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Review Question - QID 100552

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QID 100552 (Type "100552" in App Search)
A 4-year-old Caucasian male suffers from cyanosis and dyspnea relieved by squatting. Which of the following abnormalities is most likely present?

Left ventricular hypertrophy

5%

10/185

Atrial septal defect

9%

17/185

Ventricular septal defect

77%

142/185

Coarctation of the aorta

3%

5/185

Bicuspid aortic valve

4%

7/185

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The patient described is experiencing a "tet spell", characteristic of Tetrology of Fallot (TOF). By definition, TOF consists of 4 components: pulmonic stenosis, ventricular septal defect, right ventricular hypertrophy, and overriding aorta (straddling the VSD).

In "tet spells", patients with TOF present with cyanosis relieved by squatting. Cyanosis occurs because right-to-left shunting decreases blood flow to the lungs, while squatting provides relief by increasing total systemic vascular resistance, thereby decreasing the right-to-left shunt.

Incorrect Answers:
Answers 1, 2, 4, and 5: These congenital abnormalities are not commonly associated with TOF.

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