Congenital heart disease

Question 1

What in infants shunts about 2/3 of blood from right atrium to the left atrium?

Answer 1

foramen ovale

Question 2

What in infants shunts blood from the pulmonary artery directly into systemic circulation?

Answer 2

ductus arteriosis

Question 3

Syndrome when left to right shunt becomes right to left

Answer 3

eisenmenger syndrome

Question 4

What defect could this be:
Asymptomatic and often resolves on own – larger defect causes symptoms of HF (respiratory distress, poor weight gain, fatigue) - 4-6 weeks

Answer 4

VSD

Question 5

VSD is acyanotic or cyanotic

Answer 5

acyanotic (MC)

Question 6

ASD is acyanotic or cyanotic

Answer 6

acyanotic

Question 7

PDA is acyanotic or cyanotic

Answer 7

acyanotic

Question 8

coarcation of the aorta is acyanotic or cyanotic

Answer 8

acyanotic

Question 9

teratology of fallot is acyanotic or cyanotic

Answer 9

cyanotic

Question 10

transposition of the great vessels is acyanotic or cyanotic

Answer 10

cyanotic

Question 11

What does this indicate:
High pitched harsh holosystolic murmur at lower left sternal border
Handgrip increases intensity

Answer 11

VSD

Question 12

How do you manage VSD?

Answer 12

⅓ will close spontaneously

Severe w/ HF signs: diuretics + digoxin

Surgery if no response to decongestion

Question 13

ASD is mostly

Answer 13

asymptomatic

Question 14

ASD is commonly associated with

Answer 14

anomalies of AV valves

Question 15

What does this indicate:
RV heave at lower left sternal border, systolic crescendo- decrescendo ejection murmur over pulmonic area, wide, fixed split S2

TTE is initial test w/ Doppler - RA and RV dilation

Answer 15

ASD

Question 16

How do you manage ASD?

Answer 16

Symptomatic - surgical or catheterization closure

Asymptomatic - elective closure at 3 years of age

Question 17

What does this indicate:
Widened pulse pressure – “bounding pulses”, cyanosis

Most asymptomatic

Answer 17

PDA

Question 18

What are these risk factors for:
Higher altitudes, females>males
Prematurity and maternal rubella infection

Answer 18

PDA

Question 19

What does this indicate:
Machine-like continuous murmur at pulmonic area, crescendo-decrescendo at 2nd IC space

Echo - increased LAE and LVE

Answer 19

PDA

Question 20

How do you manage PDA?

Answer 20

Asymptomatic = watchful waiting

Symptomatic = IV indomethacin – promoting closure

surgical closure

Question 21

What does this indicate:
Poor feeding, respiratory distress, shock

Older kids = asymptomatic but may have leg discomfort with exercise, nose bleeds, HTN (headache)

Cyanosis

Bilateral claudication

Answer 21

coarctation of the aorta

Question 22

What is coarctation of the aorta associated with?

Answer 22

turner syndrome and bicuspid aortic valve

Question 23

What does this indicate:
Absent or diminished femoral pulses

BP: LE < UE

Blowing, harsh systolic murmur in left interscapular area of back

CXR: marked cardiomegaly + pulmonary edema, rib notching “3 sign”
EKG: normal in infants, LVH in older children
Dx: ECHO to confirm, angiography is gold standard

Answer 23

coarcation of the aorta

Question 24

How do you manage coarctation of the aorta?

Answer 24

Prostaglandin E1 (alprostadil) to keep ductus arteriosus open in preoperatively

Neonates = anastomosis

Balloon angioplasty for older kids

Question 25

What does this indicate: Hypoxic “spells” during infancy - “TET SPELLS” - restlessness, agitation, crying spells, toddlers may squat - cyanosis by 4 months, easy fatigability and DOE Hyperpnea and increasing cyanosis

Answer 25

tetralogy of fallot

Question 26

What are the 4 findings of tetralogy of fallot?

Answer 26

Large VSD Pulmonary stenosis - murmur Overriding aorta RVH (heave)

Question 27

What does this indicate: Large VSD Pulmonary stenosis - murmur Overriding aorta RVH (heave) Loud single S2 Lab: hemoglobin, hematocrit, RBC elevated CXR: normal heart, RV hypertrophy (boot shape) EKG: RVH, RAD ECHO: anomaly

Answer 27

tetralogy of fallot

Question 28

How do you manage tetralogy of fallot?

Answer 28

Treat spells with oxygen + knee-chest position IV Morphine to relax pulmonary infundibulum + sedation Consider phenylephrine to increase vascular resistance Prostaglandin therapy to maintain ductal patency Beta blockers to decrease risk of Tet spells until surgery Surgical repair Birth - 2 years Close VSD + repair stenosis Bacterial endocarditis prophylaxis indicated until 6 months or until VSD repaired

Question 29

Tetralogy of fallot patients usually need later in life a

Answer 29

pulmonary valve replacement

Question 30

What does this indicate: Profoundly cyanotic w/o respiratory distress + significant murmur Infants with a large VSD = less cyanotic + more noticable murmur Severe cyanosis and tachypnea w/n 30 days of life, diaphoresis and poor feeding

Answer 30

transposition of the great vessels

Question 31

Transposition of the great vessels is most common

Answer 31

newborn period

Question 32

Patients with transposition of the great vessels also have

Answer 32

ASD, VSD, PDA

Question 33

What does this indicate: EKG: RAD + RVH CXR: increased pulm vascularity + “egg on a string” - cardiomegaly + narrowed mediastinum Get ECHO Loud and single S2

Answer 33

transposition of the great vessels

Question 34

How do you treat transposition of the great vessels?

Answer 34

Prostaglandin E1 (alprostadil) Balloon atrial septoplasty Arterial switch operation performed w/n 2 weeks of life