Congenital Heart Disease Flashcards
Atrial Septal Defects
Ostium Secundum
Ostium Primum
Sinus Venosus
Coronary Sinus
Ostium Secundum ASD
- most common
- located in center of fossa ovalis
- defect in embryologic septum primum
- associated with MVP
- amenable to closure in cath lab
- crista terminalis is superior/cephalad to the secundum ASD
Ostium Primum ASD
•second most common
•located near atrioventricular valves and associated with cleft septal TV and anterior MV leaflets
•represent an endocardial cushion defect
•makes up part of an AV canal
Primum —> partial [MV + TV in same plane but separate rings]
Primum + restrictive inlet VSD —> transitional [MV + TV share a ring]
Primum + non restrictive inlet VSD —> complete [MV + TV share a ring] (won’t see any anterior structures — AV or LVOT)
•found in trisomy 21
•associated with conduction abnormalities - can develop complete heart block after repair
•visualize in low ME4C view
•major problem is RV volume overload due to L->R shunting in diastole
Sinus Venosus ASD
•located near SVC (most common) or IVC
•very cephalad defect so have to pull probe back from ME4C view to visualize
•associated with anomalous pulmonary venous return
•defect in common wall that separates the SVC from the RUPV
Superior sinus venosus ASD —> RUPV
Inferior sinus venosus ASD —> scimitar syndrome [hypoplastic RLL of lung, aorto-pulmonary collateral blood flow to RLL w/ anomalous pulmonary venous drainage from RLL to a scimitar vein draining to IVC]
•Warden procedure: ligate SVC and reattach to RAA, create baffle between LA/RA, now blood goes from RUPV —> LA
Coronary Sinus ASD
•least common
•represents a hole or unroofing of the CS
•higher pressured LA blood drains into lower pressure RA through the CS (L -> R shunt)
•associated with PLSVC draining into CS
Very dilated CS
Injection of contrast in left arm will light up CS before the RA
Ventricular Septal Defects — Anatomical locations + Numerical
Relative to crista supraventricularis — infracristal vs supracristal
•the ONLY supracristal defect is the subpulmonic VSD
•all other defects are infracristal
Relative to RV inflow + outflow
•inlet vs outlet
Relationship to semilunar valves
•subaortic vs subpulmonic
Relationship to septum
•malalignment
•membranous vs muscular
Numerical ( 1 - 4 ) Type 1 — outlet / subpulmonic •only supracristal defect Type 2 — membranous / subaortic •most common type ( > 70% ) Type 3 — inlet / AV canal type Type 4 — muscular / trabecular
Membranous VSD
(Para, peri, infracristal, subaortic)
Type 2
•most common type
•located near TV just below the AV (anterior)
Associated with:
•LV septal aneurysms where subvalvular apparatus of TV plugs defect
•prolapse of RCC and AI
•TOF
Best view —> ME RV inflow-outflow
Gradient highest from LV->RV during systole
Outlet VSD
(Subpulmonic, supracristal, conal, intraconal, subarterial, doubly committed)
Type 1
- Occurs just below the PV and is supracristal (anterior)
- most likely associated with prolapse of RCC and AI
- rare defect, least common — unlikely to see on exam
Inlet VSD
(AV canal type, endocardial cushion defect)
Type 3
•atrioventricular leaflets are in the same plane (TV and MV in same plane next to each other)
•more posterior —> don’t see anterior structures (LVOT or AV)
•defect in endocardial cushions
Associated with:
•primum ASD —> AV canal
•trisomy 21
•cleft atrioventricular valve leaflets (septal TV and anterior MV)
Muscular VSD
(Trabecular)
Type 4
- located more inferiorly and posteriorly in the muscular part of the septum
- this area is highly trabecular therefore referred to as ‘trabecular defects’
Anterior or posterior malalignment VSDs
- Shifting of the septum during embryologic development
- anterior / posterior and right / left
- TOF — all pathology explained by septum being shifted anterior and rightward causing RVOTO [VSD, overriding aorta, pulmonary obstruction, RVH]
- opposite occurs when septum shifted posterior and leftward — posterior malalignment VSD [bicuspid AV, interrupted aortic arch, coarctation, hypoplastic left heart, MV stenosis]
- considered membranous or conoventricular VSD
Outlet VSD vs Perimembranous VSD
Best view is ME RV inflow-outflow
Membranous —> just below AV next to TV
Outlet —> just below PV next to AV
Both associated with prolapse of RCC and AI
Hypoplastic left heart syndrome
•atrial septectomy created for LA->RA shunt so the RV can act as systemic ventricle
•AI would be a problem due to decreased forward LV stroke volume
•maintain low mean airway pressures
•obstruction at interatrial level problematic because RV needs to maintain adequate preload for systemic perfusion
•pre-repair — PDA dependent systemic blood flow
•repair :
1. Ascending aorta, AV, and main PA —> neo-aorta connected to RV ; atrial septectomy to allow pulmonary venous blood to preload for systemic RV
2. BT shunt (lower EDP from diastolic aorta runoff predisposes to coronary ischemia) OR Sano shunt (requires right ventriculotomy)
3. Bidirectional Glen from SVC —> Right PA
4. Fontan with IVC —> Right PA
Ebstein’s Anomaly
- sail like anterior TV leaflet
- apical displacement of septal TV leaflet
- atrialization part of RV
- WPW / VT
- secundum ASD
- dilated sick RV with severe volume overload due to TR
Williams Syndrome
- defect in chromosome 7 — elastin gene (arteropathy)
- elephant facies, short stature, friendly, musically inclined, developmental delay
- supravalvular AS with hour glass STJ
- physiology similar to AS with similar hemodynamic goals: SVR - high, HR - low/normal and sinus, Preload - full, Contractility - maintain
- wide pulse pressure due to wide, stiff, thick, non compliant arterial system
- obstruction can occur at level of ostium of the coronary artery and flow turbulence
- low aortic DBP and elevated LVDP therefore low coronary perfusion pressure
- at risk for sudden cardiac death with sedation
