Congenital Heart Defects

Question 1

Shunts that progress from R to L present _________ than shunts from L to R

Answer 1

earlier

Question 2

How do we remember the R to L shunts?

Answer 2

5 T’s

are Terrible, i.e. present earlier

(Tetralogy of Fallot, Transposition, Truncus arteriosus, Tricuspid atresia, TAPVR)

Question 3

How to we remember the L to R shunts?

Answer 3

VSD/ASD/PDA

L to R = Late

Question 4

Tetralogy refers to what in Tetralogy of Fallot?

Answer 4

Pulmonary Stenosis
RVH
Overriding aorta
VSD

(PROVe it!)

Question 5

A very young child presents because his mom notices his face turns blue every now and then. He squats down, and the symptoms get better. What is the most important determinant in the prognosis of the child?

Answer 5

Degree of PULMONARY STENOSIS

presents young, so must start with a T and be a R to L shunt

Question 6

What is the mechanism of squatting in a Tetralogy of Fallot patient?

Answer 6

increases systemic pressure, which decreases the R to L shunt, which pushes blood into the pulmonary artery (decreasing symptoms)

Question 7

What is often the shape of the heart on x ray in Tetralogy of Fallot?

Answer 7

boot shaped

seen in RVH

Question 8

The cause of all the symptoms of Tetralogy of Fallot comes down to what anatomical defect?

Answer 8

anterosuperior displacement of the infundibular septum

Question 9

VSD/ASD/PDA is to pulmonary HYPERtension as Tetralogy of Fallot is to __________

Answer 9

HYPOtension

due to stenosis; will show decreased pulmonary vascular markings on x ray

Question 10

Describe the flow of events (Eisenmenger syndrome) that occurs in children with late cyanosis.

Answer 10

starts as L-R shunt (VSD/ASD/PDA)
slowly develop pulmonary HTN (due to hypertropy)
resistance becomes super hi, get reversal of shunt and appearance of cyanosis/clubbing/polycythemia

Question 11

Contrast the location of the two types of coarctation of the aorta

Answer 11

Infantile: often btw branches of aorta and ductus

Adult: often AFTER the ligamentum arteriosum

(INfantile = IN closer; aDult=Distal to Ductus)

Question 12

In adult form of coarctation of the aorta, what vessels will be grossly dilated?

Answer 12

IMA artery (internal mammary, aka internal thoracic)

sends blood via anterior intercostal arteries to bypass the stenotic aorta

leads to RIB NOTCHING

Question 13

Describe the physical exam of a pt with coarctation of the aorta.

Answer 13

HTN/flushing/strong pulse in the UE

hypotension/cyanosis/weak pulse in the LE

Question 14

What two key associations are linked to infantile coarctation of the aorta?

Answer 14

Turner syndrome (45 XO)

PDA

Question 15

Adult coarctation of the aorta is associated with ________ and can lead to ______________

Answer 15

bicuspid aortic valve

aortic regurgitation

Question 16

An infant pops out and has cyanosis. Her aorta is anterior to the pulmonary trunk. What should be given immediately?

Answer 16

PGE1 (alprostadil)

keeps ductus arteriosus open until sx

Question 17

Alprostadil is also used for?

Answer 17

erectile dysfunction

Question 18

An infant pops out and has cyanosis. Her aorta is anterior to the pulmonary trunk. What condition did her mother most likely have?

Answer 18

Maternal diabetes

high assoc with transposed great vessels and sacral agenesis

Question 19

D-transposition of great vessels is to ‘failure to spiral’ as Persistent Truncus Arteriosus is to _____________

Answer 19

failure of aorticopulmonary septum to develop

Question 20

A toddler presents with lower extremity cyanosis. He has a holosystolic, machine like murmur. What treatment is given to correct the issue?

Answer 20

Indomethacin closes a PDA

Question 21

A toddler presents with lower extremity cyanosis. He has a holosystolic, machine like murmur. What are the characteristics of the most likely infectious organism that mom had?

Answer 21

Rubella (German measles)

Togavirus family; Enveloped RNA, SS + linear

Question 22

A toddler presents with lower extremity cyanosis. He has a holosystolic, machine like murmur. His mom had a congenital infection. What should we check this little dude for?

Answer 22

cataracts (assoc with maternal Rubella)

Question 23

A toddler presents with lower extremity cyanosis. He has a holosystolic, machine like murmur. What are the most likely manifestations mom had as a result of an infection?

Answer 23

Fine Rash (starts at head, moves down)
LAD
Arthraligia/arthritis

Question 24

An infant presents with early cyanosis. Imaging reveals a hypoplastic RV and an ASD. What is the most likely etiology?

Answer 24

Tricuspid Atresia

Question 25

VSDs are associated with

Answer 25

Fetal Alcohol syndrome

Question 26

Paradoxical emboli are associated with

Answer 26

ASDs (emboli lodge in brain or periphery by going thru ASD)

Question 27

ASDs are most commonly problems in development of what embryonic structure

Answer 27

ostium secundum

Question 28

ASDs can be due to a problem in the ostium primum, and must be immediately connected to

Answer 28

Down Syndrome

Question 29

Down’s syndrome is associated with an ASD due to a defect in ____________, which serves as what in the fetus?

Answer 29

Ostium primum

VALVE of foramen ovale

Question 30

Total anomalous pulmonary return (TAPVR) is when the pulmonary veins drain?

Answer 30

anywhere but normal (most often to to RA)

Question 31

What characteristic finding is heard on auscultation of an ASD?

Answer 31

Fixed splitting of S2

delayed closure of pulmonary valve after aortic valve, caused by L to R shunt

Question 32

A diabetic mom gives birth to a baby with an aorta on top of the pulmonary trunk. What cell type is to blame for the defect?

Answer 32

failure of neural crest cells to migrate

D-transposition

Question 33

A little kid comes in with cyanosis. He has no thymic shadow. Anti-CD20+ are positive, and anti-CD2+ are negative. What are the 2 most likely cardiac defects that he has?

Answer 33

kid has DiGeorge

either patent Truncus Arteriosus, or Tetralogy of Fallot

Question 34

A little kid comes in with cyanosis. He has no thymic shadow. Anti-CD20+ are positive, and anti-CD2+ are negative. Deletion of what chromosome will lead to what developmental abnormality?

Answer 34

deletion of Ch 22q11

failure to develop 3rd and 4th pharyngeal pouch