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 Table of Contents  
Year : 2021  |  Volume : 5  |  Issue : 3  |  Page : 247-249

An Unusually Aggressive Idiopathic Annuloaortic Ectasia

Department of Cardiology, AIIMS, Bhubaneswar, Odisha, India

Date of Submission08-May-2021
Date of Acceptance21-Jun-2021
Date of Web Publication19-Oct-2021

Correspondence Address:
Debasish Das
Department of Cardiology, AIIMS, Bhubaneswar - 751 019, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_11_21

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How to cite this article:
Das D, Acharya D, Das T, Pramanik S. An Unusually Aggressive Idiopathic Annuloaortic Ectasia. J Indian Acad Echocardiogr Cardiovasc Imaging 2021;5:247-9

How to cite this URL:
Das D, Acharya D, Das T, Pramanik S. An Unusually Aggressive Idiopathic Annuloaortic Ectasia. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2021 [cited 2022 Jan 27];5:247-9. Available from: https://www.jiaecho.org/text.asp?2021/5/3/247/328668

Dear Editor,

Annuloaortic ectasia (AAE) is a disorder characterized by aneurysmal dilatation of the ascending aorta with aortic valve regurgitation. Idiopathic AAE is more common in men than in women and appears between fourth and sixth decades.[1] In AAE, the proximal ascending aorta is pear shaped and symmetrically dilated. Aortic valve incompetence in AAE is secondary to dilatation of the aortic wall at the commissural level so that the aortic cusps cannot effectively converge during diastole. This aneurysmal process generally spares the aortic arch. Dissections may occur in the ectatic aorta , and are usually small and limited to the ascending aorta. Aortic rupture in AAE may also occur without dissection.[1] We report an unusually aggressive form of AAE in which the ascending aorta dilated 30 mm within a short span of 2 years, i.e. 15 mm/year which is five times that of the average rate of aortic root dilatation usually reported in Marfanoid aortopathy. It is extremely rare to encounter such aggressive dilatation of ascending aorta across common aortopathies.

  Case Top

A 45-year-old gentleman, an administrative officer in central government, presented to the cardiology outpatient department with New York Heart Association functional class II effort intolerance for the past 2 years. He also had palpitations but no angina, presyncope, or syncope. He did not have diabetes, hypertension, dyslipidemia, or history of smoking. At the time of presentation, he had pulse rate of 82 beats/min with blood pressure of 150/50 mmHg in the right arm supine position with peripheral signs of severe aortic regurgitation (AR). He also had significant cardiomegaly, loud A2, and long early diastolic murmur in aortic area, which was better audible in right parasternum suggestive of severe AR secondary to dilated aorta. He was not having Marfanoid habitus and did not reveal any history of rheumatic fever in childhood; no history was suggestive of any congenital heart disease in the past. Laboratory investigations were unremarkable with normal blood sugar, lipid profile, and renal parameters with erythrocyte sedimentation rate of 17 mm/h with normal C-reactive protein. Venereal Disease Research Laboratory test was also negative. Echocardiographic examination revealed unusual dilatation of the ascending aorta with a diameter of 74 mm [Figure 1], along with aortic annular dilatation and severe AR. The aortic valve was tricuspid [Figure 2]. Left ventricle was hugely dilated with mild left ventricular systolic dysfunction. Echocardiography done at our institute by the same operator 2 years back had revealed the presence of moderate AR with ascending aorta 44 mm in diameter. At that time, he was also evaluated by the ophthalmologist for the presence of ectopia lentis which was absent. The patient did not come for follow-up for the next 2 years due to the ongoing coronavirus disease crisis. During this period, the ascending aorta diameter increased markedly, from 44 mm to 74 mm, i.e., 15 mm/year which is very unusual to encounter across common aortopathies in routine clinical practice. The patient was on combination therapy with losartan and metoprolol in this aforesaid period of 2 years. He was advised to immediately undergo Bentall's repair to curtail the risk of aortic dissection and rupture.
Figure 1: Dilated ascending aorta of 74 mm

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Figure 2: Tricuspid aortic leaflet in annuloaortic ectasia

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  Discussion Top

In 1961, Ellis et al.[1] proposed the concept of AAE to denote aneurysmal dilatation of the proximal ascending aorta and aortic annulus. The term AAE is usually restricted to cases of idiopathic cystic medial degeneration (CMD) of the aorta.[2] Medial degeneration leads to weakening of the aortic root with dilatation and subsequent aneurysm formation. Ascending aorta looks pear shaped in AAE. Idiopathic AAE is twice as common in men than in women and typically presents between fourth and sixth decade. In the past, the tendency to group idiopathic AAE and Marfan's syndrome together emanated from the difficulty of clearly separating the two entities clinically. Many genetic disorders causing AAE besides Marfan's are now recognized along with culprit mutations. Echocardiographic features of AAE are marked dilation of the aortic root with a unique posterior movement (“paradoxical” motion) of the posterior aortic wall during the early-middle ejection period in 75% of the patients and premature systolic partial closure of the aortic valve[3] causing AR. Patients with AAE may have aortic dissection, aneurysm, or rupture.[4] Other less common causes behind AAE are inflammatory diseases such as Takayasu aortitis, giant cell aortitis,[5] bacterial infections like syphilitic or luetic aortitis,[6] and fungal aortitis. Aneurysms from atherosclerosis have also been described in the ascending aorta but AAE usually does not occur. AAE is also encountered in connective tissue disorders such as Ehlers–Danlos[7] and Loeys–Dietz syndromes.[8] Garcia-Macedo et al.[6] reported a group of 27 patients with AAE and found that 23 (85.2%) cases were due to cystic medial necrosis, 3 (11.1%) were due to chronic aortic dissection, and 1 (3.7%) was due to syphilitic aortic aneurysm. Bicuspid aortic valve and coarctation of aorta also cause aortic root dilatation. Aortic expansion rate is higher in bicuspid aortic valve (0.19 cm/year) than in normal tricuspid aortic valve (0.13 cm/year). Systemic hypertension is an important contributor to aortic dilatation due to hypertension-induced wall stress.

Aortic dissection is the second most common complication of AAE after AR. Aortic diameter is the principal deciding factor behind the development of aortic dissection in AAE. Consensus statement for optimal timing for surgery in ascending aorta aneurysm is when the aortic diameter exceeds 5.5 cm because a large number of patients with aortic dissection have a diameter of >5.5 cm. Svensson et al.[9] showed that 12.5% of the patients with Marfan's syndrome with aortic dissections had an aortic diameter of <5.0 cm, and 12.5% of patients with bicuspid aortic valve and dissection had an aortic diameter similar to patients with Marfan's syndrome. Patients with Loeys–Dietz syndrome[10] or with a family history of thoracic aortic aneurysm leading to acute aortic dissection develop aortic dissection with minimum aortic enlargement, and it is recommended that patients with a transforming growth factor β-receptor type II[11] mutation should undergo surgical repair of the aortic aneurysm when the diameter reaches 4.2 cm.

Aortic root aneurysms across aortopathies grow at an average of 1–4 mm/year with a faster rate of growth noted in patients with bicuspid aortic valve, Marfan's syndrome, end-stage renal disease, males, and smokers. Annual growth rate more than 5 mm/year warrants immediate surgical intervention in Marfan syndrome.[12] Saeyeldin et al.[13] analyzed the rate of dilatation in Marfanoid aortopathy. The average ascending aorta diameter at presentation was 4.54 ± 1.3 cm (range: 2.2–8.3 cm), and the annual growth rate was estimated to be 0.26 ± 0.05 cm/year. The minimum growth rate was 0.132 cm and the maximum growth rate was 0.355 cm. We encountered this rare case of idiopathic AAE in which the ascending aorta dilated at a rate of 15 mm/year which is quite unusual to encounter in routine clinical practice. Rapid progression of cystic medial necrosis may be the plausible explanation behind this unusually aggressive form of AAE. Although literature describes rapid progression of Marfanoid aortopathy in few individuals, our take-home message is that idiopathic AAE can also be unusually faster progressing in nature warranting minimum quarterly echocardiographic evaluation in a year.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Ellis PR, Cooley DA, DE bakey ME. Clinical considerations and surgical treatment of annulo-aortic ectasia. Report of successful operation. J Thorac Cardiovasc Surg 1961;42:363-70.  Back to cited text no. 1
Yuan SM, Jing H. Cystic medial necrosis: Pathological findings and clinical implications. Rev Bras Cir Cardiovasc 2011;26:107-15.  Back to cited text no. 2
Atsuchi Y, Nagai Y, Komatsu Y, Nakamura K, Shibuya M, Hirosawa K. Echocardiographic manifestation of annuloaortic ectasia: Its “paradoxical” motion of the aorta and premature systolic closure of the aortic valve. Am Heart J 1977;93:428-33.  Back to cited text no. 3
Hirooka Y, Sunamori M, Tanaka A, Suzuki A. Surgical treatment of annulo-aortic ectasia. Jpn Circ J 1982;46:214-24.  Back to cited text no. 4
Evans JM, O'Fallon WM, Hunder GG. Increased incidence of aortic aneurysm and dissection in giant cell (temporal) arteritis. A population-based study. Ann Intern Med 1995;122:502-7.  Back to cited text no. 5
García-Macedo R, Kalil RA, Prates PR, Lucchese FA, Sant'Ana JR, Pereira EM, et al. Tratamento cirúrgico para ectasia ânulo-aórtica. Rev Bras Cir Cardiovasc 1986;1:44-8.  Back to cited text no. 6
Fujii T, Sumiyoshi S, Koga T, Nishizaka M, Matsukawa R, Kuwano H, et al. An autopsy case report of annuloaortic ectasia with cardiac tamponade ruptured from an aneurysm of the right Valsalva sinus. Pathol Res Pract 2007;203:671-5.  Back to cited text no. 7
Yamamoto M, Iguchi M, Yamamoto M, Hiroi M. Loeys-Dietz syndrome with aortic wall necrosis secondary to external wrapping. Eur J Cardiothorac Surg 2018;53:1288-9.  Back to cited text no. 8
Svensson LG, Adams DH, Bonow RO, Kouchoukos NT, Miller DC, O'Gara PT, et al. Aortic valve and ascending aorta guidelines for management and quality measures. Ann Thorac Surg 2013;95:S1-66.  Back to cited text no. 9
Loughborough WW, Minhas KS, Jonathan CL, Rodrigues JC, Lyen SM, Helen E, et al. Cardiovascular manifestations and complications of loeys-dietz syndrome: CT and MR imaging findings. Vasc Int Radiol 2018;38:275-85.  Back to cited text no. 10
Loeys BL, Chen J, Neptune ER, Judge DP, Podowski M, Holm T, et al. A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet 2005;37:275-81.  Back to cited text no. 11
Aburawi EH, O'Sullivan J. Relation of aortic root dilatation and age in Marfan's syndrome. Eur Heart J 2007;28:376-9.  Back to cited text no. 12
Saeyeldin A, Zafar MA, Velasquez CA, Ip K, Gryaznov A, Brownstein AJ, et al. Natural history of aortic root aneurysms in Marfan syndrome. Ann Cardiothorac Surg 2017;6:625-32.  Back to cited text no. 13


  [Figure 1], [Figure 2]


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