Congenital Heart Diseases

Question 1

Patent Ductus Arteriosus: Definition, Pathophys, & Risks

Answer 1

• Definition: persistent communication between the descending thoracic aorta and the main pulmonary artery after birth → L to R Shunt
• Pathophys:
  
  • continued Prostaglandin E1 production & low arterial oxygen content promotes patency. → Enlarged R atrium, R Ventricle, & L Ventricle
• Risks:
  
  • Prematurity, female, fetal hypoxia

Question 2

Patent Ductus Arteriosus: S/sxs, PE

Answer 2

• S/sxs:
  
  • Most are asymptomatic:
    
    • but some have → poor feeding, weight loss, frequent URIs
  • if they experience Eisenmenger Syndrome → left-to-right shunt switches to a right-to-left shunt → Cyanosis
• PE:
  
  • Continuous Machinery Murmur loudest at the Left upper sternal border (PDA → personal digital assistant = machine)
  • wide pulse pressure (bounding pulses)

Question 3

Patent Ductus Arteriosus: Dx & Tx

Answer 3

• Dx: ECHO = best initial test
• Tx:Indomethacin, ibuprofen (inhibits prostaglandin)
  
  • surgical: left thoracotomy
    
    • clip the premature babies
    • ligate the neonates & infants
    • divide and oversew the toddlers & infants
  • Catheter based:
    
    • coil device → for older infants & children

Question 4

Eisenmenger Syndrome

Answer 4

• Pulmonary HTN & Cyanotic heart disease occuring when a left-to-right shunt shunt switches to a right-to-left shunt → Cyanosis

Question 5

Tetralogy of Fallot: 4 components

Answer 5

Most common cyanotic congenital heart disease (right-to-left shunt)

• 4 components’
  
  1. RV outflow obstruction (sometimes call pulm stenosis)
  2. R ventricular hypertrophy
  3. Overriding Aorta (shifted to the R; sits right over the VSD)
  4. Ventricular Septal Defect

Question 6

Tetralogy of Fallot: S/sxs & PE

Answer 6

• S/sxs:
  
  • infants: Cyanosis
  • Older Children: TET spells: paroxysms of cyanosis relieved with squatting or pulling legs up
    
    • (increased peripheral vascular resistance causes the shunt to switch to left-to-right shunt )
• PE:
  
  • Harsh systolic murmur at left mid-to-upper sternal border changes similar to HOCM (louder with decrease in ventricular volume- blue, softer with increase in ventricular volume -pink) → children will squat or pull legs to chest to increase peripheral vascular resistance
  • R. ventricular heave
  • digital clubbing
  • cyanosis
  • *Often dynamic (TOF spells) & progressive

Question 7

Tetralogy of Fallot: Dx, Tx, and Mnemonic

Answer 7

• Dx: Echo = test of choice
  
  • CXR: Boot shaped heart (prominent R ventricle)
  • EKG: RVH, R. Atrial Enlargement
• Tx:
  
  • DO NOT LET THEM GET DEHYDRATED → TOF spells (need to treat gastroenteritis aggressively)
  • surgical repair by 3-6 months or sooner if blue

Question 8

Answer 8

Boot Shaped Heart

Associated with Tetralogy of Fallot

demonstrates prominent R ventricle due to RV outflow obstruction

Question 9

Ventricular Septal Defect: Definition & Types

Answer 9

• Definition:
  
  • abnormal opening in the ventricular septum, associated with a left-to-right shunt. Most common type of congenital heart defect!!
  • Most common defect in Trisomy 21 (Down Syndrome)
• Types:
  
  • Membranous = most common type, hole in the LV outflow tract near tricuspid valve (higher up on the septum)
  • Muscular: multiple holes in “swiss cheese” pattern → these may resolve on their own

Question 10

Ventricular Septal Defect: S/sxs & PE

Answer 10

• S/sxs:
  
  • Small VSDs: may be asymptomatic
  • Large VSDs: symptoms manifest within 6 months of age:
    
    • Failure to thrive (have to pump more blood, breath harder → higher caloric demand, and decreased ability to feed)
    • Poor growth
    • Tachypnea
    • GERD
      
      • (liver enlarges due to heart failure and pushes on stomach)
    • Frequent URIs
      
      • (due to wet lungs)
• PE:
  
  • high pitched holosystolic murmur best heard at the LLSB
    
    • smaller VSDs are louder with more palpable thrills
  • normal pulses

Question 11

Ventricular Septal Defect: Dx, Tx, & Mnemonic

Answer 11

• Dx:
  
  • Echo = determines size & location of VSD
  • CXR: cardiomegaly &/or congestion
• Tx:
  
  • Diuretics, ACEI +/- Digoxin
    
    • some may close or get smaller on their own
  • Indications for Surgery:
    
    • unmanageable heart failure
    • failure of medication management
    • shunt greater than 1.5-2x with or without sxs (babies hearts can accommodate 1.5-2x the volume often before symptoms)
  • **Want to try to get them to 3-6 months of age before surgery, but not necessary if FTT or other issues**

Question 12

Coarctation of the Aorta: Definition & Pathophys

Answer 12

• Definition:
  
  • congenital narrowing of the aortic lumen at the juxtaductal area (insertion of ductus arteriosus distal to left subclavian artery)
• Often associated with Turner syndrome and bicuspid aortic valve
• Pathophys:
  
  • narrowing of the aorta most commonly → HTN in the arteries proximal to the lesion with hypotension in the lower extremities
    
    • overtime, the body develops collaterals around the coarctation

Question 13

Coarctation of the Aorta: S/sxs & PE

Answer 13

• S/sxs:
  
  • Neonates: Failure to thrive
    
    • poor feeding
    • tachypnea, irritability
    • CHF
    • **Severe Coarctation: LV failure & Shock after birth when the ductus closes*
  • Older children/Teens:
    
    • headache
    • nosebleeds
    • absent or diminished femoral pulses
    • Unexplained HTN***
• PE:
  
  • upper extremity systolic HTN with lower extremity hypotension
  • Diminished or delayed lower extremity pulses (vs. VSD that has normal pulses
  • Systolic murmur at LUSB with radiation to the scapula (back)
• Dx:
  
  • CT-angiography = Gold standard
  • CXR: cardiomegaly
    
    • posterior rib notching (due to increased intercostal artery collateral flow) after several years
    • 3 sign (narrowed aorta looks like the notch of the number 3)
  • ECG: LVH
  • Echo: useful but difficult to visualize coarctation

Question 14

Coarctation of the Aorta: Dx & Tx

Answer 14

• Dx:
  
  • CXR: cardiomegaly
    
    • posterior rib notching (due to increased intercostal artery collateral flow) after several years
    • 3 sign (narrowed aorta looks like the notch of the number 3)
  • ECG: LVH
  • Echo: useful but difficult to visualize coarctation
• Tx:
  
  • surgical: left thoracotomy with extended end-to-end anastomosis; stents reserved for recurrent coarctations
  • Prostaglandin E1 (Alprostadil) can be used to open the ductus arteriosus and relax the narrowed aortic segment
  • Early referral saves lives!

Question 15

Answer 15

3 Sign

associated with Coarctation of the Aorta

narrowed aorta looks like the notch of the number 3

Question 16

Answer 16

Coarctation-Rib Notching

Associated with Coarctation of the Aorta

due to increased intercostal artery collateral flow after several years

Question 17

How do you distinguish hypoxia from pulmonary disorders from cardiac disease?

Answer 17

Hyperoxia test

Question 18

Truncus Arteriosus

Answer 18

• truncus did not separate into aorta and pulmonary artery
• Large VSD → R-L shunt
• repair: VSD closure, RV-PA conduit, usually operate in first 2 weeks of life
• associated with DiGeorge Syndrome 22q11

Question 19

Still’s Murmur

Answer 19

Benign Murmur

• SYSTOLIC, grade 1-2/6
• best heard at APEX of heart & LL sternal border
• best heard with BELL of stethoscope
• represents the normal sound of blood gushing out into the aorta during contraction
• healthy 3-7 year old children

Question 20

Murmurs that are never normal

Answer 20

• anything DIASTOLIC
• anything >II/VI
• → get an echo

Question 21

3 problems with developmental heart processes that leads to congenital heart disease

Answer 21

• Rotation:
  
  • tetralogy of fallot
  • transposition of the great arteries (TGA)
• Septation:
  
  • ventricular septal defects
  • atrial septal defects
  • truncus arteriosus
• Migration:
  
  • total anomalous pulmonary venous drainage (TAPVR)

Question 22

Total Anomalous Pulmonary Venous Drainage (TAPVR)

Answer 22