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 Exam: general appearance, growth chart, vital signs including blood pressure, signs of respiratory distress, cyanosis Cardiac exam: inspection and palpation; 1st heart sound @ LLSB, single, mitral and tricuspid valve closure; 2nd heart sound @ LUSB; split, aortic and pulmonary valve closure; 3rd and 4th heart sounds @ apex Murmurs: Gr I- barely heard; Gr II- soft, but easily audible; Gr III- loud, recognized immediately; Gr IV-loud and associated with thrill; Gr V- audible with edge of stethoscope, thrill; Gr VI- audible without stethoscope Pulses: weak or bounding, symmetric

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

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

Maternal risk factors associated with congenital heart disease Cardiac teratogen exposure Lithium Amphetamines Alcohol Anticonvulsants: phenytoin, valproic acid, carbamazepine,and trimethadione Isotretinoin

Lungs vs heart: Differential cyanosis and the hyperoxia test PaO2 <50 and SpO2 <85% pre-ductal despite 100% FiO2 -PPHN -left-heart abnormalities Post-ductal saturation higher than pre-ductal saturation -TGA -TAPVR above diaphragm with PDA

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

Normal heart

Pulmonary Hypertension

Pulmonary Hypertension Preductal SpO2 Postductal SpO2 PA Ao

Transposition of great arteries Ao PA

Transposition Preductal SpO2 Postductal SpO2 Ao PA

TAPVR Preductal SpO2 Postductal SpO2

CXR Heart size Pulmonary blood flow Cardiac position Ebsteins, critical PS

Ebstein’s anomaly

Cyanotic with decreased pulmonary blood flow

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

Tetrology of Fallot

Tetrology of Fallot

Tetrology of Fallot Infundibular septum angled anteriorly

Tricuspid Atresia

Tricuspid Atresia

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

Management right sided obstructive lesions PGE Supplemental O2 is OK (may slightly improve pulmonary vasodilatation) Surgical intervention

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

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

Hypoplastic left heart syndrome

Aortic stenosis

Hypoplastic Left Heart Syndrome PDA supplies: body lungs head coronaries

Coartation of aorta

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 Normal pulmonary vessels should not be seen beyond the middle 1/3 of lung

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

d - Transposition of the Great Vessels

Transposition of Great Arteries Mixing at PFO and PDA

Truncus arteriosus

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 Normal pulmonary vessels should not be seen beyond the middle 1/3 of lung

Treatment of mixing lesions: TGA PGE Avoid too much PBF, may worsen patient Balloon septostomy Supplemental O2 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

Supracardiac TAPVR

Management TAPVR Ventilation with PEEP Diuretics PGE may worsen patient iNO will worsen patient Surgical intervention

Initial stabilization Airway management: use of neuromuscular blockade Titrate Fi02 to keep Sp02 80%-85%. Use of PGE1 (0.02 to 0.05 mcg/kg/min)

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

Stabilization for transport Reliable vascular access Intubation if on PGE1 Oxygen delivery, Sp02 Monitor HR, tissue perfusion, blood pressure, and acid-base status Calcium and glucose status

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 Superior axis: tricuspid atresia, primum ASD, complete endocardial cushion defect.

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

Ventriculo septal defect

Cardiac malpositions and heterotaxy

Dextrocardia Heterotaxy syndrome: abnormal cardiac and visceral situs, abnormal bronchial anatomy, associated malrotation Right sided isomerism: two right lungs (tri-lobed lungs) and asplenia Left sided isomerism: two left lungs (bi-lobed lungs) and polysplenia