Pathology: Pediatric Cardiology I and II Flashcards Preview

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Flashcards in Pathology: Pediatric Cardiology I and II Deck (33):
1

Overview of Congenital Heart Disease

  • What do most defects that allow live birth have in common?
  • What is the most common defect?
  • When do most defects occur during gestation?
  • What are the environmental causes of CHD?
  • What are the common genetic causes?

  • What do most defects that allow live birth have in common?
    • Effect a single chamber region of the heart
  • What is the most common defect?
    • VSD
  • When do most defects occur during gestation?
    • Between weeks 3 and 8
  • What are the environmental causes of CHD?
    • Rubella, teratogens, maternal diabets
  • What are the common genetic causes?
    • Trisomies
      • 13, 15, 18, 21
    • Turner Syndrome

2

What are the 5 key steps in correct cardiac development?

3

What are the 6 key molecular pathways needed for correct cardiac development?

4

Overview

  • What is the type of inheritance do most genetic defects follow?
  • What 3 key transcription factors are needed to coregulate the target genes for proper cardiac involvement?

5

DiGeorge Syndrome

  • What chromosome is implicated in half of these patients?
    • What are the 5 main conditions/signs of mutations of this chromosome?
  • What developmental structures are affected by this mutation?
    • What organs are thus affected (besides the heart)?

6

CATCH 22

  • What is the gene implicated most in CHD?
    • What 2 key functions does it have related to development?

7

Genetics of CHD

  • What condition affects the X chromosome that is implicated in many cases of CHD?
  • What is the most common genetic condition associated with CHD?
    • What is the most common structure that is affected by this condition?

8

  • What are the common left to right shunts?
  • What are the common right to left shunts?

  • What are the common left to right shunts?
    • ASD
    • VSD
    • PDA
  • What are the common right to left shunts?
    • TOF
    • TGV
      • w/ VSD
      • w/o VSD

9

What is the biggest indication/sign of R-L shunts? Why?

10

Left to Right Shunts

  • What happens to the blood flow to the pulmonary circulation?
    • What does this do the lung vascular resistance?
    • How does this affect heart development?
  • What L to R shunts eventually cause if untreated?

11

How can you reverse the significant pulmonary HTN seen in L to R shunts?

You can't

12

Patent Foramen Ovale

  • What are the 2 fenestrations that eventually become the foramen ovale?
  • How common is this condition?
  • What can it lead to?
    • How can it manifest clinically?

13

ASDs

  • What is the most common developmental structure associated with ASDs?
    • What is the common name for this type of defect?
  • Hemodynamic induced changes
    • What happens to the right atria and ventricles?
      • What happens to the RV alone?
    • What happens to the pulmonary artery?
    • What is the mechanical reason for the changes?

14

ASDs

  • What defect usually affects the lowest part of the atrial septum?
    • What valves are implicated in this?
  • What defect usually affects the highest part of the atrial septum?
    • How does affect flow in the pulmonary veins?

15

Clinical Features of ASD

  • When do symptoms manifest?
  • This is a hole in between the atria. Why is this a L to R shunt and not an R to L shunt?
  • Why would someone develop pulmonary hypertension from this?
  • What is the Tx for it?

16

VSDs

  • How common is this defect?
  • Why do most VSDs occur at the basal (membranous) region of the ventricular septum?
  • Does this defect usually occur in isolation or with other cardiac defects?

17

VSDs

  • What happens to the right ventricle?
  • What happens to the pulmonary artery? Why?

18

VSDs

  • Small to medium VSDs
    • These may be asymptomatic, and many close spontaneously if they are in the muscular portion. However, they can still cause pathology. How?
  • Larger VSDs
    • These are associated with pulmonary hypertension, just like ASDs. How does the pulmonary HTN differ with VSDs, compared to ASD?

19

PDA

  • Where is a normal ductus arteriosis located?
  • Normally, when/Why does the ducts close? (3 reasons why)
  • What is left after the patent ductus arteriosis completely obliterates?

20

PDA

  • What can cause the ductus to not close or at the very least delay the closing?
  • How common is this?
    • Does it usually occur with some other pathology?

21

PDAs

  • What type of carditis can occur with this defect?
  • Why would you suspect this upon physical examination? 
  • How do small PDAs present?
  • Large PDA: What condition can manifest?
  • What 2 conditions could a PDA be a life-saving for?
    • What would be needed in order to keep a PDA open?

22

R to L shunts

  • What is the early sign of these?  Why does it occur?
  • What are the 3 common clinical consequences that these shunts have? (different than the above question?
  • What are the 2 common conditions associated with this?

23

TOF

  • What are the 4 cardinal features of this?

24

TOF

  • What shape does the heart have? Why?
  • What happens to the aorta and pulmonary trunk?
  • What happens to the left side of the heart?

25

TOF

  • Is the VSD small or large?
  • Where does it occur in the septum?
  • What causes the right ventricular flow obstruction?

26

TOF

  • Hemodynamics
    • What happens to pulmonary blood flow?
    • What happens to aortic volumes?
  • What does the severity of this condition largely depend on?

27

TOF

  • Why would TOF present as isolated VSD?
  • Pulmonic Stenosis
    • What condition can this cause early in life?
    • As the patient gets older, pulmonic stenosis can be a "good" thing. Why?

28

TOF

  • What are the typical manifestations of cyanotic heart disease?
  • Can you cure a TOF?
  • What do all R to L shunts increase the risk for?

29

TGA

  • Where does the aorta arise from? Pulmonary artery?
  • What is the functional outcome?
  • What other defects occur with this condition?
  • What happens to each ventricle?

30

TGA

  • What is the dominant manifestation of this?
  • What are the 3 factors that predict prognosis?
  • What are some examples of congenital obstructions?

31

COA

  • What kind of congenital heart defect is this? What other common defects are in this group?
  • What other defect is seen in almost half of the cases?
  • What gender gets this more?
  • What genetic condition is associated with this?
  • What are the two types?

32

COA

  • Preductal Coarctation
    • Where does the narrowing occur?
    • Where does the deoxygenated blood go?
    • What happens to the pulmonary trunk?
    • What happens to the right ventricle?
  • Postductal
    • What happens to the aorta?
      • Where does it dilate?
    • What happens to the left ventricle?

33

Clinical Features of COA

  • Preductal with a PDA
    • What happens the lower body?
  • Postductal without PDA
    • Why would this not be dx until later in life?
  • What happens to the upper extremity blood flow?
  • What happens to vessels around the coarctation, and how is the seen in an x-ray?
  • What can heard/felt upon PE?
  • How do you treat this?