BACKGROUND: -Quadricuspid aortic valve (QAV) is a rare @entity299 . This study sought to determine QAV frequency in a large echocardiography database, characterize associated @entity65 , and describe long-term outcomes. METHODS AND RESULTS: -Fifty @entity1 (age, mean [SD], 43.5 [21.8] years at the time of index diagnosis; female sex, 52%) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006%). The QAV was type A in 32% and type B in 32% (Hurwitz and Roberts classification). @entity384 was present in 29% of the @entity1 , and 26% had moderate or severe @entity337 ( @entity337 ) at index diagnosis. @entity850 affected only 8% of the valves and was mild. Other findings, including @entity299 , @entity1757 , persistent @entity4264 , and @entity2461 , were present in 32% of @entity1 . During a mean (SD) follow-up of 4.8 (5.6) years, 8 @entity1 underwent aortic valve surgery, with severe @entity337 being the surgical indication in 7 @entity1 . One @entity1 with mild to moderate @entity337 underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No @entity884 or @entity1580 was found. Overall survival was 91.5% and 87.7% at 5 and 10 years. CONCLUSIONS: - @entity384 and other structural @entity299 were relatively common among @entity1 with QAV. @entity337 was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most @entity1 who received surgery. Long-term survival was excellent.

Quadricuspid Aortic Valve: Characteristics, Associated XXXX , and Clinical Outcomes.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity4264 :: ('MESH:D013479', 'Disease') :: ['left superior vena cava'] @entity384 :: ('MESH:D002311', 'Disease') :: ['Aortic dilatation'] @entity1757 :: ('MESH:D006345', 'Disease') :: ['septal defects'] @entity2461 :: ('MESH:D004374', 'Disease') :: ['patent ductus arteriosus'] @entity1580 :: ('MESH:C562834', 'Disease') :: ['aortic dissection'] @entity337 :: ('MESH:D001022', 'Disease') :: ['AR', 'aortic valve regurgitation'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac abnormalities', 'congenital cardiac defect', 'abnormalities of other cardiac valves'] @entity850 :: ('MESH:D003251', 'Disease') :: ['Stenosis'] @entity65 :: ('MESH:D002318', 'Disease') :: ['cardiovascular abnormalities'] @entity884 :: ('MESH:D004696', 'Disease') :: ['infective endocarditis']
@entity65 :: (MESH:D002318,Disease) :: ['Structural Cardiovascular Abnormalities']
