Extracorporeal Membrane Oxygenation Support After the Fontan Operation
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CONGENITAL HEART DISEASE Extracorporeal membrane oxygenation support after the Fontan operation Kelly L. Rood, MD,a,c Sarah A. Teele, MD,a,c Cindy S. Barrett, MD,a,c Joshua W. Salvin, MD, MPH,a,c Peter T. Rycus, MPH,e Francis Fynn-Thompson, MD,b,d Peter C. Laussen, MBBS,a,c and Ravi R. Thiagarajan, MBBS, MPHa,c CHD Objective: Extracorporeal membrane oxygenation has been used to support children with cardiac failure after the Fontan operation. Mortality is high, and causes of mortality remain unclear. We evaluated the in-hospital mortality and factors associated with mortality in these patients. Methods: Extracorporeal Life Support Organization registry data on patients requiring extracorporeal mem- brane oxygenation after the Fontan operation from 1987 to 2009 were retrospectively analyzed. Demographics and extracorporeal membrane oxygenation data were compared for survivors and nonsurvivors. A multivariable logistic regression model was used to identify factors associated with mortality. Results: Of 230 patients, 81 (35%) survived to hospital discharge. Cardiopulmonary resuscitation was more frequent (34% vs 17%, P ¼ .04), and median fraction of inspired oxygen concentration was higher (1 [confi- dence interval, 0.9–1.0] vs 0.9 [confidence interval, 0.8–1.0], P ¼ .03) before extracorporeal membrane oxygen- ation in nonsurvivors compared with survivors. Extracorporeal membrane oxygenation duration and incidence of complications, including surgical bleeding, neurologic injury, renal failure, inotrope use on extracorporeal membrane oxygenation, and bloodstream infection, were higher in nonsurvivors compared with survivors (P < .05 for all). In a multivariable model, neurologic injury (odds ratio, 5.18; 95% confidence interval, 1.97–13.61), surgical bleeding (odds ratio, 2.36; 95% confidence interval, 1.22–4.56), and renal failure (odds ratio, 2.81; 95% confidence interval, 1.41–5.59) increased mortality. Extracorporeal membrane oxygenation du- ration of more than 65 hours to 119 hours (odds ratio, 0.33; 95% confidence interval, 0.14–0.76) was associated with decreased mortality. Conclusions: Cardiac failure requiring extracorporeal membrane oxygenation after the Fontan operation is as- sociated with high mortality. Complications during extracorporeal membrane oxygenation support increase mortality odds. Prompt correction of surgical bleeding when possible may improve survival. (J Thorac Cardi- ovasc Surg 2011;142:504-10) Extracorporeal membrane oxygenation (ECMO) has been (extracorporeal cardiopulmonary resuscitation [ECPR]) as used to provide mechanical circulatory support for patients a bridge to recovery or transplantation.8-12 Morbidity and with congenital or acquired cardiac disease in cardiac fail- mortality associated with ECMO use are high; survival in ure when conventional medical management has failed. children with heart disease requiring ECMO support is ECMO has been used with increasing frequency to support reportedtobe33% to 60%.1-5,8,10-12 Factors associated with the postoperative patient with cardiac failure after repair or mortality in children supported with ECMO after cardiac palliation of congenital heart disease.1-9 Indications for surgery for congenital heart disease remain variable.1-8,11 ECMO support in the postoperative period include severe The Fontan procedure is an established surgical palliation cardiac dysfunction from postcardiotomy cardiac failure, procedure for single ventricle cardiac anomalies in which hemodynamically unstable and refractory arrhythmias, completion of a total cavopulmonary connection is and circulatory support during and after cardiac arrest achieved by anastomosis of the inferior vena cava to the pul- monary arteries, thus allowing passive return of all systemic 13 From the Departments of Cardiologya and Cardiac Surgery,b Children’s Hospital venous blood into the pulmonary circulation. The use of Boston, Mass; Departments of Pediatricsc and Surgery,d Harvard Medical School, e ECMO to support severe cardiac dysfunction in children Boston, Mass; and Extracorporeal Life Support Organization, University of 14 Michigan, Ann Arbor, Mich. with the Fontan circulation has been reported. However, Disclosures: Authors have nothing to disclose with regard to commercial support. current knowledge of outcomes after ECMO support in Received for publication July 12, 2010; revisions received Oct 8, 2010; accepted for this population is limited to case reports and small single- publication Nov 25, 2010; available ahead of print Feb 28, 2011. Address for reprints: Kelly L. Rood, MD, Children’s Hospital Boston, 300, Long- center series. It is therefore difficult to draw inferences re- wood Avenue, Boston, MA 02115 (E-mail: [email protected]). garding the applicability of ECMO for support of severe 0022-5223/$0.00 cardiac dysfunction in this population.4-7,10,11,15-18 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery The purpose of this study was to use a large multi- doi:10.1016/j.jtcvs.2010.11.050 institutional data set to better evaluate in-hospital mortality 504 The Journal of Thoracic and Cardiovascular Surgery c September 2011 Rood et al Congenital Heart Disease ‘‘aorta’’ or ‘‘right atrium’’ were considered to be cannulated via the tho- Abbreviations and Acronyms racic route. Secondary procedural codes and discharge procedure CPT co- ¼ des for heart transplantation (CPT code 33945) were used to identify CI confidence interval patients who underwent cardiac transplantation before hospital discharge. CNS ¼ central nervous system ECMO complications were categorized using codes created by the CPR ¼ cardiopulmonary resuscitation ELSO registry for the explicit purpose of reporting complications. We cat- CPT ¼ Common Procedural Technology egorized complications as follows: 1. Mechanical complications: defined ECMO ¼ extracorporeal membrane oxygenation as mechanical failure of an ECMO circuit component, report of circuit ¼ air embolus, or mechanical problems related to the cannula; 2. Circuit ECPR extracorporeal cardiopulmonary thrombus: included the report of thrombus in any area of the ECMO circuit; resuscitation 3. Surgical bleeding: defined as bleeding from the surgical site or cannula- ELSO ¼ Extracorporeal Life Support tion requiring an intervention with transfusion or surgical intervention; 4. CHD Organization Neurologic injury (central nervous system [CNS] injury): defined as the HLHS ¼ hypoplastic left heart syndrome presence of a clinical diagnosis of brain death, clinical or electroencepha- ¼ lographic evidence of seizures, or radiologic evidence (head ultrasound or IQR interquartile range computed tomography) of cerebral infarction or bleeding; 5. Renal failure: IRB ¼ institutional review board defined as serum creatinine greater than 1.5 mg/dL regardless of age or OR ¼ odds ratio need for renal replacement therapy; 6. ECMO-related complications: included continued need for inotropic support while on ECMO, need for cardiopulmonary resuscitation (CPR) while on ECMO, and continued metabolic acidosis defined as pH less than 7.2 despite ECMO support; 7. for children with severe cardiac failure requiring ECMO Cardiac complications: included cardiac arrhythmia during ECMO support support after the Fontan operation. We aimed to identify requiring treatment and cardiac tamponade requiring intervention; 8. Respiratory complications: defined as pulmonary hemorrhage or pneumo- factors associated with mortality to potentially direct man- thorax requiring drainage during ECMO support; 9. Infectious complica- agement decisions and to help improve patient outcomes. tions: included culture-proven bloodstream infection; 10. Metabolic complications: included hypoglycemia defined as a blood glucose level less than 40 mg/dL for all ages, and hyperglycemia defined as blood glu- MATERIALS AND METHODS cose level greater than 240 mg/dL for all ages; and 11. Gastrointestinal Data Source complications: defined as gastrointestinal hemorrhage requiring interven- Data for this study were obtained from the Extracorporeal Life Support tion or hyperbilirubinemia defined as total serum bilirubin total greater Organization’s (ELSO) data registry. The ELSO registry collects data on than 15 mg/dL, indirect bilirubin greater than 13 mg/dL, or direct bilirubin ECMO used for purposes of supporting critically ill patients with cardiore- greater than 2 mg /dL. For purposes of purposes of this study, we defined spiratory failure from all indications. Data are reported to the registry from mortality as death before hospital discharge from the institution providing 116 reporting centers, including 14 international centers. Data are collected ECMO support. The ELSO registry does not collect information regarding using a standardized data-collection form containing patient demographic timing of ECMO use after the index operation, pre-Fontan operation hemo- information, diagnosis, and procedures at the time of ECMO onset and at dynamic data, type of Fontan operation (lateral tunnel or extracardiac con- death or discharge, information regarding the indication and conduct of duit), information regarding the use of a fenestration in the Fontan pathway, ECMO including details of equipment used, ECMO complications and sur- functional neurologic outcomes at discharge, quality of life information for vival to hospital discharge, and discharge disposition. Data reported to the survivors, and longer-term survival data; thus, these data were not available registry are approved at each member institution