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A vascular ring: right aortic arch and descending aorta with left ductus arteriosus
Ann Thorac Surg 2001;71:729 –31
that time confirmed the diagnosis and 22 steel Gianturco coils (Cook, Bloomington, IN) were implanted in the aneurysm in an attempt to occlude it. Six months after this procedure she was evaluated at our institution. Repeat cardiac catheterization revealed systemic oxygen saturation of 75%, proximal pulmonary artery pressures equal to systemic levels, and a long segment distal left pulmonary artery stenosis in the area of a large left lower lobe pulmonary artery aneurysm (Figs 1A and 1B). The aneurysm had increased in size by approximately 20% since placement of the coils. The enlargement of the aneurysm (which was subjected to systemic arterial pressure) over a period of 6 months suggested that the risk of rupture was significant. The distal location of the aneurysm, the child’s multiple previous surgical procedures, and the presence of cyanosis were thought to increase the risk of surgical repair of the aneurysm significantly. Therefore, the decision was made to proceed with percutaneous implantation of an endovascular stent graft across the lesion. Compassionate release of two devices was granted from our institutional review board and the supplying companies. Informed consent was obtained from the family. At angiography a clearly identifiable aneurysm neck was identified as well as a residual pulmonary artery stenosis. An attempt to pass a 10 mm x 5 cm Hemobahn endovascular device (W. L. Gore, Flagstaff, AZ) was unsuccessful because of the rigidity of the device. A 10 mm x 30 mm self-expanding Wallgraft (Boston Scientific, Boston, MA) was then positioned across the area of stenosis, straddling the neck of the aneurysm. A single branch supplying a posterior basal segment of the left lower lobe arose in close proximity to the aneurysm neck. After angiographic confirmation of positioning, the device was deployed, resulting in complete exclusion of the aneurysm and improvement of the left pulmonary artery stenosis (Figs 2A and 2B). The posterior basal segmental branch no longer filled with contrast suggesting occlusion by the device. Further expansion of the graft was performed with a 10 mm angioplasty balloon to eliminate residual pulmonary artery stenosis. The procedure was well tolerated and the patient was discharged 48 hours later.
Comment Endovascular grafts have been used for many indications, including aortopulmonary fistulas and aortic, iliac, and femoral aneurysms [7, 8]. This patient presented after six previous operations and multiple percutaneous procedures with an enlarging and potentially lethal pulmonary artery pseudoaneurysm. Attempted closure with coil embolization had been unsuccessful. Her underlying problem of pulmonary atresia with ventricular septal defect had not been corrected, resulting in significant desaturation and pulmonary hypertension increasing her surgical risk. Given the size and location of the aneurysm, a left thoracotomy and aneurysm repair or pneumonectomy were suboptimal choices. The ability to exclude the aneurysm while simultaneously improving the left pulmonary artery stenosis made delivery of an endo© 2001 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
CASE REPORT HAN ET AL DUCTUS ARTERIOSUS VASCULAR RING
729
vascular graft attractive. This case represents a novel use of an endovascular stent graft and underscores the need for access to these newer prostheses for treatment of life-threatening problems in the pediatric population.
References 1. Gebitekin C, Yilmaz M, Senkaya I, Saba D, Sagdic K, Ozer G. Fatal haemoptysis due to pulmonary artery aneurysm in Behc¸et’s disease. Eur J Vasc Endovasc Surg 1997;13:233– 6. 2. Bowler RP, Durham J, Schwarz MI. Massive hemoptysis from a pulmonary artery aneurysm associated with an emphysematous bulla. Chest 1998;113:1130–1. 3. Oliver TB, Stevenson AJ, Gillespie IN. Pulmonary artery pseudoaneurysm due to bronchial carcinoma. Br J Radiol 1997;70:950–1. 4. Podbielski FJ, Wiesman IM, Yaghmai B, Owens CA, Benedetti E, Massad MG. Pulmonary artery pseudoaneurysm after tube thoracostomy. Ann Thorac Surg 1997;64:1478– 80. 5. Boubaker A, Payot M, Genton CY. Fatal rupture of an acquired aneurysm of the pulmonary artery: rare complication after surgical palliation of tricuspid atresia. Pediatr Cardiol 1997;18:392–5. 6. Boulden TF, Tonkin IL, Burton EM, et al. Case of the day: pediatric mycotic pseudoaneurysm and thrombosis of modified left Blalock-Taussig shunt. Radiographics 1990;10:119–21. 7. Miyata T, Ohara N, Shigematsu H, et al. Endovascular stent graft repair of aortopulmonary fistula. J Vasc Surg 1999;29: 557– 60. 8. Campagna AC, Wehner JH, Kirsch CM, et al. Endovascular stenting of an aortopulmonary fistula presenting with hemoptysis: a case report. J Cardiovasc Surg (Torino) 1996;37: 643– 6.
A Vascular Ring: Right Aortic Arch and Descending Aorta With Left Ductus Arteriosus Jae Jin Han, MD, Sejung Sohn, MD, Hae Soon Kim, MD, Tae Hee Won, MD, and Jae Ho Ahn, MD Departments of Cardiac Surgery and Pediatric Cardiology, Mokdong Hospital, Ewha Womans University, Seoul, Korea
A case is presented of vascular ring caused by right aortic arch with mirror-image branching and left ductus arteriosus. In this case, the descending aorta was located right of the tracheoesophagus and the left ductus arteriosus connected to the descending aorta far below the arch, producing compression of the esophagus only. Through median sternotomy, the ligation and division of the ductus was performed with concomitant repair of ventricular septal defect. (Ann Thorac Surg 2001;71:729 –31) © 2001 by The Society of Thoracic Surgeons
Accepted for publication April 15, 2000. Address reprint requests to Dr Han, Department of ThoracicCardiovascular Surgery, Ewha Womans University Mokdong Hospital, 911-1, Mokdong, Yangcheongu, Seoul, 158-710, Korea; e-mail: jjhan@mm. ewha.ac.kr.
0003-4975/01/$20.00 PII S0003-4975(00)02352-3
730
CASE REPORT HAN ET AL DUCTUS ARTERIOSUS VASCULAR RING
Ann Thorac Surg 2001;71:729 –31
aortic arch and left ligamentum arteriosum were identified. Under the extracorporeal bypass the descending aorta, located right to the trachea and esophagus, was dissected through the space between the superior vena cava and ascending aorta. A diverticulum of 10 mm in diameter originating from the descending aorta, traversed leftward all the way behind the esophagus, and was connected to the ligamentum arteriosum at the left side of trachea and esophagus. So the diverticulum eventually compressed the esophagus from behind as a pulsating blind-pouch (Fig 2). After ligation and division of the diverticulum and ligamentum of ductus, a ventricular septal defect was closed as usual. Postoperatively, the patient was free of dysphagia and vomiting. The esophagogram was repeated 2 months after the operation and showed the indentation had markedly decreased (Fig 3).
Comment The right–sided aortic arch results from regression of the left dorsal aortic root, and regression between the left ductus arteriosus and the descending aorta produces a mirror image branching of the aorta. A left-sided ductus arteriosus may connect the left pulmonary artery to the left subclavian portion of the innominate artery or to the descending aorta [4]. In the latter case, a complete vas-
Fig 1. Lateral view of preoperative esophagogram showing a posteriorly deep impression.
C
ongenital vascular ring that may cause compression of the airway and the esophagus, or both, usually includes a double aortic arch, right or left aortic arch with aberrant opposite-sided subclavian artery, and a right aortic arch with left ductus arteriosus [1, 2]. Other rare types of aortic arch anomalies have also been described [3]. We encountered a variant form of right aortic arch with left ductus arteriosus, accompanied with underlying ventricular septal defect. An 18-month-old boy, weighing 9.2 kg, with frequent aspiration and vomiting during feeding, was admitted to the hospital. The esophagogram showed a posterior impression of esophagus without airway stenosis (Fig 1). Echocardiogram and aortic angiogram showed a ventricular septal defect and suggested a type of vascular ring such as right aortic arch, mirror-image branching and left ductus arteriosus. Through median sternotomy, a right
Fig 2. Drawing of the vascular ring in the present case. A vascular ring is performed by the right aortic arch with right-sided descending aorta (DA), pulmonry artery (PA), and a left ductus arteriosus (arrow) originating from the descending aorta as a diverticulum, compressing the esophagus posteriorly, and connecting to the left pulmonary artery. (AA ⫽ ascending aorta.)
Ann Thorac Surg 2001;71:729 –31
CASE REPORT HAN ET AL DUCTUS ARTERIOSUS VASCULAR RING
731
(posterior) circumflex descending aorta at the arch level [1, 5]. Others reported tracheoesophagus compressed at the junction between aortic arch and descending aorta by a diverticulum [6] or a ligamentum [7], and they are usually not associated with intracardiac defects. In this case, however, the ductus arteriosus arose below the aortic arch and a ventricular septal defect coexisted. The operation for the vascular ring of the right aortic arch with a mirror image branching and a left ductus arteriosus was usually done through the left thoracotomy [1, 2, 8]. For this patient, we performed a median sternotomy for the concomitant repair of ventricular septal defect. In this particular patient, even without coexisted intracardiac defect, we believed that the conventional left thoracotomy would not be good for direct access to the originating portion of the diverticulum, which plays a major role in esophageal compression.
References
Fig 3. Lateral view of postoperative esophagogram showing that the posteriorly deep impression was now significantly widened 2 months after the operation.
cular ring exists. The previous reports of this type of vascular ring usually described that tracheoesophagus was compressed by either a left dorsal aortic root or a left
1. Van Son JAM, Julsrud PR, Hagler DJ, et al. Surgical treatment of vascular rings. The Mayo Clinic experience. Mayo Clin Proc 1993;68:1056– 63. 2. Roberts CS, Othersen HB, Jr, Sade RM, Smith CDIII, Tagge EP, Crawford FA, Jr. Tracheoesophageal compression from aortic arch anomalies: analysis of 30 operatively treated children. J Pediatr Surg 1994;29:334– 8. 3. Moes CAF, Freedom RM. Rare types of aortic arch anomalies. Pediatr Cardiol 1993;14:93–101. 4. De Leval M. Vascular rings. Surgery for congenital heart defects, 2nd ed. Philadelphia: WB Saunders,1994:307–16. 5. D’Souza VJ, Velasquez G, Glass TA, Formanek AG. Mirror image right aortic arch. A proposed mechanism in symptomatic vascular ring. Cardiovasc Intervent Radiol 1985;8:134– 6. 6. Garti IJ, Aygen MM, Vidne B, Levy MJ. Right aortic arch with mirror image branching causing vascular ring. A new classificaton of the right aortic arch patterns. Br J Radiol 1973;46: 115–9. 7. Schlesinger AE, Mendeloff E, Sharkey AM, Spray TL. MR of right aortic arch with mirror image branching and a left ligamentum arteriosum: an unusual cause of a vascular ring. Pediatr Radiol 1995;25:455–7. 8. Hartyanszky IL, Lozsadi K, Marcsek P, Huttl T, Sapi E, Kovacs AB. Congenital vascular rings: surgical management of 111 cases. Eur J Cadiothorac Surg 1989;3:250– 4.
that time confirmed the diagnosis and 22 steel Gianturco coils (Cook, Bloomington, IN) were implanted in the aneurysm in an attempt to occlude it. Six months after this procedure she was evaluated at our institution. Repeat cardiac catheterization revealed systemic oxygen saturation of 75%, proximal pulmonary artery pressures equal to systemic levels, and a long segment distal left pulmonary artery stenosis in the area of a large left lower lobe pulmonary artery aneurysm (Figs 1A and 1B). The aneurysm had increased in size by approximately 20% since placement of the coils. The enlargement of the aneurysm (which was subjected to systemic arterial pressure) over a period of 6 months suggested that the risk of rupture was significant. The distal location of the aneurysm, the child’s multiple previous surgical procedures, and the presence of cyanosis were thought to increase the risk of surgical repair of the aneurysm significantly. Therefore, the decision was made to proceed with percutaneous implantation of an endovascular stent graft across the lesion. Compassionate release of two devices was granted from our institutional review board and the supplying companies. Informed consent was obtained from the family. At angiography a clearly identifiable aneurysm neck was identified as well as a residual pulmonary artery stenosis. An attempt to pass a 10 mm x 5 cm Hemobahn endovascular device (W. L. Gore, Flagstaff, AZ) was unsuccessful because of the rigidity of the device. A 10 mm x 30 mm self-expanding Wallgraft (Boston Scientific, Boston, MA) was then positioned across the area of stenosis, straddling the neck of the aneurysm. A single branch supplying a posterior basal segment of the left lower lobe arose in close proximity to the aneurysm neck. After angiographic confirmation of positioning, the device was deployed, resulting in complete exclusion of the aneurysm and improvement of the left pulmonary artery stenosis (Figs 2A and 2B). The posterior basal segmental branch no longer filled with contrast suggesting occlusion by the device. Further expansion of the graft was performed with a 10 mm angioplasty balloon to eliminate residual pulmonary artery stenosis. The procedure was well tolerated and the patient was discharged 48 hours later.
Comment Endovascular grafts have been used for many indications, including aortopulmonary fistulas and aortic, iliac, and femoral aneurysms [7, 8]. This patient presented after six previous operations and multiple percutaneous procedures with an enlarging and potentially lethal pulmonary artery pseudoaneurysm. Attempted closure with coil embolization had been unsuccessful. Her underlying problem of pulmonary atresia with ventricular septal defect had not been corrected, resulting in significant desaturation and pulmonary hypertension increasing her surgical risk. Given the size and location of the aneurysm, a left thoracotomy and aneurysm repair or pneumonectomy were suboptimal choices. The ability to exclude the aneurysm while simultaneously improving the left pulmonary artery stenosis made delivery of an endo© 2001 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
CASE REPORT HAN ET AL DUCTUS ARTERIOSUS VASCULAR RING
729
vascular graft attractive. This case represents a novel use of an endovascular stent graft and underscores the need for access to these newer prostheses for treatment of life-threatening problems in the pediatric population.
References 1. Gebitekin C, Yilmaz M, Senkaya I, Saba D, Sagdic K, Ozer G. Fatal haemoptysis due to pulmonary artery aneurysm in Behc¸et’s disease. Eur J Vasc Endovasc Surg 1997;13:233– 6. 2. Bowler RP, Durham J, Schwarz MI. Massive hemoptysis from a pulmonary artery aneurysm associated with an emphysematous bulla. Chest 1998;113:1130–1. 3. Oliver TB, Stevenson AJ, Gillespie IN. Pulmonary artery pseudoaneurysm due to bronchial carcinoma. Br J Radiol 1997;70:950–1. 4. Podbielski FJ, Wiesman IM, Yaghmai B, Owens CA, Benedetti E, Massad MG. Pulmonary artery pseudoaneurysm after tube thoracostomy. Ann Thorac Surg 1997;64:1478– 80. 5. Boubaker A, Payot M, Genton CY. Fatal rupture of an acquired aneurysm of the pulmonary artery: rare complication after surgical palliation of tricuspid atresia. Pediatr Cardiol 1997;18:392–5. 6. Boulden TF, Tonkin IL, Burton EM, et al. Case of the day: pediatric mycotic pseudoaneurysm and thrombosis of modified left Blalock-Taussig shunt. Radiographics 1990;10:119–21. 7. Miyata T, Ohara N, Shigematsu H, et al. Endovascular stent graft repair of aortopulmonary fistula. J Vasc Surg 1999;29: 557– 60. 8. Campagna AC, Wehner JH, Kirsch CM, et al. Endovascular stenting of an aortopulmonary fistula presenting with hemoptysis: a case report. J Cardiovasc Surg (Torino) 1996;37: 643– 6.
A Vascular Ring: Right Aortic Arch and Descending Aorta With Left Ductus Arteriosus Jae Jin Han, MD, Sejung Sohn, MD, Hae Soon Kim, MD, Tae Hee Won, MD, and Jae Ho Ahn, MD Departments of Cardiac Surgery and Pediatric Cardiology, Mokdong Hospital, Ewha Womans University, Seoul, Korea
A case is presented of vascular ring caused by right aortic arch with mirror-image branching and left ductus arteriosus. In this case, the descending aorta was located right of the tracheoesophagus and the left ductus arteriosus connected to the descending aorta far below the arch, producing compression of the esophagus only. Through median sternotomy, the ligation and division of the ductus was performed with concomitant repair of ventricular septal defect. (Ann Thorac Surg 2001;71:729 –31) © 2001 by The Society of Thoracic Surgeons
Accepted for publication April 15, 2000. Address reprint requests to Dr Han, Department of ThoracicCardiovascular Surgery, Ewha Womans University Mokdong Hospital, 911-1, Mokdong, Yangcheongu, Seoul, 158-710, Korea; e-mail: jjhan@mm. ewha.ac.kr.
0003-4975/01/$20.00 PII S0003-4975(00)02352-3
730
CASE REPORT HAN ET AL DUCTUS ARTERIOSUS VASCULAR RING
Ann Thorac Surg 2001;71:729 –31
aortic arch and left ligamentum arteriosum were identified. Under the extracorporeal bypass the descending aorta, located right to the trachea and esophagus, was dissected through the space between the superior vena cava and ascending aorta. A diverticulum of 10 mm in diameter originating from the descending aorta, traversed leftward all the way behind the esophagus, and was connected to the ligamentum arteriosum at the left side of trachea and esophagus. So the diverticulum eventually compressed the esophagus from behind as a pulsating blind-pouch (Fig 2). After ligation and division of the diverticulum and ligamentum of ductus, a ventricular septal defect was closed as usual. Postoperatively, the patient was free of dysphagia and vomiting. The esophagogram was repeated 2 months after the operation and showed the indentation had markedly decreased (Fig 3).
Comment The right–sided aortic arch results from regression of the left dorsal aortic root, and regression between the left ductus arteriosus and the descending aorta produces a mirror image branching of the aorta. A left-sided ductus arteriosus may connect the left pulmonary artery to the left subclavian portion of the innominate artery or to the descending aorta [4]. In the latter case, a complete vas-
Fig 1. Lateral view of preoperative esophagogram showing a posteriorly deep impression.
C
ongenital vascular ring that may cause compression of the airway and the esophagus, or both, usually includes a double aortic arch, right or left aortic arch with aberrant opposite-sided subclavian artery, and a right aortic arch with left ductus arteriosus [1, 2]. Other rare types of aortic arch anomalies have also been described [3]. We encountered a variant form of right aortic arch with left ductus arteriosus, accompanied with underlying ventricular septal defect. An 18-month-old boy, weighing 9.2 kg, with frequent aspiration and vomiting during feeding, was admitted to the hospital. The esophagogram showed a posterior impression of esophagus without airway stenosis (Fig 1). Echocardiogram and aortic angiogram showed a ventricular septal defect and suggested a type of vascular ring such as right aortic arch, mirror-image branching and left ductus arteriosus. Through median sternotomy, a right
Fig 2. Drawing of the vascular ring in the present case. A vascular ring is performed by the right aortic arch with right-sided descending aorta (DA), pulmonry artery (PA), and a left ductus arteriosus (arrow) originating from the descending aorta as a diverticulum, compressing the esophagus posteriorly, and connecting to the left pulmonary artery. (AA ⫽ ascending aorta.)
Ann Thorac Surg 2001;71:729 –31
CASE REPORT HAN ET AL DUCTUS ARTERIOSUS VASCULAR RING
731
(posterior) circumflex descending aorta at the arch level [1, 5]. Others reported tracheoesophagus compressed at the junction between aortic arch and descending aorta by a diverticulum [6] or a ligamentum [7], and they are usually not associated with intracardiac defects. In this case, however, the ductus arteriosus arose below the aortic arch and a ventricular septal defect coexisted. The operation for the vascular ring of the right aortic arch with a mirror image branching and a left ductus arteriosus was usually done through the left thoracotomy [1, 2, 8]. For this patient, we performed a median sternotomy for the concomitant repair of ventricular septal defect. In this particular patient, even without coexisted intracardiac defect, we believed that the conventional left thoracotomy would not be good for direct access to the originating portion of the diverticulum, which plays a major role in esophageal compression.
References
Fig 3. Lateral view of postoperative esophagogram showing that the posteriorly deep impression was now significantly widened 2 months after the operation.
cular ring exists. The previous reports of this type of vascular ring usually described that tracheoesophagus was compressed by either a left dorsal aortic root or a left
1. Van Son JAM, Julsrud PR, Hagler DJ, et al. Surgical treatment of vascular rings. The Mayo Clinic experience. Mayo Clin Proc 1993;68:1056– 63. 2. Roberts CS, Othersen HB, Jr, Sade RM, Smith CDIII, Tagge EP, Crawford FA, Jr. Tracheoesophageal compression from aortic arch anomalies: analysis of 30 operatively treated children. J Pediatr Surg 1994;29:334– 8. 3. Moes CAF, Freedom RM. Rare types of aortic arch anomalies. Pediatr Cardiol 1993;14:93–101. 4. De Leval M. Vascular rings. Surgery for congenital heart defects, 2nd ed. Philadelphia: WB Saunders,1994:307–16. 5. D’Souza VJ, Velasquez G, Glass TA, Formanek AG. Mirror image right aortic arch. A proposed mechanism in symptomatic vascular ring. Cardiovasc Intervent Radiol 1985;8:134– 6. 6. Garti IJ, Aygen MM, Vidne B, Levy MJ. Right aortic arch with mirror image branching causing vascular ring. A new classificaton of the right aortic arch patterns. Br J Radiol 1973;46: 115–9. 7. Schlesinger AE, Mendeloff E, Sharkey AM, Spray TL. MR of right aortic arch with mirror image branching and a left ligamentum arteriosum: an unusual cause of a vascular ring. Pediatr Radiol 1995;25:455–7. 8. Hartyanszky IL, Lozsadi K, Marcsek P, Huttl T, Sapi E, Kovacs AB. Congenital vascular rings: surgical management of 111 cases. Eur J Cadiothorac Surg 1989;3:250– 4.