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Home , Tetralogy of Fallot

Tetralogy of Fallot UC Irvine Echo Essentials November 3, 2018 Tetralogy of Fallot

• Most common cyanotic ACHD defect • Incidence: 4 in 10,000 live births • 7-10% of congenital disease • Incidence equal across genders • Palliative shunt and surgical correction • without surgery: 11% survival at 20 years, 3% survival at 40 years Tetralogy of Fallot

• Demographics • Defining TOF • TOF – not just one disease!

Tetralogy of Fallot

•Malalignment VSD

•Overriding

•RVOT obstruction

•Right

Tetralogy of Fallot

• Associated anomalies • Right aortic arch (25%) • LAD from RCA (9%) • Muscular VSDs • Aortopulmonary collaterals • ASD (“pentalogy”)

Tetralogy of Fallot

Minimal RVOT obstruction Complete RVOT obstruction

“Pink Tet” “Balanced” Tet Moderate Very cyanotic Acyanotic Minimal cyanosis * Most TOF pts* Tetralogy of Fallot

Minimal RVOT obstruction Complete RVOT obstruction “Pink Tet” - Physiologically similar to a nonrestrictive VSD Very cyanotic - HF symptoms early in life due to overcirculation as PVR decrease; PA banding may be required to decrease pulmonary blood flow Tetralogy of Fallot

Minimal RVOT obstruction Complete RVOT obstruction

“Pink Tet” “Balanced” Tet Moderate cyanosis Very cyanotic Acyanotic Minimal cyanosis * Most TOF pts* Tetralogy of Fallot

• Tetralogy of Fallot/ • “a” = without, “tresia” = perforation • 10% of pts with TOF • Associated with hypoplastic central and branch pulmonary arteries • Unifocalization = reconstruction of branch pulmonary arteries • Pulmonary hypertension is common post-repair • Repair includes RV-PA conduit Tetralogy of Fallot

Minimal RVOT obstruction Complete RVOT obstruction

“Pink Tet” “Balanced” Tet Moderate cyanosis Very cyanotic Acyanotic Minimal cyanosis * Most TOF pts* Case 1: “balanced” tetralogy of Fallot Case 1: “balanced” tetralogy of Fallot Case 1: “balanced” tetralogy of Fallot Case 1: “balanced” tetralogy of Fallot Case 1: “balanced” tetralogy of Fallot Case 1: “balanced” tetralogy of Fallot Case 1: “balanced” tetralogy of Fallot Case 1: “balanced” tetralogy of Fallot • In patient with “balanced” TOF • RVOT stenosis is mild enough that it allows sufficient pulmonary blood flow • RVOT stenosis is severe enough that it protects the pulmonary arteries from overcirculation

Tetralogy of Fallot

Minimal RVOT obstruction Complete RVOT obstruction

“Pink Tet” “Balanced” Tet Moderate cyanosis Very cyanotic Acyanotic Minimal cyanosis * Most TOF pts* Tetralogy of Fallot

Lillehi 1954 – single-stage, complete repair • Patch closure of VSD • Enlargement of RVOT • Resection of sub/infundibular muscles • Transannular patch (A) • Results in regurgitation • Valve-sparing approach • May result in residual RVOT obstruction if small annulus • Valved conduit (B)

Tetralogy of Fallot

Geva 2006 Pediatric Annual Tetralogy of Fallot

• Late issues and complications

• Sudden death • - atrial & ventricular • Conduction defects- pacemaker? • LV/RV function and contractile reserve • Prosthetic valve/conduit stenosis • Aortic root dilation and aortic regurgitation • Endocarditis • and genetics

Case 2: “repaired” tetralogy of Fallot

LV/RV size and function Aortic dilation Evidence for RV pressure/volume overload Case 1: “repaired” tetralogy of Fallot

Residual pulmonary valve stenosis/regurgitation, sub/supravalvar stenosis. CW/PW RVOT. PW branch PAs for diastolic flow reversal Case 1: “repaired” tetralogy of Fallot

LV/RV size and function Case 2: “repaired” tetralogy of Fallot, post PVR Case 2: “repaired” tetralogy of Fallot, post PVR Take home points

• Tetralogy of Fallot – one name, many different presentations • Most patients have moderate+ PR after initial repair • Requires lifelong care by congenital heart disease specialists • Most will require pulmonary • Echocardiograms guide clinical decision making • RVOT obstruction (subvalvar, valvar, supravalvar) • Pulmonary valve regurgitation • RV size/function; LV size/function • Aortic root dilation and AR