Structural Cardiac Disorders and CM

Question 1

What is the MC type of congenital heart disease?

Answer 1

VSD

Question 2

What are the 4 types of VSD? Which is MC?

Answer 2

1. Perimembranous (MC): hole in LV outflow near tricuspid valve
2. Muscular: multiple holes in “swiss cheese” pattern
3. Inlet: posterior to tricuspid valve
4. Supracristal: Beneath pulmonic valve

Question 3

What are the s/s of small (restrictive) VSD?

Answer 3

Asx or mild
Found incidentally due to murmur
Restrictive = normal pressure btwn ventricles maintained

Question 4

What are s/s of moderate VSD?

Answer 4

Excessive sweating or fatigue, esp. during feeds

Question 5

What are s/s of Eisenmenger’s syndrome?

Answer 5

Right to left shunt

Asx at rest, but +/- cyanosis*, dyspnea, CP, & syncope

Question 6

What does VSD look like on PE?

Answer 6

Loud high-pitched holosystolic murmur at lower left sternal border

Mod VSD: +/- thrill, diastolic rumble at mitral area

Large VSD: signs of CHF

Question 7

How do you dx VSD?

Answer 7

CXR: LA enlargement, RV hypertrophy
Echo: preferred over cath
ECG: LVH/RVH 
MRI
Cath

Question 8

How do you treat VSD?

Answer 8

Most close spontaneously within 10 years

Patch closure if sx

Larger shunts are repaired by age 2 to prevent pulmonary HTN

Question 9

What are characteristics of coarctation of the aorta?

Answer 9

Increased LV afterload –> HTN, LVH, CHF

70% also have bicuspid aortic valve

Question 10

What are s/s of coarctation of the aorta?

Answer 10

Secondary HTN*

Bilateral claudication*, dyspnea, syncope

Question 11

What does coarctation of the aorta look like on PE?

Answer 11

Systolic murmur that radiates to back/scapula/chest

Increased BP upper > LE

Delayed/weak femoral pulses

Question 12

How do you dx coarctation of the aorta? What is gold standard?

Answer 12

CXR: Rib notching, “3 sign”

ECG: LVH

Angiogram = GOLD

Question 13

How do you treat coarctation of the aorta?

Answer 13

Surgical repair
Balloon angioplasty +/- stent
PGE1

Question 14

What is the MC cyanotic congenital heart disease?

Answer 14

Tetralogy of fallot (right to left shunt)

Question 15

What are causes of tetralogy of fallot?

Answer 15

1. RV outflow obstruction (pulmonary artery stenosis)
2. RV hypertrophy
3. VSD (large unrestrictive)
4. Overriding aorta

Question 16

What are s/s of tetralogy of fallot?

Answer 16

Blue baby syndrome (cyanosis)

“Tet-spells”: older children relieve spells by squatting

Eisenmenger’s syndrome (seen w/ VSD, PDA, & TOF)

Question 17

What does tetralogy of fallot look like on PE?

Answer 17

Harsh holosystolic murmur @ left upper sternal border

Right ventricular heave

Question 18

How do you dx TOF? What is GOLD?

Answer 18

CXR: boot shaped heart

ECG: RVH, RAE

Echo: GOLD

Question 19

How do you treat TOF?

Answer 19

Surgical repair performed in 1st 4-12 mos of life

PGE1 infusion (prevents ductal closure)

Question 20

What is patent ductus arteriosus?

Answer 20

Communication btwn descending thoracic aorta & pulmonary artery

Left to right shunt

Question 21

What causes PDA?

Answer 21

Prematurity
Perinatal distress & hypoxia
Rubella inf in 1st trimester

Continued PGE2

Question 22

What are s/s of PDA?

Answer 22

Most asx

Poor feeding, wt loss

Eisenmenger’s syndrome: left to right shunt switches & becomes right to left shunt

Question 23

What does PDA look like on PE?

Answer 23

Continuous machinery murmur loudest @ pulmonic area

Wide pulse pressure: bounding peripheral pulses

Question 24

How do you dx PDA? What is gold standard?

Answer 24

CXR: normal or cardiomegaly
ECG: LVH, LA enlargement
Echo = GOLD

Question 25

How do you treat PDA?

Answer 25

IV indomethacin 1st line (closes PDA) Surgical correction if indomethacin fails. Best to be performed before 1-3yo

Question 26

What are characteristics of dilated CM?

Answer 26

Systolic dysfunction --> ventricular dilation --> dilated weak heart MC in men, 20-60yo

Question 27

What causes dilated CM?

Answer 27

- Idiopathic MC - Viral myocarditis (enteroviruses MC, PB19, Chagas) - Toxic: Etoh, cocaine, anthracyclines (doxorubicin) - Pregnancy

Question 28

What are s/s of dilated CM?

Answer 28

Systolic HF sx: Both L & R sided + S3 Viral myocarditis: viral prodrome a few wks --> signs of HF or CP, + cardiac enzymes

Question 29

What does dilated CM look like on PE?

Answer 29

L sided HF: pulmonary congestion R HF: peripheral edema, increased JVP, hepatic congestion

Question 30

How do you dx dilated CM?

Answer 30

Echo: - LV dilation: thin walls - Decreased EF - Regional or global LV hypokinesis CXR: Cardiomegaly

Question 31

How do you treat dilated CM?

Answer 31

Standard systolic HF tx: ACEI, diuretics, etc. Implantable defibrillator if EF < 35%

Question 32

What are characteristics of restrictive CM?

Answer 32

Diastolic dysfunction Ventricular rigidity impedes ventricular filling (decreased ventricular compliance)

Question 33

What causes restrictive CM?

Answer 33

Infiltrative diseases: - Amyloidosis MC - Sarcoidosis

Question 34

What are s/s of restrictive CM?

Answer 34

R sided HF MC than L sided tachyarrhythmias

Question 35

What does restrictive CM look like on PE?

Answer 35

Kussmaul sign (increased JVP w/ inspiration) R-sided HF: peripheral edema, increased JVP, hepatic congestion

Question 36

How do you dx restrictive CM?

Answer 36

Echo: - marked dilation of both atria - diastolic dysfunction

Question 37

How do you treat restrictive CM?

Answer 37

No specific tx | Treat underlying cause

Question 38

What are characteristics of hypertrophic CM?

Answer 38

Diastolic dysfunction (due to impaired ventricular relaxation/filling) Subaortic outflow obstruction: hypertrophied septum + systolic anterior motion of mitral valve increased w/ increased contractility & decreased LV volume

Question 39

What causes hypertrophic CM?

Answer 39

Inherited genetic d/o of L &/or R ventricular hypertrophy

Question 40

What are s/s of hypertrophic CM?

Answer 40

``` Often asx dyspnea 90%. MC initial complaint Angina, syncope Arrhythmias Sudden cardiac death (esp. exertion) due to VF ```

Question 41

What does hypertrophic CM look like on PE?

Answer 41

Harsh systolic cres-decresc murmur @LLSB (sounds like aortic stenosis) - decreased murmur intensity: increased VR (squatting, supine); handgrip - increased murmur: decreased VR (valsalva & standing) & exertion, amyl nitrate Usually no carotid radiation. Loud S4

Question 42

How do you dx hypertrophic CM?

Answer 42

Echo: asymmetric wall thickness (esp. septal) ECG: LVH

Question 43

How do you treat hypertrophic CM?

Answer 43

BBs*, verapamil, ICD placement* Cautious use of digoxin, nitrates & diuretics Septal myomectomy, Etoh ablation Avoid dehydration & extreme exertion/exercise