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Diagnosis and Early Management of the Infant with Suspected Congenital Heart Disease. Introduction. Congenital heart disease occurs in 1% of live-born infants Almost 1/2 of all cases of congenital heart disease are diagnosed during the 1st week of life
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Diagnosis and Early Management of the Infant with Suspected Congenital Heart Disease
Introduction • Congenital heart disease occurs in 1% of live-born infants • Almost 1/2 of all cases of congenital heart disease are diagnosed during the 1st week of life • The most frequently occuring anomalies seen during the 1st week are: PDA, D-transposition of the great arteries, hypoplastic left heart syndrome, TOF, and pulmonary atresia
Maternal Risk Factors Associated With Congenital Heart Disease • Congenital heart disease • Cardiac teratogen exposure • Lithium • Amphetamines • Alcohol • Anticonvulsants: phenytoin, valproic acid, carbamazepine, and trimethadione • Isotretinoin
Maternal Metabolic Disorders or Infection • Diabetes mellitus • PKU • Hyperthyroidism • Lupus, collagen vascular disease • Rubella, CMV, Coxsackie, Parvovirus
Fetal Risk Factors Associated With Congenital Heart Disease • Trisomies, Turner’s syndrome, abnormal karyotype • Congenital malformations: duodenal atresia, TEF, omphalocele, diaphragmatic hernia, renal dysgenesis, and hydrocephalus • Fetal arrhythmias • IUGR • Nonimmune hydrops • ?2 vessel cord
Cyanosis • Etiology: CV, pulmonary, airway obstruction, neurological, neuromuscular, or hematological (methemoglobinemia or polycythemia) • Infants can appear cyanotic when the deoxygenated Hgb concentration is at least 3g/dL; it is not related to the percent saturated • 2 babies with sats of 80%: one with a hgb of 20g/dL and 4g/dL of desaturated hgb will be cyanotic, but an anemic infant with 10g/dL with 2g/dL deoxygenated hgb will not be cyanotic
Evaluation • ABC’s • PE: murmur, pulses, precordium, respiratory status, HSM, color, capillary refill • 4 ext BPs: if SBP >10mmHg in right hand compared to lower ext, concerning for arch anomaly (though if normal may not rule it out) • Pre/post ductal saturations: if see a difference >5%, concerning for PPHN or left heart abnormalities
Evaluation (Continued) • Hyperoxia test: baseline pre-ductal ABG when infant in room air, then repeat on 100% FiO2 • Reason for ABG and not just sats: with a saturation of 100%, you can have a PaO2 of 80 or 300; very different • CXR: cardiomegaly; normal, increased, or decreased pulmonary vascularity • EKG • Echo
Cyanotic With Decreased Pulmonary Blood Flow • Tetrology of Fallot • Ebsteins Anomaly • Tricuspid Atresia with PA or PS • Pulmonary atresia with intact septum • Critical pulmonic stenosis • PPHN
Right Sided Obstructive Lesions • Cyanosis • No respiratory distress • Normal pulses and perfusion • Single second heart sound • Murmur • Moderate to marked hypoxemia • CXR: normal to large sized heart, decreased pulmonary blood flow (PBF)
EKG : QRS axis • Tricuspid atresia with PS or PA with intact ventricular septum: superior (0— -90) • Critical PS or PA : 0 to 90 degree quadrant • TOF and TOF with PA: 90-180 degree quadrant
Cyanotic With Increased Pulmonary Blood Flow • d-Transposition of the great vessels • Truncus arteriosus • Total anomalous pulmonary venous return, above diaphragm • Single ventricle • Endocardial cushion defect
Inadequate Mixing Lesions • Cyanosis • Mild tachypnea • Normal pulses • Single heart sound • Murmur • ABG: marked hypoxemia, + acidosis • CXR: cardiomegaly, normal or increased PBF
Lesions with Poor Gas Exchange • Cyanosis • Marked tachypnea • Fair perfusion, normal pulses • May or may not have a single heart sound • May or may not have a murmur • CXR: normal heart size, pulmonary congestion
Left Sided Obstructive Lesions • Coarctation of aorta, interrupted aortic arch • Hypoplastic left heart syndrome • Aortic stenosis • Mitral stenosis • Total anomalous pulmonary venous return, below diaphragm
Left Sided Obstructive Lesions • Grey or ashen color • Tachypnea • Poor perfusion • Decreased pulses/differential pulses • Single second heart sound • Murmur + gallop • Hepatomegaly • ABG: metabolic acidosis • CXR: cardiomegaly with increased PBF
Acyanotic With Increased Pulmonary Blood Flow • VSD • ASD • PDA • Endocardial cushion defect
Initial Stabilization • ABC’s: Volume resuscitation, ionotorpic support, correction of metabolic acidosis, r/o sepsis • Intubate if needed, titrate Fi02 to keep Sp02 80%-85% to prevent pulmonary overcirculation • Placement of umbilical lines • Infants who present in shock within the first 3 weeks of life, consider ductal dependent lesions • Use of PGE1 (0.025 to 0.1mcg/kg/min)
Stabilization for Transport • Reliable vascular access • Intubation if on PGE1, OG placement • Oxygen delivery, Sp02 • Monitor HR, tissue perfusion, blood pressure, and acid-base status • Calcium and glucose status (increased risk for DiGeorge)
Prostaglandin E1 • Failure to respond: diagnosis incorrect, older infant with unresponsive ductus, ductus absent, obstructed pulmonary venous return • Clinical deterioration after PGE1: obstructed blood flow out of pulmonary veins or left atrium, HLHS with restrictive FO, TGA with intact ventricular septum and restrictive FO, obstructed TAPVR, mitral atresia with restrictive FO
PGE 1 - Side Effects • Common: Apnea, fever, leukocytosis, cutaneous flushing, and bradycardia. • Uncommon: seizures, hypoventilation, hypotension, tachycardia, cardiac arrest, sepsis, diarrhea, DIC, fever • Rare: urticaria, bronchospasm, hemorrhage*, hypoglycemia, and hypocalcemia *inhibits platelet aggregation