Congenital Heart Defects

1.)  You are the NNP on call and receive a phone call at 2 a.m. regarding Baby Boy M who is now 24 hours old.  Baby boy M has become tachypneic and the newborn nursery personnel report him as appearing "blue" at times.  The infant has a pulse ox reading of 85% on room air.  The pediatrician is requesting an in-house transfer to the NICU.  Upon arrival to the NICU, you note that Baby Boy M is cyanotic and the pulse ox reading is fluctuating between 80-85% on room air and his respiratory rate is 60-80 per minute.  He is placed on high flow nasal cannula @50% with no noted improvement in O2 saturations.  Among ordering a chest x-ray what other initial diagnostic test could be ordered to assist in making a respiratory vs. cardiac diagnosis?

A.)  Echocardiogram

B.)  Hyperoxia test

C.)  EKG

D.)  Cardiac catheterization

2.)  Baby Boy M's chest xray reveals lung fields which appear normal, but the heart has an "egg-on-a-string" appearance.  Which cyanotic heart defect would this be most indicative of?

A.)  Tetralogy of Fallot

B.)  Truncus Arteriosus

C.)  d-Transposition of the great arteries

D.)  Aortopulmonary window

3.)  As the NNP caring for an infant with a presumed cyanotic heart defect, you order an echocardiogram to confirm the diagnosis and plan to immediately initiate which medication to maintain ductal patency?

A.)  Indomethacin

B.)  Ibuprofen

C.)  Dexamethasone

D.)  Prostaglandin E₁

4.)  Baby Girl F is a 35 week neonate born to a G2P1 mother.  The mother is of advanced maternal age and Baby Girl F was diagnosed in-utero as having Down's Syndrome.  Of the congenital heart defects, which is the most common defect in neonates with Down's Syndrome?

A.)  AV-canal defect/Endocardial cushion defect

B.)  L-Transposition of the great arteries

C.)  Ebstein's anomoly

D.)  Hypoplastic Left Heart Syndrome

5.)  Complete the mnemonic below which indicates the four defects associated with Tetraology of Fallot:

D________________

R________________

O________________

P________________

6.)  Baby boy B is admitted to the NICU at 36 hours of age for cyanosis and respiratory distress.  Diagnostic tests performed after admission reveal a diagnosis of Truncus Arteriosus.  As the NNP caring for Baby boy B, you realize that infant's who have a confirmed diagnosis of truncus arteriosus must also be screened for:

A.)  Duodenal atresia

B.)  Chromosome 22 microdeletion

C.)  Hirschsprungs

D.)  ABO incompatibility

7.)  Baby Boy B becomes tachypneic and begins to exhibit additional signs of symptoms of congestive heart failure.  You realize congestive heart failure may accompany truncus arteriosus and order additional medications?

A.)  Phenobarb

B.)  Lasix

C.)  Ibuprofen

D.)  Digoxin

E.)  Both B and C

F.)  Both B and D

G.)  None of the above

8.)  Baby Girl D is a former 27 week neonate now on day of life 3.  Baby Girl D has been successfully weaning from ventilator support, but over the past few hours, she has required a noticeable increase in her ventilator settings.  A likely reasoning for the noted change in respiratory status includes:

A.)  Transient tachypnea of the newborn

B.)  Diaphragmatic hernia

C.)  Patent ductus arteriosus 

9.)  Based upon your response to Baby Girl D's worsening respiratory status, what would be your next step once confirmation of your diagnosis is made?

A.)  Order breathing treatments of Albuterol q 6hrs

B.)  Maintain current ventilator settings and consult surgery immediately

C.)  Order Indomethacin 0.2mg/kg IV q 12 hrs x 3

10.) Baby Girl D's status continues to deteriorate despite interventions.  What would be your next step in managing Baby Girl D and why?

A.)  Continue breathing treatments and order diuretics in hopes of preventing broncopulmonary dysplasia

B.)  Consider transferring infant to another facility because the surgeon on call is on vacation

C.)  Consider consultation for PDA ligation.  Your rationale for expediting PDA closure includes prevention of Eisenmenger's syndrome