Exam 2

Question 1

transposition of the great arteries (or vessels); TGA

Answer 1

Defect causing Mixed Blood Flow: switched aorta and pulmonary artery. no communication between lungs and body. Arterial Switch procedure performed as soon as possible after birth. give prostaglandin to keep PDA open until surgery

Question 2

total anomalous pulmonary venous return (TAPVR)

Answer 2

defect that cases mixed blood flow: pulmonary veins empty into R atrium instead of L atrium. Mild cyanosis increases with feedings. Small LA and LV; put on prostaglandins immediately to keep PDA open. Balloon septostomay until baby is old enough to withstand the Surgery the reconnects pulmonary veins to LA.

Question 3

Truncus Ateriorus

Answer 3

defect causing mixed blood flow. a single large vessel acts as both the aorta and pulmonary artery. VAD often present. Rastelli procedure closes VSD and creates passage to pulmonary arteries. repeated surgeries often needed. No contact sports.

Question 4

hypoplastic left heart syndrome (HLHS)

Answer 4

defect causing mixed blood flow. Very small LV and aorta. One of the most severe defects. Give Prostin to keep PDA open. NO SUPPLEMENTAL O2 to prevent pulmonary vasoconstriction. Treated with Norwood surgical procedure (RV pumps to lungs and body), transplant, or palliative care. limited physical activity due to single ventricle. Neurodevelopmental probs.

Question 5

ECMO

Answer 5

extra-corporeal membrane oxygenation: artificial heart and lung machine. outcomes decline if on this machine longer than two weeks.

Question 6

Indomethacin

Answer 6

closes PDA

Question 7

Prostin

Answer 7

keeps PDA open until surgery

Question 8

Congestive Heart Failure (CHF)

Answer 8

inability of heart to meet metabolic demands of body. Caused by increased workload or decreased myocardial contractility. Failure of one side causes failure of the other.

Question 9

when do you hold digoxin in an infant?

Answer 9

heart beats less than 80 beats/min (remember adults under 60) and 70 beats/min in toddlers

Question 10

signs of digoxin toxicity

Answer 10

vomiting, pulse irregularities

Question 11

tachycardia, sweating at rest, poor urine output, fatigue, poor feeding, poor pulses

Answer 11

CHF

Question 12

hypoxemia vs hypoxia

Answer 12

hypoxemia: low oxygen in the blood
hypoxia is low oxygen in the tissues
cyanosis at 80-85% saturation

Question 13

bacterial endocarditis

Answer 13

infection of valves/lining; result of abnormal/artificial valves, recent surgery, rheumatic fever. Nursing care focuses on Abx, repeat blood cultures, and observe for embolus. Educate on prophylaxis for invasive procedures.

Question 14

rheumatic fever

Answer 14

autoimmune? commonly follows a strep infection. pain in joints, CHF symptoms, tachycardia, rash. give Abx and anti-inflammatories, rest. Educate on chorea (ticks of face and hands pill roll)-decreases once antiinflammatories do their thing. Abx for 6 months!!!

Question 15

Kawasaki Disease

Answer 15

Phase 1 (1-10 days): fever > 5 days unresponsive to antipyretics, conjunctivitis, crusted/fissured lips, swollen hands and feet, erythema, lymphadenopathy
Phase 2 (10-25 days): fever diminishes, irritability, anorexia, desquamation of hands and feet, arthritis, arthralgia, carditis
Phase 3 (26-40+days): drop in ESR, diminishing signs of illness but inflammation still present

Question 16

Kawasaki Disease Tx

Answer 16

treat with aspirin and IVIG

call physician if child refuses to walk-disease often affects spinal cord

Question 17

hyperlipidemia

Answer 17

lower the content of sat fat and cholesterol; educate the entire family about proper nutrition

Question 18

nursing care after cath

Answer 18

Direct pressure for 15 minutes without peeking
Pressure dressing for at least 6 hours
Vitals and neurovascular checks, including pulses, q 15 mins for 1 hour, then q 30 mins for 1 hour, then q hour for 4-8 hours
Bedrest for 6 hours, “quiet play” for first 24 hours
Monitor for: bleeding, arrhythmias, infection, excessive diuresis from dye
Promote fluid intake to flush out dye

Question 19

shock

Answer 19

Perfusion no longer meets metabolic needs of body
Early: fear, irritability, narrowing pulse pressure, mild ↑HR, ↓UO, slower CRT
LATE: decreased LOC, weakening pulses, hypotension, tachypnea or apnea, anuria

Question 20

shock Tx

Answer 20

Open airway, 100% oxygen
20 ml/kg boluses of LR or NS as ordered
IV epinephrine or other vasopressors as ordered
EKG and Pulse Ox, monitor temp
Consider cause: Sepsis? Allergy? Drugs? SCI?

Question 21

pediatric cardiac assessment

Answer 21

Familial history of CHD
Other genetic defects
Maternal rubella in pregnancy
Maternal age 40
Maternal weight under 100 or over 200 pounds
Maternal IDDM
Fetal Alcohol Syndrome
Presence of other extracardiac birth defects

Question 22

Unique aspects of childhood cardiac function until age 5

Answer 22

heart rate varies according to oxygen needs, accounting for higher resting heart rate in children
cardiac output dependent on heart rate until age 5
increased risk of heart failure due to inability to adapt to volume or pressure increases
muscle fibers of heart less developed

Question 23

most frequent complaints from parents of kids with cardiac dysfunction

Answer 23

Trouble breathing
Trouble feeding
Colic and crying
Lack of bonding

Question 24

hypopituitarism

Answer 24

Pituitary gland does not produce enough Growth Hormone (GH)
Child “falls off” growth chart, usually 3rd-5th percentile by age 2
Assessment: short stature, fontanels don’t close, poor muscle mass, teeth crowded/crooked
Treatment/Interventions: daily SQ GH ($$$), growth plotted every 6 months
Education/Teaching: social interaction, treat child based on age not size

Question 25

SIADH

Answer 25

SIADH: problems stem from low sodium: tx strict I/O, daily weights, fluid overload, seizure precautions, long term ADH antagonists

Question 26

precocious puberty

Answer 26

Early onset before 8 (girls) or 9 (boys)
Synthetic LRH to slow, or no treatment
Support with dress, peers

Question 27

diabetes insipidus

Answer 27

Improper secretion of ADH–>uncontrolled diuresis
Polyuria, polydipsia, enuresis
Urine SG <1.005, weight loss 3-5%
Often temporary, NOT diabetes mellitus

Question 28

excessive sweating and very high tachycardia, fever

Answer 28

thyroid storm-whenever you do a thyroidectomy-treat with beta blocker (propranolol), PTU, and MTZ

Question 29

1 cause of ambiguous genetalia at birth

Answer 29

CAH: Congential adrenal hyperplasia
Tx: cortisone, salt
GENDER ASSIGNMENT: wait on gender assignment until you see how the child develops

Question 30

acanthosis nigricans

Answer 30

darkening of skin in fold areas
cutaneous manifestation of hyperinsulinemia
precursor to DMII
BCP’s can also cause this. Never normal, bc elevated hormones cause this.