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