Congenital Heart Disease Flashcards

1
Q

Atrial Septal Defects

A

Ostium Secundum

Ostium Primum

Sinus Venosus

Coronary Sinus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Ostium Secundum ASD

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Ostium Primum ASD

A

•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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Sinus Venosus ASD

A

•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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Coronary Sinus ASD

A

•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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Ventricular Septal Defects — Anatomical locations + Numerical

A

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Membranous VSD
(Para, peri, infracristal, subaortic)
Type 2

A

•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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Outlet VSD
(Subpulmonic, supracristal, conal, intraconal, subarterial, doubly committed)
Type 1

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Inlet VSD
(AV canal type, endocardial cushion defect)
Type 3

A

•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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Muscular VSD
(Trabecular)
Type 4

A
  • located more inferiorly and posteriorly in the muscular part of the septum
  • this area is highly trabecular therefore referred to as ‘trabecular defects’
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Anterior or posterior malalignment VSDs

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Outlet VSD vs Perimembranous VSD

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Hypoplastic left heart syndrome

A

•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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Ebstein’s Anomaly

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Williams Syndrome

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Stepwise approach to diagnosis of congenital heart disease

  1. Thoraco-abdominal situs
  2. Cardiac position (apex)
  3. Segment by segment analysis (atria -> AV valves -> ventricles -> outflow tracts -> semilunar valves + great vessels)
  4. Associated abnormalities
A
Segment by segment analysis — morphology determines identity
—> atrial situs
•solitus = normal (RA on right)
•inversus = mirror image of solitus
•ambiguous = complicated
—> RA morphology
•pectinate muscles outside RAA
•RAA broad based, triangular, anterior
•septum secundum (limbus of fossa ovalis)
•IVC, SVC, and CS (usually)
•crista terminalis, tinea Sagittarius
—> LA morphology
•pectinate muscles confined to LAA
•LAA long and narrow, posterior
•septum primum
•pulmonary veins (normally, unless TAPVR)
—> Atrioventricular valves
Oversimplification but good general rule:
•TV connects to RV (TV = 3 leaflets)
•MV connects to LV (MV = 2 leaflets)
—> Ventricles
•D-Loop  — normal, right handed looping, anterior + rightward RV, posterior + leftward LV
•L-Loop — opposite direction
—> Ventricle morphology
Left ventricle
•smoother walls (less trabeculations)
•2 well defined papillary muscles
•attached to MV
•no muscular outflow tract
•fibrous continuity between AV and MV
Right ventricle
•wraps around the heart
•muscular conus
•attached to TV
•complicated papillary muscles
•moderator band
•no fibrous continuity between TV and PV
—> Outflow tracts (conus = infundibulum, semilunar valves, and great vessels
•subpulmonary — normal
•subaortic — D-transposition [RV -> aorta, LV -> PA]
•L-transposition [LV on right -> PA ; RV on left -> aorta]
•bilateral — DORV (uncommon)
•bilaterally absent — DILV (uncommon)
•identifying PA
17
Q

L-TGA (Levo transposition of the great arteries)

RV on left —> aorta
LV on right —> PA

Ventricles are switched

A

“Double discordance” (atrioventricular and ventriculoarterial) or ‘ventricular inversion’, ‘congenitally corrected’ (normal in series flow without a shunt)
•Systemic RV and TV that is almost always abnormal

90% associated with other lesions
•70-80% VSD
•30-60% pulmonary outflow obstruction (LVOTO)
•90% TV abnormalities (leads to failure of the systemic RV)
•55% MV abnormalities
•complete heart block most common arrhythmia (unstable conduction system)

•PA banding —> decreases TR
Increases LVOTO causing septum to shift left improving RV geometry and function which decreases TR and retrains the LV to be the systemic ventricle prior to surgical correction

18
Q

D-TGA (Dextro transposition of the great arteries)

LV on left —> PA
RV on right —> aorta

Great vessels are switched

A

•Normal D-loop ventricles but transposed great vessels
•Results in 2 parallel circulations and ‘reverse differential cyanosis’ [post ductal stats > pre ductal sats —> higher saturations in the feet than the right upper extremity]
•Need adequate mixing via ASD/VSD/PDA to sustain life otherwise perform a balloon atrial septostomy at birth
•Fibrous continuity between PV and left sided MV
Repairs:
•atrial switch : baffle shunts blood from SVC to LA and from pulm veins to RA but still have a systemic RV w/ multiple atrial suture lines
•arterial switch : switches the PA and aorta to avoid a systemic RV but have to reimplant the coronaries
•Rarely associated with other non cardiac anomalies
•Associated VSD in 50% — allows mixing and trains LV to becomes the systemic ventricle