Congenital Heart Defects

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Congenital Heart Defects

• Margaret Strieper DO
• Director, Pacing and Electrophysiology
• Sibley Heart Center Cardiology

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Objectives

• Identify congenital heart defects, diagnosis,
  treatment, outcome and management
• Describe the relationship of pulmonary and
  systemic pressures in CHD
• Describe cyanotic, acyanotic and obstructed
  anatomy and current treatment options

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Congenital Heart Disease

• Structural or functional defects of the heart
  present from birth.
• CHD has the greatest effect on infant mortality
  1 cause of death in infancy from birth
  defects
• Incidence
• 8 - 10 per 1000 live births
• Similar from country to country Recurrence in
  families is 2 - 6

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• Acyanotic defects with increased pulmonary blood
  flow (PBF)
• Cyanotic defects with decreased/ increased/
  variable PBF
• Defects that obstruct blood flow
• Mixed lesions

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• Multifactorial Inheritance
• Multifactorial Interaction of genetic,
  environment and
  random effects (90).
• Genetic Chromosomal defects (5 -10)
• Mendelian Single Gene Defect (3).
• Environment Teratogens, virus, or other exposure
  during the vulnerable period of
  heart development.

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Shunting

• Left to Right
• Acyanotic
• Congestive Heart Failure

• Right to Left
• Cyanotic
• CNS complications

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Magnitude of the Shunt

• Dependent upon
• Pulmonary vascular resistance
• PVR systemic at birth decreases to adult levels
  by 6-8 weeks
• Size of the defect
• Location of defect

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Left to Right Shunting Pathophysiology

• Acyanotic defects
• Oxygenated blood from the left side to
  unoxygenated blood on right side
• RV volume overload
• Pulmonary overcirculation
• Recirculation of pulmonary venous blood through
  the pulmonary capillary bed
• Increased pulmonary blood flow
• High pressure to lower pressure

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Signs / Symptoms

• Congestive Heart Failure (CHF)
• Tachycardia, Tachypnea, Diaphoretic
• Pulmonary congestion
• Cardiomegaly, hepatomegaly, ascites
• Failure to Thrive (FTT)
• Poor feeding
• Low cardiac output syndrome

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Treatment

• Limit O2
• Diuretics
• Digoxin

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Acyanotic Defects

• Include
• PDA, ASD, VSD, AVC
• TAPVC, DORV, dTGA
• AP Window, Truncus Arteriosus

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Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Atrial Septal Defect
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Atrial Septal Defect Secundum Defect ASD II
LA
RA
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Ventricular Septal Defect
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Atrioventricular Canal Defect
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Right to Left ShuntingPathophysiology

• Cyanotic Defects
• Obstruction of pulmonary blood flow
• Decreased pulmonary blood flow
• High pressure to lower pressure
• Right to Left shunting of deoxygenated blood
• Deoxygenated blood from the right side to
  oxygenated blood on the left side
• Bypassing the lungs
• Oxygen depleted blood enters the systemic
  circulation causing cyanosis

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Signs / Symptoms of R to L shunting

• Decreased pulmonary blood flow
• Systemic desaturation
• Hypoxemia
• Cyanosis
• Acidosis
• /- Grow well

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Cyanotic defects with decrease PBF

• Tricuspid Atresia / TA
• Pulmonary Atresia / PA
• Pulmonary Stenosis / PS
• Pulmonary Stenosis with IVS
• Tetralogy of Fallot / TOF
• Ebsteins Anomaly (of Tricuspid Valve)

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Treatment of right to left shunts

• PGE1
• Oxygen
• Fluids
• Correct acidosis
• Accept saturations of 75

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Tricuspid Atresia
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Pulmonary Atresia with a VSD
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Pulmonary Atresia with IVS and Sinusoids
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Pulmonary Stenosis
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Tetrology Of Fallot
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TOF
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Tet Spell
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Tet Spell

• Treatment of Spells
• Oxygen
• Sedation
• Knee Chest
• Drugs
• Surgery

• Increased infindibular spasm
• Decreased PBF
• Decreasing amount of blood to be oxygenated
• Potentiation of cyanosis / acidosis can lead
  to death

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TOF Repair
Surgical Outcomes Follow Up
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Ebsteins Anomaly
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Cyanotic Defects with increased pulmonary blood
flow

• Cyanosis caused by mixing of pulmonary and
  systemic blood / circuits
• Mixing occurs at the atrial or ventricular level
• With no obstruction to PBF there is increased
  flow to the lungs as pulmonary vascular
  resistance falls

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Cyanotic Defects with increased pulmonary blood
flow

• TAPVC
• Truncus Arteriosus
• HLHS

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Treatment

• PGE1
• Fluids
• Balance pulmonary and systemic circulation

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TAPVR
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Truncus Arteriosus
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Hypoplastic Left Heart Syndrome
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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HLHS
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HLHS
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Hypoplastic Left Heart Syndrome
RV
1) Mitral valve atresia 2) Severe aortic valve
stenosis 3) Hypoplastic left ventricle 4)
Hypoplastic ascending aorta 5) Patent ductus
arteriosus 6) Patent foramen ovale
LV
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Hypoplastic Left Heart Syndrome Treatment with
Norwood Procedure
External view of Stage I Norwood
Procedure
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Stage I Sano Procedure

• Stage I Sano
• Arch reconstruction
• Atrial Septectomy
• RV to PA conduit

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Stage I Sano - Angiogram
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(Stg II) Bi-directional Glenn Shunt
BDG
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(Stg III) Fontan ProcedureLateral Tunnel
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(Stg III) Fontan ProcedureExtracardiac
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Defects that obstruct blood flowPathophysiology

• Pressure before the obstruction is increased the
  pressure after the obstruction is decreased
• Location of obstruction is usually near a valve
  Valvar narrowing at the valve
• Subvalvar narrowing below valve
• Supravalvar narrowing above valve
• Blood meets an area of anatomic narrowing
• Sx usually do not appear until the PDA closes
• Increased pressure and workload on the ventricle
  and decreased cardiac output

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Signs / Symptoms of obstructive defects

• Significant ventricular outflow obstruction will
  result in congestive heart failure within the
  first few weeks of life
• Ventricular dysfunction may result
• Difficult decision on when to do surgery. Mild
  obstructions may be asymptomatic.
• If postponed until symptomatic irreversible
  damage may have taken place.

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Obstructive Lesions

• Coarctation of the Aorta
• Interrupted Aortic Arch
• Aortic Stenosis
• Pulmonary Stenosis

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Coarctation of the Aorta
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Coarctation of Aorta -angiogram
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Aortic Stenosis
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Aortic Stenosis- Subvalvular
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Aortic Stenosis - Supravalvular
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Interrupted Aortic Arch / IAA
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Pulmonary Stenosis
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Pulmonary Valve Stenosis
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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Pulmonary Stenosis - PS

• Chest Xray Cath Lab
• Dilation

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Cyanotic Defects with variable pulmonary blood
flow

• Cyanosis caused by mixing of pulmonary and
  systemic blood
• Mixing occurs at the atrial / ventricular level
• Streaming of blood flow can occur
• Blood will follow the path of least resistance
  depending on the defect this will result in
  increased or decreased pulmonary blood flow

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Transposition of the Great Vessels
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TGA
Incidence Diagnosis Management Medical
Surgical Outcomes Follow Up
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TGA - Echo
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Complex CHD

• Combinations of defects
• One ventricular pumping chamber (R or L)
• Too little or too much PBF
• Resuscitate neonates alleviate acidosis
• Inotropic support

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Surgery

• Reparative / Primary vs. Palliative
• Earlier vs. Later
• Staged Repairs
• Fix and follow over the years

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