´lez-Lo´pez
Abstract Extracardiac total cavopulmonary connection between the inferior vena cava and right pulmonary artery has become the most popular Fontan modification nowadays, yielding good results. However, certain situs anomalies along with systemic venous drainage variations give rise to an issue about the best way for extracardiac conduit routing. Three cases of apicocaval juxtaposition are described. The situs was inversus with the apex and inferior vena cava on the same side (left), whereas the superior vena cava (Glenn) was located on the right in all cases. The literature is revisited and technical options are discussed. Surgery was carried out with excellent short- and midterm results.
Keywords Blood vessel prosthesis implantation, fontan procedure, heart bypass, right, heart defects, congenital, heart septal defects, vena cava, inferior
Introduction Extracardiac total cavopulmonary connection between the inferior vena cava and right pulmonary artery has become the most popular Fontan modification nowadays, yielding good results.1 Its wide acceptance relies on its simplicity compared to previous techniques (because of a beating-heart approach) and avoidance of atrial enlargement on follow-up. The surgical result is a short and straight conduit connecting both venae cavae (on the right side) opposite to the apex on the left. However, certain situs anomalies along with systemic venous drainage variations may give rise to an issue about the best way for extracardiac conduit routing.
Case reports Three out of 47 Fontan patients recorded in the last 18 months presented with apicocaval juxtaposition. The situs was inversus with the apex and inferior vena cava on the same side (left) in all cases (Table 1). The first patient was a 4-year-old 15-kg girl with situs inversus, crisscross, and pulmonary atresia with ventricular septal defect. After a previous Glenn anastomosis, the apex and inferior vena cava were on the same side (left). The second patient was a 9-year-old 26-kg boy with situs inversus, unbalanced atrioventricular septal
defect (regurgitant valve), hypoplastic right ventricle, and a pacemaker for previous ablation. The Glenn anastomosis was on the right side, whereas the apex and inferior vena cava were on the left. The third patient was a 7-year-old 21-kg girl with situs inversus, heterotaxia, and inferior vena caval interruption (suprahepatic veins entering the right atrium). Her Glenn anastomosis (Kawashima procedure), apex, and inferior vena cava were on the left. The surgical strategy was standard, cannulating the ascending aorta, superior vena cava (Glenn) and inferior vena cava (with an additional femoral vein in one patient). After establishing extracorporeal circulation, the diaphragmatic surface of the heart and the apex were thoroughly dissected, the inferior vena cava was severed and anastomosed in a beveled fashion to an expanded polytetrafluoroethylene conduit. The distal end of the conduit was connected to the right pulmonary artery,
Pediatric Cardiac Surgery, Hospital General Universitario Gregorio, Maran˜o´n, Madrid, Spain Corresponding author: Juan-Miguel Gil-Jaurena, Pediatric Cardiac Surgery, Hospital GU Gregorio Maran˜o´n, C/O’Donnell 50, 28009 Madrid, Spain. Email: [email protected]
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Asian Cardiovascular & Thoracic Annals 0(0)
Table 1. Pre- and post-Fontan data in 3 patients with apicocaval juxtaposition. Age Weight (years) (kg) Diagnosis
LA Fontan pressure pressure Fontan Glenn Apex IVC size (mm) (mm Hg) (mm Hg) SO2 Follow-up
4
15
Left
Left
Left 16
17
9
9
26
Right
Left
Left 20
16
8
80% 12 months SO2 86% (stent in IVC) 80% 10 months SO2 94%
7
21
Left
Left
Left 16
16
6
82% 6 months SO2 87%
Situs inversus, crisscross, PA with VSD Situs inversus, AV septal defect, hypoplastic RV, ablation, PM Situs inversus, heterotaxia, IVC interruption
AV: atrioventricular; IVC: inferior vena caval; LA: left atrial; PA: pulmonary atresia; PM: pacemaker; RV: right ventricle; SO2: oxygen saturation; VSD: ventricular septal defect.
Figure 1. Extracardiac Fontan crossing the midline. The inferior vena cava and apex are ipsilateral (left), with the distal anastomosis in the right pulmonary artery.
crossing from the lower left side to the upper right corner of the thorax (Figure 1). A 4–5-mm fenestration was fashioned between the conduit and the right atrium in all cases. Two operations were performed on a beating-heart basis, and a short period of cardioplegic arrest was needed for atrioventricular valve repair in the second patient. The Fontan circuit performance was correct both postoperatively and during follow-up.
Discussion After the report by Marcelletti and colleagues1 on extracardiac conduit Fontan completion, many surgical teams gradually adopted this technique. de Leval and colleagues2 applied computational fluid dynamics in several Fontan models in search of better hemodynamic
offset and energy loss avoidance. Recent studies by the same group analyzed the interactions between infradiaphragmatic venous flow and both hemidiaphragms.3 We fashion a short and straight conduit between the inferior vena cava and the right pulmonary artery, assuming this to be the best hemodynamic model. However, certain situs anomalies along with inferior vena cava variations related to the apex challenge this theory. In such cases, we have to consider whether to complete the Fontan, leave the patient in a Glenn status, or even make a transplant referral. On the other hand, 3 surgical options for an appropriate Fontan completion can be considered. The first is intracardiac; aortic crossclamping and ischemia are needed, but intraatrial variants make the intracardiac routing cumbersome and there is a long-term risk of atrial enlargement. The second is ipsilateral: a short and straight conduit but in a retrocardiac setting. This involves extensive dissection behind the heart and a risk of conduit compression (by the heart) or heart and pulmonary vein distortion (even with ringed conduits). The third is contralateral which is easy to perform with less surgical dissection and a low risk of atrial compression, but a long and non-straight conduit (energy loss) has a risk of kinking. Literature on apicocaval juxtaposition is scarce. Most papers come from Asian countries where heterotaxy is most prevalent.4,5 The contralateral method is preferred, with a long conduit from the left apex to the right pulmonary artery (provided there is a good size for the distal anastomosis). Previous reports (on 5 and 8 patients) relied on the simplicity of the procedure, avoiding retrocardiac dissection and the risk of conduit compression.4,5 A group in Pittsburgh suggested an inferior vena caval preoperative index to choose the proper conduit route according to overriding of the inferior vena cava over the spine.6 They described 13 contralateral, 11 ipsilateral, and 9 intracardiac conduits, underlining avoidance of conduit distortion over energy loss (in the contralateral routing). In our
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3 patients, we favored simplicity (the dissection is slightly longer over the diaphragmatic surface) assuming some energy loss in a longer conduit. The short- and midterm results did not differ from other Fontan patients in the same time frame. Should a future transplant be needed, a short conduit segment from the inferior vena caval anastomosis can be used in the same fashion as for a situs inversus transplant. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Marcelletti C, Corno A, Giannico S and Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg 1990; 100: 228–232.
2. de Leval MR, Dubini G, Migliavacca F, et al. Use of computational fluid dynamics in the design of surgical procedures: application to the study of competitive flows in cavopulmonary connections. J Thorac Cardiovasc Surg 1996; 111: 502–513. 3. Hsia TY, Khambadkone S, Bradley SM and de Leval MR. Subdiaphragmatic venous hemodynamics in patients with biventricular and Fontan circulation after diaphragm plication. J Thorac Cardiovasc Surg 2007; 134: 1397–1405. 4. Kawahira Y, Nishigaki K and Ueno T. Extracardiac Fontan procedure bridging the vertebra for apico-caval juxtaposition. Ann Thorac Surg 2006; 82: 350–352. 5. Morizumi S, Kato H, Kanemoto S, et al. Appropriate route selection for extracardiac total cavopulmonary connection in apicocaval juxtaposition. Ann Thorac Surg 2012; 94: 179–184. 6. Yoshida M, Menon PG, Chrysostomou C, et al. Total cavopulmonary connection in patients with apicocaval juxtaposition: optimal conduit route using preoperative angiogram and flow simulation. Eur J Cardiothorac Surg 2013; 44: 46–52.
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(AAN)
[1–3] [PREPRINTER stage]
Case Study
Extracardiac Fontan in apicocaval juxtaposition
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314553613 aan.sagepub.com
Juan-Miguel Gil-Jaurena, Ramo´n Pe´rez-Caballero, Ana Pita and Mayte Gonza´lez-Lo´pez
Abstract Extracardiac total cavopulmonary connection between the inferior vena cava and right pulmonary artery has become the most popular Fontan modification nowadays, yielding good results. However, certain situs anomalies along with systemic venous drainage variations give rise to an issue about the best way for extracardiac conduit routing. Three cases of apicocaval juxtaposition are described. The situs was inversus with the apex and inferior vena cava on the same side (left), whereas the superior vena cava (Glenn) was located on the right in all cases. The literature is revisited and technical options are discussed. Surgery was carried out with excellent short- and midterm results.
Keywords Blood vessel prosthesis implantation, fontan procedure, heart bypass, right, heart defects, congenital, heart septal defects, vena cava, inferior
Introduction Extracardiac total cavopulmonary connection between the inferior vena cava and right pulmonary artery has become the most popular Fontan modification nowadays, yielding good results.1 Its wide acceptance relies on its simplicity compared to previous techniques (because of a beating-heart approach) and avoidance of atrial enlargement on follow-up. The surgical result is a short and straight conduit connecting both venae cavae (on the right side) opposite to the apex on the left. However, certain situs anomalies along with systemic venous drainage variations may give rise to an issue about the best way for extracardiac conduit routing.
Case reports Three out of 47 Fontan patients recorded in the last 18 months presented with apicocaval juxtaposition. The situs was inversus with the apex and inferior vena cava on the same side (left) in all cases (Table 1). The first patient was a 4-year-old 15-kg girl with situs inversus, crisscross, and pulmonary atresia with ventricular septal defect. After a previous Glenn anastomosis, the apex and inferior vena cava were on the same side (left). The second patient was a 9-year-old 26-kg boy with situs inversus, unbalanced atrioventricular septal
defect (regurgitant valve), hypoplastic right ventricle, and a pacemaker for previous ablation. The Glenn anastomosis was on the right side, whereas the apex and inferior vena cava were on the left. The third patient was a 7-year-old 21-kg girl with situs inversus, heterotaxia, and inferior vena caval interruption (suprahepatic veins entering the right atrium). Her Glenn anastomosis (Kawashima procedure), apex, and inferior vena cava were on the left. The surgical strategy was standard, cannulating the ascending aorta, superior vena cava (Glenn) and inferior vena cava (with an additional femoral vein in one patient). After establishing extracorporeal circulation, the diaphragmatic surface of the heart and the apex were thoroughly dissected, the inferior vena cava was severed and anastomosed in a beveled fashion to an expanded polytetrafluoroethylene conduit. The distal end of the conduit was connected to the right pulmonary artery,
Pediatric Cardiac Surgery, Hospital General Universitario Gregorio, Maran˜o´n, Madrid, Spain Corresponding author: Juan-Miguel Gil-Jaurena, Pediatric Cardiac Surgery, Hospital GU Gregorio Maran˜o´n, C/O’Donnell 50, 28009 Madrid, Spain. Email: [email protected]
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(AAN)
[1–3] [PREPRINTER stage]
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Asian Cardiovascular & Thoracic Annals 0(0)
Table 1. Pre- and post-Fontan data in 3 patients with apicocaval juxtaposition. Age Weight (years) (kg) Diagnosis
LA Fontan pressure pressure Fontan Glenn Apex IVC size (mm) (mm Hg) (mm Hg) SO2 Follow-up
4
15
Left
Left
Left 16
17
9
9
26
Right
Left
Left 20
16
8
80% 12 months SO2 86% (stent in IVC) 80% 10 months SO2 94%
7
21
Left
Left
Left 16
16
6
82% 6 months SO2 87%
Situs inversus, crisscross, PA with VSD Situs inversus, AV septal defect, hypoplastic RV, ablation, PM Situs inversus, heterotaxia, IVC interruption
AV: atrioventricular; IVC: inferior vena caval; LA: left atrial; PA: pulmonary atresia; PM: pacemaker; RV: right ventricle; SO2: oxygen saturation; VSD: ventricular septal defect.
Figure 1. Extracardiac Fontan crossing the midline. The inferior vena cava and apex are ipsilateral (left), with the distal anastomosis in the right pulmonary artery.
crossing from the lower left side to the upper right corner of the thorax (Figure 1). A 4–5-mm fenestration was fashioned between the conduit and the right atrium in all cases. Two operations were performed on a beating-heart basis, and a short period of cardioplegic arrest was needed for atrioventricular valve repair in the second patient. The Fontan circuit performance was correct both postoperatively and during follow-up.
Discussion After the report by Marcelletti and colleagues1 on extracardiac conduit Fontan completion, many surgical teams gradually adopted this technique. de Leval and colleagues2 applied computational fluid dynamics in several Fontan models in search of better hemodynamic
offset and energy loss avoidance. Recent studies by the same group analyzed the interactions between infradiaphragmatic venous flow and both hemidiaphragms.3 We fashion a short and straight conduit between the inferior vena cava and the right pulmonary artery, assuming this to be the best hemodynamic model. However, certain situs anomalies along with inferior vena cava variations related to the apex challenge this theory. In such cases, we have to consider whether to complete the Fontan, leave the patient in a Glenn status, or even make a transplant referral. On the other hand, 3 surgical options for an appropriate Fontan completion can be considered. The first is intracardiac; aortic crossclamping and ischemia are needed, but intraatrial variants make the intracardiac routing cumbersome and there is a long-term risk of atrial enlargement. The second is ipsilateral: a short and straight conduit but in a retrocardiac setting. This involves extensive dissection behind the heart and a risk of conduit compression (by the heart) or heart and pulmonary vein distortion (even with ringed conduits). The third is contralateral which is easy to perform with less surgical dissection and a low risk of atrial compression, but a long and non-straight conduit (energy loss) has a risk of kinking. Literature on apicocaval juxtaposition is scarce. Most papers come from Asian countries where heterotaxy is most prevalent.4,5 The contralateral method is preferred, with a long conduit from the left apex to the right pulmonary artery (provided there is a good size for the distal anastomosis). Previous reports (on 5 and 8 patients) relied on the simplicity of the procedure, avoiding retrocardiac dissection and the risk of conduit compression.4,5 A group in Pittsburgh suggested an inferior vena caval preoperative index to choose the proper conduit route according to overriding of the inferior vena cava over the spine.6 They described 13 contralateral, 11 ipsilateral, and 9 intracardiac conduits, underlining avoidance of conduit distortion over energy loss (in the contralateral routing). In our
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(AAN)
[1–3] [PREPRINTER stage]
Gil-Jaurena et al.
3
3 patients, we favored simplicity (the dissection is slightly longer over the diaphragmatic surface) assuming some energy loss in a longer conduit. The short- and midterm results did not differ from other Fontan patients in the same time frame. Should a future transplant be needed, a short conduit segment from the inferior vena caval anastomosis can be used in the same fashion as for a situs inversus transplant. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Marcelletti C, Corno A, Giannico S and Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg 1990; 100: 228–232.
2. de Leval MR, Dubini G, Migliavacca F, et al. Use of computational fluid dynamics in the design of surgical procedures: application to the study of competitive flows in cavopulmonary connections. J Thorac Cardiovasc Surg 1996; 111: 502–513. 3. Hsia TY, Khambadkone S, Bradley SM and de Leval MR. Subdiaphragmatic venous hemodynamics in patients with biventricular and Fontan circulation after diaphragm plication. J Thorac Cardiovasc Surg 2007; 134: 1397–1405. 4. Kawahira Y, Nishigaki K and Ueno T. Extracardiac Fontan procedure bridging the vertebra for apico-caval juxtaposition. Ann Thorac Surg 2006; 82: 350–352. 5. Morizumi S, Kato H, Kanemoto S, et al. Appropriate route selection for extracardiac total cavopulmonary connection in apicocaval juxtaposition. Ann Thorac Surg 2012; 94: 179–184. 6. Yoshida M, Menon PG, Chrysostomou C, et al. Total cavopulmonary connection in patients with apicocaval juxtaposition: optimal conduit route using preoperative angiogram and flow simulation. Eur J Cardiothorac Surg 2013; 44: 46–52.
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