IJCA-18068; No of Pages 2 International Journal of Cardiology xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Aortic dissection in a patient with a dilated aortic root following tetralogy of Fallot repair Vishva A. Wijesekera ⁎, Marla C. Kiess, Jasmine Grewal, Rudy Chow, Rekha Raju, Jonathon A. Leipsic, Amanda J. Barlow University of British Columbia, Pacific Adult Congenital Heart Service, St Paul's Hospital, Vancouver, Canada
a r t i c l e
i n f o
Article history: Received 2 April 2014 Accepted 13 April 2014 Available online xxxx Keywords: Aortic dissection Tetralogy of Fallot Aortic aneurysm
With improved longevity, aortic root dilation has been increasingly recognized among patients with tetralogy of Fallot (TOF). Aortic dissection is thought to be rare with only three previous cases [1,3,2] reported in the literature. We describe the case of a patient who presented following aortic root dissection late after TOF repair. This 60 year old male had TOF repair at age 14, and was known to have a dilated aortic root. Infrequent (five yearly) transthoracic echocardiograms from 1999 to 2011 showed no progressive aortic root dilation although the ascending aorta was not always well visualized. There was no significant residual valvular disease, outflow tract obstruction or ventricular septal defect. Computed tomography (CT) of the aorta in January 2012 measured a trans-sinus diameter of 53 mm (24 mm/m2; normal b20 mm/m2) and proximal ascending aorta diameter of 49 mm which were similar to measurements obtained on an ascending aortogram a few months later. Apart from being obese (height 173 cm, weight 100 kg), he had no comorbidities and was on no regular medications. He presented in April 2013 with a two week history of new onset class III-IV dyspnea that was preceded by an episode of acute chest pain lasting three hours. Clinically he had left ventricular failure with a loud aortic regurgitant murmur, bilateral pleural effusions and a serum BNP 1400 ng/L (normal b40 ng/L). A CT of the aorta (Fig. 1) showed a trans-sinus diameter of 55 mm but there was now a limited dissection involving the
⁎ Corresponding author. E-mail address: [email protected] (V.A. Wijesekera).
right coronary cusp causing severe aortic regurgitation confirmed by echocardiography. He underwent a Bentall operation. Various hypotheses have been proposed for the mechanism of aortic root dilatation in this group of patients including: increased flow through the aorta due to right to left shunting before repair [4]; embryological defects of spiral septum growth leading to a larger aortic diameter at the expense of the pulmonary artery [5]; and an intrinsic aortopathy related to cystic medial degeneration [5]. The prevalence of aortic root dilation in TOF patients has been reported in a few studies and ranges between 15 and 87% depending on the definitions used [6], and when defined as an observed-to-expected ratio indexed to body surface area and age, was only present in 6.6% of patients [6]. In this case, the indexed observed-to-expected aortic dimension was increased at 1.58. In the previous three reports of aortic dissection in repaired TOF, the aortic dimensions were 71 mm [1], 93 mm [3] and 70 mm [2]; significantly larger than in this case. There is no consensus as to how TOF patients with aortic root dilation should be managed. Intervention when the ascending aorta reaches 55 mm [4] has been suggested as aortic dilatation is associated with increased stiffness and hence increased risk of aortic dissection [7]. The low number of reported cases may suggest that the risk is in fact not that great, however, a significant number of deaths may go unreported. Current Canadian guidelines state that intervention may be considered when the ascending aortic dimension reaches at least 55 mm [8]. When the aortic root is not well visualized by echocardiography, we would strongly advocate for alternate imaging, such as CT or magnetic resonance imaging, to document baseline dimensions and monitor for progressive aortic root dilation. In addition to absolute aortic dimensions, timing of aortic surgery should take into account other factors such as a family history of dissection, the rate of dilation and other planned cardiac surgery. It is important to highlight that this case was a “limited aortic dissection” which is characterized by an intimal tear but no intimal flap or hematoma. In patients with an aortic aneurysm N45 mm with strong clinical suspicion for aortic dissection and a nondiagnostic transesophageal echocardiogram or CT, the clinician should consider a dedicated aortogram to exclude a limited dissection [9]. This case is especially unique in that the dissection occurred at a lower aortic dimension than in previous case reports. In addition, the patient did not have any risk factors for dissection. Furthermore, this was a limited dissection that may not be well visualized by usual
http://dx.doi.org/10.1016/j.ijcard.2014.04.167 0167-5273/Crown Copyright © 2014 Published by Elsevier Ireland Ltd. All rights reserved.
Please cite this article as: Wijesekera VA, et al, Aortic dissection in a patient with a dilated aortic root following tetralogy of Fallot repair, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.04.167
2
V.A. Wijesekera et al. / International Journal of Cardiology xxx (2014) xxx–xxx
imaging such as CT. These observations suggest that although dissection seems to be a rare complication in TOF patients, a high clinical suspicion should always be maintained and pursued depending on the clinical presentation. References [1] Kim WH, et al. Aortic dissection late after repair of tetralogy of Fallot. Int J Cardiol 2005;101(3):515–6. [2] Konstantinov IE, et al. Aortic dissection and rupture in adolescents after tetralogy of Fallot repair. J Thorac Cardiovasc Surg 2010;140(5):e71–3. [3] Rathi VK, et al. Massive aortic aneurysm and dissection in repaired tetralogy of Fallot; diagnosis by cardiovascular magnetic resonance imaging. Int J Cardiol 2005;101(1):169–70. [4] Dearani JA, et al. Management of the aortic root in adult patients with conotruncal anomalies. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:122–9. [5] Yetman AT, Graham T. The dilated aorta in patients with congenital cardiac defects. J Am Coll Cardiol 2009;53(6):461–7. [6] Mongeon FP, et al. Aortic root dilatation in adults with surgically repaired tetralogy of Fallot: a multicenter cross-sectional study. Circulation 2013;127(2):172–9. [7] Cheung YF. Arterial stiffness in the young: assessment, determinants, and implications. Korean Circ J 2010;40(4):153–62. [8] Silversides CK, et al. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Can J Cardiol 2010;26(3):e80–97. [9] Svensson LG, et al. Intimal tear without hematoma: an important variant of aortic dissection that can elude current imaging techniques. Circulation 1999;99(10):1331–6.
Fig. 1. ECG synchronized contrast CT (A, B, and C) shows dilation of the ascending aorta and an eccentric incomplete intimal flap in-keeping with a limited aortic dissection (arrow) above the aortic valve extending to the level of the pulmonary trunk.
Please cite this article as: Wijesekera VA, et al, Aortic dissection in a patient with a dilated aortic root following tetralogy of Fallot repair, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.04.167
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Aortic dissection in a patient with a dilated aortic root following tetralogy of Fallot repair Vishva A. Wijesekera ⁎, Marla C. Kiess, Jasmine Grewal, Rudy Chow, Rekha Raju, Jonathon A. Leipsic, Amanda J. Barlow University of British Columbia, Pacific Adult Congenital Heart Service, St Paul's Hospital, Vancouver, Canada
a r t i c l e
i n f o
Article history: Received 2 April 2014 Accepted 13 April 2014 Available online xxxx Keywords: Aortic dissection Tetralogy of Fallot Aortic aneurysm
With improved longevity, aortic root dilation has been increasingly recognized among patients with tetralogy of Fallot (TOF). Aortic dissection is thought to be rare with only three previous cases [1,3,2] reported in the literature. We describe the case of a patient who presented following aortic root dissection late after TOF repair. This 60 year old male had TOF repair at age 14, and was known to have a dilated aortic root. Infrequent (five yearly) transthoracic echocardiograms from 1999 to 2011 showed no progressive aortic root dilation although the ascending aorta was not always well visualized. There was no significant residual valvular disease, outflow tract obstruction or ventricular septal defect. Computed tomography (CT) of the aorta in January 2012 measured a trans-sinus diameter of 53 mm (24 mm/m2; normal b20 mm/m2) and proximal ascending aorta diameter of 49 mm which were similar to measurements obtained on an ascending aortogram a few months later. Apart from being obese (height 173 cm, weight 100 kg), he had no comorbidities and was on no regular medications. He presented in April 2013 with a two week history of new onset class III-IV dyspnea that was preceded by an episode of acute chest pain lasting three hours. Clinically he had left ventricular failure with a loud aortic regurgitant murmur, bilateral pleural effusions and a serum BNP 1400 ng/L (normal b40 ng/L). A CT of the aorta (Fig. 1) showed a trans-sinus diameter of 55 mm but there was now a limited dissection involving the
⁎ Corresponding author. E-mail address: [email protected] (V.A. Wijesekera).
right coronary cusp causing severe aortic regurgitation confirmed by echocardiography. He underwent a Bentall operation. Various hypotheses have been proposed for the mechanism of aortic root dilatation in this group of patients including: increased flow through the aorta due to right to left shunting before repair [4]; embryological defects of spiral septum growth leading to a larger aortic diameter at the expense of the pulmonary artery [5]; and an intrinsic aortopathy related to cystic medial degeneration [5]. The prevalence of aortic root dilation in TOF patients has been reported in a few studies and ranges between 15 and 87% depending on the definitions used [6], and when defined as an observed-to-expected ratio indexed to body surface area and age, was only present in 6.6% of patients [6]. In this case, the indexed observed-to-expected aortic dimension was increased at 1.58. In the previous three reports of aortic dissection in repaired TOF, the aortic dimensions were 71 mm [1], 93 mm [3] and 70 mm [2]; significantly larger than in this case. There is no consensus as to how TOF patients with aortic root dilation should be managed. Intervention when the ascending aorta reaches 55 mm [4] has been suggested as aortic dilatation is associated with increased stiffness and hence increased risk of aortic dissection [7]. The low number of reported cases may suggest that the risk is in fact not that great, however, a significant number of deaths may go unreported. Current Canadian guidelines state that intervention may be considered when the ascending aortic dimension reaches at least 55 mm [8]. When the aortic root is not well visualized by echocardiography, we would strongly advocate for alternate imaging, such as CT or magnetic resonance imaging, to document baseline dimensions and monitor for progressive aortic root dilation. In addition to absolute aortic dimensions, timing of aortic surgery should take into account other factors such as a family history of dissection, the rate of dilation and other planned cardiac surgery. It is important to highlight that this case was a “limited aortic dissection” which is characterized by an intimal tear but no intimal flap or hematoma. In patients with an aortic aneurysm N45 mm with strong clinical suspicion for aortic dissection and a nondiagnostic transesophageal echocardiogram or CT, the clinician should consider a dedicated aortogram to exclude a limited dissection [9]. This case is especially unique in that the dissection occurred at a lower aortic dimension than in previous case reports. In addition, the patient did not have any risk factors for dissection. Furthermore, this was a limited dissection that may not be well visualized by usual
http://dx.doi.org/10.1016/j.ijcard.2014.04.167 0167-5273/Crown Copyright © 2014 Published by Elsevier Ireland Ltd. All rights reserved.
Please cite this article as: Wijesekera VA, et al, Aortic dissection in a patient with a dilated aortic root following tetralogy of Fallot repair, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.04.167
2
V.A. Wijesekera et al. / International Journal of Cardiology xxx (2014) xxx–xxx
imaging such as CT. These observations suggest that although dissection seems to be a rare complication in TOF patients, a high clinical suspicion should always be maintained and pursued depending on the clinical presentation. References [1] Kim WH, et al. Aortic dissection late after repair of tetralogy of Fallot. Int J Cardiol 2005;101(3):515–6. [2] Konstantinov IE, et al. Aortic dissection and rupture in adolescents after tetralogy of Fallot repair. J Thorac Cardiovasc Surg 2010;140(5):e71–3. [3] Rathi VK, et al. Massive aortic aneurysm and dissection in repaired tetralogy of Fallot; diagnosis by cardiovascular magnetic resonance imaging. Int J Cardiol 2005;101(1):169–70. [4] Dearani JA, et al. Management of the aortic root in adult patients with conotruncal anomalies. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:122–9. [5] Yetman AT, Graham T. The dilated aorta in patients with congenital cardiac defects. J Am Coll Cardiol 2009;53(6):461–7. [6] Mongeon FP, et al. Aortic root dilatation in adults with surgically repaired tetralogy of Fallot: a multicenter cross-sectional study. Circulation 2013;127(2):172–9. [7] Cheung YF. Arterial stiffness in the young: assessment, determinants, and implications. Korean Circ J 2010;40(4):153–62. [8] Silversides CK, et al. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Can J Cardiol 2010;26(3):e80–97. [9] Svensson LG, et al. Intimal tear without hematoma: an important variant of aortic dissection that can elude current imaging techniques. Circulation 1999;99(10):1331–6.
Fig. 1. ECG synchronized contrast CT (A, B, and C) shows dilation of the ascending aorta and an eccentric incomplete intimal flap in-keeping with a limited aortic dissection (arrow) above the aortic valve extending to the level of the pulmonary trunk.
Please cite this article as: Wijesekera VA, et al, Aortic dissection in a patient with a dilated aortic root following tetralogy of Fallot repair, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.04.167
