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The pulmonary vasculature: Achilles heel of the Fontan circulation
  1. Floris-Jan S Ridderbos1,
  2. Tjark Ebels2,3,
  3. Rolf M F Berger1
  1. 1 Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children’s Hospital, University Medical Center Groningen, Groningen, The Netherlands
  2. 2 Center for Congenital Heart Diseases, Department of Cardiothoracic Surgery, University Medical Center Groningen, Groningen, The Netherlands
  3. 3 Department of Cardiothoracic Surgery, University Medical Center Amsterdam, Amsterdam, The Netherlands
  1. Correspondence to Mr Floris-Jan S Ridderbos, Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, Groningen 9700 RB, The Netherlands; f.j.s.ridderbos{at}umcg.nl

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The Authors’ reply:

We thank Yalta and colleagues1 for their comments concerning our article reporting the relation between pulmonary artery size and functional capacity in patients with a Fontan circulation, recently published in this Journal.2 The emerging paradigm in Fontan physiology considers the pulmonary vasculature a key determinant of cardiac output.3 In the absence of obstructions in the cavo-pulmonary connection, both proximal pulmonary arteries and pulmonary vascular bed, in the absence of a subpulmonary pump, may represent a bottleneck limiting transpulmonary flow, and thus preload of the single ventricle and ultimately cardiac output.

In normal physiology, pulmonary vascular resistance is predominantly determined by the pulmonary vascular bed, whereas pulsatile load formed by the proximal elastic conduit arteries is minimal. However, in the Fontan circulation, the proximal elastic pulmonary arteries may significantly contribute to the total pulmonary resistance due to hypoplasia as a consequence of reduced flow conditions and abnormal growth, both prenatally …

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