Cards

Question 1

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

Answer 1

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

Question 2

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

Answer 2

Right ventricle

Question 3

Neonate -> QRS axis ranges from

Answer 3

+55 to +200

Question 4

Primary myocardial substrate used by adults is

Answer 4

Fatty acids

Question 5

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

Answer 5

Blood vessel diameter

Question 6

Mediator most responsible for normal pulmonary vascular transition at birth is

Answer 6

Nitric oxide

Question 7

Pompe disease involves a ? Cardiomyopathy with short ? Intervals

Answer 7

Hypertrophic
PR

Question 8

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

Answer 8

Ectopic atrial tachycardia

Question 9

Current approach for management of HLHS

Answer 9

Norwood palliation

Question 10

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

Answer 10

Nitric oxide
Prostaglandin

Question 11

Conotruncal heart defect prompts testing for what syndrome

Answer 11

Di George

Question 12

avoid ACE inhibitors in preterm babies and those with

Answer 12

Renal failure and hyperkalemia

Question 13

Most common brain MRI findings in complex CHD before surgery

Answer 13

White matter injury and infarction

Question 14

HLHS with aortic atresia is high risk bc

Answer 14

Coronary cameral fistulas

Question 15

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

Answer 15

Dilates renal and splanchnic vessels Dopaminergic

Question 16

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

Answer 16

Nitric oxide

Question 17

Milrinone has inotropic effect caused by

Answer 17

Decreased breakdown of cyclic adenosine mono phosphate

Question 18

Mutations in HAND1 and NOTCH1 have been found in cases of

Answer 18

HLHS

Question 19

Most common EKG anomaly in ebsteins

Answer 19

RBBB

Question 20

Most commonly affected gene in prolonged QT

Answer 20

KNCQ1

Question 21

Difference between functional saturation and fractional saturation

Answer 21

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

Question 22

Most common CHD associated with uncontrolled maternal DM

Answer 22

VSD

Question 23

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

Answer 23

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

Question 24

Typical course of diabetes related HCM?

Answer 24

Regression or resolution by one month of age regardless of tx

Question 25

First line for tx of mother with fetus with persistent SVT

Answer 25

Digoxin

Question 26

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

Answer 26

70-80 percent

Question 27

Does vasopressin have chronotropic effects ?

Answer 27

No

Question 28

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

Answer 28

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

Question 29

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

Answer 29

Increases peripheral vascular resistance by stimulating vascular alpha 1 receptors

Question 30

22q11 at risk for what neuro developmental issues ?

Answer 30

Seizures Delay Behavioral

Question 31

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

Answer 31

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

Question 32

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

Answer 32

HLHS

Question 33

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

Answer 33

Doppler imaging pattern of pulmonary venous flow

Question 34

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

Answer 34

TAPVR

Question 35

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

Answer 35

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

Question 36

Most common mechanism of SvT in neonate is

Answer 36

Orthodromic reciprocating tachycardia

Question 37

Possible negative effects of milrinone

Answer 37

Systemic hypotension, tachycardia, tachyarrythmias, thrombocytopenia

Question 38

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

Answer 38

TOF tetra teenager acne

Question 39

What do you send PCR for in dilated cardiomyopathy?

Answer 39

Viruses

Question 40

Two metabolic disorders that have hypertrophic cardiomyopathy

Answer 40

Noonan Pompe

Question 41

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

Answer 41

Lactate dehydrogenase

Question 42

Are diuretics a good idea in diabetic HCM?

Answer 42

Usually no

Question 43

Epinephrine acts on what and does what?

Answer 43

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

Question 44

Uptake of glucose in fetal heart depends on what transporters?

Answer 44

Glucose transporters GLUT1 and GLUT 4

Question 45

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

Answer 45

Propranolol

Question 46

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

Answer 46

Ischemia Hemorrhage

Question 47

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

Answer 47

TGA with PDA and coarc

Question 48

Clinical approach that reflects cerebral perfusion is measurement of

Answer 48

SVC blood flow with Doppler

Question 49

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

Answer 49

Compete usually due to maternal auto antibodies

Question 50

What are the echo parameters for dx of dilated cardiomyopathy?

Answer 50

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

Question 51

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

Answer 51

Carnitine palmitoltransferase

Question 52

Wandering atrial pacemaker is most associated with what tone?

Answer 52

High vagal tone

Question 53

BT shunt involves what

Answer 53

Subclavian artery to ipsilateral PA. Malala took subway in Boston