Adult Congenital Heart Diseases Flashcards
ASD
- open communication b/w atria via a defect in the intra-atrial septum
- second most common adult congenital disease after bicuspid AV, usually asymp. until adulthood
- left to right shunt (originally)
- complications: atrial arrhythmias, paradoxical embolus, cerebral abcess, right heart failure, pulmonary HTN –> Eisenmenger syndrome
patent foramen ovale
often of little significance
= foramen covered by septum primum but is not sealed shut in 20% of normal subjects
which echo is needed for operative closure and ddx of ASD?
trans-esophageal echo
two most common in adult cardiology?
- Bicuspid aortic valve
- myxomatous mitral valve (MVP)
Eisenmenger sydrome
- irreversible pulmonary HTN
- results in reversal from left to right, to right to left shunting due to noncompliance of the lungs
- this can happen with any kind of shunting, i.e. ASD, VSD, etc.
most common type of ASD?
- secundum ASD, occurs in the middle of atria
- 70% of ASD’s
- due to defects in foramen ovale, not usually assoc. w/ other cardiac defects
- usually closed with catheterization and plug
second most common ASD?
= primum defect
- at bottom of atrial septa
- 15-20% of patients
- much more serious
- sometimes involves portions of top of ventricular septum and mitral/tricuspid valve= endocardial cushion defect (or AV canal defect- could be in any chamber through putting probe in)
- Almost always associated with defects in the AV valves or Ventricular septum (almost always assoc. w/ other defects)
Sinus Venosus ASD
third most common, about 5% of ASD’s
- located high in the right atrium
- 90% are assoc. w/ anomalous pulmonary vein insertion (one or more of four pulmonary veins enters into right atria rather than left)
Two types of sinus venosus ASDs?
Superior Sinus Venosus - SVC defect
Inferior Sinus Venosus- IVC defect
Scimitar Syndrome
Triad:
- Partial anomalous venous return
- Hypoplasia of a lobe of the right lung
- Thoracic aorta>Pulmonary artery collaterals
Pathophys of shunting in ASD?
- shunt depends on the size of the defect, the compliance of the right and left ventricles, and the phases of contraction (systole/diastole, atrial ventricular, early or late in phase)
- Most shunts start L>R, but all large shunts have some R>L *(even small shunts will have a small amount of R>L)
- Shunt flow leads to a “useless circuit” of blood through the defect
- This leads to right heart volume overload, well tolerated for many years, but can cause pulmonary HTN
sizes of ASDs?
lesions 2:1 will become symptomatic and req. surgical repair by age 40
clinical manifestations of ASD?
- atrial arrhythmia: see increased HR, due to scarred/irritated SA node
- see 20% due to atrial fibrillation/flutter that increases with advancing age, A fib in older pt. is more imp.
- see risk of embolic events, including stroke. paradoxical stroke, systemic emboli
- migraine cephalgia: might be assoc. though no evidence to back this up
- pulmonary HTN and Eisenmenger syndrome can result, which requires >2.5:1 shunt
cyanosis?
know that shut is occuring in right to left - usually have Eisenmenger sydnrome or pulmonary valve stenosis
PE of ASD?
- Physical findings are related to three things:
- Size and location of defect
- Size of the shunt
- Pulmonary Artery Pressure (resistance)
** feel heaves at left sternal border, or retrosternal heaves (over the pulmonic valve) **
Shock = when you can feel a sound
Heart sounds: hear wide fixed split S2 (ASD makes splitting stay equal due to shunting), increased P2 with pulmonary HTN-
- usually heart S1 split w/ increase in tricuspid component
heart murmurs hear w/ ASD?
Heart Murmurs:
- SEM Upper LSB from increased flow
- Early DM, upper LSB from PI secondary to pulmonary hypertension
Heart sound heard with ASD?
Heart sounds: hear wide fixed split S2 (ASD makes splitting stay equal due to shunting), increased P2 with pulmonary HTN-
- usually heart S1 split w/ increase in tricuspid component
where do you feel heaves of ASD?
left sternal border, or retrosternal heaves (over the pulmonic valve) ***
VSD
- Most common congenital heart disease at birth, only accounts for 10% of adult congenital due to spontaneous closure
small VSD
Small or Restrictive VSD have orifice diameter
R shunt with no LV volume overload
No pulmonary Hypertension
moderate sized VSD?
Moderate sized defect, >25%-<75%
Mild-moderate volume overload of PA, LA,LV
No pulmonary hypertension
large VSD?
Large VSD, >=75% of annulus
Moderate to large L>R shunts with LV volume overload, if uncorrected leads to PHTN, develops with pulmonary arterial obstructive disease
VSD path?
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
VSD clinical?
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 Aortic regurgitation (b/c membranous defects are directly below aortic valve, and can cause the prolapse of the aortic valves)
Large VSD = Usually early presentation with CHF in infancy or Eisenmenger’s in late childhood, early adulthood.
PE of VSD?
- hear long, loud holosystolic murmur at LSB, 2nd or 3rd ICS (usually w/ associated thrill)
- 2/3 of EKG will be normal
- Echo will show it directly
- MRI/CT is excellent tool for looking at this
- Cardiac Catheterization is less important for ddx now
- defects are not usually closed percutaneously, must go in and close with surgery
4 features of Tetralogy of Fallot
- RVOT (right ventricular outflow) obstruction
- VSD
- Aorta overrides IVS (aorta serves right and left ventricle)
- Concentric RVH
–> results in cyanosis
- 10% of CHD, one of most common cyanotic congenital heart disease
- this defect affects many things that are happening at the same time during embryonal development
- The need for medical intervention is dependent on the degree of RVOT obstruction
