Cardiology Flashcards by Jenny Camacho

What is the most common neonatal cardiac tumor?

Rhabdmyoma. A/w tuberous sclerosis

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What is the two most common causes of complete vascular rings

double aortic arch (40%) and right aortic arch with a PDA or ligamentum arteriosus (30%). Double aortic arch secondary to persistence of R and L 4th brachial arches; Right aortic arch with PDA secondary to persistence of R 4th brachial arches

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What are causes of incomplete vascular rings?

aberran R subclavian (20%,)anomalous innominate artery (10%), abberant pulmonary artery (rare)

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What is the physiology of a widely split fixed S2 with and ASD

The second heart sound is appreciated because of the a dely in the R ventricular depolarization and littlee change in the venous return to the R atrium with inspiration. Because the pressure difference between the RA and LA is minimal, the blood flow across the ASD does not produce a murmur. However, if the R to L atrial shunting is excessive, infants may have a soft systolic ejection murmur from the increased flow across the RV outflow trct and or a diastolic murmur as a results of a relative tricuspid valve stenosis

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What vessel in the fetus has the highest O2 sat

umbilical vein; the oxygen saturation in the heart of the fetus, tend to be higher on the L side of the heart compared to the R side

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What vessel/chamber in the heart as the lowest O2 sat?

What vessel in the infant has the lowest O2 sat?

SVC = 40%

Umbilical artery is 30%

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What is the effects of high dose epi?

stimulates alpha 1 and beta 1 receptors; decrease HR because of increased in vagal tone on SA and AV nodes; increases contractility (beta 1 on myocardial cells); increases SVR due to increased alpha 1 (more important than beta 2); increases BP mostly because of increase SVR; high dose epi is similar to dopa

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What some characteristics of milrinone?

PDE 3 inhibitor that increases cAMP which leads to increased Ca cellular entry, some increase in contractility, relaxation of vascular smooth muscle cells (leading to vasodiltion and decreased afterload); it is independent of receptors and thus, no tolerace; may induce thrombocytopenia

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What the the drugs effects of digoxin?

inhibits the N/K ATPase pump in the cardiac myocytes leading to increased Ca influx; is a negative chronotrope because of vagal effect and also effect of reducing SA node conduction; +inotrop; decreases SVR; Its + inotrope effects are due to inhibition of sarcolemmal Na/K ATPase. This enhances contractility by increasing intracellular Na and Ca. The decreased chronotropy is due to prolonged SA node conduction rate, evident by prolonged PR interval. Digoxin also increases the refractory period through the AV node, accounting for is anti-arrythmic action.

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What are some characteristics of digoxin?

decreased afterload; ideal inotrope for CHF; anti-arrhythmic by decreasing AV conduction; NOTE: other medications may alter digoxin levels; toxicity creates the following syptoms: GI symptoms decreased HR, prolonged PR, AV block, monitor K+ and Ca levels (greater risk of toxicity with lower K concentrations)

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What the mechanisms of action of dobutamine?

Beta 1 and some Beta 2; + chronotrope (less of an increase in HR compared to dopamine) + inotrope, decrease SVR; increase cardiac output (by increasing SV)

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What are the receptors dose Dopamine effect a low, ,medium and high doses?

endogenous catecholamine that is a precursor to epi and norepi. Effects Beta 1 and Alpha 1 receptors. At low dose, it effects the dopamine receptors in the kidneys. At medium doses effects Beta 1 receptors and at high dose it effects alpha 1 receptors.

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What are the effects of dopamine on HR, contractility, and SVR and BP?

increases HR at med dose (Beta -1 effect), increase contractility at med dose (Beta 1); increases SVR at high doses (due to alpha 1 effect); increases CO (incr HR and SV) and SVR

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What is the mechanism of action of dopamine?

inhibits the Na/K ATPase and Na/H+ pump’ at low doses, uniquely dilates renal vasculature; 2 microg/kg/min( and lower): dopamine receptors (renal, splanchnic, coronary, and cerebral) increases renal blood flow, increases GFR and increases FeNA; doses 2-6: Beta 1 and dopaminergic increases inotrope and HR: doses > 6-20: B1 and some alpha 1; > 20; alpha 1 (controversial if this effect occurs in neonates); less effective with prolonged usage becasue of its effects on decreasing endogenous norepi stores

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What are the effects of low dose epi?

beta 1 and beta 2 stimulator; increases HR (beta 1 effect on SA and AV node); increases contractility (+inotrope, due to Beta 1 effect on myocardial cells), decreases SVR (beta 2 effect); BP effect depends on greatest effect (increase HR/SV vs decrease SVR); similar to dobutamine

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What are some adverse side effects of epi?

hypokalemia, local tissue ischemia, renal vascular ischemia, severe HTN; higher dose with enhanced diastolic pressure and improvement in coronary artery perfusion

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What is isoproteronal and how does it act?

synthetic catecholamine wih B (1 and 2); dose 0.05-1 microg/kg/min (usually > 0.5 not required); +++ chronotrope and thus, helpful for complete heart block; + inotrope; decreases SVR leading to peripheral vasodilation

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What are some characterisitics if isoproteronol?

May decrease coronoary blood flow because of vasodilation of muscular regions; not helpful in shock because leads to vasodilaton in skin and muscle vascular beds; may lead to hypoglycemia because it induces insulin secretion

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What are the effects of nitroprusside on SVR?

decreases SVR

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What are the charaacteristics of nitrorpusside?

vasodilator (arterial greater than venous); inceases cGMP levels, rapid onset with short duration; increased ICP; light sensitive; may lead to cyanide toxicity; extravasation may lead to titssue sloughing/necrosis

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What are the alpha and beta effects of norepi? Chronotrope or inotrope?

alpha> B1>B2; + chronotrope; + inotrope; increases SVR

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What are the characteristics of norepi?

Epi preferred in neonates; profound vasocontriction; renal vasoconstriction; may lead to hypocalcemia and hypoglycemia

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what is the most common cause of hypertropic cardiomyopathy in in neonates and chldren under 4?

Noonan syndrome. Other syndromes a/w hypertrophic cardiomyopahty include Beckwith-Weidemann, T21, Costello syndrome (fasciocutaneoskeletal syndrome); Eagle Barret syndrome.

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Are congenital heart defects more prevalen in the recipient twin of TTTS?

Yes- there is a 3 fold incease. The most frequent defects are VSD, ASD and pulmonary stenosis.

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What are side effects of dopamine in the neonate?

decreased thyrotropin, prolactin and thyroxine. NOTE: With prolong use- Because it works via increasing release of endogenous Norepi, norepi stores can be depleted in the heart in as little as 12 hours

What are conotruncal defects of the heart?

occur when the normal development of the cardiac flow tracts is disrupted. Defects include truncus arteriosus, TGA, DORV, tetology of Fallow ( Pulm stenosis, RVH, overriding aorta, and VSD).

What is the most commpn type of differential cyanosis and when is it seen?

When the O2 sat in the R hand is greater than either foot and this is seen wit PPHN with a PDA.

What does a lower pre-ductal sat compared to the post-ductal sat in the foot

This is reversed differential cyanosis and is seen in newborns with D-transposition of the great arteries

What do tall peaked P waves indicate on a neonatal EKG?

RA enlargement and further investigation is needed.

Does the heart arise from endoderm or mesoderm?

mesoderm

What is the first system to function in utero?

cardiovascular. Heart starts beating at D17-D20

When is heart formation complete?

8 weeks gestation

what ventricle supplies most of the cardiac output in utero?

: RV supplies 66% and the LV supplies 34% of total blood volume

Which side of the intrauterine heart has the higher oxygen saturations?

Let side of heart because the higher oxygenated blood from UV via the ductus venosus is shunt across the PFO to the LA. Also the RA is lower O2 sat because the SVC abd IVC blood returning the the RA has a lower O2 sat. This is beneficial because blood to the brain and the coronaries has a higher O2 sat

What ventricle supplies the lower body of the fetus and placenta with blood?

What ventricle supplies the heart, brain, and upper body of the fetus with blood?

Place the following blood vessels in order from highest to lower O2 sat: umbilcal artery, umbilical vein, uterine artery, uterine vein

uterine artery, uterine vein, umbilical vein, umbilical artery

Name all of the functions of nitric oxide

decreases intrapulmonary shunting, decreases PVR, decreases V/Q mismatching, increases PaO2; it does not alter gas diffusion in the lung

What is the most common type of VSD?

perimembranous

What is the most common heart lesion seen in the first week of life?

d-TGA

You examine an infant with a flat occiput, hypoplastic midface and upward slanting palpebral fissures. You auscultate a loud systolic murmur on the LSB. Name the most common lesion and what you will see on the EKG

T21- Endocardial cushion defect and will see superior axis deviation

What is the most common symptomatic pediatric arrhythmia?

SVT

What does VACTERL stand for?

vertebral anomalies, anal atresia, CV anomalies, TEF, esophageal atresia, renal anomalies, limb defects)

What is the most common heart lesion in children with VACTERL?

VSD, comprising > 50% of cases

What is the most common cause of HTN in the neonate?

reno-vacular with renal artery thrombosis secondary to UAC placement being the most common etiology

What syndrome is the most common cause of hypertrophic cardiomyopathy in neonates and children under 4 years?

Noonan syndrome; AD inheritance. Features include pulmonary stenosis (60%), hypertelorism, down slanting palpebral fissures, low set ears, short stature, short webbed neck, pectus excavatum, cryptorchidism, cognitive deficits, bleeding disorders, lymphedema

What is the most common congenital heart disease lesion?

VSD

When does cardiogeneis begin? When does the heart begin beating? When does septation occur?

Cardiogenesis begins in the 5th week. Heart starts beating in 6th week and septation occurs between weeks 7 and 8

What embryonic stucture does the ductus arteriosus arise from?

Left 6th aortic arch

True or false: The neurodevelopmental outcome in children with congenital heart disease is associated with the duration of cardiac by-pass time

True.

What is the most common form of intracranial injury on imaging studies in infants with CHD?

white matter injury (Pre-op 20% and new WMI post-op in 44%)

Congenital heart defects (3 fold increase) are more common in the recipient twin of of TTTS? What are the defects that are more frequently see?

ASD, VSD, and pulmonary stenosis

What is the second most common CHD presenting with cyanosis in the first week of life?

HLHS

What is the primary myocardial substrate in adults? Fetus?

In adults it is fatty acids, in the fetus it is glucose

evaluation of a fetus with a rapid heart rate by using fetal Doppler imaging reveals atrial contractions that precede ventricular contractions. The atrial rate ranges from 180-240 bpm and seems to speed up and slow down. Whats the diagnosis?

ectopic atrial tachycardia- the speed up and slow down is characteristic of this arrhythmia

What is the most common association with non-renal HTN in neonates?

BPD

What is the surgical procedure done in most neonates with HLHS?

Norwood palliation

What antiarrythmic drug is most effective in inducing a normal heart rhytm in the fetus with resisant AN nodal reentrant tachycardia?

amiodarone (note: for fetal SVT, dogoxin is first line, but when unsuccessful, use amiodarone)

what class of anti-hypertensive drugs should be avoided in preterm neonates (<36 weeks), those with renal failure, and those with hyperkalemia?

ACE inhibitors

In what cardiac lesion is iNO contraindicated?

interrupted aortic arch, critical AS, infradiaphagmatic/obstructive TAPVR, HLHS

a 4 week old has a right dominant unbalanced AV canal defect. He is tacyhypneic and has gained only 40 g in the past week. His Qp:Qs is 4:1. Medical management is directed at efforts to decreased Qp by increasing pulmonary vascular resistance and decreasing systemic vascular resistance. What is the clinical strategy the needs to be used?

intubation and controlled hypoventilation as this will increase the PVR. Hypoventilation leads ro respiratory acidosis which causes pulmonary vasoconstriction and increases PVR. Giving O2 would decrease PVR and so would hyperventilation

Name ways to decrease PVR

alveoloar expansio, Alkalosis (decrease pCO2), oxygen, iNO, sildenafil, Bosentan

Name was to increase PVR

Pulmomary vasonconstriction --> Hypoxia, acidosis,; Increased interstitial pressure --> atelectasis, pulm edema, PTX, pleural effusion, mechanical ventilation, excess PEEP; lung hypoplasia, polycythemia

a 4 week old has a right dominant unbalanced AV canal defect. He is tacyhypneic and has gained only 40 g in the past week despite medical management. He is not a candidate for a bidrectional Glenn operation right now. What is the most appropriate intervention?

Narrow the MPA with a PA band

Over the first several weeks of life, a newborn with a complete AV canal develops tachypnea and decreased energy. What are these signs of?

pulmonary over circulatoion with a Qp:Qs >1

What are the R-sdided obstructive lesionas and what are the clinical signs that develop when the PDA closes?

TS/TA; PS/PA, TOF, Severe Ebsteins anomaly. These are ductal dependent for pulmonary blood flow so when the ductus closes, baby will have severe cyanosis , acidosis, cardiogenic shock; pre and post-ductal hypoxemia

A 4 day old ex 38 week female appears tachypneic. The preductal sat is 92% and post is 75%. HR 160, RR 70 with increase WOB. There is a normal S2 and a 2/6 continous murmur at the LUSB and back. CXR with increased pulmonary vacular markings. Name the cardiac defect

interrupted aortic arch aka L sided obstructive lesion

a 4 day old ex 38 week female is noted to have a small chin, flattened nasal bridge, protruding toinge, and single palmar crease. HR 160, pre ductal sat 92% and post 93%, 2/6 systolic murmur but not diastolic murmur. CXR with increased pulmonary vascular markings and cardiomegaly. Name the cardiac dfect

Complete AV canal

A 4 dya old ex 38 weeker female is noted to have low set malformed ears, cleft palate and persistently low Ca. HR 160, RR55, pre ductal sat 92% and post 93%, single S2, 2/6 systolic murmur and no diastolic murmur. CXR with increased pulmonary vascular markings and cardiomegaly. Name the cardiac defect

truncus arteriosus; this is DiGeorge, 22q11 deletion; hear a single S2 due to lack of pulmonary valve

4 day old ex 38 weeker female with a deformation of the RUE. HR 130, RR 44, Pre and post ductal sats 96%. 1/6 systolic mumur only. CXR with normal pulmonary vascular markings and heart size. Name the defect

ASD; this is Holt Oram syndrome. Can also have a VSD. Due to TBX5 mutation

Which R sided obstructive lesions require PGE and BT shunt within 1 week of life

PA with intact ventricular septum and PDA; TA with PA with ASD and PDA; PA/VSD and PDA

What intervention does critical PS with PDA require?

PGE and balloon dilation within 1 week

A 4 day old ex 38 week female appears cyanotic. The pre-ductal sat is 82%, post-ducal is 84%. HR 160, RR71 but no increaseed WOB. There is a single S2 and 2/6 systolic murmur at the LUSB. CXR with decreased pulmonary vascular markings. Name the defect.

Tricuspid and pulmonary atresia (R sided obstructive lesion)

What clinical signs do you see when an infant has a L sided obstructive lesion as the PDA closes?

acidosis, hypotension, cardiogenic shock

Which L sided obstructive lesions require immediate catheter/surgical intervention after birth?

HLHS with intact atrial septum and PDA; obstructive TAPVR and PDA

A 7 do ex 38 week female presents with tachypnea, tachycardia, lethargy, diminished pulses, gallop rhythm. The pre and post ductal sats at 58%. There is a 1/6 systolic murmur on exam. CXR with increased pulmonary vascular markings. Whats the pathophysiology?

PDA closing in an infant with a L sided obstructive lesions

A 1 do former 38 week male with tachypnea. Pre ductal 67% and post 75%. HR 140 RR 80 and increased WOB. Single S2 and 2/6 continuous mumur at the LUSB and back. CXR normal. Name the cardiac defect

D-TGA

What are the indications for repairing a ASD? VAD?

ASD + RA/RV enlargement or ASD with worsening CLD; for VSD --> VSD + FTT/CHF or VSD+ PPHN due to pulmonary over circulation

Prominent forehead, butterfly vertebrae, anterior chamber abnormalities of the eye. Name the syndrrome, the genetic defect, and the cardiac anomalies associated with this

Alagille;s JAG 1 mutations, branch PA stenosis, PS, TOF

hypoplasia of the thymus and parathyroid (low Ca), cleft palate; name the disorder, the genetic defect and the associated cardiac issues

DiGeorge; 22q11 deletion, VSD, truncus, TOF, interrupted aortic arch

Infant with upper limb anomalies only and a soft 1/6 systolic murmur. Name the syndrome, genetic mutation, and associated cardiac problems

Holt Oram, TBX5 mutations, ASD, VSD

long limbs and scoliosis, pectus. Name the disorder, the genetic defect and the associated cardiac issues

Marfans, FBN1 mutations, aortic root dilation, valve prolapse

widely spaced eyes, ptosis, low set ears, webbed neck, pectus, cryotorchidism. name the disorder, the genetic defect and the associated cardiac issues

Noonans syndrome; PTPN mutations; PV stenosis, TOF

bulateral epicanthal folds, tongue protrusions, low nasal bridge, hypotonia, brushfield spots. Name the disorder, the genetic defect and the associated cardiac issues

Downs, T21, ASD, VSD, complete AV canal

webbed neck , broad chest with widely spaced nipples, streak gonads. Name the disorder, the genetic defect and the associated cardiac issues

Turners, XO, bicuspic Aortic valve, AS, Coarctation, IAA

Elfin facies, stellate pattern of the iris, short anteverted nose, long philtrum, prominent lips. Name the disorder, the genetic defect and the associated cardiac issues

Williams, 7q11 deletion, supravalvular AS, branch PA stenosis

What factors play a role in the phyiologic closure of the PDA?

bradykinin released from the lungs and withdrawal of PGE2 from the placenta at birth

What arrhythmia is L-TGA associated with?

neonatal heart block

On an EKG, what indicates right axis deviation? What lesions have this?

Down Lead I and up in AVF = 100-190 degrees, seen in normal newborn, RHV, TOF, Coarctation (lead I is positive toward the L chest and AVF is positive downward)

What will you see on EKG to indicate Left axis deviation?

up in 1 and down in AVF; will see this in AV canal defect, Primum ASD, and tricuspid atresia

What are causes of PACs?

increased vagal tone, central line within the atrium, electrolyte abnormalities (hypoglycemia), hypoxemia, hyper or hypothyroid, Drugs (digoxin, caffiene, alpha and beta agonists.

What causes PVCs

immature myocardium cardiomyopathy, electrolyte disturbance, metabolic disease, intracardiac tumors, Long QTc;

When would you get an echo for PVCS? What are you looking for?

If PVCs are persistent > 60 in 1 hour, get echo to evaluate for myocarditis, cardiomyopahty, and cardiac tumors

What two structural cardiac defects are a/w complete heart block?

congenitally corrected L-TGA and complete AV canal

What structural heart defect is most commonly a/w an accessory pathway?

Ebstein's anomaly, will see very large peaked P waves (b/c the RA is enlarged), RBBB (bunny ears in lead 3). 60% of patients with EA have WPW and 70-80% have RBBB

What are causes for prolonged QT?

low calcium, low mag, low K, myocarditis, channelopathy (~75 of cases are cause by mutation in 3 genes: KCNQ1, KCNH2, SCN5A)

What is the treatmen for Torsades?

IV Mag sulfate

What cardiac anomaly wil lead to lower intrauerine oxygen saturations to the brain, cornary vessels and upper body?

dTGA with intact ventricular septum

At birth, which cardiac lesions is more likely to have a large PDA- L sided obstructive lesions or R sided obstructive lesions?

Left sided obstructive lesions

Gallop, hepatomegaly, pulmonry edema- name the type of shock

Cardiogenic (impaired cardiac filling, impaired ventricular emptying, impaired contractility)

well perfused, bounding pulses, wide pulse pressure

distributive shock (vasodilation)

Name the 3 causes of reversed differential cyanosis

1. dTGA with intact ventricular septum, PHTN and PDA; 2. dTGA intact VS, interrupted aortic arch and PDA; 3. dTGA with intact VS, CoA and PDA

a newborn fails her pulse ox screen with pre and post ductal sats of 805. CXR showed severe pulmonary congestion. Echo showw a normal 4 chamber heeart and an ADS right --> L flow and large PDA with R --> L flow. What lesion could this be?

TAPVR

A 3 week old infant has recurrrent episodes of irritability, pallor and inconsolability. On exam there is a high frequency systolic murmur at the apex radiating to the L axilla. The EKG shows ST changes. What is the most likely diagnosis

Anomalous origin of the L main coronary from the the pulmonary artery

A cyanotic newborn is found to have a single S2 and bounding arterial pulses. What could the diagnosis be?

P2 no heart -->dTGA, TOF, severe PS ; single semi-lunar valve as in PA, AA, truncus and TOF with absent PV; pulm HTN,; most likely is truncus arteriosus (single S2 b/c one arterial vessel exiting the heart; bounding pulses b/c of diastolic runoff into PA

Stimulation of this adrenergic receptor increases SVR and increases contractility (positive inotrope); These receptrs also signal phosopholipase c

Alpha 1

Stimulation of this adrenergic receptor decreases SVR; this receptor also inhibits adenylyl cyclase (adenylyl cyclase converts ATP to cAMP)

Alpha 2

Stimulation of this adrenergic receptor increases contractility (mostly in the ventricles), increases HR (SA and AV nodes), and increases conduction velocity (higher risk for arrhythmias). This receptor induces cAMP production which causes heart muscle to contract and smooth muscles to relax

Beta 1

Stimulation of this adrengeric receptor decreases SVR and also causes bronchodilation

Beta 2

Infant with neonatal shock (gallop rhythm, hepatomegaly, and pulmonary edema) and echo showing thickened myocardium likely from glycogen storage disease. Would you use dobutamine or dopamine and why?

Dobutamine; DOpamine increases SVR and would cause the heart to work harder. Dobutamine DECREASED SVR

Neonate with "warm shock" (well perfused, bounding pulses, wide pulse pressure) with increased skin perfusion, and normal function by echo. Would you use high dose dopa, low dose dopa or dobutamina?

Use high dose dopa because it will increase SVR and lead to vasoconstriction which is what you want in this kind of distributive shock

What receptors does norepi act and what are the effects on HR, contractility, SVR, and BP

acts on Beta 1 and alpha 1 with a small amt of beta 2. Decreases HR 2/2 increase in vagal tone on SA and AV nodes. increase contractility (beta 1 on myocardial cells), increases SVR (alpha 1) and increases BP mostly 2/2 increase in SVR

When used for the management of hypotension, this class of drugs decreases the breakdown of catecholamines, increasing Ca 2+ levels in the myocardial cells, and upregulating adrenergic receptors.

Corticosteroids

What is the most common CHD a/w maternal DM?

VSD

What is the treatment for and infant with hypertrophic cardiomyopathy due to maternal GDM?

maintance IVF, mechanical vent if needed, correct hypoglycemia, echo and avoid inotropes, (avoid digitalis) because they could made the patients condition worse. Use beta blockers

When will HCM from maternal DM resolve/regress?

by 1 month of age irrespective of therapy

therapy for fetal SVT? What if the fetus has hydrops?

digoxin is first line. If there is hydrops, use flecainide or sotalol to the pregnant woman but monitor for arrythmia

What syndromes are a/w HLHS?

Holt-Oram, Jacobsen, T13, T18. and Turner's

Name the genes a/w HLHS?

NOTCH 1, HAND 1, GJA1, NKX2-5

What is the most common mechanism for SVT in the neonate?

orthodromic reciprocating tachycardia

What are possible negative side effect of milrinone?

hypotension, tachycardia, tachyarrhythmias, and thrombocytopenia