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_MS2 Cardio > 8/18- Congenital Heart Disease > Flashcards

8/18- Congenital Heart Disease Flashcards

1
Q

A child with cyanotic heart disease most likely has a heart defect that shunts?

A. Right to left

B. Left to right

A

A child with cyanotic heart disease most likely has a heart defect that shunts?

A. Right to left

B. Left to right

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2
Q

Which of the following patients is most likely to have irreversible pulmonary hypertension?

A. A 10 yo with tetrology of Fallot

B. A 1 yo with muscular VSD

C. A 10 yo with truncus arteriosus

D. A 10 yo with ASD

A

Which of the following patients is most likely to have irreversible pulmonary hypertension?

A. A 10 yo with tetrology of Fallot

B. A 1 yo with muscular VSD

C. A 10 yo with truncus arteriosus

D. A 10 yo with ASD

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3
Q

Epidemiology of Congenital Heart Disease

  • Child
  • Adult
A

Child

  • Most common heart disease
  • May become symptomatic with transitional circulation
  • Most repaired in childhood–but sequelae

Adult

  • >85% childhood CHD become adults
  • More adults with CHD than children (repaired lesions)
  • Most cardiologosits trained in CHD are pediatric…
  • Short and long term sequelae require care*
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4
Q

What is the definition of congenital heart disease?

Non-heart structures affected?

A
  • Structural malformations
  • Present at birth
  • Result from abnormal embryogenesis (3-8 wks gestation)

Associated extracardiac anomalies: 16-39%

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5
Q

What is a shunt (basic def)?

A

Abnormal communication between systemic and pulmonary circulation

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6
Q

In a shunt, the direction and amount of blood flow is determined by what factors?

A
  • Size of defect
  • Vascular resistance
  • Pressure gradient
  • Distensibility of chambers
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7
Q

Clinical consequences of a shunt?

A
  • Increased blood flow in one circulation; decreased in the other
  • Decreased oxygen in systemic circulation
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8
Q

What is cyanosis (def)?

A

Arterial O2 desaturation with > 3-5 gm/dL of unsaturated Hb

  • Blue discoloration of nailbeds and mucus membranes
  • Clubbing: convexity of nailbed +/- expansion of distal end of digit
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9
Q

Functional classifications of shunts? Examples?

A

1. Left to right shunt (systemic -> pulmonary)

  • ASD
  • VSD
  • PDA

2. Right to left shunt (pulmonary -> systemic)

  • Tetrology of Fallot
  • TV atresia
  • Truncus arteriosus
  • TAPVC
  • TGA
  • HLHS

3. Obstructions

  • Coarction of aorta
  • Valve stenosis or atresia
  • Subvalve or supravalve stenosis
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10
Q

Functional consequences due to changes in what factors?

A
  • Oxygen saturation
  • Blood volume
  • Blood pressure
  • Ultimate effects on tissue perfusion*
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11
Q

Evaluation of pts with CHD

A

Physical exam:

  • Murmurs, thrills, heart sounds, pulses/BP/ cyanosis (upper and lower extremity differences)

CXR

ECG

Labs:

  • Arterial blood gas
  • Lactate

Diagnostic

  • Echocardiogram
  • Cardiac cath
  • Cardiac MRI
  • CT angiography
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12
Q

What are the left to right shunts?

A
  • ASD
  • VSD
  • PDA
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13
Q

Characteristics of left to right shunts?

A
  • Increased pulmonary blood flow
  • Decreased systemic CO
  • No direct effect on oxygenation
  • > Pulmonary edema
  • > Congestive Heart Failure
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14
Q

What are the 3 major types of atrial septal defects? What percentage of all CHD do these comprise?

A

ASD are 10% of all congenital heart defects 3 major types:

  1. Secundum
  2. Primum
  3. Sinus venosus
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15
Q

Which ASD is the most common?

A

Secundum (90%)

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16
Q

What causes secundum ASD/where does it occur?

A

Valve of foramen ovale (septum primum) not well developed

  • usually an isolated lesion
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17
Q

What are the consequences/effects of ASD?

A
  • Increased RA and RV preload -> dilation
  • Increased pulmonary bloodflow (but not pressure)
  • High RV compliance
  • Slow increase in PA pressure

Usually asymptomatic until ~age 30

  • Pulmonary HTN
  • RV heart failure

Large ASD:

  • Early heart failure
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18
Q

Treatment of ASD?

A

Repair

  • ASD closure device or patch
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19
Q

VSDs are responsible for __% of all CHDs

A

VSDs are responsible for 40% of all CHDs

20
Q

What are the 3 major types of VSD? Which is the most common?

A
  1. Membranous (perimembranous)
  2. Infundibular (conal septal)
  3. Muscular Peri/Membranous is the most common (90%)
21
Q

What is this?

A

Membranous ventricular septum (light shining through)

22
Q

What is this?

A

Perimembranous VSD

23
Q

What are the consequences/effects of VSD?

A
  • Increased pulmonary blood flow AND pressure
  • Increased LV preload/volume
  • Decreased CO
  • > Pulmonary edema
  • > Congestive Heart Failure (left sided! volume load on left side)

Size affects symptoms!

Symptom onset often with drop in neonatal pulmonary vascular resistance

24
Q

Spontaneous closure of perimembranous VSD may be due to what? Muscular VSD?

A

Perimembranous VSD: adherence of TV

Muscular VSD: growth of heart muscle

25
Q

What is shown here on the left? right?

A

Left: perimembranous VSD (clsoed by TV adherence)

Right: muscular VSD (closed by heart muscle growth)

26
Q

How is VSD treated? When to treat?

A
  • May close spontaneously
  • Usually patch closure if no spontaneous closure

When considering repair, think of:

  • Likelihood of spontaneous closure (usually before 5 yo)
  • Severity of clinical Sx (may correlate with size of defect)
  • Risk of irreversible pulmonary HTN (unusual before 2 yo)
27
Q

What are the long term sequelae for patching a VSD?

A
  • Infective endocarditis (prosthetic patch)
  • Patch dehiscence
  • Arrhythmias
28
Q

What is the “dreaded” complication for a left to right shunting lesion?

A

Pulmonary HTN and shunt reversal

  • Uncontrolled, persistent increased pulmonary pressure/resistance will reverse the direction of a left to right shunt
  • > Late onset cyanosis

Irreversible pulmonary HTN is the dreaded complication of a L to R shunt

  • Once it develops, repair of defect is not indicated
29
Q

What are CHDs of Right to Left shunt?

How may they be divided?

A

Decreased pulmonary bloodflow (more common):

  • Tetralogy of Fallot (TOF)
  • TV atresia

Increased pulmonary bloodflow:

  • Truncus arteriosus
  • TAPVC
  • TGA
  • HLHS
30
Q

Characteristics of right to left shunts with decreased pulmonary blood flow?

A

(Decreased pulmonary bloodflow)

Associated with:

  • R sided obstruction distal to shunt (e.g decreased pulmonary outflow tract), OR
  • Elevated pulmonary pressure

Desaturated venous blood enters systemic circulation

  • > cyanotic congenital heart disease
  • > poor tissue oxygenation
31
Q

What is the Tetaralogy of Fallot (TOF)?

A

Malaligned outflow tract septum resulting in:

  • SubPA (RVOT) stenosis
  • VSD
  • Aortic over-ride of VSD
  • RV hypertrophy

Stenosis severity determines direction of magnitude and shunt

32
Q

What is this?

A

Tetralogy of Fallot

33
Q

Physiology (consequences) of TOF?

A
  • Increased RV preload/volume
  • Increased afterload/pressure -> RV dilated and hypertrophied (more blood going into aorta; more returning to right A/V -> R sided failure)
  • PA is small (hypoblastic)
  • Aorta s dilated
34
Q

What are the systemic consequences of TOF?

A

Poor tissue oxygenation

  • Hypoxemia and polycythemia
  • Cyanosis that worsens with exercise
  • Clubbing after 3 mo of age

Paroxysmal hypoxia

  • RVOT spasms
  • (TET spells)
35
Q

How is TOF treated?

A

Surgical repair: as early as feasible

  • Improve tissue perfusion
  • Decrease risk of post-operative arrhythmias

Common follow-up = valve replacement before pulmonary regurg causes permanent problems with volume load on _ventricle

36
Q

What is the most common form of cyanotic congenital heard disease?

A

Tetralogy of Fallot (TOF)

37
Q

TOF is responsible for __% of congenital heart disease?

A

TOF is responsible for 5% of congenital heart disease?

38
Q

What is the current survival rate of TOF?

A

> 75-80% 30 yrs

TOF is one of the most common anomalies seen in adult with congenital heart disease

39
Q

What is TV atresia? Characteristics? Consequences?

A

CHD that involves a right to left shunt and decreased pulmonary blood flow

  • Obligate RA -> LA shunt at atrial septum (no communication between R heart and pulmonary artery?)
  • Increased LA and LV volume
  • Pulmonary blood flow is dependent on VSD usually decreased
  • > cyanotic CHD
40
Q

What is truncus arteriosus? Characteristics? Consequences?

A

CHD that involves a right to left shunt and increased pulmonary blood flow

  • Due to failure of embryonic truncus and usually outflow tract of heart to develop a septum
  • Single great artery gives rise to aorta, pulmonary, and coronary arteries
  • Single semilunar (truncal) valve that may be stenotic or regurgitant

Consequences:

  • Variably increased pulmonary bloodflow
  • High risk for pulmonary HTN
  • > cyanotic CHD)
41
Q

What is TAPVC?

A

Total anomalous pulmonary venous connection

  • Obligate right -> left shunt at atrial septum
  • Pulmonary veins don’t connect to LA

May connect to various sites:

  • Most common = innominate vein

Consequences:

  • Increased RA and RV volume
  • Increased pulmonary bloodflow -> cyanotic CHD
42
Q

Most obstructive lesions are ___

A

Most obstructive lesions are valvular

43
Q

Physiology (characteristics) of obstructive lesions?

A
  • High pressure proximal to obstruction
  • Decreased bloodflow distal to obstruction
44
Q

What is shown here?

A

Obstructive lesion: aortic valve stenosis

45
Q

Treatment for obstructive lesions?

A
  • Balloon repair
  • Valve replacement

_MS2 Cardio (35 decks)

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