Chapter 129: Pulmonary Hypertension

DEFINITION

Elevation of pulmonary artery (PA) pressure (i.e., mean PA pressure >22 mmHg) due to pulmonary vascular or parenchymal disease, increased left heart filling pressures, or a combination. Table 129-1 lists etiologies of pulmonary hypertension (PH) by categories.

TABLE 129-1: Classification of Pulmonary Hypertension
Pulmonary Arterial HypertensionIdiopathic Heritable (mutations/familial cases) Collagen vascular diseases (e.g., scleroderma, SLE, rheumatoid arthritis) Congenital systemic to pulmonary shunts (e.g., ventricular septal defect, patent ductus arteriosus, atrial septal defect) Portal hypertension HIV infection Drugs or toxins (e.g., fenfluramine) Pulmonary Hypertension Due to Left Heart DiseaseLV systolic or diastolic dysfunction Left-sided valvular disease Pulmonary Hypertension Due to Lung Disease or HypoxiaChronic obstructive lung disease Interstitial lung disease Sleep-disordered breathing Chronic hypoventilation Chronic Pulmonary Thromboembolic DiseaseChronic pulmonary embolism MiscellaneousSystemic conditions (e.g., sarcoidosis, pulmonary histiocytosis) Hematologic conditions (e.g., myeloproliferative diseases)

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