Hypoplastic left heart syndrome (HLHS) is a rare and severe congenital cardiac defect. Approximately 1000 infants are born with HLHS in the United States every year. Healthcare collaboratives over the last decade have focused on sharing patient experiences and techniques in an effort to improve outcomes. In 2010, cardiologists and patient families joined together to improve the care of HLHS patients by forming the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). Sixty-six of the approximately 110 institutions caring for patients with HLHS in the United States and Canada are now members of NPC-QIC. In 2017, cardiovascular perfusionists joined the collaborative as another specialty involved in the care of HLHS patients. Perfusionists and cardiac surgeons developed the collaborative’s first conduct of perfusion survey for the Norwood Stage 1 procedure, specifically targeting the provision of cardiopulmonary bypass for patients with HLHS. This manuscript discusses the results of this survey, unveiling a significant variance in the conduct of perfusion for this patient population.
ParkerSEMaiCTCanfieldMAet al. Updated National Birth Prevalence estimates for selected birth defects in the United States, 2004–2006. Birth Defects Research. Part A, Clinical and Molecular Teratology2010; 88: 1008–1016.
2.
WangYLiuGCanfieldMAet al. Racial/ethnic differences in survival of United States children with birth defects: a population-based study. J Pediatrics2015; 166: 819–826.e1–2.
3.
McGuirkSPGriselliMStumperOFet al. Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience. Heart2006; 92: 364–370.
4.
MahleWTSprayTLWernovskyGGaynorJWClarkBJ3rd. Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation2000; 102: Iii136–141.
5.
AndersonJBBeekmanRH3rdKuglerJDet al. Improvement in interstage survival in a national pediatric cardiology learning network. Circ Cardiovasc Qual Outcomes2015; 8: 428–436.
ReagorJAGaoZLombardiJPMillinBBTweddellJSCooperDS.Accuracy of the Spectrum Medical M4 and Terumo CDI 500 compared to the Radiometer ABL90 FLEX benchtop blood analyzer. Perfusion2017; 32: 523–528.
8.
SouthworthRSuttonRMizeSet al. Clinical evaluation of a new in-line continuous blood gas monitor. J Extra Corpor Technol1998; 32: 166–170.
9.
SchreurANilesSPloesslJ.Use of the CDI blood parameter monitoring system 500 for continuous blood gas measurement during extracorporeal membrane oxygenation simulation. J Extra Corpor Technol2005; 37: 377–380.
10.
FriedDWLeoJMattioniGJet al. CDI Blood Parameter Monitoring System 500–a new tool for the clinical perfusionist. J Extra Corpor Technol2000; 32: 25–30.
11.
SommersteinRSchreiberPWDiekemaDJet al. Mycobacterium chimaera outbreak associated with heater-cooler devices: piecing the puzzle together. Infect Control Hosp Epidemiol2016; 38: 103–108.
12.
MatteGS.Perfusion for congenital heart surgery. Notes on Cardiopulmonary Bypass for a Complex Patient Population. Hoboken, New Jersey: John Wiley & Sons, Inc.; 2015.
13.
GravleeGPDavisRFHammonJKussmanB.Cardiopulmonary Bypass and Mechanical Support: Principles and Practice. 4th ed.Philadelphia, Pennsylvania: Lippincott Williams & Wilkins; 2015.
14.
DreherMGlatzACKennedyARosenthalTGaynorJW.A single-center analysis of methylprednisolone use during pediatric cardiopulmonary bypass. J Extra Corpor Technol2015; 47: 155–159.
SchmidtBSJordanJELaneMRet al. Timing of adding blood to prime affects inflammatory response to neonatal cardiopulmonary bypass. Cardiol Young2017: 27: 480–487.
17.
Operators Manual, CDI Blood Parameter Monitoring System 500.236603J ed. Ann ArborMichigan: Terumo Cardiovascular System Corporation; 2015.
18.
GuzzettaNAMonitzHGFernandezJDFazlollahTMKnezevicAMillerBE.Correlations between activated clotting time values and heparin concentration measurements in young infants undergoing cardiopulmonary bypass. Anesth Analg2010; 111: 173–179.
19.
PiKD.Lack of correction between activated clotting time and plasma heparin during cardiopulmonary bypass. Ann Surg1983; 198: 115.
20.
DespotisGJSummerfieldALJoistJHet al. Comparison of activated coagulation time and whole blood heparin measurements with laboratory plasma anti-Xa heparin concentration in patients having cardiac operations. J Thorac Cardiovasc Surg1994; 108: 1076–1082.
21.
Shore-LessersonLBakerRAFerrarisVAet al. The Society of Thoracic Surgeons, The Society of Cardiovascular Anesthesiologists, and The American Society of ExtraCorporeal Technology: Clinical Practice Guidelines*—Anticoagulation During Cardiopulmonary Bypass. Ann Thorac Surg2018; 105: 650–662.
22.
GuzzettaNAMillerBEToddKSzlamFMooreRHTosoneSR.An evaluation of the effects of a standard heparin dose on thrombin inhibition during cardiopulmonary bypass in neonates. Anesth Analg2005; 100: 1276–1282.
23.
MatteGSdel NidoPJ.History and use of del Nido cardioplegia solution at Boston Children’s Hospital. J Extra Corpor Technol2012; 44: 98–103.
24.
KotaniYTweddellJGruberPet al. Current cardioplegia practice in pediatric cardiac surgery: a North American multiinstitutional survey. Ann Thorac Surg2013; 96: 923–929.
25.
PreusseCJ.Custodiol cardioplegia: a single-dose hyperpolarizing solution. J Extra Corpor Technol2016; 48: 15–20.
26.
ModiPImuraHCaputoMet al. Cardiopulmonary bypass-induced myocardial reoxygenation injury in pediatric patients with cyanosis. J Thorac Cardiovasc Surg2002; 124: 1035–1036.
27.
del NidoPJBensonLNMickleDAKielmanowiczSColesJGWilsonGJ.Impaired left ventricular postischemic function and metabolism in chronic right ventricular hypertrophy. Circulation1987; 76: 168–173.
28.
del NidoPJMickleDAWilsonGJet al. Inadequate myocardial protection with cold cardioplegic arrest during repair of tetralogy of Fallot. J Thorac Cardiovasc Surg1988; 95: 223–229.
29.
CaputoMMokhtariARogersCAet al. The effects of normoxic versus hyperoxic cardiopulmonary bypass on oxidative stress and inflammatory response in cyanotic pediatric patients undergoing open cardiac surgery: a randomized controlled trial. J Thorac Cardiovasc Surg2009; 138: 206–214.
30.
CaputoMMokhtariAMiceliAet al. Controlled reoxygenation during cardiopulmonary bypass decreases markers of organ damage, inflammation, and oxidative stress in single-ventricle patients undergoing pediatric heart surgery. J Thorac Cardiovasc Surg2014; 148: 792–801.
31.
EngelmanRBakerRALikoskyDSet al. The Society of Thoracic Surgeons, The Society of Cardiovascular Anesthesiologists, and The American Society of ExtraCorporeal Technology: Clinical Practice Guidelines for Cardiopulmonary Bypass—temperature management during cardiopulmonary bypass. J Extra Corporeal Technol2015; 47: 145–154.
32.
NussmeierNAChengWMarinoMet al. Temperature during cardiopulmonary bypass: the discrepancies between monitored sites. Anesth Analg2006; 103: 1373–1379.
33.
ShannKGLikoskyDSMurkinJMet al. An evidence-based review of the practice of cardiopulmonary bypass in adults: a focus on neurologic injury, glycemic control, hemodilution, and the inflammatory response. J Thorac Cardiovasc Surg2006; 132: 283–290.
34.
NewlandRFTullyPJBakerRA.Hyperthermic perfusion during cardiopulmonary bypass and postoperative temperature are independent predictors of acute kidney injury following cardiac surgery. Perfusion2013; 28: 223–231.
35.
EnomotoSHindmanBJDexterFSmithTCutkompJ.Rapid rewarming causes an increase in the cerebral metabolic rate for oxygen that is temporarily unmatched by cerebral blood flow. A study during cardiopulmonary bypass in rabbits. Anesthesiology1996; 84: 1392–1400.
36.
DavisPJCladisEKMotoyamaEK.Smith’s Anesthesia for Infants and Children. 8th ed: Elsevier Health Sciences; 2011.
37.
OppidoGNapoleoneCPTurciSet al. Moderately hypothermic cardiopulmonary bypass and low-flow antegrade selective cerebral perfusion for neonatal aortic arch surgery. Ann Thorac Surg2006; 82: 2233–2239.
38.
KilpackVDStayerSAMcKenzieEDFraserCDJr.AndropoulosDB.Limiting circulatory arrest using regional low flow perfusion. J Extra Corpor Technol2004; 36: 133–138.
39.
FraserCDAndropoulosDB.Principles of antegrade cerebral perfusion during arch reconstruction in newborns/infants. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu2008: 61–68.
40.
GuoZHuR-JZhuD-MZhuZ-QZhangH-BWangW.Usefulness of deep hypothermic circulatory arrest and regional cerebral perfusion in children. Ther Hypothermia Temp Manag2013; 3: 126–131.
41.
LyMRoubertieFBelliEet al. Continuous cerebral perfusion for aortic arch repair: hypothermia versus normothermia. Ann Thorac Surg2011; 92: 942–948.
42.
SpielvogelDTangGHL. Selective cerebral perfusion for cerebral protection: what we do know?Ann Cardiothorac Surg2013; 2: 326–330.
43.
HalsteadJCMeierMWurmMet al. Optimizing selective cerebral perfusion: deleterious effects of high perfusion pressures. J Thorac Cardiovasc Surg2008; 135: 784–791.
44.
WilliamsGDRamamoorthyCChuLet al. Modified and conventional ultrafiltration during pediatric cardiac surgery: clinical outcomes compared. J Thorac Cardiovasc Surg2006; 132: 1291–1298.
45.
RajaSGYousufuddinSRasoolFNubiADantonMPollockJ.Impact of modified ultrafiltration on morbidity after pediatric cardiac surgery. Asian Cardiovasc Thorac Ann2006; 14: 341–350.
46.
CholetteJMPowersKSAlfierisGMet al. Transfusion of cell saver salvaged blood in neonates and infants undergoing open heart surgery significantly reduces RBC and coagulant product transfusions and donor exposures: results of a prospective, randomized, clinical trial. Pediatr Crit Care Med2013; 14: 137–147.
