NPS Pathology

Question 1

Four defects involved in Tetralogy of Fallot

Answer 1

Ventricular Septal Defect
Overriding Aorta
Right ventricular hypertrophy
Pulmonary Stenosis

Question 2

Treatment for Tetralogy of Fallot

Answer 2

100% O2
Give prostaglandins to keep ductus open
Surgical correction

Question 3

Boot-shaped heart indicates ___.

Egg-shaped heart indicates ___.

Answer 3

Tetralogy of Fallot.

Transposition of Great Vessels.

Question 4

What are the symptoms of a preductal coarctation?

Answer 4

Right to left shunt

Cyanosis will be present.

Question 5

What are the symptoms of a postductal coarctation?

Answer 5

Left to right shunt.
No cyanosis
Pulmonary HTN
Pulmonary edema

Question 6

(Left to right/right to left) shunts result in hypoxemia.

Answer 6

Right to left. (More dangerous than left to right)

Question 7

(Left to right/right to left) shunts result in pulmonary congestion..

Answer 7

Left to right.

Question 8

If blood pressure in the arms is a lot higher than in the legs, suspect this pathology:

Answer 8

Coarctation of aorta.

Question 9

What is the pathology of a truncus arteriosus? What is the treatment (besides surgery)?

Answer 9

Single common vessel for both the aorta and pulmonary artery. Also a septal defect.
Give O2 but not too much because you have to keep PVR high.

Question 10

What is the pathology of hypoplastic left heart syndrome (x3)?

Answer 10

Severely underdeveloped left ventricle.
Stenosis (narrowing) of mitral valve
Small left atrium

Question 11

What is the treatment for hypoplastic left heart syndrome?

Answer 11

Target SpO2 sat 75-85%
Give prostaglandins to keep ductus open.
Surgery.
(If you give too much O2 things will close that you need to stay open)

Question 12

What is the pathology of Total Anomalous Pulmonary Venous Return?

Answer 12

Pulmonary veins don’t return to left atria.

“Snowman” heart shape on CXR.

Question 13

What is the pathology of an atrial-septal defect?

Answer 13

Foramen ovale doesn’t close. L to R shunt.
May require supportive O2.
Not usually critical. Can be fixed at 5-6 years old.

Question 14

What is the pathology of a ventricular septal defect? How do you treat it small defects? Large defects?

Answer 14

An opening between ventricles.
Small defects: O2, digoxin, diuretics.
Large defects: Surgery

Question 15

How do you treat PDA?

Answer 15

High FiO2
Indomethacin.
Give Lasix.
Fluid restrictions.

Question 16

How can you use PO2 to diagnose PDA or PPHN?

Answer 16

Preductal PO2 increased, post-ductal PO2 decreased. Difference must be 15 torr.

Question 17

What is the pathology of Atrial-ventricular canal?

It is common with what syndrome?

Answer 17

ASD, VSD, Tricuspid valve defect, and mitral valve defect.

Common in Down syndrome.

Question 18

What is the pathology of PPHN? What’s it caused by?

Answer 18

Severe hypoxemia and cyanosis right after birth.
Caused by increased pulmonary vascular resistance.
Similar to PDA.

Question 19

How do you treat PPHN?

Answer 19

Hyperoxygenate. (This dilates pulmonary arteries)
Give Tolazoline (Priscoline) (Vasodilator)
Nitric oxide
HFOV
ECMO

Question 20

How do you treat Transient Tachypnea of the Newborn (TTNB)?

Answer 20

40-60% FiO2 via hood. May require CPAP.

Usually self limiting.

Question 21

How do you treat MAC?

Answer 21

Intubate and suction thoroughly via ET tube.
Percussion and drainage with suctioning x 8 hours
Nitric oxide if PPHN develops.

Question 22

If the patient has CDH, do not ___ until you get a ___.

Answer 22

Do not ventilate until you get an NG tube.

Question 23

If an infant shows signs of shock and mother is sick, you should suspect ___.

Answer 23

Strep B infection.

Question 24

What is the pathology of Pierre-Robin Syndrome? To treat it, place a ___. Position patient in ___ position. Feed with a ___. And if it’s severe, perform a ___.

Answer 24

Tongue is too big and jaw is too small.
Place a nasal airway. 
Place pt in prone position. 
Feed by NG tube. 
If severe, tracheostomy may be necessary.

Question 25

What is the pathology of Tracheoesophagea fistula?

Answer 25

Esophagus is a dead end. A hole goes into the trachea. Place pt. in reverse trendelenberg. Put replogle tube to keep esophageal pouch empty. Clear secretions with a bulb syringe. Requires surgery.

Question 26

What is the pathology of PIE? CXR will show __ or __. May also show ___ in the hilar area.

Answer 26

Air leaking from lungs into interstitial space. CXR will show pneumothorax or pneumomediastinum. Bubbles.

Question 27

How do you treat PIE?

Answer 27

Keep PIP down. Give 100% O2 for 10-15 min intervals. Single-lobe intubation. HFOV

Question 28

What is the pathology of Necrotizing Entercolitis?

Answer 28

Ischemic necrosis to part of the GI tract. | CXR shows distended loops of bowel.

Question 29

How do you treat Necrtoizing Entercolitis?

Answer 29

``` No oral feeding. Abdominal decompression via NG tube. Blood replacement Antibiotics Surgical resection of bowel. ```

Question 30

What are two vaccines for RSV? | What do you use to treat RSV (3 answers)

Answer 30

Respigam or Synagis | Treat with Ribavirin with SPAG and scavenger system.

Question 31

A normal sweat chloride test reading is around ___. CF reading is ___. If reading is positive, what should you do?

Answer 31

28 60 Repeat a second time to confirm.

Question 32

What is the most common infection for a CF patient? | What two drugs treat it?

Answer 32

Pseudomonas. | Tobramyacin or aztreonam

Question 33

How do you treat asthma? | If asthma is poorly controlled, give ___ or ___.

Answer 33

Always start with O2 (40-60%) Bronchodilators Inhaled steroids & mast cell inhibitors if poorly controlled

Question 34

A moderate asthma patient who is 5-11 year old has an FEV1 of ___. Their FEV1/FVC is ___. They use their SABA ___ times a week. And ___ times a week, they wake up in the night.

Answer 34

60-80% 75-80% 7 (every day) Not more than 2 nights per week

Question 35

Yellow zone of asthma action plan: Peak flow will be ___. Take ___. Begin or increase regimen of ___.

Answer 35

``` 50-80% Take albuterol or SABA. Begin or increase oral steroids. Call doctor. Return to Green when symptoms improve. ```

Question 36

To remove a foreign body that has been aspirated, use a (flexible or rigid) bronchoscopy.

Answer 36

Rigid

Question 37

What is the treatment for croup?

Answer 37

Mist tent, 30-40%. Racemic epinephrine. Rest Throat and blood cultures.

Question 38

If croup is severe, treat in the ICU. Severe croup presents with ___ and ___. (Treatment is the same)

Answer 38

Inspiratory and expiratory stridor and Retractions

Question 39

What is the treatment for epiglottitis?

Answer 39

``` Maintain child's position. Take lateral X ray, look for thumb sign. Intubate (or tracheostomy in OR only) Antibiotics Humidified O2 by T tube or CPAP. ```