TOF 10% (m/c Cyanosis, Pulmonary stenosis murmur, ECG: RAD, RVH Rx hypoxia: O2 (minimal benefit), Coronary anomalies present in 5% cyanotic single S2 & RV impulse (LSB), hypoxic CXR: “boot-shaped” heart knee-chest position (squat, of TOF patients CHD) (Tet) spells (restless, agitated, crying) VR), morphine sulfate, Natural hx is progression and unconsciousness, convulsions, phenylephedrine ( SVR). worsening of PS and cyanosis hemiparesis, death. Cyanotic spell: Surgery (complete, Subacute beacterial endocarditis risk of cerebral TE, cerebral abscess palliative shunt surgery for prophylaxis indicated until 6 m complex TOF) after repair & for residual VSDs. TOGA 5% VS intact: Cyanosis, quiet tachypnea, ECG: RAD, RVH Prostaglandin E1 Most common cyanotic lesion single S2, no murmur CXR: pulmonary vascularity, Balloon atrial septostomy present in newborn period VSD: less cyanosis, signs of HF, cardiac shadow “egg on Complete surgical repair: Atrial Depends on presence of ASD, palpable left & right ventricular string” switch within 2 w of life. VSD, PDA for survival impulses, loud VSD murmur, single S2 Echo: transposition, mixing TCA 2% Infant severely cyanotic, single S2, ECG: LVH, superior QRS axis VS intact: PE1 until surgery, or VSD Hypoplastic right ventricle frequently no murmur (VSD murmur, (between 0 & -90°) Surgery staged (3): subclavian AP Depends on PDA or VSD for diastolic murmur over mitral valve) CXR: normal-mildly enlarged shunt (Blalock-Taussig survival cardiac silhouette, PBF procedure), 2-stage procedure (biD Glenn, Fontan) TA <1% Cyanosis, HF, PVR, tachypnea, cough, ECG: combined ventricular Anticongestive medication Left and right ventricle empty into bounding peripheral pulses. hypertrophy, cardiomegaly Surgical repair: VSD closure, a single trunk that then divides Single S2, SEC, systolic murmur (LSB) CXR: pulmonary blood, conduit between RV & PA displaced PA Echo: VSD, origin of PA TAPVR 1% Infants without obstruction: minimal Iw/oO: ECG: RV volume Surgery: common pulmonary vein cyanosis, asymptomatic, hyperactive overload. CXR: cardiomegaly, is opened into the left atrium RV impulse (LUSB), mid-diastolic PBF murmur (LLSB), poor growth. IwO: ECG: RAD, RVH, CXR: Infants with obstruction: cyanosis, pulmonary edema (similar to marked tachypnea, signs of RSHF HMB or pneum.) (hepatomegaly), no murmur. HLHS 1% HF, weak to absent pulses, single loud ECG: RVH, LV forces PE1, correct acidosis, supportive Most common cause of death S2, no heart murmur, minimal CXR: right sided enlargement, Surgical repair (staged): Norwood, from cardiac defects in the first cyanosis, low CO, grayish color of skin pulmonary venous congestion bidirectional Glenn and Fontan month of life & edema procedures. Failure of development of mitral or Echo: small left side of heart, aortic valve or aortic arch hyoplastic ascending aorta Dependent on right-to-left shunting at DA for survival TOF, Tetralogy Of Fallot; TOGA, Transposition Of The Great Arteries; TCA, Tricuspid Atresia; TA, Truncus Arteriosus; TAPVR, Total Anomalous Pulmonary Venous Return; HLHS, Hypoplastic Left Heart Syndrome