Aortic Root Dilatation in Taiwanese Patients with Mucopolysaccharidoses and the Long-Term Effects of Enzyme Replacement Therapy.

Hsiang-Yu Lin Ming-Ren ChenChung-Lin LeeShan-Miao LinChung-Lieh HungDau-Ming NiuTung-Ming ChangChih-Kuang ChuangShuan-Pei Lin

Published in: Diagnostics (Basel, Switzerland) (2020)

Aortic root dilatation was common in the patients with all types of MPS, with the most severe aortic root dilatation observed in those with MPS IV. The severity of aortic root dilatation worsened with increasing age, reinforcing the concept of the progressive nature of this disease. ERT for MPS appears to stabilize the progression of aortic root dilatation.

Keyphrases

aortic valve
pulmonary artery
left ventricular
replacement therapy
aortic dissection
multiple sclerosis
coronary artery
heart failure
pulmonary hypertension
early onset
pulmonary arterial hypertension