Pulmonary arterial hypertension PAH

1.1 Primary pulmonary hypertension a) Sporadic disorder b) Familial disorder

1.2 Associated with a) Collagen vascular disease b) Congenital (right-left) systemic-pulmonary shunt c) Portal hypertension d) HIV-associated pulmonary hypertension e) Drugs f) Persisting PAH of the newborn

Pulmonary hypertension is classified into three clinical stages: Latent pulmonary hypertension is characterized when mean pulmonary arterial pressures (PAP) are below 21 mmHg with an exercise-induced increase to values above 30 mmHg. The patients suffer from dyspnea upon exercise. In manifested pulmonary hypertension, the mean PAP exceeds 25 mmHg at rest. Patients already suffer from dyspnea on light exercise. Severe pulmonary hypertension is characterized by a severely reduced cardiac output at rest, which cannot be increased upon exercise, due to the increase in right ventricular afterload. Thus, patients are unable to perform any physical activity without distress.

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