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
Q

What is the pathophysiology of large ventricular septal defects?

A

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
Q

How/when will a baby present with a large VSD? (5)

A

Several weeks after birth with dyspnea, tachypnea, failure to thrive, poor feeding, early HF symptoms

27
Q

What is the most common congenital heart abnormality?

A

VSD

28
Q

What is VSD associated with?

A

Fetal alcohol syndrome

29
Q

What is a possible course of a small VSD (%)? What is another name of this condition? Describe.

A

Closure on its own (50%); restrictive VSD in which the resistance of the hole itself impedes flow through it (resistance in series)

30
Q

What is a possible course of a large VSD? What is this called?

A

Pulmonary hypertension –> R to L shunt; Eisenmenger syndrome

31
Q

What are three symptoms of Eisenmenger syndrome?

A

RV hypertrophy, polycythemia (due to hypoxia), clubbing

32
Q

What is a nonrestrictive VSD?

A

A large VSD that does not restrict transmission of pressure (aka, pressure in ventricles is equal)

33
Q

What % of atrial septum defects are not diagnosed until adulthood?

A

25%

34
Q

Ostium primum defect is associated with what?

A

Down’s syndrome

35
Q

Physical sign of an ASD? Why?

A

Split S2: delayed closure of pulmonic valve due to increased volume

36
Q

One frightening complication of an ASD

A

Paradoxical embolism

37
Q

Why is there L –> R shunting in an ASD?

A

Because the L ventricle is less compliant (NOT because of systemic vs pulmonic circulation, from which the atria are guarded)

38
Q

What can ASD lead to in adulthood? (3)

A

Hypertension, ischemic heart disease, and a less compliant left heart

39
Q

Describe Eisenmenger syndrome

A

Elevated pulmonary vascular resistance causes reversal of the original shunt (to the right-to-left direction) and systemic cyanosis

40
Q

What is the most common cause of cyanosis after infancy?

A

Tetralogy of Fallot

41
Q

What are the 4 features of Tetralogy of Fallot

A

VAD, subvalvular pulmonic stenosis, overriding aorta that receives blood from both ventricles, RV hypertrophy

42
Q

What is the primary cause of the shunt flow across the VSD in Tetralogy of Fallot?

A

Severity of the pulmonic stenosis

43
Q

When does Tetralogy of Fallot manifest most strongly? Why? What do children do to compensate?

A

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
Q

What is tx for Tetralogy of Fallot?

A

Surgical correction to close VAD and enlarge subpulmonary infundibulum

45
Q

What are four other (pathoma) congenital heart abnormalities that cause cyanosis?

A
  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
Q

PDA and infantile coarctation of the aorta are associated with what physical finding? Why?

A

Cyanosis of the lower extremities because the PDA is after the branches of the aortic arch