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CASE REPORT
Year : 2013  |  Volume : 23  |  Issue : 4  |  Page : 102-105

Giant proximal aorta aneurysm: A successfully managed time bomb


Department of Medical Science, Division of Internal Medicine, Hypertension Unit, San Giovanni Battista Hospital, University of Torino, Torino, Italy

Date of Web Publication20-Feb-2014

Correspondence Address:
Alberto Milan
Department of Medical Science, Division of Internal Medicine, Hypertension Unit, San Giovanni Battista Hospital, University of Torino, Via Genova 3, Torino
Italy
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2211-4122.127411

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  Abstract 

Management of aortic aneurismatic disease is often care of specialists, from vascular to cardiac surgeons. However, initial diagnosis and management are not unfrequently responsibility of an emergency staff as the disease presentation may be dramatically acute. Thoracic aortic aneurysms (TAA) in particular have a silent clinical history until they become evident with dissection or rupture with a high global mortality rate. The importance of a rapid diagnosis and of correct management in such a subsetting is clear, but recent guidelines where published with the declared rationale of emphasizing the importance of an early detection of the disease. The goal is to reduce morbidity and mortality and improvement of quality of life of such patients. We present a case of successfully managed asymptomatic giant proximal aortic aneurysm in a healthy young man. On a routine transthoracic echocardiogram, severe dilatation of the proximal aorta was detected, with severe aortic regurgitation in a normal tricuspid valve determining left ventricle (LV) dilatation and impaired contractility. Computed tomography scan was scheduled, confirming the findings and open heart surgery performed within 1 week. Clinical and echocardiographical follow-up was started; after 2 months imaging studies showed good surgical results with well-functioning, non-regurgitant prosthetic aortic valve and initial recovery of left ventricular dilatation; at the last control, 14 months later, LV mass and dimensions where markedly improved, with no more signs of hypertrophy nor dilatation. TAA needs a rapid diagnosis and appropriate management. Clinicians should be aware of proper diagnostic tools and of applicable therapeutic strategies in order to grant the better assistance to the patient. In this setting, the role of echocardiography remains pivotal.

Keywords: Aortic aneurysm, aortic regurgitation, cardiac murmur, echocardiography


How to cite this article:
Milan A, Avenatti E, Naso D, Veglio F. Giant proximal aorta aneurysm: A successfully managed time bomb. J Cardiovasc Echography 2013;23:102-5

How to cite this URL:
Milan A, Avenatti E, Naso D, Veglio F. Giant proximal aorta aneurysm: A successfully managed time bomb. J Cardiovasc Echography [serial online] 2013 [cited 2019 May 19];23:102-5. Available from: http://www.jcecho.org/text.asp?2013/23/4/102/127411


  Introduction Top


Management of aortic aneurismatic disease is often care of specialists, from vascular to cardiac surgeons depending on anatomical characteristics of the lesion. However, initial diagnosis and management are not unfrequently responsibility of an emergency staff as the disease presentation may be dramatically acute. Latest available data state that aortic disease account for 45,000 death every year in the United States; [1] thoracic aortic aneurysm (TAA) in particular has a silent clinical history until they become evident with dissection or rupture [2] with a global mortality rate that by far exceed the one of abdominal aortic aneurysm. [3] The importance of a rapid diagnosis without delay and of the correct management in such a subsetting is clear but recent guidelines [3] were published with the declared rationale of emphasizing the importance of early detection of the disease for reduction of morbidity and mortality and improvement of quality of life of such patients.

We present a case of successfully managed asymptomatic giant proximal aortic aneurysm in a healthy young man. On a routine transthoracic echocardiogram, severe dilatation of the proximal aorta was detected, with severe aortic regurgitation in a normal tricuspid valve determining left ventricle (LV) dilatation and impaired contractility. Computed tomography (CT) scan was scheduled, confirming the findings and open heart surgery performed within 1 week. Clinical and echocardiographical follow-up was started; after 2 months imaging studies showed good surgical results with well-functioning, non-regurgitant prosthetic aortic valve and initial recovery of left ventricular dilatation; at the last control, 14 months later, LV mass and dimensions where markedly improved, with no more signs of hypertrophy nor dilatation.

TAA needs a rapid diagnosis and appropriate management. Clinicians should be aware of proper diagnostic tools and of applicable therapeutic strategies in order to grant the better assistance to the patient. In this setting, the role of echocardiography remains pivotal.


  Case Report Top


A 35-year-old man was referred to our outpatient clinic for an echocardiographic exam due to the detection of a murmur by his general practitioner. He was in excellent global clinical condition, was playing judo in a semi-professional fashion and reported no relevant signs or symptoms. His past clinical history was completely silent. On physical examination, the only relevant finding was actually a well-audible - 4-5/6 - holodiastolic murmur, with a decrescendo, better heard at the basal left sternal edge.

Transthoracic echocardiography was then performed; parasternal long axis view [Figure 1] showed a giant aneurysm of the ascending aorta involving Sinuses of Valsalva (75 mm) with the disappearance of SinoTubular junction and extending up to the aortic arch, which had normal dimensions (32 mm). Thoracic aorta was normal in size in explorable segments. The aortic valve was clearly tricuspid on short axis view [Figure 2]b. Severe aortic regurgitation was present [Figure 2]a, not determining functional mitral stenosis. LV showed to have mildly impaired global contractility (ejection fraction 50%) without segmental hypo-or a-kinesia, eccentric hypertrophy with markedly increased cavity dimensions - both linear and volumetric measures, with a telesystolic and telediastolic volumes of 145 and 296 cc (145 cc/m 2 ) respectively. Left atrium was moderately enlarged; right chambers were normal in dimensions and function.
Figure 1: Silent giant aortic aneurysm: Parasternal long axis showing severely dilated aortic root

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Figure 2: Severe aortic regurgitation in parasternal long axis (a) with a normal tricuspid aortic valve (b)

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The patient was promptly scheduled for a CT scan that confirmed the echocardiographic data and subsequent cardio-surgery evaluation. Open heart surgery was performed within 1 week, with surgery on both aortic valve and ascending aorta - substitution of damaged structures with Carbomedics valvular tube 27/30. The post-surgical course was complicated by onset of atrial fibrillation, successfully treated with intravenous amiodarone and post procedural pericarditis, treated with non-steroidal anti-inflammatory drugs.

Clinical and echocardiographical follow-up was started; after 2 months imaging showed good surgical results with well-functioning, non-regurgitant prosthetic aortic valve and initial recovery of left ventricular dilatation; minimal pericardial effusion was detectable.

At the last control, 14 months later [Figure 3] and [Figure 4] LV mass and dimensions where markedly improved, with no more signs of hypertrophy nor dilatation; pericardium was normal. The patient is in excellent clinical condition, without recurrence of arrhythmic episodes nor thoracic pain.
Figure 3: Follow-up after surgical repair: Parasternal long axis view pre (a) and post cardiosurgery (b)

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Figure 4: Follow-up after surgical repair: Comparison of ejection fraction computed by Simpson rule on an apical four chamber view pre (a) and post (b) cardiosurgery

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  Discussion Top


Studies on natural history of the TAA have shown how the major cause of death in such patients is represented by complications-rupture or dissection. Moreover, as previously reminded, patients are often completely asymptomatic until such catastrophic events. Rupture risk is directly related, with a strong linear correlation [2] to aneurysm dimensions. Recent guidelines [3] indicate at 5.5 cm the cut off values of the ascending aorta or sinuses of Valsalva for cardio surgical evaluation in asymptomatic patients and lower cutoffs for patients affected by congenital syndromes correlated to TAA such as Marfan, Ehler-Danlos Syndrome or connective tissue diseases.

On the other hand, in the clinical scenario of a chronic presentation, the first sign of aortic root dilatation is often a murmur indicating aortic regurgitation, as it happened in our case. It is an old acquaintance in fact that aortic root dilatation is the major cause of aortic insufficiency, exceeding in prevalence any other valvulopathy [4] and studies have related severity of aortic regurgitation linearly to aortic root dimensions. [5] Presence of aortic regurgitation leads in time to left ventricular hypertrophy, dilatation and eventually to left ventricular disfunction. Chronic aortic regurgitation represent actually a situation of both volume and pressure overload: LV responds to regurgitant flow with increased compliance and concentric and eccentric hypertrophy. These modifications grant increased end diastolic volume that maintains normal filling pressure and trough Frank Starling mechanism, increased cardiac output and normal forward-flow even in the presence of severe aortic regurgitation. With disease progression, compensating mechanisms become progressively insufficient: hypertrophic rearrangement may become inadequate and from such a point on, any further increase in preload will imply systolic function impairment. Current Guidelines for valvular heart disease underline as crucial for surgical decision timeline evaluation of LV disfunction (surgical option as class I recommendation for depressed LV systolic function) and dimensions (Class IIa for enlarged LV). [6]

In our case, intervention was obviously mandatory and urgent, due to the high risk of rupture related to the giant dimensions of the aneurism; left ventricular dilatation was present as a sign of chronic regurgitation that had led to structural remodeling. Beside obvious treatment of the aneurysm and prevention of rupture risk, cardio surgery granted a recovery of left ventricular dysfunction consequent to aortic regurgitation. Efficacy of surgical valve replacement was demonstrated by echocardiographic follow-up that showed progressive improvement of left ventricular function and dimensions, until basically normalized findings after 14 months.

Even though echocardiography is not considered the better choice in the recently published guidelines, [3] non-invasive imaging techniques may play a fundamental role in TAA diagnosis, evaluation of complication and subsequent follow-up.

 
  References Top

1.Svensson LG, Rodriguez ER. Aortic organ disease epidemic, and why do balloons pop? Circulation 2005;112:1082-4.  Back to cited text no. 1
    
2.Elefteriades JA. Natural history of thoracic aortic aneurysms: Indications for surgery, and surgical versus nonsurgical risks. Ann Thorac Surg 2002;74:S1877-80.  Back to cited text no. 2
    
3.Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE Jr, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. J Am Coll Cardiol 2010;55:e27-129.  Back to cited text no. 3
    
4.Roman MJ, Devereux RB, Niles NW, Hochreiter C, Kligfield P, Sato N, et al. Aortic root dilatation as a cause of isolated, severe aortic regurgitation. Prevalence, clinical and echocardiographic patterns, and relation to left ventricular hypertrophy and function. Ann Intern Med 1987;106:800-7.  Back to cited text no. 4
    
5.Alegret JM, Vernis JM, Palazón O, Borràs X, Duran I. Factors related to aortic regurgitation in the presence of a dilated aortic root. Am J Cardiol 2005;95:417-20.  Back to cited text no. 5
    
6.Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr, Faxon DP, Freed MD, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008;118:e523-661.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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