Pulmonary hypertension: when the acute event leads to diagnosis. Case report in a patient with Ehlers-Danlos syndrome.

Abstract

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a pulmonary vascular pathology caused by the chronic obstruction of the major pulmonary arteries, usually being the consequence of recurrent episodes of pulmonary embolism. Such events are usually unknown; the delay in such a diagnosis can therefore lead to a deterioration of the clinical picture, worsening the overall prognosis.  This is a case of a 55-year-old man who came to the Emergency Room (ER) because of an acute exacerbation of chronic dyspnea, that he experienced for several years, in the absence of an explanatory diagnosis. Acute pulmonary embolism was diagnosed in the setting of the ER, with bedside echography resulting to be crucial to the work up. A multidisciplinary approach allowed proper treatment, management and a favourable outcome.