Adult congenital heart disease

Question 1

ASD

Answer 1

open communication b/w the atria via a defect in the intra-atrial septum

second most common adult congenital abnormality after bicuspid AV

usually asymptomatic until adulthood

Question 2

patent formane ovale

Answer 2

is a foramen covered by the septum primum but is not sealed shut in 20% of normal subjects

this is different than an atrial septal defect

as long as the shunting is left to right–> this is tolerable, but if it switches, then cyanosis occurs

Question 3

how do you diagnose patent foramen ovale?

Answer 3

bubble studies

do an ordinary echo and inject bubbles into the IV –> can see them going across atrial septum !

blood without bubbles going across atrial septum and clearing the bubbles? left to right shunt

Question 4

ordinary echo?

Answer 4

transthoracic echo

Question 5

special echo?

Answer 5

transesophageal echo

swallow material, and ends up in esphagus and the esophagus butts up against the left atrium and you don’t have to go through the lung and other tissue in the front

real atrial septal defects are diagnosed this way

this test will also tell you if the defect will require surgery and if it can be closed percutaneously through the femoral vein

Question 6

transient R>L shunting?

Answer 6

occurs during the onset of ventricular contraction, explaining neurologic events in non cyanotic patients.

Question 7

right sided volume overload?

Answer 7

well tolerated by the heart for many years

mild to moderate amounts of regurg rarely lead to problems

EXCEPT if the shunt is large- such as doubling the volume overload–> patient will develop right sided heart failure

Question 8

what re the 2 most common defects in adult heart?

Answer 8

myxomatous valve (Mitral valve prolapse)

bicuspid aortic valve

Question 9

What are the complications of ASD?

Answer 9

Atrial Arrhythmias
Paradoxical Embolus
Cerebral abscess
Right Heart Failure

Pulmonary Hypertension>Eisenmenger Syndrome (as pulmonary vascular stiffens you get this shunt reversal and it’s not reversible)

Question 10

secundum ASD?

Answer 10

most common type of ASD (70%)

located in the middle where the foramen ovale is

more common in females

due to defects in the foramen ovalis

usually closed percutaneously

usually NOT associated with other cardiac defects

Question 11

primum ASD

Answer 11

second most common type of ASD (15-20%)

poor prognosis

large defect - and NEED to be closed

almost always associated with defects in the AV valves or ventricular septum

AV canal or endocardial cushion defect is the complete form

Question 12

Sinus Venosus ASD?

Answer 12

3rd most common type of ASD

5-10% of ASD’s

Often associated (>90%) with anomalous pulmonary vein insertion- where one or more of the pulmonary veins, instead of emptying into the left atrium empty into the right atrium (now have oxygenated blood emptying into de-oxygenated chamber)

Two types:
Superior Sinus Venosus-SVC Defect
Inferior Sinus Venosus Defect-IVC Defect

CANNOT be closed percutaneously

Question 13

Scimitar syndrome (triad?)

Answer 13

Triad:

1. Partial anomalous venous return
2. Hypoplasia of a lobe of the right lung
3. Thoracic aorta>Pulmonary artery collaterals

Question 14

all large shunts have some….

Answer 14

R>L shunting

Question 15

what is the outcome of ASD?

Answer 15

shunt flow leads to a “useless circuit” of blood through the defect

This leads to Right heart volume overload, well tolerated for years, but can cause Pulmonary Hypertension and Eisenmengers (shunt reversal)

Vessels that are young are very compliant- dilates as it accepts volume and this keeps the pressure down
but after years of pulmonary flow being high, pulmonary arteries get stiff and aorta gets stiff–> this is why you see systolic pressure rise in the elderly

Question 16

what size lesion in ASD are symptomatic

Answer 16

> 8mm

Question 17

what are the clinical manifestations of ASD?

Answer 17

Atrial Arrhythmias
-HR Increases in A-fib b/c random portions of heart is firing and SA node is not in charge. AV node saves the heart from V-fib. A-fib is electrical and mechanical.

20% atrial fibrillation or flutter, increases with advancing age

At risk for embolic events, including stroke, including paradoxical stroke, systemic emboli

Migraine cephalgia may be associated

Pulmonary Hypertension and Eisenmenger syndrome, requires >2.5:1 shunt

cyanosis- concomitant Pulm valve stenosis or Eisenmengers

Question 18

what syndrome can conduct 1:1 - a-fib to V-fib to death!

Answer 18

Wolf parkinson white syndrome

Question 19

Physical exam findings of ASD?

Answer 19

Precordium:
RV heave - left sternal border or retrosternal - feel the sternum lift
Palpable PA at upper LSB

Heart sounds:
Very loud S2 at the left sternal border (palpable heart sound = shock)
Wide fixed split S2
S1 split with increase in tricuspid component

fixed split- right and left equal, shunting is equal
split on exhalation but not inhalation- paradoxical split caused by severe aortic stenosis or LBBB

Heart murmurs:
SEM upper LSB from increased flow
Early DM, upper LSB from PI secondary to pulmonary HTN

Question 20

What is the most common congenital heart disease at birth?

Answer 20

VSD–> only accounts for 10% of adult congenital defects due to spontaneous closure

Question 21

VSD murmur?

Answer 21

the larger the hole- the quieter the murmur

mostly heard as holo-cystolic, left sternal border (2nd or 3rd ICS) - very loud

Question 22

what are the four types of VSD

Answer 22

1. Infundibular VSD, below the aortic and pulmonic valves, leading to progressive aortic regurgitation, the hallmark.
2. Membranous VSD, also called conoventricular, deficency of the membranous septum.
3. Inlet Defect VSD, av canal, Down’s
4. Muscular VSD, in the trabecular system, 5-20%

Question 23

moderate sized defect ?

Answer 23

> 25% -<75%

no pulmonary HTN

mild-moderate volume overload of PA, LA, LV

Question 24

large VSD?

Answer 24

> =75% of annulus

moderate to large L>R shunts with volume overload, if uncorrected leads to PHTN – > develops with pulmonary arterial obstructive disease

Question 25

what does VSD lead to

Answer 25

Progressive pulmonary hypertension leads to RV
pressures approaching systemic

Leads to reversal of shunt with R>L flow

Hypoxemia and cyanosis develop

This is Eisenmenger syndrome, and when coupled with VSD is called Eisenmenger Complex

Question 26

Physical exam findings on VSD?

Answer 26

large holosystolic, LSB, 2nd or 3rd ICS, thrill

EKG-66% of small are normal.

Echo-test of choice

CT/MRI-excellent for complex lesions

Cath- LESS important now with advanced Echo and MR

Question 27

why do people with VSD have aortic regurg?

Answer 27

because the membranous defects are right below the aortic valve and the leaflets prolapse into the defect

Question 28

small, moderate and large VSD clinical presentations>

Answer 28

Small defect-small L>R shunt, asymptomatic adults

Moderate sized defect- either asymptomatic or mild CHF in children, usually gets smaller with growth and may have AR

Large VSD- Usually early presentation with CHF in infancy or Eisenmenger’s in late childhood, early adulthood.

Question 29

4 features of tetralogy of fallot ‘

why does this happen at the same time?

Answer 29

1. RVOT (outflow tract) obstruction
2. VSD
3. Aorta overrides IVS (aorta overrides inter-ventricular septum)
4. Concentric RVH

***one of the most cyanotic congenital heart disease

happens at the same time b/c it happens during embryologic development. all these parts are being formed around the same time.

Question 30

the need for medical intervention for tetralogy of fallot depends on what?

Answer 30

depends on the degree of RVOT obstruction