Rare encounters in echo lab : Abnormal right atrium and right ventricle

A female child aged 14 was referred for progressive breathlessness  and  abdominal distension

Abnormally dilated right atrium with significant pericardial effusion .www.drsvenkatesan.com

Can you guess the diagnosis ?

Apart form RA ,RV dilatation , the RV apex is seen filled with coarse treabeculations.This is believed to be a type of non compaction www.drsvenkatesan.com

Still difficult to conclude  ?   Look at the following Image.

Tricuspid regurgitation is significant . www.drsvenkatesan.com

If you have thought  . . .

• ASD with TR
• Severe PAH/COPD
• RV cardiomyopathy

All are  acceptable  differential diagnosis

But the real diagnosis is none of the above .

Need  more time  . . . the following   Doppler tracing  will settle the issue !

Doppler velocity in RVOT at 88mmhg. www.drsvenkatean.com

The final diagnosis was . . .

• Severe valvular pulmonary stenosis
• Marked RV,RA dilatation
• Acquired non compaction of right ventricle
• TR -Moderate
• Pericardial effusion -Moderate
• This patient also had dilated IVC, Hepatic veins that  lead to clinical ascites.

Here , RV functional assessment becomes vital , but it is difficult many times. A simple clue is , as  the RV is able to generate 88mmhg pressure it implies ,   the   contractility  should be near normal .

RV EF %,  RV Dp/Dt , Tricuspid annular motion by  tissue Doppler are additional measures. Cine MRI can be a useful investigation prior to intervention.

Final message

• VPS is a common acyanotic disease. Most are benign  and  milder  forms are the rule.
• Dysplastic valves preclude balloon valvotomy. (In late stages   little  difference between dysplastic / non dysplastic VPS is noted  )
• Severe progressive VPS  , like in this patient needs immediate balloon dilatation or surgery.
• Long term outcome  is excellent except in advances cases where irreversible RV dysfunction sets in.