CV System Abnormal Development Flashcards

1
Q

CHD is the most common cardiac condition in childhood. What is the most frequent cause of CHD?

A

Unknown causes

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2
Q

What are left to right shunts?

A

Cyanosis several months or years after birth
Atrial Septal Defect
Ventricular Septal Defect
Patent (persistent) Ductus Arteriosus

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3
Q

What are right to left shunts?

A

Cyanosis early in postnatal life (O2 stats below 75%)

  • Tetralogy of Fallot
  • Transposition of the great arteries (TGA)
  • Truncus arteriosus (persistent)
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4
Q

What does the foramen ovale usually serve as in the fetus?

A

R –> L shunt!

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5
Q

What is the physiology of ASD?

A
  • Pulmonary blood flow 2-4X normal

- Only large ones lead to pulmonary hypertension that leads to cyanosis and heart failure

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6
Q

What are complications of ASD?

A
  • RV failure

- Paradoxical embolism (& brain infarction or abscess)

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7
Q

Atrial septal defects (ASDs) are common congenital heart anomalies. What is the most common form of ASD?

A

Patent foramen ovale

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8
Q

VSDs account for 40% of congenital heart defects. Which of the following is responsible for most forms of VSDs?

A

Abnormal formation of the endocardial cushions and aorticopulmonary septum (these make up the membranous portion of the ventricular septum).

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9
Q

What does the reversed blood flow in PDA cause?

A

Cardiac enlargement and increased pulmonary vascularity.

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10
Q

What aortic arch does the ductus arteriosus develop from?

A

6th - left side

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11
Q

What maternal problem is related to patent ductus arteriosus (PDA)?

A

Maternal rubella (german measles) during early weeks of pregnancy

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12
Q

What are other defects caused by maternal rubella?

A
  • Congenital cataracts
  • Deafness
  • Other congenital heart defects - pulmonary stenosis (PS), ventricular septal defect (VSD)
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13
Q

What type of murmur does PDA produce?

A

Machinery-like

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14
Q

What are clinical complications associated with PDA?

A
  • Infective endocarditis common

- Pulmonary hypertension common

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15
Q

What are treatments for PDA?

A

Surgery
-Catheter placed occluder
Also give ibuprofen to pre-matures

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16
Q

After birth, the ductus arteriosus usually closes. If the ductus remains patent, what clinical sign or symptom is most diagnostic of a patent ductus?

A

Machinery-like murmur

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17
Q

What is Tetralogy is Fallot?

A

(1) Pulmonary stenosis
(2) Overriding (Large) aorta
(3) Right ventricular hypertrophy
(4) Ventricular septal defect

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18
Q

What might you observe in clinic with Tetralogy of Fallot?

A
  • Murmur due to pulmonary stenosis

- Cyanosis (depending on size of defect it may be present early on)

19
Q

What complications are associated with TF?

A
  • Paradoxical embolism (brain abscess)

- Infective endocarditis

20
Q

Which of the four developmental consequences of the tetralology of Fallot would be the last to occur?

A

Right ventricular hypertrophy

21
Q

What clinical sign would be most obvious on examination of a patient with TF?

A

Cyanosis

22
Q

TF is a cardiac malformation that involves during early development, which of the following septa?

A

Aorticopulmonary septum

23
Q

What causes transposition of the great arteries?

A

Failure of normal spiraling of the aorticopulmonary septum. This means that the aorta comes off the right ventricle and the pulmonary trunk comes off the left ventricle.

24
Q

When would TGA be compatible with life?

A

If an atrial and ventricular septal defect and an associated patent ductus arteriosus is present

25
Q

In what groups is TGA common?

A

More common in males of diabetic mothers.

26
Q

Transposition of the great arteries (TGA) is a cardiac malformation that involves during early development which septa?

A

Aorticopulmonary septum

27
Q

What is Truncus Arterioles (Persistent)?

A
  • Common aorticopulmonary outlet, receiving blood from both ventricles
  • Due to failure of bulbar and truncal ridges (aorticopulmonary septum) to develop
  • Cyanosis present
  • Increased pulmonary blood flow that leads to irreversible pulmonary hypertension
28
Q

A persistent or common truncus arteriosus is a cardiac malformation that involves during early development, which septa?

A

Aorticopulmonary septum

29
Q

What three CV congenital defects involve bulbar and truncal ridges/aorticopulmonary septum?

A
  • Common/Persistant Truncus
  • Tetralogy of Fallot
  • Transposition of the great arteries (TGA)
30
Q

What is coarctation of the aorta?

A

Narrows aortic lumen infolding of the aorta opposite the closed ductus arteriosus (DA)

31
Q

What are clinical signs of coarctation of the aorta?

A
  • BP in arms increased
  • BP in legs decreased
  • Intercostal artery blood flow increased (NOTCHED RIBS)
  • HTN in upper extremities
  • Weak pulse & lower bp in lower extremities
32
Q

What are complications associated with coarctation of the aorta?

A
  • CHF
  • Cerebrovascular accident
  • Rupture
  • Infection
33
Q

How to best treat Coarctation of the aorta?

A

-Excision
-Bypass
[Experimental: balloon angioplasty]

34
Q

What can infantile (5%) (preductal) form of coarctation of the aorta cause?

A

It is tubular hypoplasia of the aortic arch proximal to a patent ductus arteriosus

  • Early CHF
  • Patent ductus arteriosus
  • Cyanosis, lower body
  • Mortality increased
35
Q

What causes aortic or pulmonary stenosis or atresia?

A

-Asymmetrical division or spiraling of the aorticopulmonary septum (bulbar and truncal ridges)

36
Q

13 yr old boy has no femoral pulses, increased BP in upper extremities, enlarged intercostal vessels. What abnormality is suspected?

A

Postductal coarctation of the aorta

37
Q

What is involved in aortic or pulmonary stenosis?

A

Aorticopulmonary septum

38
Q

What congenital defects are associated with blue babies?

A
  • Tetralogy of Fallot
  • Transposition of great vessels
  • Truncus arteriosus (persistent or common trunk)
39
Q

What is DiGeorge Syndrome?

A

Deletion in chromosome 22q11 - involved with neural crest cells

  • Tetralogy of Fallot
  • Truncus arteriosus (Persistent)
40
Q

What is down syndrome associated with?

A

Trisomy 21

  • Atrial septal defect
  • Ventricular septal defect
41
Q

What is turner syndrome involved in?

A

45, XO

-Coarctation of the aorta

42
Q

What is Marfan syndrome involved in?

A

Fibrillin-1 glycoprotein abnormality; FBN1 gene; chromosomes 15q21.1 and 5q23.31

43
Q

What is heard upon auscultation with ASD?

A

Fixed split S2!!

-Pulmonary valve takes longer to close due to increased volume/right ventricular filling (widened fixed split)