Aortic pseudocoarctation associated with a stenotic congenitally bicuspid aortic valve

The diagnosis of aortic coarctation is when a high-velocity Doppler jet is seen in the proximal descending aorta. Such a jet was seen in a 23-year-old man with bicuspid aortic valve complicated by severe aortic insufficiency in the presence of a normal aorta with slight tapering confirmed by magnetic resonance imaging. After aortic replacement, the gradient was no longer seen. In conclusion, high flow in the aorta induced by large regurgitant volumes can result in the appearance of "relative stenosis" or pseudocoarctation.     

Frequency of aortic root dilation in children with a bicuspid aortic valve

Bicuspid aortic valve (BAV) is associated with aortic root dilation and dissection in adults, but the age and conditions when dilation begins are unknown. Using echocardiographic data, we compared the aortic root dimensions and valve hemodynamics of 76 children with BAV with valves derived from 41 normal children. The children with BAV had significantly larger aortic roots (p <0.001) regardless of the presence of aortic stenosis or regurgitation.     

Presence of a congenitally bicuspid aortic valve among patients having combined mitral and aortic valve replacement

Although bicuspid aortic valve occurs in an estimated 1% of adults and mitral valve prolapse in an estimated 5% of adults, occurrence of the 2 in the same patient is infrequent. During examination of operatively excised aortic and mitral valves because of dysfunction (stenosis and/or regurgitation), we encountered 16 patients who had congenitally bicuspid aortic valves associated with various types of dysfunctioning mitral valves. Eleven of the 16 patients had aortic stenosis (AS): 5 of them also had mitral stenosis, of rheumatic origin in 4 and secondary to mitral annular calcium in 1; the other 6 with aortic stenosis had pure mitral regurgitation (MR) secondary to mitral valve prolapse in 3, to ischemia in 2, and to unclear origin in 1. Of the 5 patients with pure aortic regurgitation, each also had pure mitral regurgitation: in 1 secondary to mitral valve prolapse and in 4 secondary to infective endocarditis. In conclusion, various types of mitral dysfunction severe enough to warrant mitral valve replacement occur in patients with bicuspid aortic valves. A proper search for mitral valve dysfunction in patients with bicuspid aortic valves appears warranted.     

Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation.

OBJECTIVE--To investigate the morphology of congenitally bicuspid aortic valves causing pure valve regurgitation. DESIGN--A case series collected over five years. SETTING--An academic hospital. PATIENTS AND METHODS--One hundred and forty eight excised congenitally bicuspid aortic valves. The morphological findings were correlated with sex, age, clinical history, and data on haemodynamic function before operation. Pure valve regurgitation was defined as grade 3-4/4 with a gradient less than 30 mm Hg. Aortic root dilatation was evaluated angiographically or echocardiographically or both. RESULTS--Three types were recognised: valves that were purely bicuspid (23%), bicuspid valves with a raphe (34%), and valves with an additional indentation of the free edge of the conjoined cusp (43%). In 14 cases pure valve regurgitation was present. Dilatation of the aortic root was present in 47 cases. The relative risk for regurgitation when the aortic root was dilated (compared with no dilatation) was 3.99. The relative risk for valve regurgitation when there was indentation of the conjoined cusp (compared with no indentation) was 4.95. The mean age at operation in patients with pure regurgitation was 56 years, which is significantly younger (p = 0.0008) than that of patients with a congenitally bicuspid valve with combined valve stenosis and regurgitation (64.7 years). CONCLUSIONS--Congenitally bicuspid aortic valves with a central indentation of the free edge of the conjoined cusp seem particularly likely to develop pure aortic valve regurgitation.     

Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation.

OBJECTIVE--To investigate the morphology of congenitally bicuspid aortic valves causing pure valve regurgitation. DESIGN--A case series collected over five years. SETTING--An academic hospital. PATIENTS AND METHODS--One hundred and forty eight excised congenitally bicuspid aortic valves. The morphological findings were correlated with sex, age, clinical history, and data on haemodynamic function before operation. Pure valve regurgitation was defined as grade 3-4/4 with a gradient less than 30 mm Hg. Aortic root dilatation was evaluated angiographically or echocardiographically or both. RESULTS--Three types were recognised: valves that were purely bicuspid (23%), bicuspid valves with a raphe (34%), and valves with an additional indentation of the free edge of the conjoined cusp (43%). In 14 cases pure valve regurgitation was present. Dilatation of the aortic root was present in 47 cases. The relative risk for regurgitation when the aortic root was dilated (compared with no dilatation) was 3.99. The relative risk for valve regurgitation when there was indentation of the conjoined cusp (compared with no indentation) was 4.95. The mean age at operation in patients with pure regurgitation was 56 years, which is significantly younger (p = 0.0008) than that of patients with a congenitally bicuspid valve with combined valve stenosis and regurgitation (64.7 years). CONCLUSIONS--Congenitally bicuspid aortic valves with a central indentation of the free edge of the conjoined cusp seem particularly likely to develop pure aortic valve regurgitation.     

An exceptional form of congenitally bicuspid aortic valve resulting in pure aortic regurgitation

A case with an exceptional form of congenitally bicuspid aortic valve is described. A fibrous strand connected to the supra-aortic ridge supported the conjoined cusp, and severe pure aortic regurgitation was present although the fibrous strand remained intact. Aortic valve replacement was successfully performed.     

Long-term risk of aortic events following aortic valve replacement in patients with bicuspid aortic valves

Bicuspid aortic valve (BAV) is associated with ascending aortopathy predisposing to aneurysmal dilatation and dissection, even after successful aortic valve replacement (AVR). There is, however, scant evidence on which to make recommendations for prophylactic replacement of the ascending aorta at the time of AVR. The medical records of patients who underwent AVR for BAV without aortic replacement or repair from 1960 to 1995 were reviewed. Follow-up was by review of the medical record and postal questionnaire. Among 1,286 patients, the mean age at operation was 58 ± 14 years. During the follow-up interval (median 12 years, range 0 to 38), there were 13 documented aortic dissections (1%), 11 ascending aortic replacements (0.9%), and 127 documented cases of progressive aortic enlargement (9.9%). Fifteen-year freedom from aortic dissection, enlargement, or replacement was 89% (95% confidence interval [CI] 87% to 91%) and was lower in patients with documented aortic enlargement at the time of AVR (85%, 95% CI 81% to 89%) compared to those whose aortic dimensions were normal (93%, 95% CI 90% to 96%) (p = 0.001). Multivariate predictors of aortic complications included interval (subsequent) AVR (hazard ratio [HR] 3.5, 95% CI 2.3 to 5.4, p <0.001), concomitant coronary artery bypass grafting (HR 2.6, 95% CI 1.7 to 4.0, p <0.001), enlarged aorta (HR 1.8, 95% CI 1.3 to 2.6, p = 0.001), and history of tobacco abuse (HR 1.8, 95% CI 1.2 to 2.6, p = 0.003). Aortic dilatation did not predict mortality. In conclusion, despite a true risk for aortic events after AVR for BAV, the occurrence of aortic dissection was low. Any incremental surgical risk imposed by prophylactic replacement of the ascending aorta must be equally low.     

Changes in size of ascending aorta and aortic valve function with time in patients with congenitally bicuspid aortic valves

Bicuspid aortic valve (BAV) is associated with premature valve dysfunction and abnormalities of the ascending aorta. Limited data exist regarding serial changes of aortic dilation in patients with BAV. We studied paired transthoracic echocardiograms of 68 patients with BAV (mean age 44 years) and with at least 2 examinations >12 months apart (mean follow-up 47 months) to characterize the progression of aortic dilation and the natural history of valve function. We measured aortic root and ascending aortic diameters at baseline and follow-up. We measured aortic gradients and severity of aortic regurgitation (AR). During follow-up, aortic diameters increased at the sinuses of Valsalva by 1.9 mm (95% confidence interval [CI] 1.3 to 2.5), at the sinotubular junction by 1.6 mm (95% CI 0.8 to 2.3), and at the proximal ascending aorta by 2.7 mm (95% CI 1.9 to 3.6). Mean rate of diameter progression was 0.5 mm/year at the sinuses of Valsalva (95% CI 0.3 to 0.7), 0.5 mm/year at the sinotubular junction (95% CI 0.3 to 0.7), and 0.9 mm/year at the proximal ascending aorta (95% CI 0.6 to 1.2). Progression was observed regardless of hemodynamic function at baseline. Mean aortic valve gradient increased significantly from baseline to follow-up (17.6 mm Hg vs 25.7 mm Hg, p <0.001). The degree of AR increased during follow-up in 17 patients (25%). In addition, progression of aortic diameter dilation occurred irrespective of baseline valve function in adult patients with BAV. We also observed considerable progression of aortic gradients and AR over time.