Congenital Heart Disease

Question 1

What are the three major groups of Congenital Heart Disease?

Answer 1

• left-to-right shunts, right-to-left shunts, and obstruction

Question 2

What occurs in a Right-to-Left Shunt?

Answer 2

• the pulmonary circulation is decreased/bypasses, so deoxygenated blood enters the systemic circulation

Question 3

Four main Right-to-Left Shunt defects

Answer 3

• tetralogy of Fallot, transposition of the great vessels, truncus arteriosus, and tricuspid atresia

Question 4

What occurs in a Left-to-Right Shunt?

Answer 4

• systemic blood inappropriately enters the pulmonary circulation, increasing the pressure in the pulmonary circulation and eventually causing RV hypertrophy and failure

Question 5

Three main Left-to-Right Shunt defects

Answer 5

• atrial septal defect (ASD), ventricular septal defect (VSD), and patent ductus arteriosus (PDA)

Question 6

Which type of shunt is more common? What is the most common congenital heart defect?

Answer 6

• left to right shunts are more common

- VSDs are the most common

Question 7

Atresia

Answer 7

• complete obstruction of a chamber, valve, or vessel from a congenital malformation

Question 8

T or F: patients with Right-to-Left shunts will usually present with immediate cyanosis.

Answer 8

• true!
• these patients have deoxy blood entering their systemic circulation
• (in contrast, left-to-right shunts don’t immediately cause cyanosis, as blood still enters the pulmonary circulation)

Question 9

Eisenmenger’s Syndrome

Answer 9

• cyanosis that eventually occurs in left-to-right shunts
• the RV hypertrophy resulting from increased pulmonary volume and pressure eventually causes the pressure in the right side of the heart to exceed that of the left side
• the shunt is now a right-to-left shunt and cyanosis will occur

Question 10

In an infant with aortic or pulmonary atresia, why is PGE given?

Answer 10

• PGE = prostaglandin-E; it keeps the ductus arteriosus open
• we want it to stay open because it is the only thing that is maintaining systemic/pulmonary circulation due to the atresia
• (PGE is given to keep it open until surgery can be done)

Question 11

Tetralogy of Fallot

Answer 11

• P.R.O.Ve.
• Pulmonary stenosis, RV hypertrophy, Overriding aorta, Ventricular septal defect
• these four defects result in a right-to-left shunt, where blood flows from the RV straight into the aorta

Question 12

Transposition of the Great Vessels

Answer 12

• a right-to-left shunt
• the RV pumps blood into the aorta
• the LV pumps blood into the pulmonary trunk
• creates two separate circulations that do not interact

Question 13

Infantile Aortic Coarctation

Answer 13

• an obstructive congenital heart defect
• hypoplasia of the aortic arch results in a narrowing of the vessel, restricting blood flow through the area
• LV hypertrophy will result from the increased resistance to blood flow; dilation will occur proximal to the coarctation
• HTN in upper extremities, hypotension in lower

Question 14

Which septal defect can result in paradoxical emboli?

Answer 14

• ASD

- allows venous emboli to end up in the systemic system

Question 15

Which defect is associated with Fetal Alcohol Syndrome?

Answer 15

• VSD

Question 16

Which defect is associated with Down Syndrome?

Answer 16

• ASD

Question 17

Which defect is associated with Congenital Rubella?

Answer 17

• PDA

Question 18

____ keeps the Ductus Arteriosus open, while ______ closes it.

Answer 18

• PGE keeps it open

- indomethacin closes it

Question 19

T or F: Eisenmenger’s Syndrome in PDA will result in cyanosis.

Answer 19

• true!
• however, it will only result in cyanosis of the lower extremities, as the PDA occurs after the main branches of the aorta supplying the upper extremities

Question 20

What type of murmur is associated with PDA?

Answer 20

• a machine-like murmur

Question 21

Why do patients with Tetralogy of Fallot squat?

Answer 21

• squatting increases systemic resistance, increasing the afterload, and increasing the pressure on the aorta/left side of the heart, allowing more blood to enter the now more easily accessible pulmonary trunk (so that the blood can get oxygenated)

Question 22

Which defect is associated with Maternal Diabetes?

Answer 22

• transposition of the great vessels

Question 23

Truncus Arteriosus

Answer 23

• a right-to-left shunt

- a single large vessel arising from both ventricles due to the truncus failing to divide

Question 24

Which defect is associated with Turner’s Syndrome?

Answer 24

• coarctation of the aorta