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Flashcards in Abnormal CV Dev Deck (44):
1

When do most congenital heart disease arise? and what from?

weeks 3-8
- Genetics- 9%
-Environment-1%
-Unknown- 90%

2

In terns of Congenital heart diseases what makes up the 9% genetic defects?

-Familial like Marfan 2-3
-Chromosomal defects (non-familial) 5%

3

What are the left to right shunts?

Atrial septal defect
Ventricular septal defect
Patent Ductus arteriosus

4

What are the symptoms of a left to right shunt?

Cyanosis (sats

5

What are the right to left shunts and its symptoms presentation?

Tetralogy of Fallot
Transposition of Great arteries
Truncus arteriosus
- Cyanosis early in postnatal life

6

What are the 2 anomalies that create most atrial septal defects?

1. Excessive resorption of primary septum (Spider like appearance, Apoptosis)

2. Incompetent foramen ovale due to hypoplastic growth of secondary septum

7

What are other ways to have a atrial septal defect?

1. inadequate dev of the primary septum producing a basal opening

2. Sinus venosus dev defect

8

What happens to the physiology in a ASD?

-Pulmonary flow 2-4x normal
-Large ones lead to pulmonary HTN and cyanosis tardive and cardiac failure

9

What are some complications of a ASD?

-Right ventricular failure
-Paradoxical embolism

10

How would one treat a ASD?

Surgery
- Secondary atrial septal percutaneous transvenous closure device

11

What do shunts do to the heart?

Normal pressure 1:6 Pulmonary:aorta
- increases right ventricular pressure and pulmonary artery pressure increase leading to pulmonary fibrosis
- Can lead to ventricular septal defects

12

What is the most common congenital heart defect?

Ventricular septal defect
(VSD)
- 40%

13

What is the most common cause of VSD?

Membranous portion of ventricular septum fails to close
- swiss- cheese septum

14

How does one fix a VSD?q

50% close spontaneously
- surgery

15

What other anomalies are VSD associated with?

1. Pulmonary stenosis
2. Tetraology of Fallow
3. Coarctation of aorta
4. Transposition of aorta

16

What does the ductus arteriosus derive from?

6th aortic arch

17

What is common flow of a patent ductus ateriosus after birth?

From aorta to pulmonary artery
- if reversed cause cardiac enlargement and increase pulmonary vascularity
- at day 30 DA should be a ligament

18

What is the most common cause of patent ductus arteriosus PDA?

Maternal rubella during early weeks of pregnancy

19

What are some other complications of maternal rubella?

-Cataracts
-Deafness
-Pulmonary stenosis and VSD

20

What does the murmur sound like and how is a PDA fixed?

Thrill
- Catheter occluder or Ibuprofen if premature

21

What are some clinical complications of a PDA?

Infective endocarditis
Pulmonary HTN

22

What are the defects in a Tetraology of Fallot?

1. VSD
2. Pulmonary stenosis
3. Right ventricular hypertrophy
4. Over ridding large aorta

23

What is clinical significant in T of F?

Cyanosis is size dependent
- murmur due to stenosis

24

What are some complications of T of F and how is it treated?

Paradoxical embolism
Infective endocarditis
- surgery

25

What is the cause of Transposition of the great arteries TGA?

Failure of normal spiraling of the aorticopulmonary septum (bulbotruncal septum)

26

Is TGA compatible with life and how is it fixed?

Yes if there is a VSD other no
- fixed via surgery

27

What other shunts are common in TGA?

ASD and PDA

28

What is the male to female ratio of TGA?

M:F 3:1
- offspring of a diabetic mother

29

What is Persistent Truncus arteriosus?

Aorta and Pulmonary trunk receive blood from both ventricles
- leads to irreversible Pulmonary HTN
- Cyanosis present
-

30

What is the cause of Persistent truncus arteriosus?

failure of bulbar and truncal ridges to to develop

31

What are the obstruction CHD?

1. Coarctation of aorta
2. pulmonary stenosis and atresia
3. aortic stenosis and atresia

32

What is Coarctation of the aorta?

Narrowed aortic lumen
- Infolding of the aorta opposite the closed ductus arteriosus

33

What are the sx's of coarctation?

1. increase BP in arms, low in legs
2. intercostal artery blood flow increased (notched ribs)
3. Strong pulse in UE and decreased in LE

34

Is coartation more common in males or females? Postductal

males 2x

35

What the some complications of the a coartation Postducatal?

CHF
CVA
Rupture
Infection

36

How are coartations fixed?

Excision
Bypass
Ballon angioplasty

37

What causes pulmonary/aortic stenosis and atresia?

Asymmetrical division or spiraling of the aorticopulmonary septum

38

How often does a preductal coarctation of the aorta heppne?

5%

39

What is the cause of preductal coarctation?

Tubular hypoplasia of the aortic arch proximal to the PDA

40

What are some complications of a preductal coarctation?

CHF
PDA
Cyanosis, lower body
Mortality increased

41

What is diGeorge syndrome and what is its association?

Deletion of chromosome 22q11
- Tetralogy of Fallot
Truncus arteriosus (Persistent)

42

Down syndrome has association with what CHD?

ASD
VSD

43

Turner syndrome as ass with what CHD?

Coarctation of aorta

44

What is Marfan syndrome and what is its ass with (CHD)?

Aortic stenosis
- Fibrillin-1 glycoprotein abnormality, FBN1 gene
- Chromosomes 15q21.1 and 5q23.31