Table of Contents

introduction
clinical features
aetiology
- Group 1
- Group 2
- Group 3
- Group 4
- Group 5

pulmonary hypertension (PAH)

see also:

pulmonary fibrosis

introduction

defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise
characterized by a progressive and sustained increase in pulmonary vascular resistance that eventually leads to RVF and cor pulmonale

clinical features

dyspnoea
weakness
recurrent syncope
+/- features of cor pulmonale

aetiology

Group 1

primary PAH
- rare
heritable PAH
drug or toxin induced PAH:
- aminorex
- fenfluramine derivatives
- toxic rapeseed oil
small pulm. arteriolar pathologies:
- connective tissue (CT) disorders such as scleroderma
- congenital left-to-right shunts
- portopulmonary hypertension
- HIV-associated pulmonary hypertension
- schistosomiasis
- chronic haemolytic_anaemia
persistent pulmonary hypertension of the newborn

Group 2

secondary to left-sided heart disease
- left-sided myocardial and valvular diseases
- extrinsic compression of the pulmonary veins (eg, tumors)
- pulmonary veno-occlusive disease

Group 3

secondary to lung diseases and/or hypoxia
- lung disease
  - chronic obstructive pulmonary disease (COPD)
  - pulmonary fibrosis
- impaired respiration
  - obstructive sleep apnea
  - alveolar hypoventilation disorders
- long-term exposure to high altitude

Group 4

chronic thromboembolic pulmonary hypertension (CTEPH)
- pulmonary embolism (PE)

Group 5

unclear or multifactorial aetiologies
haematologic disorders
- myeloproliferative disorders
systemic disorders
- sarcoidosis
- pulmonary Langerhans cell histiocytosis
- lymphangioleiomyomatosis
- neurofibromatosis
- vasculitis
metabolic disorders
- glycogen storage disease
- Gaucher disease
- thyroid disorders
miscellaneous conditions
- tumor obstruction
- mediastinal fibrosis
- chronic renal failure on dialysis