Cards Flashcards
How does milrinone help improve systemic blood flow in single ventricle physiology?
Systemic after load reducer reduces vascular resistance leading to better blood flow
Functional status of the ? Ventricle determines the approach to palliation in HLHS
Right ventricle
Neonate -> QRS axis ranges from
+55 to +200
Primary myocardial substrate used by adults is
Fatty acids
Factor that causes most significant change in blood flow with a minor alteration is
Blood vessel diameter
Mediator most responsible for normal pulmonary vascular transition at birth is
Nitric oxide
Pompe disease involves a ? Cardiomyopathy with short ? Intervals
Hypertrophic
PR
Fetus with atrial before ventricle contractions. Atrial rate 180-240
Ectopic atrial tachycardia
Current approach for management of HLHS
Norwood palliation
Hydrocortisone inhibits the expression of inducible ? ? Synthase and vasodilatory ? Action
Nitric oxide
Prostaglandin
Conotruncal heart defect prompts testing for what syndrome
Di George
avoid ACE inhibitors in preterm babies and those with
Renal failure and hyperkalemia
Most common brain MRI findings in complex CHD before surgery
White matter injury and infarction
HLHS with aortic atresia is high risk bc
Coronary cameral fistulas
DA at low dose (0.5-2) does what and acts on what receptors
Dilates renal and splanchnic vessels Dopaminergic
Hyper capnia induced vasodilation mediated through extra cellular H+ requires a basal level of
Nitric oxide
Milrinone has inotropic effect caused by
Decreased breakdown of cyclic adenosine mono phosphate
Mutations in HAND1 and NOTCH1 have been found in cases of
HLHS
Most common EKG anomaly in ebsteins
RBBB
Most commonly affected gene in prolonged QT
KNCQ1
Difference between functional saturation and fractional saturation
Functional - measures oxyhemoglobin and reduced hemoglobin alone
Fractional - above plus carboxyhemoglobin and methemoglobin so this one is 2 points higher than functional
Most common CHD associated with uncontrolled maternal DM
VSD
Is maternal DM related infant HCM histology same as familial HCM histology?
No. Infant one is more similar to other forms of CHD
Typical course of diabetes related HCM?
Regression or resolution by one month of age regardless of tx
First line for tx of mother with fetus with persistent SVT
Digoxin
At normal levels of hemoglobin at what oxygen saturation do you see cyanosis ?
70-80 percent
Does vasopressin have chronotropic effects ?
No
At moderate doses, 5-10, DA does what and stimulates what
Increase cardiac contractility and heart rate by stimulating cardiac B1, B2, alpha 1 adrenergic and dopaminergic receptors
At high doses, 10-20, DA does what and acts on what
Increases peripheral vascular resistance by stimulating vascular alpha 1 receptors
22q11 at risk for what neuro developmental issues ?
Seizures
Delay
Behavioral
What did Boston circulatory arrest study say about neuro development in Neonates with d TGA?
Seizures within 48 hours of surgery was strong predictor of neuro and cognitive abnormalities
Jacobsen and holt Oran syndromes have been associated with what heart problem?
HLHS
What echo measure can be used in fetus for assessment of adequacy of atrial communication of hypoplastic left side of heart ?
Doppler imaging pattern of pulmonary venous flow
Fetal echo has lowest detection rate in which cyanotic heart lesion?
TAPVR
What drug type is dobutamine and what does it act on?
Synthetic sympathomimetic
Directly on alpha and beta receptors without the release of NE
Most common mechanism of SvT in neonate is
Orthodromic reciprocating tachycardia
Possible negative effects of milrinone
Systemic hypotension, tachycardia, tachyarrythmias, thrombocytopenia
Variation in ALDH1A2, an enzyme important for retinoic acid production, has been associated with what cyanotic heart lesion
TOF tetra teenager acne
What do you send PCR for in dilated cardiomyopathy?
Viruses
Two metabolic disorders that have hypertrophic cardiomyopathy
Noonan
Pompe
Major pathway of energy production in neonatal heart in hypoxic condition?
Lactate dehydrogenase
Are diuretics a good idea in diabetic HCM?
Usually no
Epinephrine acts on what and does what?
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
Uptake of glucose in fetal heart depends on what transporters?
Glucose transporters GLUT1 and GLUT 4
If a patient had SVT with WPW the usual first line med is
Propranolol
Preterm neonates are more susceptible to ? With low pressure and to ? With high blood pressure
Ischemia
Hemorrhage
Hours old neonate has cyanotic upper extremities and pink lower- what do they have
TGA with PDA and coarc
Clinical approach that reflects cerebral perfusion is measurement of
SVC blood flow with Doppler
Most significant bradycardia secondary to AV conduction dz is related to which type of heart block
Compete usually due to maternal auto antibodies
What are the echo parameters for dx of dilated cardiomyopathy?
Shortening fraction < 25%
Ejection fraction <40%
LV dimension z score >2 SD FROM mean
Primary site of regulation of fatty acid oxidation by the fetal and neonatal cardiac myocytes is the reaction catalyzed by
Carnitine palmitoltransferase
Wandering atrial pacemaker is most associated with what tone?
High vagal tone
BT shunt involves what
Subclavian artery to ipsilateral PA. Malala took subway in Boston
