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Congential heart defects Flashcards

1
Q

Atrial septal defect

A

left to right shunt d/t a hole in the septum leading to a high pressure symptoms

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2
Q

What are the symptoms of HF in general?

A

Small = asymptomatic
Over time = CHF

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3
Q

s/s of atrial septal defect

A

Fixed, split S2 and pulmonic ejection murmur (louder with age)
Infants and children :Respiratory infections, Failure to thrive
Adults (before 40): Palpitations, exercise intolerance, dyspnea, fatigue

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4
Q

growth of septum primum

A

septum primum leads to sotrium primum and then we have osteum secundum

septic secundum

this leads to the foramen ovale

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5
Q

What is the function of foramen ovale

A

allows blood to flow from LA to RA

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6
Q

What is the ostium secondum defect

A

defect where the foramen ovale

MC of the ASD

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7
Q

What is ostium primum common of?

A

down syndrome

because this happens earlier in development

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8
Q

Why do you get a split S2 ASD?

A

Some of the blood goes from the RA to the LA allowing it to close slightly before (2 sounds)

turbulence leads to a murmur

pulmonic systolic murmur increases with time

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9
Q

How wo diagnose ASD

A

ECHO

also right heart cath

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10
Q

what would you see on cxr of ASD?

A

Right heart dilation because of the shunt allowing it to have to pump harder

  • Prominent pulmonary vascularity
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11
Q

What do you see for increased concentration in for ASD?

A

RA
RV
pulmonary artery

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12
Q

Treatment of ASD

A

wait and watch for small lesions <8 mm

try not to rush the surgery and let them grow unless hemodynamic

surgery at 1-3 yo

if you wait too long you will get reverse flow and worse prognosis

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13
Q

What is ventricular septal defect?

A

VSD

Right heart failure d/t blood flow leading from LV to RV

Heart failure
▸ Pulmonary HTN
▸ Arrhythmias
▸ Stroke

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14
Q

How does the septum grow

A

from bottom to top

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15
Q

How does the top and bottom septum differ?

A

Upper is more membranous
Lower is more muscular (more concerning)

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16
Q

What is the MC VSD?

A

Membranous - upper septum (most common but less concerning)

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17
Q

What is an inlet VSD?

A
  • in the posterior portion of the V septum beneath the TV
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18
Q

Why is VSD typically a-cyanotic?

A

left to right shunt

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19
Q

When do you likely not need treatment for VSD?

A

< 6 mm

if greater than this, then surgery after 2

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20
Q

What happens if there is too big of a LV -> RV shunt

A

pulmonary HTN, which can eventually lead to a right to left shunt instead (cyanosis and dyspnea)

want to get surgery done before this

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21
Q

VSD murmur

A

holosystolic murmur (bigger than ASD because of ventricular)

poor feeding
failure to thrive
CHF
worsening tachypnea
hepatomegaly

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22
Q

when is congenital heart defects most likely to lead to death

A

birth up to a month

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23
Q

VSD diagnostic

A

Echo (1st choice)
MRI
CXR might show LAE

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24
Q

What do you do if echo is undiagnostic for VSD? When would you also get this no matter what?

A

Cardio cath

always order if pulmonary HTN

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25
Treatment of VSD
small VSD close on it's own Lasix if CHF higher calories because they will have tachypnea (concentrate the formula!) surgery after 2 years old
26
what are the 2 surgeries of VSD
Patch closure over ventricular septaI defect (preferred treatment) Transcatheter closure : Mesh to close VSD (higher risk)
27
When would you do surgery <2 yo
VSD > 8 mm unstable pulmonary HTN Aortic insufficiency LA/LV dilation
28
What is patent ductus arteriorsis and why is it concerning?
Can actually be useful to survive often have other problems though
29
What does the PDA connect
Persistence of the normal fetal vessel that joins the PA to the Aorta.
30
When does the PDA normally close?
Normally w/in the first week of life
31
MC and 2nd MC heart defect
VSD MC PDA 2nd MC
32
What is the RF of PDA
Higher incident in preterm infants weighing​ <1500 grams and infants born at higher altitudes >10,000 feet Females > Males (2:1)​
33
PDA physiology
Blood goes to aorta and bypasses the
34
2 Pathognomic for PDA
holosystolic machine-like murmur also differential cyanosis (upper extremities are normal because blood first goes to UE but feet are blue because the blood is not oxygenated d/t bypass)
35
s/s of PDA
Depend on size of PDA Smaller Usually asymptomatic Neonates: holosystolic "machine-line" murmur on auscultation Infants, children, adults: continuous murmur Moderate Exercise intolerance Continuous murmur Wide systemic pulse pressure Displaced ventricular apex Larger Infants: leads to heart failure Children: shortness of breath, fatigability, Eisenmenger syndrome some of the oxygenated blood goes back to the lungs do not see cyanosis until deoxygenated blood goes from aorta to the extremeties
36
Dx of PDA
Echo EKG shows Left ventricular hypertrophy, left atrial enlargement
37
When do you see murmur of PDA
increased O2 demand
38
treatment of small PDA
monitor
39
treatment of PDA if you wanna close it?
protoglandan inhibitor Symptomatic moderate/large PDA During heart failure : Digoxin, furosemide monitor
40
If prostoglandin inhibitor fails to close, what is required?
surgery
41
pulmonary stenosis 3 scenarios
Stenosis of the valve itself ​ Most Common - valve has 3 leaflets and they are either thickened or fused ​ Thickened muscle below valve​ Stenosis of the pulmonary artery below valve​
42
Microangiopathic hemolytic anemia
pathgonomic for PULMONARY STENOSIS meaning that there is mechanical damage
43
what are the s/s of pulmonary stensosis
Systolic ejection murmur at the LUSB which increases with inspiration​ opening click 
44
diagnosis of pulmonic stenosis
ECHO: EKG shoes RVH d/t trying to push against it
45
treatment of pulmonary stenosis
valvoplasty or surgery if unsuccessful
46
Coarctation of aorta
Part of the aortic arch (usually the proximal descending aorta) is narrower than usual causing blockage of normal blood flow to the body, backing blood flow into the left ventricle. This causes the muscles to work harder to get the blood out of the heart​
47
Coarctation of aorta MC population
females w/ Turner syndrome
48
What is the aortic valve of coarctation?
Coarctation of aorta
49
What is coarctation pathophys 2 types
narrowing leading to not enough O2 in systemic circulation
50
What helps Coartcation of aorta?
If you have PDA so that there can actually be more blood flow into systemic circulation (even though it is deoxygenated) also leads to RAAS system
51
BP of coartaction?
UE have higher BP (above coarctation) but LE have lower BP difference of more than 20 BP is most likely this
52
Adult coarctation
PDA closes increased BP upstream increased brain aneursym increased risk of aortic dissection LE is opposite: pale, decreased BP
53
S/s of adult coarctation
54
what might be the only way that coarctation presents? What should you do?
no femoral pulse ORDER AN ECHO need to measure pressure on the right side
55
What is the presentation of coarctation of the aorta?
Secondary hypertension Severe heart failure, shock if/when PDA closes Other symptoms may more apparent with age Chest pain, cold extremities, claudication on exertion Left ventricular impulse palpable, sustained Pulsations felt in intercostal spaces
56
What does a CXR show of coarctation of aorta?
Rib notching: 3-sign (narrowed aorta resembles notch of number 3 due to prestenotic of aortic arch & postenotic of descending aorta dilatation) pathognonimic!
57
Treatment of coarctation of aorta
Prostoglandin in order to keep PDA so that there is an increase of flow the LE eventually surgery
58
coarctation prognosis
Survival through the neonatal period without developing heart failure tend to do well throughout childhood and adolescence​  Infective endocarditis is rare before adolescence, but can occur in both repaired and unrepaired coarctation​  correction after age 5 yrs are at increased risk for HTN and myocardial dysfunction​​
59
What is mandatory for coarctation if they do athletic activities?
Exercise testing is mandatory for these children prior to their participation in athletic activities​
60
What happens with aortic stenosis?
ventricle has to work hard to pump the blood, leading to a delay in the opening - leading to a click murmur will increase with time, leading to cresendo-decresendo murmur
61
what ventricle is hypertrophic with aortic stenosis?
left
62
when do you order a cath for aortic stenosis?
60​ - 80 mmHg
63
what is the treatment of aortic stensosi
similar to pulmonic stenosis balloon valve (ross procedure)
64
What is the prognosis of aortic stenosis
pretty good severe obstruction can lead to arrythmia (cannot clear for sports)
65
What is worrying of cyanosis? What typically causes this?
MUCOUS MEMBRANES Presence > 3 g/dl deoxy HgB correlates with 80-85% SpO2
66
Why can anemia make it hard to tell if there is cyanosis?
Less blood cells making it harder to visualize because of less concentration
67
What is hyperoxia test?
Give 100% O2 and if no improvement, then it is cardio! other etiologies (sepsis, ect) will improve
68
hyperoxia test and what to do if cardio
O2 stat increases to 100% = lung disease O2 stat to 75% = cardio orgin cardio origin = prostoglandins
69
What are the 5 Ts of cuanotic heart disease?
Truncus Arteriosus TGA (d-Transposition of the great arteries) Tricupsid Atresia Tetralogy of Fallot Total anomalous pulmonary venous return
70
What is the MC cyanotic heart defect?
tetralogy of fallot
71
tetraology of fallot
Pulmonary stenosis Large VSD Overriding Aorta Right Ventricular Hypertrophy
72
Pulmonic stenosis in tetraology of fallot
narrowing of RV outlet causing decrease BF to the pulmonary artery causing thickening of the ventricular wall makes it harder for deoxy blood to get to to pulmonary circulation
73
what is pathognomic for tetralogy of fallot?
Boot shape cxr
74
what is a tet spell?
patient might look ok, but there is HYPERcyanotic epsiodes with increased demands anything that increases O2 demand or decreases SVR Pathognomic! fever, exercise, agitation can be life threatening if untreated!
74
How to stop an acute tet spell
increase venous return by squatting or flexing the knees, which will reverse the right to left shunt (because the veins squeeze, backing to the left side of the heart). calm the baby to stop crying (to decrease the O2 demand) hydration meds
75
What are the meds for a tet spell
Morphine (relaxes) Bicarbonate (to correct metabolic stenosis, dilating pulmonary pressure) Phenylephrine to increase SVR BB to decrease dynamic RVOT obstruction
76
Long term management of tet spell
SURGICAL REPAIR
77
Transposition of great arteries heart defect?
Aorta comes off RV (instead of LV) PA comes off LV critical! deoxygenated blood comes to the RA, RV, then aorta to the body (never to the lungs)
78
D-transpostion of great arteries
Oxygenated blood goes from pulmonary artery to the lungs and never goes to the body parallel circuit focus on this one
79
What is the L type?
switch of the ventricles, not the arteries not as sick, not going to die leads to HF over time
80
D-transpostion of great arteries shunts that help
PDA or VSD
81
MC patient population for transposition of great arteries?
Males > females 3:1 NEED to treat immediately
82
RF of mom having kid with transposition of great artery
Diabetes Rubella Poor nutrition Consumption of alcohol > 40 years old
83
If pulse ox is higher in lower extremetiy than upper extremetiy, what is there?
transposition of great artery start prostoglandin to keep the PFO open
84
Classic triad of CXR of transposition of great arteries
"egg on a string" narrowed mediastinum (near the trachea) Lung congestion Cardiomegaly
85
treatment of transposition of great arteries
prostoglandins while waiting for helicpoter to ICU
86
What is hypoplastic left heart syndrome?
Underdeveloped left ventricle, ascending aorta - Aortic/mitral valves may also be affected, narrow, or absent (atresia) If untreated: left-sided heart failure >cardiogenic shock > death no blood is leaving left side to go to the rest of the body because of narrowing need PDA or ASD
87
s/s of hypoplastic heart
same as cyanotic heart failure
88
treatment of all cyanotic heart defects
prostoglandins to maintain PDA 3 step surgery
89
can hypoplastic heart kids play in sports?
NO only one that is completely restricted
90
6 common innocent murmurs in childhood
Newborn murmur (1st days or hours) Peripheral pulmonary stenosis Still's murmur Pulmonary ejection murmur venous hum carotid bruit if soft, not as worried.
91
what is pathognomic for still murmur
Loud when supine; disappears with inspiration or sitting Loud with anemia and fever

Pediatrics (7 decks)

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