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Original research article
Fenestration in the Fontan circulation as a strategy for chronic cardioprotection
  1. Hirofumi Saiki1,
  2. Seiko Kuwata1,
  3. Yoichi Iwamoto2,
  4. Hirotaka Ishido2,
  5. Mio Taketazu3,
  6. Satoshi Masutani2,
  7. Takashi Nishida1,
  8. Hideaki Senzaki1
  1. 1 Department of Pediatrics and Pediatric Cardiology, Kitasato University School of Medicine, Sagamihara, Japan
  2. 2 Department of Pediatric Cardiology, Saitama Medical University, Kawagoe, Japan
  3. 3 Department of Pediatrics, Hokkaido Ryoiku-en, Asahikawa, Japan
  1. Correspondence to Professor Hideaki Senzaki, Department of Pediatrics and Pediatric Cardiology, Kitasato University School of Medicine, Sagamihara 350-1298, Japan; hsenzaki{at}


Background Fenestration in the Fontan circulation potentially liberates patients from factors leading to cardiovascular remodelling, through stable haemodynamics with attenuated venous congestion. We hypothesised that a fenestrated Fontan procedure would possess chronic haemodynamic advantages beyond the preload preservation.

Methods We enrolled 35 patients with fenestrated Fontan with a constructed pressure–volume relationship under dobutamine (DOB) infusion and/or transient fenestration occlusion (TFO). Despite the use of antiplatelets and anticoagulants, natural closure of fenestration was confirmed in 11 patients. Cardiovascular properties in patients with patent fenestration (P-F) were compared with those in patients with naturally closed fenestration (NC-F). To further delineate the roles of fenestration, paired analysis in patients with P-F was performed under DOB or rapid atrial pacing with/without TFO.

Results As compared with P-F, patients with NC-F had a higher heart rate (HR), smaller ventricular end-diastolic area, better ejection fraction and higher central venous pressure, with higher pulmonary resistance. While this was similarly observed after DOB infusion, DOB markedly augmented diastolic and systolic ventricular stiffness in patients with NC-F compared with patients with P-F. As a mirror image of the relationship between patients with P-F and NC-F, TFO markedly reduced preload, suppressed cardiac output, and augmented afterload and diastolic stiffness. Importantly, rapid atrial pacing compromised these haemodynamic advantages of fenestration.

Conclusions As compared with patients with NC-F, patients with P-F had robust haemodynamics with secured preload reserve, reduced afterload and a suppressed beta-adrenergic response, along with a lower HR at baseline, although these advantages had been overshadowed, or worsened, by an increased HR.

  • Fontan physiology
  • heart failure
  • congenital heart disease
  • congenital heart surgery

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  • Contributors Conception or design of the work. HSa, SK and HSe. Data collection: HSa, SK, YI, HI, MT, SM, TN and HSe. Data analysis and interpretation: HSa, SM and HSe. Drafting the article. HSa. Critical revision of the article: HSa, SK, YI, HI, MT, SM, TN and HSe. Final approval of the version to be published: HSa, SK, YI, HI, MT, SM, TN and HSe.

  • Funding Grant from Kawano Memorial Foundation.

  • Competing interests None declared.

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

  • Patient consent for publication Not required.