Marius M Hoeper1, Hossein-Ardeschir Ghofrani, Ekkehard Grünig, Hans Klose, Horst Olschewski, Stephan Rosenkranz. 1. Department of Pneumology, Hannover Medical School; German Center for Lung Research (DZL); Universities of Gießen and Marburg Lung Center (UGMLC), Gießen; Department of Pneumology, Kerckhoff Hospital Bad Nauheim; Department of Medicine, Imperial College, London, UK; Center for Pulmonary Hypertension, Chest Hospital, University Hospital Heidelberg; Pneumology Section, Center for Pulmonary Hypertension Hamburg, University Hospital Hamburg-Eppendorf; Department of Pneumology, University Hospital Graz; Department of Internal Medicine III and Cologne Cardiovascular Research Center (CCRC), Cardiac Center, University of Cologne.
Abstract
BACKGROUND: About 1% of adults suffer from pulmonary hypertension (PH). The various types of PH differ widely with respect to their incidence, clinical significance, and treatment. METHODS: Selective review of the literature in association with a consensus conference. RESULTS: Pulmonary hypertension is divided into five major categories. Those that are of particular clinical relevance are pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension due to left heart and lung diseases. Ten drugs from five different substance classes are now available for the treatment of PH and are often given in combination. The treatment strategy is determined by risk stratification based on the severity of disease, along with the clinical phenotype and possible accompanying illnesses. The preferred treatment for chronic thromboembolic pulmonary hypertension is surgical pulmonary endarterectomy; inoperable patients are treated with drugs and endovascular interventions. PH due to left heart and lung diseases generally calls for specific treatment of pulmonary hypertension only if there is severe right-heart strain. CONCLUSION: The diagnosis and treatment of severe forms of PH, in particular, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, are complex and are best carried out in close cooperation between the local physician and specialized centers.
BACKGROUND: About 1% of adults suffer from pulmonary hypertension (PH). The various types of PH differ widely with respect to their incidence, clinical significance, and treatment. METHODS: Selective review of the literature in association with a consensus conference. RESULTS:Pulmonary hypertension is divided into five major categories. Those that are of particular clinical relevance are pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension due to left heart and lung diseases. Ten drugs from five different substance classes are now available for the treatment of PH and are often given in combination. The treatment strategy is determined by risk stratification based on the severity of disease, along with the clinical phenotype and possible accompanying illnesses. The preferred treatment for chronic thromboembolic pulmonary hypertension is surgical pulmonary endarterectomy; inoperable patients are treated with drugs and endovascular interventions. PH due to left heart and lung diseases generally calls for specific treatment of pulmonary hypertension only if there is severe right-heart strain. CONCLUSION: The diagnosis and treatment of severe forms of PH, in particular, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, are complex and are best carried out in close cooperation between the local physician and specialized centers.
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