PRESCRIBING IN PRACTICE ■ P ulmonary hypertension refers to increased pressure in the pulmonary arterial circulation. The pulmonary circulation has to accommodate the entire cardiac output in each cardiac cycle, and evolution has adapted to this by making it a low-pressure high-flow system. However, pathology can affect both the arterial and venous components of this system. Pulmonary venous hypertension mainly refers to diseases that result in elevated venous pressure and occurs mainly from mitral valve and left-sided heart disease; these will not be discussed in this article. As a result of greater understanding of the molecular and cellular pathways involved in the pathobiology of pulmonary arterial hypertension (PAH), novel and exciting treatments have become available to treat this condition. These new drugs represent a huge step forward in the treatment of this universally fatal disease in that they allow improvement in quality of life and survival; however, they do not as yet offer a cure. Definition PAH is defined by consensus as a mean pulmonary artery pressure of above 25mmHg in the setting of a normal or reduced cardiac output, with a normal pulmonary capillary wedge pressure (PCWP) and elevated pulmonary vascular resistance (PVR). The normal PCWP is required to exclude the presence of significant left heart disease and pulmonary venous hypertension. As a result, the diagnosis of pulmonary hypertension requires invasive right-heart catheterisation. Classification Pulmonary hypertension is an umbrella term that refers simply to elevated pressure in the pulmonary vasculature. There are a wide variety of causes of this and the 2008 WHO conference in Dana Point attempted to clarify and organise these into a more defined classification (see Figure 2). 1 This article will focus on PAH (group 1).

Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary pulmonary hypertension" and to replace it with "idiopathic pulmonary hypertension"; to reclassify pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for pulmonary arterial hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-pulmonary shunts.

The articles are identical except for minor stylistic and spelling differences in keeping with each journal's style. Either citation can be used when citing this article.

Рабочие группы esC: Сердечно-сосудистая фармакотерапия, Сердечно-сосу-дистая хирургия, Врожденные пороки сердца у взрослых, Легочное крово-обращение и правожелудочковая функция, Клапанная болезнь сердца.Содержание данных рекомендаций, подготовленных Европейским Общест-вом Кардиологов (european society of Cardiology, esC) и Европейским Общест-вом Пульмонологов (european respiratory society, ers), опубликовано исклю-чительно для использования в личных и образовательных целях. Не допуска-ется коммерческое использование содержания рекомендаций. Рекомендации esC не могут быть переведены на другие языки либо воспроизведены, полно-стью или частично, без письменного согласия esC. Для получения данного согласия письменная заявка должна быть направлена в Oxford University Press -организацию, издающую european Heart Journal и официально упол-номоченную esC, рассматривать подобные заявки.Отказ от ответственности. Рекомендации esC/ers отражают взгляды esC/ ers и основаны на тщательном анализе научных данных, доступных во время подготовки данных рекомендаций. Медицинским работникам следует придер-живаться данных рекомендаций в процессе принятия клинических решений. В то же время, рекомендации не могут заменить личную ответственность медицинских работников при принятии клинических решений с учетом инди-видуальных особенностей и предпочтений пациентов и, при необходимости, предпочтений их опекунов и попечителей. Медицинские работники также несут ответственность в отношении дополнительной проверки всех надлежа-щих требований и правил перед назначением лекарственных средств и использованием медицинского оборудования. Ключевые слова: рекомендации, легочная гипертензия, легочная артериаль-ная гипертензия, хроническая тромбоэмболическая легочная гипертензия, врожденные пороки сердца, системные заболевания соединительной ткани, сердечная недостаточность, дыхательная недостаточность, антагонисты рецепторов эндотелина, ингибиторы фосфодиэстеразы 5 типа, аналоги про-стациклина, заболевания легких, заболевания левых камер сердца.

No abstract

Chronic obstructive lung disease (COPD) and diffuse parenchymal lung diseases (DPLD), including idiopathic pulmonary fibrosis (IPF) and sarcoidosis, are associated with a high incidence of pulmonary hypertension (PH), which is linked with exercise limitation and a worse prognosis. Patients with combined pulmonary fibrosis and emphysema (CPFE) are particularly prone to the development of PH. Echocardiography and right heart catheterization are the principal modalities for the diagnosis of COPD and DPLD. For discrimination between group 1 PH patients with concomitant respiratory abnormalities and group 3 PH patients (PH caused by lung disease), patients should be transferred to a center with expertise in both PH and lung diseases for comprehensive evaluation. The task force encompassing the authors of this article provided criteria for this discrimination and suggested using the following definitions for group 3 patients, as exemplified for COPD, IPF, and CPFE: COPD/IPF/CPFE without PH (mean pulmonary artery pressure [mPAP] <25 mm Hg); COPD/IPF/CPFE with PH (mPAP ≥25 mm Hg); PH-COPD, PH-IPF, and PH-CPFE); COPD/IPF/CPFE with severe PH (mPAP ≥35 mm Hg or mPAP ≥25 mm Hg with low cardiac index [CI <2.0 l/min/m(2)]; severe PH-COPD, severe PH-IPF, and severe PH-CPFE). The "severe PH group" includes only a minority of chronic lung disease patients who are suspected of having strong general vascular abnormalities (remodeling) accompanying the parenchymal disease and with evidence of an exhausted circulatory reserve rather than an exhausted ventilatory reserve underlying the limitation of exercise capacity. Exertional dyspnea disproportionate to pulmonary function tests, low carbon monoxide diffusion capacity, and rapid decline of arterial oxygenation upon exercise are typical clinical features of this subgroup with poor prognosis. Studies evaluating the effect of pulmonary arterial hypertension drugs currently not approved for group 3 PH patients should focus on this severe PH group, and for the time being, these patients should be transferred to expert centers for individualized patient care.

Acute right ventricular (RV) failure is a complex clinical syndrome that results from many causes. Research efforts have disproportionately focused on the failing left ventricle, but recently the need has been recognized to achieve a more comprehensive understanding of RV anatomy, physiology, and pathophysiology, and of management approaches. Right ventricular mechanics and function are altered in the setting of either pressure overload or volume overload. Failure may also result from a primary reduction of myocardial contractility owing to ischaemia, cardiomyopathy, or arrhythmia. Dysfunction leads to impaired RV filling and increased right atrial pressures. As dysfunction progresses 227to overt RV failure, the RV chamber becomes more spherical and tricuspid regurgitation is aggravated, a cascade leading to increasing venous congestion. Ventricular interdependence results in impaired left ventricular filling, a decrease in left ventricular stroke volume, and ultimately low cardiac output and cardiogenic shock. Identification and treatment of the underlying cause of RV failure, such as acute pulmonary embolism, acute respiratory distress syndrome, acute decompensation of chronic pulmonary hypertension, RV infarction, or arrhythmia, is the primary management strategy. Judicious fluid management, use of inotropes and vasopressors, assist devices, and a strategy focusing on RV protection for mechanical ventilation if required all play a role in the clinical care of these patients. Future research should aim to address the remaining areas of uncertainty which result from the complexity of RV haemodynamics and lack of conclusive evidence regarding RV-specific treatment approaches.