Ambrisentan Improves Exercise Capacity and Symptoms in Patients with Portopulmonary Hypertension

Zusammenfassung

Einleitung: Ambrisentan, ein selektiver Endothelinrezeptor-Antagonist, ist in vielen Ländern zur Therapie der pulmonal-arteriellen Hypertonie zugelassen. Daten, die eine verbesserte Belastbarkeit bei Patienten mit einer portopulmonalen Hypertonie (PoPH) zeigen, wurden bisher nicht publiziert. Patienten und Methoden: Wir untersuchten retrospektiv die Sicherheit und Effektivität von Ambrisentan bei Patienten mit einer PoPH. Die Untersuchung erfolgte an 4 deutschen Universitätskliniken. Ergebnisse: 14 Patienten mit einer moderaten bis schweren PoPH wurden eingeschlossen. Die mediane Beobachtungszeit betrug 16 Monate (IQR, 12 – 21). Nach 6 und 12 Monaten stieg die Gehstrecke im 6-Minuten-Gehtest signifikant von 376 Meter (IQR, 207 – 440) vor Therapiebeginn auf 415 Meter (IQR, 393 – 475; p = 0,011) bzw. auf 413 Meter (IQR, 362 – 473, p = 0,005) an. Die WHO-Funktionsklasse verbesserte sich ebenfalls signifikant (p = 0,014). Die Blutgasanalysen und die Leber-Funktionstests (Aspartat-Aminotransferase, Alanin-Aminotransferase, Total-Bilirubin, International Normalized Ratio) wurden nicht signifikant durch die Ambrisentantherapie beinflusst. Schlussfolgerung: Die vorliegende Studie zeigt eine Verbesserung der Belastbarkeit und der Symptomatik unter Therapie mit Ambrisentan bei Patienten mit einer PoPH ohne Hinweise auf relevante Nebenwirkungen.

Abstract

Introduction: Ambrisentan, a selective endothelin receptor antagonist has been approved in several countries for pulmonary arterial hypertension. No data have been published on the efficacy of ambrisentan on improvement of exercise capacity in patients with portopulmonary hypertension (PoPH). Patients and Methods: We retrospectively analyzed the safety and efficacy of ambrisentan in patients with PoPH in four German university hospitals. Results: 14 patients with moderate to severe PoPH were included. The median follow-up was 16 months (IQR, 12 – 21). 6 minute walk tests after 6 and 12 months improved from 376 meters (IQR, 207 – 440) at baseline to 415 meters (IQR, 393 – 475; p = 0.011) and 413 meters (IQR, 362 – 473, p = 0.005), respectively. WHO- functional class after 1 year of therapy with ambrisentan also improved significantly (p = 0.014). No significant changes in blood gas analysis and liver function tests (aspartate aminotransferase, alanine aminotransferase, total bilirubin, and international normalized ratio) during therapy with ambrisentan were detectable. Conclusions: The present study demonstrates significant improvement of exercise capacity and clinical symptoms without relevant safety concerns during ambrisentan treatment in patients with PoPH.

Schlüsselwörter

Ambrisentan - Leberzirrhose - portopulmonale Hypertonie - pulmonale Hypertonie

Key words

ambrisentan - cirrhosis - portopulmonary hypertension - pulmonary hypertension

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