7th May 2025

Eisenmenger Syndrome

This rare syndrome results from a congenital heart defect and a large shunt, which impacts both the heart and lungs. Initially, there is an acyanotic left-to-right shunt, which leads to increased vascular resistance and severe pulmonary arterial hypertension. Over time, the increased pulmonary resistance eventually reverses the blood flow, resulting in a cyanotic right-to-left shunt. A summary of the key features is detailed in Table Three.

Feature	Summary
Epidemiology	A rare condition typically presents in areas with poor access to healthcare resulting in late presentation.
Aetiology	It can develop from any CHD where pulmonary arterial hypertension results. For example, ASD, ventricular septal defects, patent ductus arteriosus, and unrepaired tetralogy of Fallot.
Clinical History	A patient with known CHD presenting with worsening exertional dyspnoea Other symptoms can include palpitations, haemoptysis, syncope, progressive cyanosis and swelling. Also, due to chronic hypoxaemia, the patient can develop symptoms related to polycythaemia. These include headache, dizziness, stroke and visual changes.
Clinical Examination	Signs of pulmonary hypertension Right ventricular heave – forceful contracture against increased pulmonary pressures Raised jugular venous pressure Peripheral oedema Loud Second Heart Sound – due to forceful pulmonary valve closure Signs related to the underlying CHD ASD: mid-systolic, crescendo-decrescendo murmur, loudest at the upper left sternal edge VSD: pan-systolic murmur, loudest at the level of left lower sternal border PDA: “machinery” murmur, continuous crescendo-decrescendo Signs of right-to-left shunt and chronic hypoxaemia Dyspnoea Clubbing Cyanosis Plethoric complexion (secondary to polycythaemia)
Diagnostic Investigations	Echocardiogram ECG Chest X-ray Bloods, including full blood count and iron studies
Management	The ideal management is preventative, i.e. correcting the potentially causative CHD prior to the development of Eisenmenger syndrome. Once Eisenmenger syndrome has developed, reversal of the high pulmonary arterial pressure is not possible, and a cardiopulmonary transplant is needed. This is not always possible and is associated with high mortality. Supportive treatment options can include: Vasodilators for pulmonary hypertension Venesection for polycythaemia Anticoagulation to reduce thrombus risk Treatment of any resulting arrhythmias
Follow Up	Patients with Eisenmenger have a reduced life expectancy and high mortality in the 3rd/4th decade of life. The most common cause of death is stroke, haemoptysis, ventricular failure and pregnancy complications. Management is required from a multidisciplinary team, including cardiologists.

Table 3: This table describes the key areas of Eisenmenger Syndrome. Sourced and adapted from Basit et al, Eisenmenger Syndrome: StatPearls Publishing; 2023 and Watchman, Eisenmenger Syndrome: Zero to Finals; 2019.

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