Congenital heart defects (CV) (Seth's Additional info) (10%)

Question 1

MC congenital heart disease in childhood

Answer 1

Ventricular Septal Defect

Question 2

Which congenital cardiac defect is associated with a “3 sign” on CXR? Boot-shaped heart on CXR?

Answer 2

3 sign = Coarctation of the aorta (notching)
Boot-shaped heart = TOF (d/t a prominent RV that looks like the heel of a boot)

Question 3

What is a VSD and what does it lead to overall?

Answer 3

Hole in the ventricular septum leading to a left-to-right shunt

left to right shunt for mild/mod

high pressure to low pressure

Question 4

While mild/moderate VSD is associated with a left to right shunt, large (unrestricted) VSD defects may lead to a ____ shunt, which is called _____.

Answer 4

Right to left shunt

Eisenmenger syndrome

Question 5

What is the MC type of VSD?

Answer 5

Perimembranous

Question 6

Where is the hole of a perimembranous VSD?

Answer 6

LV outflow tract near the tricuspid valve

Question 7

which congenital heart defect is associated with a “swiss cheese” pattern?

Answer 7

VSD muscular type

muscles from eating cheese

Question 8

Where is an inlet VSD located? What is it aka?

Answer 8

posterior to the septal leaflet of the tricuspid valve

aka posterior VSD

Question 9

Where is an outlet VSD located? What is it aka?

Answer 9

beneath the pulmonic valve, may have aortic valve insufficiency

aka supracristal VSD

Question 10

Small VSD is typically asymptomatic (may have mild symptoms), but a moderate VSD is associated with these symptoms

Answer 10

Excessive sweating or fatigue especially during feeds
lack of adequate growth (not getting enough blood)
frequent respiratory infxs (due to congested pulmonary vasculature

Question 11

what type of VSD is associated with no pressure difference between the ventricles?

Answer 11

Large (unrestricted)

further develops into right-to-left shunt = Eisenmenger syndrome

Question 12

describe the murmur associated with VSD

Answer 12

High-pitched harsh holosystolic murmur best heard @ lower left sternal border

Question 13

what part of the PE often differs a small vs larger VSD?

Answer 13

small is louder and often has a palpable thrill

diastolic rumble at MV sometimes

Question 14

Echo dx VSD showing size and extent
EKG often shows LVH because of left to right shunt
Sometimes EKG shows RVH + LVH in more severe disease, and this is called ______.

Answer 14

Katz-Wachtel phenomenon

Question 15

you choose to observe a VSD because it is small, but it must close by ____

Answer 15

12 months

otherwise surgery

Question 16

Surgery for VSD and when to do it

Answer 16

Patch closure

symptomatic infants or uncontrolled CHF, growth delay, recurrent respiratory infections, or no closure by 12 months

Question 17

2nd MC congenital heart disease

Answer 17

Atrial septal defect (ASD)

Question 18

What fetal heart component is a feature of an ASD?

Answer 18

Associated with a PFO

foramen ovale allows blood to flow from the RA to the LA

Question 19

Because there is a PFO associated with ASD, what type of shunt develops

Answer 19

left to right

acyanotic heart defect

Question 20

MC type of ASD and the others

Answer 20

Ostium secundum

there is also ostium primum, sinus venosis, and coronary sinus

Question 21

how does the age of first noticing symptoms differ in ASD vs VSD

Answer 21

ASD typically develops symptoms much later in life (like 3rd decade) as opposed to VSD (3rd week)

the atrium is not as big and it is easier to compensate early on

just as a rule of thumb, but any age can have symptoms

Question 22

How do the symptoms of ASD differ in infants and young children vs adolescence and adults?

Answer 22

Infants & young children: recurrent respiratory infx, failure to thrive, DOE
Adolescents & young adults: exertional dyspnea, easy fatigability, palpitations, atrial
arrhythmias, syncope, heart failure

Question 23

One of the main worries of ASD is the development of ____

Answer 23

paradoxical emboli

stroke from venous clots

Question 24

Explain the murmur associated with ASD

Answer 24

Systolic ejection crescendo-decrescendo flow murmur @ pulmonic area (LUSB – 2nd or 3rd intercostal space)
* Wide, fixed split S2 that does not vary with respirations, loud S1 & hyperdynamic right ventricle

Systolic ejection atrial = SEA

Question 25

definitive diagnosis of ASD and VSD

Answer 25

Cardiac cath ## Footnote rarely indicated though

Question 26

small ASD less than < () may be observed and often close by ()

Answer 26

<5mm 12 months (like VSD)

Question 27

Symptomatic treatment of ASD

Answer 27

Diuretics ACEI Digoxin

Question 28

What is the surgical correction for ASD and when is it typically done?

Answer 28

1cm+/symptomatic (between 2-4 y/o)→perQ transcatheter closure v. surgical intervention

Question 29

A patent ductus arteriosus (PDA) allows persistent communication between the ____ & ______ after birth

Answer 29

Persistent communication between the **descending thoracic aorta** & **main pulmonary artery** after birth

Question 30

t/f, like ASD and VSD, PDA is associated with a left to right shunt

Answer 30

True

Question 31

Use of ___ keeps the ductus arteriosus open

Answer 31

prostaglandin E1 ## Footnote continued production of this leads to a PDA

Question 32

What gender is MC for PDA?

Answer 32

female 2x risk | think PD**F**

Question 33

Like VSD and ASD, most PDAs are asymptomatic, but infants may develop ___ but the characteristic difference in severe disease is ___

Answer 33

poor feeding weight loss Lower respiratory tract infections Pulm congestion Endocarditis Eisenmenger syndrome **Cyanotic lower extremities**

Question 34

Murmur of PDA

Answer 34

* Continuous **machine-like** or “to and fro” murmur **loudest @ pulmonic area** (LUSB – 2nd intercostal space) * Wide pulse pressure (**bounding peripheral pulses**) with low DB, loud S2 ## Footnote P = pulmonic

Question 35

Best test to dx PDA

Answer 35

ECHOOOOO | like everything ## Footnote cardiac cath is definitive but rarely needed

Question 36

ECG and CXR findings of PDA

Answer 36

ECG: LVH, left atrial enlargement CXR: normal or cardiomegaly

Question 37

Treatment of PDA

Answer 37

NSAIDs | IV indomethacin or ibuprofen ## Footnote **inhibits PGE1**

Question 38

MCC of sudden cardiac death in young athletes in the US

Answer 38

Hypertrophic cardiomyopathy ## Footnote HCM

Question 39

Inheritance pattern of HCM

Answer 39

Autosomal dominant ## Footnote Gotta screen FHx in athletes

Question 41

Overall, what worsens the murmur of HCM?

Answer 41

Anything that causes **decreased preload**

Question 42

Affect of squatting vs standing/valsava for HCM murmur

Answer 42

1. Squatting decreases the murmur 2. Standing/valsava increases the murmur

Question 43

Why does exercise lead to a louder murmur and worse obstruction with HCM?

Answer 43

Higher demand increases contractility ## Footnote anytime your body requires more effort to pump blood = worse murmur and more dangerous

Question 44

What does dehydration do to HCM murmur?

Answer 44

louder murmur ## Footnote less preload and more effort to pump blood

Question 45

What drug (1) and class (1) are associated with increased contractility of the heart and therefore a worsened HCM murmur?

Answer 45

1. Digoxin 2. Beta antagonists ## Footnote Makes sense why BBs would be a treatment as a result!

Question 46

What drug worsens the murmur of HCM by decreasing AFTERload

Answer 46

Amyl nitrate

Question 47

Most common presenting symptom of HCM ## Footnote and some others

Answer 47

Dyspnea on exertion ## Footnote fatigue, **angina**, dizziness, etc

Question 48

What arrythmia is most associated with sudden death with extreme exertion and HCM

Answer 48

V fib

Question 49

Murmur of HCM () pitched () murmur () (systolic/diastolic) (cresendo/decrendo/cresendo) loudest at () extra heart sound () with () lift

Answer 49

Medium-pitched harsh mid-systolic crescendo-decrescendo murmur heard @ left sternal border often with a loud S4 gallop w/ apical lift.

Question 50

Like all murmurs, you should get an echo with HCM, which will show () EKG shows ()

Answer 50

Echo = **asymmetric** ventricular wall thickness (especially septal thickness) EKG: LVH

Question 51

1st line medical mng of HCM

Answer 51

BBs to lessen contractility of the heart ## Footnote Early detection is KEY

Question 52

Your young patient still has HCM that is not controlled with a BB, so you consider ___.

Answer 52

Myomectomy ## Footnote remove the hypertrophy

Question 53

The cardiologists deems that your patient is not a candidate for a myomectomy for HCM refractory to a BB, so the cardiologist may consider this procedure () or this invasive prevention () for syncope or sudden cardiac arrest

Answer 53

Alcohol septal ablation ICD

Question 54

What is the pathophys / description of coarctation of the aorta

Answer 54

Congenital **narrowing** of the **aortic lumen** at the **distal arch &/ descending aorta**

Question 55

Coarctation of the aorta is MC in ___ PDA is MC in ___ ## Footnote gender/sex

Answer 55

Coarc = male PDA = female ## Footnote think CAM (**C**o**A**rc **M**ale) think PDF (**PDF**emale)

Question 56

50% of patients with Coarctation of the aorta also have this cardiac abnormality

Answer 56

Bicuspid aortic valve ## Footnote COA BOA

Question 57

What is the overall difference between adult type and infantile type coarctation of the aorta

Answer 57

Adult = postductal Infantile = preductal ## Footnote classified by WHERE the narrowing occurs

Question 58

Explain the anatomic abnormality associated with POSTductal coarctation of the aorta ## Footnote adult type

Answer 58

narrowing occurs **distal** **to the ductus arteriosus**

Question 59

Explain the anatomic abnormality associated with PREductal coarctation of the aorta ## Footnote infantile type

Answer 59

narrowing occurs **proximal to the ductus arteriosus**

Question 60

what is the function of the ductus arteriosus

Answer 60

a short blood vessel that **connects the pulmonary artery to the aorta** in a fetus ## Footnote normally closes after birth

Question 61

Neonatal presentation of coarctation of the aorta is similar to most noncyanotic heart defects and is associated with ().

Answer 61

FTT poor feeding

Question 62

2 buzz word symptoms/presentations of coarcation of the aorta ## Footnote not talking about the murmur

Answer 62

1. Bilateral claudication 2. UE HTN with LE hypotension/diminished pulses ## Footnote also see DOE and syncope

Question 63

Explain the murmur associated with Coarcation of the aorta. () (systolic/diastolic) () (cresendo/decrendo/continuous) radiation to () loudest at ()

Answer 63

Late systolic ejection murmur/continuous murmur radiating to the left back, left scapula or chest, heard in the aortic area ## Footnote ARC at the sea

Question 64

T/F, unlike most other murmurs, the diagnositc test of choice for Coarctation of the aorta is actually a CXR instead of an echo

Answer 64

FALSEEEEEE | echo is the confirmatory test ## Footnote ALWAYS GET AN ECHOOOOOOO

Question 65

Echo and EKG finding associated with Coarctation of the aorta

Answer 65

Echo = narrowing of aortic lumen EKG = LVH (has to work harder)

Question 66

CXR finding of coartaction of the aorta

Answer 66

1. **3 sign** (aortic notch looks like a 3) 2. posterior rib notching (d/t increased intercostal artery collateral flow)

Question 67

What is the gold standard diagnostic test for coarctation of the aorta

Answer 67

Angiography | very invasive though ## Footnote makes sense

Question 68

Treatment of coarctation of the aorta

Answer 68

Corrective surgery or transcatheter-based intervention (balloon angioplasty) by 2-4 yo (emergent if dangerous s/s)

Question 69

Most patients receive ___ pre-op for coarctation of the aorta to stabilize prior to surg

Answer 69

Prostaglandin E1 (Alprostadil) ## Footnote maintains a patent ductus arteriosus, reducing symptoms @ improves lower extremity blood flow

Question 70

What is the life-expectancy and cause of death(s) associated with untreated coarctation of the aorta?

Answer 70

Most adults die by 50 y/o d/t aortic rupture, CVA, or aortic dissection

Question 71

You should r/o coarctation of the aorta in a young, healthy adult with ___

Answer 71

HTN

Question 72

MC cyanotic congenital heart disease

Answer 72

TOF

Question 73

What is the shunt associated with TOF and what causes is

Answer 73

Right to left ## Footnote d/t pulmonary stenosis

Question 74

What is the genetic abnormality sometimes associated with TOF

Answer 74

chromosome 22 deletion ## Footnote Think **t**e**t** = twenty-two

Question 75

Four abnormalities associated with TOF

Answer 75

PROV 1. Pulmonary stenosis (RV outflow obstruction) 2. RVH 3. Overriding aorta 4. VSD

Question 76

MC associated symptom with TOF

Answer 76

**Cyanosis** (blue-baby syndrome) especially with crying/feeding/increased energy requirement ## Footnote can see exertional dyspnea and progressive cyanosis in older patients

Question 77

How to relieve a tet spell in an infant vs child

Answer 77

Infant = flex knees to chest Child = squatting | kinda like HCM ## Footnote both increase preload and allow the heart to receive more blood, therefore decreasing right-to-left shunt

Question 78

Explain the murmur associated with TOF () (systolic/diastolic) loudest at ()

Answer 78

harsh systolic murmur @ left mid to upper sternal border (VSD), right ventricular heave (RVH)

Question 79

All tests of choices for congenital heart defects in these objectives involve

Answer 79

ECHOOOO ## Footnote also for TOF

Question 80

EKG finding of TOF

Answer 80

RVH RAE ## Footnote sometimes increased QRS = risk for sudden death

Question 81

CXR of TOF and why

Answer 81

Boot-shaped heart ## Footnote d/t prominent right ventricle (RV almost looks like the heel of a boot)

Question 82

Treatment of choice for TOF and when it should be done

Answer 82

Surgical repair ## Footnote ideally in first 4-12 months of life

Question 83

t/f, PGE1 is used prior to surgery for TOF in order to maintain the PDA and allow blood to flow from the pulmonary artery to the aorta

Answer 83

TRUE ## Footnote also do prophylatic ABX for endocarditis

Question 84

Complications of surgery of TOF

Answer 84

arrhythmias, pulmonary regurgitation, residual outflow obstruction, heart failure

Question 85

MCC of death of TOF

Answer 85

Sudden cardiac death and HF :(