Definitions and Diagnosis of Pulmonary Hypertension (original) (raw)

Pulmonary arterial hypertension: guidelines and unmet clinical needs

Reumatismo, 2021

The term pulmonary arterial hypertension (PAH) identifies a heterogeneous group of diseases characterized by a progressive increase in pulmonary arterial resistance (PVR), which causes a significant burden in terms of quality of life, right heart failure and premature death. The pathogenesis of PAH is not completely clear: the remodeling of the small pulmonary vessels is crucial, causing an increase in the resistance of the pulmonary circle. Its diagnosis is based on cardiac catheterization of the right heart. According to the present hemodynamic definition of pulmonary hypertension (PH) proposed by the Guidelines of the European Society of Cardiology/European Respiratory Society (ESC-ERS), the mean pulmonary arterial pressure (mPAP) values are ≥25 mmHg. In case of PAH, apart from an mPAP value ≥25 mmHg, patients must have a >3 Wood units increase in PVR and normal pressure values of the left heart. PH is a pathophysiological condition observed in more than 40 different diseases,...

Diagnosis and Assessment of Pulmonary Arterial Hypertension

Journal of the American College of Cardiology, 2009

The diagnosis and assessment of pulmonary arterial hypertension is a rapidly evolving area, with changes occurring in the definition of the disease, screening and diagnostic techniques, and staging and follow-up assessment. The definition of pulmonary hypertension has been simplified, and is now based on currently available evidence. There has been substantial progress in advancing the imaging techniques and biomarkers used to screen patients for the disease and to follow up their response to therapy. The importance of accurate assessment of right ventricular function in following up the clinical course and response to therapy is more fully appreciated. As new therapies are developed for pulmonary arterial hypertension, screening, prompt diagnosis, and accurate assessment of disease severity become increasingly important. A clear definition of pulmonary hypertension and the development of a rational approach to diagnostic assessment and follow-up using both conventional and new tools will be essential to deriving maximal benefit from our expanding therapeutic armamentarium.

Clinical update on pulmonary hypertension

Journal of Investigative Medicine, 2020

Pulmonary hypertension (PH) is a chronic, complex and challenging disease. Advances in treatment are for the subset of patients with pulmonary arterial hypertension. Selected review of the literature was conducted incorporating the European Society of Cardiology/European Respiratory Society 2015 guidelines and recommendations from the Sixth World Symposium on Pulmonary Hypertension. PH is classified into five groups based on WHO classification. Echocardiography remains the initial test of choice, and careful assessment of the right system aids in the diagnosis and prognosis of the disease. Right heart catheterization remains the gold standard of diagnosis and key guidance of treatment. Multidisciplinary approach is recommended for the care of patients with PH. Treatment selection is based on individual risk stratification of patients, and early referral to specialized PH centers improves outcomes of patients. Treating PH is complex and is best carried out in PH centers and with mult...

Pulmonary Arterial Hypertension: Evaluation of the Newly Diagnosed Patient

Seminars in Respiratory and Critical Care Medicine, 2005

With a quickly growing range of therapeutic options precise prognostic stratification became particularly important for clinical decision making in patients with pulmonary arterial hypertension (PAH). Assessment of disease-specific functional class remains the simplest method and is a fairly reliable way of assessing severity of PAH, and current guidelines recommend a therapeutic algorithm based on functional class evaluation. Six-minute walk is the most widely used test assessing exercise tolerance both in clinical practice and in clinical trials testing new therapies in PAH. Right heart catheterization with a vasoreactivity test is mandatory to identify a subgroup that has good long-term prognosis when treated with calcium channel blockers alone. Echocardiographic variables of documented prognostic value in PAH are not those directly correlated with pulmonary arterial pressure (PAP). Instead, the presence of pericardial effusion, enlarged right atrium, and elevated Doppler index of right ventricular performance have been the most consistently reported signs of poor prognosis. Low or decreasing brain natriuretic peptide (BNP) suggests a good outcome whereas troponin T leak persisting despite therapy is a poor prognostic sign. Biomarkers seem particularly useful for noninvasive follow-up of patients with PAH, and due to simplicity and low costs may successfully compete with echocardiography.

Pulmonary arterial hypertension: an update

Netherlands Heart Journal, 2011

Pulmonary arterial hypertension (PAH), defined as group 1 of the World Heart Organisation (WHO) classification of pulmonary hypertension, is an uncommon disorder of the pulmonary vascular system. It is characterised by an increased pulmonary artery pressure, increased pulmonary vascular resistance and specific histological changes. It is a progressive disease finally resulting in right heart failure and premature death. Typical symptoms are dyspnoea at exercise, chest pain and syncope; furthermore clinical signs of right heart failure develop with disease progression. Echocardiography is the key investigation when pulmonary hypertension is suspected, but a reliable diagnosis of PAH and associated conditions requires an intense work-up including invasive measurement by right heart catheterisation. Treatment includes general measures and drugs targeting the pulmonary artery tone and vascular remodelling. This advanced medical therapy has significantly improved morbidity and mortality in patients with PAH in the last decade. Combinations of these drugs are indicated when treatment goals of disease stabilisation are not met. In patients refractory to medical therapy lung transplantation should be considered an option.

Pulmonary Arterial Hypertension

American Journal of Respiratory and Critical Care Medicine, 2005

Echocardiography is a key screening tool in the diagnostic algorithm of pulmonary arterial hypertension (PAH). It provides an estimate of right ventricular function and pulmonary artery pressure, either at rest or during exercise, and is useful in ruling out secondary causes of pulmonary hypertension (PH) such as left heart disease or congenital heart disease. Several studies have showed that echocardiography is insufficiently precise as single tool for the ultimate diagnosis of PH respect to the right heart catheterization, considered the gold standard technique. Echocardiography is valuable in assessing prognosis and treatment options, monitoring the efficacy of specific therapeutic interventions, and detecting the preclinical stages of disease. The ideal imaging modality for accurate noninvasive assessment of the right heart should be accurate and precise, not influenced by loading conditions, routinely practicable and easily repeatable. For all such reasons and considering that PAH is a rare and severe condition, a complete noninvasive assessment of right heart function requires a deep knowledge of the disease and a multimodality approach. (Echocardiography 2014;0:1-15)