CHD Lesions

Question 1

List 3 characteristics of Tricuspid Atresia with VSD

Answer 1

1) Normal PDA size
2) RV/outflow/PAs are adequate in size
3) Often {S,D,S} or {S,D,D}

Question 2

Tricuspid Atresia INTACT IVS

Answer 2

PDA only supplies pulmonary flow and is usually small
Ascending aorta and isthmus are large bc they carry entire combined outflow
RV/PA/branch hypoplasia or atresia

Question 3

Ebstein’s Anomaly

Answer 3

Failure of delamination creating dysplastic TV with inferior displacement—-> leaflet most affected is SEPTAL, then posterior, then anterior.
*valve can also be stenotic or imperforate
The TV orifice often directs towards RVOT not RV apex.
Atrialized RV
Sometimes can cause ‘functional pulmonary atresia’

Question 4

1) What TV leaflets do you see in the RVIT view?
2) PSAX?
3) Apical?

Answer 4

1) posterior (left of screen) and anterior (right of screen)
2) anterior (left of screen) and septal (right of screen)
3) anterior (left of screen) and septal (right of screen)

Question 5

List 5 characteristics of Complete AVSD

Answer 5

1) Large primum ASD
2) Large inlet VSD
3) Common AV valve (typically 5 leaflets—-3 lateral (free walls) and 2 medial (superior and inferior “bridging” leaflet)
4) Balanced vs unbalanced
5) Present in 40% of Trisomy 21

Question 6

List 5 characteristics of Partial AVSD

Answer 6

1) 2 distinct AV valve orifices at same level
2) Cleft in anterior LAVV leaflet— oriented towards septum (& sometimes cleft septal RAVV leaflet)
3) often small to moderate Inlet VSD
4) often small to moderate primum ASD
5) Less common in Trisomy 21

Question 7

Transitional AVSD

Question 8

AVSD Rastelli classifications

Question 9

1) What variation of AVSD is often seen in Trisomy21?

2) In asplenia syndrome?

Answer 9

1) CAVC with TOF

2) CAVC with DORV

Question 10

What is Ebstein’s associated with

Answer 10

Cardiomegaly
LTGA with PS vs PA
WPW
Maternal exposure to Lithium

Question 11

What is sometimes called “functional pulmonary atresia” and why?

Answer 11

Ebsteins. The pulmonary insufficiency can cause a steal from the brain and body bc of PDA flow

Question 12

Truncus Arteriosus

Question 13

Interrupted arch

Question 14

T/PAPVR

Question 15

Describe DORV and list 3 types of

Answer 15

Overriding aorta > 50% with mitral aortic discontinuity.

TOF type— associated PS
VSD type- no outflow obstruction
TGA-like type— malposed great arteries with positions transposed (not true TGA bc not coming off opposing ventricle)

Question 16

Shone’s Complex

Question 17

ALCAPA

Question 18

HLHS

Question 19

Heterotaxy

Question 20

DTGA

Question 21

LTGA

Question 22

Vein of Galen

Question 23

Ventricular noncompaction

Question 24

SVC syndrome

Question 25

Endocardial Fibroelastosis

Question 26

Types of aortic coarctation

Question 27

Absent pulmonary valve syndrome

Question 28

Cardiac tumors

Question 29

Infiltrative disorders

Question 30

Types of conoseptal abnormalities

Question 31

Interrupted aortic arch types

Question 32

List 3 qualities of the LV and 3 qualities of the RV

Answer 32

LV: Smooth walled, mitral valve follows the ventricle and is septophobic, the mitral valve is further from the apex

RV: Hypertrabeculated with moderator band, the tricuspid valve follows the ventricle and is septophilic, the tricuspid valve is closest to the apex

** AV canal the AV valves are at the same level

Question 33

List the normal aortic arch branching anatomy from patient’s right to left (right of screen to left)

Answer 33

1) Innominate artery (bifurcating into right subclavian artery and right common carotid)
2) Left common carotid
3) left subclavian artery

Question 34

4 Types of coarctations and which is most common?

Answer 34

1. Juxtaductal (most common)— next to ductal region
2. Discrete coarctation— sudden significant narrowing of the aorta, often with posterior shelf
3. Long segment— from post isthmus down through descending
4. Distal coarctation— past the isthmus (original arch view can look very normal)

Question 35

What are 3 ways to identify VSD size (per CCHMC)

Answer 35

1) diameter size
2) pressure gradient
3) VSD/aortic annulus ratio

SMALL— 3mm or less, gradient above 60mmHg, 25% or less the size of ao annulus

MOD— 3-5mm, 25-60mmHg, 26-74% size of ao annulus

LARGE— 6-10mm, unrestrictive shunting, > 75% of aortic annulus

Question 36

The most severe form of HLHS is ____. It usually has a _____ (look for ______). It usually has a ______ while most other forms of HLHS don’t.

Answer 36

MS with aortic atresia — usually has very hard to identify LV— look for LV coronary fistulas/sinusoids—- usually has VSD but most other HLHS types don’t have sig VSD

Question 37

MS/AS type HLHS tends to have ____ and ____

Answer 37

A small- formed LV and EFE

Question 38

In HLHS _____ Is important to know and can cause ______ in utero. It is also important to evaluate for _____ , ____ and ______

Answer 38

1) Intact IAS (vs not)
2) Intact can cause vascular changes which can persist even after balloon septostomy
3) degree of TR (bc tv can be abnormal too)
4) Decompression vein (levocardinal vein—- most often to SVC—— can cause normal saturations even with intact IAS
5) Coarctation (80% of HLHS)