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Overview of pulmonary hypertension in adults

Authors
Lewis J Rubin, MD
William Hopkins, MD
Section Editor
Jess Mandel, MD
Deputy Editor
Geraldine Finlay, MD

INTRODUCTION

Pulmonary hypertension (PH) refers to elevated pulmonary arterial pressure. PH can be due to a primary elevation of pressure in the pulmonary arterial system alone (pulmonary arterial hypertension; pre-capillary PH), or secondary to elevations of pressure in the pulmonary venous and pulmonary capillary systems (pulmonary venous hypertension; post-capillary PH). PH can be a progressive, fatal disease if untreated, although the rate of progression is highly variable. Most patients with PH should be evaluated in a center with expertise in the diagnosis and management of this population.

The classification, etiologies, epidemiology, and prognosis of PH are reviewed here. The pathogenesis, clinical features, diagnostic evaluation, diagnostic criteria, and treatment of PH are discussed in detail separately. (See "Pathogenesis of pulmonary hypertension" and "Clinical features and diagnosis of pulmonary hypertension in adults" and "Treatment of pulmonary hypertension in adults" and "Cor pulmonale".)

DEFINITION

Pulmonary hypertension (PH) is defined by a mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest usually confirmed by right heart catheterization [1]. Normal pulmonary arterial systolic pressure ranges from 15 to 30 mmHg, diastolic pressure from 4 to 12 mmHg, and normal mPAP is ≤20 mmHg. Many clinicians consider mPAP of 21 to 24 mmHg as borderline elevated and of uncertain clinical significance. (See 'Diagnosis' below.)

PH can be due to a primary elevation of pressure in the pulmonary arterial system alone (pulmonary arterial hypertension; pre-capillary PH), or secondary to elevations of pressure in the pulmonary venous and pulmonary capillary systems (pulmonary venous hypertension; post-capillary PH) (table 1). Although pulmonary hypertension can be measured on echocardiography, the gold standard for diagnosis is right heart catheterization.

CLASSIFICATION

Pulmonary hypertension (PH) was previously classified as primary pulmonary hypertension (no identifiable cause) or secondary pulmonary hypertension (identifiable cause). Primary pulmonary hypertension is now called idiopathic pulmonary arterial hypertension (IPAH; part of group 1 PAH). It has a pathophysiology, natural history, and response to therapy that is distinct from the other types of PH. Many additional causes of PH have been identified. It was recognized that some types of secondary PH closely resemble IPAH, such that the World Health Organization (WHO) has reclassified PH based upon etiology and mechanism into the five groups listed below (table 2) [2,3]. Pulmonary arterial hypertension (PAH) refers to group 1 PAH. PH refers to any of group 2 through group 5 PH, and is also used when referring to all five groups collectively.

                    

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