Stenosis that occurs below the pulmonic valve

Before valve. Associated with other defects such as Tetrology of Fallot, infundibular muscle bundle hypertrophy (double chamber RV).

Stenosis that occurs above the pulmonic valve

May occur in association with the genetic disorders (Noonan syndrome (affects connective tissue, CHD), Alagille syndrome (affects the liver, jaundice), Williams syndrome, etc.(heart and blood vessel disorder)) or as an isolated defect.

Stenosis that occurs in the left or right pulmonary artery branch

Branch PAs are found to be slightly smaller than normal with mild flow turbulence through the right and left PAs (usually <2 m/s). The stenosis is usually bilateral and tends to regress without the need for any intervention by about 3 months of age. Other forms of branch PA stenosis can be unilateral or bilateral and are often found in conjunction with other CHD (e.g., TOF, pulmonary atresia with VSD, other cyanotic heart defects), genetic syndromes (Williams, Noonan, Alagille), or acquired after cardiac surgery.

most commonly fusion of valve leaflets

thickened leaflets with poor mobility

Clinical results of PS include:

Based on systolic peak velocity or maximal instantaneous pressure gradients:

peak systolic velocity less than 3 m/s, PG = 36mmHg

Based on systolic peak velocity or maximal instantaneous pressure gradients:

peak systolic velocity, 3 to 4 m/s, PG = 36 to 64mmHg

Based on systolic peak velocity or maximal instantaneous pressure gradients :

peak systolic velocity greater than 4 m/s, PG = 64mmHg

The 4 characteristic features of TOF:

a cardiac malformation in which a single semilunar valve and great vessel emerge from the heart. Conotruncal abnormality.

There are 2 classification systems for Truncus Arteriosus:

There are 2 classification systems for Truncus Arteriosus, Collett-Edwards:

A main PA segment is present

There are 2 classification systems for Truncus Arteriosus, Collett-Edwards:

The branch PAs arise separately but close to each other from the posterior aspect of the truncus, no MPA.

There are 2 classification systems for Truncus Arteriosus, Collett-Edwards:

The branch PAs arise separately from the right and left aspects of the truncus, like Type II but PAs are farther apart from each other.

There are 2 classification systems for Truncus Arteriosus, Van Praagh Classification:

Pulmonary trunk arises from the truncus

There are 2 classification systems for Truncus Arteriosus, Van Praagh Classification:

2 PAs arise form the truncus

There are 2 classification systems for Truncus Arteriosus, Van Praagh Classification:

One PA arises from truncus, PDA or major aortopulmonary collateral artery (MAPCA).

There are 2 classification systems for Truncus Arteriosus, Van Praagh Classification:

With interrupted aortic arch

Involves an assortment of lesions that share the common feature that both the aorta and PA arise from the RV. Embryological persistence of the subaortic conus. Most forms include a VSD

Uncommon defect involving the abnormality of the tricuspid valve.

Tricuspid valve is abnormally formed and is more apically displaced than usual in the heart.

Apical displacement of the valves creates atrialization of a portion of the right ventricle.

Variable thinning of the wall of the “atrialized” RV (aRV) occurs, along with redundancy and tethering of the anterior leaflet of the TV.

True or False

The goal of imaging is to define the anatomy associated with Ebstein’s anomaly and to describe the severity of the lesion