International Journal of Cardiology xxx (2014) xxx–xxx

IJCA-18068; No of Pages 2

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International Journal of Cardiology

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Letter to the Editor

Aortic dissection in a patientwith a dilated aortic root following tetralogyof Fallot repair

Vishva A. Wijesekera ⁎, Marla C. Kiess, Jasmine Grewal, Rudy Chow, Rekha Raju,Jonathon A. Leipsic, Amanda J. BarlowUniversity of British Columbia, Pacific Adult Congenital Heart Service, St Paul's Hospital, Vancouver, Canada

⁎ Corresponding author.E-mail address: vwijesekera@hotmail.com (V.A. Wijes

http://dx.doi.org/10.1016/j.ijcard.2014.04.1670167-5273/Crown Copyright © 2014 Published by Elsevie

Please cite this article as: Wijesekera VA, etCardiol (2014), http://dx.doi.org/10.1016/j.ij

a r t i c l e i n f o

Article history:

Received 2 April 2014Accepted 13 April 2014Available online xxxx

Keywords:Aortic dissectionTetralogy of FallotAortic aneurysm

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: increasedflow through the aorta due to right to left shunting before repair[4]; embryological defects of spiral septum growth leading to a largeraortic diameter at the expense of the pulmonary artery [5]; and anintrinsic aortopathy related to cystic medial degeneration [5]. Theprevalence of aortic root dilation in TOF patients has been reportedin a few studies and ranges between 15 and 87% depending on the

With improved longevity, aortic root dilation has been increas-ingly recognized among patients with tetralogy of Fallot (TOF).Aortic dissection is thought to be rare with only three previouscases [1,3,2] reported in the literature.We describe the case of a patientwho presented following aortic root dissection late after TOF repair.

This 60 year old male had TOF repair at age 14, and was known tohave a dilated aortic root. Infrequent (five yearly) transthoracicechocardiograms from 1999 to 2011 showed no progressive aorticroot dilation although the ascending aorta was not always well visu-alized. There was no significant residual valvular disease, outflowtract obstruction or ventricular septal defect. Computed tomography(CT) of the aorta in January 2012 measured a trans-sinus diameter of53 mm (24 mm/m2; normal b20 mm/m2) and proximal ascendingaorta diameter of 49 mm which were similar to measurements ob-tained on an ascending aortogram a few months later. Apart frombeing obese (height 173 cm, weight 100 kg), he had no comorbiditiesand was on no regular medications. He presented in April 2013 witha two week history of new onset class III-IV dyspnea that was pre-ceded by an episode of acute chest pain lasting three hours. Clinicallyhe had left ventricular failure with a loud aortic regurgitant murmur,bilateral pleural effusions and a serum BNP 1400 ng/L (normalb40 ng/L). A CT of the aorta (Fig. 1) showed a trans-sinus diameterof 55 mm but there was now a limited dissection involving the

ekera).

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al, Aortic dissection in a patiecard.2014.04.167

right coronary cusp causing severe aortic regurgitation confirmed

definitions used [6], and when defined as an observed-to-expectedratio indexed to body surface area and age, was only present in6.6% of patients [6]. In this case, the indexed observed-to-expectedaortic dimension was increased at 1.58. In the previous three reportsof aortic dissection in repaired TOF, the aortic dimensions were 71mm [1], 93 mm [3] and 70mm [2]; significantly larger than in this case.

There is no consensus as to how TOF patients with aortic root di-lation should be managed. Intervention when the ascending aortareaches 55 mm [4] has been suggested as aortic dilatation is associat-ed with increased stiffness and hence increased risk of aortic dissec-tion [7]. The low number of reported cases may suggest that the riskis in fact not that great, however, a significant number of deaths maygo unreported. Current Canadian guidelines state that interventionmay be considered when the ascending aortic dimension reaches atleast 55 mm [8].

When the aortic root is not well visualized by echocardiography, wewould strongly advocate for alternate imaging, such as CT or magneticresonance imaging, to document baseline dimensions and monitor forprogressive aortic root dilation. In addition to absolute aortic dimen-sions, timing of aortic surgery should take into account other factorssuch as a family history of dissection, the rate of dilation and otherplanned cardiac surgery. It is important to highlight that this case wasa “limited aortic dissection” which is characterized by an intimal tearbut no intimal flap or hematoma. In patients with an aortic aneurysmN45 mm with strong clinical suspicion for aortic dissection and a non-diagnostic transesophageal echocardiogram or CT, the clinician shouldconsider a dedicated aortogram to exclude a limited dissection [9].

This case is especially unique in that the dissection occurred at alower 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

nt with a dilated aortic root following tetralogy of Fallot repair, Int J

Fig. 1. ECG synchronized contrast CT (A, B, and C) shows dilation of the ascending aortaand 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.

2 V.A. Wijesekera et al. / International Journal of Cardiology xxx (2014) xxx–xxx

Please cite this article as: Wijesekera VA, et al, Aortic dissection in a patiCardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.04.167

imaging such as CT. These observations suggest that although dissec-tion seems to be a rare complication in TOF patients, a high clinical sus-picion should always be maintained and pursued depending on theclinical presentation.

References

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[3] Rathi VK, et al. Massive aortic aneurysm and dissection in repaired tetralogy ofFallot; diagnosis by cardiovascular magnetic resonance imaging. Int J Cardiol2005;101(1):169–70.

[4] Dearani JA, et al. Management of the aortic root in adult patients with conotruncalanomalies. 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 ofFallot: a multicenter cross-sectional study. Circulation 2013;127(2):172–9.

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[8] Silversides CK, et al. Canadian Cardiovascular Society 2009 Consensus Conference onthe 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 dis-section that can elude current imaging techniques. Circulation 1999;99(10):1331–6.

ent with a dilated aortic root following tetralogy of Fallot repair, Int J