NBME 10. 50Q SLE + AV block 02-17 (1)

Question 1

A 30-year-old primigravid woman with systemic lupus erythematosus comes to the physician for a prenatal visit at 34 weeks’ gestation. Examination shows that her disease is in remission. She is concerned about the possible effects of her disease on her fetus. The fetus is at greatest risk for which of the following?

Answer 1

Complete atrioventricular block

Question 2

Congenital atrioventricular (AV) block is a rare complication that can affect children born to mothers with…. 2 diseases?

Answer 2

systemic lupus erythematosus (SLE) or Sjögren syndrome.

Question 3

Congenital atrioventricular (AV) block is assoc. with what? mechanism

Answer 3

The condition is associated with the presence of anti-Ro and anti-La antibodies during pregnancy which cross the placenta and interfere with normal formation of the fetal AV node and surrounding myocardium.

Question 4

Congenital atrioventricular (AV) block. when antibodies bind AV block, what pathomechanism happens?

Answer 4

Improper necrosis caused by binding of autoantibodies with cardiomyocytes results in replacement of normal myocardium with fibrous tissue.

Question 5

In addition to the presence of autoantibodies, congenital AV block can be caused by structural heart abnormalities from other types of congenital heart disease or may be idiopathic.

Answer 5

.

Question 6

Patients may develop first-, second-, or third-degree AV block, with a significant risk for mortality in infants with third-degree AV block.

Answer 6

Clinical manifestations vary depending on the severity of block; most cases are diagnosed in the perinatal period or by early childhood

Question 7

Congenital AV block caused by maternal autoantibodies most often results in third- degree, or complete, fetal AV block.

Answer 7

.

Question 8

Choice A. Atrial septal defect. assoc with what? 3

Answer 8

fetal alcohol syndrome, Down syndrome, and trisomy 13 syndrome.

Question 9

Choice A. Atrial septal defect may occur in isolation or in combination with other congenital heart disease syndromes.

Answer 9

.

Question 10

(Choice C) Endocarditis is extremely rare in the fetal and neonatal periods

Answer 10

.

Question 11

(Choice D) most common form of fetal tachyarrhythmia ?????

Answer 11

Supraventricular tachycardia

It may affect fetuses with and without congenital heart disease.

Question 12

Choice E) most common CONGENITAL heart disease?

Answer 12

Ventricular septal defect

Question 13

(Choice E) Ventricular septal defect assoc with what? 4

Answer 13

Down syndrome, thymic aplasia (DiGeorge syndrome), Turner syndrome, and tetralogy of Fallot.