P1485 multiple hindrances in and outside the heart : right ventricular outflow tract obstruction in a child with severe post-subclavian coarctation of aorta and ventricular septal defect

Abstract

Abstract Funding Acknowledgements Not applicable OnBehalf Not applicable 11 years boy with shortness of breath/easy fatigability. On examination obese, comfortable at rest, pulse : 80bpm, regular, normal JVP. Blood pressure : 170/100 in upper limb, 120/80 in lower limb. Mild cardiomegaly, normal S2, no S3/S4/click, harsh 4/6 ESM in left 3rd intercostal space, faint continuous murmur at the back. CXR : mild cardiomegaly, adequate pulmonary blood flow. ECG : sinus rhythm, normal QRS axis/intervals, biventricular hypertrophy. Echo : severe pulmonary stenosis (predominantly subvalvular, gradient 100mmHg) coarctation (gradient 50mmHg) preserved biventricular function. Cardiac CT : confirmed echo" findings, additional ? DCRV, anomalous RCA from left sinus. Cardiac MRI couldn"t yield more information. Hence, catheterization was performed. Pressure data : severe infundibular and mild valvular RVOT obstruction, normal PA pressure, severe coarctation. Oximetry : small L–>R shunt. RV angiogram : obstruction at sub-valvular/valvular levels, doming/reverse doming of pulmonary valve. LV angiogram : small subaortic VSD, L–>R. Aortic root angiogram : bicuspid aortic valve, no AR, anomalous RCA from left sinus, severe coarctation, no PDA. Plan : Single antihypertensive, surgical resection of infundibular RVOTO, pulmonary valvotomy, VSD closure, coarctation repair. Cath data Pressure in mm of Hg Right atrium (mean) 8 Right ventricle (apex) systolic 100 end diastolic 12 Right ventricle (outflow) systolic 60 end diastolic 12 Main pulmonary artery 25/10 (15) Left ventricle systolic 158 end diastolic 14 Ascending aorta 165/75 (140) Descending aorta 116/80 (100) Right Femoral artery 117/76 (98) Oximetry Superior vena cava 68% Inferior vena cava 74% Pulmonary artery 82% Aorta 99% Pulmonary vein 100% Qp/ Qs 1.6:1 Abstract P1485 Figure. CT/MRI/Cath