Congenital aortic regurgitation caused by a rudimentary noncoronary cusp: Report of a case

Abstract

A 14-year-old asymptomatic boy was admitted to our department for investigation of a diastolic murmur which had been discovered by his family doctor during a routine examination. Echocardiography showed aortic regurgitation with dilatation of the left ventricle. Inspection of the aortic valve at the time of operation revealed normal left and right cusps with a rudimentary noncoronary cusp. An aortic commissuro-plication was performed and a new bicuspid aortic valve successfully reconstructed. His postoperative course was uneventful and he has been well and leading an active life since his discharge from hospital.

Fetal severe aortic regurgitation due to absence of left coronary leaflet of aortic valve

“…Isolated AAV is rare and solitary absence of one of the aortic cusp is exceedingly rare. Only 3 cases have been reported and all of them were during postnatal period [11][12][13] To the best of our knowledge, there is no case report of absent aortic valve leaflet in antenatal period.…”

Surgical repair of congenital aortic regurgitation by aortic root reduction: A finite element study

“…Congenital AR is most commonly caused by a bicuspid aortic valve (i.e., a valve with only two leaflets). However, congenital AR has also been reported in three-leaflet valves where one leaflet is abnormally small (Cromme-Dijkhuis and Meuzelaar, 1991; Donofrio et al, 1992; Hashimoto et al, 1984; Hioki et al, 1994; Karimi et al, 2010; Line et al, 1979). Often in these cases, there is annular dilatation (Cromme-Dijkhuis and Meuzelaar, 1991; Donofrio et al, 1992; Hashimoto et al, 1984; Hioki et al, 1994).…”

“…Case reports describe treatment by replacing the valve with a mechanical valve (Donofrio et al, 1992; Hashimoto et al, 1984) or tissue valve (Cromme-Dijkhuis and Meuzelaar, 1991), however, in children, recent surgical practice favors valve repair (Baird and del Nido, 2009; Jonas, 2010) over replacement. One repair strategy has been to suture the rudimentary leaflet closed to create a bicuspid aortic valve (Hioki et al, 1994), but bicuspid aortic valves are known to have a high early failure rate (Robicsek et al, 2004). A more typical repair strategy would be to augment or replace the undersized RC leaflet with graft material to restore tri-leaflet symmetry and valve competence.…”