Congenital Heart Disease

Question 1

What is the most common congential heart defect?

Answer 1

VSD

(also believed 1/2 of these repair themselves and never come to medical attention)

Question 2

What is an atrial septal defect?

Answer 2

persistent opening in the interatrial septum after birth that allows for direct communication between the l. and r. atria

Question 3

What is the prevalence of ASD?

Answer 3

1:1500 live births

10% of all congenital heart disease

Question 4

What is the consequence of an ASD?

Answer 4

blood ordinary shunted from left–>right

volume overload in right atrium

Question 5

What is Einsemenger Syndrome?

Answer 5

When a shunt that was formerly left-to-right becomes right-to-left

Question 6

What is the common presentation for ASD?

Answer 6

• May be asymptomatic
• DOE
• Fatigue
• Recurrent lower respiratory tract infections

Question 7

What are the exam/test findings with ASD?

Answer 7

RV heave along LSB

S2 wide, fixed splitting

murmur is mid-systolic left USB

cardiac cath - measures higher O2 in right atria

echo - shows shunt on doppler

Question 8

Rx for ASD?

Answer 8

Surgical-

• direct suture closure
• pericardial or synth patch
• percutaneous apporahc with septal occluder device

Question 9

How prevalent is VSD?

Answer 9

1.5-3.5:1000 live births

Question 10

How do you diagnose VSD?

Answer 10

echocardiography

Question 11

What are the symptoms and signs of VSD?

Answer 11

• depends upon the size of the defect ranges from no symp. to heart failure
• harsh, holosystolic murmur along LSB
• systolic thrill
• if reversed shunt, cyanosis and dyspnea

Question 12

What is the Rx for VSD?

Answer 12

• 1/2 close spontaneously by age 2
• closure indicated with s/sx of CHF or pulmonary vascular disease
• same as ASD methods for closure

Question 13

What is the tetraology of Fallot?

Answer 13

4 Defects:

1. VSD
2. pulmonic stenosis
3. overridng aorta (communicates with right ventricle through VSD)
4. RVH

Question 14

How common is tetralogy of Fallot?

Answer 14

Pretty rare: 5:10k live births

but most common cyanotic heart disease in childhood

Question 15

What are the s/sx of tetralogy of Fallot?

Answer 15

• “Tet” Spells - hyperventilation, cyanosis, syncope and squatting after exertion, feeding, crying
• dyspnea on exertion
• mild cyanosis and clubbing
• RV heave
• systolic ejection murmur at left USB

Question 16

What is the Rx for tetralogy of Fallot?

Answer 16

surgery-close VSD and increase pulmonary artery width

*if necessary, temporarily create connection between aorta and pulmonary artery to reduce hypertension

Question 17

What is transposition of the great vessels?

Answer 17

aorta arises from the RV

pulmonary artery arises from the LV

so, aorta pumps deoxygenated blood and the pulmonary artery carries oxygenated blood to the heart

Question 18

What is the prevelence of transposition of the great vessels?

Answer 18

40:100k births (7% congenital)

most common neonatal cyanosis

Question 19

What are the s/sx of transposition of the great vessels?

Answer 19

blue baby - extremely hypoxic and cyanotic

Question 20

How do you diagnose transposition of the great vessels?

Answer 20

echocardiogram

Question 21

What is the Rx for transposition of the great vessels?

Answer 21

arterial switch surgery is definitive

until that can occur, use prostaglandins to keep ductus arteriosis open (only way to get some oxygenated blood circulating)

Question 22

Who still gets pre-dental/surgical antibiotic prophylaxis with congenital heart disease?

Answer 22

unreparid cyanotic heart disease

post-repair for six months

post-repair with residual defects

Question 23

What is coarctation of the aorta?

Answer 23

narrowing of the aortic lumen

(can be postductal -98% or preductal - 2% for the DA)

Question 24

how common is coarctation of the aorta?

Answer 24

1:6k live births

Question 25

What are the s/sx of coarctation of the aorta?

Answer 25

* most asymptomatic, but severe will be evident in a newborn * preductal has cyanosis in LE * femoral pulses weak and delayed * midsystolic murmur * elevated UE BP

Question 26

How do you diagnose coarctation of the aorta?

Answer 26

* x-ray may show indented aorta and notches on inferior part of the ribs * echo confirms

Question 27

What is the treatment for coarctation?

Answer 27

severe - prostaglandin to keep DA open definitive - excision and reanastamosis (balloon +/- stenting)

Question 28

What is patent ductus arteriosus (PDA)

Answer 28

when embryological anastamosis between aorta and pulmonary artery fails to close after birth

Question 29

What is the prevalence of PDA?

Answer 29

1:2500-5000 live births

Question 30

What are the s/sx of PDA?

Answer 30

small are asymptomatic moderate - fatigue, dyspnea, palpitation large - left-to-right shunt HF Afib can develop if left atria dilates "machine" like murmur

Question 31

How do you diagnose PDA?

Answer 31

echo

Question 32

What's the treatment for PDA?

Answer 32

* prostaglandin synthesis inhibitor - indomethacin * surgical ligation or division * transcather coiling

Question 33

What is congenital aortic stenosis?

Answer 33

Abnormal development of the aortic valve with resulting obstruction of flow. Usually bicuspid leaflet or unicuspid (more severe)

Question 34

What are the s/sx of aortic stenosis?

Answer 34

most are symptomatic, but when symptoms occur, looks like AS in grown-ups.

Question 35

What is pulmonic stenosis and what is its prevalence?

Answer 35

obstruction across the pulmonic valve due to abnormal development of the valve, outflow tract, or artery. It's very rare

Question 36

What are the signs and symptoms of pulmonic stenosis?

Answer 36

usually asymp. when there are symptoms, DOE, exercise intolerance, right-sided HF * On exam: * prominent jugular venous a wave * RV heave and thrill * widely split S2 and soft P2 * loud, late-peaking, crescendo-decrescendo systolic murmur at upper LSB

Question 37

How do you dx and grade pulmonic stenosis?

Answer 37

echo to confirm and assess pressure gradient Mild \<50 mmHg Moderate 50-80 mmHg Severe \>80 mmHg

Question 38

What's the Rx for pulmonic stenosis?

Answer 38

moderate or severe cases treated - transcatheter balloon valvuloplasty RV hypertrophy usually regresses!