Cardiology

Question 1

6-year-old girl sees you in follow-up for a small perimembranous VSD. Her BP is 125/something. There is a grade II/VI regurgitant systolic murmur at the LLSB and a grade II/VI systolic ejection murmur at the LUSB that can be auscultated at the back. What is the next best step?
Echo
4 limb BP
24 hour AMBP
ECG

Answer 1

Echo looking for consequences of VSD

Question 2

You are called to see a 2-day-old infant with a murmur that had not been heard before today. Baby has been feeding well and growing. On examination, there is a grade II/VI systolic murmur at the LUSB. What is your next step?
Send back to the family doctor because clearly (LOL definitely said clearly) this is a benign murmur
Follow very closely but no further intervention needed right now
Echo
ECG

Answer 2

follow closely–benign peripheral pulmonic stenosis
flow into pulmonary branch arteries from main larger artery
LUSB
neonates, low pitched, radiates to axilla and back
usually disappears 3-6months
Differential includes PDA and pulmonary stenosis

Question 3

You started PGE-1 for concerns of a ductal dependent cyanotic cardiac lesion. What is the most important side effective to monitor for?
Hypoglycemia
Hypoventilation
Hypothermia
Hypocalcemia

Answer 3

Hypoventilation
Also causes hypotension

Question 4

A 6 year-old child has a history of TAPVR that was fully repaired as an infant. They are now in grade 1 and you have diagnosed them with ADHD. In addition to a thorough history and physical examination what do you need to do before prescribing stimulant medications?
Stimulants contraindicated
Prescribe stimulant
ECG
Echo

Answer 4

Prescribe stimulant
ECG is Dr Alsalehi’s answer

Question 5

A 15-year-old male with Marfan’s syndrome wants to begin weight-lifting. He has a normal cardiac exam. What do you suggest?
Echocardiogram before weight-lifting
Can participate in weight-lifting program
Can participate in light weight-lifting exercises
No aerobic exercise

Answer 5

Echocardiogram before weight-lifting
As per AAP, look for aortic dilation and mitral valve regurg to determine what activity they can participate in.
Echo at diagnosis, 0-12 months as indicated, yearly from one year onwards

Question 6

A 14-year-old male has stage I hypertension that has persisted despite lifestyle modifications. His BMI is 50%. His parents both have essential hypertension. His family physician has already ordered the following investigations: creatinine, urea, urinalysis, urine albumin to creatinine ratio, lipids and HbA1c. What else do you need to order?
Renal ultrasound with doppler
Abdominal ultrasound
Echo
24-hour AMBP

Answer 6

24 hr ABPM

Question 7

Normal result on CCHD screen

Answer 7

> =95% in right hand OR foot and <3% difference between right hand and foot

Question 8

Abnormal result on CCHD screen

Answer 8

<90% in right hand or foot

Question 9

Borderline result in CCHD screen

Answer 9

90-94% in right hand and foot OR >3% different between right hand and foot

Question 10

A 24 hour old term infant has their congenital heart disease screen. The right arm SpO2 is 94% and left foot SpO2 is 96%. What do you do?
Repeat in 1 hour
Urgent cardiology referral
No further assessment
SpO2 of left hand and left foot

Answer 10

No further assessment. Pass if >= 95% in right hand OR foot AND <3% difference between right hand and foot

Question 11

A 12 year old female presents to the ED with dizziness. She is otherwise well and is awake and talking to you. Her ECG shows the following rhythm. (torsades)
What is your next best step?
Magnesium sulfate
IV Adenosine
Debrillate 2J/kg
Cardioversion 1J/kg

Answer 11

Mag sulf

Question 12

A neonate has a grade III/VI systolic murmur and hyperdynamic precordium. He is an infant of a diabetic mother. What do you advise the parents about his hypertrophic cardiomyopathy?
Resolves with steroids
It is usually treated with lasix
Resolves without treatment
Improves with labetalol

Answer 12

Resolves without treatment

Question 13

16 year old male who sometimes feels like his “heart is skipping a beat” and has had two paternal uncles who have passed away from “heart attacks” in their 30’s. He is at the 95% for height and weight. Normal exam with the exception of a Grade 2 systolic murmur, loudest at the left lower sternal border, louder with standing. His BP was 125/60. What test is most likely to reveal the diagnosis?
Echocardiogram
Holter
Exercise test
Lipids

Answer 13

Echo (HOCUM)

Question 14

A teen male has a 3 week history of worsening headache. He has a BP of 180/105. He has a history of renal scarring and asthma. On examination his neck was supple and his reflexes were brisk. What do you use to treat him?
IV nitroprusside
IV labetalol
SL nifedipine
PO amlodipine

Answer 14

IV nitro

Question 15

which CHDs require endocarditis prophylaxis for dental procedures

Answer 15

1. prosthetic cardiac valve or prosthetic material used for valve repair
2. previous endocarditis
3. unrepaired cyanotic, including palliative shunts and conduits
4. repaired cyanotic if surgery <6 months ago
5. repaired with residual leak (at site or adjacent to site) (forever)
6. cardiac transplant recipients with valvulopathy

Question 16

Which of the following requires endocarditis prophylaxis with dental extraction?
Unrepaired VSD
Unrepaired ASD
TGA repaired 1 year ago
VSD repaired 1 year ago with residual defect

Answer 16

VSD repaired 1 year ago with residual defect

Question 17

Normal result on CCHD screen

Answer 17

> =95% in right hand OR foot and <3% difference between right hand and foot

Question 18

What maneuver will make a HOCM murmur louder?
What maneuver will make it softer?

Answer 18

Valsalva will make it louder (decreased venous return to heart)
Squatting will make it softer (increased venous return to heart)

Question 19

what monitoring and how often should you do for patients with suspected HOCM?

Answer 19

AHA:
1st degree relatives of patients with HCM, ECG and echo q 1-2 yrs in adolescents and q 3-5 yrs in adults

Question 20

12 yo male with father who has HOCM. Father refuses genetic testing. What do you do?
Screen with ECG and echo every 5 years
Screen with ECG and echo every year until adulthood
Do genetic testing on child - if genetic testing negative can stop ECHO/ECG
Screen yearly for symptoms

Answer 20

screen with ecg and echo every 1 yr until adulthood

Question 21

6w/o with continuous murmur at LUSB, tachypnea, hepatomegaly. What’s the likely cause?
A- PDA
B- VSD
C- Coarct
D- Pulmonary Stenosis

Answer 21

PDA

VSD: LLSB, pan systolic
PS: crescendo decrescendo SEM

Question 22

Hypertension confirmed by ambulatory BP monitor. Now BP >95th percentile for age. Normal renal function.
Renal u/s
HVA, VMA
Start captopril
Repeat ambulatory BP - if change in status to confirm BP

Answer 22

Renal ultrasound

Question 23

Endocarditis ppx
Unrepaired TOF
VSD with leak 8 months out
VSD repaired 10 months ago

Answer 23

unrepaired TOF (cyanotic CHD)
not VSD with leak as leak must be adjacent to patch or prosthetic material

Question 24

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

Answer 24

Sync cardio version

Question 25

cardiac defect associated with DiGeorge Syndrome (22q11 deletion)

Answer 25

Aortic arch abnormalities: interrupted aortic arch, right aortic arch Conotruncal abnormalities: truncus arteriosis, TOF, pulmonary atresia with VSD

Question 26

CHD with T21

Answer 26

AVSD, VSD, PDA, anomalous subclavian artery

Question 27

CHD with trisomy 18

Answer 27

VSD, polyvalvular disease, coronary abnormalities

Question 28

CHD with trisomy 13

Answer 28

PDA, septal defects, pulmonic and aortic stenosis (atresia)

Question 29

CHD with William syndrome

Answer 29

supravalvular aortic stenosis pulmonary artery stenosis

Question 30

CHD with Turner syndrome

Answer 30

coarctation of the aorta, bicuspid aortic valve

Question 31

CHD with Noonan syndrome

Answer 31

Dysplastic pulmonic valve, ASD

Question 32

Cardiac defect with Marfan syndrome

Answer 32

Dilation of the ascending aorta/aortic sinus, aortic and mitral insufficiency

Question 33

cardiac defect in fetal alcohol syndrome

Answer 33

VSD

Question 34

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

Answer 34

DiGeorge

Question 35

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

Answer 35

blood pressure lying and standing

Question 36

Infant, vomiting unwell for a few days, with HR 260bpm, resp distress and RR70, poor pulses, liver 4cm below costal margin - cause? SVT Myocarditis heart failure Pompe disease Lactic acidosis

Answer 36

SVT

Question 37

24 hour old term baby presenting with tachypnea of 70/min and preductal saturation 90% and post ductal is 70%; the most likely diagnosis is: 1- TOF 2- Coarctation 3- TGA 4- truncus arteriosus

Answer 37

Coarct. Note others cause cyanosis but not differential cyanosis 3 causes of differential cyanosis: PDA with coarc or severe PPHN, interrupted aortic arch Reversed cyanosis: TGA or supra cardiac TAPVC

Question 38

13 yo male growing at 25% centile with BP 130/84 (average from 3 readings from three visits), what do you do? **I think it was average 3 readingsfrom THAT ONE visit** ambulatory BP Repeat BP in the next three office visits do lytes, ur, cr, US do lytes, ur, cr, US, echo, ECG

Answer 38

ambulatory BP (if 3 readings from 3 separate visits) If it was 3 readings from one visit would repeat in 3 more visits

Question 39

Kid with double right outlet ventricle and interrupted arch? Trisomy 21 Trisomy 13 Trisomy 18 DiGeorge

Answer 39

DiGeorge

Question 40

Define elevated blood pressure for age >=13

Answer 40

120/<80 to 129/80

Question 41

stage 1 hypertension in age >=13

Answer 41

130/80 to 139/89

Question 42

stage 2 hypertension age >=13

Answer 42

>=140/90

Question 43

Hypertensive crisis definition and plan

Answer 43

elevated BP with evidence of end organ effects send to ED

Question 44

What do you do if patient has stage 2 HTN at visit? (>=140/90)

Answer 44

First visit: Lifestyle mods and check 4 limb BP, repeat BP in 1 week Second visit: If BP remains high, diagnostic eval and subspec referral within 1 week

Question 45

MGMT for elevated BP and stage 1 HTN

Answer 45

First visit: lifestyle mods and repeat BP in 6 months (elevated) and 1-2 weeks (stage 1) Second visit: Check 4 limb BP and repeat BP in 6 months (elevated) and 3 months (stage 1) Third visit: If BP remains high ambulatory blood pressure monitoring + diagnostic evaluation +/- subspec referral

Question 46

Teenage girl with exercised-induced syncope. She has been diagnosed with long QT syndrome. What is your next step in management: Calcium channel blocker with high intensity exercise restriction Beta Blocker with high intensity exercise restriction Calcium channel blocker without high intensity exercise restriction Beta blocker without high intensity exercise restriction

Answer 46

Beta Blocker with high intensity exercise restriction As per nelson's: B-blocker first line treatment. Inplantable cardiac defibrillator if aborted cardiac arrest, syncopal events despite beta blockade, not compliance, or intolerance of medication. All patients are recommended to follow exercise restriction although those recommendations are changing.

Question 47

A child in grade 1 has a history of repaired TAPVR. He has been diagnosed with ADHD. After taking a thorough history and physical, your next step is: ECG ECHO Start a stimulant medication with no further investigation Stimulants are contraindicated

Answer 47

ecg

Question 48

Female newborn with puffy hands/feet, low hairline. Most likely cardiac lesion Bicuspid aortic valve Pulmonic stenosis TOF TAPVD

Answer 48

Bicuspid aortic valve (Turner syndrome) Pulmonic stenosis found in Noonan syndrome and Williams syndrome TOF--DiGeorge syndrome

Question 49

3 day baby with cyanosis, CXR shows large heart and decreased pulmonary markings. Diagnosis? 1. TGA 2. Truncus 3. TOF 4. TAVPR

Answer 49

TOF: pulmonic stenosis leads to diminished pulmonary blood flow and increased right to left shunting across the VSD (blue tet) TGA: deoxygenated blood returning from the body is sent to the aorta and to the body instead of the lungs, and oxygenated blood returning from the lungs is sent back to the lungs through the pulmonary artery (parallel circulations). Can lead to pulmonary hypertension. Truncus: Single common arterial trunk that supplies everything. As PVR drops blood will preferentially go to the pulmonary arteries leading to pulmonary over circulation and heart failure (pulmonary edema). TAPVR: pulmonary over circulation due to pulmonary veins going back to right sided circulation instead of left atria

Question 50

6mo with depressed nasal bridge and elfin facies. Labs show elevated calcium level. What is the most common associated cardiac condition? A) Endocardial cushion defects - T21 C) Supravalvar aortic stenosis D) Tetralogy of fallot

Answer 50

supravalvular aortic stenosis