Cardio

Question 1

Most common cyanotic heart defect in kids

Answer 1

TOF

Question 2

TOF

Answer 2

Genetic and enviro factors
Assoc with 22q deletions
Cyanosis lips, extremities, HSM LLSB
Increased preload
Increased SVR
Boot shaped heart, increased pulm vasc markings

Tetralogy:
VSD
Pulm stenosis
RVH
overriding aorta

Question 3

Holosystolic murmurs

Answer 3

MR, TR, VSD

Question 4

TGA

Answer 4

No blood oxygenation without PDA, ASD, or VSD. Early and severe cyanosis. Single S2.
“Egg and string” CXR
Must have PDA.
NSAIDs contraindicated (cause PDA closure)
Requires two-step surgery, each sx carrying 50% mortality

Question 5

Pulsus alternans

Answer 5

LV systolic dysfunction

Question 6

Pulsus bigeminus

Answer 6

Sign HOCM

Question 7

Pulsus bisferiens

Answer 7

AR

Question 8

Pulsus parvus et tardus

Answer 8

AS

Question 9

Pulsus paradoxus

Answer 9

Tamponade and tension pneumo

Question 10

Hypoplastic Left Heart Syndrome

Answer 10

LV hypoplasia, mitral valve atresia, aortic valve lesions
Absent pulses with single S2, increased RV impulse
Gray (vs blue) cyanosis
CXR-globular heart with pulm edem. Echo most diagnostic
Tx: 3 separate sx or transplant. Each sx has high mortality

Question 11

Truncus arteriosus

Answer 11

Sxs within few days of life. Severe SOB, early and freq resp infxns.
CXR-cadiomegaly, increased pulm markings
Single S2 (only 1 semilunar valve) and SEM (valve leaflets usu abnormal in functionality)
Most serious sequelae is pulmonary HTN-develops in 4/12

Question 12

TAPVR

Answer 12

No venous return between pulm veins and LA-oxygenated blood to SVC. W or w/o obstruction of venous return

Question 13

VSD

Answer 13

Presents w SOB w/resp distress, high pitched HSM over LLSB, loud pulmonic S2.
Increased vascular markings
Small lesions usu close in first 1-2 years life
Larger or more symptomatic lesions req sx

Question 14

ASD

Answer 14

Twice as comm in women
Vast majority close spontaneously. Sx or transcath closure x’d for all symptomatic pts
Can have arrhythmia, poss paradoxical emboli

Question 15

ASD-primum defect associated with what?

Answer 15

Concomitant MV abnormalities

Question 16

ASD-secundum defect

Answer 16

Most common type ASD

Located in center of atrial septum

Question 17

ASD-sinus venosus defect

Answer 17

Least common type ASD

Question 18

PDA

Answer 18

Usu closes when PO2 >50mmHg
Normal finding in first 12 hours life; pathological after 24 hrs
Increased pressure, bounding pulses, high incidence of resp infxns
IE most comm complication later in life
ECG may show LVH due to high SVR
Indomethacin closes; prostaglandind keep open

Question 19

Pear-shaped heart on CXR

Answer 19

Pericardial effusion

Question 20

Boot shaped heart on CXR

Answer 20

TOF

Question 21

Jug handle appearance (heart) on CXR

Answer 21

Primary pulmonary artery hypertension

Question 22

3-like appearance of heart on CXR

Answer 22

coarc

Question 23

Coarc-presentation

Answer 23

Severe CHF, resp distress, differential pressures and pulses between upper and lower extremities

Question 24

Causes obstructed systemic circulation

Answer 24

Hypoplastic left heart
Critical aortic valve stenosis (congenital–bicuspid)
Severe coarc
Interruption of aortic arch

Question 25

Heart defects causing high pulmonary flow

Answer 25

I.e. Left to right shunts

VSD
ASD
Large PDA

Question 26

Eisenmenger syndrome

Answer 26

Originally left to right shunt….then right side hypertrophies and has more pressure than left side, so shunt reverses and becomes right-to-left.

Question 27

RFs congenital heart disease

Answer 27

Maternal drug use in prengnancy: lithium, booze
Maternal diabetes
Maternal PKU
Intrauterine infections, e.g. RUBELLA
Prematurity

In summary: it’s all mom’s fault.

Question 28

What do you want to ask about in hx to rule out/in congenital heart defects?

Answer 28

Cyanosis! Worse on feeding, crying?
Grey color changes
Sweating or resp distress on feeding or crying
Squatting when breathless (older children)
Collapse/syncope