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2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Author/s: 
Humbert, M., Kovacs, G., Hoeper, M. M., Badagliacca, R., Berger, R. M. F., Brida, M., Carlsen, J., Coats, A. J. S., Escribano-Subias, P., Ferrari, P., Ferreira, D. S., Ghofrani, H. A., Ginnakoulas, G., Kiely, D. G., Mayer, E., Meszaros, G., Nagavci, B., Olsson, K. M., Pepke-Zaba, J., Quint, J. K., Rådegran, G., Simonneau, G., Sitbon, O., Tonia, T., Toshner, M., Vachiery, J. L., Noordegraaf, A. V., Delcroix, M., Rosenkranz, S., ESC/ERS Scientific Document Group

Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and may be associated with a variety of cardiovascular and respiratory diseases. The complexity of managing PH requires a multifaceted, holistic, and multidisciplinary approach, with active involvement of patients with PH in partnership with clinicians. Streamlining the care of patients with PH in daily clinical practice is a challenging but essential requirement for effectively managing PH. In recent years, substantial progress has been made in detecting and managing PH, and new evidence has been timeously integrated in this fourth edition of the ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Reflecting the multidisciplinary input into managing patients with PH and interpreting new evidence, the Task Force included cardiologists and pneumologists, a thoracic surgeon, methodologists, and patients. These comprehensive clinical practice guidelines cover the whole spectrum of PH, with an emphasis on diagnosing and treating pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH).

Keywords 
Chronic Thrombo-embolic pulmonary hypertension clinical practice cardiovascular respiratory disease Pathophysiological disorder

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Author/s: 
Humbert, M., Kovacs, G., Hoeper, M. M., Badagliacca, R., Berger, R. M. F., Brida, M., Carlsen, J., Coats, A. J. S., Escribano-Subias, P., Ferrari, P., Ferreira, D. S., Ghofrani, H. A., Ginnakoulas, G., Kiely, D. G., Mayer, E., Meszaros, G., Nagavci, B., Olsson, K. M., Pepke-Zaba, J., Quint, J. K., Rådegran, G., Simonneau, G., Sitbon, O., Tonia, T., Toshner, M., Vachiery, J. L., Noordegraaf, A. V., Delcroix, M., Rosenkranz, S., ESC/ERS Scientific Document Group

Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and may be associated with a variety of cardiovascular and respiratory diseases. The complexity of managing PH requires a multifaceted, holistic, and multidisciplinary approach, with active involvement of patients with PH in partnership with clinicians. Streamlining the care of patients with PH in daily clinical practice is a challenging but essential requirement for effectively managing PH. In recent years, substantial progress has been made in detecting and managing PH, and new evidence has been timeously integrated in this fourth edition of the ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Reflecting the multidisciplinary input into managing patients with PH and interpreting new evidence, the Task Force included cardiologists and pneumologists, a thoracic surgeon, methodologists, and patients. These comprehensive clinical practice guidelines cover the whole spectrum of PH, with an emphasis on diagnosing and treating pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH).

Keywords 
Chronic Thrombo-embolic pulmonary hypertension clinical practice cardiovascular respiratory disease Pathophysiological disorder

Diagnosis and Treatment of Pulmonary Arterial Hypertension: A Review

Author/s: 
Ruopp, N. F., Cockrill, B. A.

Importance: Pulmonary arterial hypertension (PAH) is a subtype of pulmonary hypertension (PH), characterized by pulmonary arterial remodeling. The prevalence of PAH is approximately 10.6 cases per 1 million adults in the US. Untreated, PAH progresses to right heart failure and death.

Observations: Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 20 mm Hg and is classified into 5 clinical groups based on etiology, pathophysiology, and treatment. Pulmonary arterial hypertension is 1 of the 5 groups of PH and is hemodynamically defined by right heart catheterization demonstrating a mean pulmonary artery pressure greater than 20 mm Hg, a pulmonary artery wedge pressure of 15 mm Hg or lower, and a pulmonary vascular resistance of 3 Wood units or greater. Pulmonary arterial hypertension is further divided into subgroups based on underlying etiology, consisting of idiopathic PAH, heritable PAH, drug- and toxin-associated PAH, pulmonary veno-occlusive disease, PAH in long-term responders to calcium channel blockers, and persistent PH of the newborn, as well as PAH associated with other medical conditions including connective tissue disease, HIV, and congenital heart disease. Early presenting symptoms are nonspecific and typically consist of dyspnea on exertion and fatigue. Currently approved therapy for PAH consists of drugs that enhance the nitric oxide-cyclic guanosine monophosphate biological pathway (sildenafil, tadalafil, or riociguat), prostacyclin pathway agonists (epoprostenol or treprostinil), and endothelin pathway antagonists (bosentan and ambrisentan). With these PAH-specific therapies, 5-year survival has improved from 34% in 1991 to more than 60% in 2015. Current treatment consists of combination drug therapy that targets more than 1 biological pathway, such as the nitric oxide-cyclic guanosine monophosphate and endothelin pathways (eg, ambrisentan and tadalafil), and has shown demonstrable improvement in morbidity and mortality compared with the previous conventional single-pathway targeted monotherapy.

Conclusions and relevance: Pulmonary arterial hypertension affects an estimated 10.6 per 1 million adults in the US and, without treatment, typically progresses to right heart failure and death. First-line therapy with drug combinations that target multiple biological pathways are associated with improved survival.

Keywords 
Pulmonary Arterial Hypertension diagnosis treatment PAH PH
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