Key result
Echocardiography revealed severe restrictive aortic regurgitation caused by an aortic fibrous strand connecting the left coronary sigmoid cusp to the sinotubular junction in a 70-year-old male.
Case Report (n=1)
No
This case highlights a rare cause of severe aortic regurgitation due to an embryonic remnant fibrous strand restricting the left coronary cusp, diagnosed via multimodality echocardiography.
Rare fibrous strand may cause restrictive AR; extends differential but leaves open prevalence and management implications.
A 70-year-old male was referred to the echo laboratory for a reevaluation. One year ago he suffered a traumatic tetraplegia. During the preoperatory evaluation he was diagnosed with severe aortic regurgitation. A transthoracic echocardiogram showed a tricuspid aortic valve with severe aortic regurgitation (Panel A). Aortic annulus and root were not dilated. Transoesophageal echocardiography showed a tricuspid aortic valve with normal opening movement and an adequate valve coaption between right coronary and non-coronary cusps (Panel B). A linear mobile echo like a chordae tendineae strand was connecting the left coronary sigmoid cusp to the sinotubular junction (Panel C), leading an important tenting and restriction of this cusp (Panel D and E, see Supplementary data online,Supplementary Data). Colour flow displayed a severe aortic regurgitation (Panel F, see Supplementary data online,Supplementary Data). Live three-dimensional echocardiography was performed, improving the assessment of the fibrous strand (Panel G, see Supplementary data online,Supplementary Data). Left ventricle was dilated and has a moderate dysfunction (ejection fraction 45%) with global hypokinesis. The aortic chordae tendineae strand may be an embryonic remnant during the semilunar cusp formation process of the aortic valve. There are few reported cases, most of them from Asia, describing aortic regurgitation due to spontaneous rupture of chordeae tendineae. To our knowledge, there is only one case reported similar to ours, submitting an aortic valvular incompetence owing to this restrictive mechanism (fibrous strand) without another mechanism: annular dilatation, prolapse or any congenital heart defect. Supplementary data are available at European Heart Journal – Cardiovascular Imaging online.
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Esteve‐Ruiz et al. (2015) conducted a case report in Severe aortic regurgitation (n=1). Echocardiography was evaluated on Diagnosis of the mechanism of aortic regurgitation. Echocardiography revealed severe restrictive aortic regurgitation caused by an aortic fibrous strand connecting the left coronary sigmoid cusp to the sinotubular junction in a 70-year-old male.
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