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WHAT IS PH?

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WHAT IS PH? : DEFINITION AND CLASSIFICATION

DEFINITION AND CLASSIFICATION / PATHOPHYSIOLOGY / EPIDEMIOLOGY / CLINICAL PRESENTATION / MANAGEMENT / NATURAL HISTORY


Pulmonary arterial hypertension (PAH) is caused by a progressive increase in pulmonary vascular resistance (PVR) leading to right ventricular failure and premature death. The condition is diagnosed at right heart catheterisation by physiological variables, namely:

 

  • sustained elevation of mean pulmonary arterial pressure to >=25 mmHg AND
  • a mean pulmonary artery occlusion pressure of <=15 mmHg AND
  • a normal or reduced cardiac output

 

Pulmonary hypertension (PH), conversely, simply represents an elevated mean pulmonary artery pressure and can be a consequence of PAH or other conditions such as left-sided heart disease or hypoxic lung disease. The current classification of conditions leading to pulmonary hypertension is clinically based and is called the Dana Point classification (shown below).

 

1.  Pulmonary arterial hypertension

1.1 Idiopathic

1.2 Heritable

1.2.1 BMPR2

1.2.2 ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia)

1.2.3 Unknown

1.3 Drug and toxin induced

1.4 Associated with (APAH)

1.4.1 Connective tissue disease

1.4.2 HIV infection

1.4.3 Portal hypertension

1.4.4 Congenital heart disease

1.4.5 Schistosomiasis

1.4.6 Chronic haemolytic anaemia

1.5 Persistent pulmonary hypertension of the newborn

1'. Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis

 

2.  Pulmonary hypertension due to left heart disease

2.1 Systolic dysfunction

2.2 Diastolic dysfunction

2.3 Valvular disease

3.  Pulmonary hypertension due to lung diseases and/or hypoxaemia

3.1 Chronic obstructive pulmonary disease

3.2 Interstitial lung disease

3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern

3.4 Sleep disordered breathing

3.5 Alveolar hypoventilation disorders

3.6 Chronic exposure to high altitude

3.7 Developmental abnormalities

4.  Chronic thromboembolic pulmonary hypertension

 

5.  Pulmonary hypertension with unclear and/or multifactorial mechanisms

5.1 Haematological disorders: myeloprolferative disorders, splenectomy

5.2 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis

5.3 Metabolic disorders: glycogen storage disease, Gaucher's disease, thyroid disorders

5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis

 

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