Congenital Heart Disease

Question 1

Most common congenital heart lesion in adults

Answer 1

atrial septal defect

Question 2

shunt in atrial septal defect

Answer 2

left atrium to right atrium shunt

Question 3

types of atrial septal defect

Answer 3

sinus venosus, ostium secundum, and ostium primum

Question 4

what additional problems are caused by sinus venosus ASD besides the hole between the atria?

Answer 4

R-sided pulmonary veins drain back to the R atrium instead of the left atrium- so all the oxygenated blood coming to RA instead

Question 5

Patient presents with pulmonary HTN, R ventricular failure, atrial arrhythmias. Echo shows dilated right atrium and ventricle. Increased RV impulse along LSB. You hear BOTH systolic (along LSB) and mid-diastolic ejection murmur (along apex). You also hear splitting of S2 (pulmonary valve closing a little later due to increased volume). Diagnosis?

Answer 5

Atrial septal defect

Question 6

Problem with coronary sinus ASD

Answer 6

drains into LA instead of RA

Question 7

What is patent foramen ovale associated with?

Answer 7

Cryptogenic stoke and migraine headaches

Question 8

Function of foramen ovale

Answer 8

Hole between R and L atria that shunts blood from RA to LA to bypass lungs

Question 9

shunting of blood in PFO

Answer 9

Right to left atrium (opposite of other atrial septal defects)

Question 10

Why is PFO associated with strokes?

Answer 10

Because blood bypasses filtering system of lungs from RA directly into LA. Clots can get into LA from RA and into brain causing stroke

Question 11

What would echocardiography show in ASD?

Answer 11

Dilated RV, septal defect, and saline injection can be administered

Question 12

Surgical and percutaneous treatment options in ASD

Answer 12

Surgical- direct suture repair or patch repair. Percutaneous- catheter-implanted occlusion device- passage of catheter across atrial septal defect through femoral vein, deployed like umbrella

Question 13

In which location does catheter treatment work best in ASD?

Answer 13

Centrally located secundum defects

Question 14

Examples of percutaneous treatments for ASD

Answer 14

Catheter-implanted occlusion device, gore-helix, and amplatzer

Question 15

shunting in VSD

Answer 15

left to right (pressure higher in left)

Question 16

Patient presents with RV dilation and hypertrophy, LV hypertrophy, and pulmonary HTN. Displaced PMI to left. Harsh, holosystolic murmur, aortic regurgitation. Chest X ray also shows cardiac enlargement. Diagnosis?

Answer 16

VSD

Question 17

atrioventricular defect aka

Answer 17

endocardial cusion defect

Question 18

shunting of blood in endocardial cushion defect

Answer 18

shunting can occur either way

Question 19

Atrioventricular defect associated with

Answer 19

Downs syndrome

Question 20

What cardiac events is downs syndrome associated with?

Answer 20

Most common- atrioventricular septal defect. others include ASD, VSD, tetrology of fallot, hypoplastic left heart syndrome, and isolated elevated pulmonary artery pressure

Question 21

ductus arteriosus function

Answer 21

in fetus, blood from pulmonary artery shunts to aorta to bypass lungs

Question 22

patent ductus arteriosus shunting

Answer 22

shunting occurs in opposite direction of fetus- oxgenated blood goes from aorta to pulmonary artery (goes back to lungs- not as much blood getting perfused to body tissues)

Question 23

Neonate presents with shortness of breath, pulmonary hypertension, volume overloaded LV. CONTINUOUS murmur (machinery-like), and bounding pulses. Diagnosis and tx?

Answer 23

Patent ductus arteriosus. Tx- catheter-based closure or surgical closure

Question 24

Most congenital defects produce left to right shunting due to increased pressure on left side. What congenital defects cause right to left shunting?

Answer 24

Patent foramen ovale (ASD), and Eisenmenger syndrome, tetrology of fallot

Question 25

"Blue baby" in NICU. Blueing all over body, tachypnea, grunting, flaring, weak peripheral pulses. ECG shows RAD. Large R's in V1 and V2, and large S minimal R in V5 and V6 suggesting RVH. Normal S1, but only single S2 (do not hear closing of aortic valve). Murmum in diastole heard due to tricuspid regurgitation. Diagnosis?

Answer 25

Hypoplastic left ventricular syndrome

Question 26

Describe shunting and defects in hypoplastic left ventricle syndrome

Answer 26

Atrial septal defect with left to right flow, patent ductus arteriosus with right to left shunt in systole and diastolic flow reversal

Question 27

What shunts/defects left open to help in HLHS?

Answer 27

keep ductus arteriosus open and patent foramen ovale (so blood can flow from right to left- too much on right)

Question 28

What is the main problem with transposition of the great vessels?

Answer 28

The RV cannot keep up with pumping through aorta to entire body

Question 29

2 types of trasnposition of the great vessels

Answer 29

dextro and levo

Question 30

dextro vs. levo

Answer 30

dextro- atrio-ventricular concodonance and ventriculo-arterial discordance. levo- atrio-ventricular discordonance and ventriculo-arterial discordance

Question 31

repair for d-TGA

Answer 31

atrial inversion, ventricular inversion, or arterial switch

Question 32

Which TGA is cyanotic and is a parallel circle in which there is no connection between the R and L sides of the heart?

Answer 32

dextro- TGA

Question 33

What is coarctation of the aorta most often associated with?

Answer 33

bicuspid aortic valve (stenosis), also HTN in younger kids

Question 34

Patient presents with diminished lower limb pulses. But radial, brachial, carotid pulses are normal. patient also has systolic murmur due to bicuspid aortic stenosis. What do you suspect?

Answer 34

coarctation of the aorta

Question 35

Which type of repair is preferred in patients with dextro-transposition of the great vessels who have severe pulmonic stenosis?

Answer 35

Ventricular inversion

Question 36

Child appears especially blue around nail beds and lips during episodes of crying and feeding. During auscultation, you only hear single S2. Hear systolic thrill at LSB, systolic ejection murmur. You notice dretarded growth an delayed development. Xray shows "boot-shaped heart." ECG shows right atrial enlargement, RV hypertrophy, ventricular septal defect, overriding aorta, Right axis deviation, and RV conduction abnormlality. Diagnosis?

Answer 36

Tetrology of fallot

Question 37

Classic "boot shaped heart" associated with...

Answer 37

Tetrology of fallot

Question 38

What is the most common type of abnormal aortic valve?

Answer 38

Bicuspid aortic valve

Question 39

Average age that aortic valve stenosis calcification of valves occurs/

Answer 39

elderly- 45 to 65 years old

Question 40

4 abnormalities associated with tetrology of fallot

Answer 40

VSD, displacement of aorta over VSD, pulmonary artery narrowing, and RVH

Question 41

Shunting of blood in tetrology of fallot

Answer 41

RV to LV

Question 42

Shunting of blood in "pink" tetrology of fallot

Answer 42

LV to RV

Question 43

What is Ebstein's anomaly associated with

Answer 43

Septal defect of tricuspid valve is displaced into RV, so tricuspid regurgitation may occur, and also many patients also have coexistent ASD, Wolff-parkinson white syndrome

Question 44

What kind of shunt would cause more symptoms of cyanosis?

Answer 44

Right to left because mixing of deoxy with oxy blood going to body. Left to right shunt causes less blood to get to body, but it is still oxygenated blood

Question 45

What electrical abnormalities are associated with ebstein's anomaly?

Answer 45

WPW syndrome, Right atrial enlargement, and right ventricular bundle branch block

Question 46

Therapy for ebstein's anomaly

Answer 46

Tricuspid valve replacement and ASD closure

Question 47

Patient presents with cyanosis. PE shows wide splitting of S1 and S2, and also S3 and S4. Hear holosystolic murmur. Large v wave in neck veins, pulsatile liver, palpitations, peripheral edema. ECG shows Right atrial enlargement and WPW syndrome. Echo shows atrial septal defect. Diagnosis?

Answer 47

ebstein's anomaly associated with ASD and TR

Question 48

Cardio defects in marfan's syndrom

Answer 48

mitral valve prolapse and aortic aneurysm that may dissect (dilation of aorta due to weaknening of blood vessel walls)