DISEASE SCANNER

Global Incurable Diseases Tracker

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Pulmonary Disease

Pulmonary Hypertension (PAH)

HIGH SEVERITY

A rare but serious condition characterized by high blood pressure in the pulmonary arteries, leading to right heart failure. Group 1 PAH is a progressive disease affecting the small pulmonary arteries, causing vessel narrowing and increased resistance to blood flow.

Global Affected

1.0M

Countries

15

Symptoms

Dyspnea on exertion
Fatigue
Chest pain
Syncope (fainting)
Palpitations
Lower extremity edema
Abdominal distension (ascites)

Treatment Options

Endothelin receptor antagonists (bosentan, ambrisentan)
PDE-5 inhibitors (sildenafil, tadalafil)
Prostacyclins (epoprostenol, treprostinil, iloprost)
Soluble guanylate cyclase stimulators (riociguat)
Calcium channel blockers (vasoresponsive patients)
Lung transplantation
Diuretics and oxygen

Risk Factors

1Female sex
2Age 30-60
3Connective tissue diseases (scleroderma, lupus)
4HIV infection
5Liver disease
6Congenital heart disease
7Family history (BMPR2 mutations)
8Anorexigen use

Diagnostic Methods

  • 1Echocardiogram
  • 2Right heart catheterization (gold standard)
  • 3Pulmonary function tests
  • 4CT pulmonary angiography
  • 5V/Q scan
  • 6Blood tests (BMP, autoimmune markers)
  • 76-minute walk test

Prognosis

Improved with modern therapy; median survival 7-10 years. Without treatment, median survival 2.8 years. Prognosis depends on functional class and hemodynamics at diagnosis.

Prevention

  • No primary prevention
  • Genetic counseling for familial cases
  • Avoid anorexigen drugs
  • Early recognition and treatment of underlying causes

Research Status

Multiple targeted therapies (prostacyclins, endothelin receptor antagonists, PDE-5 inhibitors) improve symptoms and survival. Lung transplantation for advanced disease. Early diagnosis critical.

Affected Countries

United StatesUnited KingdomCanadaAustraliaGermanyFranceItalySpainJapanBrazilIndiaChinaNigeriaSouth AfricaMexico

Sources

  • https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-hypertension
  • https://www.hematology.org/education/patients
  • https://www.ncbi.nlm.nih.gov/books
  • https://rarediseases.org/rare-diseases

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.