Cardiovascular System

Question 1

What cardiovascular defect is most commonly associated with Edward’s syndrome (micrognathia, microcephaly, rocker bottom feet, overlapping fingers & absent palmar creases)?

Answer 1

Ventricular septal defect

Edward’s syndrome is trisomy 18. 80% of affected children die in the first month. 90% die by year 1. The remaining 5-10% are mentally retarded.

Question 2

Treatment:

Patient with a history of rheumatic fever.

Answer 2

Intramuscular benzathine penicillin G every 4 weeks.

Continuous antibiotic prophylaxis to prevent recurrent group A Streptococcus pharyngitis and limit the progression to rheumatic heart disease.

Question 3

Congenital cardiac defects associated with Down syndrome:

Answer 3

1. 40% Complete atrioventricular septal defects (failed endocardial fusion fushion)
2. 30% Ventricular septal defect
3. 15% Atrial septal defect

Question 4

What is workup is required for a child who presents with breath-holding spells?

Answer 4

1. Evaluate for iron-deficiency anemia

Breath-holding spells are otherwise benign and no additional workup is necessary.

Question 5

Most common cause of sudden death in an otherwise healthy individual?

Answer 5

Hypertrophic cardiomyopathy (HCM).

Adolescents with a family history of sudden death in young relatives should refrain from sports until a ECG and echocardiograph are performed.

Question 6

Treatment:

Maintaining a patent ductus arteriosus

Answer 6

Prostaglandin E1

• Avoid excessive inspired oxygen and indomethacin as these interventions would constrict the ductus arteriosus.

Question 7

Diagnosis:

Tricuspid valve atresia on

• CXR
• ECG

Answer 7

• CXR: decreased pulmonary markings due to hypoplasia of the right ventricle and pulmonary outflow tract
• ECG: left axis deviation, peaked P waves, short-minimal R waves