Pulmonary Hypertension Causes

Aka: Pulmonary Hypertension Causes, Causes of Pulmonary Hypertension, Pulmonary Arterial Hypertension Causes, Medication Causes of Pulmonary Arterial Hypertension, Drug-Induced Pulmonary Arterial Hypertension

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II. General

  1. Pulmonary Arterial Hypertension (Primary Pulmonary Hypertension) is idiopathic
  2. Causes below relate to Secondary Pulmonary Hypertension

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
    9. Post-Covid Long-Term Effects
      1. Persistent Dyspnea may be related to pulmonary vasculopathy (microthrombi related)
      2. Pulmonary Hypertension may develop in 22% after ICU care of Covid19
        1. Oktaviono (2021) Arch Med Sci 18(5): 1169-80 [PubMed]
  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. Heart Failure (with or without preserved ejection fraction)
  2. Valvular heart disease
  3. Other Congenital or Acquired cardiovascular conditions causing postcapillary 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. Obesity Hypoventilation Syndrome (marked Obesity)
    4. Sleep Apnea or other sleep disordered breathing
    5. Chronic exposure to high altitude

VI. Causes: Thrombotic or Thromboembolic - WHO Group 4

  1. Recurrent Pulmonary Embolus
  2. Sickle Cell Anemia
  3. Pulmonary Capillary Hemangiomatosis

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 Hemodialysis

VIII. References

  1. Rounds (April 1998) Consultant 797-819
  2. Abenhaim (1996) N Engl J Med 335:609-16 [PubMed]
  3. Chaouat (1996) Chest 109:380-6 [PubMed]
  4. Dunlap (2016) Am Fam Physician 94(6):463-9 [PubMed]
  5. Galie (2009) Eur Heart J 30(20): 2493-537 [PubMed]
  6. Mesa (1998) Mayo Clin Proc 73:37-45 [PubMed]
  7. Moser (1990) Circulation 81:1735-43 [PubMed]
  8. Simonneau (1998) Chest 114:195S-9S [PubMed]

IX. Resources

  1. World Symposium on Primary Pulmonary Hypertension
    1. http:/www.who.int/ncd/cvd/pph.html

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