Cards Flashcards

1
Q

How does milrinone help improve systemic blood flow in single ventricle physiology?

A

Systemic after load reducer reduces vascular resistance leading to better blood flow

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2
Q

Functional status of the ? Ventricle determines the approach to palliation in HLHS

A

Right ventricle

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3
Q

Neonate -> QRS axis ranges from

A

+55 to +200

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4
Q

Primary myocardial substrate used by adults is

A

Fatty acids

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5
Q

Factor that causes most significant change in blood flow with a minor alteration is

A

Blood vessel diameter

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6
Q

Mediator most responsible for normal pulmonary vascular transition at birth is

A

Nitric oxide

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7
Q

Pompe disease involves a ? Cardiomyopathy with short ? Intervals

A

Hypertrophic
PR

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8
Q

Fetus with atrial before ventricle contractions. Atrial rate 180-240

A

Ectopic atrial tachycardia

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9
Q

Current approach for management of HLHS

A

Norwood palliation

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10
Q

Hydrocortisone inhibits the expression of inducible ? ? Synthase and vasodilatory ? Action

A

Nitric oxide
Prostaglandin

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11
Q

Conotruncal heart defect prompts testing for what syndrome

A

Di George

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12
Q

avoid ACE inhibitors in preterm babies and those with

A

Renal failure and hyperkalemia

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13
Q

Most common brain MRI findings in complex CHD before surgery

A

White matter injury and infarction

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14
Q

HLHS with aortic atresia is high risk bc

A

Coronary cameral fistulas

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15
Q

DA at low dose (0.5-2) does what and acts on what receptors

A

Dilates renal and splanchnic vessels Dopaminergic

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16
Q

Hyper capnia induced vasodilation mediated through extra cellular H+ requires a basal level of

A

Nitric oxide

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17
Q

Milrinone has inotropic effect caused by

A

Decreased breakdown of cyclic adenosine mono phosphate

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18
Q

Mutations in HAND1 and NOTCH1 have been found in cases of

A

HLHS

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19
Q

Most common EKG anomaly in ebsteins

A

RBBB

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20
Q

Most commonly affected gene in prolonged QT

A

KNCQ1

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21
Q

Difference between functional saturation and fractional saturation

A

Functional - measures oxyhemoglobin and reduced hemoglobin alone
Fractional - above plus carboxyhemoglobin and methemoglobin so this one is 2 points higher than functional

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22
Q

Most common CHD associated with uncontrolled maternal DM

A

VSD

23
Q

Is maternal DM related infant HCM histology same as familial HCM histology?

A

No. Infant one is more similar to other forms of CHD

24
Q

Typical course of diabetes related HCM?

A

Regression or resolution by one month of age regardless of tx

25
Q

First line for tx of mother with fetus with persistent SVT

A

Digoxin

26
Q

At normal levels of hemoglobin at what oxygen saturation do you see cyanosis ?

A

70-80 percent

27
Q

Does vasopressin have chronotropic effects ?

A

No

28
Q

At moderate doses, 5-10, DA does what and stimulates what

A

Increase cardiac contractility and heart rate by stimulating cardiac B1, B2, alpha 1 adrenergic and dopaminergic receptors

29
Q

At high doses, 10-20, DA does what and acts on what

A

Increases peripheral vascular resistance by stimulating vascular alpha 1 receptors

30
Q

22q11 at risk for what neuro developmental issues ?

A

Seizures
Delay
Behavioral

31
Q

What did Boston circulatory arrest study say about neuro development in Neonates with d TGA?

A

Seizures within 48 hours of surgery was strong predictor of neuro and cognitive abnormalities

32
Q

Jacobsen and holt Oran syndromes have been associated with what heart problem?

A

HLHS

33
Q

What echo measure can be used in fetus for assessment of adequacy of atrial communication of hypoplastic left side of heart ?

A

Doppler imaging pattern of pulmonary venous flow

34
Q

Fetal echo has lowest detection rate in which cyanotic heart lesion?

A

TAPVR

35
Q

What drug type is dobutamine and what does it act on?

A

Synthetic sympathomimetic
Directly on alpha and beta receptors without the release of NE

36
Q

Most common mechanism of SvT in neonate is

A

Orthodromic reciprocating tachycardia

37
Q

Possible negative effects of milrinone

A

Systemic hypotension, tachycardia, tachyarrythmias, thrombocytopenia

38
Q

Variation in ALDH1A2, an enzyme important for retinoic acid production, has been associated with what cyanotic heart lesion

A

TOF tetra teenager acne

39
Q

What do you send PCR for in dilated cardiomyopathy?

A

Viruses

40
Q

Two metabolic disorders that have hypertrophic cardiomyopathy

A

Noonan
Pompe

41
Q

Major pathway of energy production in neonatal heart in hypoxic condition?

A

Lactate dehydrogenase

42
Q

Are diuretics a good idea in diabetic HCM?

A

Usually no

43
Q

Epinephrine acts on what and does what?

A

Non selective alpha agonist , activates beta one and beta two adrenergic receptors. Increases blood pressure and systemic blood flow by increasing systemic vascular resistance and CO

44
Q

Uptake of glucose in fetal heart depends on what transporters?

A

Glucose transporters GLUT1 and GLUT 4

45
Q

If a patient had SVT with WPW the usual first line med is

A

Propranolol

46
Q

Preterm neonates are more susceptible to ? With low pressure and to ? With high blood pressure

A

Ischemia
Hemorrhage

47
Q

Hours old neonate has cyanotic upper extremities and pink lower- what do they have

A

TGA with PDA and coarc

48
Q

Clinical approach that reflects cerebral perfusion is measurement of

A

SVC blood flow with Doppler

49
Q

Most significant bradycardia secondary to AV conduction dz is related to which type of heart block

A

Compete usually due to maternal auto antibodies

50
Q

What are the echo parameters for dx of dilated cardiomyopathy?

A

Shortening fraction < 25%
Ejection fraction <40%
LV dimension z score >2 SD FROM mean

51
Q

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

A

Carnitine palmitoltransferase

52
Q

Wandering atrial pacemaker is most associated with what tone?

A

High vagal tone

53
Q

BT shunt involves what

A

Subclavian artery to ipsilateral PA. Malala took subway in Boston