Congenital heart disease 2

Question 1

During a surgical repair of TOF, the patient’s blood pressure declines by 25%, and the SpO2 decreases by 10%. What are the MOST likely explanations for these findings? (select 2)
a. pulmonary vascular resistance decreased
b. systemic vascular resistance decreased
c. myocardial contractility increased
d. preload increased

Answer 1

b. systemic vascular resistance decreased
c. myocardial contractility increased

Question 2

Tetralogy of Fallot is characterized by the following four defects:

Answer 2

ventricular septal defect
aorta that overrides the RV and LV
pulmonic stenosis (obstruction to RV ejection)
RV hypertrophy

Question 3

What is the most common cyanotic congenital heart anomaly?

Answer 3

Tetralogy of Fallot

Question 4

A “tet spell” presents as

Answer 4

hypoxemia and cyanosis

Question 5

With TOF, the best induction agent is

Answer 5

ketamine

Question 6

With TOF< the drugs to avoid include

Answer 6

morphine, meperidine, and atracurium (histamine release)

Question 7

A “tet spell” is precipitated by

Answer 7

increased sympathetic activity such as crying, agitation, pain, defecation, fright or trauma

Question 8

Why might the child experiencing a tet spell assume the squatting position?

Answer 8

it increases intraabdominal pressure and compresses the abdominal arteries which increases RV preload, SVR, and blood flow through the RVOT

Question 9

Perioperative Tet spell treatment includes

Answer 9

FiO2 100%
fluids to expand intravascular volume
increase SVR with phenylephrine
Reduce SNS stimulation- deepen anesthesia, short-acting beta-blocker
avoid inotropes- can worsen RVOT obstruction
avoid excessive airway pressure
place the infant in a knee-chest position to mimic squatting

Question 10

What are the hemodynamic goals of tetralogy of Fallot:

Answer 10

increase SVR- phenylephrine
decrease PVR- nitric oxide
maintain contractility and heart rate- esmolol
increase preload- crystalloid

Question 11

Why are some patients with tetralogy of Fallot polycythemic?

Answer 11

chronic hypoxemia stimulates increased RBC production

Question 12

Failure of the fossa ovalis to close results in what type of atrial septal defect?
a. primum
b. secundum
c. sinus venosus
d. perimembranous

Answer 12

b. secundum

Question 13

The most common type of ventricular septal defect is the

Answer 13

perimembranous VSD

Question 14

The most common congenital cardiac anomaly in children is

Answer 14

a ventricular septal defect

Question 15

The most common site of atrial septal defect is

Answer 15

fossa ovalis

Question 16

Flow through an atrial septal defect is typically

Answer 16

left-to-right (acyanotic)

Question 17

The most common site of ventricular septal defect is the

Answer 17

ventricular septum

Question 18

Flow through the VSD is typically

Answer 18

left-to-right (acyanotic)

Question 19

What situations should be avoided in a VSD?

Answer 19

situations that decrease PVR or increase SVR b/c they can increase shunt flow

Question 20

The physiologic consequence of the VSD is a function of_______________

Answer 20

the pressure gradients between the RV and LV which depend on PVR and SVR

Question 21

Early signs of an ASD include

Answer 21

poor exercise tolerance

Question 22

Late signs of an ASD include

Answer 22

atrial flutter
atrial fibrillation
CHF

Question 23

______________ can cause paradoxical embolism during Valsalva-like maneuvers if ____________

Answer 23

ASDs; RAP>LAP

Question 24

Describe antibiotic prophylaxis with ASD & VSD?

Answer 24

not indicated for an isolated ASD but it’s indicated within six months of surgical repair

Question 25

VSD is associated with the following conditions:

Answer 25

trisomy 13, 18, and 21
VACTERL
CHARGE

Question 26

The primary concern of a VSD is the

Answer 26

development of a left-to-right shunt

Question 27

VSD can cause ________ during valsalva-like maneuvers if RAP>LAP

Answer 27

paradoxical embolism

Question 28

Most VSDs close by the age of:

Answer 28

2

Question 29

In adults, the most common congenital defect is

Answer 29

bicuspid aortic valve

Question 30

A patient is undergoing surgical repair for coarctation of the aorta. Select the BEST site to monitor the arterial blood pressure.
a. right arm
b. right leg
c. left arm
d. left leg

Answer 30

a. right arm

Question 31

Coarctation of the aorta is the

Answer 31

narrowing of the thoracic aortic lumen

Question 32

Where can coarctation of the aorta occur?

Answer 32

typically before or after the ductus arteriosus but in rare instances, proximal to the left subclavian artery

Question 33

______________ usually goes unnoticed for years

Answer 33

Mild to moderate coarctation

Question 34

Obstruction of blood flow at the level of the coarctation leads to

Answer 34

increased LV afterload

Question 35

Describe systolic blood pressure in coarctation of the aorta.

Answer 35

SBP is elevated in the upper extremities
SBP is reduced in the lower exprtemities

Question 36

Severe obstruction of the aorta presents

Answer 36

very early in life

Question 37

Severe obstruction of coarctation of the aorta

Answer 37

requires prostaglandin E1 to keep the PDA open until surgery can be performed because it relies on a patent ductus arteriosus

Question 38

_____________ is strongly associated with coarctation of the aorta

Answer 38

Turner syndrome

Question 39

If the coarctation occurs proximal to the left subclavian artery takeoff, then the

Answer 39

SBP in the RUE> then the SBP in the LUE

Question 40

Severe coarctation of the aorta in the neonate can present as

Answer 40

pink, well-perfused upper body and blue poorly-perfused lower body

Question 41

Indications for surgical repair in the patient with mild to moderate coarctation of the aorta includes

Answer 41

exercise intolerance
chest pain
headaches
lower extremity claudication

Question 42

Diagnosis of mild to moderate coarctation of the aorta may be delayed until adulthood when the patient presents with

Answer 42

secondary hypertension

Question 43

Preductal coarctation is commonly found in

Answer 43

infants

Question 44

Postductal coarctation is commonly found in

Answer 44

adults