A female child aged 14 was referred for progressive breathlessness and abdominal distension
Can you guess the diagnosis ?
Still difficult to conclude ? Look at the following Image.
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 !
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.
- 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.