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

Indications for Fetal Echocardiography

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

Specific Heart Disease Abnormalities

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)

Tetralogy of Fallot

Tetrology of Fallot

Ebstein’s Anomaly

Tricuspid Atresia

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: 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

Transposition of the Great Arteries

d - Transposition of the Great Vessels

Truncus Arteriosus

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

Total Anomalous Pulmonary Venous Return

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

Coarctation of the Aorta

Hypoplastic Left Heart Syndrome

Aortic Stenosis

Acyanotic With Increased Pulmonary Blood Flow VSD ASD PDA Endocardial cushion defect

Ventricular Septal Defect

Atrial Septal Defect

Atrioventricular Canal

Patent Ductus Arteriosus

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