Deck 12: Cyanotic Heart Defects

Question 1

What Congenital heart diseases are associated with an Ebstein anomaly
I)Hypoplastic left heart syndrome
II)Ventricular septal defect
III)Patent foramen ovale
IV)Atrial septal defect
V)Truncus arteriosus

A)I, II, and III
B)II, III, and IV
C)III, IV, and V
D)II, III, and IV

Answer 1

D

Question 2

What are the clinical signs of total anomalous pulmonary venous return
I)Elevated T-waves on electrocardiogram
II)Right ventricular heave
III)Periods of hypercyanosis
IV)Left ventricular hypertrophy
V)Snowman cardiac silhouette
VI)A fixed split S2

A)I, II, III, IV, and V
B)II, IV, and V
C)I, II, V, and VI
D)I, III, V, and VI

Answer 2

C

Question 3

If an infant with transposition of the great arteries is presenting with severe cyanosis, what life-saving procedure should be immediately performed at the patient's bedside

A)Endotracheal tube placement
B)Rashkind procedure
C)Echocardiogram
D)None of the above

Answer 3

B

Question 4

What percentage of patients with hypoplastic left heart syndrome die prior to stage 2 repair

A)10%
B)20%
C)30%
D)40%

Question 5

What is the appropriate PaCO₂ range needed to achieve a high pulmonary vascular resistance and maintain an appropriate systemic flow for a patient with hypoplastic left heart syndrome

A)25-30 mm Hg
B)35-45 mm Hg
C)40-50 mm Hg
D)30-40 mm Hg

Question 6

What percentage of patients with Ebstein anomaly will present with arrhythmias during their lifetime

A)30%-35%
B)25%-30%
C)20%-40%
D)20%-30%

Question 7

Surgical intervention is not indicated in patients with truncus arteriosus.

Question 8

Which of the following statements are true regarding transposition of the great arteries (TGA)
I)The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle.
II)The aorta arises from the left ventricle and the pulmonary artery arises from the right ventricle.
III)TGA is fatal without treatment.
IV)TGA occurs more often in males than females.

A)I, III, and IV
B)I, II, and III
C)II, III, and IV
D)I, II, and IV

Question 9

Successful management of hypoplastic left heart syndrome requires an understanding of the delicate balance between pulmonary blood flow and systemic blood flow.

Question 10

What pulse oximetry range should be maintained by the registered respiratory therapist for a patient with hypoplastic left heart syndrome until after stage 3 repair

A)SpO₂ 80%-90%
B)SpO₂ 65%-75%
C)SpO₂ 75%-85%
D)SpO₂ greater than or equal to 92%

Question 11

The physical examination of transposition of the great arteries includes all of the following except:

A)No cardiac murmur detected
B)Electrocardiogram normal
C)Chest radiograph normal
D)Slow progressive cyanosis

Question 12

What forms of treatment can be utilized in the management of hypoplastic left heart syndrome
I)Norwood procedure
II)Sano modification
III)Bidirectional Glenn procedure
IV)Fontan procedure
V)Heart transplant

A)I, III, and IV
B)I, II, III, and V
C)I, II, III, IV, and V
D)II, III, and IV

Question 13

Tetralogy of Fallot includes what four defects
I)Ventricular septal defect
II)Arterial septal defect
III)Aorta that overrides the ventricular septal defect
IV)Obstruction of the left ventricle outflow tract
V)Obstruction of the right ventricle outflow tract
VI)Right ventricular hypertrophy
VII)Left ventricular hypertrophy

A)I, III, V, and VI
B)II, IV, V, and VII
C)III, IV, VI, and VII
D)I, II, IV, and VI

Question 14

What is deemed the industry standard for diagnosis and differentiation of Congenital cardiac malformation

A)MRI
B)CT scan
C)Positron emission tomography
D)Echocardiography

Question 15

Hypoplastic left heart syndrome is an example of what larger category of Congenital cardiac defects

A)Modified Blalock-Taussig shunt
B)Ebstein anomaly
C)Single ventricle syndrome
D)Patent foramen ovale

Question 16

What is the mortality rate for infants with tetralogy of Fallot

A)Less than 10%
B)Greater than 25%
C)Less than 30%
D)Greater than 50%

Question 17

What tests need to be performed to decipher whether a patient's hypoxemia is related to lung disease or Congenital heart disease
I)Chest radiograph
II)EKG
III)Hyperoxia test
IV)Pulmonary function test
V)Echocardiogram
VI)Cardiac auscultation

A)II, III, V, and VI
B)I, II, IV, V, and VI
C)II, III, IV, V, and VI
D)I, II, III, V, and VI

Question 18

Total anomalous pulmonary venous return is a life-threatening problem because:

A)There is no blood delivery into the right side of the heart.
B)It presents with a Congenital long QT syndrome on electrocardiogram
C)There is no blood delivery into the left side of the heart.
D)It presents with a Congenital short QT syndrome on electrocardiogram

Question 19

What will happen to a patient with total anomalous pulmonary venous return if administered inhaled nitric oxide
I)Improved cardiac output
II)Constricted pulmonary vasculature
III)Worsened cardiac output
IV)Dilated pulmonary vasculature

A)I and II
B)III and IV
C)I and IV
D)II and III

Question 20

A patient diagnosed with an Ebstein anomaly will present with which of the following findings on physical examination
I)Saturation less than 85% in patients who are older children or are adults
II)Cyanosis
III)Hepatomegaly
IV)Jugular vein distention
V)Dyspnea on exertion

A)I, III, IV, and V
B)I, II, III, IV, and V
C)I, III, IV, and V
D)II, III, IV, and V