Results of staged palliation for hypoplastic left heart syndrome: a complete population-based series.
OBJECTIVE
To study the outcome of staged palliation for classic hypoplastic left heart syndrome.


METHODS
Retrospective chart review. Risk factors for mortality were analysed using Cox's proportional hazard regression modelling.


RESULTS
From 1993 to 2004, 55 infants underwent Norwood stage I procedure at a median age of 8 d (range 1-19 d). Hospital survival was 39/55 (71%), and there were six late deaths (before stage II). Birthweight, circulatory arrest time and cardiopulmonary bypass time were independent risk factors for stage I hospital mortality (p=0.029, p=0.001 and p=0.003, respectively). Poor right ventricular function prior to stage I was a significant predictor for interstage mortality (p=0.02). Thirty-two patients underwent bidirectional cavopulmonary anastomosis, at a median age of 6.5 mo (range 2.0-9.5 mo), with seven late deaths. Two patients had a heart transplant after stage II. Total cavopulmonary connection (TCPC) was performed in 13 patients, at a median age of 33 mo (range 21-45 mo), without mortality. Kaplan-Meier survival was 58%, 52% and 45% at 6, 12 and 48 mo, respectively.


CONCLUSIONS
Low birthweight, long time on circulatory arrest and cardiopulmonary bypass were risk factors for stage I mortality. Poor right ventricular function was detrimental to intermediate outcome.
Nilsson
Boris
B
Department of Cardiothoracic Surgery, Sahlgrenska University Hospital/Queen Silvia Children's Hospital, Göteborg, Sweden. boris.nilsson@vgregion.se
Mellander
Mats
M
Südow
Göran
G
Berggren
Håkan
H
D002675:Child, Preschool; D001026:Coronary Artery Bypass; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality* / Q000473:pathology / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D011182:Postoperative Care; D011300:Preoperative Care; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis