pulmonary hypertension (PAH)

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⁾

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

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

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

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