"Congenital heart disease mumurs pediatric electrocardiograms"
Newborn Congenital Heart Disease Matthew Snyder, DO, Maj Family Medicine/Obstetrics 31 Mar 10 Pre-Test • Question 1: • Question 2: • Question 3: List 3 murmur referral criteria • Question 4: What’s your next move? Objectives • Become comfortable with newborn cardiac exam • Identify cardiac exam “red flags” • Describe referral criteria • Describe initial mgnt of cyanotic newborn First things first! • Epidemiology ▫ CHD occurs in 8/1000 births ▫ Spectrum ranges from asymptomatic/benign to fatal ▫ Most cases multifactorial, but may be due to: Chromosomal disorders Teratogens Maternal metabolic disease Initial Newborn Assessment • Points to consider: ▫ Have high index of suspicion ▫ Consider maternal/antenatal risk factors and prenatal testing ▫ Don’t rush through exam ▫ Perform in quiet environment ▫ Perform cardiac exam before infant cries if possible The Basics • Inspection for cyanosis – central cyanosis is NEVER normal ▫ Common causes: Respiratory Distress Syndrome Sepsis Cyanotic heart disease ▫ May present with little or no tachypnea ▫ Peripheral cyanosis with tachypnea/tachycardia - sepsis Neonatal Cyanosis Differential Diagnosis System/Disease Mechanism Pulmonary Respiratory distress syndrome Surfactant deficiency Sepsis, pneumonia Inflammation, pulmonary hypertension, ARDS Meconium aspiration pneumonia Mechanical obstruction, inflammation, pulmonary hypertension Persistent pulmonary Pulmonary hypertension hypertension of the newborn Diaphragmatic hernia Pulmonary hypoplasia, pulmonary hypertension Transient tachypnea Retained lung fluid Cardiovascular Cyanotic heart disease with Right-to-left shunt as in pulmonary atresia, tetralogy of Fallot decreased pulmonary blood flow Cyanotic heart disease with Mixing lesion as in single ventricle or truncus arteriosus increased pulmonary blood flow Cyanotic heart disease with Right-to-left shunt with pulmonary edema and poor cardiac output as in hypoplastic left congestive heart failure heart and coarctation of aorta Heart failure alone Pulmonary edema and poor cardiac contractility as in sepsis, myocarditis, supraventricular tachycardia, or complete heart block; high-output failure as in PDA or vein of Galen or other arteriovenous malformations CNS Maternal sedative drugs Hypoventilation, apnea Asphyxia CNS depression Intracranial hemorrhage CNS depression, seizure Neuromuscular disease Hypotonia, hypoventilation, pulmonary hypoplasia Hematologic Acute blood loss Shock Chronic blood loss Congestive heart failure Polycythemia Pulmonary hypertension Methemoglobinemia Low-affinity hemoglobin or red blood cell enzyme defect Metabolic Hypoglycemia CNS depression, congestive heart failure Adrenogenital syndrome Shock (salt-losing) The Basics • Palpate pulses in all four extremities • Consider PMI/thrill to r/o: ▫ Dextrocardia ▫ Outflow tract obstruction ▫ Restrictive VSD The Basics • Heart Rate – increased with shock, heart failure, dysrhythmias • Resp. Rate – increased with left-to-right shunting ▫ Hypoplastic left heart ▫ Critical AS ▫ Coarctation The Basics • Blood pressure (if indicated) ▫ Perform right arm first (preductal), if elevated then left arm/leg ▫ R/o aortic coarctation Listen to the Beat! • Note the heart sounds (S1,S2) first • S2 may be split with inspiration • Widely split S2: ▫ ASD ▫ Pulmonary stenosis ▫ MR ▫ AS What’s that sound? • Murmur evaluation: ▫ Systolic ▫ Diastolic ▫ Continuous • Note intensity and frequency • May have murmur during first few hours of life as PDA closes Telling the difference… • Innocent murmurs ▫ Intensity ≤ grade II, LSB ▫ Normal S2 ▫ No audible clicks ▫ Normal pulses ▫ No other abnormalities Telling the difference… • Still Murmur ▫ Age 3-6 mo, LUSB, Grade II-III • Pulmonary Flow Murmur ▫ Newborn (Premie), systolic, LUSB, radiates to back, Grade I-II, resolves by 3-6 mo. • Venous Hum ▫ Systolic/diastolic, supraclavicular, Grade I-III ▫ Key: disappears/diminishes when head turned to the side or jugular vein compressed Telling the difference… • CHD murmurs – referral criteria ▫ Intensity ≥ grade III ▫ Harsh quality ▫ Pansystolic duration ▫ Loudest ULSB ▫ Abnormal S2 ▫ Absent or diminished femoral pulses ▫ Other abnormalities (clicks, S3, etc) ▫ Diastolic murmur Classification • Acyanotic CHD • L-to-R • Either left-to-right or ▫ VSD obstructive stenotic ▫ ASD lesions ▫ PDA • Increased pulmonary • Obstructive flow with normal SpO2 ▫ Pulm. Stenosis ▫ Aortic Stenosis ▫ Coarctation of the Aorta Classification • Cyanotic CHD • 5 “Ts” • Right-to-left shunting ▫ Tetralogy of Falot • Bypasses the lungs ▫ Transposition of the • Present clinically in Great Arteries the first 2 wks of life ▫ Tricuspid Atresia ▫ Truncus Arteriosis ▫ Total Anomalous Pulmonary Venous Return CHD Newborn Management • Start with basics: ▫ Call for help – Pediatrics/Tertiary Care Unit ▫ ABCs via NRP • May require intubation/respiratory support • Establish venous and arterial umbilical access CHD Newborn Management • If cyanosis, determine if from cardiac or pulm etiology • If unknown cause – assume sepsis and begin abx (Amp/Gent or Amp/Claforan) CHD Newborn Management • Obtain the following: ▫ CBC ▫ Blood cx ▫ UA ▫ CXR Cardiomegaly Pulm vascular markings – decreased in cyanotic CHD ▫ EKG ▫ ABG CHD Newborn Management • Hyperoxia Test ▫ Distinguishes b/n cardiac and pulm causes of cyanosis ▫ Give 100% O2 via oxyhood or ETT for 10 min ▫ Measure ABG or pulse ox from right arm (preductal) ▫ If PO2 rises ≈ likely pulmonary cause CHD Newborn Management • Hyperoxia Test (cont) ▫ If fails the test (no rise in PaO2): Likely ductal-dependent cardiac abnormality ▫ If echo not immediately available or metabolic acidosis: Talk with Peds/Neonatology and consider Prostaglandin E1 to maintain ductus arteriosis Post-Test • Question 1: • Question 2: • Question 3: List 3 murmur referral criteria • Question 4: What’s your next move? References • Kliegman, Rm, et al. Nelson Essentials of Pediatrics, 5th ed. 2006. • Uptodate.com • http://depts.washington.edu/physdx/heart/de mo.html Ventricular Septal Defect • 25% of all CHD • Involves muscular or (peri)membranous septum (67%) • Small – asymptomatic with loud pansystolic murmur (LLSB) • Split S2 • LAH & LVH on EKG • 35% close spontaneously Atrial Septal Defect • 10% of CHD • Secundum defect (foramen ovale) – most common • SEM Grade I-II, split S2 • R axis deviation/RVH • If present age 3 = surgical closure Patent Ductus Arteriosis • 5-10% of CHD • Widened pulse pressure • Continuous machine- like murmur/thrill • Diuretics/Digoxin vs surgical closure Endocardial Cushion Defect • Failure of septal fusion with endocardial cushion ≈ abnormal AV valves • CHF sx, prominent S2 • L axis deviation, VH & AH • Diuretics/Digoxin vs surgical closure Tetralogy of Falot • 10% of CHD – most common • VSD, Pulm Stenosis, Overriding Aorta, RVH • First finding – pulm stenosis murmur • Hypoxic (“Tet”) spells • Boot-shaped heart • R axis deviation, RVH Transposition of the Great Vessels • 5% of CHD • Cyanosis depends on mixing via ASD, VSD, PDA • Quiet tachypnea • R axis deviation, RVH • “Egg on a string” • Prostaglandin E1, surgery Triscuspid Atresia • 2% of CHD • Hypoplastic RV • Requires PDA or VSD • Severe cyanosis, single S2 • LVH on EKG • Prostaglandin E1 and/or surgery Truncus Arteriosis • <1% of CHD • Large arterial trunk with VSD • Single S2, SEM • Bil VH, cardiomegaly • Surgical repair Total Anomalous Pulm Ven Return • 1% of CHD • ASD required • Widely split S2, SEM • Obstruction = cyanosis • R axis deviation, RVH • Pulmonary edema on CXR • Surgical repair Hypoplastic Left Heart • 1% of CHD • Failed development of MV, AV or aortic arch • PDA dependent • CHF, low cardiac output • RVH on EKG • Prostaglandin E1/surgical repair