Pathology: Congenital Heart Defects

Question 1

Right to left shunts result in

Answer 1

Cyanosis (hypoxemia)

Question 2

Left to right shunts result in

Answer 2

Acyanosis (pulmonary congestion)

Question 3

4 specific defects in tetralogy of fallot

Answer 3

VSD
Overriding aorta
Pulmonary artery stenosis
R ventricular hypertrophy

Question 4

How is blood shunted in tetralogy of fallot?

Answer 4

R ventricle -> large VSD -> overriding aorta

Question 5

TOF: what causes profound cyanosis?

Answer 5

Closing of PDA

Question 6

How does TOF appear on CXR?

Answer 6

Boot-shaped

Question 7

What causes “tet” spells

Answer 7

Coughing, crying, BM

Question 8

What confirms dx of TOF?

Answer 8

Echo

Question 9

Treatment/management of TOF

Answer 9

Supportive care/O2 prn
Prostaglandin E1
Surgery

Question 10

What do prostaglandins do

Answer 10

Maintain PDA

Question 11

Anatomical changes in transposition of great vessels (TGV)

Answer 11

Reversal of the origin of the aorta and the pulmonary artery, R ventricle -> aorta and L ventricle -> pulmonary artery

Question 12

TGV: venous and arterial blood mixes through

Answer 12

ASD
VSD
PDA

Question 13

TGV: when does cyanosis and hypoxia occur?

Answer 13

During first week of life as PDA closes

Question 14

TGV: CXR

Answer 14

Egg-shaped

Question 15

TGV: confirmation

Answer 15

Echo

Question 16

TGV: treatment

Answer 16

Prostaglandins
Supportive care, O2 prn (decrease FiO2 if baby gets worse)
Balloon arterial septostomy (rashkind procedure) to create shunt for mixing
Surgery

Question 17

What is coarctation of the aorta?

Answer 17

Severe narrowing of the aorta, causing decreased blood flow through aorta

Question 18

Preductal coarctation of aorta

Answer 18

Narrowing occurs before PDA, results in R to L shunt

Question 19

Postductal coarctation of aorta

Answer 19

Narrowing occurs after PDA, results in L to R shunt

Question 20

Why is measuring BP in all four extremities when coarctation of aorta is suspected?

Answer 20

BP is higher in upper extremities

Question 21

Coarctation of aorta: treatment

Answer 21

Supportive care with digitalis for heart failure
Prostaglandins
Surgery for resection of coarctation

Question 22

Anatomical changes in truncus arteriosus

Answer 22

Single common vessel for both the aorta and pulmonary artery over VSD

Question 23

Blood flow in truncus arteriosus

Answer 23

Systemic and pulmonary circulations delivered through same ventricle

Question 24

Truncus arteriosus treatment

Answer 24

Be conservative with oxygen admin to keep PVR high
Rule of fourties’
Pulmonary artery banding
VSD closure
Surgery

Question 25

What is the rules of 40’s?

Answer 25

PaCO2: 40 PaO2: 40 SaO2: 75% Balances systemic and pulmonary flow

Question 26

Anatomical changes in hypoplastic left heart syndrome

Answer 26

Severely underdeveloped L ventricle with stenosis or atresia of mitral valve Dependent upon right ventricle for blood supply to both circulations

Question 27

Techniques to balance pulmonary and systemic vascular resistance in hypoplastic left heart syndrome

Answer 27

Withhold oxygen therapy, subambient O2 (17-20%) Target SpO2: 70-80% Rule of 40’s

Question 28

Hypoplastic left heart syndrome: treatment

Answer 28

Prostaglandins Subambient FiO2 Hypercarbic gas mixtures Complex surgical repair

Question 29

Total anomalous pulmonary venous return (TAPVR) anatomical changes

Answer 29

Pulmonary veins fail to connect to left atria, may connect to superior or inferior vena cava

Question 30

TAPVR: what two things are crucial for survival?

Answer 30

ASD PDA

Question 31

TAPVR: CXR

Answer 31

Snowman shaped heart

Question 32

TAPVR: treatment

Answer 32

Prostaglandins Balloon septostomy (ASD) Surgery

Question 33

atrial septal defect: anatomical changes

Answer 33

Failure of the foremen ovale to close

Question 34

Cause of R heart failure in ASD

Answer 34

Increased workload of R heart

Question 35

ASD: presentation in newborn

Answer 35

Initially healthy, failure to thrive Difficulty feeding Turns blue after crying, feeding, burping, etc (increased intrathoracic pressure)

Question 36

ASD: treatment

Answer 36

Surgery Supportive oxygen prn

Question 37

ventricular septal defect: anatomical changes

Answer 37

Opening in septum between ventricles (most common)

Question 38

How are small VSDs treated?

Answer 38

Spontaneous Oxygen Diuretics Digoxin

Question 39

How are large VSDs treated?

Answer 39

Surgery to patch pulmonary artery banding

Question 40

Why does blood shunt from the aorta into the pulmonary circulation (PDA)

Answer 40

Left to right, if left heart pressures are higher than right heart pressures

Question 41

How is a pt evaluated for a PDA?

Answer 41

Pre- and post-ductal studies (PaO2 10-15 higher in right hand vs left) (15% if SpO2)

Question 42

How is a newborn with a PDA treated?

Answer 42

Indomethacin (indocin) or ibuprofen (prostaglandin inhibitors) O2 therapy Fluid restriction and diuretics Surgery

Question 43

Atrioventricular septal defect (ASVD) other terms

Answer 43

Atrial-ventricular canal or endocardial cushion defect

Question 44

ASVD: anatomical changes

Answer 44

Absence of atrial and ventricular septum

Question 45

ASVD results in

Answer 45

Intra-atrial and intra-ventricular shunting, and atrio-ventricular regurgitation

Question 46

ASVD most common in

Answer 46

Trisomy 21 (down syndrome)

Question 47

ASVD: treatment

Answer 47

Children without CHF: diuretics and digoxin SpO2 75-90% Careful oxygen administration Surgery

Question 48

ASVD: higher SpO2 and oxygen results in

Answer 48

Reduced PVR and increased pulmonary blood flow (bad)