The study assess effects of inhaled nitric oxide on pulmonary haemodynamics, gas exchange and oxygen transport in patients with primary pulmonary hypertension (PPH) and evaluates factors predicting the response to inhaled nitric oxide. Ten patients with PPH (8 females, 2 males) were observed. Oxygenation and haemodynamic variables were measures and calculated at room air and then 15 min after each sequential addition of 10, 20 and 40 ppm nitric oxide to the gas mixture. Patients were considered responders if the mean pulmonary artery pressure or pulmonary vascular resistance decreased by 20%. An electrochemical gas sensor device PrinterNOx provided continuous analysis of nitric oxide and oxidative nitric oxide products. There was a dose-dependent improvement in haemodynamic variables that was maximal at 40 ppm nitric oxide (mean pulmonary artery pressure decreased from 67±7 to 56±5 mm Hg, pulmonary vascular resistance index decreased from 1335±302 to 790±102 dyne sec/cm -5 , and cardiac index increased from 2.3 to 2.7 L/min/m 2 , all p <0.05). There was a significant improvement in oxygen transport at 20 and 40 ppm nitric oxide (DO 2 improved from 684±108 to 791±110 and 835±98, respectively, p <0.05). Five patients (50%) responded to NO. The responders differed from non-responders in such factors as distance during 6-minute walking test, p a 0 2 and p a C0 2 (all p ≤0.01). Our data show an improvement in pulmonary haemodynamics and oxygen transport in patients with primary pulmonary hypertension.

Aim: To assess the state of cardiac and respiratory function in adult cystic fibrosis patients. 36 patients participated in the study. Lung function tests and Doppler echocardiography were performed to each patient. The majority of adult CF patients develop generalized obstructive pattern of lung function and hyperinflation. Significant decrease of VC and its part in TLC was noted. Assessment of exercise tolerance revealed significant decrease of hemoglobin oxygen saturation and heart rate increase. Right atrium and ventricle dilatation and an increase of systolic pressure in pulmonary artery were reported in 20% of patients, all of them had severe bronchial obstruction (FEV 1 <35% pred), in 28% of patients left ventricle dysfunction of both systolic and diastolic nature was registered. Our finding suggest that generalized obstructive disorders, hyperinflation and reduction in diffusion capacity are characteristic for adult cystic fibrosis patients. Pulmonary hypertension and cor pulmonale develop in patients with severe obstructive disorders.

The study was designed to investigate pulmonary haemodynamics and to evaluate its role in dyspnoe occurrence in Chernobyl clean-up workers suffering from chronic bronchitis. The study involved 38 Chernobyl patients with chronic bronchitis observed in 2000-2002. The mean age was 48.1 ±5.1 yrs, their smoking history was 18.0±12.7 pack-yrs. A severity of dyspnoe was evaluated with the MRC questionnaire; body plethysmography, lung diffusing capacity test, 6 -MWT, echocardiography, bronchoscopy with transbronchial lung tissue biopsy were also performed. Results demonstrated that pulmonary hypertension develops in Chernobyl workers even without respiratory functional disorders. This fact is probably due to incorporation of radionuclide dust particles to the lungs and occurrence of radiation-induced pulmonary vasculitis. The elimination of the dust particles out of the respiratory system is thought to delay the pulmonary hypertension development.

Ultrasound diagnostic methods completely respond to all current requirements for diagnostic procedures and are valuable, safe, and relatively independent on the patient's condition. As ultrasound scanners are improved and new diagnostic regimens are developed, their use in different clinical field is extended. The most common ultrasound methods in pulmonology are echocardiography and ultrasonography of pleural cavities. Ultrasound investigation of the lung parenchyma is limited due to full reflection of ultra-sound from the aerated lung tissue. The lung density is increased in respiratory diseases; this gives an opportunity to use ultrasonography together with radiological methods for diagnosis and, therefore, to decrease radiation dose for the patient.

Аннотация В статье рассматривается клинический пример современной терапии идиопатической легочной артериальной гипертензии у пациентки с клиническим ухудшением течения заболевания. Показана тактика замены одного препарата (бозентан) другим (мацитентан) в рамках одной группы патогенетической терапии (антагонисты рецепторов эндотелина). Представлена последняя классификация легочной гипертензии (Ницца, 2013 г.) Обсуждаются вопросы современной стратегии патогенетической терапии легочной гипертензии.

Было проведено сравнительное рандомизированное перекрестное контролируемое исследование режимов неинвазивной вентиляции легких (НВЛ) - СРАР и BiPAP - для сравнения их влияния на показатели центральной гемодинамики у больных с декомпенсацией хронической сердечной недостаточности (ХСН). Критериями включения в исследование больных, госпитализированных в стационар, были: декомпенсация ХСН, возникшая на фоне ишемической болезни сердца или дилатационной кардиомиопатии; фракция выброса левого желудочка (ЛЖ) 40 лет; нарастание одышки в течение периода 2 . Больные случайным методом были рандомизированы, после чего им проводилась НВЛ в режиме СРАР - 7,5 см вод. ст. или BiPAP (PS / PEEP) - 10 / 5 см вод. ст. При проведении НВЛ в режиме BiPAP в течение 30 мин отмечалось достоверное снижение частоты дыхания (ЧД) и сердечных сокращений (ЧСС) (р < 0,01), увеличение фракции выброса (р < 0,01) и ударного объема (р < 0,01), уменьшение систолического давления в легочной артерии (р < 0,05), снижение трикуспидальной и митральной регургитации (р < 0,05) и положительные изменения индекса Tei (р < 0,05), отражающего глобальную систоло-диастолическую функцию миокарда. При проведении НВЛ в режиме СРАР также отмечалось снижение ЧД (р < 0,05), ЧСС (р < 0,01), улучшение гемодинамических показателей, однако изменение индекса Tei не являлось достоверным. Таким образом, режимы НВЛ СРАР и BiPAP достоверно улучшают показатели сердечной гемодинамики у больных с декомпенсацией ХСН, однако BiPAP более значимо влияет на интегральный индекс систоло-диастолической функции ЛЖ.

In the available literature, there are single papers on the association of the values of external respiratory function and the EchoCG sings of pulmonary hypertension with the morphological parameters of pulmonary arterial branches in idiopathic pulmonary hypertension (IPH) and secondary pulmonary hypertension (SPH). The present investigation comprised a comprehensive study of life-time functional changes in the cardiopulmonary system and postmortem morphometric changes in the heart and pulmonary vessels in idiopathic pulmonary fibrosis (IPF)-induced SPH and IPH. The investigation has indicated that PPH and SPH are characterized by the same-type rearrangement of pulmonary arterial branches as intimal hyperplasia and medial hypertrophy; however, IPH, as compared with SPH, induces a more significant cor pulmonale. The magnitude of right and left ventricular dysfunction corresponds to the degree of pulmonary hypertension (PH) and cor pulmonale. In IPF, the decreases in tidal volumes and pulmonary diffusion capacity favor PH progression.

Morphological and morphometric studies have shown that secondary pulmonary hypertension is characterized by more pronounced pathological changes in the pulmonary arterial branches in severe chronic obstructive lung disease (COLD) than in idiopathic fibrosing alveolitis. Secondary pulmonary hypertension does develop in atopic bronchial asthma. Moreover, there are more significant pathological changes in the pulmonary arteries than in the bronchial ones. In severe COLD, the development of emphysema affects that of pulmonary arterial hypertension.

Examination covered 213 workers of asbestos technical ware plant. Standard X-ray examination no pulmonary and pleural changes were revealed. External respiration studies demonstrated moderate ventilation disorders in half of the examinees: in hardware workshop workers--restrictive type disorders, in autofriction components workshop workers--obstructive type ones with hyperventilation. Lower diffusing lung capacity in one-third of the workers in one of two autofriction components workshops proves most likely distributive disorders. Moderately increased serum alveolar mucin 3EG5 in 20% of the examinees could be a predictive criterion of slow formation of chisotyle pulmonary fibrosis.

Bronchoalveolar lavage fluid (BALF), broncho- and transbronchial biopsies were examined in Chernobyl wreckers eleven years after the accident. Cytologically and electron-microscopically, there was predominance of alveolar macrophages (AM) with many cytoplasmic foreign bodies containing clear needle-like crystals. Roentgenospectral analysis and laser mass-spectrometry revealed chemical elements which are part of non-active "hot particles" (Al, Si, Ca, Fe) as well as reactor fuel elements and the station constructive materials which are the active part of "hot particles". Programmed BALF allowed to decrease cytosis and the amount of AM containing foreign-body particles as well as concentration of the majority of chemical elements in AM.