Constrictive pericarditis is a rare disease with an often unclear etiology.
There may be a long delay between the onset of the underlying disease and
the onset of clinical symptoms, which are fatigue, abdominal swelling, peri
pheral edema and breathlessness. However, if clinically apparent, these sym
ptoms may progress rapidly and severely disable the affected patient. Diagn
osis is achieved by the clinical presentation, echocardiography and/or MRI
and right heart catheterization.
The standard therapy is an extended pericardiectomy to restore an unlimited
inflow and outflow as well as an unrestricted diastolic function of both v
entricles. The risks of this procedure are related to dense adhesions betwe
en the 2 pericardial layers and severe calcifications especially of the epi
cardium. Incomplete removal results in persistent diastolic restriction whi
le lacerations of the underlying myocardium may lead to diffuse and extensi
ve bleeding and finally to myocardial dysfunction. Also, the postoperative
course may be complicated by persistent low output syndrome or acute ventri
cular dilatation. However, early surgical intervention in the hands of expe
rienced surgeons offers the best prognosis.