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Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies
  1. Maurice Beghetti
  1. Pediatric Cardiology Unit, University Hospital of Geneva, Geneva, Switzerland
  1. Correspondence to Professor Maurice Beghetti, Unité de Cardiologie Pédiatrique, Département de L'Enfant et de L'Adolescent, Hôpital des Enfants, 6 rue Willy Donzé, 1211 Genève 14, Switzerland; maurice.beghetti{at}


In recent decades, the Fontan procedure and its variations have resulted in markedly improved outcomes of patients with single-ventricle physiology. These improvements are expected to increase greatly the number of surviving patients, particularly those surviving long into adulthood. However, there is still a progressive risk of attrition and failure of the Fontan circulation over time, the underlying pathophysiology of which is not fully understood. Current evidence suggests that alterations in the structure and function of the pulmonary vasculature may play a pivotal role. Recent evidence suggests that therapies approved for pulmonary arterial hypertension may provide benefits in this increasingly important patient population.

  • Fontan procedure
  • paediatric surgery
  • pathology
  • pulmonary heart disease

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  • Competing interests MB has served on advisory boards/consulting for Pfizer, Actelion Pharmaceuticals, Bayer Schering, Encysive Pharmaceuticals, GlaxoSmithKline, INO Therapeutics, Eli Lilly and Mondobiotech and has received lecture fees from Actelion Pharmaceuticals, Encysive Pharmaceuticals and Bayer Schering.

  • Provenance and peer review Not commissioned; externally peer reviewed.