Hemodynamics, anatomy, and outcomes of quadricuspid aortic valves: Multimodality imaging assessment.

BACKGROUND

Quadricuspid aortic valve (QAV) is a rare congenital heart disease with a limited body of literature. This retrospective cohort study investigates QAV morphology, function, and clinical outcomes.

METHODS

Echocardiography was used to assess valvular function. Morphological characteristics such as phenotypes, raphe, regurgitant orifice area (ROA), and aortic dilation (diameter >40 ​mm) were assessed by cardiac CT. Patients were followed up for the combined event of all-cause death and aortic valve replacement (AVR).

RESULTS

Ninety QAV patients (screened from 322385 CT scans) were included (mean age 55.2 ​± ​13.6 years, 61.1 ​% male). Isolated significant aortic regurgitation (AR) was present in 75.6 ​% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 ​%) and type II (3 larger and 1 smaller leaflets, 42.2 ​%) QAV. Fused raphe was present in 26.7 ​% of patients. ROA was correlated with AR severity and aortic dilation (41.1 ​%, n ​= ​37). Among patients without AVR at baseline (n ​= ​60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3-62.8). ROA was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 ​mm, HR ​= ​4.25, 95%CI 1.49-12.17, p ​= ​0.007; as a continuous variable (per mm increment), HR ​= ​1.04, 95%CI 1.01-1.07, p ​= ​0.003). Additionally, ROA had incremental prognostic value when added to the AR severity model (area under the receiver-operating characteristic curve increased from 86.8 to 88.4, p ​= ​0.004).

CONCLUSION

QAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROA may be a potential ancillary prognostic marker in patients with QAV.