"Case Study: Congenital Heart Defects" by Patricia Lincoln, RN, MS, CCRN, CNS-BC, for OPENPediatrics

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Question 1:
James is a four week old diagnosed at birth with Tetralogy of Fallot. He was scheduled for surgery in 2 weeks, but because of hypercyanotic spells he is admitted to the intensive care unit from the emergency room.
As James’ nurse, you know that Tetralogy of Fallot is a combination of which four defects?

The correct answer is: Ventricular septal defect, pulmonary stenosis, overriding aorta, right ventricular hypertrophy.

Question 2:
The hypercyanotic spells that James is experiencing are primarily related to:

The correct answer is: Pulmonary stenosis. The greater the amount of obstruction in the pulmonary outflow tract or the greater the degree of pulmonary stenosis the more cyanotic the patient will be. Because blood is unable to flow from the right side of the heart to the lungs to receive oxygenation the blood will shunt right to left across the Ventricular Septal Defect and return to body without being oxygenated.

Question 3
What causes hypercyanotic spells?

The correct answer is: an increased oxygen requirement together with increased resistance to pulmonary flow.

Question 4:
If James were to experience a hypercyanotic spell while in your care, your first intervention would be to:

The correct answer is: Place James in a knee-chest position and administer oxygen.

Question 5:
Other clinical signs and symptoms of Tetralogy of Fallot include:

The correct answer is: Chest radiograph showing decreased pulmonary markings, electrocardiogram with right ventricular hypertrophy, systolic ejection murmur.
Question 6:
James undergoes surgical correction of his Tetralogy of Fallot, including a ventricular septal defect closure and right ventricular outflow tract patch.
Intraoperatively, a transthoracic pulmonary artery catheter is placed.
Before James had his pulmonary artery line removed, right atrial and pulmonary artery saturations were measured. The right atrial saturation = 72, and the pulmonary artery saturation = 95.
What might this indicate?

The correct answer is: A residual ventricular septal defect.

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