Chronic thromboembolic pulmonary hypertension (CT-E PH) is a rare and
aberrant outcome of acute pulmonary embolism. Because it has become a
potentially curable form of pulmonary hypertension, the frequency of r
ecognized cases has increased. We report a case series of 72 patients
with CT-E PH evaluated in our institution between 1984 and 1993, and d
iscuss diagnostic clues and therapeutic approaches. All patients compl
ained of dyspnea on exertion, a history of acute thromboembolic event,
and lung murmurs were found in 60% and 17% of patients, respectively.
The presence of a disorder of coagulation was found in 30% of the pat
ients tested, the most common abnormality being lupus anticoagulant. T
he key noninvasive study for diagnosis was the lung perfusion scan whi
ch showed at least one segmental or larger perfusion defect in all pat
ients. Pulmonary angiography confirmed the diagnosis in all cases, and
sometimes associated to intravascular ultrasound imaging, established
the feasibility of thromboendarterectomy, Medical therapy included th
e use of long-term oral anticoagulant, and in the case of lower limb v
enous thrombosis, inferior vena cava filtration. Finally two surgical
procedures were discussed in selected patients: thromboendarterectomy
and lung transplantation. Since 1988, eight patients have benefited fr
om lung transplantation (six patients are still alive), and 11 patient
s underwent thromboendarterectomy which was successful in 9 patients w
ith a dramatic functional and hemodynamic improvement.