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Advanced Therapy in Eisenmenger Syndrome: A Systematic Review

Hjortshøj, Cristel S. MD, PhD; Jensen, Annette S. MD, PhD; Søndergaard, Lars DMSc

doi: 10.1097/CRD.0000000000000107
Review Articles

Treatment options for patients with the Eisenmenger syndrome have until recently been scarce, but new knowledge in the field of pulmonary arterial hypertension has expanded the therapeutic possibilities for these patients. Advanced therapy with pulmonary vasodilators has become part of the standard treatment, offering long-term benefits on exercise capacity, clinical symptoms, and possibly survival. However, there are currently only few studies to guide the use of advanced therapies in this population, and important questions such as indications for initiation or escalation of advanced therapy and valid effect parameters and treatment goals remain unanswered. This review covers the pharmacology, therapeutic options, risk stratification, and treatment strategy of pulmonary arterial hypertension-specific drugs in patients with Eisenmenger syndrome.

From the Department of Cardiology, Rigshospitalet, Copenhagen, Denmark.

Disclosure: Dr Hjortshøj received an educational grant from Actelion Pharmaceuticals. Dr Jensen received a research grant and speaker’s fees from Actelion Pharmaceuticals and Pfizer. Dr Søndergaard received a research grant and speaker’s fees from Actelion pharmaceuticals.

Correspondence: Cristel S. Hjortshøj, MD, Department of Cardiology, 2013, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark. E-mail: cristel.maria.soerensen.hjortshoej@regionh.dk.

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