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

OBJECTIVESData 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).METHODSFrom September 1999 to August 2012, 139 patients with a FSV underwen...

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Published in:Interactive cardiovascular and thoracic surgery 2017-05, Vol.24 (5), p.747-754
Main Authors: Polimenakos, Anastasios C, Subramanian, Sujata, ElZein, Chawki, Ilbawi, Michel N
Format: Article
Language:English
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Summary:OBJECTIVESData 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).METHODSFrom 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.RESULTSThe 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 range 25-75, 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).CONCLUSIONSA 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.
ISSN:1569-9293
1569-9285
DOI:10.1093/icvts/ivw413