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Endovascular repair of blunt aortic injury in a patient with situs inversus and dextrocardia
Endovascular repair of blunt aortic injury in a patient with situs inversus and dextrocardia John F. Bilello MD, FACS, Peter L. Birnbaum, MD, MSc, FRCSC, Chandra Venugopal, MD, and Leo L. Fong, MD, Fresno, Calif A 24-year-old male pedestrian with situs inversus and dextrocardia was struck by a car and sustained multiple injuries, including a pseudoaneurysm of the proximal descending thoracic aorta. A thoracic endograft was deployed to exclude the blunt aortic injury. We are not aware of any report of endovascular repair of blunt aortic injury in a patient with this congenital finding. A brief review of the literature is also included. ( J Vasc Surg 2011;54:857-9.)
Endovascular repair of blunt aortic injury (BAI) has become a safe and acceptable method of repair in selected trauma patients. The presence of multiple injuries and concomitant risks associated with heparinization and prolonged surgery have made the endovascular approach an attractive option. Only open repairs of BAI in patients with situs inversus and dextrocardia have been reported in the literature. We are not aware of any report of endovascular repair of BAI in a patient with this congenital finding. CASE REPORT A 24-year-old male pedestrian was struck by a fast-moving automobile and sustained numerous injuries. He was hypotensive in the field (blood pressure, 76/52; pulse,128 beats/min), with a Glasgow Coma Scale (GCS) score of 6, and required prehospital endotracheal intubation. However, the paramedics stated that the patient initially was awake and moving all his extremities and talking in the field. In the trauma bay, the patient was hypotensive (blood pressure, 82; pulse, 123 beats/min) but responded to intravenous fluids prescribed by Advanced Trauma Life Support protocol. His vital signs normalized after 3 L of intravenous (IV) crystalloid. His initial serum base deficit was –11 but improved with IV fluid and 2 U of packed red blood cells. His blood alcohol level was 0.12%. He was noted to have a deep scalp laceration, abrasions on the torso, and open grade IIIc fractures of the right elbow and right distal femur. The initial Focused Abdominal Sonogram for Trauma (FAST) examination was of good quality and showed no fluid but was perplexing due to inability to correlate normal anatomy to the topographic landmarks. An anteroposterior chest radiograph (Fig 1) was originally thought to be technically unreliable due to the right-sided heart, From the Department of Surgery, Community Regional Medical Center, University of California, San Francisco–Fresno Campus. Competition of interest: none. Correspondence: Dr John F. Bilello, University of California, San FranciscoFresno Campus, Department of Surgery, Community Regional Medical Center, 1st Flr 2823 Fresno St, Fresno, CA 93721-1324 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a competition of interest. 0741-5214/$36.00 Copyright © 2011 by the Society for Vascular Surgery. doi:10.1016/j.jvs.2011.03.220
but further evaluation identified dextrocardia, with no evidence of hemothorax or pneumothorax. The mediastinum appeared widened and there was loss of the aortic knob. Result of the pelvis radiograph was negative. Once the patient stabilized hemodynamically, he underwent computed tomography (CT) scanning. The CT image of the head and face showed an old head injury with some encephalomalacia and previous plating of the frontal skull, as well as new mandibular fractures. No acute intracranial findings were noted. In addition to pulmonary contusions, manubrial, and scapular fractures, the chest CT image (Figs 2 and 3) confirmed dextrocardia with mediastinal hematoma and associated pseudoaneurysm of the descending aorta, 2.1 cm distal to the right subclavian artery. CT scan of the abdomen and pelvis (Fig 4) showed situs inversus, a grade I splenic laceration, and a grade II right renal laceration. The open fractures were irrigated, splinted, and dressed. The patient was evaluated by our orthopedic, cardiothoracic, and interventional radiology consultants. After resuscitation and normalization of hemodynamic status, the patient’s blood pressure and pulse were controlled with IV -blockade. Central venous access and left radial arterial cannulation were obtained. Systolic blood pressure was kept at about 100 mm Hg. Because the patient’s mental status improved and he was not comatose, there was less concern regarding cerebral perfusion and intracranial pressure, and an intracranial pressure monitor was not required. Urine output was adequate, and the base deficit improved. After full resuscitation and evaluation in the trauma intensive care unit, the GCS continued to improve to 11 T, and the patient remained neurovascularly intact. The open grade IIIc extremity fractures were debrided, washed, and stabilized by external fixation on hospital day 2 (by 12 hours of admission) in the operating room. Under the same general anesthetic, he subsequently went to the interventional radiology suite where a 22- ⫻ 116-mm Talent thoracic endograft (Medtronic Vascular, Minneapolis, Minn) was deployed in the proximal descending thoracic aorta (Fig 5) via the right common femoral artery. The pseudoaneurysm was successfully covered and the patency of the right subclavian artery was maintained because the proximal bare metal section was deployed over this vessel. The patient was admitted at about 2200 hours on his date of admission, and his evaluation, resuscitation, and initial orthopedic and endovascular procedures were all completed ⱕ24 hours of admission on hospital day 2.
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Fig 1. Admission anteroposterior chest radiograph.
Fig 3. The aorta and pseudoaneurysm are shown in a 3-dimensional reconstruction. The arrow points to the injury.
Fig 2. Computed tomography imaging of the chest shows dextrocardia and the aortic injury. The arrow points to the pseudoaneurysm. The patient subsequently required serial extremity debridement, and open reduction and internal fixation of his extremity orthopedic injuries and the mandibular fractures, tracheostomy, autologous split-thickness skin grafting, and complex wound care of his extremity injuries. He recuperated and was discharged to a rehabilitation facility on hospital day 33, with no focal neurologic deficits. He continues to do well at the 3-month follow-up evaluation. He was contacted by telephone and appeared to be undergoing physical therapy at home. Despite our recommendations, the patient has so far not returned for a follow-up or CT evaluation of his descending thoracic aorta.
DISCUSSION Total situs inversus with dextrocardia describes an autosomal recessive condition in which the thoracic and abdominal viscera are perfect mirror images of their normal
Fig 4. Total situs inversus is shown in this computed tomography image of the abdomen.
anatomic positions. It occurs in approximately 2/10,000 live births and is associated with congenital structural anomalies in approximately 3%.1 Kartagener syndrome, an associated primary ciliary dysfunction, occurs in about 20% of patients with complete situs inversus and dextrocardia. These patients can have concomitant lung disease (bronchiectasis) and sinusitis from the ciliary impairment.1 Our patient did not demonstrate complications of this syndrome during the course of his admission. The human embryonic heart starts as a mesenchymal tube in week 3 of gestation.2 The primitive heart tube then elongates and normally bends (“loops”) to the right on day 23 of gestation. In dextrocardia, the cardiogenic tube
JOURNAL OF VASCULAR SURGERY Volume 54, Number 3
Fig 5. Endograft deployment in is shown in the proximal descending aorta.
“loops” to the left. By week 4 of gestation, the six branchial arches receive arteries, or aortic arches, from the aortic sac. At 8 weeks, the first, second, and fifth arches have involuted to contribute to the formation of the normally left-sided aorta and its great vessels. The left fourth branchial arch forms part of the aortic arch. The right fourth arch becomes the right subclavian artery. The complex growth and involution of the various arches can contribute to numerous anomalies that can also involve the esophagus and trachea.2 Blunt descending aortic injury is usually caused by rapid deceleration and shear forces.3,4 BAI accounts for ⬍1% of trauma admissions but is the second-leading cause of death from blunt trauma after head injuries and has a mortality rate ⬎90%.5,6 Treatment has traditionally involved open surgery with aortic cross-clamping and is associated with significant mortality and morbidity, including bleeding from anticoagulation, thoracotomy, and single-lung ventilation, as well as paraplegia.6,7 Endovascular repair has become a safe and satisfactory method of aortic repair in selected patients.5-8 The Talent endograft has been our endograft of choice. The bare metal stent allows accurate placement in the proximal landing zone with a lower risk of compromise to the carotid artery. BAI typically occurs just distal to the subclavian artery,3 which is frequently covered partially or completely after our endograft deployment. The angiograms after deployment for this particular patient (not available) showed unobstructed flow through the right subclavian artery. A review by Hershberger et al4 concluded that a selective approach
Bilello et al 859
should be used regarding the need for subclavian revascularization when stenting aortic injuries, especially since most of the studies that cite subclavian arterial sequelae include patients with thoracic aortic aneurysms and atherosclerosis. Aortic injury and acute aortic dissection with open repair has been reported in patients with situs inversus and dextrocardia,9,10 but we are not aware of any report of endovascular repair of BAI in a patient with situs inversus and dextrocardia. The anomalous anatomy of our patient did not hinder endovascular repair of his injury. Other than concerns for possible associated cardiac or pulmonary disease, preoperative planning differed only in placement of the arterial catheter in the left wrist instead of the right. During the short delay for resuscitation and treatment of his other limb-threatening injuries, inotropic/chronotropic control was used. The patient’s initial evaluation, resuscitation, and repair of immediate life-threatening and limb-threatening injuries occurred within the first 24 hours of admission. Although this young patient’s right-sided aorta, the small aortic diameter, arch anatomy, and aortic angulation still presented the usual challenges of endograft deployment, successful placement was obtained with no adverse sequelae and an overall good outcome. REFERENCES 1. Fulton DR. Congenital heart disease in children and adolescents. In: Hurst’s the heart. 12th ed, vol 2. New York: McGraw Hill; 2008. p. 1900-1902. 2. Moore KL. The circulatory system. The developing human: clinically oriented embryology, 3rd ed. Philadelphia, PA: W. B. Saunders Company; 1982. p. 298-343. 3. Karmy-Jones R, Jackson N, Long W, Simeone A. Current management of traumatic rupture of the descending thoracic aorta. Curr Card Rev 2009;5:6187-95. 4. Hershberger RC, Aulivola B, Murphy M, Luchette FA. Endovascular grafts for treatment of traumatic injury to the aortic arch and the great vessels. J Trauma 2009;67:660-71. 5. Estrera AL, Gochnour DC, Azizzadeh A, Simeone A. Progress in the treatment of blunt thoracic aortic injury: 12-year single-institution experience. Ann Thorac Surg 2010;90:64-71. 6. Rahimi SA, Darling C 3rd, Mehta M, Roddy SP, Taggert JB, Sternbach Y. Endovascular repair of thoracic aortic traumatic transections is a safe method in patients with complicated injuries. J Vasc Surg 2010;52: 891-6. 7. Garcia-Toca M, Naughton PA, Matsumura JS, Morasch MD, Kibbe MR, Rodriguez HE, et al. Endovascular repair of blunt traumatic thoracic aortic injuries: seven-year single-center experience. Arch Surg 2010;145:679-83. 8. Neschis DG, Scalea TM. Endovascular repair of traumatic aortic injuries. Adv Surg 2010;44:281-92. 9. Magishi K, Izumi Y, Ishikawa N, Kimura F. Stanford type A acute aortic dissection caused by blunt trauma in a patient with situs inversus. Ann Thorac Surg 2006;81:2294-6. 10. Kulick DM, Park SJ, Harrison BS, Shumway SJ. Traumatic aortic and diaphragmatic rupture in a patient with dextrocardia and situs inversus: case report. J Trauma 1998;45:397-8. Submitted Jan 19, 2011; accepted Mar 4, 2011.
Endovascular repair of blunt aortic injury (BAI) has become a safe and acceptable method of repair in selected trauma patients. The presence of multiple injuries and concomitant risks associated with heparinization and prolonged surgery have made the endovascular approach an attractive option. Only open repairs of BAI in patients with situs inversus and dextrocardia have been reported in the literature. We are not aware of any report of endovascular repair of BAI in a patient with this congenital finding. CASE REPORT A 24-year-old male pedestrian was struck by a fast-moving automobile and sustained numerous injuries. He was hypotensive in the field (blood pressure, 76/52; pulse,128 beats/min), with a Glasgow Coma Scale (GCS) score of 6, and required prehospital endotracheal intubation. However, the paramedics stated that the patient initially was awake and moving all his extremities and talking in the field. In the trauma bay, the patient was hypotensive (blood pressure, 82; pulse, 123 beats/min) but responded to intravenous fluids prescribed by Advanced Trauma Life Support protocol. His vital signs normalized after 3 L of intravenous (IV) crystalloid. His initial serum base deficit was –11 but improved with IV fluid and 2 U of packed red blood cells. His blood alcohol level was 0.12%. He was noted to have a deep scalp laceration, abrasions on the torso, and open grade IIIc fractures of the right elbow and right distal femur. The initial Focused Abdominal Sonogram for Trauma (FAST) examination was of good quality and showed no fluid but was perplexing due to inability to correlate normal anatomy to the topographic landmarks. An anteroposterior chest radiograph (Fig 1) was originally thought to be technically unreliable due to the right-sided heart, From the Department of Surgery, Community Regional Medical Center, University of California, San Francisco–Fresno Campus. Competition of interest: none. Correspondence: Dr John F. Bilello, University of California, San FranciscoFresno Campus, Department of Surgery, Community Regional Medical Center, 1st Flr 2823 Fresno St, Fresno, CA 93721-1324 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a competition of interest. 0741-5214/$36.00 Copyright © 2011 by the Society for Vascular Surgery. doi:10.1016/j.jvs.2011.03.220
but further evaluation identified dextrocardia, with no evidence of hemothorax or pneumothorax. The mediastinum appeared widened and there was loss of the aortic knob. Result of the pelvis radiograph was negative. Once the patient stabilized hemodynamically, he underwent computed tomography (CT) scanning. The CT image of the head and face showed an old head injury with some encephalomalacia and previous plating of the frontal skull, as well as new mandibular fractures. No acute intracranial findings were noted. In addition to pulmonary contusions, manubrial, and scapular fractures, the chest CT image (Figs 2 and 3) confirmed dextrocardia with mediastinal hematoma and associated pseudoaneurysm of the descending aorta, 2.1 cm distal to the right subclavian artery. CT scan of the abdomen and pelvis (Fig 4) showed situs inversus, a grade I splenic laceration, and a grade II right renal laceration. The open fractures were irrigated, splinted, and dressed. The patient was evaluated by our orthopedic, cardiothoracic, and interventional radiology consultants. After resuscitation and normalization of hemodynamic status, the patient’s blood pressure and pulse were controlled with IV -blockade. Central venous access and left radial arterial cannulation were obtained. Systolic blood pressure was kept at about 100 mm Hg. Because the patient’s mental status improved and he was not comatose, there was less concern regarding cerebral perfusion and intracranial pressure, and an intracranial pressure monitor was not required. Urine output was adequate, and the base deficit improved. After full resuscitation and evaluation in the trauma intensive care unit, the GCS continued to improve to 11 T, and the patient remained neurovascularly intact. The open grade IIIc extremity fractures were debrided, washed, and stabilized by external fixation on hospital day 2 (by 12 hours of admission) in the operating room. Under the same general anesthetic, he subsequently went to the interventional radiology suite where a 22- ⫻ 116-mm Talent thoracic endograft (Medtronic Vascular, Minneapolis, Minn) was deployed in the proximal descending thoracic aorta (Fig 5) via the right common femoral artery. The pseudoaneurysm was successfully covered and the patency of the right subclavian artery was maintained because the proximal bare metal section was deployed over this vessel. The patient was admitted at about 2200 hours on his date of admission, and his evaluation, resuscitation, and initial orthopedic and endovascular procedures were all completed ⱕ24 hours of admission on hospital day 2.
857
858 Bilello et al
JOURNAL OF VASCULAR SURGERY September 2011
Fig 1. Admission anteroposterior chest radiograph.
Fig 3. The aorta and pseudoaneurysm are shown in a 3-dimensional reconstruction. The arrow points to the injury.
Fig 2. Computed tomography imaging of the chest shows dextrocardia and the aortic injury. The arrow points to the pseudoaneurysm. The patient subsequently required serial extremity debridement, and open reduction and internal fixation of his extremity orthopedic injuries and the mandibular fractures, tracheostomy, autologous split-thickness skin grafting, and complex wound care of his extremity injuries. He recuperated and was discharged to a rehabilitation facility on hospital day 33, with no focal neurologic deficits. He continues to do well at the 3-month follow-up evaluation. He was contacted by telephone and appeared to be undergoing physical therapy at home. Despite our recommendations, the patient has so far not returned for a follow-up or CT evaluation of his descending thoracic aorta.
DISCUSSION Total situs inversus with dextrocardia describes an autosomal recessive condition in which the thoracic and abdominal viscera are perfect mirror images of their normal
Fig 4. Total situs inversus is shown in this computed tomography image of the abdomen.
anatomic positions. It occurs in approximately 2/10,000 live births and is associated with congenital structural anomalies in approximately 3%.1 Kartagener syndrome, an associated primary ciliary dysfunction, occurs in about 20% of patients with complete situs inversus and dextrocardia. These patients can have concomitant lung disease (bronchiectasis) and sinusitis from the ciliary impairment.1 Our patient did not demonstrate complications of this syndrome during the course of his admission. The human embryonic heart starts as a mesenchymal tube in week 3 of gestation.2 The primitive heart tube then elongates and normally bends (“loops”) to the right on day 23 of gestation. In dextrocardia, the cardiogenic tube
JOURNAL OF VASCULAR SURGERY Volume 54, Number 3
Fig 5. Endograft deployment in is shown in the proximal descending aorta.
“loops” to the left. By week 4 of gestation, the six branchial arches receive arteries, or aortic arches, from the aortic sac. At 8 weeks, the first, second, and fifth arches have involuted to contribute to the formation of the normally left-sided aorta and its great vessels. The left fourth branchial arch forms part of the aortic arch. The right fourth arch becomes the right subclavian artery. The complex growth and involution of the various arches can contribute to numerous anomalies that can also involve the esophagus and trachea.2 Blunt descending aortic injury is usually caused by rapid deceleration and shear forces.3,4 BAI accounts for ⬍1% of trauma admissions but is the second-leading cause of death from blunt trauma after head injuries and has a mortality rate ⬎90%.5,6 Treatment has traditionally involved open surgery with aortic cross-clamping and is associated with significant mortality and morbidity, including bleeding from anticoagulation, thoracotomy, and single-lung ventilation, as well as paraplegia.6,7 Endovascular repair has become a safe and satisfactory method of aortic repair in selected patients.5-8 The Talent endograft has been our endograft of choice. The bare metal stent allows accurate placement in the proximal landing zone with a lower risk of compromise to the carotid artery. BAI typically occurs just distal to the subclavian artery,3 which is frequently covered partially or completely after our endograft deployment. The angiograms after deployment for this particular patient (not available) showed unobstructed flow through the right subclavian artery. A review by Hershberger et al4 concluded that a selective approach
Bilello et al 859
should be used regarding the need for subclavian revascularization when stenting aortic injuries, especially since most of the studies that cite subclavian arterial sequelae include patients with thoracic aortic aneurysms and atherosclerosis. Aortic injury and acute aortic dissection with open repair has been reported in patients with situs inversus and dextrocardia,9,10 but we are not aware of any report of endovascular repair of BAI in a patient with situs inversus and dextrocardia. The anomalous anatomy of our patient did not hinder endovascular repair of his injury. Other than concerns for possible associated cardiac or pulmonary disease, preoperative planning differed only in placement of the arterial catheter in the left wrist instead of the right. During the short delay for resuscitation and treatment of his other limb-threatening injuries, inotropic/chronotropic control was used. The patient’s initial evaluation, resuscitation, and repair of immediate life-threatening and limb-threatening injuries occurred within the first 24 hours of admission. Although this young patient’s right-sided aorta, the small aortic diameter, arch anatomy, and aortic angulation still presented the usual challenges of endograft deployment, successful placement was obtained with no adverse sequelae and an overall good outcome. REFERENCES 1. Fulton DR. Congenital heart disease in children and adolescents. In: Hurst’s the heart. 12th ed, vol 2. New York: McGraw Hill; 2008. p. 1900-1902. 2. Moore KL. The circulatory system. The developing human: clinically oriented embryology, 3rd ed. Philadelphia, PA: W. B. Saunders Company; 1982. p. 298-343. 3. Karmy-Jones R, Jackson N, Long W, Simeone A. Current management of traumatic rupture of the descending thoracic aorta. Curr Card Rev 2009;5:6187-95. 4. Hershberger RC, Aulivola B, Murphy M, Luchette FA. Endovascular grafts for treatment of traumatic injury to the aortic arch and the great vessels. J Trauma 2009;67:660-71. 5. Estrera AL, Gochnour DC, Azizzadeh A, Simeone A. Progress in the treatment of blunt thoracic aortic injury: 12-year single-institution experience. Ann Thorac Surg 2010;90:64-71. 6. Rahimi SA, Darling C 3rd, Mehta M, Roddy SP, Taggert JB, Sternbach Y. Endovascular repair of thoracic aortic traumatic transections is a safe method in patients with complicated injuries. J Vasc Surg 2010;52: 891-6. 7. Garcia-Toca M, Naughton PA, Matsumura JS, Morasch MD, Kibbe MR, Rodriguez HE, et al. Endovascular repair of blunt traumatic thoracic aortic injuries: seven-year single-center experience. Arch Surg 2010;145:679-83. 8. Neschis DG, Scalea TM. Endovascular repair of traumatic aortic injuries. Adv Surg 2010;44:281-92. 9. Magishi K, Izumi Y, Ishikawa N, Kimura F. Stanford type A acute aortic dissection caused by blunt trauma in a patient with situs inversus. Ann Thorac Surg 2006;81:2294-6. 10. Kulick DM, Park SJ, Harrison BS, Shumway SJ. Traumatic aortic and diaphragmatic rupture in a patient with dextrocardia and situs inversus: case report. J Trauma 1998;45:397-8. Submitted Jan 19, 2011; accepted Mar 4, 2011.