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Post-operative Management Following the Unifocalization Procedure in Patients with TOF/PA/MAPCAs: The Stanford Experience Sandra Staveski RN, PhD, CPNP-AC, CNS, CCRN Assistant Professor, Research in Patient Services/Heart Institute Cincinnati Children’s Hospital Medical Center Formerly Nurse Practitioner in Cardiovascular ICU at Lucile Packard Children’s Hospital Stanford No disclosures Objectives • Anatomical and physiologic considerations • ICU course following the unifocalization procedure • Standardized management and daily goal directed therapy • Specific areas of nursing focus Anatomical Considerations • Less common, complex subset of TOF – TOF/PA ~ 20% of TOF*; 30-65% have MAPCAs+ • Pulmonary blood supply: – Collateral-dependent – Severe abnormalities of native pulmonary arteries – Marked heterogeneity between patients • MAPCAs – Derived from splanchnic vascular plexus – May supply all pulmonary blood flow, be part of “dual supply", or supply a single lung segment – Propensity for stenosis++ *Baltimore-Washington Infant Study 1981-89 +Shimazaki Y 1990 ++Ma et al., 2014 Unifocalization Concept Surgical reconstruction of the pulmonary arterial tree, incorporating as many lung segments as possible, by maximal recruitment of true pulmonary arteries and MAPCAs Haworth and Macartney,1980 Malhotra and Hanley, 2009 Conceptual Goals • To create a single-compartment, unobstructed, low-pressure pulmonary circulation in a separated, 2-ventricle circulation as early as possible • Achieve normal circulatory physiology – Low RV systolic pressure (< 50% LVp) • • • • Maximize lung segments incorporated Minimize pulmonary vascular resistance Eliminate VSD shunt Eliminate proximal RV outflow obstruction Bull & Somerville et al., 1995 Pulmonary Vascular Beds of Children with TOF/PA/MAPCAs • “Microclimates” – Hypoperfused – High pressure, high flow – Normal • Morphology and physiology determines surgical approach and surgical approach determines post-operative management ICU Course Following Unifocalization Surgery Problems encountered - Cardiac • Low cardiac output syndrome (LCOS) – Often long cardiopulmonary bypass (CPB) times – Pharmacologic support of RV function • Monitor for tachycardia, high filling pressures (RAp > 10-12 mmHg), and poor perfusion – Dysrhythmias (sinus tachycardia, JET) • Fever control (inflammation from CPB) – Bleeding (suture lines, long CPB, coagulopathy) • Tolerate mild permissive hypotension – RV lines (relationship between RVp and SBP) • Monitor measures of cardiac output – Physical exam, bicarbonate, lactate, urine output Bronicki, & Chang, 2011 – NIRS ICU Course Following Unifocalization Surgery Problems encountered - Cardiac • Treatment – Low dose epinephrine and milrinone infusions – Delayed sternal closure – Normalization of pH and correction of alveolar hypoxia with titration of end-expiratory pressure to achieve a normal functional residual capacity • Ventilation increases RV afterload • Acidosis and hypoxemia increases RV afterload – Inhaled nitric oxide (iNO) • Judicious use of analgesia and sedation – Chemical paralysis Bronicki, & Chang, 2011 ICU Course Following Unifocalization Surgery Problems encountered – Pulmonary Figure 1: Differen al Diagnosis New or resolving pulmonary hemorrhage Diaphragm paralysis Obstruc ve breathing pa ern Lung parenchymal disease (contusion, hematoma, pneumonia, atelectasis, RPE) Airway compression Upper airway obstruc on Possible Prolonged Respiratory Failure Hemodynamic altera ons Asija et al., 2014 Stanford Pathway for TOF/PA/MAPCAs ICU Course Following Unifocalization Surgery Problems encountered - Pulmonary • Pulmonary reperfusion edema – Incidence ~ 50% – Severity of stenosis and bilateral unifocalizations are associated with development of reperfusion injury – Does not affect duration of respiratory failure and mechanical ventilation • Clinically self-limiting and does not appear to involve vascular injury Maskatia et al., 2012 Asija et al., 2013 Asija et al., 2014 Left-sided Lung Reperfusion Injury PRE-OP EARLY POST-OP POD 3 POD 4 POD 1 POD 2 DISCHARGE ICU Course Following Unifocalization Surgery Problems encountered - Pulmonary • Treatment – Judicious ventilation strategies • Permissive hypercarbia – Pulmonary toilet – Diuretic therapy ICU Course Following Unifocalization Surgery Problems encountered - Pulmonary • Pulmonary hemorrhage – Related to extensive dissection • Other parenchymal processes – Contusion, hematoma, altectasis, pneumonia *Asija et al., 2013 ICU Course Following Unifocalization Surgery Problems encountered - Pulmonary • Bronchospasm – Frequent post-op complication – > 70% of patients receive treatment for significant airflow limitation and wheezing • This is thought to be caused by the extensive dissection and disruption of lymphatics and blood vessels around the bronchopulmonary tree • Treatment – Ventilation strategies – Pulmicort, albuterol (Continuous, ATC, and PRN) – Analgesia, sedation, and at times chemical paralysis Asija et al., 2013 Maskatia et al., 2012 ICU Course Following Unifocalization Surgery Problems encountered - Variety • Phrenic nerve injury – Hilar dissection, more frequent in reoperations • Prolonged pleural fluid drainage – Likely related to lymphatic disruption from extensive mediastinal dissection • Infection – Increased incidence of 22q11 microdeletion, DiGeorge syndrome and associated immune dysfunction • Acute, severe hepatic ischemia – Low incidence (1-1.5%), but high mortality rate – Causative factor(s) unclear; no cases > 10 years ICU Course Following Unifocalization Surgery • Defense…. – Handwashing – De-intensifying – Getting them up and moving – Pulmonary toilet – Early intervention with infectious disease processes Standardize Post-operative Management CVICU Clinical Pathway Guideline (CPG) for patients with TOF/PA/MAPCAs Inclusion Criteria: All patients undergoing unifocalization surgery age > or = to 3 months with no other significant cardiac abnormalities. Includes patients undergoing partial unifocalization surgery via right or left lateral thoracotomy, with or without CPB. Includes patients with delayed sternal closure, though the guidelines are initiated on the day of sternal closure. CPG members- Ritu Asija, Catherine Krawczeski, Julie Bushnell (CVICU), Frank Hanley (CT surgery), Frandics Chan (Radiology), Sumit Bhargava (Pulm), Stan Perry (Cath), Anna Messner (ENT), Vickie Arnold (RT), Alaina Kipps (3W), Chandra Ramamoorthy (CV anesth) Monitoring Cardiac Resp Fluids Diagnostics Medications/ IV therapy POD 0 Vital signs per CVICU routine CR monitoring Central venous line LA line and RV line (if complete repair) Arterial line and pacing wires secure Breath sounds CT drainage Measure I/O, Foley CXR CBC, cardiac metabolic panel (CMP), coags, ABG q 2 hr, lactate q 2 until < 2 Antibiotic x 24 hours * [1], milrinone [2], epinephrine dopamine ** fentanyl infusion sedative infusion (midazolam or dexmetetomidine) POD 1 Vital signs per CVICU routine CR monitoring Central venous line LA line and RV line (if complete repair) Arterial line and pacing wires secure Breath sounds CT drainage Measure I/O, Foley CXR CBC, CMP, ABG q 6 and prn, lactate prn POD 2 Vital signs per CVICU routine CR monitoring Central venous line LA line and RV line (if complete repair) Arterial line and pacing wires secure Breath sounds CT drainage Measure I/O, Foley CXR BMP, Ca, Mg, PO4, ABG q 12 and prn POD 3 Vital signs per CVICU routine CR monitoring Central venous line Arterial line and pacing wires secure POD 4 Vital signs per CVICU routine CR monitoring Central venous line Arterial line and pacing wires secure POD 5 Vital signs per CVICU routine CR monitoring Central venous line Arterial line and pacing wires secure Breath sounds CT drainage Measure I/O CXR BMP, Ca, Mg, PO4, ABG q 12 and prn Breath sounds CT drainage Measure I/O CXR BMP, Ca, Mg, PO4, ABG q 12 and prn Breath sounds CT drainage Measure I/O CXR BMP, Ca, Mg, PO4 milrinone epinephrine dopamine morphine infusion sedative infusion (midazolam or dexmetetomidine) acetaminophen atc Zantac and any milrinone dopamine furosemide and additional diuretic if required to achieve negative fluid balance morphine infusion sedative infusion furosemide acetaminophen atc morphine prn Zantac and any other home antireflux meds If patient has signs of airflow obstruction, furosemide acetaminophen atc ibuprofen prn morphine prn Zantac and any other home antireflux meds If patient has signs of airflow furosemide acetaminophen prn ibuprofen prn morphine prn Zantac and any other home antireflux meds If patient has signs of airflow Asija et al., 2014 Stanford Pathway for TOF/PA/MAPCAs Daily Goal Directed Therapy Daily Goals POD 0 POD 1 POD 2 POD 3 Cardiac and respiratory stability Initiate diuresis with goal negative fluid balance for next morning Wean ventilator Continue Continue diuresis diuresis Discontinue Extubate intracardiac lines Wean ventilator Wean inotropes POD 4 POD5 Nutrition Pulmonary toilet Wean NIPPV as tolerated Nutrition Pulmonary toilet Wean NIPPV as tolerated Asija et al., 2014 Stanford Pathway for TOF/PA/MAPCAs Specific Areas for Nursing Focus • Keeping everyone’s eye on the prize – Personal patient safety officer – Nursing assessment is key! • Establishment of physical and occupational therapy – Feeding evaluation – Movement/activities of daily living • Get them up and moving as soon as it is safe • Pulmonary toilet Specific Areas for Nursing Focus • Nutrition – Early enteral feeds • Comfort management – Standardized comfort management protocols – Pharmacologic and nonpharmacologic supports • Family education – Starts pre-operatively and goes throughout their child’s hospitalization Important Considerations with Family Education • “Tending the garden” – pulmonary vascular bed (long-term management) – Growth and development of pulmonary bed is commensurate with visceral growth – Lung perfusion scans • Hospital discharge • Before further surgical interventions – Surveillance cardiac catheterization (6 months to 1 year) to assess the pulmonary vasculature – Echocardiograms should be done routinely to monitor Asija et al., 2014 right ventricular function Hanley, 2010 Mainwaring et al., 2013 Important Considerations with Family Education • Children undergone unifocalization surgery without intracardiac repair have aortopulmonary shunts providing PBF – At risk for specific complications • Thrombosis or pulmonary overcirculation • Require close monitoring for any changes in their oxygen saturation or ventricular overload Asija et al., 2014 Important Considerations with Family Education • Complete intracardiac repair – Monitored for recurrent PA stenosis, right ventricle-to-pulmonary artery conduit stenosis or insufficiency, and RV hypertension/dilation • Endocarditis prophylaxis – 6 months after repair, and beyond that for patients who have repairs that include a prior episode of endocarditis, and have residual intracardiac shunt who remain cyanotic or have patch leaks Asija et al., 2014 Thank you! I would like to acknowledge the work and support of my colleagues at Lucile Packard Children’s Hospital Stanford Frank Hanley MD V. Mohan Reddy MD Stephen Roth MD, MPH Ritu Asija MD