© 2004 by European Society of Cardiology
Copyright © 2003, The European Society of Cardiology
Aortic valve regurgitation due to cusp aneurysm: a case report
aDepartment of Cardiology, Onze Lieve Vrouwe Gasthuis, P.O. Box 95500, 1090 HM Amsterdam, The Netherlands
bDepartment of Cardiology, Leids Universitair Medisch Centrum, The Netherlands
cDepartment of Cardiology, Thoraxcenter Rotterdam, The Netherlands
Received 3 July 2003; received in revised form 25 September 2003; accepted after revision 25 September 2003.
* Corresponding author. Tel.: 31-20-5993032; fax: 31-20-5993997. a.j.h.a.scholte{at}olvg.nl
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Abstract |
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 Top Abstract 1 Case report2 DiscussionReferences |
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Two-dimensional echocardiography is a valuable tool in visualizing and monitoring aortic valve and root abnormalities. We present a rare case of a patient with massive aortic regurgitation due to cusp aneurysm, which was accurately diagnosed by echocardiography and treated by valve replacement. A complicated course with recurrent aneurysms of the aortic wall after aortic valve replacement was remarkable in this case. Although different possible etiologies could not be determined, endocarditis and/or aortitis may be the most likely explanation of the complicated and finally fatal course of this patient.
Keywords: echocardiography; aortic regurgitation; aortic aneurysm; endocarditis
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1 Case report |
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TopAbstract1 Case report2 DiscussionReferences |
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A 49-year-old woman was admitted to the hospital following a 3-week period of progressive dyspnea accompanied by acute orthopnea with chest pain. The blood pressure measured 180/60 mmHg and the pulse was regular with a rate of 112 beats per minute. The resting ECG showed a sinus tachycardia and left ventricular hypertrophy. Auscultatory examination of the chest revealed a diastolic murmur at the left sternal border. Transthoracic echocardiography was performed and demonstrated a moderately dilated left ventricle (LVEDD 63 mm, LVESD 47 mm) with a grade 4 aortic regurgitation revealed by color Doppler echocardiography. Malcoaptation of the aortic valve due to a remarkable diastolic bulging of the left coronary cusp (Fig. 1) resulted in an eccentric aortic regurgitation. The clinical history was not suspect for endocarditis. Multiplane transesophageal echocardiography was performed and showed aortic root dilatation (annulus 27 mm, sinus 45 mm, sino-tubular junction 43 mm); the severe aortic regurgitation was confirmed (Fig. 2) together with the aneurysm of the left coronary cusp (Fig. 3). The patient was scheduled for surgery. At intra-operative inspection a dilated ascending aorta (46 mm) was observed with a thickened and inflammatory aspect of the wall of the aorta. The left coronary cusp was severely deformed with a prolapse and aneurysmatic dilatation of the leaflet. The aortic annulus itself showed a defect with a diameter of 1 cm, suggestive for an old abscess, possibly due to endocarditis. However, no vegetations were observed during surgery and (blood) cultures remained negative.
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Surgical replacement of the aortic valve by a mechanical valve (Carbomedics 27 mm) with closure of the defect was performed without complications.
Histo-pathological examination of the native aortic valve showed mucoid degeneration and focal sclerosis without signs of inflammation. The initial recovery was uneventful, but four months after surgery the patient again complained of progressive shortness of breath and orthopnea.
The ECG showed marked changes, consisting of a new 1st degree AV block (0.26 s) with a new interventricular block of 0.12 s and ST-T segment changes.
Echocardiography showed a dilated aortic root of 52 mm with an aneurysm of the ascending aorta impressing the left atrium (Figs. 4 and 5
). A diastolic flow pattern between the aorta and the aneurysm was also observed.
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Three days later re-operation showed a more than 6 cm dilated ascending aorta. The three sinuses of Vasalva were expanded enormously and the ostium of the right coronary cusp was displaced to 7 cm above the mechanical valve. Between the mechanical aortic valve and the left main coronary artery a defect of 4 mm in diameter was present. A Bentall procedure with implantation of a St Jude 27 mm mechanical valve was performed. Repeat transesophageal echocardiography showed a grade 4 mitral regurgitation, possibly due to the fixation of the Bentall prosthesis into the septum of the left ventricle near the anterior leaflet of the mitral valve. After restrictive annuloplasty with a 26 mm Physio-ring and a commisuroplasty, a grade 1 mitral regurgitation remained.
Pathological examination of the aortic root showed extensive degeneration with atherosclerosis and chronic focal inflammation, but no signs of systemic diseases. Again, no microorganism was cultured.
Implantation of a DDD pacemaker was necessary because of the development of a total AV-block, but otherwise the patients' recovery was uneventful. Six months thereafter the patient presented again in the emergency room because of chest pain radiating to the shoulders and back accompanied with nausea and vomiting. The ECG showed sinus rhythm with right ventricular pacing and significant new ST-T segment elevation in lead AvR, and ST-T segment depression in the lateral leads. Since plasma levels of troponine-T were elevated (0.12 µg/L, normal reference < 0.01 µg/L) coronary angiography was performed. Significant luminal narrowing in both the proximal right and left coronary artery was noted, which was possibly due to pressure on the origin of both coronary arteries (Figs. 6 and 7).
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Both contrast angiography and echocardiography of the aortic root showed again a false aneurysm and two connections between the aortic root and the aneurysm (Fig. 7). Both connections were possibly located just below the newly stitched coronary buttons. A third operation of the aorta was planned but could not be performed due to progressive shock followed by untimely death.
At autopsy the aortic root showed a circumferential aneurysm of 5 cm in diameter between the pre-existent aorta and the outer wall of the Bentall prosthesis. This aneurysm impressed the origin of the coronary arteries, resulting in almost complete occlusion of both proximal segments of the coronary arteries.
A defect at the side of the button of the left coronary artery was responsible for the aneurysm. Except for a recent myocardial infarction with extension into both papillary muscles no sign of systemic inflammatory disease of the aortic wall could be detected.
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2 Discussion |
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TopAbstract1 Case report2 DiscussionReferences |
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Primary disease of either the aortic valve leaflets or the wall of the aortic root or both may cause aortic regurgitation. In a large series of 218 consecutive patients with aortic regurgitation, none of the patients presented with aortic cusp aneurysm underlying aortic regurgitation (as described in the present case report).1 Although the reported series consisted of patients with chronic aortic regurgitation, acute aortic regurgitation due to cusp aneurysm as described in the current report is exceptional.
Possible etiologies underlying the aortic regurgitation in the current patient include hypertension, Marfan (-like diseases) and endocarditis. The dilatation of the ascending aorta and the electrocardiographic and echocardiographic signs of left ventricular hypertrophy were suspect for longstanding, previously undetected, hypertension. The dilatation of the aortic root may have had secondary effects on the aortic valve, since this can result in tension and bowing of the individual cusps.
Despite the mucoid degeneration of the valve tissue, Marfan (-like diseases) and collagen disorders could not be determined at autopsy; syphilis and aortic dissection were also excluded. Infective endocarditis as cause of aortic valve aneurysm has occasionally been reported in the literature.2 Although (blood) cultures and (transesophageal) echocardiography could not confirm the diagnosis of endocarditis, this could not exclude an old endocarditis or aortitis as the primary cause of aortic valve aneurysm. Observations during surgery including the changes of the aortic wall, the defect in the aortic annulus (suspect for an old abscess) and the defect in the aortic wall (visible during re-operation), all favor the diagnosis of endocarditis and/or aortitis. In addition, the focal inflammation observed on histo-pathological analysis supports this diagnosis.
Although the excellent accuracy of (transesophageal) echocardiography for diagnosing aortic valve prolapse and (in second instance) aortic root abnormalities was demonstrated, the underlying pathophysiology for these abnormalities could not be assessed by this technique.
All discussed etiologies are possible causes, although endocarditis and/or aortitis appear the most likely cause(s). In retrospect, aortic root placement (Bentall procedure) and antibiotics targeting endocarditis may have been preferred.
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References |
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TopAbstract1 Case report2 DiscussionReferences |
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- Evangelista A, del Castillo H.G, Calvo F, Permanyer-Miralda G, Brotons C, Angel J, et al. Strategy for optimal aortic regurgitation quantification by Doppler echocardiography: agreement among different methods. Am Heart J (2000) 139:773–781.[Web of Science][Medline]
- Plein D, Van Camp G, Derluyn M, Vandenbossche J.L. Aortic valve aneurysm after acute endocarditis. Clin Cardiol (1997) 20:969–970.[Web of Science][Medline]
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