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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 •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
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