Quadricuspid Aortic Valve: An Introduction for Clinicians

Abstract

Quadricuspid aortic valve (QAV) is a congenital heart anomaly in which the aortic valve has four cusps of various size possibilities, as opposed to the three symmetrical cusps generally observed. This cardiac valvular abnormality is rarely identified, with an estimated incidence rate of 0.013% to 0.043%, although recent technological advancements in diagnostics have contributed to an increase in detection. Historically, it had been typically encountered during open heart surgery or postmortem; however, it is presently diagnosed primarily via ultrasound echocardiography, and could go undetected unless specifically considered. It was first reported by Babington in 1847, and since then approximately 300 cases have been published. This condition is sporadically associated with additional congenital cardiovascular defects, with coronary artery irregularities being the most common. In more than half of published QAV incidences it has led to the progressive development of aortic regurgitation (AR) usually sans aortic stenosis, particularly amongst elderly patients, often requiring surgical intervention after 50 years of age. A fifth of total instances, but two-thirds of instances with AR, warrant surgery seldom amidst complications, with reconstructive tricuspidization preferred over valve replacement.

Transcatheter aortic valve replacement in patients with quadricuspid aortic valve in a single center

“…Compared with patients who underwent TAVR, the patients who underwent openheart surgery were relatively younger, with an average age of approximately 45 years. Therefore, the actual incidence of QAV may be higher than that reported previously in the literature, (1,2,5) and data obtained from autopsies and echocardiographic screenings alone may have limitations.…”

“…The Hurwitz and Roberts classification, ( 27 ) based on the relative size of the supernumerary cusp, divides QAVs into seven types from A to G ( Figure 4 ). Because most patients with QAVs have undergone aortic valve replacement during surgery, ( 1 , 5 ) this classification has limited significance for surgical guidance. For patients with QAV who underwent TAVR procedures, the preoperative assessment, and strategy planning were based on the current principles of routine TAVR assessment.…”

“…Limited studies and case reports have shown that more than half of patients with QAV will progress to the stage requiring surgery. Most patients also have progressive aortic valve regurgitation (AR), whereas aortic stenosis (AS) and ascending aortic enlargement are uncommon (4)(5)(6). Patients aged 50 to 70 years with aortic valve disease often require surgery.…”

Quadricuspid aortic valve and right ventricular type of myocardial bridging in an asymptomatic middle-aged woman: A case report

“…However, echocardiography is a subjective method — it is operator-dependent and its diagnostic value depends on several factors ( e.g. , suboptimal acoustic windows, subject body habitus, and in cases of uncertain valvular anatomy, differential diagnosis[ 24 ] (Table 2 ) and may require additional imaging)[ 25 ]. MSCT and MRI are complementary methods in detecting possible associated anomalies and differential diagnosis[ 12 , 25 , 26 ].…”

A quadricuspid aortic valve with incomplete partitioning can mimic a tricuspid aortic valve

“…It was first reported in 1847, and the incidence rate reported to be 0.013%–0.043% in general population, but the actual incidence could be underreported due to rarity of the anomaly. [ 1 2 3 ] The age of clinical presentation is usually 4 th –5 th decades of life and is a little more prominent in male. [ 4 5 ]…”