8/18- LAB: Pathology of Congenital Heart Disease Flashcards Preview

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Flashcards in 8/18- LAB: Pathology of Congenital Heart Disease Deck (27):
1

Describe the circuit of fetal circulation

- Ductus venosus takes oxygenated UV blood to suprahepatic IVC

- IVC blood is directed across PFO to LA-LV-Ascending aorta

- SVC blood directed to RV-PA

- High pulmonary vascular resistance directs PA blood to PDA and descending aorta

- Umbilical arteries take blood back to placenta 

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2

Describe the postnatal transitional circulation

- Closure of fetal shunts

- Gradual decrease in pulmonary vascular resistance

3

What is patent ductus arteriosus? Consequences?

Left to right shunt (from aorta to pulmonary artery)

Consequences:

- Increased PA bloodflow -> LV dilation

- Increased PA pressure -> RV hypertrophy

- Increased risk for pulmonary HTN! 

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4

Closure of the ductus arteriosus occurs how?

Functional closure (intimal cushions*)

- 1-2 d muscular contraction

- Followed by:

Anatomic closure

- Ductal ligament "ligamentum arteriosum"

- 6 wks - 3 mo 

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5

Treatment of PDA?

- Pharmacologic: Indomethacin (PGE Inhib)

- Percutaneous catheterization: occlusion devices

- Surgical ligation

6

What is ductus dependent congenital heart disease?

Certain lesions that may be asymptomatic in-utero will lead to early death following birth due to closure of the fetal shunts, largely the ductus arteriosus.

- In this scenario, administration of Prostaglandin E (PGE) to maintain ductal patency is “life saving”.

7

Clinical Case)

- Full term male, uncomplicated birth Hx

- At 2 d, he becomes fussy, feeding poorly, dusky blue nail beds and grunting with tachypnea

- Parents take to ER but he collapses and becomes unresponsive en route

- In ER, he has tachycardia with weak pulses and cool skin

These signs/symptoms point to what conditions/diseases?

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Cyanosis is indicated by blue nailbeds

Congestive Heart Failure is indicated by:

- "Fussy", feeding poorly

- Grunting with tachypnea

Shock is indicated by:

- Collapsing, unresponsive tachycardia

- Weak pulses and cool skin

Heart structure:

- ASD

- No continuity between LV and aorta; there is no aortic valve. LV ends blindly at the base of the aorta

- Patent ductus arteriosus (PDA)

8

Think ductus dependent lesion with what part of the Hx?

Onset of symptoms at 2 days

9

What is shown here?

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- ASD

- No continuity between LV and aorta; there is no aortic valve. LV ends blindly at the base of the aorta

- Patent ductus arteriosus (PDA) 

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10

Case)

If there is no aortic valve; the LV ends blindly at the base of the aorta. The aortic valve is described as?

A. Hypoplastic

B. Atretic

C. Dysplastic

D. Atrophic

If there is no aortic valve; the LV ends blindly at the base of the aorta. The aortic valve is described as?

A. Hypoplastic

B. Atretic

C. Dysplastic 

D. Atrophic

Hypoplastic- decreased size

Atretic- imperforate; no opening AT ALL

Dysplastic- poorly formed

Atrophic- started normally sized, but decreased due to physiologic/other reasons

11

Case)

The left ventricle and ascending aorta are described as?

A. Hypoplastic

B. Atretic

C. Dysplastic

D. Atrophic 

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The left ventricle and ascending aorta are described as? 

A. Hypoplastic 

B. Atretic 

C. Dysplastic

D. Atrophic 

12

Case)

Which of the following is true?

A. The baby was cyanotic because of a right to left shunt at the foramen ovale.

B. The baby was cyanotic because of a left to right shunt at the foramen ovale.

C. The baby was cyanotic because of a left to right shunt at the ductus arteriosus.

D. The baby was cyanotic because of a right to left shunt at the ductus arteriosus.

Which of the following is true? 

A. The baby was cyanotic because of a right to left shunt at the foramen ovale.

B. The baby was cyanotic because of a left to right shunt at the foramen ovale. 

C. The baby was cyanotic because of a left to right shunt at the ductus arteriosus.

D. The baby was cyanotic because of a right to left shunt at the ductus arteriosus.

13

Case)

The ductus arteriosus(*) provides bloodflow to:

A. The systemic circulation

B. The systemic and coronary circulation

C. The pulmonary circulation

D. All of the above 

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The ductus arteriosus(*) provides bloodflow to:

A. The systemic circulation

B. The systemic and coronary circulation 

C. The pulmonary circulation

D. All of the above 

- Coronary arteries can't get bloodflow from aorta (no flow across it or valves); flow actually going backwards down aorta to coronary arteries

14

Case) With closure of the ductus arteriosus:

A. This baby will develop shock.

B. his baby will develop worsening cyanosis.

C. This baby will have congestive heart failure.

D. All of the above.

Case) With closure of the ductus arteriosus:

A. This baby will develop shock. 

B. his baby will develop worsening cyanosis. 

C. This baby will have congestive heart failure.

D. All of the above.

15

In this case, the obstructive lesion in the aortic arch is what?

Aortic coarction that sits opposite the entry of the PDA(*)

- In this position it is called jxutaductal 

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16

What other lesions are associated with coarction of the aorta?

A. Bicuspid aortic valve

B. Congenital and acquired aortic stenosis

C. Tubular hypoplasia of the arch

D. All of the above

What other lesions are associated with coarction of the aorta? 

A. Bicuspid aortic valve

B. Congenital and acquired aortic stenosis 

C. Tubular hypoplasia of the arch 

D. All of the above

- Tubular hypoplasia: abnormal elongation/decreased size

17

What is the most common congenital cardiac anomaly causing:

- Death in neonate

- Reason for heart transplantation

Hypoplastic Left Heart Syndrome

18

What is surgical repair look like in hypoplastic left heart syndrome?

Occurs in stages:

- Norwood-Glenn-Fontan

- Occurs over first 2 years of life 

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19

Many pts with full repair of Hypoblastic Left Heart Syndrome typically develop what?

RT heart failure

- require heart transplant 

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20

Ductus dependent lesions are typically asymptomatic in-utero, but incompatible with prolonged postnatal life.

Which of the following fall into this category?

- Severe aortic stenosis

- Pulmonary valve atresia

- Ventricular septal defect

- Transposition of the great arteries

- Total anomalous pulmonary venous connection (TAPVC)

- Severe aortic stenosis

- Pulmonary valve atresia (depend on ductus to get blood from aorta to pulm art to get to lungs)

- Ventricular septal defect

- Transposition of the great arteries 

- Total anomalous pulmonary venous connection (TAPVC) (don't need a ductus here; need a foramen ovale)

21

Case 2)

Same Hx as before

- Full term male, uncomplicated birth Hx

- At 2 d, he becomes fussy, feeding poorly, dusky blue nail beds and grunting with tachypnea

- Parents take to ER but he collapses and becomes unresponsive en route

- In ER, he has tachycardia with weak pulses and cool skin

In this case, this is transposition of the great arteries (similar presentation to other ductus dependent conditions)

22

What is transposition of the great arteries?

- RV pumps into aorta while LV pumps into pulmonary a.

- Need ductus to get oxygenated blood to body! 

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23

Which statement is true in regard to transposition of the great arteries (TGA)?

A. Survival depends on patency of the foramen ovale.

B. Survival depends on patency of the ductus arteriosus.

C. A ventricular septal defect would benefit this patient.

D. All of the above

Which statement is true in regard to transposition of the great arteries (TGA)? 

A. Survival depends on patency of the foramen ovale. 

B. Survival depends on patency of the ductus arteriosus. 

C. A ventricular septal defect would benefit this patient.

D. All of the above

- If VSD was present, would have another means of oxygenating the blood going out to the body; may not even depend on ductus (might not present after 2ish days)

24

Treatment for TGA?

Arterial switch repair

- Connect aorta back to LV and pulmonary artery back to the right

- Have to leave the right valve (with coronary artery outflow) so take button of coronary arteries off and reattach to the L (patch the holes left behind)

- Arterial switch repair is performed in the 1st 2 wks of life while the LV still has its muscle mass form the high pulmonary vascular resistance of the fetus (preferred repair method)

- (before this, an "atrial switch" was done by excising the atrial septum and placing a tortuous patch to redirect pulmonary venous blood to the RV and systemic venous blood to the LV 

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25

How common is TGA?

- One of the most common congenital heart anomalies seen in adults (most repaired with "atrial switch") 

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26

Lon gterm complications of TGA?

- RV "systemic ventricle" failure

- Tricuspid "systemic" valve regurgitation

- Atrial/ventricular arrhythmias

- Obstruction of systemic or pulmonary veins by the patch that redirects atrial blood flow

27

What are the three most common congenital abnormalities in adults?

1. Tetralogy of Fallot (FOT)

2. Transposition of the Great Arteries (TGA)

3. Broad combo of Sx similar to hypoplastic L heart syndrome