exam 3 study guide

Question 1

what are the cyanotic defects

Answer 1

• Tetralogy of Fallot (TOF)
• Pulmonary Atresia
• Tricuspid Atresia

Question 2

Tetralogy of Fallot (TOF)

Answer 2

• Decreased pulmonary blood flow.
• Cyanosis
• O2 saturation baseline may be 93-94% RA
  Hypercyanotic, Tet or Blue spells - anoxia occurs when oxygen requirements exceed supply (Place in knee – chest position, squatting, give oxygen, Morphine, Inderal)
• Fe Supplement
• Polycythemia – Hydration needed
• Complete surgical repair first year of life.
• Palliative surgery if need to delay surgical repair. (Blalock-Taussig shunt procedure increases oxygenation by attaching the subclavian artery to the pulmonary artery. Post-op CHF possible
• Bacterial endocarditis – PCN prophylaxis

Question 3

Four defects of tetralogy of fallot (TOF)

Answer 3

• ventricular septal defect (VSD)
• Pulmonary stenosis (degree determines severity of symptoms)
• an overriding aorta
• right ventricular hypertrophy.

Question 4

Blalock-Taussig shunt procedure?

Answer 4

• increases oxygenation by attaching the subclavian artery to the pulmonary artery
• Post-op CHF possible

Question 5

Tricuspid Atresia

Answer 5

• Failure of the tricuspid valve to develop.
• Decreased pulmonary blood flow.
• Cyanosis
• Must have other defects at birth to survive: ASD & VSD &/or PDA
• Initially given Prostaglandin E 1 (PGE 1 ) until surgical repair.

Question 6

Pulmonary Atresia

Answer 6

• Absence of opening between (R) ventricle and pulmonary artery
• Open ASD and PDA required for survival
• Tachypnea
• CHF
• Clubbing
• Polycythemia
• Growth delay
• Prostaglandin E1 needed
• Digoxin and diuretics

Question 7

acyanotic obstructive defects

Answer 7

• Aortic Stenosis (AS)
• Pulmonic Stenosis (PS)
• Coarctation of the Aorta (COA)

Question 8

Aortic Stenosis (AS) (acyanotic)

Answer 8

• Narrowing at the entrance to the aorta.
• Decreased cardiac output.
• Mitral insufficiency - murmur
• (L) ventricular hypertrophy
• Pulmonary vascular congestion → pulmonary hypertension.
• Weak thready pulses
• Hypotension
• Dizziness
• Syncope
• Repair can be done during cardiac catheterization. Often not permanent.
• Surgery – aortic valvulotomy.
• Now can be done in utero.
• Digoxin & diuretics for heart failure.
• Bacterial endocarditis – PCN prophylaxis

Question 9

Pulmonic Stenosis (PS) (acyanotic)

Answer 9

• Narrowing at the entrance to the pulmonary artery.
• Decreased pulmonary blood flow → (R) ventricular hypertrophy → (R) sided heart failure.
• Asymptomatic → CHF & mild cyanosis. Depends on the extent of the narrowing.
• Digoxin & diuretics if CHF
• Surgical – valvulotomy with cardiac bypass
• Repair can be done during cardiac catheterization.
• Bacterial endocarditis – PCN prophylaxis

Question 10

Coarctation of the Aorta (COA) (acyanotic)

Answer 10

• Localized narrowing of the aorta near the insertion of the ductus arteriosus.
• Increased BP upper extremities. Decreased BP in lower extremities (20 mm Hg or ≥ ).
• Bounding peripheral pulses in arms and decreased or absent pulses on femoral & pedal pulses.
  CHF with severe.
• Older children – cool lower extremities, headaches, dizziness, fainting, epistaxis from hypertension
• Repair can be done during cardiac catheterization- Balloon angioplasty
• Surgical resection with an end to end anastomosis via thoracotomy. Post-op hypertension
• Best treated less than 6 month of age
• Bacterial endocarditis – PCN prophylaxis

Question 11

Atrial Septal Defect (ASD)

Answer 11

• Abnormal opening between the (R) and (L) atria.
• Often at foramen ovale.
• Increased pulmonary blood flow.(L) → (R) shunt
• ↑ pulmonary congestion → pulmonary HTN
• Systolic murmur
• Surgical repair at 2 →4 yrs.
• Repair during cardiac catheterization more common.

Question 12

Ventricular Septal Defect (VSD)

Answer 12

• Most common defect
• Abnormal opening between the (R) and (L) ventricle
• Increased pulmonary blood flow. (L) → (R) shunt
• CHF common
• Pulmonary Hypertension
• Murmur (L) sternal border
• Repair can be done during cardiac catheterization if small opening.
• Surgical repair with cardiopulmonary bypass.
• At risk for bacterial endocarditis – PCN prophylaxis

Question 13

Patent Ductus Arteriosis (PDA)

• PDA remains open when poor oxygenation is occurring in the newborn

Answer 13

• Failure of the fetal ductus arteriosus to close
• Functional closure first 5 hours after birth. Permanent closure 5 – 7 days after birth
• Common in preterm births
  Increased pulmonary blood flow
• (L) → (R) shunt
• Machine like murmur
• Asymptomatic → CHF
• Bounding peripheral pulses
• Wide pulse pressure (Systolic pressure minus the diastolic pressure. This expresses the tone in the arterial walls. ≥40 – 50 mm Hg difference.)
• Initial treatment: Indomethasin (Indocin®) or Ibuprophen– prostaglandin inhibitors
• Repair can be done during cardiac catheterization.
• Surgery if needed.
• Pulmonary hypertension if not treated early.
• Bacterial endocarditis – PCN prophylaxis

Question 14

Cardiac Catheterization

Answer 14

• Pressure & location of abnormal openings
• Pressure within structures
• O2 saturation of chambers
• Repair

Question 15

where is cardiac catheterization done

Answer 15

Done using arteries &/or veins with contrast media

• (R) Side catheterization- Most common- Femoral vein → (R) atria
• (L) Side catheterization → artery → aorta → heart

Question 16

cardiac catheterization PRE TEST

Answer 16

• Requires Consent
• NPO before test – 4-6 hours or more
• Check allergies: Especially to iodine or shell fish
• Baseline V/S & O2 saturation
• Assess skin: Especially in groin area
• Ht/Wt
• Mark pedal pulses
• Administer sedation, analgesics
• Void on call if toilet trained

Question 17

Post test: Cardiac Catheterization

Answer 17

Typically: q 15 x 4h
                q 30 x 4h
                q 1h x 4h
                q 4h x 16h
Include: Apical pulse – detect arrhythmias & tachycardia
- BP decreased if hemorrhage

Question 18

Cardiac Catheterization ASSESSMENTS

Answer 18

• Peripheral Pulses: Assess equality & symmetry
  (especially in extremity used)
• Assess LOC
• Assess color warmth of extremity
• Pressure dressing x 24 hours –Assess bleeding (If noted: Apply pressure 1 inch above site, place flat position, call MD) ***
• Decrease movement of leg ( Keep straight 4-8 hours )
• I&O: Contrast medium acts as diuretic- Especially important in infants
• Dye is nephrotoxic
• Increase fluids to get rid of dye
• Assess glucose level R/T possibility of hypoglycemia

Question 19

Kawasaki Disease

sign and symptoms

Answer 19

• Fever ( note high fever for 5 days that is unresponsive to antibiotics )
• Chills
• Headache
• Malaise
• Extreme irritability
• Vomiting
• Diarrhea
• Abdominal and joint pain

Question 20

Heart failure sign and symptoms

Answer 20

• Failure to thrive
• Lethargy
• Tires with feeding/play
• Tachypnea
• Nasal flaring
• Intercostal retractions
• Crackles
• Tachycardia → cardiomegaly
• Cool extremities
  Diaphoresis
• Edema – periorbital
• Dependent edema – older child
• Slow capillary refill
• Decreased peripheral pulses
• Hepatomegaly
• Oliguria
• Jugular vein distension – older child
• Increased respiratory infections

Question 21

Nephrotic Syndrome

Answer 21

Results from disorder within the glomerulus of the kidney where the glomerus becomes permeable to proteins especially albumin and immunoglobulin

• proteinuria
• hypoalbuminemiam= leads to decreased osmo pressure in capillaries/serum albumin levels leads to decrease protein in blood
• hyperlipidemia
• edema

= Primary nephrotic syndrome = minimal change nephrotic syndrome = MCNS
Secondary to systemic lupus, diabetes

• onset by 6 years (most common)
• more males than females

Question 22

patho of nephrotic syndrome

Answer 22

• Hypoalbuminemia leads to decreased osmotic pressure in capillaries.
• Leads to Fluid accumulating in interstitial spaces (EDEMA).
• This fluid shift decreases vascular fluid volume causing hypovolemia. ECF to ICF
• This stimulates the renin-angiotensin system and secretion of antidiuretic hormone & aldosterone.
• Tubular reabsorption of sodium & water increases.
• Attempt is to increase intravascular volume.
• Protein loss also leads to immunoglobulin loss. Leads to altered immunity.
• Liver increases synthesis of lipoprotein (cholesterol) leading to hyperlipidemia.(R/T protein loss)
• There is also loss of antithrombin III & decreased levels of factors IX,XI,& XII due to urinary loss.
• This can lead to hypercoagulability.
• Child increased risk for thrombosis.

Question 23

Clinical Manifestations of nephrotic manifestations

Answer 23

• Dark, foamy, and frothy urine
• Decreased urine production
• Edema — Generalized
• Stretched, shiny skin with a waxy pallor
• Fatigue & lethargy
• BP Normal to slightly decreased
• Hypertension (Late)

1. Weight gain
2. Periorbital (Early sign) Especially in AM
3. Abdominal ascites with increased abdominal girth (Progresses through day
4. scrotal edema
5. ankle edema
6. Diarrhea, anorexia & malnutrition result from edema of the intestinal mucosa