Abnormal CV Dev Flashcards

1
Q

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

A

weeks 3-8

  • Genetics- 9%
  • Environment-1%
  • Unknown- 90%
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2
Q

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

A
  • Familial like Marfan 2-3

- Chromosomal defects (non-familial) 5%

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3
Q

What are the left to right shunts?

A

Atrial septal defect
Ventricular septal defect
Patent Ductus arteriosus

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4
Q

What are the symptoms of a left to right shunt?

A

Cyanosis (sats

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5
Q

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

A

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

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6
Q

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

A
  1. Excessive resorption of primary septum (Spider like appearance, Apoptosis)
  2. Incompetent foramen ovale due to hypoplastic growth of secondary septum
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7
Q

What are other ways to have a atrial septal defect?

A
  1. inadequate dev of the primary septum producing a basal opening
  2. Sinus venosus dev defect
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8
Q

What happens to the physiology in a ASD?

A
  • Pulmonary flow 2-4x normal

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

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9
Q

What are some complications of a ASD?

A
  • Right ventricular failure

- Paradoxical embolism

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10
Q

How would one treat a ASD?

A

Surgery

- Secondary atrial septal percutaneous transvenous closure device

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11
Q

What do shunts do to the heart?

A

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
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12
Q

What is the most common congenital heart defect?

A

Ventricular septal defect
(VSD)
- 40%

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13
Q

What is the most common cause of VSD?

A

Membranous portion of ventricular septum fails to close

- swiss- cheese septum

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14
Q

How does one fix a VSD?q

A

50% close spontaneously

- surgery

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15
Q

What other anomalies are VSD associated with?

A
  1. Pulmonary stenosis
  2. Tetraology of Fallow
  3. Coarctation of aorta
  4. Transposition of aorta
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16
Q

What does the ductus arteriosus derive from?

A

6th aortic arch

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17
Q

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

A

From aorta to pulmonary artery

  • if reversed cause cardiac enlargement and increase pulmonary vascularity
  • at day 30 DA should be a ligament
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18
Q

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

A

Maternal rubella during early weeks of pregnancy

19
Q

What are some other complications of maternal rubella?

A
  • Cataracts
  • Deafness
  • Pulmonary stenosis and VSD
20
Q

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

A

Thrill

- Catheter occluder or Ibuprofen if premature

21
Q

What are some clinical complications of a PDA?

A

Infective endocarditis

Pulmonary HTN

22
Q

What are the defects in a Tetraology of Fallot?

A
  1. VSD
  2. Pulmonary stenosis
  3. Right ventricular hypertrophy
  4. Over ridding large aorta
23
Q

What is clinical significant in T of F?

A

Cyanosis is size dependent

- murmur due to stenosis

24
Q

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

A

Paradoxical embolism
Infective endocarditis
- surgery

25
Q

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

A

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

26
Q

Is TGA compatible with life and how is it fixed?

A

Yes if there is a VSD other no

- fixed via surgery

27
Q

What other shunts are common in TGA?

A

ASD and PDA

28
Q

What is the male to female ratio of TGA?

A

M:F 3:1

- offspring of a diabetic mother

29
Q

What is Persistent Truncus arteriosus?

A

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

30
Q

What is the cause of Persistent truncus arteriosus?

A

failure of bulbar and truncal ridges to to develop

31
Q

What are the obstruction CHD?

A
  1. Coarctation of aorta
  2. pulmonary stenosis and atresia
  3. aortic stenosis and atresia
32
Q

What is Coarctation of the aorta?

A

Narrowed aortic lumen

- Infolding of the aorta opposite the closed ductus arteriosus

33
Q

What are the sx’s of coarctation?

A
  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
Q

Is coartation more common in males or females? Postductal

A

males 2x

35
Q

What the some complications of the a coartation Postducatal?

A

CHF
CVA
Rupture
Infection

36
Q

How are coartations fixed?

A

Excision
Bypass
Ballon angioplasty

37
Q

What causes pulmonary/aortic stenosis and atresia?

A

Asymmetrical division or spiraling of the aorticopulmonary septum

38
Q

How often does a preductal coarctation of the aorta heppne?

A

5%

39
Q

What is the cause of preductal coarctation?

A

Tubular hypoplasia of the aortic arch proximal to the PDA

40
Q

What are some complications of a preductal coarctation?

A

CHF
PDA
Cyanosis, lower body
Mortality increased

41
Q

What is diGeorge syndrome and what is its association?

A

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

42
Q

Down syndrome has association with what CHD?

A

ASD

VSD

43
Q

Turner syndrome as ass with what CHD?

A

Coarctation of aorta

44
Q

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

A

Aortic stenosis

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