Objective: To elucidate sex differences in valve morphology, disease phenotype, progression, and outcomes among children and young adults with bicuspid aortic valve (BAV).
Patients and Methods: This is a retrospective cohort study examining all children and young adults (aged [less than or equal to]22 years) with isolated BAV diagnosed, by excluding patients with concomitant congenital heart defects or genetic syndromes, from January 1, 1990, through December 1, 2016, at Mayo Clinic in Rochester, Minnesota.
Results: Of 1010 patients with BAV, 558 had isolated BAV. Distributions of morphology were rightleft in 65.8% (n=367), right-noncoronary in 34% (n=190), and left-noncoronary cusp fusion in 0.2% (n=1) of patients; with no sex differences. Male to female ratio was 3:1. At the first echocardiographic evaluation in the study, there were no sex differences in terms of frequency of aortic valve stenosis or regurgitation. However, males had significantly higher grades of aortic valve regurgitation at 17 years of age onward (P<.0001). Males had significantly larger mid-ascending aorta (P=.01) and sinus of Valsalva dimensions (z score; P=.0001) as compared with females, with a novel Ending of peak aortic dimensions around 8 years of age. Males also had more than 2-fold higher risk for sinus of Valsalva dilation (z score >2) as compared with females (odds ratio, 2.3; 95% CI, 1.2 to 4.2; P=.01). There were no significant sex differences in the primary cardiac outcomes of interventions on aortic valve and/or aorta, aortic dissection, or death.
Conclusion: In children and young adults with BAV, males have a higher grade of aortic regurgitation in late adolescence, significantly larger aortic dimensions, different patterns of aortic growth, and more frequent sinus of Valsalva dilation as compared with females. Overall, the rate of primary cardiac events is lower in young patients, with no significant sex differences.
Bicuspid aortic valve (BAV) is one of the most common congenital heart defects, with an estimated prevalence of 0.5% to 2%, and is 3 times more prevalent in males than females. (1-4) Bicuspid aortic valve has a wide spectrum of clinical presentation ranging from asymptomatic patients with normal aortic valve function to those with hemodynamically significant aortic valve disease or aortopathy. (5) Although most of these patients present as adults, a considerable number of pediatric patients may also present with premature aortic valve disease and aortopathy requiring intervention during a young age. (6,7) With improved family screening and readily available echocardiography, there is a large pediatric population with BAV that requires lifelong surveillance. Bicuspid aortic valve disease is complex due to the heterogeneity of its presentation and progression. (8) Determination of different risk factors associated with aortic valve disease and aortopathy is essential for risk stratification and determining adequate follow-up intervals.
In adult patients with BAV, several studies have examined sex differences in terms of various complications and survival. (9,10) Adult patients with BAV present with more frequent aortic stenosis among women and aortic regurgitation among men. (9) Men also have a higher frequency of aortopathy than women and male sex is an established...