Chapter 129: Pulmonary Hypertension


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
  1. Pulmonary Arterial Hypertension Idiopathic 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)
  2. Pulmonary Hypertension Due to Left Heart Disease LV systolic or diastolic dysfunction Left-sided valvular disease
  3. Pulmonary Hypertension Due to Lung Disease or Hypoxia Chronic obstructive lung disease Interstitial lung disease Sleep-disordered breathing Chronic hypoventilation
  4. Chronic Pulmonary Thromboembolic Disease Chronic pulmonary embolism
  5. Miscellaneous Systemic conditions (e.g., sarcoidosis, pulmonary histiocytosis) Hematologic conditions (e.g., myeloproliferative diseases)

There's more to see -- the rest of this topic is available only to subscribers.