Congenital Heart Defects

Question 1

______ defects are the most common type of birth defect

Answer 1

Congenital heart defects

Question 2

cyanotic vs acyanotic congenital heart defects

RT -> LT Shunt

Answer 2

Cyanotic

oxygen-poor blood is pumped through body

Question 3

Cyanotic vs Acyanotic Congenital Heart Defect

RT <- LT

Answer 3

Acyanotic

blood is oxygenated, there’s just not enough of it

oxygenated blood is cycled through lungs a 2nd time

Question 4

The 5 Ts of Cyanotic Heart Disease

Answer 4

Truncus Arteriosus
Transportation of the Great Arteries
Tricuspid Atresia
Tetralogy of Fallot
Total Anomalous Pulmonary Return

Question 5

What?

Answer 5

Truncus Arteriosus

Question 6

What?

Answer 6

Transposition of the Great Arteries

Question 7

What?

Answer 7

Tricuspid Atresia

tricuspid valve never formed. Pt will likely have an atrial & ventricular septal defects, which we will try to keep open to buy the pt some time before tricuspid valve Sx. poorly oxygenated blood running through your body is better than no blood.

Question 8

what?

Answer 8

Tetralogy of Fallot

1. Ventricular Septal Defect
2. Pulmonic stenosis
3. Overriding aorta - the aortic valve is enlarged and seems to open from both ventricles. Aortic valve sits directly on top of the ventricular septal defect
4. RT ventricular hypertrophy (more of a result due to the other 3 main causes)

Question 9

Tricuspid Atresia Repair Options

Answer 9

Fontan procedure connect SVC to pulmonary arteries. Direct route to lungs for oxygenation

Question 10

“Tet Spells”

Answer 10

Infants/children are agitated for mins-hrs due to cyanosis

Question 11

Why do pts with heart defects squat after physical activity?

Answer 11

squatting increases systemic vascular resistance -> decreases RT->LT shunt

This is from Dr. V. I’m a lil confused about the shunt explanation. I recall Dr. Ryan just saying it increases resistance to limbs, so more blood is pumped to brain

Question 12

Tetralogy of fallot murmur

Answer 12

Systolic ejection murmor at LT sternal border due to pulmonic stenosis

Question 13

What does Tetralogy of Fallot look like on CXR, EKG, and Echo?

Answer 13

CXR: “boot shaped heart” upturned cardiac apex due to RV hypertrophy
EKG: RV enlargement
Echo: VSD, overriding aorta, RV wall hypertrophy

Question 14

What imaging makes the definitive dx for Tetralogy of Fallot?

Answer 14

Echocardiogram shows VSD, overriding aorta, RV wall hypertrophy

IVS = interventricular septum; VSD = ventricular septal defect; A= Aorta

Question 15

Cyanotic kid + “boot shaped heart” =

Answer 15

Tetralogy of Fallot

Question 16

Trmnt for Tetralogy of Fallot

Answer 16

Very symptomatic -> Oxygen, beta-blockers, epi (the key is a lil veinoconstriction), IV fluids

Prostaglandin E1 -> *prevent PDA closure *to help maintain pulmonary blood flow

**Sx closure **(4-12months old)

Question 17

What is the #1 thing you gotta repair in Tetralogy of Fallot?

Answer 17

PULMONIC STENOSIS

Question 18

Most common cyanotic heart disease

Answer 18

Tetralogy of Fallot

Question 19

“Pink” vs “Blue” Tet

Answer 19

Pink = adequate pulmonary blood flow at birth
Blue = restricted pulm blood flow (cyanosis at birth). PDA dependent; give prostaglandin E1 to keep it open

Question 20

Which genetic syndrome is most commonly associated with Tetralogy of Fallot?

Answer 20

Trisomy 21 (Down Syndrome)

Question 21

Answer 21

Total Anomalous Pulmonary Venous Return (TAPVR)

Question 22

What does type 1 Total Anomalous Pulmonary Venous Return (TAPVR) look like on CXR?

Answer 22

Snowman

Question 23

Blue babies get ____

Answer 23

ECHOS for poss congital heart defect

Question 24

Are CXRs used to dx congenital heart defects?

Answer 24

NO, just to see issue. Can only Dx with ECHO

Question 25

After birth, an Atrial Septal Defect is initially a ____ to ____ shunt

cyanotic or acyanotic?

Answer 25

RT <- LT

Acyanotic

Bc LT side of heart is higher pressure than RT (low pressure lungs)

Question 26

Patent Ductus Arteriosus is defined as failure of DA to close within ___hrs

Answer 26

72

Question 27

What is Eisenmenger Syndrome and how does it affect ASDs and VSDs?

Answer 27

After birth, flow through the septal defects are acyanotic RT<-LT shunts Bc the pulm circuit is lower pressure than the systemic circuit. Over time you push more blood into pulm circuit and it can get so bad to the point where the pressure is greater in the pulm circuit than in the systemic circuit. Flow through septum flips RT -> LT (cyanotic)