Congenital Heart Defects Flashcards
1
Q
What heart defects are associated with…
Down’s
Turner’s
William’s
Noonan’s
A
- Down - 50% have cardiac defects; most common is AV septal defects and VS defects
- Turner’s - often L sided obstruction (coarctation of aorta or bicuspid aortic valve)
- William’s - defect in elastin gene so arterial abnormalities; supravalve aortic stenosis (narrow above valve — ascending aorta), renal artery stenosis, pulmonary artery stenosis
- Noonan’s- wide-spaced nipples; pulmonary valve stenosis and hypertrophic cardiomyopathy
2
Q
Acyanotic Septal Defects in General
A
All involve L—> R shunt which can lead to CHF and pulmonary HTN
+ vol overload –> RAD and RVH on Xray and EKG
Atrial septal, ventricular septal and AV septal defects, patent ductus arteriosus
3
Q
Atrial Septal Defect
A
- L —> R atria shunt so vol overload in RA and RV
- Parasternal heave
- R side hypertrophy & inc pulmonary vasculature (X-ray)
- Fixed split S2 b/c more pulmonary flow so P2 always later
- Ejection murmur across pulmonary valve
- Mid-diastolic murmur across tricuspid valve
- EKG - RAD and R vent hypertrophy (high R waves)
- Diff locations of defect
- Secundum - fossa ovalis in center of atrium
- Sinus Venosus - higher near superior vena cava
- Ostium Primum
- Presentation - Usually asymptomatic in childhood; complications in adulthood due to chronic high pulmonary flow (pulmonary HTN, paradoxical emboli, R heart fail, atrial arrhythmias)
- Management - if defect but no volume overload just leave it; but if volume overload - surgical closure or transcatheter closure if secundum
4
Q
Ventricular Septal Defect
A
- L—>R ventricle shunt so vol overload of L atrium and L vent, L atria and maybe R vent
- Physiology depends on size of defect and pulmonary resistance
* Larger VSD = more shunting (Pulmonary HTN, CHF, ejection murmur and loud single S2)
* Smaller VSD= less shunting (Normal S2 and no symptoms but pan-systolic murmur at lower sternal border) - Larger pulmonary resistance = less shunting
* When infant is first born they have high pulmonary resistance (less shunting) then by 4 wks pulmonary resistance is way lower than systemic resistance —> more shunting and marked volume overload/pulmonary HTN - Location determines how likely it is to close on own
- Perimembranous (most common)
- Muscular - small; high likelihood of closing
- Supracristal - high; above crust terminus; high incidence of mitral valve prolapse
- Presentation
- Often spontaneously close or decrease in size
- Complications include CHF, pulmonary HTN, bacterial endocarditis
- Management - surgical repair and treat CHF/pulmonary vascular disease
5
Q
AV Septal Defect
A
- Defect in center of heart w/ both atrial and ventricular components; L—>R shunt in all chambers; volume overload in all chambers
- Can be partial (low atrial defect and cleft mitral valve) or complete (atrial and vent defect)
- A/V valves also involved - do not form properly
- Presentation
- Most common w/ Down Syndrome
- If partial - get mitral regurg over cleft mitral; behaves like atrial septal defect
- If complete - behaves like vent septal defect (pulmonary HTN, CHF)
- Usually dx by echo
- Management - surgical repair (no cath option)
6
Q
Patent Ductus Arteriosus
A
- Tube b/n pulmonary artery and aorta stays open after birth
- Aorta —> pulmonary artery (b/c less resistance than systemic) —> LA —> LV
- So only LA and LV overload
- Presentation
- CONTINUOUS murmur at L sternal border and bounding pulses
- Can lead to pulm HTN and CHF if ductus is large
- Complications - CHF, pulm HTN, endocarditis
- Management - surgical ligation, transcatheter once 1 yr+
7
Q
Acyanotic Obstructions in General
A
Obstruction –> Pressure overload –> CHF and vent hypertrophy
Pulmonic stenosis, aortic stenosis, coarctation of aorta
8
Q
Pulmonic Stenosis
A
- Thick pulmonary valve or leaflets fuse so R vent pressure overload/hypertrophy
- Presentation
- Systolic ejection murmur at L sternal border w/ click (mobile valve as child)
- Dilated pulmonary artery on X ray
- Complications - right heart fail; if foramen ovale still open then R —> L atrial shunting because less resistance than outflow to pulmonary artery (causes cyanosis)
- Management - trans-catheter balloon valvuloplasty
9
Q
Aortic Stenosis
A
- Thick leaflets or fused leaflets (usually bicuspid) —> block LV outflow/ LV hypertrophy
- Presentation
- Systolic ejection murmur at R sternal border w/ click transmitted to apex
- Dec pulse amp if severe
- LVH on EKG
- Complications - more severe b/c dec CO (exertional syncope or sudden death), CHF, endocarditis
- Management - balloon valvuloplasty but usually need surgery later (replace valve)
10
Q
Coarctation of Aorta
A
- Obstruction of aortic arch (near where ductus arteriosus was) —> P inc in ascending aorta —> inc P in L vent
- Presentation
- HTN in arms
- Normal pulses in arms but weak in legs (because pulse pressure becomes blunted at blockage)
- Any discrepancy in BP b/n upper and lower body
- LVH on EKG
- If do not repair for 5 yrs - may see rib notching due to collateral flow through intercostal arteries
- May see “figure of 3” shape in descending aorta on X ray (see shadow of aorta pinching in)
11
Q
How to tell if cyanosis is due to cardiac abnormality
A
**Normal sat is 95% do not see cyanosis clinically until 85-90%
**Hyperoxia test - give them 100% oxygen and if sat still does not improve than likely cardiac problem
12
Q
3 Types of Cyanotic Defects (+ examples of each)
A
1- abnormal connections (transposition of great arteries or totally anomalous pulmonary venous return)
2- septal defect w/ obstruction to pulmonary flow (tricuspid atresia, pulmonic atresia, tetralogy of Fallot)
**May be ductal-dependent; use prostaglandins (keep ductus open) if not then may need aorta–>pulm shunt
3- complete mixing (aortic atresia, single ventricle, truncus arteriosus)
13
Q
Transposition of Great Arteries
A
- Pulmonary artery and aorta connected to wrong chamber
- In utero survive b/c atrial communications —> mixing; so 1st wk after birth Rashkin procedure - enlarge atrial communication (foramen ovale) w/ balloon
- Presentation -
- Cyanotic
- Normal sound, EKG an Xray
- Dx via echo
- Management - prostaglandins, balloon septostomy (Rashkin), arterial switch procedure
14
Q
Total Anomalous Pulmonary Venous Return
A
- Pulmonary veins empty back into R atrium
- Problem in embryology - pulmonary veins go back to confluence behind L atrium but do not enter L atrium; alternative veins will form to connect confluence to systemic circulation so pulmonary flow goes to R atrium NOT L atrium
- Alt vein may go superior vena cava, down to portal circulation OR to coronary sinus
- Problem in embryology - pulmonary veins go back to confluence behind L atrium but do not enter L atrium; alternative veins will form to connect confluence to systemic circulation so pulmonary flow goes to R atrium NOT L atrium
- Compensate w/ foramen ovale —> L ventricle –> mixing
- Complications - inc pulmonary blood flow, if obstruction to pulmonary blood flow thru new veins then get pulmonary edema
- Management - surgical repair; connect confluence to L atrium and ligate alternative veins
15
Q
Tricuspid Atresia
A
- Do not get flow into R ventricle; so blood goes from R atria —> L atria instead
- Leads to R vent hypotrophy b/c does not get flow in development
- Also have VSD - L ventricle —> small RV —> pulmonic artery (so L vent is source of pulmonary flow)
- Presentation
- More pulmonary blood flow you can achieve = least cyanosis
- Systolic murmur across pulmonic valve or across VSD
- Normal heart size but dec pulmonary vascularization
- LAD and LVH on EKG
- Management - prostaglandins if trying to keep ductus open OR aorta —> pulmonary artery shunt if severe cyanosis
