Rare clinical case of the diagnosis of idiopathic pulmonary artery hypertension

In this work, a rather rare (morbidity of about 1–2 cases per 1 million of adult population per year) and interesting clinical case of the diagnosis of idiopathic pulmonary artery hypertension (IPAH) was described. This case was also known as Ayerza disease or Ayerza–Arilago syndrome. At the initial stage, this pathology was characterized by an asymptomatic course that caused certain difficulties in making a correct diagnosis. In general, this disease had a poor prognosis. The main cause of death was decompensated right ventricular failure. The occurrence of complications could be significantly reduced and the life quality of such patients could be improved by the timely diagnosis and correct approach.