- Email: [email protected]
Superior Vena Cava Reconstruction for Treatment of Chylothorax Resulting From Thrombosis of Superior Vena Cava in Young Infants
Superior Vena Cava Reconstruction for Treatment of Chylothorax Resulting From Thrombosis of Superior Vena Cava in Young Infants
CONGENITAL HEART
T. K. Susheel Kumar, MD, Saradha Subramanian, MD, Shyam Sathanandam, MD, John Alexander, MD, Mohammed Ali, MD, and Christopher J. Knott-Craig, MD Departments of Pediatric Cardiothoracic Surgery and Pediatric Cardiology, Le Bonheur Children’s Hospital and University of Tennessee, Memphis, Tennessee
Background. Thrombosis and occlusion of the superior vena cava (SVC) can cause massive chylothorax resulting in significant morbidity and mortality among young infants. Medical therapy is often unsuccessful. We report a new surgical technique that entails open thrombectomy and reconstruction of the SVC and innominate vein to treat this condition. Methods. The charts of 4 consecutive infants with chylothoraces refractory to conservative management were reviewed. The operations were performed on cardiopulmonary bypass without myocardial arrest. The SVC and innominate veins were incised open, and thrombectomy was performed. This was followed by homograft patch reconstruction of both the veins. Results. The infants were aged between 5 weeks to 4 months and had an average weight of 4 kg. All of them had hypoalbuminemia and evidence of hypercoagulable state. After surgical intervention, 3 had complete relief of
SVC obstruction. Two of the 3 patients had complete resolution of chylous effusion, and the third patient had a significant decrease in chest tube drainage (70%) by the end of 1 week. The fourth patient had recurrence of high drainage after an initial improvement, and a subsequent angiogram demonstrated stenosis of the SVC without thrombosis. The chest tube drainage finally resolved after balloon angioplasty. Conclusions. Thrombotic occlusion of the SVC can result in chylothorax that is often not amenable to medical therapy. This is associated with significant loss of proteins and hypercoagulable state. A complete surgical relief of SVC obstruction by open thrombectomy and venoplasty can result in dramatic decrease in chylous output.
C
Material and Methods
ritically ill neonates and young infants are often at high risk for vascular thrombosis from a variety of reasons including indwelling central venous catheters, sepsis, and inherited and acquired coagulopathies [1–3]. Thrombosis of the superior vena cava (SVC) can lead to SVC syndrome and chylothorax secondary to elevated venous pressure. Chylothorax that results from elevated central venous pressure is of the high-output type and responds poorly to medical management [4]. Surgical interventions, including thoracic duct ligation and pleurodesis, have been described for the treatment of SVC obstruction in neonates and young infants with variable results [5, 6]. Stenting of the SVC has been more successful in older children and adults [7]. We describe a novel surgical technique for extensive thrombosis of the SVC and its proximal branches among young infants. This entails an open thrombectomy with reconstruction of the SVC and innominate veins.
Accepted for publication June 1, 2015. Address correspondence to Dr Kumar, Pediatric Cardiothoracic Surgery, Le Bonheur Children’s Hospital, University of Tennessee, 848 Adams St, Memphis, TN 38103; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
(Ann Thorac Surg 2015;100:1432–6) Ó 2015 by The Society of Thoracic Surgeons
Patients The University of Tennessee Health Science Center Institutional Review Board approved this study. A retrospective chart review of 4 infants who had complete thrombotic occlusion of the SVC as documented by color Doppler ultrasound and computed tomography scan and who subsequently underwent open surgical thrombectomy with venous reconstruction was completed. The 4 infants (3 boys, 1 girl) were between 5 weeks to 4 months of age with the following diagnoses: D-loop transposition of the great vessels after undergoing an arterial switch operation as a term newborn, bronchiolitis with respiratory failure, extreme prematurity (24 weeks’ gestational age), and persistent pulmonary hypertension of the newborn. The cause of thrombosis was related to the central venous catheter in all 4 patients. The central venous catheters were placed in the internal jugular vein in 3 patients and the subclavian vein in 1 patient. Thrombosis developed despite giving heparin at 0.5 U/ mL with total parenteral nutrition through the central catheters. Unfortunately no details of either the make or type of central catheters are available. All had significant chylous drainage (30 to 350 mL $ kg1 $ day1). The 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.06.021
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mean duration of chylothorax was 40 days. Treatment with systemic therapeutic heparin or enoxaparin had been unsuccessful in eliminating the thrombus. Octreotide and elimination of enteral long-chain fatty acids in the diet or parenteral nutrition had little to no effect on chylous drainage. Transcatheter thrombectomy was attempted in 1 patient and was unsuccessful in eliminating both thrombus and drainage. This patient with pulmonary hypertension was treated with sildenafil but without any apparent benefits on chest tube output.
Surgical Technique
Results The demographics and preoperative details of the 4 patients are summarized in Table 1. The mean age was 11 weeks, and the mean weight was 4 kg. Surgical intervention was performed after an average duration of 40 days of chylous drainage. All patients exhibited features of SVC syndrome clinically. Three of the 4 patients were assessed for hypercoagulable state during the period of high chylous drainage. All 3 had low levels of protein C (25% to 47%; normal, 74% to 161%), protein S (27% to 50%; normal, 58% to 114%), and antithrombin III (20% to 60%,y; normal, 84% to 125%). All 4 had hypoalbuminemia (1.2 to 1.7 g/dL; normal, 2.6 to 4.4 g/dL). Prealbumin levels were low in the 3 who were tested (5 to 10.3 mg/dL; normal, 17 to
CONGENITAL HEART
All patients were managed surgically in the same manner. After sternotomy, the patient was placed on cardiopulmonary bypass through aortic and inferior vena cava cannulation. Normothermia was maintained. A longitudinal incision was initially made in the innominate vein, and the thrombus was evacuated using an endarterectomy (Freer) elevator. This was followed by passage of a no. 4 Fogarty balloon-tipped catheter into the left subclavian and internal jugular veins. This was done until there was adequate back-bleeding. The SVC was then incised vertically such that the two venotomies met at a right angle to each other. The SVC incision was carried proximally onto the junction of the SVC with the right atrium with a C clamp placed across the right atrium to allow the incision to be extended into the right atrium. Myocardial arrest was avoided. An endarterectomy of the SVC was then performed, as was a thrombectomy of the right-sided veins with the Fogarty catheter (Fig 1). Once adequate backflow was achieved throughout, the innominate vein was augmented with a patch of pulmonary homograft using 7-0 Prolene (Ethicon, Somerville, NJ) for the anastomosis. A second homograft patch was used to augment the SVC, and the two patches were anastomosed together in an end-to-side fashion (Fig 2). To maintain central venous access without the need for indwelling catheters in the brachiocephalic system, a 3.5F doublelumen catheter was passed subcutaneously and placed into the right atrium through a separate pursestring suture. All patients were then weaned from cardiopulmonary bypass without inotropic agents.
Fig 1. Technique of surgical repair. Both innominate vein and superior vena cava have been incised open longitudinally. After thromboendarterectomy, a Fogarty balloon catheter is passed up the branches to evacuate additional clots.
36 mg/dL). All patients had severe hypogammaglobulinemia (<7 to 36 mg/dL; normal, 190 to 650 mg/dL). Of the 4 patients who underwent surgery, 3 had complete relief of SVC obstruction as documented by color Doppler. In 2 of these 3 patients, chylous effusions completely resolved by the end of 1 week (Table 2). In the third, chest tube drainage of chylous fluid decreased from 350 mL $ kg1 $ day1 to 100 mL $ kg1 $ day1 (reduction of 71%) at the end of the first week and subsequently stopped by the end of 3 weeks. The fourth patient initially improved but had recurrence of high drainage (250 mL $ kg1 $ day1) at the end of 1 week. An angiogram demonstrated residual SVC stenosis without thrombosis on postoperative day 15. This was successfully dilated with a balloon in the catheterization laboratory. The chest tube output showed a steady decline and finally resolved in a month. All patients survived to 30 days. Among the 4 patients, we had 2 long-term survivors (beyond 1 year). The first patient died 4 months later of sepsis. The second patient died 9 months later of complications from a respiratory tract infection. We did not face complications such as wound infections, renal failure, or neurologic injury.
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KUMAR ET AL SUPERIOR VENA CAVA RECONSTRUCTION
Fig 2. Homograft patch augmentation of innominate vein and superior vena cava. Separate patches are used to augment the vessels.
Comment Superior vena cava thrombosis is a major complication that can dramatically increase morbidity and carries greater than 60% mortality [8]. We describe 4 patients who had significant chylous drainage secondary to SVC thrombosis. Our approach included a period of medical management to attempt to reduce the volume of chyle losses and relieve SVC obstruction. We used similar dietary modifications that have been well described by others [9], including avoidance of enteral feeds, total parenteral nutrition, and enteral medium-chain triglyceride diet. In addition, octreotide was used in an attempt to further reduce chyle loss [10, 11]. Heparin or enoxaparin was administered to try to relieve the obstruction caused by the thrombus. Despite this approach, the chylous drainage continued unabated and increased in
some. In 3 (patients 1, 3, and 4), the decision to move forward with surgical therapy was because of the massive volume of output that was unresponsive to medical therapy of 28, 25, and 15 days’ duration, respectively. In the fourth patient, medical therapy had been unsuccessful for more than 3 months before being referred to us for a surgical option. It has been our experience that delaying surgery in the presence of high chylous outputs increases the morbidity of patients. They remain dependent on mechanical ventilation and require large amounts of fluid to prevent intravascular volume depletion, including the use of albumin, fresh frozen plasma, cryoprecipitate, and crystalloid solutions. Despite constant correction of intravascular volume depletion, many of these infants develop massive third spacing of fluids because of low oncotic pressure as a result of lost plasma proteins. Most need central access for medications and volume resuscitation. The risk of infection associated with central access and mechanical ventilation is significant because of the acquired immune deficiency from lost immunoglobulin. In the patient with significant chylothorax, anticoagulation is rarely successful [12]. This is likely secondary to loss of antithrombin III. Antithrombin III acts primarily on thrombin as a potent inhibitor of the coagulation cascade. Heparin binds to antithrombin III and increases the rate of reactions of antithrombin III by up to 2,000-fold [13]. Deficiency of antithrombin greatly reduces the effect of heparin [14], which is why anticoagulation is rarely successful in eliminating thrombus when a large chylothorax is present. Chylothorax secondary to SVC thrombosis is unlikely to respond to medical or conservative management. When chylothorax is caused by high venous pressure, the volume of output is high and the duration is longer than when it is caused by injury to the thoracic duct [9]. Wang and colleagues [4] have described conservative medical management of chylothorax with resolution and development of collateral vessels after 46 days. The treatment algorithm proposed by Beghetti and associates [9] suggest 4 weeks of medical therapy before undertaking surgical options. McGrath and coworkers [11] proposed a less conservative model for the treatment of chylothorax in which surgical options would be considered if drainage persisted for more than 2 weeks or if output was greater than 100 mL $ kg1 $ day1 while on medical therapy for chylothorax. We support the algorithm for earlier surgery
Table 1. Demographics and Preoperative Characteristics of Patients Patient
Age (wk)
Sex
Weight (kg)
1 2
8 16
M M
2.7 5.3
3 4
17 5
F M
4.5 3.5
D-TGA
Diagnosis Prematurity Primary pulmonary hypertension Bronchiolitis D-TGA after arterial switch operation
¼ D-loop transposition of the great arteries;
Protein C
Protein S
Antithrombin III
Albumin (g/dL)
Prealbumin (mg/dL)
IgG (mg/dL)
45% NT
40% NT
34% NT
1.5 1.7
10.3 NT
<7 <7
25% 47%
27% 50%
20% 60%
1.7 1.2
7 5
7 36
IgG ¼ immunoglobulin G;
NT ¼ not tested.
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Table 2. Outcomes of Patients After Surgical Intervention at End of 1 Week Patient 1 2 3 4
Duration of Chylothorax (days)
Preoperative Chest Drainage (mL $ kg1 $ day1)
Postoperative Chest Drainage (mL $ kg1 $ day1)
28 25 92 15
210 30 350 90
250 0 100 0
atrium through a double 5.0 Prolene pursestring suture placed over the right atrium. The tip of the catheter usually lies at the junction of the right atrium and inferior vena cava. We have had no bleeding issues after removal of the transthoracic catheter. Thrombosis of the SVC can result in massive chylothorax that is often refractory to medical therapy. It is associated with hypercoagulable state (protein C, protein S, and antithrombin III deficiency), poor nutritional state, and immunocompromised state. Surgical intervention in the form of SVC open thrombectomy and reconstruction using homograft patch can yield dramatic relief from such effusions, especially if done early enough. It is important to achieve complete relief of SVC obstruction for good results.
References 1. Mitchell L, Male C. Central venous line-related thrombosis in children with congenital heart disease: diagnosis, prevalence, outcomes, and prevention. Prog Pediatr Cardiol 2005;21:9–16. 2. Shah PS, Shah N. Heparin-bonded catheters for prolonging the patency of central venous catheters in children. Cochrane Database Syst Rev 2014;(2):CD005983. 3. Tulika S. Thrombosis in neonates and children. East J Med 2009;14:36–45. 4. Wang IJ, Lu FL, Chang CI, Wang JK. Conservative treatment in an infant with superior vena cava syndrome after cardiac surgery. J Formos Med Assoc 2002;101:352–4. 5. Van Biervliet S, De Waele K, Vande velde S, et al. Thoracic duct ligation as treatment of chylothorax due to vena cava superior thrombosis. Acta Clin Belg 2011;66:221–2. 6. Hsu HF, Chou YH, Wang CR, Wu SC. Catheter-related superior vena cava syndrome complicated by chylothorax in a premature infant. Chang Gung Med J 2003;26:782–6. 7. Hannan RL, Zabinsky JA, Hernandez A, Zahn EM, Burke RP. Hybrid treatment of superior vena cava syndrome in a child. Ann Thorac Surg 2009;88:277–8. 8. Swaniker F, Fonkalsrud EW. Superior and inferior vena caval occlusion in infants receiving total parenteral nutrition. Am Surg 1995;61:877–81. 9. Beghetti M, La Scala G, Belli D, Bugmann P, Kalangos A, Le Coultre C. Etiology and management of pediatric chylothorax. J Pediatr 2000;136:653–8. 10. Siu SL, Lam DS. Spontaneous neonatal chylothorax treated with octreotide. J Paediatr Child Health 2006;42:65–7. 11. McGrath EE, Blades Z, Anderson PB. Chylothorax: aetiology, diagnosis and therapeutic options. Respir Med 2010;104:1–8. 12. Van Veldhuizen PJ, Taylor S. Chylothorax: a complication of a left subclavian vein thrombosis. Am J Clin Oncol 1996;19: 99–101. 13. Bjork I, Lindahl U. Mechanism of the anticoagulant action of heparin. Mol Cell Biochem 1982;48:161–82. 14. Anderson JA, Weitz JI. Hypercoagulable states. Crit Care Clin 2011;27:933–52.
CONGENITAL HEART
in the presence of very large outputs. A more conservative approach would be reasonable in the presence of low-volume chylothorax or chylothorax from a different cause. Thoracic duct ligation with or without pleurodesis is an option when conservative management fails. Some report excellent success with this approach [15]. Although in some patients this approach may improve or eliminate the chylothorax [16], it will not address the primary problem, which is SVC thrombosis with SVC syndrome. Transcatheter therapy has been described in older children and adults [17]. Superior vena cava perforation during transcatheter treatment has been reported in a 10-year-old [7] and was considered to be too high risk in a neonate by Wang and colleagues [4]. In our series, this was attempted in 1 patient without any success, and we did not experience any complications. Reconstruction of the SVC for nonmalignant venous occlusive disease has been well described in adults [18, 19]. Saphenous vein graft, human allograft, and expanded polytetrafluoroethylene tubes have been used for reconstruction of the SVC. However, SVC reconstruction for thrombotic occlusion has not been described in infants. We decided to extend the concept of SVC reconstruction to infants and children with venous occlusion. We believe that unless the SVC and its branches are completely cleared of all clots, the chylous drainage does not decrease. Extensive thrombectomy and surgical reconstruction of the SVC and innominate vein using pulmonary homograft was performed in these 4 neonates. We were able to eliminate chylous drainage in 3 patients. The fourth patient needed a balloon intervention for residual stenosis of the SVC, after which there was improvement. It is important to achieve as much patency as possible during the operation. Residual clot or stenosis should be ruled out if there are no tangible benefits. We also believe that the operation should be undertaken early before the thrombus becomes organized and fibrous for complete removal. The use of transthoracic catheters instead of catheters in the SVC has greatly mitigated the incidence of SVC thrombosis and chylothorax at our institution. We use a 3.5F double-lumen umbilical venous catheter as a transthoracic catheter. This is usually placed at the end of surgery. A wide-bore needle is used to tunnel the catheter through the skin and subcutaneous tissue over the left chest wall to reach the suprasternal space. The catheter is then pulled into the anterior mediastinum and tailored to reach the level of the diaphragm. The catheter is then introduced into the right
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CONGENITAL HEART
15. Paul S, Altorki NK, Port JL, Stiles BM, Lee PC. Surgical management of chylothorax. Thorac Cardiovasc Surg 2009;57:226–8. 16. Curci MR, Dibbins AW. Bilateral chylothorax in a newborn. J Pediatr Surg 1980;15:663–5. 17. Rizvi AZ, Kalra M, Bjarnason H, Bower TC, Schleck C, Gloviczki P. Benign superior vena cava syndrome: stenting is now the first line of treatment. J Vasc Surg 2008;47:372–80.
Ann Thorac Surg 2015;100:1432–6
18. Gloviczki P, Pairolero PC, Toomey BJ, et al. Reconstruction of large veins for nonmalignant venous occlusive disease. J Vasc Surg 1992;16:750–61. 19. Kalra M, Gloviczki P, Andrews JC, et al. Open surgical and endovascular treatment of superior vena cava syndrome caused by nonmalignant disease. J Vasc Surg 2003;38: 215–23.
The Society of Thoracic Surgeons: Fifty-Second Annual Meeting Mark your calendar for the 52nd Annual Meeting of The Society of Thoracic Surgeons (STS) to be held at the Phoenix Convention Center in Phoenix, Arizona, January 23-27, 2016. The STS Annual Meeting offers you a chance to meet the experts, network with colleagues from around the world, and participate in a dynamic learning experience. This preeminent educational event is open to all physicians, residents, fellows, research scientists, perfusionists, physician assistants, nurses, and others interested in cardiothoracic surgery. Meeting participants will have the opportunity to attend traditional abstract presentations, invited lectures, surgical forums, Early Riser Sessions, Surgical Motion Pictures, and procedural hands-on courses. Parallel sessions on Monday and Tuesday will focus on specific subspecialty interests. The STS Annual Meeting offers more translational science than any other cardiothoracic surgery conference!
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
An advance program with information about housing and registration will be mailed to STS members this fall. Nonmembers may contact the Society to receive a copy of the printed advance program; however, detailed up-todate meeting information will be available on the STS website at www.sts.org/annualmeeting. I hope to see you in Phoenix. Keith S. Naunheim, MD Secretary The Society of Thoracic Surgeons 633 N Saint Clair St, 23rd Floor Chicago, IL 60611-3658 Telephone: (312) 202-5800 Fax: (312) 202-5801 E-mail: [email protected] Website: www.sts.org
Ann Thorac Surg 2015;100:1436
0003-4975/$36.00
CONGENITAL HEART
T. K. Susheel Kumar, MD, Saradha Subramanian, MD, Shyam Sathanandam, MD, John Alexander, MD, Mohammed Ali, MD, and Christopher J. Knott-Craig, MD Departments of Pediatric Cardiothoracic Surgery and Pediatric Cardiology, Le Bonheur Children’s Hospital and University of Tennessee, Memphis, Tennessee
Background. Thrombosis and occlusion of the superior vena cava (SVC) can cause massive chylothorax resulting in significant morbidity and mortality among young infants. Medical therapy is often unsuccessful. We report a new surgical technique that entails open thrombectomy and reconstruction of the SVC and innominate vein to treat this condition. Methods. The charts of 4 consecutive infants with chylothoraces refractory to conservative management were reviewed. The operations were performed on cardiopulmonary bypass without myocardial arrest. The SVC and innominate veins were incised open, and thrombectomy was performed. This was followed by homograft patch reconstruction of both the veins. Results. The infants were aged between 5 weeks to 4 months and had an average weight of 4 kg. All of them had hypoalbuminemia and evidence of hypercoagulable state. After surgical intervention, 3 had complete relief of
SVC obstruction. Two of the 3 patients had complete resolution of chylous effusion, and the third patient had a significant decrease in chest tube drainage (70%) by the end of 1 week. The fourth patient had recurrence of high drainage after an initial improvement, and a subsequent angiogram demonstrated stenosis of the SVC without thrombosis. The chest tube drainage finally resolved after balloon angioplasty. Conclusions. Thrombotic occlusion of the SVC can result in chylothorax that is often not amenable to medical therapy. This is associated with significant loss of proteins and hypercoagulable state. A complete surgical relief of SVC obstruction by open thrombectomy and venoplasty can result in dramatic decrease in chylous output.
C
Material and Methods
ritically ill neonates and young infants are often at high risk for vascular thrombosis from a variety of reasons including indwelling central venous catheters, sepsis, and inherited and acquired coagulopathies [1–3]. Thrombosis of the superior vena cava (SVC) can lead to SVC syndrome and chylothorax secondary to elevated venous pressure. Chylothorax that results from elevated central venous pressure is of the high-output type and responds poorly to medical management [4]. Surgical interventions, including thoracic duct ligation and pleurodesis, have been described for the treatment of SVC obstruction in neonates and young infants with variable results [5, 6]. Stenting of the SVC has been more successful in older children and adults [7]. We describe a novel surgical technique for extensive thrombosis of the SVC and its proximal branches among young infants. This entails an open thrombectomy with reconstruction of the SVC and innominate veins.
Accepted for publication June 1, 2015. Address correspondence to Dr Kumar, Pediatric Cardiothoracic Surgery, Le Bonheur Children’s Hospital, University of Tennessee, 848 Adams St, Memphis, TN 38103; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
(Ann Thorac Surg 2015;100:1432–6) Ó 2015 by The Society of Thoracic Surgeons
Patients The University of Tennessee Health Science Center Institutional Review Board approved this study. A retrospective chart review of 4 infants who had complete thrombotic occlusion of the SVC as documented by color Doppler ultrasound and computed tomography scan and who subsequently underwent open surgical thrombectomy with venous reconstruction was completed. The 4 infants (3 boys, 1 girl) were between 5 weeks to 4 months of age with the following diagnoses: D-loop transposition of the great vessels after undergoing an arterial switch operation as a term newborn, bronchiolitis with respiratory failure, extreme prematurity (24 weeks’ gestational age), and persistent pulmonary hypertension of the newborn. The cause of thrombosis was related to the central venous catheter in all 4 patients. The central venous catheters were placed in the internal jugular vein in 3 patients and the subclavian vein in 1 patient. Thrombosis developed despite giving heparin at 0.5 U/ mL with total parenteral nutrition through the central catheters. Unfortunately no details of either the make or type of central catheters are available. All had significant chylous drainage (30 to 350 mL $ kg1 $ day1). The 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.06.021
Ann Thorac Surg 2015;100:1432–6
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mean duration of chylothorax was 40 days. Treatment with systemic therapeutic heparin or enoxaparin had been unsuccessful in eliminating the thrombus. Octreotide and elimination of enteral long-chain fatty acids in the diet or parenteral nutrition had little to no effect on chylous drainage. Transcatheter thrombectomy was attempted in 1 patient and was unsuccessful in eliminating both thrombus and drainage. This patient with pulmonary hypertension was treated with sildenafil but without any apparent benefits on chest tube output.
Surgical Technique
Results The demographics and preoperative details of the 4 patients are summarized in Table 1. The mean age was 11 weeks, and the mean weight was 4 kg. Surgical intervention was performed after an average duration of 40 days of chylous drainage. All patients exhibited features of SVC syndrome clinically. Three of the 4 patients were assessed for hypercoagulable state during the period of high chylous drainage. All 3 had low levels of protein C (25% to 47%; normal, 74% to 161%), protein S (27% to 50%; normal, 58% to 114%), and antithrombin III (20% to 60%,y; normal, 84% to 125%). All 4 had hypoalbuminemia (1.2 to 1.7 g/dL; normal, 2.6 to 4.4 g/dL). Prealbumin levels were low in the 3 who were tested (5 to 10.3 mg/dL; normal, 17 to
CONGENITAL HEART
All patients were managed surgically in the same manner. After sternotomy, the patient was placed on cardiopulmonary bypass through aortic and inferior vena cava cannulation. Normothermia was maintained. A longitudinal incision was initially made in the innominate vein, and the thrombus was evacuated using an endarterectomy (Freer) elevator. This was followed by passage of a no. 4 Fogarty balloon-tipped catheter into the left subclavian and internal jugular veins. This was done until there was adequate back-bleeding. The SVC was then incised vertically such that the two venotomies met at a right angle to each other. The SVC incision was carried proximally onto the junction of the SVC with the right atrium with a C clamp placed across the right atrium to allow the incision to be extended into the right atrium. Myocardial arrest was avoided. An endarterectomy of the SVC was then performed, as was a thrombectomy of the right-sided veins with the Fogarty catheter (Fig 1). Once adequate backflow was achieved throughout, the innominate vein was augmented with a patch of pulmonary homograft using 7-0 Prolene (Ethicon, Somerville, NJ) for the anastomosis. A second homograft patch was used to augment the SVC, and the two patches were anastomosed together in an end-to-side fashion (Fig 2). To maintain central venous access without the need for indwelling catheters in the brachiocephalic system, a 3.5F doublelumen catheter was passed subcutaneously and placed into the right atrium through a separate pursestring suture. All patients were then weaned from cardiopulmonary bypass without inotropic agents.
Fig 1. Technique of surgical repair. Both innominate vein and superior vena cava have been incised open longitudinally. After thromboendarterectomy, a Fogarty balloon catheter is passed up the branches to evacuate additional clots.
36 mg/dL). All patients had severe hypogammaglobulinemia (<7 to 36 mg/dL; normal, 190 to 650 mg/dL). Of the 4 patients who underwent surgery, 3 had complete relief of SVC obstruction as documented by color Doppler. In 2 of these 3 patients, chylous effusions completely resolved by the end of 1 week (Table 2). In the third, chest tube drainage of chylous fluid decreased from 350 mL $ kg1 $ day1 to 100 mL $ kg1 $ day1 (reduction of 71%) at the end of the first week and subsequently stopped by the end of 3 weeks. The fourth patient initially improved but had recurrence of high drainage (250 mL $ kg1 $ day1) at the end of 1 week. An angiogram demonstrated residual SVC stenosis without thrombosis on postoperative day 15. This was successfully dilated with a balloon in the catheterization laboratory. The chest tube output showed a steady decline and finally resolved in a month. All patients survived to 30 days. Among the 4 patients, we had 2 long-term survivors (beyond 1 year). The first patient died 4 months later of sepsis. The second patient died 9 months later of complications from a respiratory tract infection. We did not face complications such as wound infections, renal failure, or neurologic injury.
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Fig 2. Homograft patch augmentation of innominate vein and superior vena cava. Separate patches are used to augment the vessels.
Comment Superior vena cava thrombosis is a major complication that can dramatically increase morbidity and carries greater than 60% mortality [8]. We describe 4 patients who had significant chylous drainage secondary to SVC thrombosis. Our approach included a period of medical management to attempt to reduce the volume of chyle losses and relieve SVC obstruction. We used similar dietary modifications that have been well described by others [9], including avoidance of enteral feeds, total parenteral nutrition, and enteral medium-chain triglyceride diet. In addition, octreotide was used in an attempt to further reduce chyle loss [10, 11]. Heparin or enoxaparin was administered to try to relieve the obstruction caused by the thrombus. Despite this approach, the chylous drainage continued unabated and increased in
some. In 3 (patients 1, 3, and 4), the decision to move forward with surgical therapy was because of the massive volume of output that was unresponsive to medical therapy of 28, 25, and 15 days’ duration, respectively. In the fourth patient, medical therapy had been unsuccessful for more than 3 months before being referred to us for a surgical option. It has been our experience that delaying surgery in the presence of high chylous outputs increases the morbidity of patients. They remain dependent on mechanical ventilation and require large amounts of fluid to prevent intravascular volume depletion, including the use of albumin, fresh frozen plasma, cryoprecipitate, and crystalloid solutions. Despite constant correction of intravascular volume depletion, many of these infants develop massive third spacing of fluids because of low oncotic pressure as a result of lost plasma proteins. Most need central access for medications and volume resuscitation. The risk of infection associated with central access and mechanical ventilation is significant because of the acquired immune deficiency from lost immunoglobulin. In the patient with significant chylothorax, anticoagulation is rarely successful [12]. This is likely secondary to loss of antithrombin III. Antithrombin III acts primarily on thrombin as a potent inhibitor of the coagulation cascade. Heparin binds to antithrombin III and increases the rate of reactions of antithrombin III by up to 2,000-fold [13]. Deficiency of antithrombin greatly reduces the effect of heparin [14], which is why anticoagulation is rarely successful in eliminating thrombus when a large chylothorax is present. Chylothorax secondary to SVC thrombosis is unlikely to respond to medical or conservative management. When chylothorax is caused by high venous pressure, the volume of output is high and the duration is longer than when it is caused by injury to the thoracic duct [9]. Wang and colleagues [4] have described conservative medical management of chylothorax with resolution and development of collateral vessels after 46 days. The treatment algorithm proposed by Beghetti and associates [9] suggest 4 weeks of medical therapy before undertaking surgical options. McGrath and coworkers [11] proposed a less conservative model for the treatment of chylothorax in which surgical options would be considered if drainage persisted for more than 2 weeks or if output was greater than 100 mL $ kg1 $ day1 while on medical therapy for chylothorax. We support the algorithm for earlier surgery
Table 1. Demographics and Preoperative Characteristics of Patients Patient
Age (wk)
Sex
Weight (kg)
1 2
8 16
M M
2.7 5.3
3 4
17 5
F M
4.5 3.5
D-TGA
Diagnosis Prematurity Primary pulmonary hypertension Bronchiolitis D-TGA after arterial switch operation
¼ D-loop transposition of the great arteries;
Protein C
Protein S
Antithrombin III
Albumin (g/dL)
Prealbumin (mg/dL)
IgG (mg/dL)
45% NT
40% NT
34% NT
1.5 1.7
10.3 NT
<7 <7
25% 47%
27% 50%
20% 60%
1.7 1.2
7 5
7 36
IgG ¼ immunoglobulin G;
NT ¼ not tested.
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Table 2. Outcomes of Patients After Surgical Intervention at End of 1 Week Patient 1 2 3 4
Duration of Chylothorax (days)
Preoperative Chest Drainage (mL $ kg1 $ day1)
Postoperative Chest Drainage (mL $ kg1 $ day1)
28 25 92 15
210 30 350 90
250 0 100 0
atrium through a double 5.0 Prolene pursestring suture placed over the right atrium. The tip of the catheter usually lies at the junction of the right atrium and inferior vena cava. We have had no bleeding issues after removal of the transthoracic catheter. Thrombosis of the SVC can result in massive chylothorax that is often refractory to medical therapy. It is associated with hypercoagulable state (protein C, protein S, and antithrombin III deficiency), poor nutritional state, and immunocompromised state. Surgical intervention in the form of SVC open thrombectomy and reconstruction using homograft patch can yield dramatic relief from such effusions, especially if done early enough. It is important to achieve complete relief of SVC obstruction for good results.
References 1. Mitchell L, Male C. Central venous line-related thrombosis in children with congenital heart disease: diagnosis, prevalence, outcomes, and prevention. Prog Pediatr Cardiol 2005;21:9–16. 2. Shah PS, Shah N. Heparin-bonded catheters for prolonging the patency of central venous catheters in children. Cochrane Database Syst Rev 2014;(2):CD005983. 3. Tulika S. Thrombosis in neonates and children. East J Med 2009;14:36–45. 4. Wang IJ, Lu FL, Chang CI, Wang JK. Conservative treatment in an infant with superior vena cava syndrome after cardiac surgery. J Formos Med Assoc 2002;101:352–4. 5. Van Biervliet S, De Waele K, Vande velde S, et al. Thoracic duct ligation as treatment of chylothorax due to vena cava superior thrombosis. Acta Clin Belg 2011;66:221–2. 6. Hsu HF, Chou YH, Wang CR, Wu SC. Catheter-related superior vena cava syndrome complicated by chylothorax in a premature infant. Chang Gung Med J 2003;26:782–6. 7. Hannan RL, Zabinsky JA, Hernandez A, Zahn EM, Burke RP. Hybrid treatment of superior vena cava syndrome in a child. Ann Thorac Surg 2009;88:277–8. 8. Swaniker F, Fonkalsrud EW. Superior and inferior vena caval occlusion in infants receiving total parenteral nutrition. Am Surg 1995;61:877–81. 9. Beghetti M, La Scala G, Belli D, Bugmann P, Kalangos A, Le Coultre C. Etiology and management of pediatric chylothorax. J Pediatr 2000;136:653–8. 10. Siu SL, Lam DS. Spontaneous neonatal chylothorax treated with octreotide. J Paediatr Child Health 2006;42:65–7. 11. McGrath EE, Blades Z, Anderson PB. Chylothorax: aetiology, diagnosis and therapeutic options. Respir Med 2010;104:1–8. 12. Van Veldhuizen PJ, Taylor S. Chylothorax: a complication of a left subclavian vein thrombosis. Am J Clin Oncol 1996;19: 99–101. 13. Bjork I, Lindahl U. Mechanism of the anticoagulant action of heparin. Mol Cell Biochem 1982;48:161–82. 14. Anderson JA, Weitz JI. Hypercoagulable states. Crit Care Clin 2011;27:933–52.
CONGENITAL HEART
in the presence of very large outputs. A more conservative approach would be reasonable in the presence of low-volume chylothorax or chylothorax from a different cause. Thoracic duct ligation with or without pleurodesis is an option when conservative management fails. Some report excellent success with this approach [15]. Although in some patients this approach may improve or eliminate the chylothorax [16], it will not address the primary problem, which is SVC thrombosis with SVC syndrome. Transcatheter therapy has been described in older children and adults [17]. Superior vena cava perforation during transcatheter treatment has been reported in a 10-year-old [7] and was considered to be too high risk in a neonate by Wang and colleagues [4]. In our series, this was attempted in 1 patient without any success, and we did not experience any complications. Reconstruction of the SVC for nonmalignant venous occlusive disease has been well described in adults [18, 19]. Saphenous vein graft, human allograft, and expanded polytetrafluoroethylene tubes have been used for reconstruction of the SVC. However, SVC reconstruction for thrombotic occlusion has not been described in infants. We decided to extend the concept of SVC reconstruction to infants and children with venous occlusion. We believe that unless the SVC and its branches are completely cleared of all clots, the chylous drainage does not decrease. Extensive thrombectomy and surgical reconstruction of the SVC and innominate vein using pulmonary homograft was performed in these 4 neonates. We were able to eliminate chylous drainage in 3 patients. The fourth patient needed a balloon intervention for residual stenosis of the SVC, after which there was improvement. It is important to achieve as much patency as possible during the operation. Residual clot or stenosis should be ruled out if there are no tangible benefits. We also believe that the operation should be undertaken early before the thrombus becomes organized and fibrous for complete removal. The use of transthoracic catheters instead of catheters in the SVC has greatly mitigated the incidence of SVC thrombosis and chylothorax at our institution. We use a 3.5F double-lumen umbilical venous catheter as a transthoracic catheter. This is usually placed at the end of surgery. A wide-bore needle is used to tunnel the catheter through the skin and subcutaneous tissue over the left chest wall to reach the suprasternal space. The catheter is then pulled into the anterior mediastinum and tailored to reach the level of the diaphragm. The catheter is then introduced into the right
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15. Paul S, Altorki NK, Port JL, Stiles BM, Lee PC. Surgical management of chylothorax. Thorac Cardiovasc Surg 2009;57:226–8. 16. Curci MR, Dibbins AW. Bilateral chylothorax in a newborn. J Pediatr Surg 1980;15:663–5. 17. Rizvi AZ, Kalra M, Bjarnason H, Bower TC, Schleck C, Gloviczki P. Benign superior vena cava syndrome: stenting is now the first line of treatment. J Vasc Surg 2008;47:372–80.
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18. Gloviczki P, Pairolero PC, Toomey BJ, et al. Reconstruction of large veins for nonmalignant venous occlusive disease. J Vasc Surg 1992;16:750–61. 19. Kalra M, Gloviczki P, Andrews JC, et al. Open surgical and endovascular treatment of superior vena cava syndrome caused by nonmalignant disease. J Vasc Surg 2003;38: 215–23.
The Society of Thoracic Surgeons: Fifty-Second Annual Meeting Mark your calendar for the 52nd Annual Meeting of The Society of Thoracic Surgeons (STS) to be held at the Phoenix Convention Center in Phoenix, Arizona, January 23-27, 2016. The STS Annual Meeting offers you a chance to meet the experts, network with colleagues from around the world, and participate in a dynamic learning experience. This preeminent educational event is open to all physicians, residents, fellows, research scientists, perfusionists, physician assistants, nurses, and others interested in cardiothoracic surgery. Meeting participants will have the opportunity to attend traditional abstract presentations, invited lectures, surgical forums, Early Riser Sessions, Surgical Motion Pictures, and procedural hands-on courses. Parallel sessions on Monday and Tuesday will focus on specific subspecialty interests. The STS Annual Meeting offers more translational science than any other cardiothoracic surgery conference!
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
An advance program with information about housing and registration will be mailed to STS members this fall. Nonmembers may contact the Society to receive a copy of the printed advance program; however, detailed up-todate meeting information will be available on the STS website at www.sts.org/annualmeeting. I hope to see you in Phoenix. Keith S. Naunheim, MD Secretary The Society of Thoracic Surgeons 633 N Saint Clair St, 23rd Floor Chicago, IL 60611-3658 Telephone: (312) 202-5800 Fax: (312) 202-5801 E-mail: [email protected] Website: www.sts.org
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0003-4975/$36.00