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 Ductus Arteriosus Aorta Pulmonary Artery Left Atrium Patent Foramen Ovale Right Atrium Left Ventricle Right Ventricle 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 QuickTime™ and a Photo - JPEG decompressor are needed to see this picture. 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 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!