RIGHT INFLOW ANOMALIES U2 L2

Which right inflow anomaly is a regurgitant lesion, and which is a stenotic lesion?

• Tricuspid dysplasia → regurgitant lesion

• Cor triatriatum dexter → stenotic lesion

How many leaflets does the tricuspid valve have?

3 leaflets.

List the 3 tricuspid valve leaflets and their characteristics.

• Anterior — large, redundant, and sail-like

• Posterior — located along the RV free wall

• Septal — connected to the ventricular septum

What is the BEST view to see the posterior leaflet of the TV?

RV Inflow In PLAX view

What is the normal distance between the hinge points of the tricuspid and mitral valves?

<0.8 cm/m²

(In A4C The TV is slightly apically displaced then the MV, In EA the displacement would be >0.8 cm/m²)

How common is tricuspid dysplasia? but its incidence is what?

It is the most common tricuspid abnormality, but its incidence is unknown.

Where is tricuspid dysplasia frequently described? List 2

• Fetal cases

• Labradors

What does congenital tricuspid dysplasia cause?

Severe tricuspid regurgitation.

List the 2 structural abnormalities that cause gaps in tricuspid leaflet coaptation.

• Thickened/myxomatous leaflet tissue

• Chordal shortening

What may happen to segments of the tricuspid leaflets in tricuspid dysplasia?

They may become flail.

Are the tricuspid valve leaflets apically displaced in tricuspid dysplasia?

No. There are no apically displaced leaflets.

What is important to remember about RV function early in tricuspid dysplasia?

RV function is still preserved

How is tricuspid regurgitation from tricuspid dysplasia usually tolerated during childhood?

It is usually well tolerated

Where does fluid eventually back up with severe tricuspid dysplasia? List 4

(Eventually once we get into further adulthood this will happen)

• Jugular veins / JVD

• Liver

• Peripheral tissues → edema

• Abdomen → ascites

Peripheral edema is the swelling of lower legs, ankles, feet, hands, or arms caused by excess fluid trapped in body tissues

What can severe tricuspid dysplasia eventually lead to? List 2

Severe TR, what will the back up of fluid lead to??????

• Severe right-heart failure

• Renal failure

List the 5 clinical symptoms/findings of tricuspid dysplasia.

• Elevated V waves in the jugular pulses (b/c of JVD)

• Peripheral edema

• RV heave (b/c there is volume overload)

• Hepatomegaly

• Exercise intolerance

List the 5 consequences of severe tricuspid regurgitation seen with tricuspid dysplasia. ECHO findings

• RA enlargement (dilation of annulus)

• RV enlargement

• Reduced RV systolic function

• Right-to-left shunting at the atrial level

• Eventually right-heart failure

List the 2 differential diagnoses for tricuspid dysplasia.

• Ebstein anomaly

• Pulmonary hypertension

List the 4 treatments for tricuspid dysplasia.

• Diuretics (To remove fluid)

• Annuloplasty ring (to reduce the size of the annulus)

• Valve/leaflet repair

• Valve replacement — not preferred for pediatric patients (b/c the valve doesnt grow with them as they age

What happens to the right side of the heart after successful surgery for tricuspid dysplasia?

The right side will reduce in size.

What is cor triatriatum dexter?

A membrane created from the Eustachian valve.

What can vary about the membrane in cor triatriatum dexter? List 2

• The membrane varies in Size & Number of fenestrations

(holes, the smaller the it is can cause stenosis, bigger has no problems)

Where does the cor triatriatum dexter membrane originate and extend?

It originates from the IVC and extends to the interatrial septum (IAS).

What does the membrane do to the right atrium in cor triatriatum dexter? and the list the 2 names ?

It separates the right atrium into 2 components

One component - Postermedial

Second component - Anterolateral

What structures drain into the posteromedial component of the RA in cor triatriatum dexter? - one component

• SVC and IVC (caval veins)

• Coronary sinus

What structures are contained in the anterolateral component of the RA? List 2

• Right atrial appendage

• Tricuspid valve vestibule

What defect is present in most patients with cor triatriatum dexter?

An ASD

What is the purpose of the ASD in cor triatriatum dexter, and what direction does blood shunt? & where is the Location?

The ASD acts like a pop-off. (Relieves pressure off the RA due to the stenosis)

The shunt is right-to-left

Location is variable

What is the Incidence / etiology of cor triatriatum dexter?

It occurs as sporadic cases.

What can tricuspid stenosis from cor triatriatum dexter cause? List 2

• Increased right atrial pressure

• Right-sided congestion

(b/c blood is backing up)

Is the degree of obstruction the same in every patient with cor triatriatum dexter?

No. The degree of obstruction varies. (But is usually stenotic)

List the 8 clinical Symptoms findings of cor triatriatum dexter.

1. Fatigue

2. Edema

3. Palpitations

4. Neck-vein distention

5. Peripheral cyanosis

6. Diastolic rumble (TS)

7. RA enlargement on X-ray

8. Atrial fibrillation

What is the best imaging window for cor triatriatum dexter? List 2

Subcostal imaging views & PSAX

List the 5 things to evaluate from the subcostal views in cor triatriatum dexter.

• Obstruction in the membrane

• Flow through the tricuspid valve

• ASD

• Obstruction in the SVC, IVC, and coronary sinus

• SVC and IVC size and respiratory variation

List the 3 things to evaluate from PSAX in cor triatriatum dexter.

• Obstruction in the membrane

• Flow through the tricuspid valve

• Obstruction in the IVC and coronary sinus

What is the differential diagnosis for cor triatriatum dexter?

Congenital tricuspid stenosis.

Is treatment necessary for non-obstructive cor triatriatum dexter?

No. No treatment is necessary if it is non-obstructive. (removing the membrane may cause it to re grow back)

List the 2 treatments for obstructive/symptomatic cor triatriatum dexter.

• Diuretics

• Surgical resection of the membrane with ASD closure

Post op

List the 2 things to interrogate after cor triatriatum dexter repair.

• Pulmonary vein stenosis

• Membrane recurrence