title: Individualized total cavopulmonary connection technique for patients with asplenia syndrome.
abstract: BACKGROUND
  Outcomes after univentricular repair for patients with asplenia syndrome remain unsatisfactory, not only because of clinical difficulties in patient selection, but also secondary to technical difficulties in the separation of the systemic and pulmonary circulations, particularly with the rerouting technique for the inferior systemic veins.
  
  
  METHODS
  Between February 1995 and May 2000, a total of 14 consecutive patients with asplenia syndrome underwent bidirectional cavopulmonary connection with obliteration of additional pulmonary blood flow, followed by a total cavopulmonary connection. The rerouting technique for inferior systemic venous blood flow was individualized to optimize laminar nonturbulent flow characteristics in the pathway, and to minimize prosthetic load and suture load on the atrial wall. The lateral tunnel or tube conduit technique was used in an extraatrial, intra-extraatrial, or intraatrial fashion. No fenestration was applied.
  
  
  RESULTS
  No hospital mortality was observed. Systemic venous flow was evaluated using magnetic resonance angiography, revealing no signs of obstruction, turbulence, or stasis either in or near the reconstructed pathways, irrespective of the rerouting technique. Postoperative catheterization revealed favorable hemodynamics including an inferior vena cava pressure of 13 +/- 2 mm Hg and arterial oxygen saturation of 93.4% +/- 3.5% at room air. All patients have remained free of symptoms, although 1 patient died of acute septic complications 3.5 years after the procedure.
  
  
  CONCLUSIONS
  The complexity of cardiac anomalies in asplenia syndrome warrants individualization of the total cavopulmonary connection technique used in reconstruction of the inferior systemic venous pathway. Optimizing flow characteristics in the pathway should be a priority. A staging approach allows suitable selection of candidates for univentricular repair.
authors: Division of Cardiovascular Surgery, Keio University, Tokyo, Japan. aeba@sc.itc.keio.ac.jp
authors: Ryo
authors: R
authors: Aeba
authors: Toshiyuki
authors: T
authors: Katogi
authors: Kenichi
authors: K
authors: Hashizume
authors: Yoshimi
authors: Y
authors: Iino
authors: Shiaki
authors: S
authors: Kawada
authors: Yuji
authors: Y
authors: Yuasa
mesh_terms: D000015:Abnormalities, Multiple; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D018610:Heart Bypass, Right / Q000379:methods*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007223:Infant; D011183:Postoperative Complications; D012189:Retrospective Studies; D013154:Spleen / Q000002:abnormalities*; D013577:Syndrome