TEXAS CHILDREN's HOSPITAL EVIDENCE-BASED OUTCOMES CENTER Postoperative Management of the Fontan Patient Evidence-Based Guideli

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DATE: January 2018 TEXAS CHILDREN’S HOSPITAL EVIDENCE-BASED OUTCOMES CENTER Postoperative Management of the Fontan Patient Evidence-Based Guideline

Definition: First described in 1971 as a surgical intervention Differential Diagnosis for patients with tricuspid atresia, the Fontan operation has Other left-sided obstructive lesions where the systemic become the most common operation performed for patients circulation is dependent on ductal flow with hypoplastic left heart syndrome or single ventricle. (1) Refinements in operative technique and perioperative Diagnostic Evaluation management have resulted in improved outcome in this high- History: Assess for risk group of patients. The Fontan is usually the final planned  A thorough clinical assessment operation in the staged single ventricle palliation pathway. The  Oximetry at rest procedure involves the placement of a conduit from the right  ECG to assess for sinus rhythm atrium to the main pulmonary artery which bypasses the hypoplastic right ventricle and results in a total cavopulmonary  Chest x-ray connection. The Fontan operation is typically performed Physical Examination between two and five years of age, depending on the specific clinical presentation and surgical history. Following the Fontan,  Cardiac catherization (ideal measurements): appropriate postoperative management is essential for o Common atrial pressure: <12 preventing postoperative mortality, reducing postoperative o PVR: <4 Woods units complications and length of stay, and promotion of optimal long o TPG: <8 term outcomes. o BDG/PA: ≤15 o Oxygen saturations: >75% Inclusion Criteria  Echocardiography:  Patients 18 years and younger o ≤ Mild atrioventricular valve regurgitation  Single ventricle o ≤ Mild ventricular dysfunction or systemic EF >45% o ≤ Mild aortic insufficiency Exclusion Criteria  24-hour Holter:  Patients over the age of 18 years o No clinically relevant dysrhythmia  Fontan conversion procedures  Fontan with concomitant procedures Laboratory Tests  Preoperative renal insufficiency/failure  Serum protein and albumin measurement. If low, increased 1  Failing Fontan anti-trypsin clearance in the stool documents the presence of PLE (protein losing enteropathy).

Critical Points of Evidence* Evidence Supports  Administration of Lasix per the following postoperative regimen: 24 hours: IV Lasix 1 mg/kg every 12 hours (initial dose not to exceed 20 mg/dose); 48 hours: IV Lasix 1 mg/kg every 8 hours; 72 hours: PO Lasix 1.5 mg/kg every 8 hours (do not exceed 20 mg/dose). (2- 6) – Strong recommendation, low quality evidence  Initiation of ACE inhibitor for patients with significant hypertension or AV valve regurgitation once taking PO if patient does not have concern for renal injury. (2,3,4-6) – Strong recommendation, low quality evidence  Administration of acetylsalicylic acid 81 mg for antithrombotic therapy beginning on postoperative day 2. (4,8-10) – Strong recommendation, low quality evidence  Initiation of fluid restriction at 50% maintenance then up to a maximum of 80% maintenance once the patient begins taking liquids by mouth. (2,5,6) – Strong recommendation, low quality evidence  Administration of supplemental oxygen for all patients at a minimum of 0.5 L/min via nasal cannula through postoperative day 3 when patient is asleep or in bed (OK to discontinue with ambulation; DO NOT limit out of bed or walking). Continue after postoperative day 3 if oxygen saturations are <94%. For patients with fenestrated Fontans, documented AV malformations and/or veno-venous collaterals, patient care should be individualized. (2,5,6) – Strong recommendation, low quality evidence  If chylous drainage is suspected or confirmed (≥5 cc/kg drainage), test for the presence of triglycerides in drainage by 48 to 72 hours. If drainage is positive for fluid triglycerides (pleural fluid triglycerides >110 + lymphocyte count = chylous effusion), initiate minimal fat diet. (5,11,12) – Strong recommendation, low quality evidence  Management of postoperative pain utilizing multimodal analgesia. Patients should be on a morphine PCA on admission to the CVICU and for 48 hours and around-the-clock acetaminophen (IV/PO) for 48 hrs. The day following surgery patients should be started on ketorolac, 6-8 doses, as long as their platelets are >100, creatinine <0.6, and they don't have bloody chest tube output. Patients should initially be on dexmedetomidine for approximately 24 hours unless they are paced or their HR is <60. When being transferred to the floor, the PCA Morphine can transition to PRN morphine. When taking PO, they should be transition from PCA morphine or scheduled morphine to scheduled Hycet (with PRN morphine). (13-18) – Strong recommendation, low quality evidence © Evidence-Based Outcomes Center 1 Texas Children’s Hospital DATE: January 2018  Encourage highest degree of mobility within 18 hours postoperatively or the morning after surgery: order physical therapy consult on admission; encourage highest degree of mobility within 18 hours or morning of surgery, with an ambulation goal of a minimum of three times a day; to initiate respiratory physiotherapy using age-appropriate device (e.g., incentive spirometry, bubbles, pinwheel) within 18 hours of surgery or morning after procedure, every hour while awake, starting on postoperative day 1. (19-22) – Strong recommendation, low quality evidence

Evidence Against  Postoperative use of ACE inhibitors for all Fontan patients. (2,3,4-6) – Strong recommendation, low quality evidence

Evidence Lacking/Inconclusive  Postoperative use of ace inhibitors for all Fontan patients. (2,3,4-6) –Unable to make a recommendation  The use of vasopressin for the reduction of chest drainage following the Fontan procedure. –Unable to make a recommendation

*NOTE: The references cited represent the entire body of evidence reviewed to make each recommendation.

Condition-Specific Elements of Clinical Management Discharge Criteria Measures  Drains: chest tubes removed Structure  Diuretics: 1) All enteral, 2) Lasix ≤3 doses/day, 3) Diuril ≤2  Bed availability doses/day Process  Mobilization: adequate mobilization  Postoperative additional procedures  Pain: pain well controlled by PO medicines (PRN or Outcome scheduled morphine or Dilaudid no longer needed)  Mortality  Feeds: tolerating regular diet  Morbidity (infection, thromboembolic events, etc…)  Elimination: normal bowel movements  Length of stay  Discharge teaching complete  Length of stay in CVICU  Discharge ECHO, EKG and CXR performed  Duration of chest tubes  Follow up appointments made with 1) Cardiology and 2) CV  Total chest tube drainage surgery  Number of days chest tube drainage  Readmissions Follow-Up Care  Patient/family satisfaction  Follow-up clinic date and time confirmed with surgical PA  Functional health status prior to discharge  Quality of life  Families instructed to call if new: redness, swelling, pain,  Cost discharge around drain, edema, abrupt change in amount or character of drainage, cough, fever, cyanosis, and/or SOB

FONTAN MANAGEMENT TABLE

POD CV Meds Diuretics2 Fluid Nutrition4 Respiratory Chest Tubes Mobilization Pain Mx Disposition

Inotropes None Crystalloid / NPO then OR Head of bed to  PCA CVICU blood clears if extubation vs. 30 degrees 0  Ofirmev product stable <6hrs ICU5 replacement Wean  IV furosemide3 50% Start enteral Min: Mediastinal out7 Encourage  PCA CVICU 6 1 Inotropes (1 mg/kg q12 max maintenance intake 0.5L/min highest degree of  IV acetaminophen 20 mg/dose) mobilization  Consider Toradol8  Initiate ASA  IV furosemide3 75-80% PO Min: Mediastinal out7 Walk in ICU at  PO opioid Transfer to 15T  Consider (1 mg/kg q8 maintenance encouraged 0.5L/min Pleural to bulb least once per +acetaminophen 2 resuming max 20 mg/dose) shift  Consider home meds  PO Diuril ketoralac8  ASA  PO furosemide3 75-80% Full PO Min: Pleural to bulb Walk x3  PO opioid Transfer to 15T  Consider (1.5 mg/kg q8 maintenance 0.5L/min +acetaminophen 3 resuming max 20 mg/dose)  PO NSAID home meds  PO Diuril  ASA Optimize PO 75-80% Full PO Stop O2 if Remove CTs if Full mobilization  PO NSAID 4 diuretics maintenance Sats >94% <2cc/kg/d each as at home  PRN PO opioid (minimum 3x)

 ASA Decrease or 75-80% Full PO Stop O2 if Remove CTs if Full mobilization  PO NSAID d/c criteria met? maintain maintenance Sats >94% <2cc/kg/d each as at home  PRN PO opioid 5 (minimum 3x)

 ASA Decrease or 75-80% Full PO Stop O2 if Remove CTs if Full mobilization  PO NSAID d/c criteria met? maintain maintenance Sats >94% <2cc/kg/day each as at home  PRN PO opioid 6 (minimum 3x)

© Evidence-Based Outcomes Center 3 Texas Children’s Hospital DATE: January 2018 References 1. Cowgill, L. D. (1991). The Fontan procedure: A historical review. Annals of Thoracic Surgery, 51(6), 1026-1030. 2. Cava, J. R., Bevandic, S. M., Steltzer, M. M., & Tweddell, J. S. (2005). A medical strategy to reduce persistent chest tube drainage after the Fontan operation. American Journal of Cardiology, 96(1), 130-133. 3. Fujita, T., Kawasaki, S., Matsushita, S., Inaba, H., Morita, T., Kuwaki, K., & Amano, A. (2014). Effect of regular medication using super high dose diuretics for pleural effusions at early stage after the Fontan procedure. Juntendo Medical Journal, 60(1), 35-42. 4. Jacobs, M. L., Pelletier, G. J., Pourmoghadam, K. K., Mesia, C. I., Madan, N., Stern, H., ... & Murphy, J. D. (2008). Protocols associated with no mortality in 100 consecutive Fontan procedures. European Journal of Cardio-Thoracic Surgery, 33(4), 626-632. 5. Pike, N. A., Okuhara, C. A., Toyama, J., Gross, B. P., Wells, W. J., & Starnes, V. A. (2015). Reduced pleural drainage, length of stay, and readmissions using a modified Fontan management protocol. Journal of Thoracic and Cardiovascular Surgery, 150(3), 481-487. 6. Sunstrom, R. E., Muralidaran, A., Gerrah, R., Reed, R. D., Good, M. K., Armsby, L. R., & Langley, S. M. (2015). A defined management strategy improves early outcomes after the Fontan procedure: the Portland protocol. Annals of Thoracic Surgery, 99(1), 148-155. 7. Jin, S. M., Noh, C. I., Bae, E. J., Choi, J. Y., & Yun, Y. S. (2007). Impaired vascular function in patients with Fontan circulation. International Journal of Cardiology, 120(2), 221-226. 8. Alsaied, T., Alsidawi, S., Allen, C. C., Faircloth, J., Palumbo, J. S., & Veldtman, G. R. (2015). Strategies for thromboprophylaxis in Fontan circulation: A meta-analysis. Heart, 101(21), 1731-1737. 9. Monagle, P., Cochrane, A., Roberts, R., Manlhiot, C., Weintraub, R., Szechtman, B., & Fontan Anticoagulation Study Group. (2011). A multicenter, randomized trial comparing heparin/warfarin and acetylsalicylic acid as primary thromboprophylaxis for 2 years after the Fontan procedure in children. Journal of the American College of Cardiology, 58(6), 645-651. 10. McCrindle, B. W., Manlhiot, C., Cochrane, A., Roberts, R., Hughes, M., Szechtman, B., & Fontan Anticoagulation Study Group. (2013). Factors associated with thrombotic complications after the Fontan procedure: A secondary analysis of a multicenter, randomized trial of primary thromboprophylaxis for 2 years after the Fontan procedure. Journal of the American College of Cardiology, 61(3), 346-353. 11. Beghetti, M., La Scala, G., Belli, D., Bugmann, P., Kalangos, A., & Le Coultre, C. (2000). Etiology and management of pediatric chylothorax. Journal of Pediatrics, 136(5), 653-658. 12. Chan, E. H., Russell, J. L., Williams, W. G., Van Arsdell, G. S., Coles, J. G., & McCrindle, B. W. (2005). Postoperative chylothorax after cardiothoracic surgery in children. Annals of Thoracic Surgery, 80(5), 1864-1870. 13. Elia, N., Lysakowski, C., & Tramer, M. R. (2005). Does multimodal analgesia with acetaminophen, nonsteroidal antiinflammatory drugs, or selective cyclooxygenase-2 inhibitors and patient-controlled analgesia morphine offer advantages over morphine alone? Meta-analyses of randomized trials. Journal of the American Society of Anesthesiologists, 103(6), 1296-1304. 14. McDaid, C., Maund, E., Rice, S., Wright, K., Jenkins, B., & Woolacott, N. (2011). Paracetamol and selective and non-selective non-steroidal antiinflammatory drugs for the reduction in morphine-related side-effects after major surgery: A systematic review. British Journal of Anaesthesia, 106(3), 292-297. 15. Rafiq, S., Steinbrüchel, D. A., Wanscher, M. J., Andersen, L. W., Navne, A., Lilleoer, N. B., & Olsen, P. S. (2014). Multimodal analgesia versus traditional opiate based analgesia after cardiac surgery, a randomized controlled trial. Journal of Cardiothoracic Surgery, 9(1), 52. 16. Hasegawa, T., Oshima, Y., Maruo, A., Matsuhisa, H., Tanaka, A., Noda, R., & Matsushima, S. (2015). Dexmedetomidine in combination with midazolam after pediatric cardiac surgery. Asian Cardiovascular and Thoracic Annals, 23(7), 802-808. 17. Jiang, L., Ding, S., Yan, H., Li, Y., Zhang, L., Chen, X., & Zhang, J. (2015). A retrospective comparison of dexmedetomidine versus midazolam for pediatric patients with congenital heart disease requiring postoperative sedation. Pediatric Cardiology, 36(5), 993-999. 18. Tokuhira, N., Atagi, K., Shimaoka, H., Ujiro, A., Otsuka, Y., & Ramsay, M. (2009). Dexmedetomidine sedation for pediatric post-Fontan procedure patients. Pediatric Critical Care Medicine, 10(2), 207-212. 19. Burtin, C., Clerckx, B., Robbeets, C., Ferdinande, P., Langer, D., Troosters, T., & Gosselink, R. (2009). Early exercise in critically ill patients enhances short-term functional recovery. Critical Care Medicine, 37(9), 2499-2505. 20. Cho, Y. J., Ryu, H., Lee, J., Park, I. K., Kim, Y. T., Lee, Y. H., & Jeon, Y. (2014). A randomised controlled trial comparing incentive spirometry with the Acapella® device for physiotherapy after thoracoscopic lung resection surgery. Anaesthesia, 69(8), 891-898. 21. Kaminski, P. N., Forgiarini, L. A., & Andrade, C. F. (2013). Early respiratory therapy reduces postoperative atelectasis in children undergoing lung resection. Respiratory care, 58(5), 805-809. 22. Wieczorek, B., Ascenzi, J., Kim, Y., Lenker, H., Potter, C., Shata, N. J., & Hoch, J. (2016). PICU Up!: Impact of a quality improvement intervention to promote early mobilization in critically ill children. Pediatric Critical Care Medicine, 17(12), e559-e566.

Clinical Standards Preparation Development, Clarity and Presentation, Applicability, and Editorial This clinical standard was prepared by the Evidence-Based Independence using a 4-point Likert scale. The higher the score, Outcomes Center (EBOC) team in collaboration with content the more comprehensive the guideline. experts at Texas Children’s Hospital. Development of this clinical This clinical standard specifically summarizes the evidence in standard supports the TCH Quality and Patient Safety Program support of or against specific interventions and identifies where initiative to promote clinical standards and outcomes that build a evidence is lacking/inconclusive. The following categories describe culture of quality and safety within the organization. how research findings provide support for treatment interventions. “Evidence Supports” provides evidence to support an Postoperative Management of the Fontan Patient Content intervention Expert Team “Evidence Against” provides evidence against an intervention. Lauren Kane, MD, Congenital Heart Surgery “Evidence Lacking/Inconclusive” indicates there is insufficient Heather Dickerson, MD, Cardiology evidence to support or refute an intervention and no conclusion Jordana Goldman, MD, Critical Care Medicine can be drawn from the evidence. Daniel Trujillo Diaz , MD, Congenital Heart Surgery The GRADE criteria were utilized to evaluate the body of evidence Amy HemingwayRN, CNP used to make practice recommendations. The table below defines Karla Resendiz Trujano, PharmD how the quality of the evidence is rated and how a strong versus Premal Trivedi, MD, Anesthesiology weak recommendation is established. The literature appraisal Kristen Neff, RN, Patient & Family Advocate reflects the critical points of evidence. Susan Rusinger, RN Miranda Rodrigues, RN, CNS Recommendation Desirable effects clearly outweigh undesirable effects or STRONG EBOC Team vice versa Sheesha Porter, MSN, RN, CNOR, Evidence-Based Practice Desirable effects closely balanced with undesirable WEAK Specialist effects Ellis M. Arjmand MD, MMM, PhD, Associate Medical Director Quality Type of Evidence Charles Macias, MD, MPH, Medical Director High Consistent evidence from well-performed RCTs or Additional EBOC Support exceptionally strong evidence from unbiased Tom Burke, Research Assistant observational studies Sherin Titus, Research Assistant Moderate Evidence from RCTs with important limitations (e.g., Karen Gibbs, MSN/MPH, RN, Evidence-Based Practice Specialist inconsistent results, methodological flaws, indirect Andrea Jackson, MBA, RN, Evidence-Based Practice Specialist evidence, or imprecise results) or unusually strong Betsy Lewis, MSN, RN, CNL, Evidence-Based Practice Specialist evidence from unbiased observational studies Jennifer Loveless, MPH, Evidence-Based Practice Specialist Low Evidence for at least 1 critical outcome from Anne Dykes, MSN, RN, Assistant Director observational studies, RCTs with serious flaws or Kathy Carberry, MPH, RN, Director indirect evidence Very Low Evidence for at least 1 critical outcome from No relevant financial or intellectual conflicts to report. unsystematic clinical observations or very indirect evidence Development Process This clinical standard was developed using the process outlined in Recommendations the EBOC Manual. The literature appraisal documents the Practice recommendations were directed by the existing evidence following steps: and consensus amongst the content experts. Patient and family 1.Review Preparation preferences were included when possible. The Content Expert - PICO questions established Team and EBOC team remain aware of the controversies in the - Evidence search confirmed with content experts postoperative management of children following the Fontan 2.Review of Existing External Guidelines Procedure. When evidence is lacking, options in care are provided - CVS: ICU Management of HLHS and Single Ventricle in the clinical standard and the accompanying order sets (if Physiology; Management of Postoperative Pain: A Clinical applicable). Practice Guideline from the American Pain Society, the American Society of Regional Anesthesia and Pain Medicine, Approval Process and the American Society of Anesthesiologists' Committee on Clinical standards are reviewed and approved by hospital Regional Anesthesia, Executive Committee, and committees as deemed appropriate for its intended use. Clinical Administrative Council standards are reviewed as necessary within EBOC at Texas 3.Literature Review of Relevant Evidence Children’s Hospital. Content Expert Teams are involved with every - Searched: PubMed, Cochrane Library, EMBASE review and update. 4.Critically Analyze the Evidence - 4 meta-analyses, 5 randomized controlled trials, and 12 Disclaimer nonrandomized studies Practice recommendations are based upon the evidence available 5. Summarize the Evidence at the time the guideline was developed. Clinical standards - Materials used in the development of the clinical standard, (guidelines, summaries, or pathways) do not set out the standard literature appraisal, and any order sets are maintained in a of care and are not intended to be used to dictate a course of care. postoperative management of the Fontan patient evidence- Each physician/practitioner must use his or her independent based review manual within EBOC. judgment in the management of any specific patient and is responsible, in consultation with the patient and/or the patient’s Evaluating the Quality of the Evidence family, to make the ultimate judgment regarding. Published clinical guidelines were evaluated for this review using the AGREE II criteria. The summary of these guidelines are Version History included in the literature appraisal. AGREE II criteria evaluate Date Comments Guideline Scope and Purpose, Stakeholder Involvement, Rigor of Jan 2018 Originally completed