Babu-Narayan, SV

Beurtheret, S

Bonello, B

Burman, ED

Diller, GP

Gatzoulis, MA

Keegan, J

Kilner, PJ

Pennell, DJ

Shiina, Y

Shore, DF

Swan, L

Uebing, A

JACC Cardiovascular Imaging

Spiral - Imperial College Digital Repository

Aortic dilatation in repaired tetralogy of Fallot: features, determinants and  progression*Beatrice Bonello, MD1 *Darryl F. Shore, FRCS1 Anselm Uebing MD,1 Gerhard-Paul Diller, MD, PhD1 Jennifer Keegan PhD,1 Elizabeth Burman,1 Yumi Shiina, MD,1 Lorna Swan, MD1  Dudley J. Pennell MD, PhD1,2  Philip J. Kilner MD, PhD, 1,2  Sylvain Beurtheret, MD1 Michael A. Gatzoulis, MD, MRCPH, PhD 1,2 **Sonya V. Babu-Narayan, MB BS, BSc, FRCP, PhD1,21 Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom2 NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College LondonRunning title: Aorta dilatation in tetralogy of Fallot* The 2 first authors contributed equally to the work**Address for correspondence:Dr Sonya Babu-Narayan MB BS, BSc, FRCP, PhD, FESCBritish Heart Foundation Intermediate Clinical Research Fellow,Consultant Cardiologist and Clinical Senior Lecturer in Adult Congenital Heart DiseaseNIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, Sydney Street, London, SW3 6NPPhone: 00 44 207 351 8803, Fax: 00 44 207 351 8816Email: s.babu-narayan@rbht.ac.uk; s.babu-narayan@imperial.ac.ukFunding and Relationship with Industry: Beatrice Bonello was supported by the French Federation of Cardiology. Sonya V. Babu-Narayan is supported by the British Heart Foundation (FS/11/38/28864). This project was supported by the NIHR Cardiovascular Biomedical Research Unit of Royal Brompton and Harefield NHS Foundation Trust and Imperial College London. This report is independent research by the National Institute for Health Research Biomedical Research Unit Funding Scheme. The views expressed in this publication are those of the author(s) and not necessarily those of the NHS, the National Institute for Health Research or the Department of Health.Conflict of interest: NoneWord Count: 800Dear Editor,Although the high prevalence of aortic root dilatation in adults with rTOF is well established (1,2), evidence to guide clinical follow-up and decision-making remains sparse.We sought to define the features, determinants and rate of progression of aortic dilatation in adults with repaired tetralogy of Fallot (rTOF) using cardiovascular magnetic resonance (CMR).  We retrospectively identified adults with rTOF who had 2 interval CMR scans. Aortic dimensions were measured at sinus, sinotubular junction (STJ) and mid-ascending aortic level at both timepoints blinded to scan order and other clinical data. Dilatation was defined as diameter >2 standard deviations larger than our published normal CMR aortic dimensions adjusted for age (3). One-hundred-and-ten patients (57 male, median age 30.9 [IQR 22.9-39.4] years) were retrospectively studied. One patient with aortic valve endocarditis requiring aortic valve surgery was excluded. Forty had a shunt prior to repair, median age at repair was 4.5 [2.1-9.2] years, 14 were repaired before 1 year of age, 9 had pulmonary atresia, 24 right-sided aortic arch and 11 were successfully treated for systemic hypertension. Twenty-nine (27%) had mild and 6 (5%) had moderate aortic regurgitation. Seventy-six (69 %) patients had aortic dilatation. Dilatation was present in 30 (27%) at sinus level, in 73 (66%) at STJ level, and in 24 (21%) at ascending aortic level. Thirty five (31%) had normal aortic dimensions (Figure, panel A).  Patients repaired before 1 year of age were less likely to have aortic dilatation at any level compared to the remainder (p=0.001). Male gender and history of palliative shunt were independent predictors of aortic dilatation at any level (p<0.0001 and p=0.023, respectively) and were independent predictors of STJ dilatation (p=0.0001 and p=0.033, respectively). Male gender and pulmonary atresia were independent predictors of aortic sinus dilatation (p=0.008 and p=0.0009, respectively). Male gender, later repair and pulmonary atresia were independent predictors of ascending aortic dilatation (p=0.008, p=0.006 and p=0.0004, respectively). During a median interval of 6.3 [IQR5.1-7.6] years, aortic diameters increased in 47% patients (25% at sinus, 21% at STJ and 35% at ascending aortic level) at rates between ~0.2-0.4 mm/year (Figure, panel B).  Even amongst patients with sinus diameter ≥45 mm at baseline (n=5) there was no increase.Predictors of aortic diameter increase at STJ level were older age, later repair and right aortic arch. No predictors of aortic diameter increase at other levels were ascertained. There were no aorta-related events during follow-up. Aortic regurgitation progressed from mild to moderate in only 2 patients without progressive aortic dilatation.In conclusion, our data show that aortic dilatation is common, most frequently at STJ level (97% of patients with dilated aorta).  Aortic dimensions increased in approximately 50% patients during a 6 year follow-up, most commonly in the ascending aorta, but with reassuringly low rates of progression. Previous studies have reported aortic root dilatation in rTOF (1,2). We also found associated ascending aortic dilatation in 21% of the patients. Risk factors for aortic dilatation were similar to those previously reported (1,2) and are those that lead to increased volume overload of the aorta prior to repair. The combination of these with the intrinsically abnormal aortic vessel wall (4) may contribute to aortic dilatation. With earlier surgical repair the importance of aortic dilatation may decrease.We demonstrated a very low rate of aortic diameter progression comparable with known age-related increase in normal volunteers. Recent consensus recommendations suggest replacement of the ascending aorta when its diameter is at least 55 mm (5). Our datawould not support a more aggressive approach or very frequent aortic assessment with CMR.1. 	Mongeon FP, Gurvitz MZ, Broberg CS et al. Aortic root dilatation in adults with surgically repaired tetralogy of Fallot: a multicenter cross-sectional study. Circulation 2013;127:172-179.2. 	Niwa K, Siu SC, Webb GD, Gatzoulis MA. Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation 2002;106:1374-1378.3. 	Burman ED, Keegan J, Kilner PJ. Aortic root measurement by cardiovascular magnetic resonance: specification of planes and lines of measurement and corresponding normal values. Circ Cardiovasc Imaging 2008;1:104-13.4.	Chowdhury UK, Mishra AK, Ray R, Kalaivani M, Reddy SM, Venugopal P. Histopathologic changes in ascending aorta and risk factors related to histopathologic conditions and aortic dilatation in patients with tetralogy of Fallot. J Thorac Cardiovasc Surg 2008;135:69-77.5. 	Hiratzka LF, Bakris GL, Beckman JA et al. ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. J Am Coll Cardiol 2010;55:e27-e129.Figure Aortic diameters and rate of progression in 110 repaired tetralogy of Fallot patients during median follow-up 6.3 years studied with CMRPanel A:  Patient aortic diameters against expected age-adjusted value illustrated by the grey zone of 2 standard deviations from our published mean. Panel B: Percentage of patients (black boxes) with aortic progression and the rate of diameter increase (blue line representing median progression) amongst patients that progress.  

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Aortic Dilatation in Repaired Tetralogy of Fallot.

Aortic Dilatation in Repaired Tetralogy of Fallot.

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