Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. Nearly one-half of patients with QAV require a surgical procedure for their aortic valve disease. Options for QAV include aortic valve repair and aortic valve replacement, but data on long-term clinical and surgical outcomes are lacking.
Mayo Clinic researchers, first author Patricia Pellikka, M.D., conducted a study to determine QAV frequency in a large echocardiography database, characterize associated cardiovascular abnormalities, and describe long-term outcomes. The study was published in the Circulation journal.
The study included 50 patients that received a diagnosis of QAV between January 1, 1975, and March 14, 2014. The QAV was type A in 32% and type B in 32%. Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation (AR) at diagnosis. Stenosis affected only 8% of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus.
During a follow-up, eight patients underwent aortic valve surgery, with severe AR being the surgical indication in seven patients. One patient with mild to moderate AR underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. The overall survival was 91.5% and 87.7% at five and 10 years.
The study provided important insights into QAV frequency, echocardiographic characteristics, natural history, and long-term outcomes. Based on the results, AR was the predominant hemodynamic abnormality, whereas AS was rare. Aortic dilatation was more
prevalent than has been reported previously. However, a majority of patients had no or
only mild progression of AR and aortic dilatation over time, and only 16% underwent aortic valve surgery during a mean follow-up of almost 5 years. Long-term survival was excellent both without and with aortic valve surgery.