Neonatal Cardiac assessment Eric Towe, MD Pediatric Cardiology Avera Children’s Hospital University of South Dakota Sanford School of Medicine

Objectives Incidence of congenital defects Discuss types of congenital heart defects Outline clinical features of congenital heart defects Overview of specific congenital heart defect care

Objectives Incidence of congenital defects Discuss types of congenital heart defects Outline clinical features of congenital heart defects Overview of specific congenital heart defect care

Congenital heart defects Most common congenital defect Occur in 6-13/1000 live births Bicuspid aortic valve Ventricular septal defect (VSD) Atrial septal defect (ASD)

Congenital heart defects Cyanotic defects – 15% Pulse ox <80% Tetralogy of Fallot Critical defects – 25-33% Require surgical or cath intervention in first year of life

Congenital heart defects Leading cause of neonatal death prenatal diagnosis 58% Operator expertise Gestational age Fetal position Type of defect

Congenital heart defects Most infants identified soon after birth Some not until after discharge 30% critical Increased morbidity and mortality if delay

Objectives Incidence of congenital defects Discuss types of congenital heart defects Outline clinical features of congenital heart defects Overview of specific congenital heart defect care

Neonatal cardiac anatomy

classification Cyanotic congenital heart disease Deoxygenated blood into systemic circulation shunts

Ductal-dependent Circulation dependent of patent ductus arteriosus (PDA) Only source of blood to lungs or body Many cyanotic lesions are ductal dependent

Critical congenital heart disease Requires surgical repair or catheter intervention within first year Ductal-dependent, cyanotic, other less forms 25% of all chd

Acyanotic heart disease Congenital defect with normal pulse ox Wide range of severity

Objectives Incidence of congenital defects Discuss types of congenital heart defects Outline clinical features of congenital heart defects Overview of specific congenital heart defect care

Initial diagnosis and evaluation History Physical exam Chest x-ray Hyperoxia test EKG/echocardiogram

history Risk factors Poor feeding Color change Irritability/sweating Poor weight gain Excessive sleeping

Risk factors Family history Multiple fetuses Genetic syndromes

Genetic syndromes 7-12% congenital heart disease Most common Trisomy 21 Turner syndrome Digeorge syndrome

Risk factors Family history Multiple fetuses Genetic syndromes Maternal factors

Maternal factors Obesity Diabetes Medications NSAIDS Ace Retnoic acid

Maternal factors Advanced maternal age Hypertension Alcohol/substance abuse Epilepsy Infection – rubella, cmv, parvo b19, etc. Assisted reproductive technology

Physical findings Can present shortly after birth Days after discharge Shock, cyanosis, tachypnea, pulmonary edema

shock Most common left heart obstructive lesions Hypoplastic left heart Critical aortic valve stenosis Critical coarctation Interrupted aortic arch

shock Infants present in shock as ductus arteriosus closes Initiation of pge1 essential Must differentiate from septic shock Cardiomegaly and lack of response to volume – suggests cardiac

Bluish skin tone caused by deoxygenated hemoglobin cyanosis Bluish skin tone caused by deoxygenated hemoglobin Important sign in multiple types of CHD May not be seen Mild desaturations (>80%) Anemia Darker skin tones

Ductal dependent lesions cyanosis Ductal dependent lesions Right heart obstruction

Pulmonary atresia

Ductal dependent lesions cyanosis Ductal dependent lesions Right heart obstruction Left heart obstruction

Critical aortic valve stenosis

Ductal dependent lesions cyanosis Ductal dependent lesions Right heart obstruction Left heart obstruction Parallel circulations

Transposition of the great arteries

Ductal dependent lesions cyanosis Ductal dependent lesions Right heart obstruction Left heart obstruction Parallel circulations Non-ductal dependent

Differential cyanosis Difference in pulse ox of >3% between right hand and lower extremity Pre versus post ductal Coarctation of the aorta

Differential cyanosis Difference in pulse ox of >3% between right hand and lower extremity Pre versus post ductal Coarctation of the aorta Persistent pulmonary hypertension of the newborn (PPHN)

cyanosis Non-cardiac causes Pulmonary disorders Abnormal hemoglobin Sepsis, hypoglycemia acrocyanosis Acrocyanosis – hands and feet – not oral mucosa

Increased work of breathing Poor feeding Respiratory symptoms Tachypnea Increased work of breathing Poor feeding Rapid increase in pulmonary blood flow Drop in pulmonary vascular resistance PDA in premature infants Truncus arteriosus

Cardiac versus pulmonary disease Further workup needed if: Persistently elevated respiratory rate (>60 bpm) Distress during feeding Cough and wheeze more likely pulmonary disease

Physical exam Key component to neonatal assessment Fails to detect more than half of infants with chd Subtle findings provide clues Findings may be absent in ductal dependent lesions if PDA still open

Findings suggestive of CHD Abnormal heart rate Abnormal precordial activity Abnormal splitting s2 Abnormal extra heart sounds Pathologic murmurs Hepatomegaly Diminished pulses in lower extremities Extracardiac abnormalities

EKG best course for further evaluation if persistent Abnormal heart rate Normal range 90-160 bpm Up to six days of life Higher or lower rates EKG best course for further evaluation if persistent

Abnormal precordial activity Precordial palpation for normal placement Left side of chest Right side – dextrocardia Complex CHD Cardiac enlargement + respiratory symptoms = likely chd Order chest x-ray if concerned

Cardiac auscultation is key component to evaluation Abnormal heart sounds Cardiac auscultation is key component to evaluation Timing of murmur S1/S2 Extra heart sounds Pathologic murmurs

Cardiac cycle Systole diastole

S2 normally splits with inspiration Abnormal splitting s2 S2 normally splits with inspiration S2 splitting reduces likelihood of CHD Infant heart rate often too high to hear 80 of newborns by 48 hours <150 bpm

Defects with Abnormal s2 Single s2 Aortic atresia Pulmonary atresia Tetralogy of fallot Pulmonary hypertension Wide fixed split s2 ASD (not all)

Presence of heart murmurs often associated with CHD Not all CHD has a heart murmur 80% of children will have a heart murmur 1% pathologic

Sound of blood moving through heart Heart murmurs Sound of blood moving through heart Turbulent flow Pressure difference Ventricular function

Timing Systolic Early Mid Late Pan Diastolic Continuous

Heart murmurs Systolic 1-6/6

Intensity Grade + Thrill - Thrill LOUDEST, Stethoscope off chest Louder, Edge of stethoscope Intensity Grade 1 2 3 4 5 6 Loud, Palpable thrill 6 Easily heard, Intermediate intensity 5 Faint, Heard immediately 4 + Thrill - Thrill Faint, with concentration 3 2 1

Heart murmurs Systolic 1-6/6 Diastolic 1-4/4 Never innocent

Location Aortic area Pulmonic area A P Mitral area Tricuspid area T M

Normal liver edge 1-3 cm below right costal margin Enlarged liver Hepatomegaly Normal liver edge 1-3 cm below right costal margin Enlarged liver Heart failure Increased central venous pressure

Diminished pulses Cool/mottled lower extremities Essential part of evaluation Decreased or absent pulses lower extremity Coarctation Aortic arch obstruction Usually accompanied with bounding pulses in upper extremities Cool/mottled lower extremities

Blood pressure Monitor upper and lower extremities Gradient > 10 mmHg (upper>lower) Appropriate cuff size Neonatal hypertension

Extracardiac abnormalities 22% of infants with chd – extracardiac defects Skeletal Hand or arm Chromosomal abnormalities 12.3% of infants with CHD had chromosomal abnormality Down syndrome Turners Digeorge

Pulse ox screening

Pulse ox screening Performed after 24 hours or as late as possible if early discharge Measure right hand (pre-ductal) and either foot Positive screen based on AAP: SpO2 < 90% in either extremity SpO2 < 95% in both upper and lower extremities on 3 measurements – one hour apart SpO2 difference > 3% between upper and lower on 3 measurements – one hour apart

Reduces diagnostic gap to 5-10% Pulse ox screening Positive screen Identify cause of hypoxia Echocardiography Consultation with pediatric cardiologist Reduces diagnostic gap to 5-10% Ductal dependent lesions

Diagnostic approach Presence or absence of symptoms determines approach Symptomatic infants Urgent consultation Physical exam Pulse ox/Hyperoxia test Chest x-ray Echocardiogram

Differentiate between cardiac and pulmonary Chest x-ray chd Differentiate between cardiac and pulmonary Obtain in cyanosis/respiratory symptoms Findings consistent with chd Cardiomegaly Dextrocardia Abnormal cardiac silhouette Arch sidedness (right) 20% TOF right sided aortic arch

Abnormal cardiac silhouette Boot shaped heart Tetralogy of Fallot

Abnormal cardiac silhouette Egg on a String Transposition of the Great Arteries

Abnormal cardiac silhouette Egg on a String Transposition of the Great Arteries

Abnormal cardiac silhouette Wall to Wall or Basketball Ebstein Abnormality

Hyperoxia test Distinguish cardiac from non-cardiac causes of cyanosis Measure arterial PaO2 right radial artery (preductal) and foot Taken during room air and after 10 minutes on 100% FiO2 If systemic PaO2 >150 mmHg after O2 administration - more likely pulmonary Abnormal PaO2 doesn’t “rule in” CHD Should not use pulse ox as surrogate to PaO2

Echocardiography Definitive diagnosis of chd Consult pediatric cardiology Shock not responsive to volume Cyanosis Failed hyperoxia test Abnormal CXR Physical findings Abnormal pulse ox screening Genetic Disorder or extracardiac malformation

Diagnostic approach Asymptomatic infants Presence or absence of symptoms determines approach Symptomatic infants Urgent consultation Physical exam Pulse ox/Hyperoxia test Chest x-ray Echocardiogram Asymptomatic infants

Asymptomatic infants Pulse ox screening Careful physical examination Careful history If questions/concerns - call

Objectives Incidence of congenital defects Discuss types of congenital heart defects Outline clinical features of congenital heart defects Overview of specific congenital heart defect care

Initial management Cyanotic infants require immediate assessment and general supportive care Airway management IV access Vital Signs Antibiotics if other cause cannot be identified Prostaglandin E1 Keeps ductus open Starting dose 0.05 mcg/kg/min Can cause apnea – have ET tube at bedside CXR, Echo if possible Consult Cardiology/Transport to tertiary care center

Initial management Heart murmur but otherwise asymptomatic Close clinical observation Vital signs Careful history and complete physical Pulse Ox screening Consider CXR/EKG/Echo Contact with any questions/Concerns

Summary Congenital heart disease is the most common congenital defect Careful history and physical exam is key Pulse Ox screening improves detection Most neonates present while in hospital Some present after discharge Careful history and physical – feeding, respiratory issues, cyanosis

Summary Consider CXR, hyperoxia test or echocardiogram if concerns Cyanotic infants require immediate care Airway/IV access Antibiotics PGE1 Echocardiogram Transfer/Cardiology consultation

Questions?

Thank you