Attrition in patients with single ventricle and trisomy 21: Outcomes after a total cavopulmonary connection

Anastasios C. Polimenakos, Sujata Subramanian, Chawki ElZein, Michel N. Ilbawi

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27.6 ± 12.1 months. RESULTS: The initial procedure was pulmonary artery banding in 9 patients, systemic-To-pulmonary shunt in 2 and Damus-Kaye-Stansel/ Norwood procedure in 2. Median follow-up was 69 months (interquartile range25-75, 21-99). There was 1 death after a Damus-Kaye-Stansel/Norwood procedure and one interstage death after a bidirectional Glenn procedure. Nine (of 11) survivors underwent a Fontan operation. A fenestrated Fontan procedure was the predominate operation in 78%. One patient was deemed unsuitable for a Fontan operation. There was 1 takedown and 1 late death after the Fontan operation. Heterotaxy syndrome did not affect outcome (P > 0.05). There was no statistical difference in the pre-Fontan McGoon ratio, hospital length of stay, duration of pleural drainage and Fontan-related adverse events between patients with a dominant right ventricle and those with a left (P > 0.05). CONCLUSIONS: A TCPC in patients with T21 and an FSV is associated with reproducible, satisfactory outcomes. An assisted-Glenn procedure with pulsatile pulmonary blood flow and a fenestrated Fontan may be associated with attenuated perioperative morbidity and late attrition.

Original languageEnglish (US)
Pages (from-to)747-754
Number of pages8
JournalInteractive Cardiovascular and Thoracic Surgery
Issue number5
StatePublished - May 1 2017
Externally publishedYes


  • Fontan operation
  • Single ventricle
  • Trisomy 21

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine


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