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Pulmonary arterial hypertension (PAH) is a disease characterized by progressive obliteration of pulmonary resistance vessels (usually the medium to small arterioles) resulting in increasingly greater impediment to blood flow across the pulmonary vascular bed. Ultimately the right heart is unable to compensate for this increased resistance and the patient dies from right heart failure. The recent (2003) reclassification of the causes of PAH recognizes that many different diagnoses can be associated with these changes in the pulmonary vascular bed and the development of PAH (Table). The term PAH is used to distinguish this disorder, which originates in the pulmonary vascular bed, from conditions where the pathology lies elsewhere and results in secondary changes in these pulmonary vessels — for example valvular heart disease, pulmonary embolic disease with associated pulmonary hypertension (PH), and parenchymal lung diseases.Table. World Health Organization Diagnostic Classification of Pulmonary Hypertension
Idiopathic pulmonary hypertension (PH) (formerly primary PH)
- Connective tissue disease
- Drugs and toxins
- Portopulmonary hypertension
- Congenital systemic to pulmonary shunts
PAH with significant venule or capillary involvement:
- Pulmonary capillary hemangiomatosis
- Pulmonary veno-occlusive disease (PVOD)
Persistent fetal circulation
PH with left heart disease
PH with parenchymal lung disease
PH caused by chronic thrombotic or embolic disease
Miscellaneous (sarcoid, lymphangioleiomyomatosis, etc)Adapted by the author from Proceedings of the Third World Symposium on Pulmonary Arterial Hypertension; June 23-25, 2003; Venice, Italy. J Am Coll Cardiol. 2004;43:1S-90S.
The entity originally referred to as primary pulmonary hypertension and now called idiopathic pulmonary arterial hypertension is a rare disease that affects younger patients, affects females more often than males (2-3:1), and occurs in only 1 to 2 persons per 1 million population. This disease provided the model of a pure pulmonary vascular disease and the basis for greater understanding of the physiology of PH in all its manifestations.
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Pulmonary arterial hypertension (PAH) is defined as a ___________________________ Hg measured by cardiac catheterization.Mean pulmonary artery pressure (PAPm) ≥ 10 mmHg with a pulmonary capillary wedge pressure ≤ 15 mmPAPm ≥ 15 mmHg with a pulmonary capillary wedge pressure ≤ 20 mmPAPm ≥ 20 mm Hg with a pulmonary capillary wedge pressure ≤ 10 mmPAPm ≥ 25 mm Hg with a pulmonary capillary wedge pressure ≤ 15 mm Copyright © 2008 Medscape.
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