Nokea
An 8-year-old from the former Soviet Union is brought to your office because of a heart murmur and dusty blue skin. He has marked clubbing of his fingers and toes, and his hematocrit is 70%. He has a grade 4/6 holosystolic murmur. His chest x-ray reveals a normal pulmonary blood flow as well as a right-sided aortic arch. What is the likely diagnosis?
- A. total anomalous venous return
- B. tetralogy of Fallot
- C. Eisenmenger syndrome
- D. transposition of the great vessels
Correct Answer: C
Rationale: Eisenmenger syndrome involves reversal of shunt direction due to long-standing pulmonary hypertension, explaining the cyanosis and clubbing.
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José is a 4-year-old child scheduled for a cardiac catheterization. What should be included in preoperative teaching?
- A. Directed at his parents because he is too young to understand
- B. Detailed in regard to the actual procedures so he will know what to expect
- C. Done several days before the procedure so that he will be prepared
- D. Adapted to his level of development so that he can understand
Correct Answer: D
Rationale: Preoperative teaching should always be directed at the child’s stage of development. The caregivers also benefit from the same explanations. The parents may ask additional questions, which should be answered, but the child needs to receive the information based on developmental level. Preschoolers will not understand in-depth descriptions and should be prepared close to the time of the cardiac catheterization.
List FIVE criteria for the diagnosis of incomplete Kawasaki Disease.
- A. Fever for ≥7 days
- B. Five days of fever with 2 or 3 compatible clinical criteria
- C. CRP ≥3.0mg/dL and/or ESR ≥40mm/h
- D. All of the above
Correct Answer: D
Rationale: The diagnosis of incomplete Kawasaki Disease requires a combination of clinical, laboratory, and echocardiographic findings.
Systolic murmur in which among the following conditions does not increase in intensity in the cardiac cycle after a premature ventricular contraction?
- A. Valvular aortic stenosis
- B. Hypertrophic obstructive cardiomyopathy
- C. Valvular pulmonic stenosis
- D. Mitral valve prolapse
Correct Answer: D
Rationale: Mitral valve prolapse does not typically increase in intensity after a premature ventricular contraction.
Rocker bottom feet and polyvalvular dysplasia are characteristic of which of the following?
- A. Trisomy 19
- B. Trisomy 18
- C. Trisomy 13
- D. Trisomy 21
Correct Answer: B
Rationale: Rocker bottom feet and polyvalvular dysplasia are classic signs of trisomy 18 (Edwards syndrome), a condition that involves multiple congenital anomalies.
Defects of complement system should be suspected in:
- A. Recurrent folliculitis
- B. Facial swelling following trauma
- C. Recurrent meningococcal infection
- D. Pneumocystis carinii pneumonia
Correct Answer: C
Rationale: Recurrent meningococcal infection: Defects in the complement system, especially the terminal complement components, increase the risk of recurrent meningococcal infections.