Выпот в полость перикарда может быть клиническим проявлением многих системных заболеваний, иногда яв-ляясь случайной находкой. К числу наиболее частых при-чин выпотного перикардита, по данным Европейской ас-социации кардиологов (European Society of Cardiology), относятся инфекционные заболевания, паранеопластиче-ский синдром, аутоиммунные болезни. Реже поражение перикарда ассоциируется с метаболическими нарушения-ми, лекарственными или другими токсическими воздей-ствиями [1]. Почти в 50% случаев причина перикардита остается неуточненной, а перикардит расценивается как идиопатический [2].

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the variants of pulmonary hypertension developing as a result of incomplete resolution of emboly in pulmonary artery (PA). It is characterized by an extremely pessimistic prognosis for the patient. The standard treatment of the patients presenting with this condition is pulmonary thrombendarterectomy under artificial circulation and circulatory arrest if possible. Recently trans-catheter balloon angioplasty of the PA has been considered as an alternative to pharmacological treatment in inoperable patients. This relatively new endovascular method implies the stage by stage intervention and makes it possible to significantly reduce mean blood pressure in the pulmonary artery down to its completely normal level resulting in lowering of the functional class of pulmonary hypertension. The most dangerous complications associated with the application of this method include hemorrhages in the case of perforation of PA branches and reperfusion pulmonary oedema. The present article contains a concise overview of the currently available approaches to the certification of reperfusion pulmonary oedema and the treatment of this condition including details of the patients' care as exemplified by a clinical situation we faced in our practice.

It is presented case report of pulmonary endarterectomy under prolonged circulatory arrest in patient with chronic thromboembolic pulmonary hypertension. Surgical treatment of these patients is followed by certain difficulties in postoperative management of patients. So, routine surgical procedures sre possible only in specialized centres for pulmonary hypertension.

Treatment algorithms for systemic sclerosis have not been completely developed. Effectivity of medications are usually used in clinical practice has a low level of evidence. Therefore, it is necessary to find a new treatment approaches for this nosological form. In the paper described clinical case of olokizumab treatment in a patient with diffuse systemic sclerosis with interstitial lung disease, polyserositis, severe microcirculatory alterations.

Pulmonary veno-occlusive disease (PVOD) is verified by identifying typical pathological changes in lungs or mutation in the EIF2AK4 gene. Clinical suspicion is based on combination of specific pulmonary pattern on computed tomography scan, hypoxemia with low diffusing lung capacity (DLCO) and inadequate response to specific therapy of pulmonary arterial hypertension (PAH). The rapid course of PVOD with high mortality determines the importance of early diagnosis and lung transplantation refferal.We present an unusual clinical case of 40-year-old woman with expected idiopathic PAH and extremely low DLCO with verified EIF2AK4 gene mutation, who demonstrated a positive vasoreactive test (VRT) and clinical response to calcium channel blockers therapy at the onset of the disease. The loss of positive VRT and subsequent PAH specific therapy escalation resulted in PVOD manifestation with severe desaturation, recurrent syncope and pulmonary edema. The combination of low DLCO and inadequate response to PAH therapy in idiopathic PAH patient should be of a paramount awareness of PVOD. Careful PAH therapy escalation with meticulous follow-up, pulmonary multispiral CT and genetic testing could improve early PVOD diagnostics and lung transplantation referral.