Abnormal CV Dev

Question 1

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

Answer 1

weeks 3-8

• Genetics- 9%
• Environment-1%
• Unknown- 90%

Question 2

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

Answer 2

• Familial like Marfan 2-3

- Chromosomal defects (non-familial) 5%

Question 3

What are the left to right shunts?

Answer 3

Atrial septal defect
Ventricular septal defect
Patent Ductus arteriosus

Question 4

What are the symptoms of a left to right shunt?

Answer 4

Cyanosis (sats

Question 5

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

Answer 5

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

Question 6

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

Answer 6

1. Excessive resorption of primary septum (Spider like appearance, Apoptosis)
2. Incompetent foramen ovale due to hypoplastic growth of secondary septum

Question 7

What are other ways to have a atrial septal defect?

Answer 7

1. inadequate dev of the primary septum producing a basal opening
2. Sinus venosus dev defect

Question 8

What happens to the physiology in a ASD?

Answer 8

• Pulmonary flow 2-4x normal

- Large ones lead to pulmonary HTN and cyanosis tardive and cardiac failure

Question 9

What are some complications of a ASD?

Answer 9

• Right ventricular failure

- Paradoxical embolism

Question 10

How would one treat a ASD?

Answer 10

Surgery

- Secondary atrial septal percutaneous transvenous closure device

Question 11

What do shunts do to the heart?

Answer 11

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

Question 12

What is the most common congenital heart defect?

Answer 12

Ventricular septal defect
(VSD)
- 40%

Question 13

What is the most common cause of VSD?

Answer 13

Membranous portion of ventricular septum fails to close

- swiss- cheese septum

Question 14

How does one fix a VSD?q

Answer 14

50% close spontaneously

- surgery

Question 15

What other anomalies are VSD associated with?

Answer 15

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

Question 16

What does the ductus arteriosus derive from?

Answer 16

6th aortic arch

Question 17

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

Answer 17

From aorta to pulmonary artery

• if reversed cause cardiac enlargement and increase pulmonary vascularity
• at day 30 DA should be a ligament

Question 18

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

Answer 18

Maternal rubella during early weeks of pregnancy

Question 19

What are some other complications of maternal rubella?

Answer 19

• Cataracts
• Deafness
• Pulmonary stenosis and VSD

Question 20

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

Answer 20

Thrill

- Catheter occluder or Ibuprofen if premature

Question 21

What are some clinical complications of a PDA?

Answer 21

Infective endocarditis

Pulmonary HTN

Question 22

What are the defects in a Tetraology of Fallot?

Answer 22

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

Question 23

What is clinical significant in T of F?

Answer 23

Cyanosis is size dependent

- murmur due to stenosis

Question 24

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

Answer 24

Paradoxical embolism
Infective endocarditis
- surgery

Question 25

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

Answer 25

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

Question 26

Is TGA compatible with life and how is it fixed?

Answer 26

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

Question 27

What other shunts are common in TGA?

Answer 27

ASD and PDA

Question 28

What is the male to female ratio of TGA?

Answer 28

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

Question 29

What is Persistent Truncus arteriosus?

Answer 29

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

Question 30

What is the cause of Persistent truncus arteriosus?

Answer 30

failure of bulbar and truncal ridges to to develop

Question 31

What are the obstruction CHD?

Answer 31

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

Question 32

What is Coarctation of the aorta?

Answer 32

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

Question 33

What are the sx's of coarctation?

Answer 33

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

Question 34

Is coartation more common in males or females? Postductal

Answer 34

males 2x

Question 35

What the some complications of the a coartation Postducatal?

Answer 35

CHF CVA Rupture Infection

Question 36

How are coartations fixed?

Answer 36

Excision Bypass Ballon angioplasty

Question 37

What causes pulmonary/aortic stenosis and atresia?

Answer 37

Asymmetrical division or spiraling of the aorticopulmonary septum

Question 38

How often does a preductal coarctation of the aorta heppne?

Answer 38

5%

Question 39

What is the cause of preductal coarctation?

Answer 39

Tubular hypoplasia of the aortic arch proximal to the PDA

Question 40

What are some complications of a preductal coarctation?

Answer 40

CHF PDA Cyanosis, lower body Mortality increased

Question 41

What is diGeorge syndrome and what is its association?

Answer 41

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

Question 42

Down syndrome has association with what CHD?

Answer 42

ASD | VSD

Question 43

Turner syndrome as ass with what CHD?

Answer 43

Coarctation of aorta

Question 44

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

Answer 44

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