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Pulmonary Hypertension.

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.

SUBMITTER: Hoeper MM

PROVIDER: S-EPMC5331483 | biostudies-literature | 2017 Feb

REPOSITORIES: biostudies-literature

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Pulmonary Hypertension.

Hoeper Marius M MM Ghofrani Hossein-Ardeschir HA Grünig Ekkehard E Klose Hans H Olschewski Horst H Rosenkranz Stephan S

Deutsches Arzteblatt international 20170201 5

<h4>Background</h4>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.<h4>Methods</h4>Selective review of the literature in association with a consensus conference.<h4>Results</h4>Pulmonary hypertension is divided into five major categories. Those that are of particular clinical relevance are pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and pulmo ...[more]

PMID: 28241922

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Project description:Pulmonary vascular arterial remodeling is an integral and well-understood component of pulmonary hypertension (PH). In contrast, morphological alterations of pulmonary veins in PH are scarcely described. Explanted lungs (n = 101) from transplant recipients with advanced chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary arterial hypertension (IPAH) were analyzed for venous vascular involvement according to a pre-specified, semi-quantitative grading scheme, which categorizes the intensity of venous remodeling in three groups of incremental severity: venous hypertensive (VH) grade 0 = characterized by an absence of venous vascular remodeling; VH grade 1 = defined by a dominance of either arterialization or intimal fibrosis; and VH grade 2 = a substantial composite of arterialization and intimal fibrosis. Patients were grouped according to clinical and hemodynamic characteristics in three groups: COPD non-PH, COPD-PH, and IPAH, respectively. Histological specimens were examined by a cardiovascular pathologist blinded to clinical and hemodynamic data. Pathological alterations of pulmonary veins were present in all hemodynamic groups, with the following incidences of VH grade 0/1/2: 34/66/0% in COPD non-PH; 19/71/10% in COPD-PH; and 11/61/28% in IPAH. In COPD, explorative correlation analysis of venous remodeling suggested a modest positive correlation with systolic and mean pulmonary artery pressure ( P = 0.032, respectively) and an inverse modest correlation with diffusion capacity for carbon monoxide ( P = 0.027). In addition, venous remodeling correlated positively with the degree of arterial remodeling ( P = 0.014). In COPD-PH and IPAH, advanced forms of pulmonary venous remodeling are present, emphasizing that the disease is not exclusively restricted to arterial lesions. In addition, venous remodeling may be related to the hemodynamic severity, but more rigorous analysis is required to clearly define potential relationships.

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Pulmonary Hypertension.

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Pulmonary Hypertension.

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