Adult congenital heart disease Flashcards
ASD
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
patent formane ovale
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
how do you diagnose patent foramen ovale?
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
ordinary echo?
transthoracic echo
special echo?
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
transient R>L shunting?
occurs during the onset of ventricular contraction, explaining neurologic events in non cyanotic patients.
right sided volume overload?
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
what re the 2 most common defects in adult heart?
myxomatous valve (Mitral valve prolapse)
bicuspid aortic valve
What are the complications of ASD?
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)
secundum ASD?
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
primum ASD
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
Sinus Venosus ASD?
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
Scimitar syndrome (triad?)
Triad:
- Partial anomalous venous return
- Hypoplasia of a lobe of the right lung
- Thoracic aorta>Pulmonary artery collaterals
all large shunts have some….
R>L shunting
what is the outcome of ASD?
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
what size lesion in ASD are symptomatic
> 8mm
what are the clinical manifestations of ASD?
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
what syndrome can conduct 1:1 - a-fib to V-fib to death!
Wolf parkinson white syndrome
Physical exam findings of ASD?
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
What is the most common congenital heart disease at birth?
VSD–> only accounts for 10% of adult congenital defects due to spontaneous closure
VSD murmur?
the larger the hole- the quieter the murmur
mostly heard as holo-cystolic, left sternal border (2nd or 3rd ICS) - very loud
what are the four types of VSD
- Infundibular VSD, below the aortic and pulmonic valves, leading to progressive aortic regurgitation, the hallmark.
- Membranous VSD, also called conoventricular, deficency of the membranous septum.
- Inlet Defect VSD, av canal, Down’s
- Muscular VSD, in the trabecular system, 5-20%
moderate sized defect ?
> 25% -<75%
no pulmonary HTN
mild-moderate volume overload of PA, LA, LV
large VSD?
> =75% of annulus
moderate to large L>R shunts with volume overload, if uncorrected leads to PHTN – > develops with pulmonary arterial obstructive disease
