Cardiac Murmurs in the Newborn Infant Carrie Phillipi MD, PhD Prevalence of Congenital Heart Disease (CHD) • <1 per 100 live births Most cardiac murmurs are benign! Risk Factors for CHD (when is CHD more likely) • Family history of CHD • Increased maternal age: increased risk for chromosomal abnormalities • Genetic syndromes (Down syndrome, Turner’s) • Finding of other major anomalies (VATER, cleft palate, neural tube, etc.) • Plurality (multiple gestation) Maternal Risk Factors for CHD • Maternal Diabetes: 3-5% risk of CHD – VSD, conotruncal defects, d-TGA – Risk reduced with good diabetic control – Importance of supplemental vitamins (Folate) • Maternal systemic lupus – Complete heart block – Transplancental passage of maternal antiSS-A or anti-SS-B autoantibodies Genetic Risk for CHD • Chromosome Disorders – Trisomy 21 (Down Syndrome) • ~50% have CHD • AVSD, VSD, PDA, ASD – Trisomy 18 and 13 • >90% have CHD • VSD, DORV, PDA, conotruncal defects Genetic Risk for CHD • Chromosome Disorders – 45X deletion syndrome (Turner syndrome): 35% have CHD: coarctation, bicuspid aortic valve, potential for developing aneurysm of aorta – 22q11 deletion (DiGeorge syndrome; Velocardio-facial syndrome, CATCH-22) • Truncus, Tetralogy, Interrupted Aortic Arch • Need to screen parents. Neonatal Manifestations of Congenital Heart Disease • Heart Murmur (~ 50%) • Cyanosis and/or Arterial Desaturation using pulse oximetry • Pallor or Poor Perfusion • Tachypnea • Tachycardia • Slow growth (FTT) Heart Murmurs • ~50% of newborns with significant CHD have minimal or no murmur at birth (large VSD, single ventricle, transposition, TAPVR) • Left to right shunt defects such as large VSD do not develop murmurs until the PVR drops (days to weeks Cardiac murmur is an insensitive marker for CHD Grading Heart Murmurs 1 Softer than Heart Sounds 2 Equal to Heart Sounds 3-6 Louder than Heart Sounds The Cardiac Exam • Observation • Palpation (precordium, pulses, perfusion, liver) • Auscultation Listening is just one step. Be as descriptive as possible. Heart Murmurs Remember most cardiac murmurs are benign, but… • Factors that increase likelihood of CHD – Persistence – Intensity – Association with any symptoms Unexplained Tachypnea • Persistent resting tachypnea (greater than 60/minute) and no murmur • Need to rule out cardiac etiology • ~10% of CHD presents with this finding • Importance of chest X-ray and Pulse Oximetry Screening tests • Pulse oximetry • Chest X-ray • 4 extremity blood pressure • EKG: best used when concern is cardiac rhythm Infant with Cardiomegaly Cyanotic vs. Acyanotic CHD • Pulse Oximetry! • The cyanotic infant needs immediate assessment! Acrocyanosis Cyanotic Infant Oxygen challenge Lung vs. Heart??? Oxygen supplementation will not correct pulse oximetry to 100% with cyanotic congenital heart disease. Transient Ductus Murmur of the Healthy Newborn • Soft systolic murmur, grade 1-2 in intensity, often higher pitched • Caused by flow in closing ductus. Typically heard best at 4-16 hours of life • Studies indicate that it can be heard in most newborn infants • Hallmark of this murmur is its transient nature • Infants are asymptomatic (normal feeding; breathing comfortably) • The Auscultation Assistant - Systolic Murmurs Delayed closure of PDA in Premature Infants • Risk factors include lower gestational age and finding of RDS • Should be suspected in any preemie with respiratory symptoms and new onset murmur • Larger PDA’s in the sickest preemie infants are often silent • CXR: ground-glass or “wet” appearance Physiologic Peripheral Pulmonary Stenosis (PPS) • Murmur of PPS is high pitched • Heard well in both axillae and into posterior lung fields • More common in infants born prematurely • Infants are asymptomatic • Resolves by 3-6 months • CXR, EKG, & Pulse oximetry: normal Innocent (Still’s) Murmur • Lower pitched • Soft, grade 1-2 in intensity • Described as “musical”, “vibratory”, “twanging”or “barking seal” • Often heard best at cardiac apex • CXR and pulse oximetry normal • The Auscultation Assistant - Systolic Murmurs Infant Heart Murmurs • Asymptomatic Infant with persistent murmur (feeding well, normal pulse oximetry and CXR): needs close follow-up, echo/cardiac referral when appropriate • Infant with murmur and any other clinical sign: (tachypnea, decreased pulses, abnormal CXR, arterial desaturation): needs evaluation ASAP! CHD with Left to Right Shunts (common cause of early cardiac murmurs) • • • • VSD ASD PDA AVSD (AV canal defect) 50% of children with CHD will have one of these defects! Ventriculoseptal Defect (VSD) • Most Common Cardiac Defect (35% of all cardiac defects) • 50% male:female • Small defects are detected earlier (1st days of life) than larger defects • Murmur is due to systolic pressure difference between LV and RV • The Auscultation Assistant - Systolic Murmurs VSD: Natural History • ~ 50%: close spontaneously • ~ 25%: persistent small shunts that do not require surgery (risk for SBE) • ~ 25%: require surgical closure ASD • Soft systolic murmur, grade 1-2 intensity, heard in pulmonary listening area (can mimic the murmur of PPS) • Typically not heard for several weeks • Most infants with ASD are asymptomatic • Mild cardiomegaly on CXR • Normal Pulse Oximetry • The Auscultation Assistant - Systolic Murmurs ASD: Clinical Findings and Natural History • 65% female prevalence • Murmur frequently sounds benign and is often overlooked! • Murmur: due to increased flow across pulmonary valve • Spontaneous closure occurs in those diagnosed in 1st month of life • PFO (defects < 3 mm) is common and benign • No risk for SBE AV Septal Defect • Also called AV Canal defect or Endocardial Cushion defect • ASD, VSD, and commonly AV regurgitation • Large VSD allows systemic pressure transmission into pulmonary vascular bed • Associated with high PVR and systemic RV pressure (and hence, absent or soft murmur) Coarctation: Clinical Findings • Systolic murmur, grade 2 intensity, at left midclavicular area to mid-scapular area • Diminished lower extremity pulses • Need for 4-extremity blood pressures • Difficult diagnosis in the newborn prior to ductal closure • 65% male prevalence • Think possible Turners syndrome in girls with coarctation Critical Coarctation (or IAA) • All pulses diminished with cool mottled extremities, delayed capillary refill, pallor, poor perfusion, acidosis, low cardiac output • Cardiac murmur often minimal; listen for gallop • Need for PGE infusion • Need for cardiac operation Heart Sounds & Murmurs • The Auscultation Assistant - Systolic Murmurs