Abstract

The treatment algorithm that has recently been developed at the 4th World Conference on Pulmonary Hypertension (PH) in Dana Point will contain a number of important innovations for patients with pulmonary arterial hypertension (PAH), but also for those with other forms of PH. In PAH patients, a targeted therapy with ERA or PDE5 inhibitors is now recommended for patients in functional class II. Combination therapy (ERA and/or PDE5i and/or prostanoids) is proposed if the clinical response to monotherapy is not adequate. In addition, supervised training programs are recommended for patients with PAH. For other forms of PH such as PH due to left heart disease or chronic lung disease, it remains valid that the underlying disease should be treated as efficiently as possible. However, a targeted PAH therapy may be beneficial in selected patients with "out of proportion PH", but these treatments should exclusively be initiated in expert centers. Pulmonary endarterectomy (PEA) remains the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). If patients are inoperable and/or if surgical treatment is not an option, targeted PAH therapy may be considered, but these patients should at present be included into clinical trials. Since there is currently no cure for PH/PAH, further development and progress in medical treatment are highly desirable. A number of promising novel compounds are currently under investigation. These include sGC stimulators, tyrosine kinase inhibitors, and serotonin antagonists.

Full Text
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