Congenital Heart Disease Initial evaluation and stabilization Priscilla

Congenital Heart Disease Initial evaluation and stabilization Priscilla Joe, MD Children’s Hospital and Research Center Oakland

Initial evaluation § History § Physical exam with 4 extremity blood pressures § Pre-ductal and post-ductal oxygen saturations § Hyperoxia test § CXR § EKG § ECHO 2

Indications for fetal echocardiography Fetal risk factors associated with CHD: § Trisomies, Turner’s syndrome, abnormal karyotype § Congenital malformations: duodenal atresia, TEF, omphalocele, diaphragmatic hernia, renal dysgenesis, and hydrocephalus § Fetal arrhythmias § IUGR § Nonimmune hydrops 3

Maternal metabolic disorders or infection § § § Diabetes mellitus PKU Hyperthyroidism Lupus, collagen vascular disease Rubella, CMV, Coxsackie, HIV 4

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 5

Lungs vs heart: Differential cyanosis and the hyperoxia test § Pa. O 2 <50 and Sp. O 2 <85% pre-ductal despite 100% Fi. O 2 -PPHN -left-heart abnormalities § Post-ductal saturation higher than preductal saturation -TGA -TAPVR above diaphragm with PDA 6

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Neonatal Heart Disease § Ductal dependent lesions § Congestive heart failure § § Right heart obstructive lesions Left heart obstructive lesions Mixing lesions Inadequate gas exchange 8

Normal heart 9

Pulmonary Hypertension 10

Pulmonary Hypertension Preductal Sp. O 2 Postductal Sp. O 2 PA Ao 11

Transposition of great arteries Ao PA 12

Transposition Preductal Sp. O 2 Postductal Sp. O 2 Ao PA 13

TAPVR Preductal Sp. O 2 Postductal Sp. O 2 14

CXR § Heart size § Pulmonary blood flow § Cardiac position 15

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Ebstein’s anomaly 17

Cyanotic with decreased pulmonary blood flow 18

Right Sided Obstructive Lesions. Blue, but comfortable § § Cyanosis No respiratory distress Normal pulses and perfusion Single second heart sound (no closing sound from abnormal pulm valve) § Murmur § Moderate to marked hypoxemia § CXR: normal to large sized heart, decreased PBF 19

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Tetrology of Fallot 21

Tetrology of Fallot 22

Tetrology of Fallot Infundibular septum angled anteriorly 23

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Tricuspid Atresia 25

Tricuspid Atresia 26

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 27

Management right sided obstructive lesions § PGE § Supplemental O 2 is OK (may slightly improve pulmonary vasodilatation) § Surgical intervention 28

Left sided obstructive lesions Acute shock

Left sided obstructive lesions § § § § § Grey or ashen color (may not be blue) Tachypnea Poor perfusion Decreased pulses/differential pulses Single second heart sound Murmur + gallop Hepatomegaly ABG: metabolic acidosis CXR: cardiomegaly with increased PBF 30

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 31

Hypoplastic left heart syndrome 32

Aortic stenosis 33

Hypoplastic Left Heart Syndrome PDA supplies: • body • lungs • head • coronaries 34

Coartation of aorta 35

HLHS Treatment § § § § Fetal diagnosis is vital to prevent end organ failure PGE Balance perfusion to body/coronaries/head vs lungs Avoid oxygen, hyperventilation, pressors to limit PBF Control ventilation; paralyze and hypoventilate Blend in nitrogen to raise PVR and limit PBF Surgical intervention

Cyanotic with increased pulmonary blood flow Inadequate mixing 37

Inadequate Mixing Lesions § § § § Cyanosis, often profound Mild tachypnea Normal pulses Single heart sound Murmur ABG: marked hypoxemia, + acidosis CXR: cardiomegaly, normal or increased PBF 38

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d - Transposition of the Great Vessels 40

Transposition of Great Arteries Mixing at PFO and PDA

Truncus arteriosus 42

Truncus arteriosus

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 45

Treatment of mixing lesions: TGA § PGE § Avoid too much PBF, may worsen patient § Balloon septostomy § Supplemental O 2 may be helpful § Surgical repair

Lesions with poor gas exchange

Lesions with poor gas exchange § Cyanosis § Marked tachypnea (difficult to differentiate from GBS pneumonia/MAS § Perfusion fair, pulses normal § Second heart sound may be single § May or may not have a murmur § CXR: normal heart size, pulmonary congestion

Total anomalous pulmonary venous return 49

Supracardiac TAPVR

Management TAPVR § § § Ventilation with PEEP Diuretics PGE may worsen patient i. NO will worsen patient Surgical intervention

Initial stabilization § Airway management: use of neuromuscular blockade § Titrate Fi 02 to keep Sp 02 80%85%. § Use of PGE 1 (0. 02 to 0. 05 mcg/kg/min) 52

Prostaglandin E 1 § Failure to respond: diagnosis incorrect, older infant with unresponsive ductus, ductus absent, obstructed pulmonary venous return § Clinical deterioration after PGE 1: 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) 53

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 54

Stabilization for transport Reliable vascular access Intubation if on PGE 1 Oxygen delivery, Sp 02 Monitor HR, tissue perfusion, blood pressure, and acid-base status § Calcium and glucose status § § 55

EKG : QRS axis § Tricuspid atresia with PS or PA : superior § Critical PS or PA : 0 to 90 degree quadrant § TOF and TOF with PA: 90 -180 degree quadrant 58

Acyanotic with increased pulmonary blood flow § § VSD ASD PDA Endocardial cushion defect 59

Ventriculo septal defect 60

Cardiac malpositions and heterotaxy 61

Dextrocardia 62