Cardiolgy ♥️ Flashcards

1
Q

Heart lesion with reverse differential cyanosis

A

D - TGA with PDA and PPHN, interrupted aortic arch or coarctation of aorta

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2
Q

Mechanism of action of milrinone

A
  1. Phosphodiesterase 3 inhibitor—increased cAMP
    * * Decreased breakdown of cyclic AMP->perpetuating Ca influx —> improve systemic blood flow
  2. Improved ventricular function Inotropic, inodilator (promotes cardiac contractility) lusitropic (promotes relaxation)
  3. Decreases SVR

Indications: RV dysfunction, improving function in cardiac patients coming off bypass (especially if need decreased SVR)

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3
Q

HLHS

A

7-9% of congenital ♥️ defects

RV + tricuspid valve represent systemic ventricle + AV valve

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4
Q

Describe normal neonatal ECG findings

A

Normal QRS measured in V5 20-80msec

QRS axis ranges from +55 to 200+ in terms and +65-+174 in preterm

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5
Q

Predominant myocardial substrate prenatally and postnatally

A

Prenatally glucose and lactate

Postnatally fatty acids

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6
Q

The factor that causes the most significant change in blood flow with minor alteration is

A

Radius/diameter

R proportional 8x viscosity x L/pi x rx4

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7
Q

Responsible for pulmonary vascular changes at delivery

A

NO

Arachidonic acid metabolites

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8
Q

Patients with Pompes have which ECG finding?

A

Short PR interval and biventricular hypertrophy given hypertrophic cardiomyopathy

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9
Q

Noonan Syndrome findings

A
Hypertelorism
Downward eye slanting 
Low set ears 
Short stature
Pulmonic stenosis (60%) 
Web neck 

**Noonan’s syndrome is the MCC of hypertrophic cardiomyopathy in neonates and children < 4yo
Hypertrophic cardiomyopathy

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10
Q

Rapid heart rate with atrial contractions precede ventricular contractions, atrial rate 180-240 which seems to speed up and slow down. What is diagnosis?

A

Ectopic atrial tachycardia

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11
Q

The most common non renal association with hypertension in preterm is?

A

BPD

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12
Q

Approach to management of HLHS is?

A

Norwood palliation

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13
Q

Treatment of fetal SVT vs AVnRT (atrioventricular nodal reentry tachycardia)

A

SVT is Digoxin

AVNRT is amiodarone

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14
Q

How does hydrocortisone express myocardial action in hypotensive patients?

A

Inhibits expression of nitric oxide synthase and vasodilators prostaglandin action

Upregulation of CV adrenergic receptors (vasoconstriction, inc Co, inc BP)

Upregulation of angiotensin II receptors to increase vascular resistance

Inhibits catecholamine metabolism and release of vasoactive factors

Inc in intracellular Ca concentration (enhances cardiac responsiveness to catecholamines)

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15
Q

Which genetic polymorphism is associated with abnormal neuro development at 1 year post cardiac surgery?

A

Genetic polymorphisms in apolipoprotein E

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16
Q

Which population of NICU patients should avoid ACE inhibitors

A

Preterm
Renal failure
Hypokalemia

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17
Q

Describe findings in HLHS and subtypes

A

Severe MV and AV stenosis/atresia
Hypoplastic LV
Aortic arch hypoplasia

30% mitral and aortic atresia
25% mitral stenosis and aortic atresia (high risk 2/2 presence of coronary-cameras fistulas in 50%)
45% mitral and aortic stenosis

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18
Q

Dopamine acts on which receptors at which doses?

A

Alpha 1: vasoconstriction (6-10)
Beta 1 and 2 chronotropy (HR) and contractility (2-6)
Dopaminergic at low dose (2-4) renal vasodilation and splanchnic vessels
At high dose 10-20, increases PVR via alpha 1

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19
Q

Hyperpercapnic-induced vasodilation in CBF is mediated by:

A

Hydrogen ion concentration requiring basal nitric oxide

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20
Q

Norepinephrine works where?

A
  • endogenous catecholamine
  • increases SVR and CO by alpha 1,2 and beta 1 receptors
  • constricts systemic vascular&raquo_space; pulmonary vascular
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21
Q

Mechanism of action of dobutamine

A

Acts directly in alpha and beta receptors without release of Norepi

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22
Q

Hydrocortisone side effects

A

Hyperglycemia
Osteopenia
Inhibits immune function and somatic growth
Associated with SIP if concurrent indomethacin

Aid in hypotension by decreasing breakdown of catecholamines, inc Ca in myocardial cells and upregulating adrenergic receptors

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23
Q

Which mutations have been found in HLHS?

A

HAND1 and NOTCH1

Recurrence HLHS sibling 8%
Recurrence of any congenital is 22%

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24
Q

What abnormalities are seen in Ebstein anomaly?

A

Apical displacement of septal and posterior leaflet of TV (atrial I zing the RV)

WPW (60%)
Right bundle branch block (70-80%)

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25
Q

Most commonly affected gene in long QT syndrome

A

KCNQ1- potassium channel gene

Abnormalities Associated with this gene also correlate with A fib later in life

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26
Q

What is the difference in functional vs fractional saturation of hgb?

A

Functional measures oxyHb and reduced Hb alone

Fractional saturation measures oxyHb ans. reduced Hb. And approximates carboxyHb and methHb.

Functional Hb 1.6-2 points higher than fractional saturation.

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27
Q

Most Common CHD in maternal diabetes

A

VSD
Double outlet RV
Truncus Arteriosus

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28
Q

What is therapy of hypertrophic cardiomyopathy in IDM neonates?

A

B blockers
Maintenance fluid
Contraindicated-inotropic support bc can make condition worse

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29
Q

What is the prognosis of HCM in neonate born with maternal diabetes?

A

excellent with resolution of ventricular hypertrophy at 1 mo of age

Mainly diastolic dysfunction

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30
Q

How is BP affected in preterms? Ie relative to SVR

A

Preterms may have low systemic blood flow because of structure of preterm myocardium is less able to overcome increased SVR after birth

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31
Q

How is preterm blood pressure influenced by chorioamnionitis

A

Chorio is associated with hypotension —> caused by release of inflammatory mediators (IL -1 and TNF)

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32
Q

When does cyanosis occur in neonates?

A

With drop of 3-5g/dL of Hgb of reduced Hgb

Cyanosis is more visible in polycythemic than in anemic babies

Cyanosis notable at this %saturation Hb: Reduced Hgb/total Hgb

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33
Q

Vasopressin mechanisms of action

A

Minimal chronotropic effects

Vasoconstriction during hypoxemia and acidosis

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34
Q

Which is the most common cause of HCM in neonates and children <4years?

A

Noonan syndrome

35
Q

Describe Heart embryology and GA at formation

A

Arises from mesoderm
Right sided or dextro looping at 21 days
Septation at 3-4 weeks
Functional at 8 weeks

36
Q

In utero, what percentage does the RV and LV supply to fetus?

A

66% RV
34% LV

7-15% of total blood volume goes to fetal lungs

37
Q

Name the order from highest to lowest O2:

Umbilical artery, vein, uterine artery and vein

A

Uterine artery, uterine vein, umbilical vein, umbilical artery

38
Q

How does the fetus compensate for hypoxemic environment?

A

Increase in erythropoietin
Fetal hemoglobin
Decreased O2 consumption

39
Q

How can you increase stroke volume?

A

Increase EDV by inc preload or inc ventricular compliance

Decrease ESV by inc contractility and dec afterload

40
Q

How best does the neonate regulate CO

A

HR

Preload

41
Q

What target conditions does critical congenital heart screening identify?

A
HLHS
Pulmonary Atresia with intact septum 
Tetralogy of Fallot
TAPVR
TGA
Tricuspid Atresia
Truncus Arteriosus
42
Q

Describe the surgical repair for HLHS

A

Norwood

Stage 1
Atrial septectomy (creating pop off for LA) — preventing pulmonary venous congestion
Proximal portion of MPA —-> ascending aorta (to provide systemic circulation)

Systemic - pulmonary artery shunt to provide systemic blood flow (BT shunt - subclavian to pulmonary artery to ensure adequate pulmonary blood flow)

Stage 2 - BiDirectional Glenn
Decrease volume overload on RV
Remove BT - connects SVC directly to pulmonary artery (increasing pulmonary blood flow without increasing RV volume load)

Stage 3 - modified Fontan 
Reroutes IVC (to now meet SVC) to PA to create separate systemic and pulmonary circulation

This occurs in multi step process to allow pulmonary pressures to decrease

GLENN (G for Glenn) goes FIRST (F for Fontan)

43
Q

Tetralogy of Fallot

A

RVH
PS
Overiding Aorta (allowing blood to leave both LV and RV)
VSD

  • Right sided obstructive lesion
44
Q

Variation in which enzyme has been associated with TOF?

A

ALDH1A2 (enzyme imp in retinoic acid production)

45
Q

Genes associated with TGA

A

NODAL and FOXH1

46
Q

Workup of dilated cardiomyopathy

A

Echo for ALCAPA
Viral PCR
Genetic/metabolic

47
Q

In hypoxic condition the major pathway for energy production in neonatal heart is?

A

Lactate dehydrogenase (facilitates reduction of pyruvate to lactate reflecting anaerobic glycolysis)

48
Q

Differentiate mechanism of action of Epi at low dose and high dose

A

Low dose 0.01-0.1mcg/kg/min Stimulates beta receptors enhancing myocardial contractility and peripheral vasodilation

High dose >0.1mvg/kg/min stimulates alpha receptors causing peripheral vasoconstriction and increase SVR

Remember it is a non selective alpha agonist and activates both B1 and B2 adrenergic receptors

49
Q

How does fetal heart facilitate glucose uptake??

A

Glucose transporters
GLUT1 and GLUT4

GLUT1 plays primary role in bringing glucose into the cardiac monocyte

Prenatally
GLUT1&raquo_space;GLUT4

Postnatally
GLUT4 > GLUT1

50
Q

Treatment of SVT with WPW

A

Propanolol

51
Q

Timing of CHD Presentation

A

TGA —-> 1-2 days
HLHS —-> 3-7 days
Coarctation Ao —> ~14 days
VSD —> ~4-6 weeks

52
Q

3.2 kg baby with tachypnea + poor perfusion. Pre/post sats 82%. O2 via hood worsens condition. Placed on ventilator (TV 20ml, PEEP 6, iT 0.35, FiO2 70%)
CBG - 7.24/38/46/18/-8.
Saturations are still low (~75%)

What is the next step in management?

A

Decrease TV
Decrease rate
Decrease FiO2

In HLHS - oxygen worsens the condition due to steal of blood from PDA

Newborn Baby within 1st week of worsening with O2 - T/C HLHS

53
Q

Qp/Qs calculation

>1

A

Ao sat-SVC sat/RV sat-PV sat

> 1 L to right shunt (inc PBF)
<1 R to L shunt (PPHN)

Remember inverse (systemic/pulmonary)

54
Q

Which type of heart failure does PDA lead to?

A

Left to right shunt

Therefore, left sided heart failure

55
Q

Characteristics of Fetal Circulation

A

Oxygenated blood —> placenta
—> IVC —> RA (SVC brings deoxygenated blood so mixing occurs) —> RV —> PA —> PDA —> descending Ao

Oxygenated blood —> placenta
—> IVC —> Ductus Venosus —> LA (PFO) —> ascending Ao

** LA, LV, ascending Ao have the highest PO2

56
Q

Name Contracting and Dilating Mediators

For example - PDA closure occurs with increasing contracting mediators and decreasing dilating mediators

A
Constrictors 
PGF2α
Αcetylcholamine 
Bradykinin
Oxygen 
Dilators 
PGE1
PGI2 (prostacyclin)
Hypoxemia
Acidosis
57
Q

EKG Measurements

A

HR - big boxes - 300 —> 150–> 75
1 big box = 0.2 seconds or 200 milliseconds
1 small box = 0.04 seconds = 1 millivolt

58
Q

Describe the expected pressure measurements with umbilical catheter in IVC, Aorta, LA

A

IVC - 1-3 mmHg
Ao - should approach systemic means
LA - mean pressure of 8mmHg

59
Q

Calculate Mean Blood Pressure

A

Mean BP=
Diastolic + 1/3 (SBP - DBP)

** Remember to average blood pressures if given more than one)

60
Q

Indirect Measure of cerebral perfusion

A

SVC blood flow + Doppler

SVC flow reflects upper body venous return and is less influenced by shunts

May reflect cerebral perfusion

61
Q

Describe echo findings of dilated cardiomyopathy in terms of

  • shortening fraction
  • ejection fraction
  • left ventricular dimension Z score
A

Shortening fraction <25%
EF<40%
LV dimension Z score greater than 2

62
Q

Which mediator is decreased in expression in pulmonary casualties in idiopathic pphn?

A

Endothelial nitric oxide synthase and downstream target soluble guanylate cyclase

Remember endothelin 1 plays goal in maintaining high vascular tone in utero

63
Q

T or F

Blood pressure has a strong relationship with cardiac left ventricle output

A

False
BP is a function of both blood flow and SVR.
Weak relationship between LV output and mean BP

64
Q

Physical exam findings in HLHS

A

Hyperdynamic precordial activity reflects right ventricular volume and pressure overload
Decrease in amplitude of peripheral pulses only after ductal constriction
Single S2 if aortic atresia present
Murmur for TR

65
Q

Primary site of fatty acid oxidation regulation by fetal and neonatal cardiac myocytes is

A

Carnitine palmitoyltransferase

66
Q

Wandering atrial pacemaker is most associated with:

A

High vagal tone

67
Q

The BT shunt involves shunting from which vessels?

A

Subclavian artery to ipsilateral pulmonary artery

68
Q

Ductal patency in utero depends on which 3 substances

A

PGE2
Prostacyclin
Thromboxane 2

69
Q

Describe 4 mechanisms how fetus tolerates lower pO2

A
  1. Fetal hemoglobin
  2. Increased O2 carrying capacity bc of elevated Hgb in fetus 2/2 hypoxemic erythropoeitin production
  3. Decreased O2 consumption
  4. Increased ability to utilize glucose by anaerobic metabolism
70
Q

Describe 3 Mechanisms by which fetus compensated for worsening hypoxemia

A

Blood flow preferentially goes to heart brain and adrenals
Dilation of DV so more oxygenated blood goes to left side of heart
Fetus goes into hybernation mode

71
Q

What is the % of fetal blood flow in 2nd trimester?

In 3rd trimester?

A

7-15% (2nd trimester)

35% (fetal growth)

72
Q

How does the fetus adjust to

the relatively low intra utérine oxygen environment ?

A

Epo

Fetal Hb (high affinity for oxygen which allows for increased oxygen uptake even in hypoxia environments)

Decreased oxygen consumption

73
Q

How does fetal

Heart increase cardiac output ?

A

CO = SV x HR

fetal tachycardia—> increases cardiac output

74
Q

Describe a pt with Qp/Qs>1

A

Tachypnea, FTT, CHF

75
Q

Baby with cyanosis, acidosis and tachypnea. What’s the Qp/Qs?

A

Qp/Qs<1

76
Q

In a patient with cardiac tamponade how does the blood pressure change?

A

Pulsus paradoxus is a decrease in systolic blood pressure during spontaneous inspiration

Pulsus paradoxus is a common finding in cardiac tamponade in a spontaneously breathing patient.

Because systolic blood pressure normally increases during inspiration while receiving positive pressure ventilation, reversed pulsus paradoxus is observed in patients with cardiac tamponade who are receiving mechanical ventilation.

77
Q

List disorders associate with single S2

A

S2 is closure of aortic and pulmonary valve

Single S2 heard with:

  • Pulmonary hypertension (both close at same time)
  • 1 semilunar valve (PA, aortic atresia, truncus, TOF/absent PV)
  • P2 not heard (TGA, TOF, severe PS)
78
Q

Single S2 with bounding pulses. Dx?

A

Truncus arteriousus

Single S2 because one arterial vessel exiting heart

Bounding arterial pulses because of diastolic runoff into PA

79
Q

Which enzyme do catecholamines (ie dopamine) regulate for renal solute transport and Na absoption?

A

Basolateral Na/K ATPse (increased Na absorption)

80
Q

Hypertrophic cardiomyopathy is seen with which genetic conditions?

A

Noonan
Beckwith Weideman
Costello Syndrome (fasciocutaneoskeletal syndrome)
Eagle Barrett (prune belly)

81
Q

Side effects of dopamine use

A

Decreased thyroyropin
Deceased prolactin
Decreased thyroxine
Increases PVR

82
Q

Long term dopa use (> 12 h) does what to the heart?

A

Depleted norepinephrine stores

83
Q

Right and left differentiation begins during which stage of embryonic development?

A

Gastrulation

84
Q

What suggests R > L shunting?

A

> 15 torr difference in pre/post ductal paO2

>10% difference in O2 saturations