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Pulmonary hypertension 

Pulmonary hypertension
Pulmonary hypertension

Nazzareno Galiè

, Alessandra Manes

, and Massimiliano Palazzini


July 27, 2017: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

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date: 11 July 2020

Pulmonary hypertension is a haemodynamic and pathophysiological condition defined as an increase in the mean pulmonary arterial pressure of ≥25 mmHg at rest, as assessed by right heart catheterization. In fact, while transthoracic echocardiography may provide clues on the presence of pulmonary hypertension, the haemodynamic evaluation offers a more precise and comprehensive assessment. Pulmonary hypertension is heterogeneous from a pathophysiological point of view, and the diversity is reflected in the haemodynamic definitions. The different haemodynamic forms of pulmonary hypertension can be found in multiple clinical conditions which have been classified into six main groups and at least twenty-six subgroups. Each main clinical group shows specific pathological changes in the lung distal arteries, capillaries, and small veins. If we combine the haemodynamic and clinical heterogeneity, we understand the complexity of an accurate diagnosis in the individual patient which is crucial for the prognostic assessment and treatment strategy. In addition, the concomitant presence of different haemodynamic and clinical mechanisms cannot be excluded in individual cases (e.g. in patients with congestive heart failure and associated lung diseases). The presence of pulmonary hypertension, as defined above, is always an ominous prognostic sign, even if the severity may differ according to the haemodynamic changes and underlying clinical condition. The therapeutic approach also is markedly different, according to the clinical group, and symptomatic and haemodynamic severity. For these reasons, the four more frequent clinical groups are discussed individually, while the classifications are described in the Introduction section.

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