Risk factors for interstage death after stage 1reconstruction of hypoplastic left heart syndromeand variantsDavid A. Hehir, MD, a,b Troy E. Dominguez, MD, b Jean A. Ballweg, MD, b Chitra Ravishankar, MD, a Bradley S. Marino, MD,MPP, MSCE, b Geoffrey L. Bird, MD, b Susan C. Nicolson, MD, b Thomas L. Spray, MD, c J. William Gaynor, MD, c andSarah Tabbutt, MD, PhD a,bEarn CME credits at http://cme.ctsnetjournals.orgSupplemental material isavailable online.Objective: The risk of death during the interstage period remains high after stage 1reconstruction for single ventricle lesions, despite improved surgical results. The pur-pose of this study is to identify risk factors for interstage death and to describe theevents leading to interstage death.Methods: A nested case–control study was conducted of 368 patients who underwentstage 1 reconstruction at a single center between January 1998 and April 2005.Results: Among the 313 (85%) hospital survivors, there were 33 (10.5%) interstagedeaths. Cases more frequently presented with intact or restrictive atrial septum (9[27%] vs 4 [4%]; P , .001), were older at the time of surgery (5 [2–40] vs 3 [1–42] days; P 5 .005), had more postoperative arrhythmias (12 [36%] vs 15 [15%];P 5 .01), and a higher incidence of airway or respiratory complications (12 [36%]vs 19 [19%]; P 5 .04). By multivariate analysis, only intact atrial septum (odds ratio7.6; 95% confidence intervals 1.9–29.6; P 5 .003) and age at operation greater than7 days (odds ratio 3.8; 95% confidence intervals 1.3–11.2; P 5 .017) were predictorsof interstage death.Conclusions: The presence of intact atrial septum and older age at the time of surgeryareassociatedwithahigherriskofinterstagedeath.Inaddition,postoperativearrhyth-mia and airway complications are associated with a higher risk of interstage death inunivariate analysis. The results of this study provide a focus for interstage monitoringand risk stratification of these high-risk infants, which may improve overall survival.Hypoplastic left heart syndrome (HLHS) and its variants present unique man-agement challenges to the cardiac intensive care team after stage 1 recon-struction (S1R). Equally challenging is the prevention of interstage death(ISD), defined as death after hospital discharge and before admission for a plannedstage 2 reconstruction (S2R). Hospital survival after S1R has improved as a resultof continued innovation and refinement in surgical technique, earlier diagnosis andreferral to tertiary care centers, and advances in perioperative care. 1-10 As a result,this group of congenital lesions once considered universally fatal in infancy nowhas a survival after S1R of 77% to 93%. 11-16 However, at most centers ISD ratesare unchanged from historical rates of 7% to 15%. 17-22 Therefore, interstage mortalityconstitutes an increasing percentage of overall mortality in the current era and isattracting increased clinical and research attention.The reported causes of ISD range from simple intercurrent illnesses to catastrophicshuntthromboses. 19,23 InattemptstoidentifythosepatientsatriskforISDevents,pastinvestigatorshaveidentifiedresidualanatomiclesions,depressedmyocardialfunction,elevated systemic vascular resistance, arrhythmia, and noncardiac factors such as sei-zures and feeding dysfunction as significant risk factors. 3,12,19-25 It is clear that afterFrom the Division of Cardiology, a Depart-ment of Anesthesiology and Critical CareMedicine, b and the Division of Cardiotho-racic Surgery c at The Children’s Hospitalof Philadelphia and University of Pennsyl-vania School of Medicine, Philadelphia, Pa.Received for publication June 13, 2007;revisions received Nov 21, 2007; acceptedfor publication Dec 18, 2007.Address for reprints: David A. Hehir, MD,Children’s Hospital of Philadelphia,Divisions of Cardiology and Critical CareMedicine, 34th St and Civic Center Blvd.,Philadelphia, PA 19104 (E-mail: hehir@email.chop.edu).J Thorac Cardiovasc Surg 2008;136:94-90022-5223/$34.00Copyright ? 2008 by The American Asso-ciation for Thoracic Surgerydoi:10.1016/j.jtcvs.2007.12.012CHD94 The Journal of Thoracic and Cardiovascular Surgeryc July 2008Surgery for Congenital Heart Disease Hehir et al
