The effect of adding an open distal anastomosis to proximal aneurysm repairs in bicuspid aortopathy.

Objectives: To determine the role of adding open distal anastomosis to proximal aortic aneurysm repairs in bicuspid aortic valve (BAV) patients.

Methods: Retrospective review was performed of 1132 patients at our Aortic Center between 2005 and 2019. Inclusion criteria were all patients diagnosed with a BAV who underwent proximal aortic aneurysm repair with open or clamped distal anastomosis.

Rare variants in SOX17 are associated with pulmonary arterial hypertension with congenital heart disease.

Background: Pulmonary arterial hypertension (PAH) is a rare disease characterized by distinctive changes in pulmonary arterioles that lead to progressive pulmonary arterial pressures, right-sided heart failure, and a high mortality rate. Up to 30% of adult and 75% of pediatric PAH cases are associated with congenital heart disease (PAH-CHD), and the underlying etiology is largely unknown. There are no known major risk genes for PAH-CHD.