Review Article

Pulmonary Hypertension Secondary to COPD

Table 1

Updated clinical classification of pulmonary hypertension (Dana Point, 2008) [1].

(1) Pulmonary arterial hypertension (PAH)
 (1.1) Idiopathic PAH
 (1.2) Heritable
  (1.2.1) BMPR2
  (1.2.2) ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia)
  (1.2.3) Unknown
 (1.3) Drug- and toxin-induced
 (1.4) Associated with
  (1.4.1) Connective tissue diseases
  (1.4.2) HIV infection
  (1.4.3) Portal hypertension
  (1.4.4) Congenital heart disease
  (1.4.5) Schistosomiasis
  (1.4.6) Chronic hemolytic anemia
 (1.5) Persistent pulmonary hypertension of the newborn

(i) Pulmonary venoocclusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)

(2) Pulmonary hypertension owing to left-heart disease
 (2.1) Systolic dysfunction
 (2.2) Diastolic dysfunction
 (2.3) Valvular disease

(3) Pulmonary hypertension owing to lung disease and/or hypoxia
 (3.1) Chronic obstructive pulmonary disease (COPD)
 (3.2) Interstitial lung disease
 (3.3) Other pulmonary diseases with mixed restrictive and obstructive pattern
 (3.4) Sleep-disordered breathing
 (3.5) Alveolar hypoventilation disorders
 (3.6) Chronic exposure to high altitude
 (3.7) Developmental abnormalities

(4) Chronic thromboembolic pulmonary hypertension (CTEPH)

(5) Pulmonary hypertension with unclear multifactorial mechanisms
 (5.1) Hematologic disorders: myeloproliferative disorders, splenectomy
 (5.2) Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis,    neurofibromatosis, vasculitis
 (5.3) Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
 (5.4) Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis

Simonneau [1].