Lung and pulmonary vascular disease in scleroderma, the role of ...
Lung and pulmonary vascular disease in scleroderma, the role of ...
Lung and pulmonary vascular disease in scleroderma, the role of ...
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PH – a syndrome<br />
1. Pulmonary Arterial Hypertension<br />
- Idiopathic PAH (IPAH)<br />
- Heritable<br />
- Drugs/tox<strong>in</strong>s<br />
- Associated PAH (APAH)<br />
connective tissue <strong>disease</strong>s<br />
HIV <strong>in</strong>fection<br />
portal hypertension<br />
congenital heart <strong>disease</strong>s<br />
1*. PVOD/PCH<br />
2. PH 2ry to left heart <strong>disease</strong><br />
- systolic <strong>and</strong> diastolic dysfunction<br />
- valvular<br />
3. PH 2ry to lung <strong>disease</strong><br />
- COPD<br />
- <strong>in</strong>terstitial lung <strong>disease</strong><br />
- sleep disordered breath<strong>in</strong>g<br />
- developmental abnormalities<br />
4. PH due to chronic thrombotic<br />
<strong>and</strong>/or embolic <strong>disease</strong><br />
- proximal CTEPH<br />
- distal CTEPH<br />
- non-thrombotic embolism<br />
5. Miscellaneous<br />
- sarcoid<br />
- PLCH, LAM<br />
- metabolic disorders<br />
Adapted from Dana Po<strong>in</strong>t Classification 2008
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