Congenital Heart Disease Flashcards

1
Q

When are most congenital heart problems manifested?

• What maternal Factors play a role?

A

Weeks 4-9

Maternal Factors: 
• Infection, RUBELLA
• EtOH
• Radiation
• Teratogenic Drugs
• Genetic
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2
Q

What common genetic disorder pre-disposes patients for increased congenital heart formations?
• What is the most common type seen in these people?

A

DOWN (trisomy 21)
• ATRIOVENTICULAR SEPTAL DEFECTS are most common

Note: these are sometimes called endocardial Cushion Defects

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3
Q

What is the most common type of shunting seen in heart defects?

A

• Left to Right Shunts are the most common

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4
Q

What are the 4 most common congenital heart defects that cause RIGHT to LEFT shunting?
• Which is most common?

A
  • Atrial Septal Defects
  • Ventricular Septal Defects - MOST COMMON
  • Patent Ductus Arteriosus
  • AV septal defect
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5
Q
What defects have a characteristic mumur out of the following: 
• ASD
• VSD
• Patent Ductus Arteriosus
• AV septal Defect
A

VSD - Pansystolic Murmur

Patent Ductus Arteriosus - MACHINE MURMUR

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6
Q
Of the following which is associated with a Rubella infection in the first 4 to 9 weeks of pregnancy: 
• ASD
• VSD
• Patent Ductus Arteriosus
• AV septal Defect
A

Patent Ductus Arteriosus

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7
Q

T or F: cyanosis is typically an early feature of Left to Right Shunts.

A

FALSE, left to right shunting doesn’t lead to mixing of oxygenated and unoxygenated blood

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8
Q

What is Eisenmenger Syndrome?

A

• Occurs when a Left to Right Shunt puts so much volume and pressure into the Right side of the heart that the RIGHT SIDE OF THE HEART ENLARGES AND STARTS PUSHING BLOOD INTO THE LEFT SIDE OF THE HEART - this will end up causing cyanosis

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9
Q

What causes the vast majority of atrial septal defects?

A

• OSTIUM SECUNDUM does not grow in as it should and fails to occlude the SECOND OSTIUM

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10
Q

T or F: atrial septal defects may lead to systemic hypertension.

A

False, they will lead to PULMONARY hypertension because blood will go LEFT to RIGHT and back into the PULMONARY circuit to cause PULMONARY HTN.

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11
Q

What will happen if Pulmonary hypertension from an ASD persists?

A
  • you will get RIGHT VENTRICULAR HYPERTROPHY from persistent larger volumes getting put in from the atria.
  • EISENMENGER’S COMPLEX will eventually happen because atria won’t be able to push into the hypertrophied ventricle
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12
Q

What is a PARADOXICAL EMBOLUS when does this happen?

A
  • Emoblus from venous circulation winds up in Systemic Circulation
  • This is allowed to happen in a RIGHT ATRIAL/VENTRICLE shunt (possible from Eisenmenger’s pushes the embolus from venous to systemic ciruculation
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13
Q

Where is the septum secundum found?

A

• Atria

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14
Q

What is the most common septal defect at birth?

• If someone is born with this why would you not automatically do surgery?

A
  • Ventricular Septal Defects are the Most common

* These often spontaneously Close

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15
Q

What defect is associated with trisomy 13?

• were specifically is it found?

A

• Ventricular Septal Defect - typically in the Membranous Septum

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16
Q

What is the risk with having small septal defects even if no shunting is produced?

A

• Having even small defects increases the risk of getting ENDOCARDITIS

17
Q

Ductus Arteriosus
• What is it?
• Type of Shunting if Patency is maintained
• Murmur

A

Ductus Arteriosus
• Connects Aortic and Pulmonary Trunks

  • Produces a Left to Right Shunt
  • Machine Murmur is produced
18
Q

What are all of the Right to Left Shunts?

A
  • Tetrology of Fallot
  • Transposition of Great Arteries
  • Persistent Truncus Arteriosus
19
Q

What are the Left to Right Shunts?

A
  • ASD
  • VSD
  • PDA
  • AV septal Defect
20
Q

WHAT ARE THE 4 COMPONENTS OF THE TETROLOGY OF FALLOT?

A
  • Pulmonary Stenosis - Right ventricle has hard time pumping into pulmonary trunk
  • Overriding Aorta - takes blood from BOTH ventricles
  • Ventricular Hypertrophy - from stenosis
  • Ventricular Septal Defect
21
Q

What is a key gross characteristic of a heart from someone that has Tetrology of Fallot?

A

• BOOT SHAPED heart

22
Q

Your kid begins crying and subsequently becomes cyanotic and passes out. What congenital Defect might they suffer from?

A

• This is likely a “Tet Spell” - kids pass out when eating or crying etc.

23
Q

In what septal defect are drugs given to PREVENT closure of the ductus arteriosus?
• what drugs?

A

• PROSTAGLANDINS are given to prevent closure of the Ductus Arteriosus

24
Q

What FOUR major things are you looking for with TRANSPOSITION OF THE GREAT ARTERIES?

A
  • DISCORDANT connection of ventricles to vascular outflow
  • ABNORMAL formation of the truncal and aortopulmonary septa
  • VSD (may or may not be present)
  • PATENTENT DUCTUS ARTERIOSUS - we want this to stay open

VSDs and PDA are good in these diseases

25
Q

PERSISTENT TRUNCUS ARTERIOSUS
• descibe this defect
• genetic connection
• Symptoms

A
  • Primative Truncus (combo of Aortic and Pulmonary valve) doesn’t divide
  • 22q11 Deletion Syndrome aka DiGeorge - about 1/3 of these kids have persistent truncus arteriosus
  • Symptoms are MILD CYANOSIS and signs of HEART FAILURE
26
Q

What is Aortic Coarctation?
• Gender Most Affected?
• Syndrome Association
• Other Abnormalities?

A

Coarctation - narrowing of aortic lumen leading to outflow obstruction

  • MALES most often affected
  • Associated with TURNERS SYNDROME (XO)
  • 50% of cases also have a BICUSPID aortic valve
27
Q

What are the two forms of Aortic Coarctation?

• how do these differ?

A

Infantile Form:
• PRE-DUCTAL
• often PATENT Ductus Arteriosus
• RIGHT ventricular HYPERTROPHY

Adult Form:
• POST-DUCTAL
• NO Patent Ductus Arteriosus
• LEFT ventricular HYPERTOPHY

28
Q

What are some unique features of Infantile and Adult forms of Aortic Coarchtation?

A

Infantile:
• LOWER BODY CYANOSIS - because PDA pumps deoxygenated blood out just past the 3 branches of the aortic arch

Adult:
• Rib Notching - caused because blood is shunted to Thoracic Vessels to Bipass semi-occluded Aorta (only seen in patients older than 5 or so)

29
Q

T or F: people with aortic coarctation are likely to get athlerosclerotic plaques

A

True, specificially they get them at the area of the stenosis due to turbulent flow

30
Q

What Heart Defect is Rubella associated with?

A

Patent Ductus Arteriosus

31
Q

What defect is Down Sydrome associated with?

A

Septal Defects

32
Q

What heart defect is Turner Syndrome Associated with?

A

Coarchtation of the Aorta

33
Q

What heart defect is associated with 22q11

A

Tetraology of Fallot

34
Q

What heart defect is associated with fetal alcohol syndrome?

A

ASD/VSD

35
Q

What heart defect is associated with Maternal Muscular Dystrophy types I and II?

A

Transposition of the Great Vessels