RESUMO
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 âmm) 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 â± â13.6 years, 61.1 â% male). Isolated significant aortic regurgitation (AR) was present in 75.6 â% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 â%) and type II (3 larger and 1 smaller leaflets, 42.2 â%) QAV. Fused raphe was present in 26.7 â% of patients. ROACT was correlated with AR severity and aortic dilation (41.1 â%, n â= â37). Among patients without AVR at baseline (n â= â60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3-62.8). ROACT was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 âmm2, HR â= â4.25, 95%CI 1.49-12.17, p â= â0.007; as a continuous variable (per mm2 increment), HR â= â1.04, 95%CI 1.01-1.07, p â= â0.003). Additionally, ROACT 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 â= â0.004). CONCLUSION: QAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROACT may be a potential ancillary prognostic marker in patients with QAV.