Congenital Heart Disease

Question 1

Genetic disorder associated with coarctation of the aorta

Answer 1

Turner Syndrome

Question 2

Genetic disorder associated with aortic insufficiency and dissection

Answer 2

Marfan’s syndrome

Question 3

Genetic disorder associated with septal and endocardial cushion defects

Answer 3

Down syndrome

Question 4

Genetic disorder associated with truncus arteriosus and tetralogy of Fallot

Answer 4

22q11

Question 5

Cardiac structure/defect that allows for alteration in normal blood flow; typically beneficial for fetuses, but can be detrimental in babies/adults

Answer 5

Shunts

Question 6

Two general categories for congenital heart defects

Answer 6

Cyanotic vs. Acyanotic

Question 7

Acyanotic congenital heart defects usually involve blood flowing in what direction (left vs. right)

Answer 7

Left to right (goes with pressure gradients)

Question 8

Cyanotic congenital heart defects usually involve blood flowing in what direction (left vs. right)

Answer 8

Right to left (deoxygenated blood bypasses lungs)

Question 9

Acyanotic Congential Heart Defects (6 total)

Answer 9

Atrial/Ventricular Septal defect
Patent Ductus Arteriosus
Congenital Aortic Stenosis
Pulmonic Stenosis
Coarctation of the aorta

Question 10

Cyanotic Congenital Heart Defects (5 T’s)

Answer 10

Truncus Arteriosus
Transposition of great vessels
Tricuspid Atresia
Tetralogy of Fallot
TAPVR (Total Anomalous Pulmonary Venous Return)

Question 11

Defect that allows blood flow between left to right atria

Answer 11

Atrial septal defect (acyanotic)

Question 12

Most common atrial septal defect

Answer 12

Ostium Secundum

Question 13

Atrial Septal Defect can cause a volume overload in what heart chamber?

Answer 13

Right Ventricle (leads to enlargement and dec. compliance over time)

Question 14

Symptoms of ASD and VSD

Answer 14

Dyspnea
Fatigue
Recurrent resp. infections (pulmonary overcirculation)
Atrial tachyarrhythmias (enlarged RA)

Question 15

Murmur in ASD is due to…

Answer 15

increased volume load

Question 16

Defect that allows blood flow between left to right ventricles

Answer 16

Ventricular septal defect (acyanotic)

Question 17

Most ventricular septal defects are (membranous/muscular)

Answer 17

Membranous (70%)

Question 18

Restrictive vs. Nonrestrictive defects

Answer 18

Restrictive: defect size determines flow rate
Nonrestrictive: pressure gradients determine flow rate

Question 19

Persistent connection between the great vessels (flow from ascending aorta to pulmonary artery); can lead to LV dysfunction due to volume overload

Answer 19

Patent Ductus Arteriosus (acyanotic)

Question 20

How can Patent Ductus Arteriosus eventually lead to LV dysfunction

Answer 20

Left-sided volume overload

Question 21

Symptoms of Patent Ductus Arteriosus

Answer 21

CHF
Recurrent resp. infections
Enlarged cardiac silhouette (Left-sided enlargement)

Question 22

Prostaglandin synthesis inhibitor for Persistent Ductus Arteriosus

Answer 22

Indomethacin

Question 23

Treatment options for Patent Ductus Arteriosus

Answer 23

Indomethacin (prostaglandin inhibitor; FIRST LINE)

Cath/surgical closure

Question 24

Uncorrected septal defects or PDA can cause pulmonary vascular hypertrophy due to pulmonary overload; increased pulmonary afterload can cause shunt reversal; can cause cyanosis, clubbing and polycythemia (but NOT acyanotic lesion)

Answer 24

Eisenmenger’s Syndrome

Question 25

Narrowed aortic valve orifice; requires lots of LV systolic pressure to pump blood; severe cases cause LVH; high velocity jet may impact proximal aorta and causes dilation of proximal aorta

Answer 25

Aortic Stenosis (acyanotic)

Question 26

Narrowed pulmonic valve orifice; requires lots of RV systolic pressure to pump blood; severe cases can cause RVH; valve fusion can cause obstruction

Answer 26

Pulmonic Stenosis (acyanotic)

Question 27

Discrete narrowing of aortic lumen; can be due to dec. flow from aortic stenosis or residual ductal tissue; may have discrepancy between upper and lower extremities

Answer 27

Coarctation

Question 28

Which congenital heart disease can result in discrepancy between upper and lower extremities

Answer 28

Coarctation (reduced flow to descending aorta)

Question 29

Failure of division of aorta and pulmonary trunk; most have accompanying VSD; mixing of oxygenated/deoxygenated blood

Answer 29

Persistent Truncus Arteriosus (cyanotic)

Question 30

failure of aorticopulmonary septum to spiral; great vessels arise from wrong ventricles; aorta leaves RV and pulmonary trunk leaves LV; 2 parallel circuits

Answer 30

Transposition of Great Arteries (cyanotic)

Question 31

Most common cause of neonatal cyanosis

Answer 31

Transposition of Great Arteries

Question 32

Why might a shunt help those with Transposition of Great Arteries?

Answer 32

Shunt allows mixing of blood, allowing adequate systemic oxygenation

Question 33

Absent tricuspid valve and hypoplastic RV; requires BOTH ASD and VSD for viability because RA is completely closed off from the RV

Answer 33

Tricuspid atresia

Question 34

most common form of cyanotic congenital heart disease VSD, Pulmonic stenosis, RVH and overriding aorta

Answer 34

Tetralogy of Fallot (cyanotic)

Question 35

Why does Tetralogy of Fallot cause right to left ventricular flow?

Answer 35

Pulmonic stenosis causes resistance to RVOT flow leading to RVH and increase in RV systolic pressure. This leads to R to L shunt causing cyanosis.

Question 36

Why might exertion (feeding & crying) cause a "Tet Spell" in those with Tetralogy of Fallot?

Answer 36

Feeding and crying cause higher contractile force which leads to increase in RV pressure. This worsens right to left shunting, bypassing lungs and worsening cyanosis.

Question 37

Pulmonary veins drain into right heart circulation; sometimes associated with ASD to maintain CO

Answer 37

Total Anomalous Pulmonary Venous Return (TAPVR) (cyanotic)

Question 38

Congenital heart defects are usually well tolerated at birth (True or False)

Answer 38

True (change in left-sided pressures after birth brings out true colors)

Question 39

Disorder associated with septal defects, PDA, pulmonary artery stenosis

Answer 39

Congential rubella

Question 40

Disorder associated with transposition of great vessels

Answer 40

infant of diabetic mother

Question 41

What 4 congenital abnormalities make up tetralogy of fallot?

Answer 41

VSD Pulmonic stenosis RVH Overriding aorta

Question 42

What is a tet spell?

Answer 42

episodes of sudden cyanosis during exertion (crying & feeding)