RVOT Stenting and Trametinib in an Infant With Noonan Syndrome, Pulmonary Stenosis, and Hypertrophic Cardiomyopathy

• DOI: 10.1016/j.jaccas.2024.103224
• Publication Date: 2025-02-12T19:20:35Z
• Authors: Theodore J. Millette; Michael A. McCulloch; Qin Yao; Michael R. Hainstock
• Keywords: Noonan Syndrome; Trametinib; Pulmonic stenosis

Right ventricular outflow tract stenting is a well-established palliative strategy primarily used for infants with tetralogy of Fallot but has not been reported pulmonary valve stenosis in Noonan syndrome. We describe an infant history extreme prematurity, syndrome, hypertrophic cardiomyopathy, and severe valvular supravalvular pulmonic who underwent right mitogen-activated protein kinase (MEK) inhibitor therapy. This unique approach ultimately allowed discharge home postponement surgical repair until 18 months age. viable mode palliation syndrome are ideal candidates. An increasing number case reports demonstrating the efficacy MEK inhibition as effective tool treatment syndrome-associated cardiomyopathy.