Pediatric Heart Disease

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Pediatric Heart Disease
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• Infants may have one of the following
• Cyanosis (cyanotic CHD or R to L shunt)
• Pulmonary and tricuspid atresia
• CHF (L to R shunt) or shock
• Shock from outflow obstruction
• Diagnostic evaluation
• CXR
• EKG

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Cyanotic Heart Defects

• Tetralogy of Fallot
• Transposition of the great vessels
• Truncus arteriosus
• Total anomalous pulmonary venous return
• Tricuspid valve Abnormalities
• Severe pulmonic stenosis

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Tetralogy of Fallot

• Consists of VSD, obstructed right vent. outflow
  tract, dextroposed overriding aorta, RVH.
• TeT spells-worsening obstruction of flow in the
  pulmonary artery leading to greater R to L shunt.
  Causes cyanosis and dyspnea.

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Transposition of Great Vessels

• Appears in 1st week of life.
• Aorta comes from R vent. and pulmonary art. Comes
  from L vent.
• Must have a VSD or ASD for survival
• If suspected, start Prostaglandin E1

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Truncus Arteriosus

• Large arterial trunk from the ventricular portion
  of the heart.
• Supplies blood to systemic and pulmonary
  circulation.
• Present with increased pulm. blood flow, dyspnea
  and CHF

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Coarctation of the Aorta

• Narrowing of the aortic lumen.
• Present with CHF and feeding difficulty.
• Decreased pulse amplitude in the lower ext.
• Hypertension in the upper ext.
• Older kids present with exercise intolerance and
  rib notching.

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Ductal Dependent heart defects

• Depend on a patent ductus arteriosus.
• Systemic blood flow depends on a R to L shunt
  from the pulmonary artery through the DA to
  aorta.
• Pulmonic blood flow depends on a L to R shunt
  from the aorta through the DA to the pulmonary
  art.

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• Defects dependent on R to L shunt via the PDA
  include
• Critical Aortic Stenosis
• Hypoplastic left heart
• Severe coarctation
• Usual Clinical Presentation
• Circulatory collapse at the end of first week of
  life

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Treatment

• Prostaglandin infusion keeps the DA open until
  surgery.
• Start at 0.1 mcg/kg/min and titrate

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CHF

• Usually w/in the first 6 months when PVR has
  decreased allowing L to R shunt (VSD or PDA)
• Triad of CHF in infancy
• Tachypnea
• Tachycardia
• Hepatosplenomegaly

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Treatment

• Supportive
• Lasik
• Digoxin except in IHSS or TOF
• Inotropes
• Pressors
• Vasodilators
• Afterload reducing drugs
• Head up
• NPO
• Treat infections

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Atrial septal defects

• RA and RV enlargement
• Pulmonary over-circulation
• High pressure
• Low volume
• ASD low morbidity and mortality
• Repair may be surgical or trans cath

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Vent. Septal Defect

• Most common CHD
• May occur in any septal location
• Hemodynamic significance depends on the size of
  the defect.
• Spontaneous closure in the first 6 mo. In 30-40.
  

• Surgical repair required if
• Infant has failure to thrive
• Pulmonary HTN
• R to L shunt

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Aortic Stenosis

• May have a mono, bi or tricuspid valve.
• If severe, avoid prosthetic valve until they are
  old enough to receive an adult sized one.

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• In critical AS, LV unable to pump adequate flow
  past the aortic valve.
• If no PDA, not compatible with life

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Kawasakis Disease

• Acute self limited multisystem vasculitis
• Clinical presentation
• Fever 5 or more days with 4 of 5 other clinical
  features
• Bilat. conjunctival inection w/o exudates
• Mucous membranes changes of upper resp. tract
  Erythema and edema of hands/feet (early) and
  desquamation (subacute phase)
• Exantham polymorphous, truncal
• Acute cervical lymphadenopathy

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• Lab findings include high WBC, left shift,
  hemolytic anemia, high platelets, high CRP/ESR,
  pyuria, bilirubinuria
• On CXR infiltrates, cardiomegaly, long PR or QT,
  dysrhythmias
• Cardiac echocardiography show coronary aneurysms
  80-90 of time

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Treatment

• Hospitalization for IVIG
• ASA
• Cardiac eval.
• Corticosteroids may decrease coronary aneurysms

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Myocarditis

• Causes
• Idiopathic
• Inflammatory
• Acute rheumatic fever
• Collagen vascular disease
• Lyme disease
• Toxins
• HIV
• Viruses
• Adenovirus
• Coxsackie A and B

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• Often misdiagnosed
• Classic presentation
• CHF or fulminant cardiogenic shock
• Syncope from dysrhythmias
• May present with cough, wheeze/tachypnea,
  congestion, or fever.
• Consider in a child with wheezing and no history
  of asthma or in a febrile child whose wheezing is
  not responding to treatment.

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• Diagnostic eval
• CXR-cardiomegaly
• EKG changes
• Elevated troponin
• Definitive diagnosis-biopsy or MRI

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• Treatment
• Bedrest
• Oxygen
• Inotropes
• May need transplant
• 35 mortality

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SVT

• Most common dysrhythmia
• Present with tachycardia, poor feeding,
  tachypnea, pallor, lethargy, chest pain.
• HR usually gt230 BPM
• Unstable sync. Cardiovert 0.5 j/kg. Lidocaine 1
  mg/kg should be given prior d/t risk of V fib.
• Adenosine 0.1 mg/kg the 0.3 mg/kg

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A fib

• Usually d/t rheumatic heart disease or dilated
  cardiomyopathy.
• Unstable-cardiovert 0.5 J/kg
• Medical therapy (dig) is usually not effective
• Anticoagulation is not necessary

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V Tach

• Usually seen with congenital heart disease, but
  also with myocarditis, cardiomyopathy, or
  prolonged QT.
• Unstable sync. Cardiovert 1-2 J/kg
• Start Lidocaine or Procainamide if pt not stable
  for transfer

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V Fib

• Initial energy for defib is 2 J/kg. Can be
  doubled.
• A trial of amiodarone 5mg/kg infusion followed by
  defib.
• If successful, Amiodarone infusion 5-15
  micrograms/kg/min.
• After 3 defib attempts and a second 2 mg/kg bolus
  infusion usually indicates futility.