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

Figures

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  Figure 1. Pre-Intervention Echocardiogram Parasternal long axis (A) and short axis (B) views demonstrating biventricular hypertrophy and severe pulmonary valve stenosis.

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  Figure 2. RVOT Stenting Angiograms PA (A) and lateral (B) projection of injection in the RV demonstrating an anterior and rightward morphologic RV, no interventricular right to left shunt, thickened and dysplastic pulmonary valve with supravalvular tethering, no branch or segmental pulmonary artery stenosis. PA (C) and lateral (D) projections of injection into the RVOT demonstrating positioning and deployment of two RVOT stents. Following deployment of the second stent, injection in the RV demonstrates increase in vessel caliber with no dissection, aneurysm, or extravasation of contrast. Distal jailing of the right pulmonary artery noted with no reduction in contrast flow. PA = posteroanterior; RV = right ventricle; RVOT = right ventricular outflow tract.

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  Figure 3. Pre-Trametinib Echocardiogram Subcostal sagittal (A,B) and apical 5-chamber (C,D) views demonstrating biventricular and interventricular septal hypertrophy, RVOTO, and dynamic LVOTO. LVOTO = left ventricular outflow tract obstruction; RVOTO = right ventricular outflow tract obstruction.

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  Figure 4. RVOT Stent Dilation Angiograms PA (A) and lateral (B) projection of injection in the ascending aorta demonstrates mild AI normal coronary origins. The LCA is approximately 3 mm from the RVOT stent. PA (C) and lateral (D) projections of follow up injection into the ascending aorta demonstrating LCA approximately 1.5 mm from RVOT stent. LCA = left coronary artery; RVOT = right ventricular outflow tract; other abbreviation as in Figure 2.

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  Figure 5. Post-Trametinib Echocardiogram Apical 4-chamber and 5-chamber views demonstrating improved left ventricular and septal hypertrophy and resolution of LVOTO. Abbreviation as in Figure 3.

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  Visual Summary. Favorable Responses to Interventions