pulmonary_hypertension
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Table of Contents
pulmonary hypertension (PAH)
see also:
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
- it appears it may in part at least, be caused by elevated levels of microRNA-224 which orchestrates BMP and TGFβ signaling and thus acts as a regulator of pulmonary vascular remodeling, and blocking this in animal models appears to help reverse the condition1)
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:
- congenital left-to-right shunts
- portopulmonary hypertension
- HIV-associated pulmonary hypertension
- 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
- impaired respiration
- obstructive sleep apnea
- alveolar hypoventilation disorders
- long-term exposure to high altitude
Group 4
- chronic thromboembolic pulmonary hypertension (CTEPH)
Group 5
- unclear or multifactorial aetiologies
- haematologic disorders
- systemic disorders
- pulmonary Langerhans cell histiocytosis
- lymphangioleiomyomatosis
- neurofibromatosis
- metabolic disorders
- glycogen storage disease
- Gaucher disease
- thyroid disorders
- miscellaneous conditions
- tumor obstruction
- mediastinal fibrosis
- chronic renal failure on dialysis
pulmonary_hypertension.1788442836.txt.gz · Last modified: 2026/09/03 13:40 by gary1