Congenital Heart Defects

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Congenital Heart Defects

• Craig T. Carter, D.O.
• Assistant Professor EM/Peds
• University of Kentucky

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• 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 ..

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

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So you sit and think..
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And think some more.
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Back to Basics-Fetal Circulation

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

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Back to Basics-Fetal Circulation
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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)

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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

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Case 1 2 day old infant

• 2 day old baby girl is being examined for
  discharge physical.
• Vitals HR120 RR40 BP r arm 75/40
• Gen low hairline, webbed neck, edemetous dorsum
  of feet

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Case 1 cardiac exam

• Normal PMI, no thrill
• No hepatosplenomegally
• unable to palpate pulses in lower extremities
• BPs all four extremities both legs systolic BP
  lower 40/30

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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

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Case 1

• What do you think is the congenital defect?

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Case 1

• What further evaluation do you need?

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Coarctation of Aorta (COA)
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Coarctation of Aorta (COA)

• Incidence 8 of all congenital heart defects
• Turners syndrome 30 have COA

• Preductal 40 associated with other cardiac
  defects,symptomatic early in life
• Postductal less likely to have symptoms early

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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
• Dopplerdisturbed flow

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COA complications

• COA can cause CHF, HTN (intracranial
  bleeding,hypertensive encephalopathy)
• Bicuspid aortic valve stenosis or regurg
• LV failure
• infective endocarditis

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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.

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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

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Case 2 Cardiac exam

• Grade III/VI holosystolic murmur at LLSB

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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

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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)

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Case 3 One day old infant

• One day old infant with tachypnea and cyanosis
• Gen cyanotic
• RR65, HR 140, BP 40/20
• pulse ox on RA less than 80

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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.

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What would you like to do?

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

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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

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Transposition of Great Arteries
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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

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TGA

• ABGhypoxemia is unresponsive to O2
• EKG RVH
• X-rays egg-on-a-string silhouette
• cardiomegally with increased pulmonary
  vascularity

• Echo PA from LV
• associated anomaliesVSD,ASD,PDA

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Immediate Treatment

• Prostaglandin E1 to reopen PDA
• Oxygen
• cardiology/surgery referral

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DDX of Cyanotic Heart Dz

• Transposition of the Great Arteries
• Tetralogy of Fallot
• Total Anomalous Pulmonary Venous Return
• Tricuspid Atresia
• Pulmonary Atresia
• Truncus Arteriosus

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Other congenital cyanotic defects

• Ebsteins anomaly
• single ventricle
• Double-outlet right ventricle (depends on
  associated defectsif cyanotic or not)

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Tetralogy of Fallot
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Tetralogy of Fallot (TOF)

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

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Tetralogy of Fallot

• 10 of all congenital heart defects
• The MOST COMMON CYANOTIC cardiac defect beyond
  infancy

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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

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TOF

• EKG RAD, RVH
• Xray boot-shaped heart (hypoplastic MPA)

• Echo image of four defects associated

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Tetralogy of Fallot
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TOF complications

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

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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)

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Treatment of TET Spell

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

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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)

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Total Anomalous Pulmonary Venous Return
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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
• EKGRAD

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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

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Tricuspid Atresia
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Tricuspid Atresia findings

• Exam cyanosis
• S2 single, often syst murmur of VSD, and occ of
  PDA present
• early CHF

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Tricuspid Atresia

• EKG superior QRS between O and -90
• LVH

• Pulmonary vascularity is decreased
• Echo defines minimal RV, and large LV

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Pulmonary Atresia

• Less than 1 of congenital heart diseases
• valve is atretic, RV cavity is hypoplastic
• need other defects ASD,PDA for survival

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Pulmonary Atresia
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Pulmonary Atresia findings

• PE S2 is single
• murmur of PDA

• EKG normal axis,LVH
• Xray decreased pulmonary vascularity
• Echoatretic pulmonary valve and hypoplastic RV

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Pulmonary Atresia

• Prostaglandin E1
• cardiac surgery

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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

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Truncus Arteriosus

• PE cyanosis
• wide pulse pressure and bounding pulses
• harsh VSD murmur LSB

• EKGnormal axis,LAH
• Xrays cardiomegally and increased pulm
  vascularity
• 50 R aortic arch
• Echosingle great artery,VSD

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Hypoplastic Left Heart
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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.

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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.