Cardiology - 2019 Updated! Flashcards

1
Q

SVT in neonates is most commonly associated with:

a) hypocalcemia
b) hyperthyroidism
c) electrolyte disturbances
d) ventricular septal defect
e) structurally normal heart

A

e) structurally normal heart

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2
Q

A baby has been diagnosed with truncus arteriosus. What is the most likely complication in the first week?

a. pulmonary edema
b. severe cyanosis
c. heart failure

A

a. pulmonary edema

→ secondary to decreased pulm vascular resistance and increased pulmonary blood flow – get pulm overload then continue to see CHF (after first month)

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3
Q

Infant with large VSD. The murmur cannot be heard. What is the cause?

a. VSD has closed
b. There is increased pulmonary outflow obstruction
c. Pulmonary arterial pressures have increased

A

c. Pulmonary arterial pressures have increased

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4
Q

A 2-day-old infant presents in congestive heart failure. He has hydrocephalus. He has a seizure 1 hour after admission.

Most likely cause:

a) vein of Galen aneurysm
b) intraventricular hemorrhage
c) hypoxic ischemic encephalopathy
d) cerebral abscess
e) meningitis

A

a) vein of Galen aneurysm

AVMs are most common cause of hemorrhagic stroke in children

O/E: continuous murmur when listen over fontanelle
Basically there is no capillary bed separating arteries from vein, so the blood flows really quickly and easily from the artery into the venous system. The heart has to pump harder and faster to increase CO to keep up with this much faster flow of blood through the brain. The baby then develops high output cardiac failure

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5
Q

First line treatment of a child with bradycardia

A
  • ABCs!!
  • CPR if HR <60/min with poor perfusion despite oxygenation
  • Epinephrine, then atropine

-Consider transthoracic pacing

If no cardiopulmonary compromise - Support ABCs, O2, Consider expert consultation

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6
Q

Epinephrine at low and high doses.

  1. What is the effect on contractility (increased, decreased, none)?
  2. What is the effect on systemic vascular resistance?
  3. What is the effect on HR?
A
Epi Low dose:
(beta1 + beta 2 stimulation)
Contractility: increased ++
SVR: decreased 
HR: Increased ++
Epi High dose
(alpha 1 + beta 1)
Contractility: increased
SVR: increased
HR: Increased
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7
Q

Infant with R arm sat of 90% and L leg sat of 70%. Pt tachypneic, RR 70, no distress. Dx?

a. CoA
b. truncus
c. TGA
d. TOF

A

a. CoA

Also see in PPHN with a PDA shunting from PA to Ao, Interrupted Ao Arch, Critical Coarct, and Critical Aortic Stenosis.

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8
Q

Newborn with meconium stained fluid, needed resuscitation, poor apgars. Cyanotic and in 100% O2 had a PO2 of 70 with a normal CO2 (Rt Radial A) . Cord gas had O2 of 30. CXR normal sized heart and decreased vascularity. Most likely diagnosis

a) PPHN
b) TGA
c) TAPVD
d) Mec aspiration syndrome

A

a) PPHN (BW implied that pre sat higher than post)

PPHN: MAS predisposes patients to PPHN and presents as PaO2 of <100mmHg in response to 100% oxygen supplementation AND Preductal > Postductal with a greater than 10% difference.

Preductal is higher b/c there is shunting across PDA R-> L due to high pulmonary pressure

Why others are wrong:

b) TGA (increased vascularity)
c) TAPVD (increased vascularity)
d) Mec aspiration syndrome (cyanosis improve with oxygen if no PPHN)

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9
Q

TAPVD repair as younger child; now has ADHD and want to start stimulant - what to do?
A) ECG
B) Go ahead and prescribe
C) Stimulants are contraindicated

A

B) Go ahead and prescribe

Ugh - CPS statement sucks.

Patients with CHD that are at increased risk of sudden death should already be under a cardiology.

There is no compelling evidence that ADHD medications raise the risk of sudden death even further, therefore initiation of ADHD medication should be primarily at the recommendation of an ADHD specialist… ALTHOUGH discussion with cardiologist is appropriate.

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10
Q

An infant is irritable and is feeding poorly. Your EKG looks something like this (but much faster! - SVT): The child is stable . What are TWO things that you would do for management?

A
  1. Vagal manuvers (ice, valsalva, breath holding)
  2. Adenosine *remember adenosine can cause a fib so be ready for DC cardioversion

Adenosine = 0.1 mg/kg max 12mg

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11
Q

A baby has cyanosis, an enlarged heart, and decreased vascularity on CXR. What is the lesion?

tetraology of Fallot
trucus arteriosus
TGA
TAPVR

A

TOF

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12
Q

First line treatment of a child with V tach with pulse

A

Adenosine if regular rhythm and QRS monomorphic.

If cardiopulmonary compromise (hypotension, aLOC, shock) then synchronized cardioversion.

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13
Q

16 year old basketball player collapses during a game. Basic life support is administered and he receives a 1st shock in the community for what the AED detects as “pulseless VT”. He is then brought to the ED where he remains in pulseless VT at your assessment. Your first step is to:

a) Continue 5 minutes of CPR – checking every 2 minutes
b) Asynchronous cardioversion 2J/kg
c) Asynchronous cardioversion 4J/kg
d) Give the first dose of IV epinephrine

A

c) Asynchronous cardioversion 4J/kg

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14
Q

(Photo of EKG showing wide complex tachycardia)
Patient in ED waiting area with poor pulses, not responsive, first line therapy?

1) Adenosine
2) Sync cardiovert
3) CPR
4) Amiodarone

A
  1. Synchronized Cardioversion
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15
Q

List 4 other signs of endocarditis in a child with a fever, murmur, tachycardia and hepatospenomegaly.

A
  • Roth spots (hemorrhage in retina)
  • petechiae
  • splinter hemorrhages (linear lesions beneath nails)
  • Osler nodes (tender pea-sized intradermal in pads of fingers + toes)
  • Janeway lesions (painless small lesions of palms/soles)
  • Heart failure
  • Arrhythmias
  • Splenomegaly
  • Clubbing
  • Tachypnea
  • Pulmonary Emboli
  • Arthritis
  • Meningitis
  • Pericarditis
  • Abscesses
  • Blood Culture +ve
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16
Q

16 year old girl complains of feeling dizzy and palpitations – it occurs within 5 minutes of standing upright. Her supine blood pressure is 118/70 and her supine heart rate is 84bpm. When she stands up, her blood pressure is 116/68 and her heart rate is 120bpm. What is the diagnosis?

Orthostatic hypotension
Long QT
Postural orthostatic tachycardia syndrome

A

Postural orthostatic tachycardia syndrome

An orthostatic heart rate of >120 beats/min and a rise in heart rate of ≥30 beats/min with 5 min of standing suggest the diagnosis.

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17
Q

Child with tall, mitral valve prolapse, hyperextensible joints, long arms, who has rectal prolapse.

a) What is the Dx
b) What is the Ddx for rectal prolapse

A

a) Marfan or Ehler-Danlos (ED has rectral prolapse- not Marfan… but they’re not tall)

b) Intestinal parasites
Malnutrition
Diarrhea
UC
Pertussis
CF
Chronic constipation
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18
Q

Features of Innocent Murmurs

A
Grade =<2
	Softer when patient sitting compared to supine
	Short systolic duration
	Minimal radiation
	Musical or vibratory quality
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19
Q

Which of the following is associated with an increased risk of necrotizing fasciitis?

a) parvo virus
b) roseola
c) Kawasaki disease
d) varicella
e) rosacea

A

d) varicella
Varicella is a risk factor for group A strep infection which causes neck fasc

Other risk factors:
diabetes
HIV
IVDU
chronic pulmonary or cardiac disease
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20
Q

Milrinone

  1. What is the effect on contractility (increased, decreased, none)?
  2. What is the effect on systemic vascular resistance (increased, decreased, none)?
  3. What is the effect on HR?
A

Contractility: Increased (systolic and diastolic fcn improved)
SVR: Decreased
HR: No change

Main issue is the long half life - can’t just stop it

Phosphdiesterase inhibitor - inotropy, lusitropy

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21
Q

A 3 week old infant presents with 3 days of progressive difficulty feeding, vomiting and tachypnea. On examination his HR is 260, BP is 80/50, CR is 3s and the liver is 5 cm below the costal margin. What is the most likely etiology:

a. SVT
b. Myocarditis
c. VSD

A

a. SVT

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22
Q

An infant is in shock with paroxysmal supraventricular tachycardia. You would give:

a) bag of ice to face
b) synchronous DC cardioversion
c) asynchronous cardioversion
d) verapamil
e) digoxin

A

b) synchronous DC cardioversion (assuming there is a pulse)

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23
Q

3 year old finger in light socket, has ECG with Vfib. Receiving CPR.
What to do?

  1. cardiovert with 1J/kg
  2. epinephrine IO/IV
  3. defibrillate with 2J/kg
  4. intubate
A
  1. Defibrillate with 2J/kg

Can go up to 4 J/kg

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24
Q

A teenager has long QT syndrome. He also has been having syncopal episodes while participating in sport. What is the best course of management:

a. Start CCB and restrict vigorous activity
b. Start CCB and do not restrict activity
c. Start beta blocker and restrict vigorous activity
d. Start beta blocker and do not restrict activity

A

c. Start beta blocker and restrict vigorous activity

Until you at least know his symptoms are under control

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25
Q

You are seeing a teenager with a history of recurrent syncopal episodes. What is the best screening test for prolonged QT syndrome.

a) EKG
b) exercise EKG
c) Holter monitor
d) echocardiogram
e) electrolytes

A

a) EKG

Findings:

  • QTc>0.47 seconds is highly indicative, >0.44 is suggestive
  • notched T waves in 3 leads
  • T-wave alternans (beat to beat variation in amplitude or shape of the T wave)
  • low resting heart rate for age
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26
Q

A 3 day old is tachypneic, cyanosed despite 100% O2. Bilateral crackles on exam with weak peripheral pulses and no heart murmur.

What is the diagnosis:

a) HLHS
b) Sepsis
c) AV fistula

A

a) HLHS

Most are acutely ill within first days/weeks of life (as PDA closes)
o Lactic acidosis
o HF
o Cardiogenic shock
o Cyanosis
o Poor pulses
o Hyperdynamic cardiac impulse

Mgmt: start PGE, surgical palliation (Norwood, Glenn, Fontan) or transplant

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27
Q

Infant with large VSD. The murmur cannot be heard. What is the cause?

a) VSD has closed
b) there is increased pulmonary outflow obstruction
c) pulmonary arterial pressures have increased

A

c) pulmonary arterial pressures have increased - known complication of unrepaired VSD

VSD has closed - large VSD unlikely to close spontaneously
There is increased pulmonary outflow obstruction - possible but occurs infrequently

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28
Q

5 year old with exercise intolerance. On examination he has a slight heave at the left lower sternal border. His first heart sound is normal, the second heart sound is split and quieter. He has a Harsh ejection systolic murmur loudest at the left upper sternal border. What is the most likely cause?

a. Mitral stenosis
b. Mitral valve prolapse
c. Aortic stenosis
d. Pulmonary stenosis

A

c. Ao Stenosis

Harsh systolic murmur with heave and P2;A2 sequence of second heart sound with obscuring (quiet) second heart sound, exercise intolerance = AS

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29
Q

A full term infant is diagnosed with meconium aspiration syndrome. He is desaturating despite 100% oxygen. An echocardiogram shows normal cardiac anatomy and R to L shunting at the ductus and atrial level. Which of the following interventions may be effective:

a. dopamine
b. indomethacin
c. nitric oxide
d. prostaglandins

A

c. nitric oxide

PPHN

  • Persistent fetal circulatory pattern of R -> L through PDA and foramen oval as result of high PVR
  • Unresponsive to 100% O2; pre/post sat differential
  • Predisposing factors: birth asphyxia, MAS, early onset sepsis, RDS, hypoglycaemia, polycythemia, maternal NSAIDS, maternal late use SSRIs, Pulmonary hypoplasia (diaphragmatic hernia, oligohydramnios, pleural effusions)
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30
Q
  1. A term IDM newborn is seen at 48 hours of age with a grade 3/6 SEM at the LSB. On echo there is hypertrophy of the septal muscle but no decrease in function. What is the clinical course:
    a. Will resolve with no treatment
    b. corticosteroids
A

a. Will resolve with no treatment

IDMs have increased risk of transient hypertrophic cardiomyopathy

  • associated with inter ventricular septal hypertrophy and decreased ventricle size (increased risk for LVOTO)
  • resolves spontaneously as plasma insulin levels normalize (2-3 weeks), usually asymptomatic, may have resp distress
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31
Q

10 Day baby with lethargy, poor feeding. Glucose normal. Femoral pulses are decreased, baby is poorly perfused.

a) Most likely Dx
b) First Step (Be specific)
c) Side effects of Tx

A

a) Critical coarctation of Aorta
b) Prostaglandin E1 0.01mcg/kg/min
c) Apnea, hypotension, flushing, hyperthermia, hypoglycemia, NEC

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32
Q

First line treatment of a child with V tach without a pulse

A
Shock
CPR
Shock
CPR
Epi q3-5 min
Shock
CPR
Amiodarone

Treat H/Ts

epinephrine, the amiodarone or lidocaine (same as V fib)

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33
Q

For which patient is prophylactic antibiotics before a dental procedure indicated for?

a. Unrepaired TOF
b. VSD that was repaired 10 months ago
c. VSD that was repaired 8 months ago with a small residual leak
d. Moderate aortic stenosis

A

a. Unrepaired TOF

Assuming VSD that was repaired with small leak was stitch closed- not patch closed

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34
Q

Meds with prolonged QT

e. Hypercalcemia
f. Hypokalemia
g. Clarithromycin
h. Digoxin

A

f. Hypokalemia
g. Clarithromycin
Both can prolong QT; also HYPOcalcemia, hypoMg, TCAs, anti-arrhythmics (procainamide, amiodarone, sotalol)

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35
Q

Description of an anorexic teenage girl. What would be the most likely finding on ECG?

a. Peaked T waves
b. prolonged QT
c. Right axis deviation
d. ST elevation

A

b. Prolonged QT
(due to hypokalemia)

  • Also: bradycardia
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36
Q

15 year old female with 5 prior episodes of syncope. Each one is associated with lightheadedness, going dark in field of vision. On one episode her father kept her upright and she had brief GTC activity with urinary incontinence. What is your initial investigation?\

  1. EEG
  2. ECG
  3. blood pressure lying and standing
  4. Echo
A
  1. blood pressure lying and standing

All patients presenting with a first episode of syncope should have an electrocardiogram obtained, looking primarily for QT interval prolongation, preexcitation, ventricular hypertrophy, T-wave abnormalities, and conduction abnormalities.

New CCS guidelines on syncope would suggest if NO concerning features on the history and physical examination, the ECG screening is not necessary

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37
Q

Cyanotic baby, presents at 5 days of age. His sats increase from 79 to 81 with oxygen. His RR – 50s, HR 180s. On exam he has single S2 loud, no murmur. Chest X-ray shows narrow mediastinum and mildly increased pulmonary markings. EKG shows mild right ventricular hypertrophy.

  1. What is your diagnosis?
  2. What is one medication you can give for treatment?
  3. What are four complications of this treatment.
A
  1. Diagnosis: TGA
  2. Mgmt: Prostaglandin E1
  3. Complications: apnea, hypotension, NEC, fever, flushing, hypoglycemia, hyperthermia

TGA (~5% of all CHD) – desaturated blood returning from the body to the right side of the heart goes inappropriately out the aorta and back to the body again, whereas oxygenated pulmonary venous blood returning to the left side of the heart is returned directly to the lungs (systemic and pulmonary circulations exist as 2 parallel circuits) ; 50% have VSDs

2nd heart sound is usually single and loud, although it may be split – because pulmonary artery is located posterior and directly behind the aorta. Thus, the aortic component of S2 is loud because of its anterior location and the softer pulmonary component is often inaudible

Murmurs usually absent

ECG - normal - expected R sided dominant neonatal pattern

CXR- Mild cardiomegaly, narrow mediastinum (egg on string), increased pulmonary blood flow as pulmonary vascular resistance drops in first weeks

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38
Q

Girl with confirmed hypertension by ambulatory BP monitor. Now BP is >95% for age. Renal function tests are normal. What is treatment?

a) renal u/s
b) HVA, VMA
c) start captopril
d) repeat ambulatory BP

A

c) start captopril

2017 HTN Guidelines:
All Patients:
- Urinalysis
- Lytes, Urea, Creatinine
- Lipid profile 
- Renal Ultrasound in those < 6 years or with abnormal UA or renal fcn

If Obese (BMI >95%ile), add:

  • Hemoglobin A1C
  • AST, ALT (screen for fatty liver)
  • Fasting Lipid profile (screen for dysplidemia)

Other Options Depending on Hx and PE:

  • Fasting glucose
  • TSH
  • Drug Screen
  • Sleep Study
  • CBC (especially with growth delay or abnormal renal function)
  • Echocardiogram (don’t do an ECG looking for LVH)
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39
Q

3-day-old infant with congenital heart disease whose cyanosis is aggravated by crying. Most likely from choice? Most likely not included in these choices?:

a) TGA
b) VSD
c) ASD
d) PDA
e) PS

A

e) PS

This is most likely actually describing TOF, but given the choices PS is the most correct

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40
Q
  1. Name 4 side effects of prostaglandin E.
A
Hypotension
Apnea
Hyperthermia
Flushing
Hypoglycemia
NEC
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41
Q

A 12 month old child is seen in your ED with a cough and fever. A RLL consolidation is seen on
CXR. On examination of the heart, you hear a loud S1, a fixed split S2 and a SEM over the LUSB.
What is the cause of the child’s heart murmur:
a. Increased flow secondary to the pneumonia
b. PS
c. ASD
d. Still’s murmur

A

c. ASD

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42
Q

7 year old with severe hypertension.

List 4 investigations you would use to identify end organ damage.

A
ECHO ( LVH)
Retinal Examination
Albumin:Creatinine first AM
Urinalysis
(ECG)
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43
Q

A patient has a murmur auscultated at the left sternal border with a normal S1, large split S2. What are you likely to find on the ECG?

a) Signs of RV overload
b) Signs of LV overload
c) Prolonged PR interval
d) Left bundle branch block

A

a) Signs of RV overload

Large split S2 is delayed closure of PV from extra volume going to R side
ASD will be most common
ECG in ASD will often show signs of RV overload

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44
Q

Romano-Ward and Jervell-Lange-Neilsen are 2 syndromes associated with what?

A

Long QT

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45
Q

A baby presents at several days with lethargy, decreased peripheral pulses. His heart rate is 180. His gas is bad. What do you do next:

a. broad spectrum antibiotics
b. prostin infusion
c. give IV carnitine
d. Intubate

A

b. prostin infusion

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46
Q

List 5 diagnostic criteria for Kawasaki disease.

A
  • 5 days of fever
  • bilateral non purulent conjunctivitis
  • lymphadenopathy >1.5cm
  • swelling/redness of hands or feet
  • mucosal involvement (strawberry tongue, cracked red lips, pharyngitis)
  • rash (not vesicular, bullous, petechial)
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47
Q

Preadolescent girl presents with a history of syncope during exertion. ECG with QT of 0.8seconds and RR of 1.2 seconds and notched (ie., bifid) T-waves.

Diagnosis and 2 next steps in management

A

QTC = QT / √ RR
= 0.73

She has LQTS
A QTc >0.47 highly indicative, QT >0.44 suggestive

Mgmt:

  • Exercise limitation, no swimming
  • Stop any QT prolonging drugs
  • Check electrolytes (K, Ca, Mg)
  • Screen Family members
  • Cardiology referral for medical management with propranolol or defibrillator
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48
Q

What are some medications that can cause long QT?

A
  • antibiotics: erythro/clarithro/azithro, septra, fluoroquinolones
  • TCAs (Amitriptyline), SSRIs (CITALOPRAM)
  • antipsychotics: haldol, risperidone, chlorpromazine
  • lasix
  • ondansetron, domperidone
  • antifungals
  • antihistamines
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49
Q

First line treatment of a child with asystole:

A

epinephrine (PEA or asystole - not a shockable rhythm)

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50
Q

What is the most common physical examination finding in congenital cyanotic heart disease?

A

Most often asymptomatic.

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51
Q

Concerning complete AV block, all are true EXCEPT:

a) may be a cause of syncopal episodes
b) ventricular rate of 30-60 bt/min
c) if you hear a systolic murmur then it is associated with a congenital heart lesion
d) diagnosis is confirmed by ECG
e) may be present in infants born to mothers with SLE

A

c) if you hear a systolic murmur then it is associated with a congenital heart lesion

Congenital complete AV block in children usually autoimmune injury to fetal conduction system caused by maternall derived antibodies (anti-SSA/RO, anti-SSB/La)
- Autoimmune disease cause of 60-70% of cases, and ~80% with structurally normal hearts

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52
Q
  1. Management of hypercholesterolemia (+Fhx)
A

Fam Hx includes MI/stroke before age 55, CAD, peripheral vascular disease, coronary intervention, parent with hyperlipidemia (>6.2 - 99% of people with LDL >6.2 have FH)

  1. evaluate for secondary causes: obesity, hyper/hypothyroid, hypercortisol, diabetes, biliary cirrhosis, nephrotic syndrome, meds (cyclosporine, estrogen, isotretinoin (accutane))
  2. diet and lifestyle modifications:
    - all kids over 2 should follow step 1 diet (<10% calories from sat fat, <30% calories from fat, <300mg/day cholesterol)
    - if patient’s fasting LDL is high, should switch to step 2 diet (<7% calories from sat fat, <200mg cholesterol)
    - refer to dietician for assistance
    - 60 minutes of mod-vigorous activity daily
    - re-evaluate in 6 months and if still high then start statin
  3. medication therapy: statins, bile acid resins
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53
Q

15 y o boy for regular check up and exam shows Ht > 95 th , Wt 50%, arm span > ht, +
pectus, flat feet. What is the most likely cardiac defect that could be found.

a. Mitral valve prolapse
b. Bicuspid Ao valve
c. Dilatation Ascending aorta
d. VSD

A

Dilatation of aortic root
Dissection of ascending aorta
Also (involvement, not major criteria): mitral valve prolapse

c. Dilatation Ascending aorta
a. Mitral valve prolapse
* either could be correct

Dilatation of the aorta is found in approximately 50% of young children with MFS and progresses with time (60-80% of adults)
Mitral valve prolapse (MVP) is frequently identified in patients with MFS (eg, 40 and 54 percent in two series of MFS patients, however it is a nonspecific feature and most patients with MVP do not have MFS

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54
Q

Teen is at a concert and feels dizzy and then passes out briefly. You suspect that this is vasovagal syncope.

List 4 signs and symptoms that would make you suspicious for a cardiac origin of syncope.

A
  1. Syncope mid exertion
  2. Syncope precipitated by loud noise or extreme emotion
  3. Syncope while supine
  4. Syncope w/o prodrome
  5. Family history of sudden death
  6. Abnormal Cardiac exam (abn HR, extra heart sound, poor pulses)
  7. Other cardiac symptoms: CP, palpitations, dyspnea
  8. History of heart disease/KD
  9. Syncope required CPR
55
Q

How do you treat long QT syndrome?

A

Beta blockers (to blunt HR response to exercise) - propranolol and nodal used

  • may then need pacemaker for drug induced bradycardia
  • if still syncopal on beta blockers or have had cardiac arrest, need ICD
56
Q

Nitroprusside
Nitroglycerine

  1. What is the effect on contractility (increased, decreased, none)?
  2. What is the effect on systemic vascular resistance (increased, decreased, none)?
  3. What is the effect on HR?
A

Contractility: None

SVR: Nitroprusside is an arterial dilator (decreased)
Nitroglycerin is a venous vasodilator (decreased)

HR: None

57
Q

Murmur at left mid-lower sternal border, otherwise well. What is it?
A) Innocent
B) Venous hum
C) Pulmonary regurg

A

A) Innocent

Venous hum - common innocent murmur, turbulence in jugular venous system. May be heard in neck or upper chest. Can disappear by change in head position.

Innocent - most common medium pitched vibratory or musical, left lower and midsternal border with no significant radiation. Softer when sits up.

58
Q

CHD with reverse differential sats.

A

Legs Pink - Arms Blue
Preductal < Post Ductal

TGA with CoArct or Interrupted Aortic Arch - oxygenated blood from pulmonary circulation enters descending Ao through PDA

59
Q

A newborn baby has tachypnea and cyanosis. With 100% O2 the O2sat improves from 80% to 85%.
On CXR the pulmonary vasculature is normal and there are no other abnormalities noted.

Which lesion is this most consistent with:

a. HLHS
b. TOF
c. Tricuspid atresia
d. TGA

A

c. Tricuspid atresia - decreased pulmonary vascular markings, no other CXR findings

HLHS - increased pulmonary vascular markings, pulmonary edema
TOF - decreased pulmonary vascular markings, boot shaped heart, 25% right aortic arch
TGA - increased pulmonary vascular markings, egg shaped heart

60
Q

A 14 year old boy was found unresponsive in a park the morning after the overnight temperature dropped to -3°C. He has been receiving resuscitation in the ER for 30

minutes. Which of the following would be an indication to stop the resuscitation?
a. Rectal temperature of 30°C
b. Barbiturates found on toxicology screen
c. Refractory ventricular fibrillation
d. Electromechanical dissociation / PEA

A

d. Electromechanical dissociation / PEA

Temp - needs to be warmed up
Drugs - need to wait for effects to wear off or reverse if toxins are an explanation

61
Q

A newborn presents with hypocalcemia. On history, you note frequent regurgitations, including up through his nose. On examination, he has an atypical facies and a heart murmur. What test will confirm the most likely diagnosis?

a) FISH 22q11
b) CGH microarray
c) Karyotype
d) Western Blot

A

a) FISH 22q11

DiGeorge

62
Q

An ECG is shown. Left axis deviation, increased forces, ST-T changes with T-wave inversion.

a) LVH
b) RVH
c) heart block
d) Wolff-Parkinson-White
e) ST-T changes associated with digoxin therapy

A

a) LVH

changes in keeping with LVH:
- depression of ST segments and inversion of T waves in left precordial leads (V5, V6) - left
ventricular strain pattern - suggest presence of a severe lesion
- deep Q wave in left precordial leads
- deep S wave in V1
- tall R wave in V6

63
Q

Infusion rate of prostaglandin

A

Prostaglandin E1 at 0.01-0.2ug/kg/min

64
Q

What are causes of atrial fibrillation?

A
PIRATES
Pulmonary Embolus or Pulmonary Disease (COPD)
o Ischemia
o Rheumatic or Regurgitation (mitral)
o Anemia or Atrial Myxoma
o Thyrotoxicosis or Toxins
o Electrolytes or Ethanol
o Sepsis or Stimulants
65
Q

CHD with DECREASED pulmonary blood flow

A

Tricuspid Atresia
Pulmonary Atresia
Tetralogy of Fallot
Ebstein’s Anomaly

CXR is black

66
Q

A teenager is being evaluated for his heart “Skipping a beat”. Two of his uncles died of “heart attacks” in their 30’s.
On examination, he is 97% for height and weight. He has a systolic murmur over the LLSB that gets louder when he stands. What investigation is most likely to reveal the cause?

a. 24 hour Holter monitor
b. Echocardiogram
c. ECG
d. CXR

A

b. Echocardiogram

An electrocardiogram (ECG) should be performed in all patients when considering a diagnosis of HCM. ECG testing is the most sensitive routinely performed diagnostic test for HCM, but the ECG abnormalities are not specific to HCM and should prompt further diagnostic evaluation, usually with echocardiography.

(UTD)

67
Q

7 y with viral URTI 2 weeks ago. Now presents in CHF and a maculopapular rash. What is the most likely diagnosis?

a. Viral myocarditis
b. Acute rheumatic fever
c. Subacute bacterial endocarditis

A

a. Viral myocarditis

Myocarditis
viral most common etiology
Patients may be asymptomatic, have nonspecific prodromal symptoms, or present with over CHF, arrhythmias or death

68
Q

An infant is referred for evaluation of a heart murmur heard on day 2 of life. Birth history is unremarkable. Feeding well. Good colour and perfusion with RR 50. Grade II/VI systolic murmur heard at the left sternal border. Your plan:

a) send back to family doctor with no follow-up
b) follow closely
c) cardiology consult
d) urgent echocardiogram
e) start prostaglandin infusion

A

b) follow closely

  • most likely pulmonary flow murmur of newborn
  • systolic murmur (early to mid systolic)
  • loudest at LUSB, with radiation to axilla and back
  • grade 1-2/6
  • blowing quality
  • usually disappears by 1-2 months
69
Q

A child comes in with episodes of syncope after exercise. Her sibling died of SIDS at 2 months old. What is the ECG finding that most helps you with a diagnosis?

a) widened QRS
b) prolonged QTc
c) prolonged PR
A

b. Prolonged QTc

70
Q

What are 2 syndromes associated with long QT?

A

Romano-Ward

Jervell-Lange-Nielsen (associated with congenital SNHL)

71
Q

Stimulation of beta-1 receptor

A

Located in the heart, increased inotropy and chronotropy

72
Q

A 3 year old child is referred to your office after a murmur is picked up by the family doctor on routine physical examination. You hear a harsh continuous murmur in the right infraclavicular area that is loudest when sitting and disappears on lying flat. The remainder of her cardiac exam
is within normal limits.

What is this most consistent with:

a. PDA
b. Venous hum
c. Still’s murmur

A

b. Venous hum

venous hum: soft humming sound heard in both systole and diastole; it can be exaggerated or made to disappear by varying the position of the head, or it can be decreased by lightly compressing the jugular venous system in the neck

Still’s murmur is an innocent murmur: vibratory or musical quality and is typically louder when the patient is supine or if there is a hyperdynamic state

PDA: murmur is continuous (“machinery murmur”), grade 3/6 or less and is heard best in the left infraclavicular region. The intensity of the murmur is max

73
Q

3/6 systolic murmur with ejection click in left upper sternal border. Quiet S2. Kid is 3y/o.

a. PS
b. PDA
c. TET
d. Coarctation

A

a. PS

Pulmonary Stenosis: high pitched high grade murmur over pulmonic, radiate to L carotid, widely split S2 with decreased P2 +/- ejection click
Tetralogy of Fallot: Single S2, harsh systolic ejection murmur of PS.
PDA: continuous machine like murmur

74
Q

Dobutamine

  1. What is the effect on contractility (increased, decreased, none)?
  2. What is the effect on systemic vascular resistance (increased, decreased, none)?
  3. What is the effect on HR?
A

Contractility: Increased
SVR: Decreased
HR: Increased ++

(Dobutamine is a derivative of dopamine, has reduction in SVR - no vasoconstriction)

Beta 1 (inotropy/chronotropy) and Beta 2 (bronchiodzaltion, vasodilation)

Dobutamine - Do(uble)Betas, get both of the betas.

75
Q
Infant with CHF. Liver 5cm. Pansystolic murmur. Increased HR, increased wob.
What to do?  
a) lasix
b) digoxin
c) beta blocker
A

a) Lasix

  • Diuretics are typically first line therapy
  • ACEi/ARBs - reduces afterload, benefical in CHF secondary to cardiomyopathy and severe MR/Aortic Insuff or L>R Shunts. Usually used in conjunction with diuretics.
  • Digoxin - used to be mainstay - more of an adjunct with ACEi and diuretics
76
Q

Child with SVT weights 8 kg. Treat with:

a. synchronized cardioversion 4 J
b. synch cardiovert 16 J
c. unsynchronized cardiovert 4 J
d. unsynchronized cardioversion 16J

A

a. synchronized cardioversion 4 J

SVT : Vagal maneuvers if stable -> adenosine (0.1mg/kg up to 6mg, then 0.2mg/kg up to 12mg) -> synchronized cardioversion starting at 0.5J/kg then up to 2J/kg

77
Q

What is most common ECG finding in acute rheumatic fever?

a. Wide QRS
b. Peaked T waves
c. Prolonged PR
d. Q waves in lateral precordial leads
e. Sinus tachycardia

A

Prolonged PR

Sinus Tach

78
Q

Stimulation of the beta-2 receptor

A

Located in lungs and blood vessels of skeletal muscles

Bronchodilation and vasodilation

79
Q

ECG features of WPW and why do we worry about it?

A
  • short PR interval
  • slow upstroke of the QRS (delta wave)
  • increased risk of a fib leading to v fib and death
  • usually the heart is normal, but can be associated with HOCM or Epstein’s anomaly of the tricuspid valve
80
Q
  1. Which of the following pulse profiles matches the diagnosis given:
    a. pulsus alternans-constrictive pericarditis
    b. atrial fibrillation-hypothyroidism
    c. pulsus bigeminus-digoxin toxicity
    d. dicrotic pulse-mitral stenosis
A

c. pulsus bigeminus-digoxin toxicity

Pulsus Alternans (regular rhythm but varying volume of pulse)
- Myocardial failure (left), aortic stenosis, hypertension, asthma, large pericardial effusion

Pulsus Bigeminus

  • Two heartbeats close together followed by longer pause (normal heart beat then a premature beat) -conduction issue
  • Causes: HOCM, digoxin toxicity, hypothyroidism, K abnormalities

Dicrotic Pulse - two atrial for every contraction. Severe HF, Hypovol shock, Tamponade

81
Q

What genetic syndrome is MOST associated with a right aortic arch and tetralogy of fallot?

1) Down syndrome
2) Williams syndrome
3) Turner syndrome
4) DiGeorge syndrome

A
  1. Di George

Genetic syndrome will be found in 20% of TOF
22q11 = 28%, T21 = 13%, VACTERL = 12%, then others

Williams - supravalv stenosis

82
Q

Dopamine

  1. What is the effect on contractility (increased, decreased, none)?
  2. What is the effect on systemic vascular resistance (increased, decreased, none)?
  3. What is the effect on HR?
A

Contractility: Increased
SVR: Increased (At high doses only)
HR: Increased

beta 1 (alpha1 at high doses)

High dose dopamine is DOPE, they get all the 1s (alpha1beta1) vasoconstriction, increased HR, increased contractility

83
Q

First line Rx treatment of a child with SVT

A

adenosine 0.1 mg/kg (max 6 mg) (push followed by NS flush)

84
Q

Picture of narrow complex tachycardia, no palpable pulse with poor perfusion:

a. Carotid massage
b. Asynchronous cardioversion
c. Adenosine
d. Verapamil

A

SVT with poor perfusion - go right to synchronized cardioversion as opposed to adenosine

If you’re in PEA - CPR… EPI… CPR… EPI

85
Q

A child received a large transfusion for a splenic injury. What is the ECG change most related to the complication he has?

increased QTc
QRS changes
Peaked T wave
U wave

A

Peaked T wave

→ hyperkalemia from transfusion (and hemolysis from splenic injury)

86
Q

When is endocarditis prophylaxis indicated?

A

When undergoing a dental procedure which manipulates the gums or perforates the oral mucosa, incision or biopsy of resp mucosa (tonsillectomy or adenoidectomy, bronch with biopsy), never for GI/GU procedures:

  • prosthetic cardiac valve
  • previous IE
  • unprepared cyanotic congenital heart disease (including partially repaired like palliative shunts)
  • completely repaired CHD with prosthetic material or device within 6 months of surgery
  • repaired CHD with residual defects adjacent to prosthetic material
  • heart transplant recipients with valvulopathy
  • rheumatic heart disease if prosthetic valve or material used in repair
87
Q

An 11 month old child with PEA. What to give:

  1. Epi 1cc of 1:1000 IV
  2. Epi 1cc of 1:10000 IV
  3. Atropine
  4. Amiodarone
A
  1. Epi 1cc of 1:10000 IV (0.01ml/kg)

PEA is not shockable
Start CPR and Epi 0.01 mg/kg (0.1ml/kg of standard 1:10,000 concentration)
Repeat q3-5 mins

88
Q

Neonate with complete heart block. ECHO normal. List 2 causes.

A

o Maternal SLE
o Maternal Sjogren Syndrome
o Idiopathic familial congenital CHB

Structural associations if echo was not normal:
ASD (mutation in homeobox gene described w/ ASD lesions)
o Post VSD closure complication
o Abnormal development of conduction system
o Myocardial Tumour
o Myocarditis
o Complication of myocardial abscess secondary to endocarditis

89
Q

What are secondary causes of PPHN?

A
Abnormal pulmonary vascular development (pul resistance up)
- Chronic fetal hypoxia/Asphyxia
- Maternal DM
- Alveolar Capillary Dysplasia
Pulmonary Hypoplasia:
- Congenital Diaphragmatic Hernia
- Potter’s Syndrome
- Prolonged Oligohydraminos
- Renal agenesis
Post-natal elevation in pul vasoconstrictors
- Sepsis
- Pneumonia
- MAS
- Perinatal Asphyxia
90
Q

What are the components of the systemic score in Ghent criteria when evaluating for Marfan syndrome?

A

Wrist and thumb sign
Pectus carinatum/excavatum
Flat foot
Spontaneous pneumothorax
Dural ectasia (widening of sac around spinal cord)
Protrucio acetabluae (displacement of acetabulum and femoral head)
Scoliosis, thoracolumbar kyphosis
Reduced elbow extension
Skin striae
Myopia
Mitral valve prolapse
Reduced upper:lower segment or increased arm span:height
3/5 facial features: dolicocephaly, downward palpebral fissures, enopthalmus, retrognathia, malar hypoplasia

Family Hx

91
Q

2 year old with signs of CHF. EKG looks like SVT. Give 3 things that would suggest SVT on EKG

A
  1. HR >220
  2. Absent or abnormal p waves (abnormal axis)→ “absent” more common in neonates
  3. Unvarying rate
    Also: narrow QRS
92
Q

Child with classic physical features of Marfan’s syndrome.

  1. How is this condition inherited?
  2. Name two life threatening cardiac conditions that it is associated with.
  3. Name 2 things you would do/follow regularly
A
  1. Autosomal dominant (75%), de Novo FBN1 variant 25%
    • Aortic rupture, Aortic aneurysm, Aortic dissection
      - Pulmonary HTN (due to MVP)
      - Supraventricular arrhythmias
      - Ventricular dysrhythmias
      - Prolonged QT
  2. Yearly Optho
    Yearly Echo
    Assessment for scoliosis
    PFTs
93
Q

Vasopressin

  1. What is the effect on contractility (increased, decreased, none)?
  2. What is the effect on systemic vascular resistance (increased, decreased, none)?
  3. What is the effect on HR?
A

Contractility: No Change
SVR: increased ++
HR: No change

v1 receptor agonist

94
Q

Newborn with persistent bradycardia. ECG given. Looks like heart block… identify the problem. What 2 things is this child at risk for?

A

Congenital heart block usually secondary to maternal autoimmune disease (i.e. SLE, Sjogren’s RA). At risk for:

  1. Cytopenias → anemia, leukopenia (less TCP)
  2. Light sensitive annular or macular rash
  3. Hepatitis
  4. Hydrops
  5. SIDS
95
Q

In what conditions do you see pulsus paradoxus?

A
  • cardiac tamponade, asthma, constrictive pericarditis, pericardial effusion
96
Q
  1. What is most characteristic of a Still’s murmur:
    a. vibratory murmur
    b. increases with sitting
    c. often radiates to axilla
A

a. vibratory murmur

Louder when lying
LLSB toward apex
Minimal radiation
Muscial quality
Louder in hyper dynamic states (fear, anxiety, fever)
Usually resolves by adolescence
97
Q

Two heart lesions with a single S2.

A
  1. TGA
  2. Pulmonary atresia
  3. Truncus arteriosis
98
Q

Features of Pathologic Murmurs

A

Grade =>3
Holosystolic
Harsh or blowing
Diastolic
Abnormal S2 - wide + fixed, or single prominent (pulm S htn)
Maximum intensity at LUSB - esp with abnormal S2 or other sounds
Systolic click
Increased intensity when upright (innocent murmurs usually diminish)
Gallop rhythm (S3)
Friction Rub

99
Q

ECG for WPW:

  1. What are the ECG abnormalities?
  2. What is the underlying diagnosis
  3. What is the most common arrhythmia
  4. If recurrent symptoms, how can this be managed
A
  1. QRS Delta wave
  2. Wolf-Parkinson-White
  3. SVT
  4. Accessory pathway ablation
    Medical Mgmt: beta-blockers

(Ca++ blockers, Digoxin are CONTRAINDICATED)

100
Q
3 day infant cyanosis with crying, investigation
a ECG
b CXR
c ABG
d bld cx
e echo
A

e echo (best test)

First test = CXR

Worry about cyanotic heart disease, especially TOF given cyanosis with crying

101
Q

Norepi and Phenylephrine

  1. What is the effect on contractility (increased, decreased, none)?
  2. What is the effect on systemic vascular resistance (increased, decreased, none)?
  3. What is the effect on HR?
A

Contractility: Increased
SVR: Increased ++
HR: Increased

Mainly alpha agonists, minimal effect on HR but ++ increased SVR

B1+A1&raquo_space; B2

102
Q

What are ECG changes in keeping with RVH?

A
  • right axis deviation
  • *note in first week of life need serial ECGs to determine if there is RVH beyond what is
    physiologically normal for a neonate (right ventricular dominance)
  • tall R waves in V1
  • deep S waves in V6
  • upright T waves in V1 and V4R
103
Q

4 risk factors for atherosclerosis.

A

high risk

  • DM
  • CKD/ESRD/post-renal transplant
  • heart transplant recipient
  • KD with current aneurysm
  • Familial hypercholesterolemia
  • Childhood Ca Survivor

moderate risk

  • Obesity
  • HTN
  • Dyslipidemia
  • Kawasaki disease with regressed coronary aneurysms
  • chronic inflamm disease (e.g. JIA, SLE)
  • HIV
  • nephrotic syndrome
  • adolescent depressive and bipolar disorders

others: smoking, diet, physical inactivity, family history

104
Q

You are seeing a toddler for a routine visit in your office. On auscultation of the chest, you note a subclavicular murmur that is loudest when the child is sitting. The most likely cause of this murmur is:

a) Venous hum
b) VSD
c) ASD
d) Aortic stenosis

A

a) Venous hum

b) VSD → pansystolic murmur best heard at LSB
c) ASD → SEM at LUSB, fixed widely split S2

105
Q

Child with cyanosis, O2 sat 80% doesn’t improve with oxygen. Pansystolic murmur grade III/VI. CXR normal and ECG shows right axis deviation. What is the diagnosis?

A

Depends on age

Could be TOF or
VSD (with high pulm pressures). Could also be a tricuspid atresia

106
Q

A child is diagnosed with a hypertrophic cardiomyopathy. After counseling the family regarding this new diagnosis and its potential impact on the family, which of the following therapies will you begin?

a) Labetalol
b) Furosemide
c) Digoxin
d) Captopril

A

A) Labetalol

Management of HOCM:
Competitive sports and strenuous physical activity should be prohibited as most sudden deaths occur during or immediately after vigorous physical exertion
Beta-adrenergic blocking agents (propranolol, atenolol) or CCB (verapamil) may be useful in diminishing ventricular outflow tract obstruction, modifying ventricular hypertrophy and improving ventricular filling

107
Q

Kid with down’s syndrome and previous CHD repair, now many years later going for
surgery. List 2 considerations

A
  • potential C spine instability (atlanto-axial)
  • Tracheal stenosis + subglottic stenosis more common
  • Hx of OSA -> pulmonary vascular disease
  • Dysrhythmias + bradycardia (esp during induction)
  • current cardiac condition and need for endocarditis prophylaxis
  • risk of pulmonary hypertension
  • risk of heme abnormalities
  • Risk of Reflux
  • suggested screening: ECG, echo, CBC
108
Q

Initial management of an infant with suspected TGA?

A

Start PGE1 - may need balloon atrial septostomy if not responding

109
Q
  1. Newborn diagnosed with interrupted aortic arch, what to start?
    a. dopamine
    b. prostaglandin
    c. nitric oxide
    d. indomethacin
A

b. prostaglandin

110
Q

Long term complications of PPHN?

A

hearing loss, developmental delay, intellectual disability

111
Q

Name 3 EKG finding of Hyperkalemia.

Name 3 ways to treat hyperkalemia

A

Three findings on ECG:

  1. Peaked T waves
  2. Lengthening of PR interval
  3. Wide QRS
  4. Flat P waves
  5. Sine wave

Mgmt:

  1. Calcium gluconate 10% solution, 1ml/kg IV over 3-5mins
  2. NaHCO3 1-2mEq/kg IV over 5-10 mins
  3. regular insulin 0.1U/kg with 50% glucose solution 1ml/kg over 1 hour
  4. Ventolin (5mg neb)
  5. Kayexalate
  6. Consider if dialysis needed
112
Q

What are the features of a venous hum on physical exam?

A

– Whining, roaring, whirring Gr 1-6, continuous, systolic/diastolic murmur (3-8y)
– Supra/infraclavicular (R>L)
– Sitting or standing (disappears when supine or applied pressure to jugular vein, or with rotation of head)
– Caused by blood cascading down jugular vein, louder in diastole as atrium empties
– DDx PDA

113
Q

Patients with ASDs have a fixed split S2. This is due to:

a) pulmonary hypertension
b) abnormal pulmonary valve
c) pooling of blood in the pulmonary vasculature
d) late eruption of the right atrium
e) prolonged right ventricle ejection

A

e) prolonged right ventricle ejection

Most often asymptomatic; lesion often discovered inadvertently during physical examination

  • characteristic finding: widely split fixed S2 during all phases of respiration
  • RV diastolic volume is constantly increased with ASD (no variation breath to breath)
  • Systolic murmur (due to increased flow across RV outflow into pulmonary artery)
  • Short, rumbling mid diastolic murmur - increased flow across tricuspid valve at LLSB
114
Q
  1. Which of the following are true?
    ( a) fetal p02 is 25-30
    (b) the incidence of asymptomatic PFO in the adult population is 10%
A

( a) fetal p02 is 25-30
(Umbilical vein pO2 - highest level of O2 to fetus is 30-35mmHg)

*15-25% of adults have asymptomatic PFOs

115
Q

What is the most common cyanotic cardiac disease in the newborn period?

A

TGA
● Cyanosis is apparent within the first few hours of life and is not responsive to oxygen.
CXR: egg on a string

116
Q
  1. 6 wk old with pansystolic murmur, increasing respiratory distress and liver edge down.
    CXR shows increased pulmonary markings. Which medication would you consider to
    help his symptoms?
    a. propanolol
    b. furosemide
    c. digoxin
    d. adenosine
A

b. furosemide

117
Q

List 3 causes of cyanotic congenital heart disease with decreased pulmonary blood flow

A
TOF
tricuspid atresia
pulmonary stenosis
Ebstein anomaly
pulmonary atresia
118
Q

Newborn baby with cyanotic congenital heart disease. Most consistent physical exam finding:

a) bounding/dynamic precordium
b) normal pulses and quiet precordium
c) decreased pulses and poor perfusion
d) tachypnea and nasal flaring
e) palpable thrill

A

b) normal pulses and quiet precordium

o Cyanosis- most have no murmur and no distress
▪ TGA, pulm or tricuspid atresia

119
Q

Name 3 types of benign murmurs in children.

Name 4 features on PE of a benign murmur

A

Stills
Venous Hum
Peripheral pulmonary stenosis
Carotid bruit

  • Grade < 3
  • Systolic
  • Soft, vibratory, musical
  • No extra heart sounds
  • Lessens in intensity upon sitting (louder supine)
  • No abnormal growth, no dysmorphisms
120
Q

First line treatment of a child with pulseless Ventricular tachycardia.

A

Defibrillate (unsynchronized) with 2J/kg then 4 J/kg

121
Q

A teenager is seen in the ER with shortness of breath. He has distended neck veins,hepatomegaly and an S3 and an S4. What are TWO abnormalities on this CXR?
(Cardiomegaly and a left sided pleural effusion). What are TWO possible diagnoses?

A

Findings on CXR in right sided heart failure
1. Cardiomegaly
2. Prominence of the pulmonary veins in the hilar region
3. Enlarged right atrium & ventricle
4. Loss of pulmonary vasculature in the periphery
5. Pleural effusion
DDx - likely acquired in a teen:
1. myocarditis
2. cardiomyopathy (hypertrophic or dilated)
3. infective endocarditis (acquired valve disease)
- acute HTN (glomerulonephritis)
- thyrotoxicosis
- sickle cell anemia

122
Q

Child with supravalvular aortic stenosis, prominent lips, developmental delay, and hypercalcemia. This is indicative of:

  1. DiGeorge
  2. Williams
  3. Noonans
  4. Downs
  5. Fetal alcohol syndrome
A
  1. Williams

o Supravalvular Aortic stenosis

  • Intellectual disability
  • Elfin facies
  • Idiopathic hypercalcemia of infancy

DiGeorge - Conotrucal (TOF, Pulm Atresia w/ VSD, DORV, TA, TGA)
Noonan - Pulmonary Stenosis
T21 - AVSD

123
Q

Neonate with PDA treated with indocid. List four side effects of indocid

A
Indocid= Indomethacin
● Decreased platelet function: GI bleed, IVH, pulmonary hemorrhage
● NEC
● Acute Kidney Injury
● Spontaneous GI perforation
●ROP
124
Q

What congenital cardiac defect is associated with Williams syndrome?

A

supravalvular aortic stenosis

Other features:
▪ ID, cocktail party personality, elf-like facies,hypercalcemia
▪ Narrowing of peripheral systemic and pulmonary arteries (may have hypertension)

125
Q

21 day old boy with 7 days of reduced feeding presenting with lethargy. HR 160 BP 45/30; bolus NS being given. Na 110 K 9.5 glucose 3.5. EKG shows peaked T waves.

a. List two key initial treatments for hi hyperkalemia
b. What is the most likely Dx
c. What one specific empiric therapy is indicated at this point

A

a. Calcium Gluconate
Ventolin
Insulin + glucose

b. CAH
c. corticosteroid infusion

126
Q

Neonatal goiter. What anti-arrhythmic was mom on?

a. Digoxin
b. Procainimide
c. Amiodarone
d. Sotalol

A

c. Amiodarone

Congenital goitre: Fetal T4 defect or maternal antithyroid drugs/ iodides - mom and baby have hypothyroid
● Amiodarone (37% iodine content) can also cause congenital goiter with hypothyroidism

127
Q

Which type of heart block is most concerning for progression to complete heart block?

A

Second degree, type II (mobitz II): no progressive prolongation of PR interval; a P wave is predictably non-conducted in a specific pattern (e.g. every 2 beats, every 3 beats)

128
Q

Child with confirmed hypertension. Urinalysis, lytes, BUN, Creatinine are all normal.

Name 4 investigations you need to do:

A
Echocardiography
Retinal exam
First AM Albumin-to-Creatinine 
Renal US
Fasting blood glucose
Serum total cholesterol, HDL and LDL, Triglycerides

± TSH
± CBC (esp. growth delay or abn renal function)
± Sleep study

129
Q

Stimulation of alpha-1 receptor

A

Arterial vasoconstriction, increases systemic vascular constriction

130
Q

4 week in obvious CHF. Huge voltages on EKG, short pr interval. Hypotonic and progressive weakness. Hepatosplenomegaly and large tongue.

What does this child have?
Treatment?

A

Pompe Disease

  • Example of inherited form of hypertrophic cardiomyopathy
  • Type 2 glycogen storage disease

Treat with enzyme replacement

Presents in early infancy with hypertrophic cardiomyopathy, shortened P-R interval on ECG, and skeletal and respiratory muscular weakness; get elevated CK
Heart failure, respiratory insufficiency and death without treatment

131
Q

Child with HTN. Best test for long standing hypertension.

ECHO
Fundoscopy
EKG

A

ECHO

132
Q

Baby boy born at term with no problems. You’re seeing him at 6 weeks now, mom had noticed he is not feeding well. You note he has been gaining weight poorly. Exam is remarkable for a harsh pansystolic murmur at the left sternal border, radiating to the right.

A) What is the diagnosis?
B) Explain the reason he is presenting with these symptoms now. (2 points)

A

a) Ventricular septal defect

b) Pulmonary pressures were initially high, as PVR falls there is more shunting of blood from left to right

133
Q

CHD with INCREASED pulmonary blood flow

A

VSD, ASD, endocardial cushion defects
PDA
Truncus Arteriosus
TAPVD

(White CXR)