Pulmonary hypertension with unclear and/or multifactorial mechanisms

Pulmonary hypertension with unclear and/or multifactorial mechanisms

Updated: 08/31/2022

© Jun Wang, MD, PhD

 

General features

  • Group 5 pulmonary hypertension
  • Associated with various disorders
    • Hematologic disorders: MPNs, postsplenectomy, sickle cell anemia, thalassemia, etc
    • Systemic disorders: Sarcoidosis, pulmonary Langerhans cell histiocytosis
    • Metabolic disorders: Autoimmune thyroid disease, glycogen storage disease, Gaucher disease
    • Other disorders: End-stage renal disease
    • Complex congenital heart disease

Pathogenesis

  • Pathogenesis not well understood

Clinical presentations

Treatment

  • Management of underlying etiology

 

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Contents

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