A patient who presents with predominantly right heart failure is an interesting clinical challenge . Constrictive pericarditis (CP) remains a popular diagnosis in this setting. However in the bed side clinical examination (and in cardiology Board exams ) the following differential diagnoses are to be considered .( And ruled out one by one)
- Restrictive cardiomyopathy* especially Right sided .In India endo myocardial fibrosis tops the list
- Primary Tricuspid valve disease( Tricuspid stenosis / Carcinoid etc)
- Chronic cor-pulmonale in terminal RV failure
- Silent Mitral stenosis with right heart failure
- Ebstein anomaly
- Severe forms of valvular pulmonary stenosis with RV dysfunction
- SVC obstruction
- Cirrhosis of liver
- Porto pulmonary hypertension
( The list is not complete , readers may contribute )
Bed side clues
- Remember a deep “y” descent is the bed side counter part of Square root sign recorded by invasive RV pressure study
- Similarly , pericardial knock is the auditory equivalent (You hear the square root ! . . .yes )as the ventricle thuds the rigid thickened pericardial shell in very early diastole !)
- Pulsus paradoxus and kussmal sign can occur in both CP and RCM.
- If a good LV apex , is palpated it goes against CP .
- Please be reminded , even restrictive cardiomyopathy will ultimately dilate their chamber pre-terminal and clinical features may be confounded with that of DCM.
- Silent heart would suggest CP.
- AV valve regurgitation would favor RCM
- Features of Pulmonary hypertension will help confirm Mitral valve disease , Cor pulmonale,
- Deep “y”descents are against any form of Tricuspid stenosis.
- Opening snap of mitral valve is to be distinguished from pericardial knock.( Opening snap high pitched and occur later than pericardial knock in diastole , best heard in expiration )
- Cirrhosis liver with hypo- proteinimic fluid retention is a traditionally close mimicker .It may be ruled out by the careful history taking as exertional dyspnea is an exception , if at all , it is a very late event in cirrhosis.
- The issue gets further weird as chronic constriction can lead on to chronic congestive liver and cardiac cirrhosis .
- Severe forms of constriction can invade the myocardium and result in features of myocardial dysfunction .It is more common than we recognise.
How to confirm ?
Following should be performed in that order
- X -Ray
- CT scan
*Cath study is no longer done (Only for academic purpose )
Even in this era of sophisticated medical imaging , clinical examination remains the key . One should realise the importance of meticulous clinical history , sequential examination and interpretation .It will “rule out or rule in” majority of cardiac disorders .
The hi tech imaging modalities should be used only to confirm , risk stratify and plan management . If you skip the clinical part , one may still arrive at a correct diagnosis but there is high chances of erring in management.
(Cardiac pearls lie in the bed side not in cath labs ! Here is one such pearl . Not every constriction require surgery !
Please note about 20 % of constrictive pericarditis are transient !)