Eur J Cardiothorac Surg 2008;34:690-692. doi:10.1016/j.ejcts.2008.05.044
Copyright © 2008, European Association for Cardio-thoracic Surgery. Published by Elsevier. All rights reserved.
Acquired supravalvular aortic stenosis: a late complication of replacement of the ascending aorta
Andrew J. Turleya,*,
John Darkb,
Philip C. Adamsa
a Department of Cardiology, Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne NE1 4LP, United Kingdom
b Department of Cardiothoracic Surgery, Freeman Hospital, High Heaton, Newcastle upon Tyne NE7 7DN, United Kingdom
Received 31 December 2007;
received in revised form 18 May 2008;
accepted 21 May 2008.
* Corresponding author.
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Abstract
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Aortic syndromes are an increasing cause of morbidity and mortality. Ascending aortic dissection is a clinical emergency with most patients requiring open surgery to replace the ascending aorta. Detection through clinical suspicion, improved non-invasive imaging and refined surgical techniques have resulted in an improved survival rate. Acquired supravalvular aortic stenosis is an extremely rare complication of cardiac surgery. We present the case of a patient who, 15 years after undergoing elective replacement of the ascending aorta for aortic dissection, required repeat surgery for symptomatic supravalvular aortic stenosis. This case elegantly highlights the need for a detailed focused assessment in patients where the clinical presentation does not correlate with initial investigations. To our knowledge this is the first reported case of late symptomatic supravalvular aortic stenosis following replacement of the ascending aorta.
Key Words: Acute ascending aortic dissection • Acquired supravalvular aortic stenosis • Ascending aorta • Graft • Complication
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1. Introduction
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Acquired supravalvular aortic stenosis (SVAS) is an extremely rare complication of cardiac surgery. We present the case of a patient who, 15 years after undergoing elective replacement of the ascending aorta for aortic dissection, required repeat surgery for symptomatic supravalvular aortic stenosis. To our knowledge this is the first reported case of late symptomatic supravalvular aortic stenosis following replacement of the ascending aorta.
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2. Case report
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In 1990 a 53-year-old male presented with severe lower abdominal pain radiating to his back. He was hypertensive and a chronic smoker. Physical examination was unremarkable; in particular there were no murmurs. A CT scan established aortic dissection from root to bifurcation. Referral to the regional cardiothoracic centre occurred 10 days after hospital admission. Following blood pressure control the patient became asymptomatic and was discharged home.
At review he complained of chest discomfort and a new early diastolic murmur was heard. Following an MRI scan the patient underwent elective replacement of the ascending aorta using a 26 mm DeBakey collagen impregnated graft. On exploration the dissection had extended down the arch although the distal false lumen was occluded with thrombus. The top-end of the graft was cut obliquely and anastamosed just below the innominate artery and extending on the underside of the arch to approximately half way down the arch. The aortic valve was re-suspended. The patient made an uneventful recovery.
A decade later the patient was re-referred after the incidental finding of atrial fibrillation. Examination revealed an early diastolic murmur, loud ejection systolic murmur and a thrill over the upper precordium. Transthoracic echocardiography showed a moderately dilated aortic root with aortic regurgitation secondary to aortic cusp coaptation failure. There was no aortic stenosis. The patient was asymptomatic and therefore discharged.
Almost 15 years after the original operation and after a complex illness leading to several emergency hospital admissions he was re-investigated. The patient described progressively worsening dyspnoea (NYHA II/III) and orthopnoea. Echocardiography from the right upper parasternal region revealed a broad high velocity systolic jet (4.47 m/s, 80 mmHg gradient) in the aorta at the superior end of the graft (Fig. 1a). There was also low velocity diastolic flow reversal consistent with aortic regurgitation. Left ventricular systolic function was mildly impaired. Three-dimensional reconstruction of a thoracic CT acquired during a previous admission confirmed stenosis at the top of the graft (Fig. 2
). Cardiac catheterisation demonstrated a 46 mmHg peak–peak gradient within the ascending aorta (Fig. 1b), moderate aortic regurgitation and non-obstructive coronary artery disease. The diagnosis was acquired supravalvular aortic stenosis.
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Fig. 1. (a and b) Transthoracic echocardiography performed from the right upper parasternal region. Continuous wave (CW) Doppler measurement in the aorta at the superior end of the ascending aortic graft revealed a broad systolic jet of high velocity (4 m/s). Cardiac catheterisation demonstrating a 46 mmHg peak–peak gradient within the ascending aorta. Ao: aorta, LV: left ventricle.
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Fig. 2. Acquired supravalvular aortic stenosis. Contrast enhanced sagittal CT scan of the thorax. Curved maximum intensity projection (MIP) view demonstrating stenosis at the top of the ascending aortic graft. Ao: aorta, LV: left ventricle, DAo: descending aorta.
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Despite escalating doses of diuretics the patient became increasingly symptomatic. At surgery there was annular and sino-tubular dilatation, a tricuspid aortic valve with a large degree of regurgitation and significant scarring around the graft anastamosis surrounding the innominate vessels. The top-end of the graft appeared kinked. The patient underwent repeat replacement of the ascending aorta and hemi-arch (Vascutek graft) plus bioprosthetic aortic valve replacement (25 mm Perimount). Following a protracted recovery period secondary to respiratory, renal and gastrointestinal complications he is currently well and under annual surveillance for an asymptomatic infra-renal aortic aneurysm.
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3. Discussion
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Aortic syndromes are an increasing cause of morbidity and mortality. Ascending aortic dissection is a clinical emergency with most patients requiring open surgery to replace the ascending aorta. Detection through clinical suspicion, improved non-invasive imaging and refined surgical techniques have resulted in improved survival rates [1,2].
Our report highlights the potentially fatal complication of acquired SVAS after replacement of the ascending aorta over a decade earlier. The majority of cases of SVAS are congenital as part of William's syndrome, although isolated acute acquired cases have been reported [3–5]. Acquired SVAS is extremely rare. Identified aetiologies include aortic kinking due to redundant donor aortic tissue following cardiac transplantation, inwardly indented aortotomy wounds secondary to multiple aortic cannulations and peri-prosthetic psuedoaneurysm formation following replacement of the ascending aorta [3,4]. The most likely aetiology in this case was kinking of a too long ascending aortic graft.
It is estimated that a third of patients surviving the initial treatment for acute dissection will experience dissection extension, aortic rupture, or aortic aneurysm formation within 5 years of presentation necessitating consideration of repeat intervention [2]. Modern endovascular techniques can treat selected patients with abdominal aortic disease, descending aortic dissections or recoarctation following previous coarctation surgery [6]. Percutaneous treatment was briefly considered but due to the location of the SVAS, its proximity to both the head and neck vessels and the graft this was felt unwise and the patient underwent open surgical repair. In patients with congenital SVAS long-term results of surgical treatment are excellent with 91% survival at 20 years. Univariate analysis identifies a LV aortic gradient >34 mmHg as a risk for repeat surgery [7]. In our patient the peak to peak gradient at the time of surgery was 46 mmHg.
SVAS may contribute to the clinical picture in patients following cardiac surgery. Our patient presented with increasing breathlessness on the background of a progressive deterioration in functional capacity. The combination of the patient's signs and symptoms were inconsistent, prompting a more detailed echocardiographic assessment of the patient's aortic root and ascending aorta. This case highlights this extremely rare but life-threatening late complication of aortic surgery and elegantly highlights the need for a detailed focused assessment in patients where the clinical presentation does not correlate with initial investigations.
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Acknowledgments
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We are grateful to Dr T. Irvine, consultant cardiologist and Dr L. Mitchell, consultant radiologist for their help in the preparation of this manuscript.
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References
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Abstract
1. Introduction
