Nomenclature

Question 1

What is the nomenclature approach used for ACHD?

Answer 1

Sequential Segmental Approach

Question 2

Who proposed the sequential segmental approach?

Answer 2

Praagh in 1960s and revised by Anderson and colleagues

Question 3

What are the basic building blocks of the SSA?

Answer 3

Atria, Ventricles and the Great Arteries, AV and VA connections

Question 4

Morphological characteristics of RAA

Answer 4

Blunt triangular appendage

Broad junction to RA

Question 5

Morphological characteristics of LAA

Answer 5

Tubular

Narrow junction to LA

Question 6

Morphological characteristics of RA

Answer 6

Pectinate muscles extend from RAA to postero-inferior wall of RA

Question 7

Morphological characteristics of LA

Answer 7

Smooth posterior wall of LA

Question 8

What atrial arrangements can you have?

Answer 8

Situs solitus
Situs inversus
Situs ambiguous (Right/Left isomerism = 2 x RA/2xLA)

Question 9

How are the morphological characteristics in the SAA identified?

Answer 9

Echocardiogram

Question 10

Morphological characteristics of RV

Answer 10

Coarse apical trabeculations
Muscular infundibulum between TV and PV
Septomarginal trabeculation and moderator band

Question 11

Morphological characteristics of LV

Answer 11

Fine apical trabeculations
Fibrous continuity valves
Smooth septal surface

Question 12

Ventricular Patterns

Answer 12

Balanced (Normal)
RV/LV Hypoplasia
Primitive morphological ventricle

Question 13

How can you determine which is the primary ventricles in hypo-plastic RV/LV?

Answer 13

Based on morphological characteristics of large ventricle
Small ventricle in front and above –> large ventricle is LV
Small ventricle behind and below –> large ventricle is RV

Question 14

What is a primitive morphological ventricle?

Answer 14

Very rare

Mixed morphological characteristics of LV and RV

Question 15

Ventricular Arrangements

Answer 15

Normal
Ventricular inversion (mirror image)

Question 16

In what order do we undergo SSA?

Answer 16

Atria
Ventricles
AV
VA

Question 17

AV Connection Analysis

Answer 17

Mode

Type

Question 18

AV Connection Mode analysis

Answer 18

Details on AV valves, Chordae, papillary muscles and annuli

Question 19

AV Connection Type

Answer 19

Which atrium is connected or not to the ventricle:

Biventricular and Univentricular Types

Question 20

Biventricular AV Connection Types

Answer 20

Concordant (Atrial Situs solitus/inversus)
Discordant (RA to LV; LA to RV)
Ambiguous (In atrial isomerism = situs ambiguous)

Question 21

Univentricular AV Connection Types

Answer 21

Double Inlet (Both atria >50% connected to LV)
Absent AV connection (Usually RA not connected to RV)
If remnants of TV tissue, it is biventricular or double inlet with imperforate valve

Question 22

Modes of AV Connection

Answer 22

2 perforate valves
1 perforate and 1 imperforate valve
Common AV valve
Straddling 
Over-riding

Question 23

Types of common AV Valve

Answer 23

Balanced AVSD (equally above both ventricles)
Unbalanced AVSD (Greater part above one ventricle)

Question 24

Straddling AV Connection

Answer 24

Of left, right or both AV valves
Concerns tension apparatus of the valves (one or more chordae/papillary muscles attached to the myocardium in the wrong ventricle)

Question 25

Over-riding AV Connection

Answer 25

AV valves is over-riding the other ventricle that has the other AV orifice Biventricular type of AV connection if over-riding <50% Univentricular (double inlet) type of AV connection if over-riding>50%

Question 26

VA Connection Mode analysis

Answer 26

Details on arterial valves and annuli

Question 27

VA type analysis

Answer 27

Which ventricle is connected or not to which great artery

Question 28

Types of VA Connection

Answer 28

Concordant (normal ventricular pattern or ventricular inversion) Discordant (Normal ventricular pattern/ventricular inversion) Double outlet (both great arteries connected to one ventricles - usually RV) Single outlet VA connection

Question 29

What are the types of single outlet VA connection?

Answer 29

Common arterial trunk (truncus arteriosus) Single outlet aorta (pulmonary atresia) Single outlet aorta (Absent pulmonary trunk and arteries)

Question 30

Modes of VA Connection

Answer 30

2 perforate valves 1 perforate and 1 imperforate valve Over-riding (Bother arteries > 50% above RV: Double outlet VA connection)