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1

When does the PFO close functionally in most normal neonates?
a) within 48 hours
b) within 3 months
c) within 6 months
d) within 1 year

b) within 3 months closes functionally in most healthy neonates
can pass a probe through the overalapping flaps in large percentage of children and 15-25% of adults

2

What is the most important factor controlling the closure of the PDA in a full term neonate?
a) oxygen
b) CO2
c) amount of prostaglandin being released
d) increase in SVR

a) oxygen is the most important factor controlling ductal closure in the term neonate
when blood passing through the ductus has a paO2 of 50 mmHg, it causes the ductal awl to contract (effect may be direct or mediated by effects on prostaglandin synthesis)

3

How many infants with congenital heart disease have extra cardiac manifestations?
a) 5-10%
b) 10-20%
c) 20-45%
d) 50%

c) 20-45% of patients with congenital heart disease have extra cardiac manifestations
5-10% have known chromosomal abnormality

4

Which of the following conditions does not usually produce bounding pulses?
a) PDA
b) AV fistula
c) anemia
d) cardiomyopathy

d) cardiomyopathy has decreases pulses in all extremities
Nelson pg 1958
increased pulses with wide pulse pressure
- PDA, aortic insufficency, AV ommunication, increased cardiac output (from anemia, anxiety, increased catecholamine or thyroid)
Decreased pulses
- pericardial tamponate, LVOT obstruction, cardiomyopathy

5

Which of the following lesions does not have a wide splitting of S2
a) ASD
b) pulmonary stenosis
c) pulmonary hypertension
d) right bundle branch block

c) pulmonary hypertension

wide splitting - ASD, pulmonary stenosis, Ebstein anomaly, TAPVR, RBBB
narrow splitting - pulmonary hypertension with accentuated pulmonic component of second sound
Single 2nd sound: pulmonary or aortic atresia (or SEVERE stenosis), truncus arteriosus, transposition

6

Which of the following is unlikely to cause a pan systolic murmur?
a) tricuspid valve insufficiency
b) aortic valve stenosis
c) VSD
d) mitral valve insufficiency

b) aortic valve stenosis - systeolic ejection murmur, between 1st heart sound and 2nd, if severe, it CAN extend beyond the second heart sound
systolic ejection murmur - imply increased flow or steonis across ventricular outflow (aortic or pulmonic valve)
**clear 1st hear sound - most likely to be ejection murmur

vs.

pansystolic or holosystolic - behind WITH the 1st heart sound continue throughout systole, occasionally decrescendo.

to remember: after closure of AV valves (tricuspid/mitral) brief period where ventricular pressure increases but semilunar valves (aortic and pulmonic) stay closed) during isovolumic contraction THEREFORE pansystolic murmurs CANNOT be related to problems with aortic or pulmonic valves (semilunar valves)
instead, pan systolic murmurs caused by blood exiting the ventricle via a abnormal opening (VSD) or AV valve insufficiency (mitral/tricuspid)

7

Which of the following is not generally the cause of a loud murmur?
a) severe anemia
b) extra cardiac AVM
c) anomalous insertion of a coronary artery
d) myocarditis

c) does not usually cause a loud murmur (Nelson 1864)
loud murmurs without structural heart disease : i.e. non cardiac AVM, myocarditis, severe anemia or hypertension
may not have loud murmur in patients with severe aortic steonosis, ASD, anomalous pulmonary venous return, AVSD, anomalous insertion of a coronary artery, coarctation

8

Which of the following is the most common innocent heart murmur in children?
a) vibratory, musical Still's murmur
b) venous hum
c) pulmonary flow murmur
d) peripheral pulmonary stenosis

a) most common innocent murmur - medium-pitched, vibratory or musical stills murmur, relatively short systolic ejection murmur, heard best along left lower and mid sterna border, no radiation to apex, base or back
age 3-7 year old most common, intensity of the murmur often changes with respiration and position
less loud in the sitting or prone position (vs organic lesion usually louder when heart brought closer to the surface)

Innocent pulmonary murmurs: children/adolescents - high pitched, blowing brief early systolic murmurs grade 1-2 intensity, best detected in the 2nd left parasternal space

Venous hum: turbulence of blood in the jugular venous system, heard in the neck of anterior test , varies with position of head




more than 30% of children may have an innocent murmur at some point

9

Which of the following ECG findings is abnormal and needs further evaluation for cardiac anomaly?
a)newborn with right axis deviation
b) 2 month old with upright T waves in V1, V3R and V4R
c) newborn with upright T waves in V1, V3R and V4R
d) 7 year old with upright T waves in V1, V3R and V4R

b) upright T waves generally disappear by 48 hours, and should become downward by 1 week at the latest. Upright T waves in V1, V3R and V4R which reflect the right ventricle forces, are abnormal BEFORE age 6, and raises suspicion for RVH even without other voltage criteria
may remain negative into adolescence

10

Which of the following conditions is not associated with a broad P wave?
a) Ebstein anomaly
b) VSD
c) PDA
d) severe mitral regurgitation

a) Ebstein is associated with RIGHT atrial enlargement, which is shown by TALL P waves (>2.5 mm) which are narrow and spiked

left arial enlargement - broad P wave, including bifid P wave
causes -L->R shunt (VSD, PDA), mitral regurg/severe stenosis (because blood spills back into left atrium causing it to get bigger)

11

What is the normal axis of a newborn baby on ECG?
a) +150 to + 220 degrees
b) +110 - +180 degrees
c) +30 to + 120 degrees
d) +10 to +90 degrees

b) is the normal axis 110-180 degrees (according to Nelson, different in different sources)

+30 to +120 is for 1-3 month old
+10 to +90 is 3-6 month old

as kid gets older the axis gets more and more leftward as the pulmonary pressures fall and the pressures change

12

Which of the following factors does not increase the risk of cerebral vein thrombosis in patients with cyanotic heart disease?
a) iron deficiency
b) dehydration
c) hot weather
d) decreased dose of diuretic

d) decreased dose of diuretic

patients with R-L shunting cyanotic heart disease are at risk of thrombosis because of polycythemia (Hct >65%) especially of the cerebral veins
this risk increase with dehydration (so need to be careful in hot weather and with GI illnesses) and the risk can worsen with iron deficiency
may need to decrease dose of diuretic when their is concern of fluid intake

13

What causes the systolic murmur in an ASD?
a) increased flow across the tricuspid valve
b) flow across the ASD
c) increased blood flow via right ventricle outflow tract to the pulmonary artery
d) increased blood volume in right ventricle in diastole

c) increased blood flow via right ventricle outflow tract to the pulmonary artery causes the murmur, not the low-pressure flow across the ASD (Nelson 1884)

diastolic murmur produced by increased volume of blood across the tricuspid valve (left lower sternal border tricuspid area where it is heard best)
increased blood volume in right ventricle in diastole (vs normally with inspiration only) leads to fixed split S2

14

What are the EKG findings in Pompe disease?
a) long PR, low voltage QRS
b) short PR, high voltage QRS
c) LVH
d) none of the above

b) short PR, high voltage QRS
Echo shows hypertrophic cardiomyopathy

Pompe disease: glycogen storage disease in lysosomes
infantile form very severe/fatal
often presents with hypotonia, macroglossia, feeding difficulties, hepatomegaly
Labs: increased CK, AST, LDH
diagnosis: demonstrate acid alpha glucosidase deficiency

15

Which of the following syndromes is not commonly associated with pulmonary stenosis?
a) Noonan
b) Alagille
c) Trisomy 18

c) Trisomy 18
the other 2 are commonly associated with PS
- associated with Noonan syndrome – PS as a result of valve dysplasia
- associated with Alagille syndrome – PS of valve or branch pulmonary arteries

16

Which of the following findings is not associated with pulmonary stenosis with an intact septum in a 5 year old?
a) cyanosis
b) systolic ejection murmur loudest at the LUSB
c) pulmonic click
d) split S2
e) upright T wave in V1 in a 5 year old

a) cyanosis is not associated with PS even with small hole, as long as there is no septum defect available for shunting
ONLY in critical pulmonary stenosis (in the neonate) we may see some cyanosis because the PFO is around for shunting to happen

the rest are findings of PS
upright T wave in V1 - indicates RVH (should be inverted till about 6-8 year old)

17

Which of the following is the least likely finding of severe pulmonary stenosis with intact ventricular septum?
a) pulmonary hypertension
b) hepatomegaly and peripheral edema
c) tall spiked P waves on ECG
d) severity graded on the ratio of pulmonary pressure to the systemic pressure

a) pulmonary pressures are either normal or decreased - remember that because the hole is so small, not much blood can get through so the pressure can't be high

the others - LVR and peripheral edema can be from right heart failure (since the blood can't get through to the lungs it backs up into the RA and then the body)

do remember that findings can vary based on the severity of the lesion
treatment: usually ballon valvuloplasty

18

Which of the following about aortic stenosis is false?
a) if moderate -severe, no competitive sports
b) supravalvular AS is associated with Williams syndrome
c) can present in infancy with heart failure and shock like state
d) needs endocarditis prophylaxis prior to repair

d) does not as per the new guidelines (double check)
however if there was a prosthetic valve then would need

19

A child presents with peripheral pulmonary stenosis and hypercholesterolemia, which of the following is not associated with the likely syndrome present in this child ?
a) hypercalcemia
b) butterfly vertebrae
c) liver disease
d) growth defect

a) NOT hypercalcemia, this is part of Williams syndrome not Alagille

Alagille: Jagged 1 gene
liver problem and PPS
peripheral pulmonary stenosis , liver disease, hypercholesterolemia, peculiar facies, butteryfly vertebrae and growth defects
high cholesterol though to be from LVR disease

20

Which of the following statements is false?
a) blood pressure in the legs is normally 10-20 mmHg higher than in the arms
b) the femoral pulse is normally felt after the radial pulse
c) blood pressure should be done in BOTH arms to rule out coarct
d) with severe coarctation, one can have palpable femoral pulses and no BP gradient , as well as pulmonary congestion

b) FALSE - normally, femoral pulse is slightly before, in radio femoral delay (which happens in coarctation) femoral is felt after because the lower limbs blood supply is via collaterals, which are slower

the rest are true
c) because if the coarctation is in the region of the left subclavian, may have right arm BP>left arm

d) truein differential cyanosis in coarctation, RV blood goes via the ductus to supply the descending aorta, since blood is getting there, you will palpate femoral pulses and there may not be BP gradient, there will be a saturation gradient though because the lower limbs won't be getting as much O2 (pre ductal >post ductal)

remember AS (can be critical with shock like picture, severe heart failure) vs coarct (differential cyanosis and BP, decreased lower limb pules, can get heart failure signs; in infants if super severe can result in similar picture as AS with hypo perfusion, acidosis and severe heart failure )

21

Which of the following is not associated with PHACE syndrome?
a) posterior fossa brain anomalies
b) facial hemangiomas
c) arterial anomalies
d) coarctation of the aorta
e) ear anomalies

e) false - in fact it is eye anomalies
the others are features
can also have other cardiac problems
the risk with this syndrome is stroke

22

What are two causes of short PR

1. glycogen storage disease - i.e. Pompe
2. where you bypass AV node - i.e. WPW

23

Causes of prolonged QT

hypocalcemia
severe hypokalemia
prolonged QT syndrome
drugs - including quinidin erythromycin TCAs etc (look at detailed answer in questions)

24

true or false - digoxin is a good choice in WPW

no, digoxin is contraindicated in WPW

25

Treatment of 3rd degree heart block

depends on ventricular rate and presence of symptoms
often needs a pacemaker

26

most common congenital heart diseases

VSD 25%
ASD 10% (secundum is most common - at the PFO area), then primum (which is near AV canal) then sinus venous
PDA (5-10%, excluding prems)
pulmonary stenosis 10%
Aortic stenosis 5%
Coarctation - 10%
TOF - most common cyanotic - 10%
transposition - 5% (but most common to present in newborn out of the cyanotic lesions)
tricuspid atresia - 2%
truncus <1% of all cases
Hypoplastic left heart - 1% - but most common cause of death from cardiac defcects in first month

27

Findings in VSD

small VSD - normal ECG and CXR
large VSD: get increased volume on left side of the heart - Left atrial and ventricular enlargement and hypertrophy (since increased blood going to lungs via VSD and coming back to La and LV.
can get pulmonary hypertension from increased flow/PVR

28

how many VSDs close spontaneously

1/3 can close, small ones usually do
initial treatment for moderate to large: diuretics, after load reduction
surgery if poor growth or pulmonary hypertension

29

ASD findings?

grade 1 -2 systolic ejection murmur at right ventricular outflow tract , and fixed split S2
ECG: increased blood flow into right atrium, right ventricle, pulmonary arteries and lungs
ECG might show RVH and right axis deviation

30

why fixed split S2 in ASD

overload of right ventricle with prolonged ejection into pulmonary circuit
larger shunt, might get a mid-diastolic murmur also