CARDIAC Flashcards
(106 cards)
1
Q
CHD
A
Congenital heart defects 1% of life births
2
Q
errors in cardiac morphogenesis
A
septation, valve formation, pattering of the great vessels
3
Q
development of the heart
A
primordia (lateral plate mesoderm)
primitive streak (ectoderm and endoderm layers)—symmetric mesoderm
body folding
“simple tube” contracts rhythmically (dilations-heart chambers; constrictions–partition between chambers)
major morphogenesis process done at 8 weeks post-conception
4
Q
the tubular and looping heart
A
looping, convergence, and wedging
5
Q
cardiac jelly
A
creates a distinct layer between the myocardium and endocardium
6
Q
looping stages
A
“S” shape; “D” Dextro; “L” Levo…results in left ventricle on the right and right ventricle on the left
defects: mutation in ciliary ultrastructures)
7
Q
convergence
A
process of proper orientation of the inflow (cranially) and outflow (ventral) tracks
defects: neural crest cell-associated diseases DiGeorge
8
Q
wedging
A
parallel with looping and convergence; outflow thack rotates;
defects: conotruncal defects: tetralogy of Fallot, double outlet right ventricle
9
Q
septation
A
begins with swelling of the extracellular matrix between the endocardium and the myocardium (AV junction, outflow track, primary atrial septum, ridge of the interventricular septum)
10
Q
neural crest cells
A
contribute to the development of the heart and associated vessels: walls of the aortic arch arteries
separation of the aortic and pulmonary trunk
formation and maturation of the cardiac conduction system
defects: inflow anomalies and abnormalities of the aortic arch arteries
11
Q
cardiac conduction system development
A
CCS
12
Q
epicardium
A
relatively late-forming cardiac tissue, the outer layer of the heart; development of coronary vessels and valve development
13
Q
cardiomyopathies
A
intrinsic disorders classified as hypertrophic, dilated, restrictive and arrhythmogenic
14
Q
can myocardial cells divide after birth?
A
NO
15
Q
primary heart field
A
L ventricle, portions of the AV canal
16
Q
second heart field
A
(adjacent to neural crest cells) outflow tract, R ventricle, atrial formation
17
Q
pulmonary vascular development
A
embryonic phase
pre-acinar vascular branching pattern present by 20th week GA
18
Q
angiogenesis
A
budding, sprouting and branching of existing vessels to form a new ones
19
Q
vasculogenesis
A
de novo organization of blood vessels produced by migration and differentiation of endothelial progenitor cells or angioblasts
20
Q
hypoxic condition of fetal life support lung vascular growth T/F
A
TRUE
21
Q
hypoxic intrauterine environment
A
HIF hypoxia inducible factors involved in angiogenesis, survival and metabolic pathways: VEGF (vascular endothelial growth factor); medicated by Nitric oxide
22
Q
mediators of fetal pulmonary vascular tone
A
pulmonary hypertension is normal in fetal life
uses foramen ovale and ductus arteriosus to bypass the lungs
low oxygen environment,
low basal production of vasodilators (PgI2, and NO)
increased production of vasoconstrictors
23
Q
pulmonary vascular transition
A
decrease in PVR at birth
close of foramen ovale
close of ductus arteriosus
longs inflation with gas and increase in oxygen tension
24
Q
central vasodilators
A
NO
Prostacyclin
25
abnormalities of pulmonary vascular development
persistent pulmonary hypertension of the newborn (PPHN)
Congenital Diaphragmatic Hernia (CDH)
Alveolar Capillary Dysplasia (ACD)
Pulmonary Hypertension (PH)
26
PPHN
right to left extrapulmonary shunting of deoxygenated blood that produces hypoxemia
three categories:
1. lung parenchymal disease (MAS, RDS, pneumonia)
2. idiopathic
3. hypoplastic (CDH)
cases: antenatal NSAIDs, SSRI
genetics: downs syndrome
elective cesarean delivery, maternal DM, asthma, high BMI
perinatal asphyxia
oxidant stress
27
CDH
abnormal diaphragm development, herniation of abdominal viscera into the chest and variable degree of lung hypoplasia
28
ACD
interstitial lung disease that presents as severe PH and hypoxemia early in life
genetic defect of a pulmonary capillary bed
fatal
29
PH
extremely premature birth, associated with oligohydramnios
caused by BPD
pulmonary vein stenosis
30
Clinical therapy of PPHN
respiratory distress, labile oxygenation, differential saturation (higher in R hand) profound hypoxemia despite oxygenation and mechanical ventilation
usually in term
ECHO mandatory to rule out congenital heart disease
31
PPHN ECHO
right to left shunting:
foramen ovale, ductus arterious, left deviation of the intraventricular velocity, tricuspid regurgitant velocity, increase in R ventricular dilation
requires lung recruitment, optimization of right and left ventricular function, and maintenance of PaO2 between 60 and 80
32
PPHN management
normal temperature, electrolytes (calcium), glucose and intravascular volume
systemic BP at normal levels
maintain normal L ventricle function
33
PPHN management guidelines
iNO then ECMO OI more than 25
iNO for PPHN w/ parechymal lung disease
evaluate for developmental disorders: ACD or surfactant deficiency
Sidenafil, inhaled prostacyclin, IV milrinone
34
PPHN maternal factors
diabetes, high body mass index, smoking, use of SSRIs or NSAIDs, and caesarean section
35
PPHN postnatal factors
perinatal asphyxia, hyperoxia, hypoxia, infection, and lung inflammation
36
best timing for fetal ECHO
18-22 weeks
37
fetal myocardium
noncontractlie elements (60%)
fetal cardiomyocytes can divide
slow removal of Ca from troponin C(slower muscle relaxation)
equal R and L wall thickess
38
increased preload cases what?
```
fetal hydrops (cause by anemia, viral illness, significant arterial to venous malformations)
caused by placental edema (AV malformations, sacroccygeal teratomas, TTTS)
```
39
what organ has the lowest vascular resistance in fetal circulation?
placenta
40
fetal indications caregories
fetal
maternal
genetics
41
what is the minimum for fetal ECHO
thorough two-dimensional imaging, color Doppler, and spectral Doppler examination of the four-chamber view, both arterial outflow tracts, three vessels and trachea view, and an assessment of pulmonary venous return
42
fetal pathology categories
```
structural anomalies (tx post)
functional anomalies (tx post)
rhythm disorders (tx prenataly)
```
43
Prostoglandin dependent CHD lesions types
too little pulmonary blood flow
| too little systemic blood flow
44
Transposition of the great arteries TGA
results in systemic venous return with relatively low oxygen content directed to teh ascending aortal results in CNS and cardiac hypoxia
45
fetal heart failure is associated with what?
placental edema and resultant hypoxia
46
DX of causes of Hydrops Fetalis
```
immune
cardiac
twin-twin transfusion
arteriovenous malformation
diaphragmatic hernia\cystic hygroma
```
47
TTTS twin to twin syndrome
DONOR: volume depletion and nephrosclerosis
RECIPIENT: increased volume, vasoconstriction
48
PPHN inodilator
milrinone; improve left ventricular function and reduce pulmonary blood flow and oxygenation
49
Prostanoids PPHN
IV PGI2; with neonates concern about systemic hypotension and/or ventilation-perfusion mismatch
inhaled PGI2 produce transient pulmonary vasodilation and enhanced oxygenation
50
fetus circulation overview
placenta (oxygenated blood) to foramen ovale to left atrium
| highly oxygenated blood: umbilical vein to ductus venosus to inferior vena cava through FO by Eustachian valve
51
two different output systems
systemic: of the LV
pulmonic: RV towards placenta
52
steps for performing fetal ECHO
segmental approach:
fetal position: right-left and cranial-caudal axis
fetal abdominal situs
morphologic markers
53
fetal rhythm abnormalities
ectopic beats
tachycardia
bradycardia
54
Ductus arteriosus
connection between the pulmonary artery and descending aorta; deoxygenated blood returning to th eright heart is diverted to the placenta for reoxygenation; excessive pulmonary blood flow causing diseases like RDS, BPD, NEC, IVH
55
what causes the PDA to close?
```
rapid postnatal increase in arterial oxygen tension;
fall in PGE 2 from fetal placenta
prenatal glucocorticoids
dynamic closure 4 days
anatomic closure 1 week
```
56
fetal PDA direction
right to left
57
newborn PDA direction
left to right
58
PDA clinical presentation
machinery murmur
increased precordial impulse
prominent arterial pulses (palms)
1000g +: decreased systemic arterial diastolic BP and increased pulse pressure
1000g -: reduction in diastolic and systolic BP withouth widened pulse pressure
59
PDA diagnostics
Doppler ECHO
BNP marker elevation (IVH risk and neurodevelopmental effects
xray: enlarged cardiac silhouette, pulmonary edema, nonspecific
60
consequences of ductal shunt on ECHO
left heart volume load, cardiac function, systemic hypoperfusion
61
PDA management
surgical ligation
indomethacin
NSAIDs ibuprofen/acetaminophen
62
indomethacin side effects
with glucocorticosteroids increase risk for bowel perforation
oliguria
NEC
63
ibuprofen PDA side effects
bilirubin displacement
64
ligation side effects
severe left ventricular disfunction and cardiorespiratory decompensation immediate post surgery
65
cyanotic heart defect: mixing dependent
D-Transposition of the Great Arteries (D-TGA)
aorta arises from RV, receives systemic deoxygenated blood
pulmonary artery arises from LV, receives venous oxygenated blood
deoxygenated blood recalculating the body
oxygenated blood recalculated the lungs
egg shaped heart
surgery: arterial switch operation
66
Cyanotic heart defect; restricted pulmonary flow ToF
tetralogy of Fallot (ToF)
large ventricular septal defect
infundibular and valve pulmonary stenosis
RV hypertrophy
large aorta overriding the ventricular septum
clinical presentation: RV outflow track obstruction determines the saturation, boot shaped heart
67
Cyanotic heart defect; restricted pulmonary flow TA
Tricuspid Atresia platelike tissue in place of the tricuspid valve with no direct communication between RA and RV
clinical presentation: cyanosis and murmur, hepatic enlargement,ECHO, ECG
surgery: bidirectional cavopulmonary anastomosis Glenn ot total cavopulmonary operation/Fontan
68
Cyanotic heart defect, restricted pulmonary flow PA/IVS
pulmonary atresia with intact ventricular septum
well-formed pulmonary annulus with a plate that obstructs outflow to complete absence of the valve
pulmonary flow depends only on PDA
clinical presentation: cyanosis, murmur; cardiomegaly
surgical: valvotomy
69
cyanotic heart defect: restricted pulmonary flow EA
Ebstein Anomaly
arrested delimitation of the leaflets during valve development; conduction abnormalities
clinical presentation; cardiomegaly
70
Cyanotic heart defect: complete mixing TAPVC/TAPVR
total anomalous pulmonary venous connection or return
all pulmonary being caring oxygenated blood drain into the systemic venous circulation rather than normally to LA
clinical presentation: symptomatic, xray: pulmonary edema, snowman sigh
71
cyanotic heart defect: complete mixing TA
trunks arteriosus
common origin of the aorta and pulmonary artery from a single arterial trunk
DiGeorge
clinical presentation: saturation in mid-80s%, murmurs, bounding pulses, increased pulse pressure
RX: diuretics and digoxin
72
cyanotic heart defect: variable physiology
Single Ventricle Anatomy
Hypoplastic Left Heart Syndrome (rx: provide adequate systemic blood flow and effective control of pulmonary blood flow)
double outlet RV (aorta and pulmonary artery both arise from RV
73
obstructive heart defects
Valvar aortic stenosis (murmur and click)
Aortic Coarctation and interrupted Aortic Arch (Turner, diminished femoral pulses and BP and O% differentials between the upper and lower extremities
Serial Obstructive Left heart defects
Valvar Pulmonary Stenosis (PS)
74
Shunting Lesions
Ventricular Septal Defect (murmur at left sternal border, oral diuretics
Atrial Septal Defect
Atrioventricular Septal Defect (trisomy21; murmur at the apex
PDA (machinery murmur, , significant L-R shunting,, wide pulse pressure and bounding pulses
Aortopulmonary Window
75
Situs
the development of morphologically right-side structures on one side of the body, and morphologically left-sided structures on the other side
solitus-normal
inverses-opposite side of the body
76
dextrocardia
base to apex axis of the heart is directed to the right side of the chest
77
mesocardia
apex is midline
78
Arteriovenous malformations
AVMs abnormal direct connection between arteries and veins via a network of vessels called the NIDUS that lack an intervening capillary bed
Galen malformation: cranial bruit, systolic ejection murmur
RX; milrinone, iNO, loop diuretics
79
hypertrophic cardiomyopathy
infants od DM moms
80
pericardial effusion
increased fluid within existing pericardial space, fetal hydrous, pericardial tamponade
81
cardiac tumor
rhabdomyoma
82
vascular rings and slings
cause compression on trachea, esophagus or both
| respiratory difictulties) stridor or wheezing)
83
Marfan Syndrome
skeletal, ocular Cvm cutaneous, urologic, pulmonary manifestation, family HX
84
sinus node
initiation of cardiac electrical activity; posterior junction of the superior vena cava and RA
85
atropine and bradycardia
increases rate almost immediately, no change in HR if primary sinus node dysfunction
86
sinus arrhythmia
"respiratory" HR increase on inspiration, and decrease with expiration; Bainbridge reflex
change in rate no change in P-wave
87
premature atrial contractions
conducted, nonconducted, aberrantly conducted
"blocked" PAC- no QRS complex after P-wave
couplet two in the row
bigeminy every other
atrial ectopic tachycardia" three or more consecutive PACs with rate fatre than 120
exclude IV placement hat might cause this
88
Atrial Flutter
rate 300-600, ventricular way slower, sawtoothhhhh pattern
| RX: cardioversion,
89
Supraventricular tachycardia
SVT
Wolff-Parkinson-White, Ebstein anomaly
1.atrial fib in the presence of an accessory pathway (adenosine usually contradicted)
2,concealed accessory pathway
3. atrioventricular nodal reentrant tachycardia, no true accessory pathway
200-300 BPM
RX: adenosine(0.1-0.4mg/kg), vagal stimulation (ice on face over 10-15 sec) or cardioversion
propranolol 4mg/kg/day
digoxin 4-5 mcg/kg
90
premature ventricular contractions
spontaneous depolarization of the ventricle myocardium resulting in an early or premature ventricular contraction
91
ventricular Tachycardia
RX:
1. normal anatomy: no treatment needed
2. 200-220 BMP use propranolol, or flecainide, aotalol, amiodarone
3. very fast or incessant tachycardia: IV amiodarone or sotalol
92
"sine wave"
The hallmark feature of severe electrolyte or acid-base disturbances is a loss of delineation between the QRS complex and the T wave.
93
First degree atrioventricular block
manifested by prolongation of the PR interval on the ECG while each sinus or atrial beat reaches the ventricles PR longer than 160msec
94
second-degree atrioventricular block
some, but not all, sinus beats do not conduct to the ventricles. On ECG, this results in some of the P waves not being followed by a QRS complex "dropped QRS"
95
complete block
there is no conduction of sinus beats from the atria to the ventricles due to abnormality of the AV node or bundle of His or both bundle branches.
MOB has: systemic lupus erythematosus or mixed connective tissue disease, particularly Sjögren syndrome
96
long QT syndrome
mulitifaced genetic, torsades de pointes, syncopal episodes and seizures
SIDS?
97
torsades de Pointes RX
dc all meds causing prolonged QT interval
avoid amiodarone
Magnesium bolus and continuous infusion even if the serum magnesium level is normal.
Isoproterenol
98
Prostoglandin E 1 dosing
0.03mcg/kg/min up to 0.2mcg/kg/min
99
preload meds
loop diuretics: furosmide, bumetanide
thiazide diuretic: chlorothiazide
potassium sparing: spironolactone
100
afterload meds
milrinone
afterload-reducing, inotropic, and lusitropic (ventricular relaxing) properties
dobutamine (significant elevation of HR)
pulmonary overload: oxygen and iNO, sidenafil or bosentan
101
contractility
digoxin
dopamine and dobutamine
epinephrine
102
tet spells: hypercyanotic spells
ToF, dynamic subpulmonary obstruction of the right ventricular outflow
103
difference between fetal and newborn myocardial function?
neonate has the ability to increase cardiac output with increasing atrial pressures (preload)
104
aneuploidy CHDs
trisomy 13: conotruncal defects, septal defects, valvular anomalies
trisomy 18: septal and polyvalvular dysplasia
trisomy 21:
AV defect, and ventricular septal defect VSD
Turner syndrome: left-sided cardiac structures: aortich arch and valve abnormalities
105
Chromosomal deletions CHDs
Digeorge: conotruncal
Jacobsen syndrome: VDS and left sided obstructive defects
Williams Syndrome: supravalvular ourtic stenosis and pulmonary stenosis
VACTERL: VSD and atrial septal defect
Beckwith-Wiedemann Syndrome: cardiomegaly, PDa, and PFO; risk for developing cancers
106
Tof F
```
pulmonary artery stenosis,
VSD
overriding aorta
RV hypertrophy
"boot shaped" heart
blue or pink ToF cyanotic/noncyanotic
tet spells paroxysmal hypercyanotic episodes
```
