• Loud, pansystolic murmur, LLSB
• NOT from flow through the defect; more from turbulent flow in the R ventricle
• Loud S2 if pulmonary HTN
• Potential for Eisenmenger Syndrome
• Also caused by ASD, PDA
• Left-to-right shunt => increased pulmonary flow => damage => pulmonary HTN => Right-to-left shunt => Cyanosis
• Presents with cyanotic episodes in teenage years

Innocent murmur

• Systolic
• Except venous hum, which is innocent but systolic + diastolic
• Soft
• Normal heart sounds
• Normal pulses
• No thrill
• No radiation
• Position dependent
• Asymptomatic
• Normal CXR and ECG
  

Kawasaki syndrome

• Autoimmune medium-vessel vasculitis
  
• Diagnosis:
• Fever of ≥5 days' duration plus 4 of:
  
• Bilateral nonsuppurative conjunctivitis
• One or more changes of the mucous membranes of the upper respiratory tract
• Pharyngeal injection, dry fissured lips, injected lips, "strawberry" tongue
• One or more changes of the extremities
• Peripheral erythema, peripheral edema, periungual desquamation, generalized desquamation
• Polymorphous rash
• Pimarily truncal
• Cervical lymphadenopathy
  
• >1.5 cm in diameter
• Can cause fatal coronary artery aneurysms
• Treatment:
• IVIG
• Aspirin
• One of the few conditions for which it's indicated in kids
• cf Rey's syndrome
• Plasma exchange
• Corticosteroids?
• Poor evidence and may increase risk of coronary artery aneurysms

IVIG therapy

• Contains the pooled, polyvalent, IgG from the plasma of over one thousand blood donors
• Effects last between 2 weeks and 3 months
• Mechanism unclear
• Activates inhibitory Fc receptors on dendritic cells
• => anti-inflammatory effects
• Indications
• Immune deficiencies
  
• X-linked agammaglobulinemia
• Hypogammaglobulinemia
• Acquired compromised immunity conditions featuring low antibody levels
• Autoimmune disease
• e.g. ITP
• Inflammatory diseases
• e.g. Kawasaki disease.
• Acute infections

Tet spells

• Problem
• Not entirely clear
  
• Sudden decrease in pulmonary blood flow and therefore oxygenation?
• Increased pulmonary vascular resistance? (Crying)
  
• Fall in systemic vascular resistance
• Begins a cycle:
• Acidosis => Hyperventilation
• => More systemic venous return
• => More R=>L shunt
• => Even less pulmonary blood flow
• Also spasm of pulmonary artery?
• Place in a knee-chest position 
• Increase systemic vascular resistance
• Oxygen
  
• IV morphine
• Venoconstriction => More peripheral venous blood pooling => Less venous return => Less R=>L shunt
• Sedative effect
  
• IV propranolol
  
• Relaxes the infundibular muscle spasm causing right ventricular (RV) outflow tract obstruction (RVOTO)

• HARD TO FIND PULSES
• Ejection systolic murmur radiating to between the shoulder blades
  
• Symptoms depend on where to coarctation is
• Pre-dutal
• Ductal
• Post-ductal

• See rib notching on CXR, due to collateral vessels
  

    

Transverse myelitis

• Pathogenesis
• Inflammatory process of the spinal cord
• => axonal demyelination
• Transverse implies that the inflammation is across the thickness of the spinal cord
• Causes
• Post infection
• CMV?
• MS
• Progresses vary rapidly
• Hours-days
• Presentation
• Depends on level
• Upper cervical cord => Quadriplegia, Resp. paralysis
• C5–T1 => Combination of upper and lower motor neuron signs in the upper limbs; exclusively upper motor neuron signs in the lower limbs
• T1–12 => Upper motor neuron signs in the lower limbs, presenting as a spastic diplegia
• L1–S5 => Combination of upper and lower motor neuron signs in the lower limbs
• Motor impairment
• Sometimes have some sensory impairment
  

Notes

• AMPLE
• Allergies
• Medications
• Past Medical History
• Last Eaten
• Events Leading
• Breathlessness in a baby makes it hard for them to feed => FTT