Treatment of adults with Eisenmenger syndrome-state of the art in the 21st century: a short overview.

Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. In developed countries, cardiac defects are being operated on in a timely manner, before pulmonary vascular disease develops. However, with rising immigration from underserved countries, we increasingly see patients with shunt lesions, that are not amenable for repair as pulmonary vascular disease has already established. ES describes a symptom complex and patients present with heterogeneous problems involving many organ systems (multisystem disorder). Care in tertiary specialist cardiac centers with access to multidisciplinary subspecialities is required. Central cyanosis with secondary erythrocytosis is one of the key features of patients with ES. Clinical consequences of longstanding hypoxia can lead to other organ complications, that involve other organs than the heart alone. Although ES patients have a better prognosis compared to other patients with pulmonary arterial hypertension, ES grossly affects quality of life and morbidity is frequent. Follow-up and care at specialist congenital heart disease centers is highly recommended to prevent, to early diagnose and to timely manage complications of ES. This is necessary to maintain functional capacity, decrease morbidity and increase life expectancy for these vulnerable patients. The leading reasons for mortality are sudden cardiac death, progressive heart failure, and infectious diseases. Various factors have been shown to be associated with mortality like decreased arterial oxygen saturation, functional class, impaired exercise tolerance, syncopal events, iron deficiency, presence of pre-tricuspid shunts, arrhythmias, increased (NT-pro) brain natriuretic peptide, echocardiographic variables of right ventricular dysfunction and hospitalization for heart failure. Although to date there is no causal therapy to reverse pulmonary vascular disease, a greater armamentarium of targeted therapies is available, which have been shown to be beneficial in patients with ES.

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2021 Cardiovascular Diagnosis and Therapy. All rights reserved.

Déclaration de conflit d'intérêts

Conflicts of Interest: The authors have completed the ICMJE uniform disclosure form (available at https://dx.doi.org/10.21037/cdt-21-135). The series “Pediatric Pulmonary Hypertension” was commissioned by the editorial office without any funding or sponsorship. Drs. Lammers served as the unpaid Guest Editor of the series. Drs. Diller and Lammers both report support from Actelion Germany/Global and Johnson and Johnson. The authors have no other conflicts of interest to declare.