Pulmonale Hypertonie: Diagnostik, Klassifikation und Therapie

Zusammenfassung

Bei der pulmonalen Hypertonie handelt es sich um eine Druckerhöhung in der Lungenstrombahn, dem sog. kleinen Kreislauf: Der pulmonalarterielle Mitteldruck (Messung im Rechtsherzkatheter) ist auf über 25 mmHg erhöht. Für die Prognose der meist stark beeinträchtigten Patienten ist eine frühe Diagnosestellung extrem wichtig. Der Beitrag widmet sich der Diagnostik, der klinischen Klassifikation und der gruppenspezifischen Therapie der pulmonalen Hypertonie.

Abstract

Pulmonary hypertension is a chronic, incurable disease with poor prognosis. The therapeutic aim is a stabilization of patients showing signs of right heart failure as well as disease progression. A pulmonary hypertension is diagnosed in patients displaying a mean pulmonary arterial pressure of > 25 mmHg in resting state. Invasively measured hemodynamics evaluated by right heart catheterization (mean pulmonary arterial pressure [mPAP], pulmonary arterial wedge pressure [PAWP], diastolic pressure gradient [DPG] and pulmonary vascular resistance [PVR]) allows to differentiate between pre-capillary, post-capillary and combined pulmonary hypertension, which constitutes the basis for classification. Diagnostics and therapy shall occur within a center of expertise. Currently, 10 medications belonging to 5 substance classes are approved. Combination therapy should be introduced early. In accordance with risk stratification, therapy is oriented towards estimated 1-year survival as opposed to single target values. If pulmonary hypertension is associated with left heart disease (group 2) or lung disease (group 3), optimal care of the primary disease should be paramount. These associations make up for a greater proportion of patients than idiopathic pulmonary arterial hypertension (PAH). In isolated cases, patients of group 2 may be treated in centers of expertise within the scope of medical studies. Patients with PAH may be categorized into typical versus atypical PAH. For patients with atypical PAH, an initial monotherapy is to be introduced. In case of chronic thromboembolic pulmonary hypertension, the possibility of an operative pulmonary endarterectomy should be evaluated. To date, the only approved drug is Riociguat, a stimulator of the soluble guanylate cyclase.

Schlüsselwörter

pulmonale Hypertonie - pulmonalarterielle Hypertonie - medikamentöse Therapie - Risikostratifizierung

Key words

pulmonary hypertension - pulmonary arterial hypertension - medical therapy - risk stratification

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