Prevalence, multimodality imaging characterization, and mid-term prognosis of quadricuspid aortic valves: an analysis of eight cases, based on 160 004 exams performed during 12 years in a tertiary care hospital.

Aims: Quadricuspid aortic valve (QAV) is a rare abnormality, which may cause aortic regurgitation (AR) requiring surgical intervention in some patients. The characteristics associated with aortic valve functional degeneration in patients with QAV are still unknown. The aim of this study is to describe QAV prevalence, characterize the disease by multimodality imaging, evaluate predictors of severe AR, and assess mid-term prognosis.

Methods And Results: Retrospective search in imaging exams database of one tertiary centre, for patients diagnosed with QAV between January 2007 and September 2019. QAV was characterized by cardiac computed tomography, transthoracic/transoesophageal echocardiography, and cardiac magnetic resonance. A total of 160 004 exams were reviewed and eight patients with QAV were identified (50% men, mean age 53.5 ± 10.7 years). The prevalence of QAV was 0.005%. During a median follow-up of 52 months (interquartile range 16-88), there were no deaths. Seven patients (88%) had pure AR (three severe, one moderate, and three mild) and one patient (12%) had moderate AR and moderate aortic stenosis. Three patients (38%) with severe AR underwent valve surgery (two replacements and one repair). Analysis of predictors of severe AR was not statistically significant.

Conclusion: QAV is a rare congenital cardiac defect, with a prevalence of 0.005% in our study. Its predominant functional abnormality was regurgitation and about one-third of the patients required aortic valve surgery. Multimodality imaging may play a pivotal role in assessing patients with QAV with significant valve dysfunction or associated congenital heart disease and improve their treatment strategy.