Congenital Heart Lesions

Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions Right side obstruction and R -> L shunt Transposition Mixing Lesions Surgical therapy

Pulmonary Artery Right Atrium Right Ventricle Left Ventricle Aorta Left Atrium Ductus Arteriosus P Patent F Foramen O Ovale

Key Points Blood flows to the path of least resistance Pulmonary resistance < systemic resistance All newborns have connections –PDA –PFO

Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions Right side obstruction and R -> L shunt Transposition Mixing Lesions Surgical therapy

Left to right shunting Right and left side connected Increased (too much) pulmonary blood flow Respiratory distress/ CHF

Left to right shunt lesions Ventricular septal defect (VSD) Atrial septal defect (ASD) AV canal Patent ductus arteriosus (PDA)

Diagnostic tools CXR-- “wet lungs” with cardiomegaly EKG-- may have RVH, IRBBB (ASD), abnormal “NW” axis (AV canal), BVH (VSD) ABG-- high CO2 late finding; PO2 in 100% not very useful; no acidosis

Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions Right side obstruction and R -> L shunt Transposition Mixing Lesions Surgical therapy

Left side obstruction Not enough blood to the body Hypo-perfusion, acidosis, shock +/- connection between right and left

Left side obstructive lesions Mitral valve obstruction Aortic valve obstruction Coarctation of the aorta Everything obstructed –Hypoplastic left heart syndrome

Diagnostic tools CXR- may be normal or “wet” EKG- often misleading; neonate will not have LVH you would expect from an older person with AS or coarct (and hypoplast will have left forces) ABG- may present with profound metabolic acidosis, low CO2 (hyperventilating), PO2 may be lo or hi

Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions Right side obstruction & R -> L shunt Transposition Mixing Lesions Surgical therapy

Cyanotic lesions Connection - right and left sides AND right side obstruction Decreased pulmonary blood flow OR Separated systems Normal or increased pulmonary blood flow

Cyanotic lesions Right side obstructions –Tricuspid obstruction –Pulmonary obstruction –Tetralogy of Fallot Separate systems –Transposition of the great vessels

Diagnostic tools CXR- classically, “black lung fields” with “boot” (TOF) or narrow mediastinum (TGA) EKG- very often normal, except tricuspid atresia classically “northeast” ABG- these are the kids who fail the hyperoxia challenge

Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions Right side obstruction & R -> L shunt Transposition Mixing Lesions Surgical therapy

When is “blue” O.K.?

Mixing lesions Very large connection Key points- –What goes into the lungs comes out of the lungs = red –What goes into the body comes out of the body = blue May have right side obstruction

Mixing Lesions Single ventricle –Double inlet left ventricle (DILV) –Double outlet right ventricle (DORV) –Primitive ventricle –Hypoplastic right or left ventricle Total anomalous pulmonary venous return (TAPVR) Truncus arteriosus

Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions Right side obstruction & R -> L shunt Transposition Mixing Lesions Surgical therapy

Repair vs. palliation Palliating a single ventricle - Example: HLHS –Stage I: Norwood and BT shunt –Stage II: Glenn shunt –Stage III: Fontan

Hypoplastic Left Heart Syndrome

Stage I: Norwood + BT shunt

Stage II: Glenn shunt

Stage III: Fontan

Take-home Congenital heart disease is not about murmurs Tachypnea, cyanosis, “shock” should all raise red flags Exam, CXR,EKG,Sats, ABG are as important as the echo!