Cardiovascular- Pediatrics

Question 1

Tx: Closure of Patent ductus arterioles

Answer 1

Indomethacin

Question 2

Signs and symptoms of transient Hypertrophic cardiomyopathy

Answer 2

Infant of gestational dibetes
Mild Respiratory Distress
Systolic Ejection Murmur 
Interventricular septal wall thickening 
LVOT obstruction

Question 3

Management of transient HCM

Answer 3

Goal: increasing LV volume and CO
Tx: Iv fluids and Beta blockers

Transient HCM self resolves when insulin level normalize

Question 4

Causes of congenital long QT syndrome

Answer 4

Romano-Ward syndrome (AD)
Jervell & Lange Nielsen syndrome (AR)

Loss of function mutation on the KCNQ1 gene located on chromosome 11p → defective slow delayed rectifier voltage-gated potassium channel → affected repolarization phase

Question 5

Differentiate between the two congenital LQTS syndromes?

Answer 5

Romano-Ward syndrome (AD)- no associated deafness

Jervell & Lange Nielsen syndrome (AR)- associated senorinueaural deafness

Question 6

Complications of LQTS

Answer 6

Prolonged QT interval (eg, >460 ms) predisposes to the development:
Torsade de pointes (polymorphic ventricular tachycardia)
Syncope
Sudden cardiac death.

worse during periods of rapid heart rate and high sympathetic activity (eg, exercise)

Question 7

Management of congenital LQTS

Answer 7

nonselective beta blockers (propranolol, nadolol)

Implantable cardioverter defibrillator

Question 8

Beta Blocker mechanism for LQTS

Answer 8

Beta blockers (class II antiarrhythmics) dampen sympathetic activity and shorten the QT interval at rapid heart rates to reduce the risk of syncope and death in patients with congenital LQTS.

Question 9

Evaluation of newborn for congenital heart disease

Answer 9

Pre and post ductal pulse oximetry