ORIGINAL ARTICLE

European Journal of Cardio-Thoracic Surgery 46 (2014) 458–464 doi:10.1093/ejcts/ezu022 Advance Access publication 19 February 2014

Management of early Fontan failure: a single-institution experience† Michael O. Murphy*, Andrew C. Glatz, David J. Goldberg, Lindsay S. Rogers, Chitra Ravishankar, Susan C. Nicolson, James M. Steven, Stephanie Fuller, Thomas L. Spray and J. William Gaynor The Cardiac Center, The Children’s Hospital of Philadelphia, PA, USA * Corresponding author. The Cardiac Center, The Children's Hospital of Philadelphia, PA 19104, USA. Tel: +1-215-5902708; fax: +1-215-5902715; e-mail: [email protected] (M.O. Murphy). Received 18 September 2013; received in revised form 15 December 2013; accepted 18 December 2013

Abstract OBJECTIVE: To analyse the incidence and outcomes of early Fontan failure (EFF) in a large contemporary cohort of palliated patients. METHODS: A retrospective, single-centre study of all patients undergoing primary Fontan from 1 July 1995 to 31 December 2009 was performed. EFF was defined as death, need for extracorporeal membrane oxygenation (ECMO), Fontan takedown to superior cavopulmonary connection (SCPC) or transplantation within 30 days of the Fontan procedure. The incidence and outcomes were summarized with descriptive statistics, and risk factors for EFF were identified. RESULTS: A total of 592 patients underwent primary Fontan procedure during the study period; 67% had a dominant right ventricle. An extracardiac conduit (ECC) was used for Fontan completion in 60.5%, with the remainder having a lateral tunnel. EFF occurred in 11 patients (1.9%), all of whom had ECC. ECMO was used in 5 patients, 5 had Fontan takedown and 2 had heart transplantation. Five of eleven, or 46%, study subjects died as opposed to an overall mortality for primary Fontan of 0.8%. Among patients who had Fontan takedown to SCPC, long-term survival was 80%. By univariate analysis, elevated ventricular end-diastolic pressure (9.5 ± 3.3 vs 7.4 ± 2.7 mmHg, P = 0.019) and total circulatory support time (99 ± 33 vs 71 ± 23 min, P = 0.001) were risk factors for EFF. The mean follow-up for the 6 hospital survivors was 5.9 years. There was one late transplant-related death. Of the 4 surviving patients who had Fontan takedown to a SCPC, 3 underwent subsequent Fontan completion and 1 underwent biventricular repair. CONCLUSIONS: EFF is rare in the current era, but is associated with significant mortality. High filling pressures and a prolonged intraoperative course are risk factors for EFF. Of the management strategies available, Fontan takedown to an intermediate pathway appears to be associated with the best outcomes. Keywords: Early Fontan failure • Fontan takedown • ECMO

INTRODUCTION In the early era of the Fontan procedure, case series demonstrated rates of early Fontan failure (EFF) of over 10%, with consequent early mortality rates of 9–15% [1–4]. EFF has been used to describe the postoperative state of low systemic perfusion, high Fontan circuit pressures (FP) and large volume requirements, which is unresponsive to inotropes and is the principal cause of early death after Fontan completion. The incidence of EFF has decreased greatly over time with large unselected series reporting perioperative mortality rates under 3% [5–11] and even below 1% in selected series [12, 13]. Strategies to avoid EFF include careful case selection, avoiding Fontan completion in those at high risk for EFF [14], the use of Fontan takedown to an intermediate circulation [2–5, 7–11, 14–21] and extracorporeal membrane oxygenation (ECMO) as a bridge to recovery or takedown [6, 8, 9, 12, 15, 17, 22]. Though emergent transplant is often included in the definition of EFF and discussed † Presented at the 27th Annual Meeting of the European Association for CardioThoracic Surgery, Vienna, Austria, 5–9 October 2013.

as an option in the management, we found only 1 case of its use for management of EFF [23]. Not surprisingly, survival rates after EFF can be low, with historical series reporting mortality rates of over 85% [1–4], but, in more recent series, survival rates of 33–66% have been achieved with the use of ECMO and/or takedown [6, 8–12, 18]. A number of important patient and operative variables have previously been described as risk factors for EFF, including heterotaxy syndrome [4, 5, 15], right ventricular morphology [15, 19], common atrioventricular valve (AVV) [4, 18, 20], increased preoperative pulmonary artery pressure (PAP) [2–4, 14, 17, 18, 20], increased preoperative ventricular end-diastolic pressure (VEDP) [1, 3], use of extracardiac conduit (ECC) [5, 8], elevated postoperative FP [1, 3, 4], prolonged cardiopulmonary bypass time (CPB) [1, 2, 4, 8, 15, 16, 20] and prolonged cross-clamp time (XC) [3, 8, 16]. Some of these risk factors seem to have been neutralized by improved understanding, experience and techniques, and so the aim of this study was to analyse the incidence of and outcomes of management strategies for EFF in a contemporary series of palliated patients and to identify risk factors for EFF.

© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

M.O. Murphy et al. / European Journal of Cardio-Thoracic Surgery

Data collection A single-centre retrospective study of a large cohort of patients palliated for single-ventricle physiology was carried out. The institutional review board of The Children’s Hospital of Philadelphia approved the study. The cardiac surgery database was interrogated for patients undergoing primary Fontan procedure from 1 July 1995 to 31 December 2009, and demographic, anatomic, preoperative haemodynamic and procedural variables were gleaned from echocardiograms, cardiac catheterisations and operative notes. Follow-up information was obtained from clinic notes. EFF was defined as early postoperative death, the need for Fontan takedown to an intermediate circulation, the need for ECMO or emergent transplant in the first 30 days after Fontan surgery. Patients requiring prolonged inotrope support with ultimate recovery to discharge without intervention were not included as EFF.

Surgical management All Fontan procedures were performed using cardiopulmonary bypass, and all but 6 patients had cold cardioplegic arrest for the procedure. Choice of Fontan technique, use of fenestration and use of deep hypothermic circulatory arrest (DHCA) were at the discretion of the operating surgeon and alpha-stat blood gas management was used. Pump flow rates were not standardized for this study. Prior to DHCA, patients underwent core cooling for at least 15 min with topical hypothermia of the head to a nasopharyngeal temperature of 18°C. Haematocrit was maintained around 30 and rewarming was usually achieved in at least 22 min. Fenestrations were created as a single punch hole in the Gore-Tex (W.L. Gore and Associates, Flagstaff, AZ, USA) baffle for the lateral tunnel (LT) group or as a single punch hole in the conduit with a side-to-side anastomosis to the atrium, for the ECC group. Perfusion data were recorded as CPB, XC, DHCA and total support time (TS), the latter defined as CPB plus DHCA.

Statistical analysis Standard summary statistics were used to describe the data, and reported as mean ± standard deviation for normally distributed continuous variables, median with range for skewed continuous variables and frequency count with percentage of total for categorical variables. Univariate logistic regression was used to identify potential factors associated with EFF. Statistical significance was determined a priori as a two-tailed P–value of

Management of early Fontan failure: a single-institution experience.

To analyse the incidence and outcomes of early Fontan failure (EFF) in a large contemporary cohort of palliated patients...
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