[Pulmonary hypertension in lung diseases].

Pulmonary hypertension (PH) eventually occurs during the course of obstructive as well as restrictive lung diseases, with highly variable prevalences between the different entities. Early detection of PH in patients with manifest lung diseases is difficult as PH and lung disease share most clinical symptoms in common. Diagnostic hints may come from clinical symptoms - out of proportion dyspnea and disability as well as exaggerated hypoxemia with hyperventilation - radiologic, ECG, echocardiographic findings, lung function test results, and laboratory parameters such as BNP and NT-proBNP are potential indicators of PH. In any case, right heart catheter is obligatory to make a firm diagnosis of PH. Optimal therapy of the underlying disease including long-term oxygen therapy is the mainstay of treatment. In eligible patients lung transplantation is a potentially life saving procedure. Specific PAH medications are so far not approved for the use in PH associated with lung diseases. Based on pathophysiological similarities with PAH and successful case reports specific PAH therapies may be employed "off-label" in an individual case by case decision. Adequately designed and powered clinical trials are urgently needed to confirm the role of PH specific therapies in patients with lung disease associated PH.