Aortic Valve Fenestrations

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A long strandlike mobile echodensity originating at the aortic leaflets may in rare cases represent a ruptured aortic valve fenestration.
The echocardiographic differential diagnosis is: Lambl excrescence, vegetation, papillary fibroelastoma.
Unruptured aortic valve fenestrations are not readily diagnosable on echocardiography. They are located beyond the line of aortic leaflet coaptation. Hence, normally there is no color flow evidence of aortic regurgitation to suggest their presence.

When the aortic root becomes dilated - the line of aortic valve closure may change, and there may be a diastolic color flow jet through a fenestration that has become incorporated into the functional component of the aortic valve.

Acute aortic regurgitation due to spontaneous rupture of a fenestrated cusp: report in a 65-year-old man and review of seven additional cases.
Blaszyk H, Witkiewicz AJ, Edwards WD.
Cardiovasc Pathol. 1999 Jul-Aug;8(4):213-6.

A 65-year-old man with chronic hypertension developed dyspnea, a cough, and a new diastolic murmur. Two-dimensional echocardiography showed severe aortic regurgitation. No valvular vegetations were identified and blood cultures were negative. Surgical intervention was recommended, but the patient died of an acute intracranial hemorrhage two weeks later. At autopsy, the posterior aortic cusp was flail, due to rupture of the residual cord above two large fenestrations. There was no acute or healed endocarditis. To our knowledge, this is the eighth reported case of aortic valve incompetence due to spontaneous rupture of a fenestrated cusp. Patients ranged in age from 31-67 years (mean, 54), and 4 (50%) were older than 60 years. Seven (88%) of the 8 were men, and 4 (57%) of 7 had chronic hypertension. Analogously, in another four reported cases, aortic insufficiency developed following spontaneous rupture of the fenestrated raphe of an atypical congenitally bicuspid aortic valve. Noninfective and nontraumatic rupture of cord-like aortic valve structures may result in severe acute aortic regurgitation, particularly in men with chronic hypertension.

A fenestrated aortic valve contributing to iatrogenic aortic insufficiency post mitral valve replacement.
Lee AP, Walley VM, Ascah KJ, Veinot JP, Davies RA, Keon WJ.
Cardiovasc Pathol. 1996 Mar-Apr;5(2):81-3.

A case of an unusual local complication of cardiac valvular surgery is presented. Distortion of the geometry of the aortic valve base by a prosthetic mitral valve sewing ring allowed aortic insufficiency through the aortic valve's central orifice, as well as through an aortic valve cusp fenestration. During the 6 years after valve surgery, this patient developed chronic left heart failure contributed to by the aortic insufficiency and eventually, at age 65, required cardiac transplantation. Surgeons and pathologists should be aware of this unusual local complication of cardiac valve surgery, as it may have serious consequences.

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The contents and links on this page were last verified December 2017 by Dr. Olga Shindler.