Marcela Boháčeková, Iveta Šimková
Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by chronic thromboembolic obstruction in
the pulmonary bed. Although multiple risk factors for the development of CTEPH have been identified so far, the definitive
pathogenesis of this rare disease remains unexplained. Echocardiography plays a key role in the diagnostic process
of pulmonary hypertension (PH) followed by a ventilation/perfusion lung scintigraphy. The right-side heart catheterization
with pulmonary angiography remains a gold standard for accurate diagnosis of CTEPH. The first choice of therapeutic
algorithm is surgical treatment – pulmonary endarterectomy (PEA), which represents a potentially curative treatment.
Symptomatic patients contraindicated for surgical treatment or those with persistent or recurrent CTEPH after
pulmonary endarterectomy may benefit from specific pharmacologic treatment, preferably with a new molecule – riociguat.
At the moment there is a prospective method with limited results called balloon pulmonary angioplasty.