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Survival in Eisenmenger syndrome: a paradigm shift in outcomes research for adult congenital heart disease?
  1. Ami B Bhatt
  1. Correspondence to Dr Ami B Bhatt, Department of Cardiology, Massachusetts General Hospital, Adult Congenital Heart Disease Program, Boston, MA 02114, USA; ABHATT{at}mgh.harvard.edu

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In 1958, Paul Wood defined Eisenmenger syndrome as ‘Pulmonary hypertension due to a high pulmonary vascular resistance with reversed or bidirectional shunt at aortopulmonary, ventricular, or atrial level.’ He went on to state ‘As life advances, usually between the ages of 20 and 30, thrombo-obstructive lesions develop in the larger pulmonary arteries which increase the resistance considerably and herald the beginning of the downhill course.’1

As the field of congenital heart disease (CHD) progressed, and individuals lived beyond the second and third decades of life, a concept was born which has been clung to by physicians and patients alike … Eisenmenger syndrome portends a better prognosis than other causes of pulmonary hypertension, and less is more.

In the article, ‘Survival Prospects of Treatment Naive Eisenmenger Patients—A Systematic Review of the Literature and Report of Own Experience’,2 our colleagues, including those from the Brompton, offer a modern-day treatise on the untreated Eisenmenger patient and a new lens through which we should assess this fatal disease.

Eisenmenger syndrome is a severe form of pulmonary arterial hypertension and arises in CHD with a systemic-to-pulmonary shunt. Individuals with Eisenmenger syndrome have multisystem involvement as a result of chronic hypoxaemia with renal and liver dysfunction, coagulation disorders, neurologic complications, heart failure, arrhythmias, and often sudden and premature death. Diller et al challenged the premise that high levels of pulmonary vascular resistance, nearly lifelong cyanosis, and multisystem organ failure portend a better prognosis. They also astutely noted that the Eisenmenger population's outcomes have not considerably improved since the 1960s.

During much of this time, treatment was symptom guided (for reduced exercise tolerance, increasing cyanosis or heart failure presentation), and stable ‘asymptomatic’ patients were not treated. Lung or heart-lung transplantation remained a last resort for the hospital-bound individual. With the advent of selective pulmonary arterial vasodilators, an …

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