Cyanotic Heart Disease

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Cyanotic Heart Disease
Casey Wong MS III
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Overview

• Specific Cyanotic Congenital Heart Diseases
• Evaluation of Cyanosis
• Case Presentation

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Epidemiology of Congenital Heart Disease

• Incidence of congenital heart disease is 1 in 100
  live births
• Critical Congenital Heart Disease 1 in 400 live
  births
• Of these, 1/3 have cyanotic heart disease

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Etiology of Congenital Heart Disease
of All Lesion OBSTRUCTIVE LESIONS
(Coarctation, aortic/pulmonic stenosis)?
ACYANOTIC LESIONS (VSD 30-35, ASD, PDA)?
CYANOTIC LESIONS 5 Ts Tetralogy of Fallot
5-7 Transposition of great
arteries 3-5Truncus arteriosus 1-2Total
Anomalous Pulmonary Venous Return 1-2Tricuspid
atresia 1-2 (pulmonary atresia) 1-2 (hy
poplastic left heart syndrome) 1-2
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Transposition of the Great ArteriesMixing
lesion/ductal independentneed PGE1 to
increase mixing
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Transposition of Great Arteries
Second most common cause of cyanosis in
infancy Pulmonary and systemic circulations form
two separate circuits Must be mixing between two
circuits for life
egg-shaped silhouette
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Clinical Findings

• Severe cyanosis present at birth with
  vasculature
• 1/3 have VSD, some have ASD
• Some have subpulmonic stenosis
• Loud, single S2
• Systolic murmur indicates VSD or pulmonic
  stenosis
• ECG reveals right ventricular hypertrophy

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Transposition of Great Arteries Tx

• PGE1 administration necessary
• Balloon atrial septostomy necessary (Rashkind
  procedure)?
• Arterial Switch procedure performed first week of
  life

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Hypoplastic left heartDuctal Dependent for
systemic flow
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Hypoplastic Left Heart
Presents first week of life, as PDA closes
symptoms develop PGE administration Ductal
dependant systemic blood flow
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Tricuspid AtresiaDuctal Dependent Pulmonary
Blood flow
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Tricuspid Atresia
Tricuspid valve fails to develop Hypoplasia of
right heart Venous blood from right atrium
depends on open ASD or PFO, VSD, PDA
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Tricuspid Atresia-Clinical Findings

• Progressive cyanosis as PDA closes
• 30 transposition of great arteries
• 70 some degree of Pulmonic stenosis
• Tacypneic, single S2
• Systolic murmur along left lower sternal border
  (VSD)?
• ECG reveals left ventricular hypertrophy

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Tricuspid Atresia Tx

• PGE1 administration necessary
• Balloon atrial septostomy
• shunt placed between subclavian artery and
  pulmonary artery in neonates when pulmonary
  resistance still high
• Eventually superior and inferior vena cava are
  connected directly to the pulmonary arteries

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Truncus Arteriosus Mixing lesion/ductal
independent
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Truncus Arteriosus
Failure of primitive truncus arteriosus to divide
into aorta and pulm A. VSD almost always
present Right Sided-arch in about 33
Cardiomegaly, increased pulmonary vascularity,
right aortic arch
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Truncus Arteriosus-Clinical Findings

• Minimal cyanosis at birth Death at 6 months
• Congestive Heart failure develops in weeks
• Pulmonary vascular resistance falls and pulmonary
  blood flow increases at the expense of systemic
  flow
• Bounding pulses, pulse pressure widened
• Loud, single S2
• Systolic murmur heard at left sternal border
• ECG reveals biventricular hypertrophy

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Truncus Arteriosus Tx

• Surgical repair at 2 to 3 months of age
• Closing VSD
• Separation of pulmonary arteries from truncal
  vessels
• Placing conduit between right ventricle and
  pulmonary arteries

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Tetralogy of FallotDuctual-dependent pulmonary
blood flow
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Tetralogy of Fallot
Most Common cause of cyanotic heart disease
beyond neonatal period Degree of Pulmonary
stenosis and size of VSD determine
presentation Variable degree of Cyanosis
Boot Shaped Heart
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Tetralogy of Fallot- Clinical Findings

• squatting
• Tet spells due to pulmonary outflow tract
  spasm
• Severe cases ---at birth---severe PS
• Mild cases ---- much later---mild PS
• Cyanosis usually
• ECG reveals right ventricular hypertrophy

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Tetralogy of Fallot Tx

• Squatting relieves tet spells venous return,
  systemic resistance
• Surgical repair performed during first 3 to 5
  years old
• VSD closed with a patch, pulmonary stenosis
  opened up with balloon

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Total Anomalous Pulmonary Venous
ConnectionDuctal-independent mixing lesion
(increased PBF)?
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Total Anomalous Pulmonary Venous Connection
Pulmonary veins are not connected to the left
atrium Systemic circulation dependant on shunting
through ASD or PFO Variable degree of
Cyanosis-dependant on presence of obstruction
snowman
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Schematic Drawing of Cardiac Defects
A Normal Circulation B Tetralogy of Fallot C
Pulmonary Atresia D Tricuspid Atresia E
Transposition of Great Arteries F Truncus
Arteriosus
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Evaluation of Cyanotic Heart Disease
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Physical Examination

• Central Cyanosis vs. Peripheral cyanosis
• Vital signs
• Lung and CNS examination to rule these out
• Cardiac Examination
• Heaves, thrills, abnormal or increased precordial
  activity
• Absent or diminished femoral pulses
• Abnormal first or second heart sound (abnormal
  splitting)?
• Extra heart sounds (gallop, ejection click,
  opening snap)?
• Murmurs that are loud, harsh, blowing

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History

• Difficulty feeding, irritablility, diaphoresis,
  failure to thrive
• Prenatal history maternal diabetes, SLE
• Congenital Infections (TORCH)?
• Drugs taken in pregnancy
• Family history heart problem before 50 y.o.
• Chromosomal Abnormalities

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Hypoxemia Differential

• Right-to-Left Shunt
• INTRACARDIAC, Great Vessels, pulmonary AV
  malformation
• V/Q Mismatch
• Pneumonia, atelectasis, aspiration, pulmonary
  hypoplasia
• Hypoventilation
• CNS depression, Neuromuscular disease, Airway
  obstruction
• Diffusion Impairment
• Pulmonary edema, pulmonary fibrosis
• Hemoglobinopathy

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Lab/Imaging Studies

• CBC/Sepsis evaluation
• Chest x-ray
• Oxygen Saturation (Arterial blood gas, pulse
  oximetry)?
• Hyperoxia test
• Electrocardiogram
• Echocardiography

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Case Presentation
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Case Presentation

• History
• 6 week old male with 2 days of clear, nasal
  congestion, no fever
• Gets bluish after feeding or crying
• Previously well, full-term baby
• The family history was negative

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Case Presentation contd

• Physical
• Vigorous male, growing appropriately
• HR 135, RR 30, normal BP, no fever
• Clear nasal discharge
• Lungs clear to auscultation b/l, no wheezes,
  ronchi, rales

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Case Presentation contd

• Purplish lips, hands and feet
• Grade III/VI systolic murmur loudest at
  lower left sternal border
• Liver was 1.5 cm below right costal margin
  and a normal spleen
• Peripheral pulses equal in upper/lower
  extremities, 1.5 sec cap refill

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Case Presentation contd
Work Up

• PaO2 of 38mm Hg and a hyperoxia test showed
  increase to 48mm Hg
• Electrocardiogram showed RVH
• Chest X-ray

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WHATS THE DIAGNOSIS?
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Case Presentation contd

• Tetralogy of Fallot
• IV antibiotics b/c of age and possible sepsis
• Echocardiogram
• Cardiac Catheterization and plan surgery