HOW TO DO IT
Orthotopic Heart Transplantation in Patients With Persistent Left Superior Vena Cava: Bicaval and Biatrial Techniques Siyamek Neragi-Miandoab, MD, PhD, David Baran, MD, Alla Godelman, MD, and Daniel J. Goldstein, MD
Persistent left superior vena cava (LSVC) is the most common congenital venous abnormality. With the increasing number of children who survive into adulthood with congenital heart malformations, the recognition of persistent LSVC among patients with advanced heart failure is likely to rise. We present two cases of
orthotopic heart transplantation in the setting of LSVC successfully managed with biatrial and bicaval techniques.
ersistent left superior vena cava (LSVC) was first described in 1850 [1]. It has a prevalence of 0.5% in the general population and approximately 4% in patients with congenital heart disease [2]. During the embryonic period, the compressive effects of left lung and left atrium obliterate the LSVC. In these situations, the right side of the head and the right arm drain mainly through the innominate vein into the LSVC, which itself drains through an extremely dilated coronary sinus. In more than 90% of cases, the LSVC drains into the coronary sinus and then into the right atrium (RA) [3]. In 80% of cases, a right SVC is also present and empties into the RA, providing uncomplicated right heart access, which explains why LSVC is not more commonly identified. Mediastinal widening, abnormal positioning of the central venous catheter on a chest radiograph, and an ultrasonic signal-free space in the retrocardiac area are signs of a dilated coronary sinus associated with this malformation. An unusual scenario in which persistent LSVC is present is in situs inversus totalis with dextrocardia. The latter has long been regarded as a contraindication for thoracic transplantation, because reconstruction would need to accommodate normal donor organs [4].
cava (RSVC) as part of the transplant evaluation. An implantable cardioverter defibrillator had been introduced into the coronary sinus via the LSVC. No other congenital cardiac abnormalities were found. After midline sternotomy, cardiopulmonary bypass was established using an arterial cannula in the ascending aorta and inferior vena cava cannula for venous drainage. After decompression of the heart, the LSVC along with a large vertical vein draining into the coronary sinus was easily identified, cannulated, and incorporated into the venous limb of the bypass circuit. After cross clamping, cardiectomy was performed leaving cuffs of left and right atrium. The vertical vein connecting the LSVC to the coronary sinus (CS) was preserved because it ran on the posterior aspect of the left atrial cuff (Fig 1). The CS and its junction in the remaining right atrium were left intact during cardiectomy. The atrial cuffs of the recipient and donor were fashioned for atrial-to-atrial anastomoses. After removing the native heart, a biatrial anastomosis was performed. At the end of right atrial anastomosis, the patient had two coronary sinuses (CS); the recipient’s native CS draining the recipient’s LSVC and the donor’s CS draining the normal donor heart. The postoperative course remained uneventful. The patient was discharged home on the ninth postoperative day with the standard immunosuppressive therapy. On 2.5-year follow-up, the patient remains well without hospitalizations or major complications.
P
Technique Patient 1: Biatrial Technique A 58-year-old man with dilated cardiomyopathy and heart failure (ejection fraction of 10% to 15% and New York Heart Association class III/IV) was found to have left superior vena cava (LSVC) with absent right superior vena Accepted for publication July 1, 2013. Address correspondence to Dr Goldstein, Department of Cardiothoracic and Vascular Surgery, 3400 Bainbridge Ave, Bronx, NY 10467; e-mail: dgoldste@montefiore.org.
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
(Ann Thorac Surg 2014;97:1085–7) Ó 2014 by The Society of Thoracic Surgeons
Patient 2: Bicaval Technique The second patient was a 67-year-old man with known situs inversus totalis and idiopathic dilated cardiomyopathy. His condition was diagnosed at 19 years of age when he left his native country and immigrated to the United States. At 65 years of age, the patient became symptomatic and developed end-stage heart failure. A preoperative venous angiogram demonstrated LSVC without RSVC. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.07.033
FEATURE ARTICLES
Department of Cardiothoracic Surgery, Montefiore Medical Center, Bronx, New York; Division of Cardiology, Newark Beth Israel Medical Center, Newark, New Jersey; Department of Radiology, Montefiore Medical Center, Bronx, New York
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HOW TO DO IT NERAGI-MIANDOAB ET AL HEART TRANSPLANTATION AND LEFT SUPERIOR VENA CAVA
Ann Thorac Surg 2014;97:1085–7
Fig 1. LSVC draining into the RA through the CS. The CS was used to direct the blood from the LSVC to the RA. (A ¼ aorta; CS ¼ coronary sinus; IVC ¼ inferior vena cava; LA ¼ left atrium; LSVC ¼ left superior vena cava; PA ¼ pulmonary artery; RA ¼ right atrium; SVC ¼ superior vena cava.)
FEATURE ARTICLES
The patient was taken to the operating room. Following a standard sternotomy, typical CS anatomy was confirmed with an LSVC, midline inferior vena cava (IVC), and leftsided right atrium. There was no RSVC. Aortic and bicaval cannulation was performed. During dissection of heart, the LSVC was divided at its junction with the leftsided RA. The IVC, which was near the midline, was divided leaving a large patulous portion of the right atrium to allow easy reach to the donor’s IVC. The implantation of donor heart was started with left atrial anastomosis using a Prolene 3-0 suture in the usual fashion. Next, the redundant left-sided IVC cuff was anastomosed to the donor IVC in running fashion with minimal torsion at the
Fig 2. Oblique reformatted image from noncontrast computed tomography of the thorax. A conduit is extending from the LSVC to the RA appendage. (A ¼ aorta; AzV ¼ azygos vein; IVC ¼ inferior vena cava; LA ¼ left atrium; LSVC ¼ left superior vena cava; PA ¼ pulmonary artery; RA ¼ right atrium; RAA ¼ right atrial appendage.)
anastomosis. This was followed by end-to-end pulmonary artery and aortic anastomoses. To direct the blood from the LSVC to the normal donor heart, we constructed a pericardial baffle with a donor’s pericardium over a Hegar’s dilator, with a size matching the recipient’s SVC. The baffle was then anastomosed to LSVC in end-to-end fashion and brought anteriorly to the great vessels and anastomosed to the donor SVC in an end-to-end fashion (Fig 2). The patient was weaned from cardiopulmonary bypass without difficulty. He had an uneventful postoperative recovery, and the usual immunotherapy was instituted. He was given daily aspirin (325 mg) without coumadin. Posttransplant endomyocardial biopsies were performed
through the femoral route. His graft function remained stable for several years without evidence of baffle stenosis or closure. He developed progressive kidney disease and eventually required dialysis.
Comment Several approaches have been advanced for the management of LSVC at the time of transplantation. Deuse and Reitz [4] preserved the donor innominate vein to connect to the recipient’s LSVC in a retroaortic configuration in a case of situs inversus totalis undergoing heartlung transplantation. Length and frailty of the innominate vein can render this approach difficult, and it can be compressed by the greater vessels. Vricella and colleagues [5] described transplantation in 15 patients with situs inversus in which the donor SVC-innominate vein complex was anastomosed end-to-end to the LSVC across the greater vessels [5]—the same route used by us with our pericardial baffle. This experience was limited mostly to infants and children and in the authors’ judgment is less likely to be useful in adults in whom the innominate is frail and the length of conduit necessary to reach the LSVC precludes a tension-free anastomosis. Rabago and colleagues [6] described a bicaval anastomosis in a recipient with LSVC. The authors performed a bicaval anastomosis isolating the patient’s native CS to drain the LSVC into the IVC in a manner similar to our approach. Michler and Sandhu [7] described the use of complex systemic and pulmonary venous intracardiac rerouting in a pediatric patient with visceroatrial situs inversus. Reproducible surgical techniques exist to achieve bicaval or biatrial anastomoses in patients undergoing transplantation in the presence of a LSVC. The biatrial
HOW TO DO IT NERAGI-MIANDOAB ET AL HEART TRANSPLANTATION AND LEFT SUPERIOR VENA CAVA
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anastomosis preserves the natural LSVC-to–coronary sinus drainage and avoids the use of intracardiac or extracardiac conduits; however, the documented superiority of bicaval technique in terms of postoperative arrhythmia, tricuspid valve geometry, and mortality [8] suggests that efforts aimed at a bicaval anastomosis even in LSVC situations may be warranted.
References 1. Marshall J. On the development of great anterior veins in man and mammalia including an account of certain remnants of fetal structure found in the adult, a comparative view of these great veins in the different mammalia and an analysis of their occasional peculiarities in the human subject. Philos Trans Roy Soc London 1850;(140):133–54. 2. Josloff RK, Kukora JS. Central venous catheterization via persistent left superior vena cava. Am Surg 1995;61:781–3. 3. Higgs AG, Paris S, Potter F. Discovery of left-sided superior vena cava during central venous catheterization. Br J Anaesth 1998;81:260–1. 4. Deuse T, Reitz BA. Heart-lung transplantation in situs inversus totalis. Ann Thorac Surg 2009;88:1002–3. 5. Vricella LA, Razzouk AJ, Gundry SR, Larsen RL, Kuhn MA, Bailey LL. Heart transplantation in infants and children with situs inversus. J Thorac Cardiovasc Surg 1998;116:82–9. 6. R abago G, Martín-Trenor A, L opez-Coronado JL, Macias A, Cosín-Sales J, Herreros JM. Bicaval anastomosis in a heart transplant recipient with left superior vena cava. Ann Thorac Surg 2002;74:1242–4. 7. Michler RE, Sandhu AA. Novel approach for orthotopic heart transplantation in visceroatrial situs inversus. Ann Thorac Surg 1995;60:194–7. 8. Davies RR, Russo MJ, Morgan JA, Sorabella RA, Naka Y, Chen JM. Standard versus bicaval techniques for orthotopic heart transplantation: analysis of the United Network for Organ Sharing database. J Thorac Cardiovasc Surg 2010;140: 700–8, 708.e1–2.
FEATURE ARTICLES
Ann Thorac Surg 2014;97:1085–7
Orthotopic Heart Transplantation in Patients With Persistent Left Superior Vena Cava: Bicaval and Biatrial Techniques Siyamek Neragi-Miandoab, MD, PhD, David Baran, MD, Alla Godelman, MD, and Daniel J. Goldstein, MD
Persistent left superior vena cava (LSVC) is the most common congenital venous abnormality. With the increasing number of children who survive into adulthood with congenital heart malformations, the recognition of persistent LSVC among patients with advanced heart failure is likely to rise. We present two cases of
orthotopic heart transplantation in the setting of LSVC successfully managed with biatrial and bicaval techniques.
ersistent left superior vena cava (LSVC) was first described in 1850 [1]. It has a prevalence of 0.5% in the general population and approximately 4% in patients with congenital heart disease [2]. During the embryonic period, the compressive effects of left lung and left atrium obliterate the LSVC. In these situations, the right side of the head and the right arm drain mainly through the innominate vein into the LSVC, which itself drains through an extremely dilated coronary sinus. In more than 90% of cases, the LSVC drains into the coronary sinus and then into the right atrium (RA) [3]. In 80% of cases, a right SVC is also present and empties into the RA, providing uncomplicated right heart access, which explains why LSVC is not more commonly identified. Mediastinal widening, abnormal positioning of the central venous catheter on a chest radiograph, and an ultrasonic signal-free space in the retrocardiac area are signs of a dilated coronary sinus associated with this malformation. An unusual scenario in which persistent LSVC is present is in situs inversus totalis with dextrocardia. The latter has long been regarded as a contraindication for thoracic transplantation, because reconstruction would need to accommodate normal donor organs [4].
cava (RSVC) as part of the transplant evaluation. An implantable cardioverter defibrillator had been introduced into the coronary sinus via the LSVC. No other congenital cardiac abnormalities were found. After midline sternotomy, cardiopulmonary bypass was established using an arterial cannula in the ascending aorta and inferior vena cava cannula for venous drainage. After decompression of the heart, the LSVC along with a large vertical vein draining into the coronary sinus was easily identified, cannulated, and incorporated into the venous limb of the bypass circuit. After cross clamping, cardiectomy was performed leaving cuffs of left and right atrium. The vertical vein connecting the LSVC to the coronary sinus (CS) was preserved because it ran on the posterior aspect of the left atrial cuff (Fig 1). The CS and its junction in the remaining right atrium were left intact during cardiectomy. The atrial cuffs of the recipient and donor were fashioned for atrial-to-atrial anastomoses. After removing the native heart, a biatrial anastomosis was performed. At the end of right atrial anastomosis, the patient had two coronary sinuses (CS); the recipient’s native CS draining the recipient’s LSVC and the donor’s CS draining the normal donor heart. The postoperative course remained uneventful. The patient was discharged home on the ninth postoperative day with the standard immunosuppressive therapy. On 2.5-year follow-up, the patient remains well without hospitalizations or major complications.
P
Technique Patient 1: Biatrial Technique A 58-year-old man with dilated cardiomyopathy and heart failure (ejection fraction of 10% to 15% and New York Heart Association class III/IV) was found to have left superior vena cava (LSVC) with absent right superior vena Accepted for publication July 1, 2013. Address correspondence to Dr Goldstein, Department of Cardiothoracic and Vascular Surgery, 3400 Bainbridge Ave, Bronx, NY 10467; e-mail: dgoldste@montefiore.org.
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
(Ann Thorac Surg 2014;97:1085–7) Ó 2014 by The Society of Thoracic Surgeons
Patient 2: Bicaval Technique The second patient was a 67-year-old man with known situs inversus totalis and idiopathic dilated cardiomyopathy. His condition was diagnosed at 19 years of age when he left his native country and immigrated to the United States. At 65 years of age, the patient became symptomatic and developed end-stage heart failure. A preoperative venous angiogram demonstrated LSVC without RSVC. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.07.033
FEATURE ARTICLES
Department of Cardiothoracic Surgery, Montefiore Medical Center, Bronx, New York; Division of Cardiology, Newark Beth Israel Medical Center, Newark, New Jersey; Department of Radiology, Montefiore Medical Center, Bronx, New York
1086
HOW TO DO IT NERAGI-MIANDOAB ET AL HEART TRANSPLANTATION AND LEFT SUPERIOR VENA CAVA
Ann Thorac Surg 2014;97:1085–7
Fig 1. LSVC draining into the RA through the CS. The CS was used to direct the blood from the LSVC to the RA. (A ¼ aorta; CS ¼ coronary sinus; IVC ¼ inferior vena cava; LA ¼ left atrium; LSVC ¼ left superior vena cava; PA ¼ pulmonary artery; RA ¼ right atrium; SVC ¼ superior vena cava.)
FEATURE ARTICLES
The patient was taken to the operating room. Following a standard sternotomy, typical CS anatomy was confirmed with an LSVC, midline inferior vena cava (IVC), and leftsided right atrium. There was no RSVC. Aortic and bicaval cannulation was performed. During dissection of heart, the LSVC was divided at its junction with the leftsided RA. The IVC, which was near the midline, was divided leaving a large patulous portion of the right atrium to allow easy reach to the donor’s IVC. The implantation of donor heart was started with left atrial anastomosis using a Prolene 3-0 suture in the usual fashion. Next, the redundant left-sided IVC cuff was anastomosed to the donor IVC in running fashion with minimal torsion at the
Fig 2. Oblique reformatted image from noncontrast computed tomography of the thorax. A conduit is extending from the LSVC to the RA appendage. (A ¼ aorta; AzV ¼ azygos vein; IVC ¼ inferior vena cava; LA ¼ left atrium; LSVC ¼ left superior vena cava; PA ¼ pulmonary artery; RA ¼ right atrium; RAA ¼ right atrial appendage.)
anastomosis. This was followed by end-to-end pulmonary artery and aortic anastomoses. To direct the blood from the LSVC to the normal donor heart, we constructed a pericardial baffle with a donor’s pericardium over a Hegar’s dilator, with a size matching the recipient’s SVC. The baffle was then anastomosed to LSVC in end-to-end fashion and brought anteriorly to the great vessels and anastomosed to the donor SVC in an end-to-end fashion (Fig 2). The patient was weaned from cardiopulmonary bypass without difficulty. He had an uneventful postoperative recovery, and the usual immunotherapy was instituted. He was given daily aspirin (325 mg) without coumadin. Posttransplant endomyocardial biopsies were performed
through the femoral route. His graft function remained stable for several years without evidence of baffle stenosis or closure. He developed progressive kidney disease and eventually required dialysis.
Comment Several approaches have been advanced for the management of LSVC at the time of transplantation. Deuse and Reitz [4] preserved the donor innominate vein to connect to the recipient’s LSVC in a retroaortic configuration in a case of situs inversus totalis undergoing heartlung transplantation. Length and frailty of the innominate vein can render this approach difficult, and it can be compressed by the greater vessels. Vricella and colleagues [5] described transplantation in 15 patients with situs inversus in which the donor SVC-innominate vein complex was anastomosed end-to-end to the LSVC across the greater vessels [5]—the same route used by us with our pericardial baffle. This experience was limited mostly to infants and children and in the authors’ judgment is less likely to be useful in adults in whom the innominate is frail and the length of conduit necessary to reach the LSVC precludes a tension-free anastomosis. Rabago and colleagues [6] described a bicaval anastomosis in a recipient with LSVC. The authors performed a bicaval anastomosis isolating the patient’s native CS to drain the LSVC into the IVC in a manner similar to our approach. Michler and Sandhu [7] described the use of complex systemic and pulmonary venous intracardiac rerouting in a pediatric patient with visceroatrial situs inversus. Reproducible surgical techniques exist to achieve bicaval or biatrial anastomoses in patients undergoing transplantation in the presence of a LSVC. The biatrial
HOW TO DO IT NERAGI-MIANDOAB ET AL HEART TRANSPLANTATION AND LEFT SUPERIOR VENA CAVA
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anastomosis preserves the natural LSVC-to–coronary sinus drainage and avoids the use of intracardiac or extracardiac conduits; however, the documented superiority of bicaval technique in terms of postoperative arrhythmia, tricuspid valve geometry, and mortality [8] suggests that efforts aimed at a bicaval anastomosis even in LSVC situations may be warranted.
References 1. Marshall J. On the development of great anterior veins in man and mammalia including an account of certain remnants of fetal structure found in the adult, a comparative view of these great veins in the different mammalia and an analysis of their occasional peculiarities in the human subject. Philos Trans Roy Soc London 1850;(140):133–54. 2. Josloff RK, Kukora JS. Central venous catheterization via persistent left superior vena cava. Am Surg 1995;61:781–3. 3. Higgs AG, Paris S, Potter F. Discovery of left-sided superior vena cava during central venous catheterization. Br J Anaesth 1998;81:260–1. 4. Deuse T, Reitz BA. Heart-lung transplantation in situs inversus totalis. Ann Thorac Surg 2009;88:1002–3. 5. Vricella LA, Razzouk AJ, Gundry SR, Larsen RL, Kuhn MA, Bailey LL. Heart transplantation in infants and children with situs inversus. J Thorac Cardiovasc Surg 1998;116:82–9. 6. R abago G, Martín-Trenor A, L opez-Coronado JL, Macias A, Cosín-Sales J, Herreros JM. Bicaval anastomosis in a heart transplant recipient with left superior vena cava. Ann Thorac Surg 2002;74:1242–4. 7. Michler RE, Sandhu AA. Novel approach for orthotopic heart transplantation in visceroatrial situs inversus. Ann Thorac Surg 1995;60:194–7. 8. Davies RR, Russo MJ, Morgan JA, Sorabella RA, Naka Y, Chen JM. Standard versus bicaval techniques for orthotopic heart transplantation: analysis of the United Network for Organ Sharing database. J Thorac Cardiovasc Surg 2010;140: 700–8, 708.e1–2.
FEATURE ARTICLES
Ann Thorac Surg 2014;97:1085–7
