Long‐term performance of bicuspid and quadricuspid aortic valves: Similarities and differences

Abstract

While bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, quadricuspid aortic valve (QAV) is rare. The usual three‐leaflet aortic valve is characterized by engineering advantages with superior long‐term performance, and thus, degenerative changes and significant functional deterioration appear at advanced age. Aim: Evaluation of long‐term performance, similarities, and differences between QAV and BAV. Methods: Screening of 19 000 consecutive echocardiographic studies was performed. Results: BAV was reported in 131 subjects with a prevalence of 0.7%, while QAV was seen in 11 with a prevalence of 0.06%, P < .00001. Age of BAV patients was younger, 45 ± 20 years vs 62 ± 17 years in QAV, P < .05, with higher proportion of females in those with QAV, 40% vs 30%. Chamber diameters were similar in both groups. Higher atrial contraction‐A‐wave mitral peak velocities and longer E‐wave deceleration times were found in subjects with QAV, P < .05 for both. Dilated ascending aorta was found in 25% of patients with BAV and in 18% of those with QAV, P = .2. Moderate and severe aortic valve stenosis were found in 21% of patients with BAV and in 27% of those with QAV, P = ns. More than moderate aortic regurgitation was found in 15.5% of BAV patients and in 9% of QAV, P = ns. Aortic valve infective endocarditis was found in 1.5% of BAV patients and in 9% of those with QAV. Conclusions: BAV is a common congenital anomaly, while QAV is rare. Similar prevalence of significant valve disease and aortopathy was found in both anomalies, though at younger age in BAV patients.

Normally functioning quadricuspid aortic valve mistaken for bicuspid valve in a septuagenarian

“…According to the Hurwitz and Roberts classification scheme, this patient's valve was Type A (four equal‐sized valve cusps) 1 . In addition to visualizing four cusps, a quadricuspid aortic valve should demonstrate the corpus Arantii (nodule located at the tip of each cusp) as was noted in this case 2,3 (Figure 4).…”

“…Additionally, a quadricuspid aortic valve may remain functionally normal. However, a patient with a quadricuspid aortic valve may develop progressive aortic regurgitation, requiring surgical intervention 3,4 …”

“… The incidence of quadricuspid aortic valve may be somewhat higher than generally thought. By TTE, a quadricuspid aortic valve may be mistakenly diagnosed as a bicuspid aortic valve 3,5 Often a patient with a quadricuspid aortic valve is asymptomatic, but may develop progressive aortic regurgitation.…”

“…However, a patient with a quadricuspid aortic valve may develop progressive aortic regurgitation, requiring surgical intervention. 3,4 Summarizing points include:…”

Transthoracic echocardiography and its limitations in the diagnosis of congenital supernumerary aortic valve in a Thoroughbred

“…Anatomical variations of the aortic valve that have been documented in humans include unicuspid, bicuspid, quadricuspid and quinticuspid valves (Meng et al., 2009 ; Oladiran et al., 2019 ). Of these variants the bicuspid valve (BAV) is the most common with a reported prevalence of ~1% with male predominance (Meng et al., 2009 ; Sharif et al., 2019 ; Sun et al., 2019 ). Prevalence of quadricuspid aortic valve (QAV) is significantly lower (0.06%), and only three sporadic reports exist in human literature that documented a quinticuspid aortic valve (Bogers et al., 1982 ; Meng et al., 2009 ; Simonds, 1923 ).…”