Congenital Heart Disease Flashcards

1
Q

What is the most common CHD?

A

Ventricular septal defect (25-30%)

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2
Q

What are the types of CHD exhibiting a left to right shunt?

A

VSD, ASD, PDA

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3
Q

How might we classify VSD according to its locations? For each, describe the chance of spontaneous closure.

A

• Perimembranous:

  • bordered directly by fibrous continuity b/n AV + arterial valves
  • intermediate chance closure, some risk aortic valve regurgitation

• Muscular:

  • completely in muscular septum
  • high chance spontaneous closure, can be difficult for surgical closure

• Doubly committed:

  • bordered by fibrous continuity between leaflets of aortic and pulmonary valves
  • Low chance spontaneous closure, significant risk aortic valve regurgitation
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4
Q

Describe the murmur produced by VSD

A
  • LLS ejection holosystolic (louder with more restrictive i.e. smaller VSD)
  • can radiate to RLS
  • smaller VSD related to more palpable thrill
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5
Q

When do symptoms usually present for a VSD?

A

When PVR fallsnormally after birth, usually 1-3 weeks

- May have very little net intracardiac shunt right after birth -> baby no Sx, no murmur

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6
Q

What can develop if a VSD remains untreated?

A
  • Left ventricle dilatation
  • Einsenmenger syndrome (now rare): long-standing left-right shunt -> pulmonary HTN -> reversal into cyanotic right-left shunt
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7
Q

What Ix could you do to diagnose a VSD?

A
  • Echo: location and number VSDs, estimation pressure gradient-assess RVP, ?LV dilatation, ?associated defects
  • ECG: prominent left and right ventricular forces
  • CXR: pulmonary plethora and cardiac enlargement
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8
Q

What are the clinical features of a VSD?

A
  • Cyanosis
  • Failure to thrive
  • Sweaty, tachypnoiec with feeds
  • SOB
  • clubbing
  • recurrent pulmonary infections
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9
Q

What kind of conservative Mx is available for a VSD?

A

Similar to actual HF, but without the BBs:
• *Diuretics (frusemide/spironolactone)
○ to reduce pulmonary extravascular water

• Afterload reduction - ACE inhibitor
○ reducing systemic vascular resistance improves oxygen delivery
○ Trying to make the balance between SVR and PVR less - so the net shunt will be lower.

• Balance risk of endocarditis with prophylactic abx - in only some pts now!

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10
Q

When might surgery be indicated for a VSD?

A
  • Failure to respond to meds
  • VSD with pulmonic stenosis
  • Large VSD with pulmonary HTN
  • VSD with aortic regurgitation
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11
Q

What kind of procedural methods are there to repair a VSD?

A
  • Surgery–standard care if closure required
    • Closure of defect - perimembranous, doubly committed, many muscular VSDs
    • Pulmonary artery band - apical muscular
  • Catheter device closure
    • Muscular
  • Surgical device closure
    • Apical and mid muscular
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12
Q

Epidemiology of ASD:

  • % of CHD
  • When do symptoms arise?
  • M/F
A
  • 8% of CHD
  • ~3rd-4th decade, very rare early on
  • F > M
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13
Q

What is the most common type of ASD?

A

Secundum defects:

- in region of fossa ovalis while sinus venosus type defects in region SVC or IVC

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14
Q

What kind of murmur is heard in ASD?

A
  • Only heard in greater degrees of left-right shunt
  • ejection systolic at ULSB i.e. pulmonary
  • fixed splitting of S2 (pathognomonic)
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15
Q

Explain the pathophysiology of the S2 splitting that occurs in ASD, and how this differs then from normal physiological splitting of S2.

A
  • Normal: splitting in insp, none in exp.
  • Insp -> neg intra-thoracic P -> inc VR -> RV volume loading -> pul valve delays closure
  • Exp -> split no longer heard bc less neg intra-thoracic P
  • Fixed splitting in ASD i.e. no variation b/n exp and insp.
  • ASD creates left to right shunt that causes pul valve to always close later than the aortic
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16
Q

When do we repair ASDs?

A
  • 80% of small lesions close in the first 2 years of life

* Large defects closed before school age because of long-term risks of developing pulmonary vascular disease or RHF

17
Q

What is the role of the ductus arteriosus at birth? What is its natural history?

A
  • Persistence of essential in utero structure (ductus arteriosus b/w pulmonary artery and aorta bypassing lungs)
  • Normally closes within 10-15hrs of life
  • Spontaneous closure rare after 2 weeks in full term infant–closure up to 3 months in premature infant
18
Q

What are possible complications of a PDA?

A

Inc pulmonary BF ->:

  • pulmonary HTN
  • CHF
  • future -> infective endocarditis risk
19
Q

How might PDA present?

A

• Premature neonate may present with prolonged ventilation requirement

  • SOB, tachypnoea
  • failure to thrive
  • apnoea
  • irritability
  • bounding peripheral pulses, widened PP
  • low diastolic BP
  • systolic thrills
  • pulmonary hypertension
20
Q

Describe the murmur seen with PDA.

A
  • Continuous murmur–heard throughout systole
  • completely ablating S2 and continues into diastole
  • if large , may only be heard in systole
21
Q

Outline the treatment options for PDA, depending on age and symptoms.

A
  • Indomethacin if premature infant
  • Surgery for small symptomatic infant
  • Catheter device closure for older individual
22
Q

Give examples of some obstructive CHD lesions.

A
  • aortic stenosis
  • pulmonary stenosis
  • aortic coarctation
23
Q

What is the natural history of pulmonary stenosis?

A
  • Mild stenosis improves with growth

* Severe stenosis progresses with growth

24
Q

How might pulmonary stenosis present?

A
  • Mild stenosis usually asymp
  • Cyanosis
  • Chest pain, fatigue, syncope, dyspnoea, cyanosis
  • Signs of RHF
25
Q

Describe the murmur seen with pulmonary stenosis

A
  • Ejection systolic with ejection click - loudest LUSE, delay in P2
26
Q

What maternal issue is PDA most associated with?

A

Maternal rubella

27
Q

Give some examples of cyanotic CHD lesions.

A
  • tetralogy of fallot

- transposition of the great arteries (TGA)

28
Q

What is the difference between ToF and TGA in terms of blood flow? What does this mean for their presentation at birth?

A
  • Can have decreased pulmonary blood flow in Tetralogy of Fallot (can be pink at birth)
  • Increased/normal pulmonary blood flow in TGA (blue at birth, very sick)
29
Q

What are the components of tetralogy of fallot?

A
  • VSD
  • Overriding aorta towards RV- outlet deviated, moving aorta towards RV
  • RVH
  • Pulmonary stenosis
30
Q

TGA: M/F greater?

A

M 70%