Questions from Nelson Flashcards

1
Q

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

A

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

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2
Q

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

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)

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3
Q

How many infants with congenital heart disease have extra cardiac manifestations?

a) 5-10%
b) 10-20%
c) 20-45%
d) 50%

A

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

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4
Q

Which of the following conditions does not usually produce bounding pulses?

a) PDA
b) AV fistula
c) anemia
d) cardiomyopathy

A

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

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5
Q

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

A

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

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6
Q

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

A

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)

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7
Q

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

A

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

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8
Q

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

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

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9
Q

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

A

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

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10
Q

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

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)

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11
Q

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

A

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

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12
Q

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

A

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

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13
Q

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

A

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

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14
Q

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

A

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

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15
Q

Which of the following syndromes is not commonly associated with pulmonary stenosis?

a) Noonan
b) Alagille
c) Trisomy 18

A

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

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16
Q

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

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)

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17
Q

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

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

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18
Q

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

A

d) does not as per the new guidelines (double check)

however if there was a prosthetic valve then would need

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19
Q

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

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

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20
Q

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

A

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 )

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21
Q

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

A

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

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22
Q

What are two causes of short PR

A
  1. glycogen storage disease - i.e. Pompe

2. where you bypass AV node - i.e. WPW

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23
Q

Causes of prolonged QT

A

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

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24
Q

true or false - digoxin is a good choice in WPW

A

no, digoxin is contraindicated in WPW

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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
31
management of ASD
if lots of shunting by age 3, then consider closure | use a device for secundum, surgery for primum and sinus venous
32
true or false - it is common for a PDA to close spontaneously in term infants after a few weeks of age
false - not common for it to close after a few weeks of age in full-term infants controversy whether small asymptomatic PDAs need to be closed
33
AVSD ECG findings
LVH (since have tones of blood in LV) combined ventricular hypertrophy may show combined atrial enlargement
34
symptoms in mild pulmonary stenosis?
mild pulmonary stenosis is usually asymptomatic moderate to severe in exertional dyspnea and easy fatigability newborns might shunt right to left and be even cyanotic with severe stenosis can be valvular, subvalvular or supravalvular
35
Clinical and ECG findings in pulmonary stenosis
systemolic murmur at second left intercostal space - radiates to back might have a thrill can hav e widely split S2 with a quiet pulmonary component if more severe get RVH impulse at LLSB
36
imaging in pulmonary stenosis
mild - nomal findings moderate to severe - right axis deviation and RVH (so same as ASD) hear size usually nomal, can have dilation of the main pulmonary artery Treatment - if mild - usually doesn't progress if more significant - consider balloon valvuloplasty or surgical repair
37
Where is the murmur of aortic stenosis
systolic ejection murmur right second intercostal space radiate to neck systolic ejection click when it is a valve problem Imaging: LVH with moderate/severe, can get dilation of ascending aorta or aortic known Treatment: can get AI as progresses, need to follow, tx balloon valvuloplasty (although can often fail) surgery if doesn't work
38
Coarctation - associated abnormalities
hypoplastic aortic arches abnormal aortic valves VSD symptoms when duct closes
39
true or false - can present mainly with shock no murmur with coarct
true - if cardiac function is poor, might not notice mumur or BP gradient until you resuscitation femoral pulses weaker and delayed compared to radial pulse
40
story for an older kid presenting with coarctation
usually asymptomatic may have history of leg discomfort with exercise, headache or epistaxis hypertension, decreased lower extremity pulse or murmur may be present abnormal aortic valve in 50% of the time
41
how many people with coarctation have abnormal aortic valve
50% (unclear if this is only in oleo peeps)
42
what cardiac abnormality is associated with rib notching in older kids
coarctation in oleo kids will also see LVH and mild large heart in these kids in younger kids - get RVSH and hypertrophy, with cardiomegaly and pulmonary edema
43
treatment of coarct
young kids - prostaglandin, iotropes, diuretics, supportive care
44
What is the core reason that tetralogy of fallout happens
abnormal septation of the truncus into the aorta and pulmonary artery at 3-4 weeks features: pulmonary stenosis, VSD, RVH, overridding aorta ECG findings: right axis deviation and RVH boot shaped heart 5% have coronary abnormalities
45
Tetralogy of fallout findings
may initially be cyanotic initially usually have pulmonary stenosis murmur amount of shunting right to left (bypassing lungs) and the degree of cyanosis increases as the degree of pulmonary stenosis increases murmur becomes shorter and softer as the pulmonary stenosis gets worse The ‘Tet spell’ (also called ‘hypoxic spell’, ‘cyanotic spell’, ‘hypercyanotic spell’ or ‘paroxysmal dyspnea’) most frequently occurs in young infants with Tetralogy of Fallot but may occur with other congenital heart defects that have pulmonary or subpulmonary stenosis and a VSD, and at any age. Common precipitants include crying, defecation, feeding, waking from naps (low systemic resistance), fever, dehydration, tachypnoea / tachycardia due to any cause, and medications (e.g. ACE inhibitors). They tend to occur in those with mild-to-moderate cyanosis at rest and are more common in children who are iron deficient. They are characterised by: · Period of uncontrollable crying / panic, · Rapid and deep breathing (hyperpnoea), · Deepening of cyanosis, · Decreased intensity of heart murmur, · Limpness, convulsions and rarely, death. Hypercyanotic spells need to be recognised quickly and effectively controlled to prevent the development of serious complications from prolonged hypoxia. While medical intervention is indicated, many episodes are self-limiting. Those involved in the care of a child with Tetralogy of Fallot should be familiar with a spell’s presentation and early management. Pathophysiology é Hypercyanotic spells may be best thought of as an imbalance between pulmonary and systemic vascular resistance favouring decreased pulmonary flow and increased right-to-left shunting. Hypoxemia, metabolic acidosis, hyperpnoea, increased systemic venous return, catecholamines, and pulmonary vasoconstriction are thought to be involved in an interaction that results in a self-perpetuating cycle. Infundibular spasm is not required. (from internet)
46
kid with tetraology of fallot, kid becomes restless, agitated, cries, ambulatory kid squats , becomes cyanotic , treatment
knee to chest position - to increase venous return morphine for pain control (will help to relax the pulmonary vessels and for sedation) can try to increase the SVR with phenylephrine **cyanotic spell - need surgery
47
Risk factors for kids with unprepared tetralogy of fallout
increased cerebral thromboemolism cerebral abscesses (from right to left shunt )
48
which cyanotic heart disease is most likely to present in newborn period?
transposition (even though it is only 5% of congenital heart defects) remember that need to have a mixing lesion to be alive with transposition
49
Transposition clinical features
``` cyanosis quiet tachypnea and single S2 ECG: right axis deviation and RVH increased pulmonary vascularity CXR: egg on a string ```
50
management of transposition
prostaglandin balloon atrial septostomy arterial switch is the main surgery
51
Tricuspid atresia ECG findings
no tricuspid so right side is teeny left side bigger therefore get LVH and axis will be superior (i.e. 0-negative 90) (which is left axis deviation i believe) CXR - less blood flow need to have VSD or PDA - way for the blood to get to lungs
52
Surgery (BASICS) for tricuspid atresia
1. connect the subclavian artery to pulmonary - Blalock-Taussig 2. bidirectional cavopulmonary shunts and Fontan - connect the systemic venous return to the pulmonary arteries
53
What determines how cyanotic someone with truncus is?
depends on how much pulmonary blood flow there is if not diagnosed, the PVR might decrease and then get heart failure usually will have a VSD below the truncal valve
54
Imaging in truncus
combined ventricular hypertrophy and cardiomegaly | CXR: increased pulmonary blood flow may show displaced pulmonary arteries
55
total anomalous pulmonary venous return what do they need to survive
atrial level communiation 2 types of TAPVR: 1. without obstruction of pulmonary venous drainage - may have minimal cyanosis and asymptomatic ; clinical : hyperactive RV impulse with widely split S2, systolic ejection murmur ; can also have a mid diastolic murmur from flow across the tricuspid valve 2. with obstruction - cyanosis, tachypnea, dyspnea, right sided heart failure ; may or may not have murmur or changes in S2 ECG findings: without obstruction: righ ventricular volume overload, increased pulmonary flow on CXR; if they have obstruction then right atrial and right ventricle are bigger (will have RAD and RVH) , left side is smaller, CXR will show increased pulmonary vasculature if they have lots of obstruction of right sided flow, will present with hepatomegaly and other signs of right sided heart failure
56
Treatment of TAPVR
pulmonary vein open into left atrium
57
most common congenital cardiac disease cause of death in first month
HLHS
58
Hypoplastic left heart findings on ECG
small left side, therefore RVH and decreased LV forces can get cardiometagly (with increased right side) and can get increased pulmonary congestion and edema depend on right to left shunt of PDA< as it constricts, the kid gets critically ill because get excess lung flow and no perfusion to body, weak pulses usually no murmur low cardiac output why it happens: failure of development of the mitral or aortic valve or the aortic arch, small left ventricle can't support
59
Treatment of HLHS
prostaglandin supportive care surgery: norwood - systemic source for lung circulation - bldnn/Fontain ext
60
name extra cardiac complications of cyanotic congenital heart disease
1. polycythemia 2. relative anemia 3. CNS abscess 4. CNS thromboembolic stroke 5. gingival disease 6. gout 7. arthritis/clubbing 8. pregnancy - can have more trouble 9. infectious disease - i.e. RSV 10. poor growth 11. psychosocial
61
Major findings in rheumatic fever
need either 2 major or 1 major and 2 minor Major Criteria: 1. polyarthritis 2. chorea 3. subcutaneous nodules 4. erythema marginatum 5. carditis Minor manifestations: fever, elevated ESR/CRP, arthralgias , increased leukocytes, prolonged PR interval **and also need evidence of antecedent strep infection
62
treatment of acute rheumatic fever
benzathine to eradicate the beta-hemolytic strep , long term penicillin prophylaxis then needed IM benzathin penicillin 1.2 million U q 28 days - ORAL not as effective
63
prognosis in rheumatic fever
depends on extent of cardiac involvement cardiac involvement gets worse with more episodes what are the common manifestations: pancarditis, valves, pericardium myocardium, can get tachycardia greater than explained by fever, new murmur of mitral or aortic insufficiency, heart failure
64
Types of cardiomyopathy
1. dilated **most common, often idiopathic, may be due to infection (echo or Coxsackie) or be postinfectius, familial or secondary to systemic disease or to cardiotoxic drugs (i.e. doxorubicin, ipecac, iron, irradiation, cocarine, amphetamines) 2. hypertrophic : usually familial with autosomal dominant inheritance canb be sporadic 3. restrictive : rare, may be idiopathic or from systemic illness - i.e. amyloidosis , saroidosis ; storage disease (hemochromatosis, Frabry)
65
Causes of dilated cardiomyopathy
infectius - most common are coxsackie and echo virus among others metabolic toxic idiopathic get decreased systolic function posivie inotropes, diuretics, after load reduction, beta blockers, antiarrhthmics, anticoagulants, may need transplant
66
Causes of hypertrophic cardiomyopathy
1. sporadic 2. inherited autosomal dominant get diastolic dysfunction - i.e. the ventricle can't fill beta blockers and ca channel blockers are used to treat this
67
Cause of restrictive cardiomyopathy
infiltrative - amyloidosis and sarcoidosis non infiltrative - idiopathic, familial storage disease - hemochromatosis, Fabry disease endomyocardial disease get diastolic dysfunction Tx. : diureics, anti-coagulant, transplant
68
which type of cardiomyopathy associated with prolonged QT
primary hypertrophic cardiomyopathy | ECG in hypertrophic cardiomyopathy will be abnormal but the changes are nonspecific
69
ECG in dilated cardiomyopathy
non specific st-t wave changes, and LVH; RVH in 25% of cardiomyopathy
70
ECG in restrictive cardiomyopathies
may show atrial enlargement on ECG
71
Causes of pericarditis
1. infectious - viral most likely (include coxsackie, echo, mumps, influenza, EBV, CMV); bacterial more likely to be series - Staph aureus and strep most likely , also mycobacterium on the list 2. collagent vascular diseases: ie RA, SLE, RF, Kawasaki disease, scleroderma, mixed connective tissue disease, IBD 3. metabolic - uremia, hypothyroidism, chylopericardium 4. trauma - i.e. including postpericardiotomy syndrome 5. neoplastic 6. other - drug, pancreatitis, MI, thalssemia, CV perforation, heart failure, hemorrhage
72
what position is most comfy for patient with pericarditis
sitting position, will also have chest pain worsened by lying down or with inspiration, dyspnea, malaise
73
ECG findings in pericarditis
tachycardia elevated ST segments reduced QRS voltage electrical alternans (variable QRS amplitude) Treatment: if Hd significant - then do pericardiocentesis; for viral pericarditis, no specific treatment, other than anti-inflammatory meds
74
Who should be checked for high cholesterol (kids)
2-10 year old with 1. family history of atherosclerosis or MI age <55 2. parent with high cholesterol 3. unknown family history 4. child with risk factors: increased BMI, diabetes, hypertension, smokers, nephrotic syndrome Treatment: lifestyle x 6 months then retest medical treatment - usually not before 8 year old - start with statin need to check liver enzymes and CK to do this