title: Fontan circulation over 30 years. What should we learn from those patients?
abstract: BACKGROUND
  This study aimed to evaluate Fontan circulation which was observed over 30 years.
  
  
  METHODS
  Forty patients who underwent a Fontan operation between 1974 and 1986 and survived (group S, n = 20) or died in the late period (group LD, n = 20) were evaluated. The median age at operation was 10 years (range 2-32 years). The diagnoses were tricuspid atresia in 21, single ventricle in 9, and others in 10. The Fontan procedure was a right atrium-pulmonary artery graft in 2, atriopulmonary connection in 28, and right atrium-right ventricle anastomosis in 10 (Björk in 4, graft in 6).
  
  
  RESULTS
  Causes of late death were congestive heart failure in 6, sudden death in 4, arrhythmia in 4, and others in 6. Sixteen patients underwent reoperation 23.3 ± 6.5 years after Fontan. Cardiac catheterization was performed at 1 month and 12 years. There were no significant differences in central venous pressure, ventricular ejection fraction, or pulmonary vascular resistance between the two groups. However, changes in ventricular end-diastolic volume suggested volume and pressure overload in group LD. In group S, the latest chest radiographs showed cardiothoracic ratio 51.3% ± 7.6%, oxygen saturation 94% ± 2%, brain natriuretic peptide 153 ± 111 pg ċ mL-1, and Holter monitoring revealed sinus rhythm in 10 patients. New York Heart Association class was I in 12 patients, II in 6, and III in 2.
  
  
  CONCLUSIONS
  For long-term Fontan survival, timely total cavopulmonary connection conversion and medication to decrease ventricular volume and pressure load might be important.
authors: Division of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan takasakamoto@yahoo.co.jp.|Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
authors: Takahiko
authors: T
authors: Sakamoto
authors: Division of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.|Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
authors: Mitsugi
authors: M
authors: Nagashima
authors: Division of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.|Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
authors: Takeshi
authors: T
authors: Hiramatsu
authors: Division of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.|Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
authors: Goki
authors: G
authors: Matsumura
authors: Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
authors: In-Sam
authors: IS
authors: Park
authors: Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
authors: Kenji
authors: K
authors: Yamazaki
mesh_terms: D000293:Adolescent; D000328:Adult; D002317:Cardiovascular Agents / Q000627:therapeutic use; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006439:Hemodynamics; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality / Q000601:surgery; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D002317:Cardiovascular Agents
keywords: Fontan procedure; Heart defects; Heart ventricles; Protein-losing enteropathies; Pulmonary artery; Treatment outcome; congenital