What is the role of apical ventriculotomy in children and young adults with hypertrophic cardiomyopathy?

Background: The transapical approach has been utilized in adult HCM patients with either midventricular obstruction or a small LV cavity; however, there are little data on its use in children.

Methods: We retrospectively reviewed all patients (age <21 years) with HCM who underwent a transapical myectomy from January 2002 to December 2016. Indication for surgery was midventricular obstruction in 19/23 (83%) and small LV cavity in 4 (17%).

Characteristic Morphologies of the Bicuspid Aortic Valve in Patients with Genetic Syndromes.

Background: In patients with bicuspid aortic valve (BAV), complications including progressive aortic stenosis and aortic dilatation develop over time. The morphology of cusp fusion is one of the determinants of the type and severity of these complications. We present the association of morphology of cusp fusion in BAV patients with distinctive genetic syndromes.

Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects.

Background: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV.

Methods: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015.