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Declining incidence and prevalence of Eisenmenger syndrome in the developed world: a triumph of modern medicine
  1. Aleksander Kempny1,2,
  2. Konstantinos Dimopoulos1,2,
  3. Michael A Gatzoulis1,2
  1. 1 Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK
  2. 2 National Heart and Lung Institute, Imperial College School of Medicine, London, UK
  1. Correspondence to Dr Aleksander Kempny, Adult Congenital Heart Centre, Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, UK; a.kempny{at}

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In 1897, Dr Victor Eisenmenger described a 32-year-old man with a history of cyanosis and progressive exertional breathlessness since childhood. The patient developed heart failure in his 30s and, thereafter, died suddenly following massive haemoptysis. His postmortem showed a large ventricular septal defect (VSD). However, the haemodynamic and pathophysiological significance of large intracardiac defects remained unclear until the advent of cardiac catheterisation. It was not until the late 1950s, when Paul Wood in his seminal work elegantly described the flow and pressure overload to the pulmonary circulation caused by large cardiac defects, resulting in a gradual increase in pulmonary vascular resistance and, over time, shunt reversal leading to cyanosis. Paul Wood also recognised that Eisenmenger syndrome (ES) may develop in patients with an isolated, large VSD and also in those with a large patent ductus arteriosus, atrioventricular septal defect or even an isolated atrial septal defect.1

In ES, the severe pulmonary arterial hypertension (PAH), low cardiac output and chronic cyanosis result in a systemic disease with multiorgan involvement, increased morbidity and mortality and poor quality of life.2 3 There is compensatory secondary erythrocytosis and a predisposition to bleeding and thrombosis, the latter often manifesting as in situ thrombosis within dilated central pulmonary arteries. Patients often suffer from recurrent haemoptysis, which is a common cause of hospitalisation, but not the leading cause of death in contemporary cohorts with ES. Furthermore, there is increased risk of infections, including endocarditis and intracranial abscess. Older patients with ES may develop progressive biventricular dysfunction, valve insufficiency and are prone to supraventricular arrhythmias. Severe exercise intolerance is prevalent, and patients …

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  • Funding AK, KD and MAG have received unrestricted educational or research grants from Bayer UK, Pfizer UK, Actelion UK and Actelion Global. Furthermore, they have received support from the Pulmonary Hypertension Association (UK) and the British Heart Foundation.

  • Competing interests we have provided information regarding disclosures in the submitted manuscript. Please copy from the ‘Disclosures’ in the submitted manuscript.

  • Provenance and peer review Commissioned; internally peer reviewed.

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