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: Failure of_______ creating _______ with _______ displacement—-> leaflet most affected is _______, then _________, then ________.
*valve can also be ______ or ______.
The TV orifice often directs towards _____ not _____.
Atrialized RV
Sometimes can cause ‘_______.’

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) septal leaflet (left of screen) and anterior (right of screen) **if CS and IVS are shown OR Posterior (left of screen) if not in view
2) posterior (left of screen) and anterior (right of screen)
3) anterior (left of screen) and septal (right of screen)

https://citoday.com/articles/2018-july-aug/echocardiography-for-tricuspid-valve-intervention

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) often cleft in anterior LAVV leaflet— oriented towards septum (& sometimes cleft septal RAVV leaflet)
3) often intact interventricular septum
4) small to moderate primum ASD
5) Less common in Trisomy 21

Question 7

Transitional AVSD/Intermediate AVSD

Answer 7

Two AV valve annuli with small to moderate ostium primum and inlet VSD and often LAVV cleft (can have RAVV cleft)

Question 8

AVSD Rastelli classifications

Answer 8

A —mural leaflet attached to crest of (conal) septum in subx en face view
B— very rare, anterior leaflet inserts to papillary muscle,
C—mural leaflet free floating

Question 9

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

2) In asplenia syndrome?

Answer 9

1) CAVSD with TOF

2) CAVSD with DORV

Question 10

What 4 things 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 types are based off of ____, _____, and _____. The Van Praagh classification may include a ______.

Describe each type.

The most common type is _____ at _____%

Answer 12

based on branch pulmonary arteries, the development of the aortic arch, and the presence of a PDA. With Van Praagh Each type may include a modifier “A” (with VSD) of “B” (intact ventricular septum).

• Type 1: The main pulmonary is present and bifurcates into the left and right pulmonary arteries (same as Collette and Edwards classification).

• Type 2: The right and left branch pulmonary arteries arise from the common trunk (usually back of trunk)

• Type 3: One branch pulmonary artery (typically the right) arises from the common trunk (usually on sides) and the other arises from a PDA or the aorta.

• Type 4: This type is defined by presence of aortic arch hypoplasia, coarctation or interrupted aortic arch and a large PDA.

Type 1 (A1) at 60%

Question 13

Describe Interrupted arch types and list 3 associations

Answer 13

Type A: interruption beyond the left subclavian artery (Most common type in AP windows)
Type B: interruption between left carotid and left subclavian artery (Most commonly associated with DiGeorge Syndrome)
- Can have aberrant right subclavian artery
LUE and LE saturations and blood pressure similar
If you Doppler the subclavian artery, will see diastolic flow reversal as it is distal to ductal flow
Type C: interruption between left innominate artery and left carotid artery

***IAA is associated with posteriorly deviated VSD

Question 14

T/PAPVR types

Answer 14

1) Supracardiac—- the veins typically join a posterior confluence that connects to vertical vein left chest which drains to innominate vein/SVC
2) Cardiac— The veins connect directly to the right atrium or to the coronary sinus
3) Infracardiac— the veins drain to the right side via the hepatic veins and IVC
4) Mixed - a mixture of any of these in all types look for obstruction!

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

Answer 16

Multiple left heart lesions often including:
1) supravalvar mitral ring
2) parachute mitral valve
3) subvalvar aortic membrane
4) coarctation

Question 17

ALCAPA

Answer 17

Anomalous origin of the left coronary artery from the pulmonary artery

• can arise from any pulmonary cusp, main pulmonary cusp, or branch PAs
• best recognized by retrograde flow in the coronaries into the PA
• 2D can look like clearly “normal origin” but if flow is retrograde then likely ALCAPA with intramural course

https://www.jacc.org/doi/10.1016/j.jcmg.2021.07.009

Question 18

Which is the worst HLHS subtype and describe 4 things to look for with HLHS

Answer 18

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

MS/AS—- tends to have some type of LV, often with EFE

1) define left heart anatomy (AS/AA, MS/MA, LV? Sinusoids?)
2) IAS patency is important!!
(Intact IAS causes vascular changes in utero which can persist postnatally even after atrial septostomy)
May see posterior devotion of IAS
3) 80% Association discrete coarctation
4) Look for decompressing vein (levo cardinal vein—- usually to SVC) —- can cause baby to still sat decently even if IAS is intact or very small
5) RV function and degree of tricuspid regurgitation is important
Tricuspid valve can be abnormal too

Question 19

Heterotaxy: Right atrial isomerism vs Left atrial isomerism

If you see ____ or ____ think LAI
If you see ____ or ____ think RAI

Answer 19

Right atrial isomerism almost always has IVC, though can be “juxtaposed”/appear higher in visceral situs view. assoc w ASPLENIA (no spleen) 2 3-lobed lungs and bronchus, 2 right atria midline liver, stomach can be anywhere, no spleen. Absent coronary sinus (the CS is a left sided feature not a right). Broad triangular “dogear” appendage. The RAA is next to the SVC. An SVC coming to a right atrium will have the crista terminus protrusion.

Left atrial isomerism = visceral situs view the IVC is interrrupted with azygous continuation lower than abdominal aorta. associated with polysplenia — 2 left lobed lungs and left bronchus, 2 left atria, midline liver, stomach can be anywhere, multiple spleen (with spleen dysfunction) Almost always has interrupted IVC. Narrow, finger-like appendage
*An SVC coming to a left atrium will not have the crista terminus protrusion
When aorta and IVC are on the same side its called “juxtaposition”

Interrupted IVC with heart block is a very high indicator of LA isomerism, also interrupted IVC or absent IVC

If you see AVSD/DORV combo or If you see IVC in wrong position at all check for right atrial isomerism!

Question 20

DTGA

Answer 20

Transposed great arteries—- RV is systemic, coronaries attached to nonpulmonary cusps (aorta is anterior and rightward)

Question 21

LTGA

Answer 21

Transposed great arteries (congenitally corrected) with isolated ventricular inversion (left sided RV is systemic) — associated with PS and VSD —- CAs arise from nonpulmonary cusp. The aorta is anterior and leftward of PA.

Question 22

Vein of Galen

Answer 22

Severe pulmonary hypertension with heart failure and hydrocephalus

develops when there is a direct connection between cerebral arteries and veins - it causes significant high output heart failure (excessive systemic venous return with ventricular noncompliance, especially on the left which further complicates a vicious cycle) and the PH is largely related to that (pressure = flow x resistance - vein of Galen means LOTS of flow) They are very difficult to medically manage and often the only treatment is embolization of the direct connections (cath procedure to block venous malformations)

Question 23

Ventricular noncompaction

Answer 23

measure the thickness of the noncompacted layer of the left ventricle’s myocardium and divide it by the thickness of the compacted layer, with a ratio greater than 2 typically indicating left ventricular noncompaction

Question 24

List 3 indications for concern for SVC syndrome and 5 symptoms

Answer 24

When your SVC gets blocked typically 1) after implanted devices or 2) cancers, most often lung cancer or 3) non-Hodgkins Lymphoma

Symptoms: Cough, face or neck swelling, feeling of fullness in your upper body, swelling in arms and hands, shortness of breath

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)

Question 39

Typical Van Praagh classifications:

1) Situs inversus totalis
2) DTGA
3) LTGA
4) Truncus
5) Interrupted IVC = _____ isomerism
6) DORV or DOLV

Answer 39

1) ILD or ILI
2) SDD/SLL or IDD/ILL
3) SDL/SLD or ILD/IDL
4) SDX or ILX
5) L/X,D,S (left atrial isomerism) (R/X for RA isomerism)
6) SDD/SLL or IDD/ILL