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Tightening Up
Cardiovascular System
Diagnosis and reasoning

This young woman has presented with rapidly progressive dyspnea associated with features of right sided heart failure.
Very few diseases give rise to this clinical picture - these include isolated right ventricular infarction, cardiac tamponade and
constrictive pericarditis.
The presence of a di use apical impulse and mu led heart sounds favor cardiac tamponade or constrictive pericarditis, although
this is by no means definite. Further di erentiation requires imaging - ideally echocardiography.
Her echocardiogram shows normal systolic function, normal cardiac contractility and no wall motion abnormalities - which
make right ventricular infarction very unlikely. In addition, the absence of a pericardial e usion excludes cardiac tamponade.
The presence of restricted diastolic filling and an abnormal septal bounce is strongly suggestive of constrictive pericarditis. While
restrictive cardiomyopathy may also give rise to these echocardiographic findings, the presence of early diastolic rapid filling
favors constrictive pericarditis.
The next step in her diagnosis is to determine the underlying cause of the disease. The history of a chronic cough and low grade
fever provide a strong clue by suggesting the possibility of tuberculosis, which is well known to cause constrictive pericarditis.
This is supported by the very high ESR.
Her chest x-ray shows apical shadowing of the right upper lung, which is characteristic (but not diagnostic) of tuberculosis.
Microscopy of sputum obtained via nebulization proves to be diagnostic by demonstrating the presence of acid-fast bacilli.
Thus the diagnosis is constrictive pericarditis complicated by right heart failure, which is secondary to active tuberculosis.
Steroids may reduce the inflammation in tuberculous pericarditis, while surgical pericardiectomy is required for definitive
treatment. There is no indication for thrombolysis or pericardiocentesis.
Discussion
Constrictive pericarditis is characterized by scarring and (usually) thickening of the pericardial sac, and is usually a sequel of
longstanding pericardial inflammation due to causes such as tuberculosis, mediastinal irradiation, cardiac surgery and chronic
idiopathic pericarditis.
In normal persons, the pericardium is moderately compliant (flexible). Thus, it is capable of accommodating physiological
increases in the cardiac volume ( i.e. following cardiac filling in diastole).
In addition, as the intrathoracic pressure rises and falls with the phase of respiration, this compliance allows the pericardium to
slightly contract or expand and thus transmit these pressure variations to the cardiac chambers.
This is important in maintaining a constant pressure gradient between the cardiac chambers and extra-cardiac but intrathoracic
pulmonary veins. This constant pressure gradient ensures equal filling of both ventricles regardless of the phase of respiration.
In constrictive pericarditis, the thickened and sti pericardium has a fixed volume. This limits cardiac expansion resulting in
impaired cardiac filling, and thus diastolic dysfunction, which later progresses to diastolic heart failure.
Note that filling is normal (and rapid) during early diastole. However, towards the end of diastole, filling stops suddenly - this is
the point where the filled ventricles attempt to accomodate additional blood by expanding, and are constrained by the rigid
pericardium.
In addition, the sti pericardium no longer transmits intrathoracic pressure changes to the cardiac chambers - although the
pulmonary veins are still subject to these pressure changes.
Thus, during inspiration, the pressure gradient between the pulmonary veins and le atrium drops, resulting in reduced le
ventricular filling. In addition, increased venous return from the vena cavae results in increased right ventricular filling. This
asymmetric ventricular filling results in the interventricular septum 'bulging' into the le ventricle.
During expiration, the converse of these events occurs, resulting in the interventricular septum bulging into the right ventricle.
This phenomenon is known as "ventricular interdependence"
The clinical manifestations of constrictive pericarditis arise from the systemic congestion and reduced cardiac output. The
systemic congestion results in an elevated JVP, tender hepatomegaly, ascites, pleural e usions and ankle edema. The reduced
cardiac output results in poor exercise tolerance, which may progress to cardiac cachexia with muscle wasting.
The classic auscultatory feature of pericardial constriction is the "pericardial knock", which is a high pitched diastolic sound
which occurs at the point where diastolic filling suddenly ceases. However, this sign may be absent and can be easily missed,
even when present.
ECGs are of limited use in these patients and may show non-specific changes or may even be completely normal. Chest X-rays
may show pericardial calcification, which is a specific but non-sensitive sign of constrictive pericarditis.
Transthoracic echocardiography may show pericardial thickening - although it is less sensitive than transesophageal
echocardiography or cardiac MRI. Note however that certain patients may have a normal pericardial thickness. In other patients,
the constriction may be localized to only one area, and thus may be missed.
Other echocardiographic features suggestive of constriction include restricted filling of both ventricles, early rapid diastolic filling
followed by a sudden rapid deceleration and interventricular septal movement with respiration.
One key issue to be kept in mind is that restrictive cardiomyopathy (especially when due to cardiac amyloidosis) may present
with clinical and echocardiographic features similar to that of constrictive pericarditis. Di erentiation is very important, as
constrictive pericarditis is potentially curable, while restrictive cardiomyopathy is rarely curable.
Features useful in di erentiating the two etiologies include absence of pericardial thickening, limited or no interventricular
septal movement with respiration, and (usually) major enlargement of the right and le atria in restrictive cardiomyopathy.
Patients with restrictive cardiomyopathy also tend to have elevated BNP levels (which is a manifestation of myocardial
stretching), while these are usually normal in constrictive pericarditis.
In certain patients it may still be impossible to di erentiate between these two etiologies - in which case catheterization and
endomyocardial biopsy may be necessary.
In general, medical therapy is ine ective in the management of constrictive pericarditis - although if a causative disease is
identified (e.g. tuberculosis), it should be treated. Surgical pericardial decortication (involving resection of the parietal and
visceral pericardium) is the treatment of choice - but is associated with a mortality rate of approximately 6%.
Take home messages

1. Constrictive pericarditis is characterized by scarring and (usually) thickening of the pericardial sac, and is usually a sequel of
longstanding pericardial inflammation.
2. The clinical manifestations of constrictive pericarditis are those of the systemic congestion and reduced cardiac output.
3. Echocardiography is key to diagnosis, although lateral chest x-rays and cardiac MRI may also impart important information.
4. Restrictive cardiomyopathy may mimic the clinical and echocardiographic findings of constrictive pericarditis. Rarely,
endomyocardial biopsy may be required for di erentiation.
5. Surgical pericardial decortication is the treatment of choice, even though the mortality rate is significant.
References
1. Current Problems in Cardiology : Cardiac Tamponade, Constrictive Pericarditis, and Restrictive Cardiomyopathy (2004)
2. Circulation : Cardiovascular Imaging : The Role of Multimodality Imaging in the Management of Pericardial Disease (2010)
3. Progress in Cardiovascular Diseases : A Modern Approach to Tuberculous Pericarditis (2007)
4. Heart : Di erential diagnosis of restrictive cardiomyopathy and constrictive pericarditis (2001)
5. Lancet : Pericarditis (2004)
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Tightening Up

Diagnosis and reasoning

Take home messages

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