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. CorrectivePalliative – 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