II. General

III. Causes: Pulmonary Arterial Hypertension (PAH) - WHO Group 1

  1. Previously known as Primary Pulmonary Hypertension (PPH)
  2. Genetic predisposition
    1. BMPR2 gene (bone morphogenetic protein receptor type 2)
    2. ALK1 gene (activin receptor-like kinase type 1)
    3. Endoglin (with or without Hereditary Hemorrhagic Telangiectasia)
  3. Human Immunodeficiency Virus (HIV) Infection
  4. Significant liver disease with Portal Hypertension
  5. Collagen vascular disease (connective tissue disease)
  6. Schistosomiasis
  7. Congenital disorders
    1. Congenital Heart Disease with systemic to pulmonary cardiac shunts (left to right shunt)
    2. Persistent Pulmonary Hypertension of the Newborn
  8. Medications
    1. Aminorex
    2. Fenfluramine
    3. Dexfenfluramine
    4. Toxic rapeseed oil
    5. Amphetamines
    6. L-Tryptophan
  9. Possible causes of PAH
    1. Human Herpes Virus 8
    2. Selective Serotonin Reuptake Inhibitors
    3. Cocaine Abuse
    4. Chemotherapy agents
    5. Pregnancy
    6. Systemic Hypertension
    7. Thyroid disease (esp. Hyperthyroidism)
    8. Severe Anemia
  10. Unlikely causes of PAH
    1. Antidepressants
    2. Oral Contraceptives
    3. Estrogen Replacement Therapy
    4. Tobacco abuse
    5. Obesity

IV. Causes: Left Heart Conditions - WHO Group 2 (post-capillary Pulmonary Hypertension)

  1. Disease of the left atrium
  2. Disease of the left ventricle
  3. Central pulmonary vein compression
    1. Fibrosing mediastinitis
    2. Adenopathy
    3. Tumor masses

V. Causes: Respiratory (Progresses to Cor Pulmonale) - WHO Group 3

  1. Pulmonary parenchymal Disease
    1. Chronic Obstructive Pulmonary Disease (COPD)
    2. Interstitial Lung Disease
    3. Bronchiectasis
    4. Cystic Fibrosis
    5. Scleroderma
    6. Bronchopulmonary Dysplasia following neonatal RDS
  2. Breathing Mechanics
    1. Kyphoscoliosis or Pectus Excavatum
    2. Neuromuscular disorder
    3. Marked Obesity
    4. Sleep Apnea or other sleep disordered breathing
    5. Chronic exposure to high altitude

VI. Causes: Thrombotic or Thromboembolic - WHO Group 4

VII. Causes: Miscellaneous or multifactorial - WHO Group 5

  1. Hematologic causes
    1. Myeloproliferative disorders
    2. Post-splenectomy
  2. Systemic disorders
    1. Sarcoidosis
    2. Pulmonary Langerhans Cell histiocytosis
    3. Lymphangioleiomyomatosis
    4. Neurofibromatosis
    5. Vasculitis
  3. Metabolic disorders
    1. Glycogen Storage Disease
    2. Gaucher disease
    3. Thyroid disorders
  4. Other
    1. Tumoral obstruction
    2. Fibrosing mediastinitis
    3. Chronic Renal Failure on Dialysis

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