A very nice Case in Govt. Gen. Hospital Chennai A female child aged 14 was referred for progressive breathlessness and abdominal distension Pic. 1. Abnormally dilated right atrium with significant pericardial effusion Pic. 2. Apart form RA ,RV dilatation , the RV apex is seen filled with coarse treabeculations.This is believed to be a type of non compaction Pic. 3. Tricuspid regurgitation is significant . Pic. 4. Doppler velocity in RVOT at 88mmhg Can you guess the Final diagnosis ?



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.

Dr suresh there is gradient of 90 mm Hg across PV IMage 3 . So this is VALVAR PS RV DYSFUNCTion RV FAILURE hence pericardial effusion . See thick RV . Normally RV wall thickness less than 5 . So with RVH ALWAYS LOOK AT PULMONARY VALVE. TR IS SECONDARY

k sir. .Thanks for your nice explanation

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need all views of Echo. It can be PS,RHEUMATIC MS,OR CARDIOMYOPATHY

sorry . tricuspid

This looks severe VALVAR PS WITH RV DYSFUNCTIOn with right sided failure. Needs proper video to rule out infundibular ps and look for morphology of PULMOARY valve. Adv DIUERTIC STABALISE THEN BPV IF VAVE SUTIABLE

Right sided heart failure. ..Pulmonary stenosis. .amyloidosis. .haemosiderosis. .constrictive pericarditis due to tb..restrictive cardiomyopathy

Nice differential

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Dr s k Pathak sir pls share CXR ECG IT WILL BE INTEresting

grossly DILATED RA and RV severe PAH mostly PS

mitral regurgitation. rheumatic heart disease

These all due to RVF. NEEDS DIURETIC

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