doi:10.1510/mmcts.2005.001578
Orthotopic heart transplantation for congenital heart defects: anomalies of the systemic venous return Olivier Raisky, Daniel Tamisier, Pascal R. Vouhe´* Department of Pediatric Cardiac Surgery, Groupe Hospitalier Necker – Enfants Malades, 149 rue de Se`vres, 75015 Paris, France Anomalies of the systemic venous return are frequently associated with other congenital heart defects. Some anomalies do not complicate really orthotopic heart transplantation (such as azygos continuation of the inferior vena cava). Other anomalies raise more difficulties; the most frequent one is persistent left superior vena cava draining into either the coronary sinus or the left atrium. Sometimes, the left superior vena cava can be ligated without untoward effect. In most cases, the left superior vena cava must be anastomosed to the right atrial compartment, preferably using extracardiac procedures. Most problems can be solved by harvesting extra lengths of donor superior vena cava and innominate vein. In rare patients, associated anomalies of the pulmonary venous return may require additional partitioning of the atria. The early risk of heart transplantation is probably not increased by the presence of such anomalies.
Keywords: Congenital heart defects; Anomalies of systemic venous return; Persistent left superior vena cava; Orthotopic heart transplantation Introduction
Anomalous return to the right atrium
Anomalies of the systemic venous return are frequently associated with other congenital cardiac defects. In situs solitus, a left superior vena cava connecting to the coronary sinus is common. In right isomerism, bilateral cavae connecting to bilateral morphologically right atria may occur. In left isomerism, multiple coexisting systemic venous anomalies are the rule.
Anomalous left superior vena cava connecting to the coronary sinus is common and occurs in 2–4% of all congenital cardiac defects. In approximatively 75% of the cases, there is hypoplasia or agenesis of the left innominate vein. The left superior vena cava originates at the confluence of the left internal jugular and subclavian veins, descends anterior to the hilus of the left lung and becomes continuous with the coronary sinus which is typically enlarged.
Anatomical considerations From a surgical stand-point, anomalies of the systemic venous return can be classified into five categories. * Corresponding author: Tel.: q33-1-44381867; fax: q33-144381911. E-mail: [email protected] 䉷 2006 European Association for Cardio-thoracic Surgery
Interrupted inferior vena cava occurs in 0.6% of patients with congenital heart defects. The hepatic and the prerenal segments of the inferior vena cava fail to fuse into a continuous channel. The prerenal segment of the interrupted inferior vena cava joins either the azygos vein and the right superior vena cava (azygos continuation of the inferior vena cava) or 1
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578 Table 1. Combined anomalies of systemic and pulmonary venous connections ● Anomalous systemic and pulmonary venous connection to the right atrium. – Left SVC to coronary sinus – right pulmonary vein(s) to right atrium. – Left SVC to coronary sinus – left pulmonary vein(s) to left SVC. – Left SVC to coronary sinus – partial or total anomalous pulmonary venous to coronary sinus. – Azygos continuation of IVC – right pulmonary vein(s) to right atrium. ● Anomalous systemic venous connection to left atrium and anomalous pulmonary venous connection. – Left SVC to left atrium – right pulmonary vein(s) to right atrium. – Left SVC to left atrium – anomalous pulmonary vein(s) to left SVC. – Anomalous systemic vein (right SVC or hepatic veins) to left atrium – anomalous pulmonary vein(s) to anomalous systemic vein.
the hemi azygos vein and a persistent left superior vena cava draining into the coronary sinus (hemi azygos continuation of the inferior vena cava).
Surgical techniques
Anomalous return to the left atrium
Precise preoperative evaluation and careful planning of the procedure are essential to solve the problems related to anomalies of the systemic venous return. Both systemic venous and pulmonary venous connections must be defined. This is usually best achieved by a combination of echocardiography, cardiac catheterization and multi slice CT scan.
The commonest anomaly is a left superior vena cava draining into the left atrium. The orifice of the left superior vena cava is situated in the left upper corner of the left atrium between the base of the left atrial appendage anteriorly and the left pulmonary veins posteriorly. Usually, there is a right superior vena cava and the innominate vein is absent. Rarely, there is no right superior vena cava. Typically, the coronary sinus is absent and the coronary veins drain individually into the atria. Other less common types of anomalous systemic return to the left atrium include drainage into the left atrium of the inferior vena cava, hepatic veins, or right superior vena cava. Anomalous return to both atria This includes a combination of the anomalies described previously. An azygos continuation of the inferior vena cava to the right superior vena cava and a left superior vena cava to the left atrium is one example.
General considerations
The technical principles of heart transplantation are twofold (1) to create a recipient left atrial cuff which receives all the pulmonary veins and is anastomosed to the donor left atrium (2) to achieve adequate systemic venous return either by direct cavo-caval anastomoses or by a combination of cavo-caval anastomoses and atrial anastomosis between the donor right atrium and a recipient right atrial cuff receiving some of the systemic veins. Some anomalies (for example azygos continuation of the inferior vena cava) do not complicate really heart transplantation which can be carried out using a conventional technique. Other anomalies raise more difficulties which can, however, all be solved by adequate planning of the procedure. Donor operation
Combined anomalous systemic and pulmonary venous return Multiple variants are possible. Table 1 is a list of such anomalies w1x. Actually, all combinations may probably be encountered. Surgically-induced anomalies These include intracardiac anomalies of systemic and pulmonary venous return secondary to atrial repair of transposition of the great arteries (Senning or Mustard procedure) and anomalies of the systemic return induced by various types of cavo-pulmonary connections. 2
According to the planned reimplantation procedures needed in the recipient, donor heart harvesting includes the superior vena cava and the innominate vein, most of the intrapericardial and transdiaphragmatic inferior vena cava and the pulmonary veins. Adequate partitioning of the veins needs collaboration with the surgical teams who eventually harvest the liver and the lungs. Recipient operation Cannulation for cardiopulmonary bypass The presence of anomalies of the systemic venous return may complicate the technique of venous cannulation
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578
Schematic 1. Cardiac anatomy in patients with persistent left superior vena cava (draining into the coronary sinus or the left atrium). The venae cavae are cannulated separately. The incisions performed for recipient heart excision are shown in red dotted lines. The left superior vena cava is transected at its entrance into the heart, near the base of the left atrial appendage. RSVC s right superior vena cava – LSVC s left superior vena cava – LA s left atrium – RA s right atrium – IVC s inferior vena cava.
Schematic 2. Appearance of the mediastium after heart excision in patients with persistent left superior vena cava. Both superior venae cavae are fully mobilized. Great care is taken to avoid injury to the phrenic nerves, particularly on the left side. Adequate mobilization needs division of both internal thoracic veins as well as azygos and hemi-azygos veins (in the absence of concomitant azygos or hemi-azygos continuation of the inferior vena cava).
for cardiopulmonary bypass. The right superior vena cava is cannulated directly, the top of the cannula being located proximal to the entrance of the azygos vein in case of azygos continuation of the inferior vena cava. A small persistent left superior vena cava may by occluded temporarily; however, in most cases, a left superior vena cava should be cannulated directly; this is, of course, the case when there is hemi-azygos continuation of the inferior vena cava. The inferior vena cava should be cannulated as distally as possible after dissection of its transdiaphragmatic segment. When the hepatic veins drain as a single vessel, this vessel is cannulated directly after careful dissection. If the hepatic veins drain as two vessels, it may be necessary to use two cannulas or if one of the veins is small, the blood return can be controlled with an intracardiac sucker.
then cannulated separately after institution of cardiopulmonary bypass. With adequate venous cannulation, the transplantation procedure can be performed under conventional normothermic cardiopulmonary bypass. Using deep hypothermic circulatory arrest may however be mandatory, very rarely, in small infants with complex anatomy.
When pericardial adhesions due to previous operations limit access to the heart, the right atrium is cannulated with a single cannula; the systemic veins are
Management of persistent left superior vena cava The coronary sinus cannot be preserved during recipient heart excision. Therefore, the surgical problems are identical whether the anomalous left superior vena cava drains into the left atrium or into the right atrium via the coronary sinus. Simple ligation When the left superior vena cava is smaller than its right-sided counterpart and when there is an innominate vein, the left superior vena cava can usually be ligated and divided. However, a central 3
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578
Schematic 4. Anatomy in a patient with concomitant left superior vena cava draining into the left atrium, isolated drainage of the hepatic veins and anomalous right superior pulmonary vein draining into the right superior vena cava. The cardiac end of both superior venae cavae are oversewn (the right one above the entrance of the anomalous pulmonary vein). A pericardial patch is used to partition the atria into a right-sided compartment (receiving inferior vena cava and hepatic veins) and a leftsided compartment (receiving all pulmonary veins). Cardiac implantation is performed using bilateral atrial anastomoses followed by extracardiac reconstruction of the superior systemic venous pathway. RSVC s right superior vena cava – LSVC s left superior vena cava – IVC s inferior vena cava – HV s hepatic veins – RSPV s right superior pulmonary vein – RIPV s right inferior pulmonary vein.
Schematic 3. Extracardiac reconstruction of the superior systemic venous pathway. According to the available length of recipient left superior vena cava, various techniques can be used. Donor heart and vessels are shown in grey. (A) When the left superior vena cava is long, its free end is brought under the aortic arch and anastomosed end-to-side to the medial aspect of the recipient right SVC. (B) Alternatively, the free end of the left superior vena cava may be brought in front of the aorta and anastomosed end-to-end to an incision made in the tip of the donor right atrial appendage. (C) When the left superior vena cava is short (particularly when adequate mobilization is not possible because of associated hemi-azygos continuation of the inferior vena cava, the free end of the left superior vena cava is anastomosed end-to-end to the donor innominate vein and the recipient right superior vena cava is anastomosed to the confluence of the donor right internal jugular and subclavian veins.
4
venous line should be placed in the left internal jugular vein and the pressure should not rise above 15– 20 mmHg after temporary occlusion of the left superior vena cava. Reconstruction of normal systemic venous pathway Recipient heart excision is performed by dividing ascending aorta, pulmonary trunk, inferior vena cava, right and left superior venae cavae and left atrial wall (Schematics 1 and 2). Various techniques can then be used to reconstruct the continuity between the left superior vena cava and the right atrial compartment w2x (Schematic 3). Cardiac implantation is completed by anastomosing the donor left atrium with the recipient left atrium, performing separate superior and inferior cavo-caval anastomoses and reconstructing aorta and pulmonary trunk.
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578 monary venous connection, cardiac reimplantation may need both atrial septation and extracardiac reconstructive procedures. Schematic 4 gives an example of such a repair. Management of patients with previous atrial repair of transposition of the great arteries w3x The recipient heart is excised in the usual fashion by dividing the aorta and the pulmonary artery just above the semi-lunar valves and incising the atria just above the atrio-ventricular junction. The previous intra-atrial baffle material (atrial wall following Senning procedure or prosthetic material after Mustard operation) is completely excised. Both venae cavae are divided and their cardiac ends are oversewn. This leaves a single atrial cuff which receives both right and left pulmonary veins and which can be connected to the donor left atrium. Superior and inferior cavo-caval anastomoses are then performed (Schematic 5). Aortic and pulmonary arterial reconstruction is achieved after adequate mobilization of both the aortic arch and the main pulmonary arteries (including division of the ligamentum arteriosum).
Comments
Schematic 5. Orthotopic transplantation in a patient with previous atrial repair of transposition of the great arteries. (A) The recipient heart is excised in the usual fashion dividing the atrial wall close to the atrio-ventricular groove. The systemic venous baffle is completely resected at the same time. Both venae cavae are divided at their cardiac entry. (B) The cardiac ends of the venae cavae are oversewn. This leaves a single atrial cuff which receives both right and left pulmonary veins and which is connected to the left atrium of the graft. Direct cavocaval anastomoses are performed subsequently before aortic and pulmonary arterial reconstruction is achieved.
Management of associated systemic venous and pulmonary venous anomalies In patients with combined anomalies of systemic venous return and pul-
Orthotopic cardiac transplantation is feasible in all patients with anomalies of the systemic venous return (sometimes associated with anomalies of pulmonary venous connection). Complete preoperative evaluation with precise description of the venous returns, careful planning, adequate donor heart harvesting and innovative reimplantation techniques are, however, often necessary. Most problems can be solved by performing extracardiac cavo-caval anastomoses. Spatulated venous anastomoses should minimize the risk of stenosis of the anastomotic site. Should such a stenosis occur, it can be easily dealt with using current interventional dilation and stenting techniques w4x. Extracardiac reconstruction avoids the need for complex intra-atrial rerouting which carries a potential risk of late intra-atrial obstruction. In patients with associated anomalies of the pulmonary venous return, adequate atrial partitioning may be necessary followed by bilateral atrial anastomoses and extracardiac reconstruction of the systemic venous pathway. Despite an increased surgical complexity, it has been shown in various series of cardiac transplantation in congenital defects that the early risk of the procedure is not influenced by the presence of anomalies of the systemic and pulmonary venous connections w4–14x. 5
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578
References w1x De Leval M. Anomalies of the systemic venous return. Surgery for congenital heart defects. Grune and Stratton Ltd. 1983:253–260. w2x Reddy VM, McElhinney DB, Hanley FL. Correction of left superior vena cava draining to the left atrium using extracardiac techniques. Ann Thorac Surg 1997;63:1800–1802. w3x Chang AC, Wernovsky G, Wessel DL, Freed MD, Parness IA, Perry SB, O’Brien P, Van Praagh R, Hanley FL, Jonas RA, et al. Surgical management of late right ventricular failure after Mustard or Senning repair. Circulation 1992;86(5 Suppl): II140–149. w4x Larsen RL, Eguchi JH, Mulla NF, Johnston JK, Fitts J, Kuhn MA, Razzouk AJ, Chinnock RE, Bailey LL. Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy. Am J Cardiol 2002;89: 1275–1279. w5x Menkis AH, McKenzie FN, Novick RJ, Kostuk WJ, Pflugfelder PW, Goldbach M, Rosenberg H. Expanding applicability of transplantation after multiple prior palliative procedures. The Paediatric Heart Transplant Group. Ann Thorac Surg 1991;52:722–726. w6x Chartrand C, Guerin R, Kangah M, Stanley P. Pediatric heart transplantation: surgical considerations for congenital heart diseases. J Heart Transplant 1990;9:608–617. w7x Mayer JE Jr, Perry S, O’Brien P, Perez-Atayde A, Jonas RA, Castaneda AR, Parness IA. Orthotopic
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w8x
w9x
w10x
w11x
w12x
w13x
w14x
heart transplantation for complex congenital heart disease. J Thorac Cardiovasc Surg 1990;99:484– 492. Chartrand C. Pediatric cardiac transplantation despite atrial and venous return anomalies. Ann Thorac Surg 1991;52:716–721. Vouhe PR, Tamisier D, Le Bidois J, Sidi D, Mauriat P, Pouard P, Lefebvre D, Albanese SB, Khoury W, Kachaner J. Pediatric cardiac transplantation for congenital heart defects: surgical considerations and results. Ann Thorac Surg 1993;56:1239– 1247. Hasan A, Au J, Hamilton JR, Hunter S, Hilton CJ, Dark JH. Orthotopic heart transplantation for congenital heart disease. Technical considerations. Eur J Cardiothoracic Surg 1993;7:65– 70. Hsu DT, Quaegebeur JM, Michler RE, Smith CR, Rose EA, Kichuk MR, Gersony WM, Douglas JF, Addonizio LJ. Heart transplantation in children with congenital heart disease. J Am Coll Cardiol 1995;26:743–749. Carrel T, Neth J, Mohacsi P, Gallino A, Turina MI. Perioperative risk and long-term results of heart transplantation after previous cardiac operations. Ann Thorac Surg 1997;63:1133–1137. Chen JM, Davies RR, Mital SR, Mercando ML, Addonizio LJ, Pinney SP, Hsu DT, Lamour JM, Quaegebeur JM, Mosca RS. Trends and outcomes in transplantation for complex congenital heart disease: 1984 to 2004. Ann Thorac Surg 2004;78:1352–1361. Groetzner J, Reichart B, Roemer U, Reichel S, Kozlik-Feldmann R, Tiete A, Sachweh J, Netz H, Daebritz S. Cardiac transplantation in pediatric patients: fifteen-year experience of a single center. Ann Thorac Surg 2005;79:53–61.
Orthotopic heart transplantation for congenital heart defects: anomalies of the systemic venous return Olivier Raisky, Daniel Tamisier, Pascal R. Vouhe´* Department of Pediatric Cardiac Surgery, Groupe Hospitalier Necker – Enfants Malades, 149 rue de Se`vres, 75015 Paris, France Anomalies of the systemic venous return are frequently associated with other congenital heart defects. Some anomalies do not complicate really orthotopic heart transplantation (such as azygos continuation of the inferior vena cava). Other anomalies raise more difficulties; the most frequent one is persistent left superior vena cava draining into either the coronary sinus or the left atrium. Sometimes, the left superior vena cava can be ligated without untoward effect. In most cases, the left superior vena cava must be anastomosed to the right atrial compartment, preferably using extracardiac procedures. Most problems can be solved by harvesting extra lengths of donor superior vena cava and innominate vein. In rare patients, associated anomalies of the pulmonary venous return may require additional partitioning of the atria. The early risk of heart transplantation is probably not increased by the presence of such anomalies.
Keywords: Congenital heart defects; Anomalies of systemic venous return; Persistent left superior vena cava; Orthotopic heart transplantation Introduction
Anomalous return to the right atrium
Anomalies of the systemic venous return are frequently associated with other congenital cardiac defects. In situs solitus, a left superior vena cava connecting to the coronary sinus is common. In right isomerism, bilateral cavae connecting to bilateral morphologically right atria may occur. In left isomerism, multiple coexisting systemic venous anomalies are the rule.
Anomalous left superior vena cava connecting to the coronary sinus is common and occurs in 2–4% of all congenital cardiac defects. In approximatively 75% of the cases, there is hypoplasia or agenesis of the left innominate vein. The left superior vena cava originates at the confluence of the left internal jugular and subclavian veins, descends anterior to the hilus of the left lung and becomes continuous with the coronary sinus which is typically enlarged.
Anatomical considerations From a surgical stand-point, anomalies of the systemic venous return can be classified into five categories. * Corresponding author: Tel.: q33-1-44381867; fax: q33-144381911. E-mail: [email protected] 䉷 2006 European Association for Cardio-thoracic Surgery
Interrupted inferior vena cava occurs in 0.6% of patients with congenital heart defects. The hepatic and the prerenal segments of the inferior vena cava fail to fuse into a continuous channel. The prerenal segment of the interrupted inferior vena cava joins either the azygos vein and the right superior vena cava (azygos continuation of the inferior vena cava) or 1
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578 Table 1. Combined anomalies of systemic and pulmonary venous connections ● Anomalous systemic and pulmonary venous connection to the right atrium. – Left SVC to coronary sinus – right pulmonary vein(s) to right atrium. – Left SVC to coronary sinus – left pulmonary vein(s) to left SVC. – Left SVC to coronary sinus – partial or total anomalous pulmonary venous to coronary sinus. – Azygos continuation of IVC – right pulmonary vein(s) to right atrium. ● Anomalous systemic venous connection to left atrium and anomalous pulmonary venous connection. – Left SVC to left atrium – right pulmonary vein(s) to right atrium. – Left SVC to left atrium – anomalous pulmonary vein(s) to left SVC. – Anomalous systemic vein (right SVC or hepatic veins) to left atrium – anomalous pulmonary vein(s) to anomalous systemic vein.
the hemi azygos vein and a persistent left superior vena cava draining into the coronary sinus (hemi azygos continuation of the inferior vena cava).
Surgical techniques
Anomalous return to the left atrium
Precise preoperative evaluation and careful planning of the procedure are essential to solve the problems related to anomalies of the systemic venous return. Both systemic venous and pulmonary venous connections must be defined. This is usually best achieved by a combination of echocardiography, cardiac catheterization and multi slice CT scan.
The commonest anomaly is a left superior vena cava draining into the left atrium. The orifice of the left superior vena cava is situated in the left upper corner of the left atrium between the base of the left atrial appendage anteriorly and the left pulmonary veins posteriorly. Usually, there is a right superior vena cava and the innominate vein is absent. Rarely, there is no right superior vena cava. Typically, the coronary sinus is absent and the coronary veins drain individually into the atria. Other less common types of anomalous systemic return to the left atrium include drainage into the left atrium of the inferior vena cava, hepatic veins, or right superior vena cava. Anomalous return to both atria This includes a combination of the anomalies described previously. An azygos continuation of the inferior vena cava to the right superior vena cava and a left superior vena cava to the left atrium is one example.
General considerations
The technical principles of heart transplantation are twofold (1) to create a recipient left atrial cuff which receives all the pulmonary veins and is anastomosed to the donor left atrium (2) to achieve adequate systemic venous return either by direct cavo-caval anastomoses or by a combination of cavo-caval anastomoses and atrial anastomosis between the donor right atrium and a recipient right atrial cuff receiving some of the systemic veins. Some anomalies (for example azygos continuation of the inferior vena cava) do not complicate really heart transplantation which can be carried out using a conventional technique. Other anomalies raise more difficulties which can, however, all be solved by adequate planning of the procedure. Donor operation
Combined anomalous systemic and pulmonary venous return Multiple variants are possible. Table 1 is a list of such anomalies w1x. Actually, all combinations may probably be encountered. Surgically-induced anomalies These include intracardiac anomalies of systemic and pulmonary venous return secondary to atrial repair of transposition of the great arteries (Senning or Mustard procedure) and anomalies of the systemic return induced by various types of cavo-pulmonary connections. 2
According to the planned reimplantation procedures needed in the recipient, donor heart harvesting includes the superior vena cava and the innominate vein, most of the intrapericardial and transdiaphragmatic inferior vena cava and the pulmonary veins. Adequate partitioning of the veins needs collaboration with the surgical teams who eventually harvest the liver and the lungs. Recipient operation Cannulation for cardiopulmonary bypass The presence of anomalies of the systemic venous return may complicate the technique of venous cannulation
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578
Schematic 1. Cardiac anatomy in patients with persistent left superior vena cava (draining into the coronary sinus or the left atrium). The venae cavae are cannulated separately. The incisions performed for recipient heart excision are shown in red dotted lines. The left superior vena cava is transected at its entrance into the heart, near the base of the left atrial appendage. RSVC s right superior vena cava – LSVC s left superior vena cava – LA s left atrium – RA s right atrium – IVC s inferior vena cava.
Schematic 2. Appearance of the mediastium after heart excision in patients with persistent left superior vena cava. Both superior venae cavae are fully mobilized. Great care is taken to avoid injury to the phrenic nerves, particularly on the left side. Adequate mobilization needs division of both internal thoracic veins as well as azygos and hemi-azygos veins (in the absence of concomitant azygos or hemi-azygos continuation of the inferior vena cava).
for cardiopulmonary bypass. The right superior vena cava is cannulated directly, the top of the cannula being located proximal to the entrance of the azygos vein in case of azygos continuation of the inferior vena cava. A small persistent left superior vena cava may by occluded temporarily; however, in most cases, a left superior vena cava should be cannulated directly; this is, of course, the case when there is hemi-azygos continuation of the inferior vena cava. The inferior vena cava should be cannulated as distally as possible after dissection of its transdiaphragmatic segment. When the hepatic veins drain as a single vessel, this vessel is cannulated directly after careful dissection. If the hepatic veins drain as two vessels, it may be necessary to use two cannulas or if one of the veins is small, the blood return can be controlled with an intracardiac sucker.
then cannulated separately after institution of cardiopulmonary bypass. With adequate venous cannulation, the transplantation procedure can be performed under conventional normothermic cardiopulmonary bypass. Using deep hypothermic circulatory arrest may however be mandatory, very rarely, in small infants with complex anatomy.
When pericardial adhesions due to previous operations limit access to the heart, the right atrium is cannulated with a single cannula; the systemic veins are
Management of persistent left superior vena cava The coronary sinus cannot be preserved during recipient heart excision. Therefore, the surgical problems are identical whether the anomalous left superior vena cava drains into the left atrium or into the right atrium via the coronary sinus. Simple ligation When the left superior vena cava is smaller than its right-sided counterpart and when there is an innominate vein, the left superior vena cava can usually be ligated and divided. However, a central 3
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578
Schematic 4. Anatomy in a patient with concomitant left superior vena cava draining into the left atrium, isolated drainage of the hepatic veins and anomalous right superior pulmonary vein draining into the right superior vena cava. The cardiac end of both superior venae cavae are oversewn (the right one above the entrance of the anomalous pulmonary vein). A pericardial patch is used to partition the atria into a right-sided compartment (receiving inferior vena cava and hepatic veins) and a leftsided compartment (receiving all pulmonary veins). Cardiac implantation is performed using bilateral atrial anastomoses followed by extracardiac reconstruction of the superior systemic venous pathway. RSVC s right superior vena cava – LSVC s left superior vena cava – IVC s inferior vena cava – HV s hepatic veins – RSPV s right superior pulmonary vein – RIPV s right inferior pulmonary vein.
Schematic 3. Extracardiac reconstruction of the superior systemic venous pathway. According to the available length of recipient left superior vena cava, various techniques can be used. Donor heart and vessels are shown in grey. (A) When the left superior vena cava is long, its free end is brought under the aortic arch and anastomosed end-to-side to the medial aspect of the recipient right SVC. (B) Alternatively, the free end of the left superior vena cava may be brought in front of the aorta and anastomosed end-to-end to an incision made in the tip of the donor right atrial appendage. (C) When the left superior vena cava is short (particularly when adequate mobilization is not possible because of associated hemi-azygos continuation of the inferior vena cava, the free end of the left superior vena cava is anastomosed end-to-end to the donor innominate vein and the recipient right superior vena cava is anastomosed to the confluence of the donor right internal jugular and subclavian veins.
4
venous line should be placed in the left internal jugular vein and the pressure should not rise above 15– 20 mmHg after temporary occlusion of the left superior vena cava. Reconstruction of normal systemic venous pathway Recipient heart excision is performed by dividing ascending aorta, pulmonary trunk, inferior vena cava, right and left superior venae cavae and left atrial wall (Schematics 1 and 2). Various techniques can then be used to reconstruct the continuity between the left superior vena cava and the right atrial compartment w2x (Schematic 3). Cardiac implantation is completed by anastomosing the donor left atrium with the recipient left atrium, performing separate superior and inferior cavo-caval anastomoses and reconstructing aorta and pulmonary trunk.
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578 monary venous connection, cardiac reimplantation may need both atrial septation and extracardiac reconstructive procedures. Schematic 4 gives an example of such a repair. Management of patients with previous atrial repair of transposition of the great arteries w3x The recipient heart is excised in the usual fashion by dividing the aorta and the pulmonary artery just above the semi-lunar valves and incising the atria just above the atrio-ventricular junction. The previous intra-atrial baffle material (atrial wall following Senning procedure or prosthetic material after Mustard operation) is completely excised. Both venae cavae are divided and their cardiac ends are oversewn. This leaves a single atrial cuff which receives both right and left pulmonary veins and which can be connected to the donor left atrium. Superior and inferior cavo-caval anastomoses are then performed (Schematic 5). Aortic and pulmonary arterial reconstruction is achieved after adequate mobilization of both the aortic arch and the main pulmonary arteries (including division of the ligamentum arteriosum).
Comments
Schematic 5. Orthotopic transplantation in a patient with previous atrial repair of transposition of the great arteries. (A) The recipient heart is excised in the usual fashion dividing the atrial wall close to the atrio-ventricular groove. The systemic venous baffle is completely resected at the same time. Both venae cavae are divided at their cardiac entry. (B) The cardiac ends of the venae cavae are oversewn. This leaves a single atrial cuff which receives both right and left pulmonary veins and which is connected to the left atrium of the graft. Direct cavocaval anastomoses are performed subsequently before aortic and pulmonary arterial reconstruction is achieved.
Management of associated systemic venous and pulmonary venous anomalies In patients with combined anomalies of systemic venous return and pul-
Orthotopic cardiac transplantation is feasible in all patients with anomalies of the systemic venous return (sometimes associated with anomalies of pulmonary venous connection). Complete preoperative evaluation with precise description of the venous returns, careful planning, adequate donor heart harvesting and innovative reimplantation techniques are, however, often necessary. Most problems can be solved by performing extracardiac cavo-caval anastomoses. Spatulated venous anastomoses should minimize the risk of stenosis of the anastomotic site. Should such a stenosis occur, it can be easily dealt with using current interventional dilation and stenting techniques w4x. Extracardiac reconstruction avoids the need for complex intra-atrial rerouting which carries a potential risk of late intra-atrial obstruction. In patients with associated anomalies of the pulmonary venous return, adequate atrial partitioning may be necessary followed by bilateral atrial anastomoses and extracardiac reconstruction of the systemic venous pathway. Despite an increased surgical complexity, it has been shown in various series of cardiac transplantation in congenital defects that the early risk of the procedure is not influenced by the presence of anomalies of the systemic and pulmonary venous connections w4–14x. 5
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2005.001578
References w1x De Leval M. Anomalies of the systemic venous return. Surgery for congenital heart defects. Grune and Stratton Ltd. 1983:253–260. w2x Reddy VM, McElhinney DB, Hanley FL. Correction of left superior vena cava draining to the left atrium using extracardiac techniques. Ann Thorac Surg 1997;63:1800–1802. w3x Chang AC, Wernovsky G, Wessel DL, Freed MD, Parness IA, Perry SB, O’Brien P, Van Praagh R, Hanley FL, Jonas RA, et al. Surgical management of late right ventricular failure after Mustard or Senning repair. Circulation 1992;86(5 Suppl): II140–149. w4x Larsen RL, Eguchi JH, Mulla NF, Johnston JK, Fitts J, Kuhn MA, Razzouk AJ, Chinnock RE, Bailey LL. Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy. Am J Cardiol 2002;89: 1275–1279. w5x Menkis AH, McKenzie FN, Novick RJ, Kostuk WJ, Pflugfelder PW, Goldbach M, Rosenberg H. Expanding applicability of transplantation after multiple prior palliative procedures. The Paediatric Heart Transplant Group. Ann Thorac Surg 1991;52:722–726. w6x Chartrand C, Guerin R, Kangah M, Stanley P. Pediatric heart transplantation: surgical considerations for congenital heart diseases. J Heart Transplant 1990;9:608–617. w7x Mayer JE Jr, Perry S, O’Brien P, Perez-Atayde A, Jonas RA, Castaneda AR, Parness IA. Orthotopic
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heart transplantation for complex congenital heart disease. J Thorac Cardiovasc Surg 1990;99:484– 492. Chartrand C. Pediatric cardiac transplantation despite atrial and venous return anomalies. Ann Thorac Surg 1991;52:716–721. Vouhe PR, Tamisier D, Le Bidois J, Sidi D, Mauriat P, Pouard P, Lefebvre D, Albanese SB, Khoury W, Kachaner J. Pediatric cardiac transplantation for congenital heart defects: surgical considerations and results. Ann Thorac Surg 1993;56:1239– 1247. Hasan A, Au J, Hamilton JR, Hunter S, Hilton CJ, Dark JH. Orthotopic heart transplantation for congenital heart disease. Technical considerations. Eur J Cardiothoracic Surg 1993;7:65– 70. Hsu DT, Quaegebeur JM, Michler RE, Smith CR, Rose EA, Kichuk MR, Gersony WM, Douglas JF, Addonizio LJ. Heart transplantation in children with congenital heart disease. J Am Coll Cardiol 1995;26:743–749. Carrel T, Neth J, Mohacsi P, Gallino A, Turina MI. Perioperative risk and long-term results of heart transplantation after previous cardiac operations. Ann Thorac Surg 1997;63:1133–1137. Chen JM, Davies RR, Mital SR, Mercando ML, Addonizio LJ, Pinney SP, Hsu DT, Lamour JM, Quaegebeur JM, Mosca RS. Trends and outcomes in transplantation for complex congenital heart disease: 1984 to 2004. Ann Thorac Surg 2004;78:1352–1361. Groetzner J, Reichart B, Roemer U, Reichel S, Kozlik-Feldmann R, Tiete A, Sachweh J, Netz H, Daebritz S. Cardiac transplantation in pediatric patients: fifteen-year experience of a single center. Ann Thorac Surg 2005;79:53–61.
