Congenital Heart Defects Craig T. Carter, D.O. Assistant Professor – EM/Peds University of Kentucky.

Congenital Heart Defects

Craig T. Carter, D.O.

Assistant Professor – EM/Peds

University of Kentucky

A nurse approaches you -

“Doctor, Doctor” - a kid just checked in with a history of Hypoplastic Left Heart syndrome – What is that again??”

You reply …..

“ I need to go to the bathroom, as soon as I get back, I will tell you all about it....”

So you sit and think…..

And think some more….

Back to Basics-Fetal Circulation

Four shunts of blood flow : placenta, ductus venosus, foramen ovale and ductus arteriosus

Back to Basics-Fetal Circulation

Changes after birth:

Shift of blood flow for gas exchange, from the placenta to the lungs.

Closure of ductus venosus :no placenta increase in pulmonary blood flow functional closure of foramen ovale

(increased LA pressure) Closure of ductus arteriosus (^O2)

Newborn Cardiac Exam

Vital signs: RR, HR, BP respiratory effort color palpate point of maximal impulse (PMI)

of heart palpate pulses of all extremities auscultate

Case 1: 2 day old infant

2 day old baby girl is being examined for discharge physical.

Vitals: HR:120 RR:40 BP: r arm 75/40 Gen : low hairline, webbed neck,

edemetous dorsum of feet

Case 1: cardiac exam

Normal PMI, no thrill No hepatosplenomegally unable to palpate pulses in lower

extremities BP’s all four extremities: both legs

systolic BP lower 40/30

Case 1: Auscultation

S2 splits normally ejection click systolic ejection murmur III/VI at the

URSB and LLSB systolic murmur radiates to the back early diastolic decrescendo murmur at

3LICS

Case 1:

What do you think is the congenital defect?

Case 1:

What further evaluation do you need?

Coarctation of Aorta (COA)

Coarctation of Aorta (COA)

Incidence: 8% of all congenital heart defects

Turner’s syndrome: 30% have COA

Preductal: 40% associated with other cardiac defects,symptomatic early in life

Postductal: less likely to have symptoms early

COA

EKG: LVH (but may be normal in 20%)

X-rays: heart size may be normal or slightly enlarged

Rib notching in older children

Echo: can see narrowing

Bicuspid aortic valve Doppler:disturbed

flow

COA complications

COA can cause CHF, HTN (intracranial bleeding,hypertensive encephalopathy)

Bicuspid aortic valve: stenosis or regurg LV failure infective endocarditis

Preductal COA in Newborn

80% of infants with preductal COA develop CHF by 3 months of age!

Symptoms of CHF: poor feeding, poor weight gain, dyspnea in first 2-6 weeks of life.

Case 2: Two week old with murmur Two week old infant, who you saw as a

newborn with normal exam, now is noted to have a heart murmur.

PMI LSB, not hyperdynamic pulses equal all extremities No HSM

Case 2: Cardiac exam

Grade III/VI holosystolic murmur at LLSB

Case 2: What would you like to do next? EKG: LVH,LAH Blood pressures all four extremities Xray: look for cardiomegally and

increase in pulmonary vascularity oxygenation Echo: VSD

Ventricular Septal Defect (VSD)

The most common form of congenital heart disease: 20-25%

may be located in different anatomical locations

may be associated with many other cardiac defects ( in many cases essential for survival)

may be small or large (can cause CHF)

Case 3: One day old infant

One day old infant with tachypnea and cyanosis

Gen: cyanotic RR:65, HR 140, BP 40/20 pulse ox on RA: less than 80

Case 3: Respiratory and Cardiac

Lungs clear, no retractions, RR rapid Cardiac: PMI at LSB pulses palpable all extremities S2 single and loud. No heart murmur.

What would you like to do?

ABG: before oxygenation Give 100% O2 and then repeat ABG EKG, 4 extremity BP CXR

Results of ABG

Before O2: PH: 7.15,PaCO2 30 and Pa O2 40

After 100% O2: PH: 7.12, PaCO2 25 and PaO2 50

Transposition of Great Arteries

Transposition of Great Arteries

5% of all congenital heart defects Aorta arises anteriorly from RV, PA

arises posteriorly from LV defects (VSD,ASD,PDA) that permit

mixing of the two circulations are necessary for survival

TGA:

ABG:hypoxemia is unresponsive to O2

EKG: RVH X-rays: egg-on-a-

string silhouette cardiomegally with

increased pulmonary vascularity

Echo: PA from LV associated

anomalies:VSD,ASD,PDA

Immediate Treatment

Prostaglandin E1 to reopen PDA Oxygen cardiology/surgery referral

DDX of Cyanotic Heart Dz

Transposition of the Great Arteries Tetralogy of Fallot Total Anomalous Pulmonary Venous

Return Tricuspid Atresia Pulmonary Atresia Truncus Arteriosus

Other congenital cyanotic defects

Ebstein’s anomaly single ventricle Double-outlet right ventricle (depends

on associated defects…if cyanotic or not)

Tetralogy of Fallot

Tetralogy of Fallot (TOF)

Large VSD RV outflow obstruction right ventricular hypertrophy overriding of the aorta

Tetralogy of Fallot

10% of all congenital heart defects The MOST COMMON CYANOTIC

cardiac defect beyond infancy

TOF: physical exam

Varying degrees of cyanosis and clubbing

systolic thrill LSB S2 single with ejection click loud III-V/VI SEM LSB continuous murmur of PDA

TOF

EKG: RAD, RVH Xray: “boot-shaped”

heart (hypoplastic MPA)

Echo: image of four defects associated

Tetralogy of Fallot

TOF complications

Hypoxic spells growth retardation with severe cyanosis brain abscess and CVA infective endocarditis polycythemia

Hypoxic Spell ( “TET Spell”

Paroxysm of hyperpnea (rapid and deep)

irritability and prolonged cry increasing cyanosis decreased intensity of heart murmur (may lead to limpness, convulsion,CVA

or death)

Treatment of “TET Spell”

Knee-chest (squat )position morphine sulfate treat acidosis oxygenation

Total Anomalous Pulmonary Venous Return One percent of all congenital heart

defects Defect: no direct communication

between the pulmonary veins and the left atrium (they can drain: supracardiac,cardiac,infracardiac or mixed)

Total Anomalous Pulmonary Venous Return

TAPVR findings

S2 widely split and fixed

S3 or S4 gallop SEM: III-IV/VI middiastolic rumble

at LLSB

Xrays: cardiomegally

“Snowman” Echo can define

anatomy EKG:RAD

Tricuspid Atresia

1-2% of all congenital heart disease in infancy

tricuspid valve is absent and RV is hypoplastic

associated defects of VSD,ASD or PDA are necessary for survival

Tricuspid Atresia

Tricuspid Atresia: findings

Exam: cyanosis S2 single, often syst murmur of VSD,

and occ of PDA present early CHF

Tricuspid Atresia

EKG: superior QRS between O and -90

LVH

Pulmonary vascularity is decreased

Echo: defines minimal RV, and large LV

Pulmonary Atresia

Less than 1% of congenital heart diseases

valve is atretic, RV cavity is hypoplastic need other defects: ASD,PDA for

survival

Pulmonary Atresia

Pulmonary Atresia: findings

PE: S2 is single murmur of PDA

EKG: normal axis,LVH

Xray: decreased pulmonary vascularity

Echo:atretic pulmonary valve and hypoplastic RV

Pulmonary Atresia

Prostaglandin E1 cardiac surgery

Truncus Arteriosus

Less than 1% of all congenital heart Dz Only a single arterial trunk leaves the

heart and gives rise to the pulmonary, systemic and coronary circulations

large VSD is always present

Truncus Arteriosus

PE: cyanosis wide pulse pressure

and bounding pulses

harsh VSD murmur LSB

EKG:normal axis,LAH

Xrays: cardiomegally and increased pulm vascularity

50% R aortic arch Echo:single great

artery,VSD

Hypoplastic Left Heart

Hypoplastic Left Heart

Hypoplastic left heart syndrome refers to underdevelopment of the left side of the heart.

This syndrome may include: – Small aorta: This is the major blood vessel

from the left ventricle to the body.

Hypoplastic Left Heart

May Include:

– Aortic valve atresia (absence): This valve normally opens and closes to let blood flow from the left ventricle to the aorta. When atresia is present, there is no connection between the left ventricle and aorta, and no forward blood flow.

– – Mitral valve stenosis or atresia: This valve normally

opens and closes to let blood flow between the left atrium and left ventricle. Stenosis causes little blood flow; atresia causes no blood flow. Either atresia or stenosis may be present.