Dumitrescu, Daniel; Gerhardt,
Felix; Viethen, Thomas; Schmidt, Matthias; Mayer, Eckhard; Rosenkranz,
Stephan. BMC

Pulmonary Medicine, December 2016, Vol. 16 Issue: Number 1
p1-4, 4p;

Abstract: Chronic thromboembolic pulmonary hypertension
(CTEPH) is a progressive disease. For patients with operable CTEPH,
there is a clear recommendation for surgical removal of persistent
thrombi by pulmonary endarterectomy (PEA). However, without the
presence of PH, therapeutic management of chronic thromboembolic
disease (CTED) is challenging – especially in highly trained subjects
exceeding predicted values of maximal exercise capacity.
A 43-year-old male athlete reported with progressive exercise
limitation since 8 months. Six months earlier, pulmonary embolism had
occurred, and was treated since with oral anticoagulation. A pulmonary
ventilation/perfusion scan showed severe ventilation/perfusion
mismatch: chest CT and pulmonary angiography revealed bilateral
wall-adherent thrombotic material, but pulmonary hemodynamics were
completely normal. His peak oxygen uptake exceeded predicted values,
however exercise ventilatory efficiency was abnormal, compared to a
matching athlete. After thoroughly discussing therapeutic options with
the patient, he successfully underwent pulmonary endarterectomy at an
expert center. Five and twelve months after surgery, his maximal
exercise capacity and ventilatory efficiency profoundly improved beyond
preoperative values, and his subjective exercise tolerance had returned
to normal.                   Significant CTED may be present without
relevant pathologic changes in pulmonary hemodynamics at rest. Reaching
normal values of maximal exercise capacity does not exclude pulmonary
vascular disease in highly trained subjects. More data are needed to
evaluate the risk-/benefit ratio of PEA in patients with CTED and
normal pulmonary hemodynamics. A thorough discussion with the patient
as well as shared decision making regarding therapy are mandatory.
Cardiopulmonary exercise testing may add important clinical information
in the non-invasive diagnostic evaluation at baseline and during
follow-up.