Lecture 25 - 27: Congenital Heart Defects Flashcards

1
Q

Describe the right atrium

A

Broad triangular appendage, trabeculated

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2
Q

Describe the left atrium

A

Finger-like appendage, smooth surface

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3
Q

T/F: The left atrium receives the pulmonary veins

A

FALSE! This is an independent variable

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4
Q

Where do we find the septum primum connections?

A

L atrial septal surface

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5
Q

How do we define the tricuspid and mitral valves?

A

By which ventricle they serve (mitral = LV, tricuspid = RV) NOT by which atrium they guard

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6
Q

Describe the right ventricle

A

Irregular shape, heavy trabeculations, tricuspid attached to septum

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7
Q

Describe the left ventrile

A

Bullet-shaped, smooth septal surface, absent of mitral attachments to septum

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8
Q

What is the pulmonary artery? What is the aorta?

A

Gives rise to pulmonary artery branches; gives rise to at least one of the coronary arteries

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9
Q

What is the normal atrial situs?

A

Situs solitus

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10
Q

What happens in sinus inversus?

A

RA is left-sided and the LA is right-sided

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11
Q

In sinus inversus, where are the septum primum attachments?

A

Right-sided (w/ the LA)

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12
Q

Describe situs ambiguous

A

Chaotic lateralization in which some portions are organized in solitus and others in inversus

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13
Q

What is the normal ventricular situs?

A

D-looped

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14
Q

What is the abnormal ventricular situs? What is an important note?

A

L-looped; normal only for situs inversus

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15
Q

In situs solitus, what is the orientation of the valves of the great arteries?

A

Aortic valve is right and posterior to the pulmonic valve

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16
Q

What is the most common cause of neonatal cyanosis?

A

Transposition of the great arteries

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17
Q

In transposition of the great arteries, circulation is in…

A

Parallel

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18
Q

What two communications allow a baby to survive if born with transposition of the great arteries?

A

Ductus arteriosus (give PGE) and foramen ovale

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19
Q

What is the corrective procedure of choice for transposition of the great arteries?

A

Arterial switch

20
Q

What is transposition of the great arteries associated with?

A

Maternal diabetes

21
Q

What are the four characteristics of the great arterials in situs solitus?

A
  1. Aorta w/ fibrous continuity to mitral valve; 2. Pulmonary valve seperated from tricuspid via subpulmonary muscle; 3. Aortic valve is to the right and posterior of the pulmonic valve; 4. LV aligned with aorta and RV aligned with PA
22
Q

What is another example of malpositions of the great arteries (besides transposition)

A

Double outlet from L or R ventricle

23
Q

What happens to the foramen ovale when a baby is born?

A

High left ventricular pressure forces it shut, remains patent in 1/5 of individuals

24
Q

When is the pulmonary resistance equivalent to adult levels?

A

At 8 weeks

25
What is the pathophysiology of large ventricular septal defects?
Because pulmonary resistance is lower than systemic resistance, and the pressures inside the single ventricle is the same, the pulmonary track will get larger flow --> inefficient pulmonic circulation
26
How/when will a baby present with a large VSD? (5)
Several weeks after birth with dyspnea, tachypnea, failure to thrive, poor feeding, early HF symptoms
27
What is the most common congenital heart abnormality?
VSD
28
What is VSD associated with?
Fetal alcohol syndrome
29
What is a possible course of a small VSD (%)? What is another name of this condition? Describe.
Closure on its own (50%); restrictive VSD in which the resistance of the hole itself impedes flow through it (resistance in series)
30
What is a possible course of a large VSD? What is this called?
Pulmonary hypertension --> R to L shunt; Eisenmenger syndrome
31
What are three symptoms of Eisenmenger syndrome?
RV hypertrophy, polycythemia (due to hypoxia), clubbing
32
What is a nonrestrictive VSD?
A large VSD that does not restrict transmission of pressure (aka, pressure in ventricles is equal)
33
What % of atrial septum defects are not diagnosed until adulthood?
25%
34
Ostium primum defect is associated with what?
Down's syndrome
35
Physical sign of an ASD? Why?
Split S2: delayed closure of pulmonic valve due to increased volume
36
One frightening complication of an ASD
Paradoxical embolism
37
Why is there L --> R shunting in an ASD?
Because the L ventricle is less compliant (NOT because of systemic vs pulmonic circulation, from which the atria are guarded)
38
What can ASD lead to in adulthood? (3)
Hypertension, ischemic heart disease, and a less compliant left heart
39
Describe Eisenmenger syndrome
Elevated pulmonary vascular resistance causes reversal of the original shunt (to the right-to-left direction) and systemic cyanosis
40
What is the most common cause of cyanosis after infancy?
Tetralogy of Fallot
41
What are the 4 features of Tetralogy of Fallot
VAD, subvalvular pulmonic stenosis, overriding aorta that receives blood from both ventricles, RV hypertrophy
42
What is the primary cause of the shunt flow across the VSD in Tetralogy of Fallot?
Severity of the pulmonic stenosis
43
When does Tetralogy of Fallot manifest most strongly? Why? What do children do to compensate?
During exercise or anything that causes systemic vasodilation (feeding, crying); children will to squat to increase arterial resistance, which decreases the R --> L shunt
44
What is tx for Tetralogy of Fallot?
Surgical correction to close VAD and enlarge subpulmonary infundibulum
45
What are four other (pathoma) congenital heart abnormalities that cause cyanosis?
1. Truncus arteriosis, due to mixing; 2. Tricuspid atresia, due to hypoplastic RV; 3. Coarctation of the aorta, if infantile it's associated with PDA and Turner Syndrome; 4. PDA, associated with congential rubella
46
PDA and infantile coarctation of the aorta are associated with what physical finding? Why?
Cyanosis of the lower extremities because the PDA is after the branches of the aortic arch