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Tetrology of Fallot. Tetrology of Fallot. Cyanosis Decreased Pulmonary Vascularity Normal Cardiac size 8 percent of Congenital heart disease 4 Components. VSD Large and located just below Aortic Valve Pulmonary Infundibular Stenosis Overriding Aorta – Straddles VSD
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Tetrology of Fallot • Cyanosis • Decreased Pulmonary Vascularity • Normal Cardiac size • 8 percent of Congenital heart disease • 4 Components
VSD Large and located just below Aortic Valve • Pulmonary Infundibular Stenosis • Overriding Aorta – Straddles VSD • Right Ventricular Hypertophy – from elevated RV pressure
Hemodynamics • Pulmonary resistance greater than systemic so right to left shunt and cyanosis • Exercise decreases systemic vascular resistance so greater right to left shunt
Cyanosis brought on by exercise, hot weather, meals • Tetrad spells – sudden increase in Rt to Lt shunt and severe hypoxemia. May be relieved with squatting- increases systemic resistance so rt to left shunt decreases • Pulmonary Systolic Ejection Murmur
Xray • Decreased Pulmonary Artery Vascularity • Large Bronchial collaterals • Boot shaped heart from RVH, enlarged aorta and concave PA • Rt Aortic Arch in 25%
Management- Palliative vs. Corrective Palliative – bring blood to PA Potts – Lt PA to Descend Aorta Waterston –Rt. PA and Ascending Aorta Blalock Taussig- Older Children – Subclavian Art. Opposite Arch anastamosted to Ipsilateral PA Central Shunt- Synthetic Graft Ascending Aorta to PA Corrective Close VSD Alleviate Rt Ventric Outflow Obsrtuction Pulmonary Valvotomy, Resection of infundibular myocadium and outflow patch
Pseudo Truncus Tetrology with Aortic Artesia