- Email: [email protected]
Palliating Severe Arteriovenous Fistulae Using Absorbable Pulmonary Artery Bands
Palliating Severe Arteriovenous Fistulae Using Absorbable Pulmonary Artery Bands Kurt R. Schumacher, MD, Albert Rocchini, MD, and Richard G. Ohye, MD Division of Pediatric Cardiology and Division of Pediatric Cardiovascular Surgery, University of Michigan School of Medicine, Ann Arbor, Michigan
We report the use of an absorbable pulmonary artery band to limit postoperative cyanosis due to severe pulmonary arteriovenous fistulae in a 5-year-old girl with post-Fontan palliation takedown to a two-ventricle repair. At 1-year postoperatively, her fistulae had nearly resolved with no distortion of her pulmonary artery anatomy. This case demonstrates a novel approach to limiting cyanosis while pulmonary arteriovenous fistulae resolved after redirection of hepatic blood flow to the affected lung. (Ann Thorac Surg 2010;89:1301–3) © 2010 by The Society of Thoracic Surgeons
P
ulmonary arteriovenous fistulae (PAVF) formation is known to develop in patients who have undergone a Fontan palliation [1]. In patients who have undergone a Fontan procedure and have hepatic venous flow preferentially to one lung, PAVF formation in the opposite lung has been reported [2, 3]. Multiple authors have hypothesized that an absence of hepatic venous blood or “hepatic factor” in the pulmonary circulation leads to formation of PAVF [2, 3]. This report provides evidence in support of the role of hepatic venous blood flow in PAVF formation and describes a novel approach to PAVF palliation. A 5-year-old girl was referred for a second opinion with diagnoses of heterotaxy, mildly unbalanced leftward atrioventricular septal defect, double-outlet right ventricle, and pulmonary stenosis, status-post Fontan palliation. Her pulmonary veins drained into the right atrium. Her systemic venous drainage consisted of bilateral superior vena cava with an interrupted inferior vena cava with azygous-continuation to the left-sided superior vena cava. At another institution, she underwent an atrial septectomy and bilateral bidirectional Glenn anastomoses, followed by an extracardiac Fontan procedure with baffling of her hepatic veins into the right pulmonary artery (PA). After her Fontan procedure, she continued to have cyanosis with systemic oxygen saturations in the high 70% range. Cardiac catheterization demonstrated severe,
Accepted for publication Aug 31, 2009. Address correspondence to Dr Schumacher, Pediatric Cardiology, University of Michigan, 1500 E Medical Center Dr, L1242 Women’s/Box0204, Ann Arbor, MI 48109; e-mail: [email protected].
© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
CASE REPORT SCHUMACHER ET AL PALLIATING SEVERE ARTERIOVENOUS FISTULAE
1301
left-sided PAVF (Fig 1). The outside hospital recommended a left pneumonectomy. At our institution, it was believed (based on her technique of Fontan reconstruction and an angiogram suggesting little mixing between her PAs) that the lack of “hepatic factor” to her left lung was responsible for the development of PAVF. There were no other apparent correctable lesions (such as stenosis between the branch PAs) that were amenable to surgical repair. A long discussion was undertaken with the patient’s mother outlining the risks and benefits of the possible approaches (summarized in Table 1). The decision was made to proceed with a Fontan takedown to a two-ventricle repair. Cardiopulmonary bypass with standard arterial cannulation and venous cannulae to her superior vena cava and hepatic veins was instituted. The extracardiac conduit was resected leaving a 2-cm cuff of the conduit on the right PA and hepatic veins. Examination of the internal anatomy revealed a complete atrioventricular septal defect with approximately two thirds of the common atrioventricular valve dedicated to the left ventricle and ipsilateral pulmonary venous return. The right ventricular volume was smaller than had been suggested by echocardiogram and catheterization. Multiple trabeculations were divided, and the muscle was resected to increase ventricular size. A standard two-patch technique was used for atrioventricular septal defect repair, with the exception that the superior most portion of the ventricular septal defect patch was not sewn to the crest of the defect for the later intracardiac double-outlet right ventricle repair. The atrial patch was constructed to baffle the right pulmonary veins to the left atrium. The branch PAs were divided just distal to the Glenn anastomoses, and the proximal ends were oversewn, leaving the central portion of the PA to serve as the “innominate vein.” A right ventriculotomy was made. The ventricular septal defect was enlarged superiorly and anteriorly as there was no outlet extension. A portion of stretch polytetrafluoroethylene tube graft was opened longitudinally and was used to form an intraventricular tunnel from the ventricular septal defect to the aorta. A 22-mm cryopreserved pulmonary allograft was bicuspidized to 16 mm due to a lack of an appropriately sized graft. Anastomosing the allograft left PA to the native left PA and using a ringed 12-mm polytetrafluoroethylene graft to reach the right PA established right ventricle to PA continuity. Systemic venous drainage was re-established by sewing the cuffs of polytetrafluoroethylene graft from the extracardiac conduit on the hepatic veins and native proximal right PA (now “innominate vein”) to the right atrium. Total cardiopulmonary bypass time was 302 minutes, and cross-clamp time was 145 minutes. The patient tolerated the procedure well, but postoperatively she continued to have cyanosis with systemic oxygen saturations of 60% to 75%, and she required significant ventilatory support. Cardiac catheterization re-demonstrated diffuse left-sided PAVF and significant improvement in saturation with balloon occlusion of the 0003-4975/10/$36.00 doi:10.1016/j.athoracsur.2009.08.077
FEATURE ARTICLES
Ann Thorac Surg 2010;89:1301–3
1302
CASE REPORT SCHUMACHER ET AL PALLIATING SEVERE ARTERIOVENOUS FISTULAE
Ann Thorac Surg 2010;89:1301–3
Fig 1. (A) Angiogram of the extracardiac conduit Fontan with preferential filling of the right pulmonary artery. (B) Left upper lobe pulmonary arteriovenous fistulae (PAVF). (C) Medial left-lower lobe PAVF. (D) Lateral leftlower lobe PAVF.
FEATURE ARTICLES
left PA. A left PA band was believed to be potentially beneficial. The patient returned to the operating room and had the placement of an absorbable polyglactin 910 (Vicryl, Ethicon, Somerville, NJ) mesh left PA band. Saturation before application of the band was 70%. The diameter of the band was based on a desire to see a rise in oxygen saturation, while minimizing the gradient to allow for good perfusion of the left lung and adequate delivery of “hepatic factor.” After band placement, saturation improved to 96% with a measured 15 mm Hg trans-band gradient. Postoperatively, she maintained systemic oxygen saturation in the 90% range on room air. She was discharged home on postoperative day 15. At her 1-year follow-up, the family reported no cyanosis, dyspnea, or exercise intolerance. Oxygen saturations were 97% to 99%. Cardiac catheterization demonstrated
resolution of the left-sided PAVF with only one small pulmonary arteriovenous fistula remaining (Fig 2). Her left PA had no structural abnormalities and no pressure gradient across the banded area.
Comment Formation of PAVF in post-Fontan patients is a significant morbidity with limited treatment options. Several authors have noted resolution of PAVF once hepatic venous flow is directed to the PA [2, 3]. Our patient also demonstrated PAVF resolution after redirection of hepatic blood flow to the affected lung. After redirection of hepatic venous flow to the PA, patients continue to have cyanosis while the PAVF resolved [2, 3]. Our patient represents a unique approach to improving cyanosis in patients with resolving unilateral PAVF. Several authors have reported their use of absorb-
Table 1. Summary of Proposed Options Procedure
Advantages
Disadvantages ● ●
●
Lowest risk Likely to deliver “hepatic factor” to left lung Intermediate risk
●
Normal physiology
● ●
Arteriovenous Shunt/Fistula Fontan revision
● ●
Two-ventricle repair
● ●
Volume overload Uncertain long-term durability Uncertain delivery of “hepatic factor” to left lung Long-term risks of Fontan Highest risk Need for future right ventricle-to-pulmonary artery conduit replacement
Ann Thorac Surg 2010;89:1301–3
CASE REPORT SCHUMACHER ET AL PALLIATING SEVERE ARTERIOVENOUS FISTULAE
1303
had complete reabsorption of the PA band with no surgery required for PA reconstruction. Using an absorbable PA band to restrict blood flow to a lung affected by PAVF has not been reported. This may immediately improve systemic oxygen saturations by limiting the right-to-left shunt through the unilateral PAVF. Also, this may obviate the need for a second surgery to remove the PA band once PAVF have resolved. In our case, the use of an absorbable PA band did not cause any distortion of PA anatomy. We expect this report will encourage further usage and study of absorbable PA bands.
Fig 2. Angiogram of the reconstructed pulmonary artery (PA) demonstrating normal blood flow to the left PA with no notable pulmonary arteriovenous fistulae.
able PA bands in patients with congenital heart disease [4 – 6]. Patients reported include ventricular septal defect [4, 5], atrioventricular septal defect [4], and coarctation of the aorta with ventricular septal defect [5, 6]. All patients
1. Cloutier A, Ash J, Smallhorn J, Williams WG, Trussler GA, Rowe RD, Rabinovitch M. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae. Circulation 1985;72:471–9. 2. Ichikawa H, Fukushima N, Ono M, et al. Resolution of pulmonary arteriovenous fistula by redirection of hepatic venous blood. Ann Thoracic Surg 2004;77:1825–7. 3. Kim S-J, Bae E-J, Lee J-Y, Lim H-G, Lee C, Lee C-H. Inclusion of hepatic venous drainage in patients with pulmonary arteriovenous fistulas. Ann Thoracic Surg 2009;87:548 –53. 4. Gutierrez de Loma J, Ferreiros Mur M, Castilla Moreno M, Garcia Pena R, Gonzalez de Vega N. Reabsorbable banding: our initial experience. Rev Esp Cardiol 1991;44:677–9. 5. Peek GJ, Arsiwala SS, Chan KC, Hickey MS. Absorbable pulmonary artery band. Ann Thoracic Surg 1997;64:539 – 41. 6. Bonnet D, Patkai J, Tamisier D, Kachaner J, Vouhe P, Sidi D. A new strategy for the surgical treatment of aortic coarctation associated with ventricular septal defect in infants using an absorbable pulmonary artery band. JACC 1999;34:866 –70.
FEATURE ARTICLES
References
We report the use of an absorbable pulmonary artery band to limit postoperative cyanosis due to severe pulmonary arteriovenous fistulae in a 5-year-old girl with post-Fontan palliation takedown to a two-ventricle repair. At 1-year postoperatively, her fistulae had nearly resolved with no distortion of her pulmonary artery anatomy. This case demonstrates a novel approach to limiting cyanosis while pulmonary arteriovenous fistulae resolved after redirection of hepatic blood flow to the affected lung. (Ann Thorac Surg 2010;89:1301–3) © 2010 by The Society of Thoracic Surgeons
P
ulmonary arteriovenous fistulae (PAVF) formation is known to develop in patients who have undergone a Fontan palliation [1]. In patients who have undergone a Fontan procedure and have hepatic venous flow preferentially to one lung, PAVF formation in the opposite lung has been reported [2, 3]. Multiple authors have hypothesized that an absence of hepatic venous blood or “hepatic factor” in the pulmonary circulation leads to formation of PAVF [2, 3]. This report provides evidence in support of the role of hepatic venous blood flow in PAVF formation and describes a novel approach to PAVF palliation. A 5-year-old girl was referred for a second opinion with diagnoses of heterotaxy, mildly unbalanced leftward atrioventricular septal defect, double-outlet right ventricle, and pulmonary stenosis, status-post Fontan palliation. Her pulmonary veins drained into the right atrium. Her systemic venous drainage consisted of bilateral superior vena cava with an interrupted inferior vena cava with azygous-continuation to the left-sided superior vena cava. At another institution, she underwent an atrial septectomy and bilateral bidirectional Glenn anastomoses, followed by an extracardiac Fontan procedure with baffling of her hepatic veins into the right pulmonary artery (PA). After her Fontan procedure, she continued to have cyanosis with systemic oxygen saturations in the high 70% range. Cardiac catheterization demonstrated severe,
Accepted for publication Aug 31, 2009. Address correspondence to Dr Schumacher, Pediatric Cardiology, University of Michigan, 1500 E Medical Center Dr, L1242 Women’s/Box0204, Ann Arbor, MI 48109; e-mail: [email protected].
© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
CASE REPORT SCHUMACHER ET AL PALLIATING SEVERE ARTERIOVENOUS FISTULAE
1301
left-sided PAVF (Fig 1). The outside hospital recommended a left pneumonectomy. At our institution, it was believed (based on her technique of Fontan reconstruction and an angiogram suggesting little mixing between her PAs) that the lack of “hepatic factor” to her left lung was responsible for the development of PAVF. There were no other apparent correctable lesions (such as stenosis between the branch PAs) that were amenable to surgical repair. A long discussion was undertaken with the patient’s mother outlining the risks and benefits of the possible approaches (summarized in Table 1). The decision was made to proceed with a Fontan takedown to a two-ventricle repair. Cardiopulmonary bypass with standard arterial cannulation and venous cannulae to her superior vena cava and hepatic veins was instituted. The extracardiac conduit was resected leaving a 2-cm cuff of the conduit on the right PA and hepatic veins. Examination of the internal anatomy revealed a complete atrioventricular septal defect with approximately two thirds of the common atrioventricular valve dedicated to the left ventricle and ipsilateral pulmonary venous return. The right ventricular volume was smaller than had been suggested by echocardiogram and catheterization. Multiple trabeculations were divided, and the muscle was resected to increase ventricular size. A standard two-patch technique was used for atrioventricular septal defect repair, with the exception that the superior most portion of the ventricular septal defect patch was not sewn to the crest of the defect for the later intracardiac double-outlet right ventricle repair. The atrial patch was constructed to baffle the right pulmonary veins to the left atrium. The branch PAs were divided just distal to the Glenn anastomoses, and the proximal ends were oversewn, leaving the central portion of the PA to serve as the “innominate vein.” A right ventriculotomy was made. The ventricular septal defect was enlarged superiorly and anteriorly as there was no outlet extension. A portion of stretch polytetrafluoroethylene tube graft was opened longitudinally and was used to form an intraventricular tunnel from the ventricular septal defect to the aorta. A 22-mm cryopreserved pulmonary allograft was bicuspidized to 16 mm due to a lack of an appropriately sized graft. Anastomosing the allograft left PA to the native left PA and using a ringed 12-mm polytetrafluoroethylene graft to reach the right PA established right ventricle to PA continuity. Systemic venous drainage was re-established by sewing the cuffs of polytetrafluoroethylene graft from the extracardiac conduit on the hepatic veins and native proximal right PA (now “innominate vein”) to the right atrium. Total cardiopulmonary bypass time was 302 minutes, and cross-clamp time was 145 minutes. The patient tolerated the procedure well, but postoperatively she continued to have cyanosis with systemic oxygen saturations of 60% to 75%, and she required significant ventilatory support. Cardiac catheterization re-demonstrated diffuse left-sided PAVF and significant improvement in saturation with balloon occlusion of the 0003-4975/10/$36.00 doi:10.1016/j.athoracsur.2009.08.077
FEATURE ARTICLES
Ann Thorac Surg 2010;89:1301–3
1302
CASE REPORT SCHUMACHER ET AL PALLIATING SEVERE ARTERIOVENOUS FISTULAE
Ann Thorac Surg 2010;89:1301–3
Fig 1. (A) Angiogram of the extracardiac conduit Fontan with preferential filling of the right pulmonary artery. (B) Left upper lobe pulmonary arteriovenous fistulae (PAVF). (C) Medial left-lower lobe PAVF. (D) Lateral leftlower lobe PAVF.
FEATURE ARTICLES
left PA. A left PA band was believed to be potentially beneficial. The patient returned to the operating room and had the placement of an absorbable polyglactin 910 (Vicryl, Ethicon, Somerville, NJ) mesh left PA band. Saturation before application of the band was 70%. The diameter of the band was based on a desire to see a rise in oxygen saturation, while minimizing the gradient to allow for good perfusion of the left lung and adequate delivery of “hepatic factor.” After band placement, saturation improved to 96% with a measured 15 mm Hg trans-band gradient. Postoperatively, she maintained systemic oxygen saturation in the 90% range on room air. She was discharged home on postoperative day 15. At her 1-year follow-up, the family reported no cyanosis, dyspnea, or exercise intolerance. Oxygen saturations were 97% to 99%. Cardiac catheterization demonstrated
resolution of the left-sided PAVF with only one small pulmonary arteriovenous fistula remaining (Fig 2). Her left PA had no structural abnormalities and no pressure gradient across the banded area.
Comment Formation of PAVF in post-Fontan patients is a significant morbidity with limited treatment options. Several authors have noted resolution of PAVF once hepatic venous flow is directed to the PA [2, 3]. Our patient also demonstrated PAVF resolution after redirection of hepatic blood flow to the affected lung. After redirection of hepatic venous flow to the PA, patients continue to have cyanosis while the PAVF resolved [2, 3]. Our patient represents a unique approach to improving cyanosis in patients with resolving unilateral PAVF. Several authors have reported their use of absorb-
Table 1. Summary of Proposed Options Procedure
Advantages
Disadvantages ● ●
●
Lowest risk Likely to deliver “hepatic factor” to left lung Intermediate risk
●
Normal physiology
● ●
Arteriovenous Shunt/Fistula Fontan revision
● ●
Two-ventricle repair
● ●
Volume overload Uncertain long-term durability Uncertain delivery of “hepatic factor” to left lung Long-term risks of Fontan Highest risk Need for future right ventricle-to-pulmonary artery conduit replacement
Ann Thorac Surg 2010;89:1301–3
CASE REPORT SCHUMACHER ET AL PALLIATING SEVERE ARTERIOVENOUS FISTULAE
1303
had complete reabsorption of the PA band with no surgery required for PA reconstruction. Using an absorbable PA band to restrict blood flow to a lung affected by PAVF has not been reported. This may immediately improve systemic oxygen saturations by limiting the right-to-left shunt through the unilateral PAVF. Also, this may obviate the need for a second surgery to remove the PA band once PAVF have resolved. In our case, the use of an absorbable PA band did not cause any distortion of PA anatomy. We expect this report will encourage further usage and study of absorbable PA bands.
Fig 2. Angiogram of the reconstructed pulmonary artery (PA) demonstrating normal blood flow to the left PA with no notable pulmonary arteriovenous fistulae.
able PA bands in patients with congenital heart disease [4 – 6]. Patients reported include ventricular septal defect [4, 5], atrioventricular septal defect [4], and coarctation of the aorta with ventricular septal defect [5, 6]. All patients
1. Cloutier A, Ash J, Smallhorn J, Williams WG, Trussler GA, Rowe RD, Rabinovitch M. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae. Circulation 1985;72:471–9. 2. Ichikawa H, Fukushima N, Ono M, et al. Resolution of pulmonary arteriovenous fistula by redirection of hepatic venous blood. Ann Thoracic Surg 2004;77:1825–7. 3. Kim S-J, Bae E-J, Lee J-Y, Lim H-G, Lee C, Lee C-H. Inclusion of hepatic venous drainage in patients with pulmonary arteriovenous fistulas. Ann Thoracic Surg 2009;87:548 –53. 4. Gutierrez de Loma J, Ferreiros Mur M, Castilla Moreno M, Garcia Pena R, Gonzalez de Vega N. Reabsorbable banding: our initial experience. Rev Esp Cardiol 1991;44:677–9. 5. Peek GJ, Arsiwala SS, Chan KC, Hickey MS. Absorbable pulmonary artery band. Ann Thoracic Surg 1997;64:539 – 41. 6. Bonnet D, Patkai J, Tamisier D, Kachaner J, Vouhe P, Sidi D. A new strategy for the surgical treatment of aortic coarctation associated with ventricular septal defect in infants using an absorbable pulmonary artery band. JACC 1999;34:866 –70.
FEATURE ARTICLES
References