One Hundred Useful References in Pediatric Cardiac Intensive Care: The 2012 Update
David M. Axelrod, MD1; Darren Klugman, MD, MS2; Gail E. Wright, MD1; Anthony Chang, MD, MBA, MPH3; Ronald Bronicki, MD4; Stephen J. Roth, MD, MPH1; and the Board Members of the Pediatric Cardiac Intensive Care Society
Objective: The specialty of pediatric cardiac critical care has undergone rapid scientific and clinical growth in the last 25 years. The Board of Directors of the Pediatric Cardiac Intensive Care Society assembled an updated list of sentinel references focused on the critical care of children with congenital and acquired heart disease. We encouraged board members to select articles that have influenced and informed their current practice or helped to establish the standard of care. The objective of this article is to provide clinicians with a compilation and brief summary of these updated 100 useful references.
Data Sources: The list of “One Hundred Useful References for Pediatric Cardiac Intensive Care” (2004) and relevant literature to the practice of cardiac intensive care.
Data Selection: A subset of Pediatric Cardiac Intensive Care Society board members compiled the initial list of useful references in 2004, which served as the basis of the new updated list. Suggestions for relevant articles were submitted by the Pediatric Cardiac Intensive Care Society board members and selected pediatric cardiac intensivists with an interest in this project following the Society’s meeting in 2010. Articles were considered for inclusion if they were named in the original list from 2004 or were suggested by Pediatric Cardiac Intensive Care Society board members and published before December 31, 2011.
Data Extraction: Following submission of the complete list by the Pediatric Cardiac Intensive Care Society board and contributing Society members, articles were complied by the two co-first authors (D.A., D.K.). The authors also performed Medline searches to ensure comprehensive inclusion of all relevant articles. The final list was then submitted to the Pediatric Cardiac Intensive Care Society board members, who ranked each publication.
Data Synthesis: Rankings were compiled and the top 100 articles with the highest scores were selected for inclusion in this publication. The two co-first authors (D.A., D.K.) reviewed all existing summaries and developed summaries of the newly submitted articles.
Conclusions: An updated compilation of 100 useful references for the critical care of children with congenital and acquired
heart disease has been compiled and summarized here. Clinicians and trainees may wish to use this document as a reference for education in this complex and challenging subspecialty. (Pediatr Crit Care Med 2013; 14:770–785)
Key Words: literature review; pediatric cardiac intensive care; pediatric cardiac surgery; pediatric cardiology; pediatric intensive care
The original list of “One Hundred Useful References in Pediatric Cardiac Intensive Care” was complied by Anthony Chang, MD, MBA, and the Board of Directors of the Pediatric Cardiac Intensive Care Society (PCICS) in 2004. It was distributed at the Fifth International PCICS conference in December 2004 in Miami, Florida. Revision and updating of the list began in 2009 at the request of the PCICS board. The purpose of this revision is to create an updated list of publications we think reflects the significant progress made in the field of pediatric cardiac intensive care and guides current diagnostic and management principles.
METHODS
Using the original compilation as a guide, PCICS board members were asked to submit new articles to be voted upon for inclusion in this revision. Articles were considered for inclusion if they were named in the original list from 2004 or were submitted by PCICS board members and published before December 31, 2011. We excluded published guidelines from major national organizations (e.g., American Heart Association). Two authors (D.A., D.K.) collected these newly suggested articles and the original 100 articles in a spreadsheet. Using the original articles as cross-reference sources, the two lead authors conducted an additional Medline search to ensure a comprehensive list of articles. Subsequently, three authors (D.A., D.K., S.J.R.) evaluated each article for its current relevance and selected the most current and relevant articles (n = 134). This list included 50 articles from the original list and 84 new articles suggested by the board. The list of 134 articles was initially reviewed by the senior author of the prior list (A.C.) and another senior member of the PCICS board who initiated this current revision (R.B.) and then subsequently e-mailed to all PCICS board members.
In an effort to provide an objective measure of each article’s impact on the field, a score was assigned to each article based on the PCICS board members’ votes. Votes were cast in the following manner: for each article, a vote of “1” signified “definitely include the article in the revised list,” “2” signified “strongly consider for inclusion,” and “3” signified “a great article but not in the Top 100.” Average votes were then calculated as the overall article score. Ninety-three articles qualified for inclusion (scores ranged from 1 to 1.86) and 18 articles tied with an average score of 2.0. The 18 tied articles were ranked based on the impact factor of the journal in which they were published, and the final seven articles were selected based on the impact factor. The final 100 articles comprise 43 articles from the original 2004 list and 57 articles newly submitted by the board.
Limitations of this publication include the inherent subjective nature of selecting a list of “best” articles. We encouraged board members to select articles that have influenced and informed their current practice or helped to establish the standard of care. Although we attempted to evaluate each article objectively with the scoring system described above, there is no truly objective measure of an individual article’s value to our field. No list of articles is inclusive; this list of 100 important references should be viewed as a starting point of notable references for the interested reader.
SUMMARY
The following articles represent the revised list of 100 important references from the PCICS, in alphabetical order of the first author’s last name. A brief summary of each article is provided; newly included articles were summarized by the two cofirst authors (D.A., D.K.).
1. Allan CK, Thiagarajan RR, del Nido PJ, et al: Indication for initiation of mechanical circulatory support impacts survival of infants with shunted single-ventricle circulation supported with extracorporeal membrane oxygenation. J Thorac Cardiovasc Surg 2007; 133:660–667
This single-center retrospective review seeks to identify risk factors for survival in shunted single-ventricle patients less than 1 year old supported with mechanical circulatory support. Forty-four infants were included; patients with the Sano modification were excluded and divided into two groups based on indication for extracorporeal membrane oxygenation (ECMO) use: those who were cannulated for circulatory collapse and those who were cannulated for hypoxemia. The majority of patients (55%) had hypoplastic left heart syndrome (HLHS), and the most common procedure performed was the Norwood operation (n = 29, 66%). Survival to hospital discharge was 48%. Of patients cannulated for hypoxemia, 81% survived to discharge as compared with only 29% survival for those cannulated for circulatory collapse. This difference persisted on multivariate logistic regression testing (95% CI, 1.2–99.9, p = 0.04). The survival reported in this series is similar to larger studies of ECMO use in postoperative cardiac surgical patients and suggests that in certain clinical situations, shunted single ventricle-anatomy should not be considered a contraindication to postoperative ECMO support.
2. Almond CS, Thiagarajan RR, Piercey GE, et al: Waiting list mortality among children listed for heart transplantation in the United States. Circulation 2009; 119:717–727
This is a multicenter cohort study using the U.S. Scientific Registry of Transplant Recipients including all patients younger than 18 years listed only for heart transplant from 1999 to 2006. A total of 3,098 patients were included, and 17% of patients (533) died waiting for transplant. Multivariate analysis for predictors of waiting list mortality identified ECMO, ventilatory support, need for dialysis support, nonwhite race/ethnicity, listing status 1A, and congenital heart disease (CHD) as risk factors. The level of invasive hemodynamic support required is the most important patient-related factor associated with waiting list mortality. Children with a more than or equal to 20% risk of waiting list mortality included most children with CHD listed status 1A and most children who required ECMO support or mechanical ventilation. This study suggests the need for alternate methods of stratifying pediatric patients awaiting heart transplantation given the current heterogeneity among the patients listed as status 1A.
3. Anand KJ, Hickey PR: Pain and its effects in the human neonate and fetus. N Engl J Med 1987; 317:1321–1329
This review provides background information on the developmental aspects of pain perception and the physiologic cardiorespiratory, hormonal, and metabolic and behavioral changes associated with pain in neonates.
4. Atz AM, Adatia I, Wessel DL: Rebound pulmonary hypertension after inhalation of nitric oxide. Ann Thorac Surg 1996; 62:1759–1764
This study described the hemodynamic response to initiation and withdrawal of inhaled nitric oxide in nine infants after total anomalous pulmonary venous connection repair and showed a decrease in mean pulmonary artery pressure from 35.6 to 23.7 mm Hg. However, all infants had a rebound pulmonary hypertension response upon cessation of therapy that dissipated within 60 minutes.
5. Barrett CS, Bratton SL, Salvin JW, et al: Neurological injury after extracorporeal membrane oxygenation use to aid pediatric cardiopulmonary resuscitation. Pediatr Crit Care Med 2009; 10:445–451
This study is a retrospective review of all children younger than 18 years requiring ECMO during cardiopulmonary resuscitation (E-CPR) from 1999 to 2005 using the Extracorporeal Life Support Organization (ELSO) registry to assess risk factors for neurologic injury. Of the 695 E-CPR events, 70% had cardiac disease and 90% were supported with venoarterial ECMO. Twenty-two percent of the patients had evidence of neurologic injury by radiographic imaging. Risk factors for neurologic injury following E-CPR in this series included lower pre-ECMO pH, need for cardiopulmonary resuscitation (CPR) on ECMO, pulmonary hemorrhage, or renal failure requiring dialysis on ECMO. Cardiac disease and higher pre-ECMO arterial pH had decreased odds of neurologic injury. Neurologic outcomes over the time period studied showed a trend toward improvement.
6. Beca J, Gunn J, Coleman L, et al: Pre-operative brain injury in newborn infants with transposition of the great arteries occurs at rates similar to other complex congenital heart disease and is not related to balloon atrial septostomy. J Am Coll Cardiol 2009; 53:1807–1811
This study of 64 infants with complex CHD including d-transposition of the great arteries (d-TGA), HLHS, and pulmonary atresia sought to define the brain injuries noted in d-TGA compared with other lesions and the risk factors for brain injury following balloon atrial septostomy (BAS) in those children. Among all patients studied, 30% had evidence of preoperative brain injury on MRI. The prevalence was not greater in patients with d-TGA as compared with other diagnostic groups, and there was no association between BAS and brain injury. No risk factors were identified for brain injury in the group studied, and risk was thought to be multifactorial.
7. Bellinger DC, Jonas RA, Rappaport LA, et al: Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. N Engl J Med 1995; 332:549–555
This study of 155 neonates randomized to either circulatory arrest of low-flow bypass showed that those assigned to circulatory arrest had a lower mean score on the Psychomotor Development Index of the Bayley Scales of Infant Development (specifically, a 6.4-point deficit) and that perioperative electro-encephalogram (EEG) seizure activity was also associated with a lower score.
8. Bernstein D, Naftel D, Chin C, et al; Pediatric Heart Transplant Study: Outcome of listing for cardiac transplantation for failed Fontan: A multi-institutional study. Circulation 2006; 114:273–280
This is a retrospective review of 97 Fontan patients listed for heart transplantation between 1993 and 2001. Actuarial survival at 1 year following transplantation of patients with failing Fontan circulation (77%) was not statistically different from patients without CHD or patients with CHD who underwent at least one prior surgery. Younger patients, patients younger than 6 months from Fontan operation, and patients who were status 1A or on mechanical ventilation were more likely to die while awaiting transplantation. Hazard analysis demonstrated that most Fontan patients who die awaiting transplantation do so in the first 6 months. Although this cohort represents patients managed during a time period when mechanical circulatory support options were different than the current era, these data support ongoing use of transplantation as a management option for failing Fontan patients.
9. Bizzarro M, Gross I: Inhaled nitric oxide for the postoperative management of pulmonary hypertension in infants and children with congenital heart disease. Cochrane Database Syst Rev 2005; 4:CD005055
All studies of nitric oxide use in infants and children younger than 14 years were considered for this study. For inclusion, the only studies considered were those using randomization or quasi-randomization with nitric oxide and placebo for postoperative patients. The small number of studies in children of this type limited the analysis to four studies which met criteria. These studies did not demonstrate any statistically significant difference in hospital mortality, mean pulmonary artery pressure, cardiac output, heart rate, number of pulmonary hypertensive crises, or PaO2:FiO2 ratio in the postoperative period. No data were available on neurodevelopmental outcomes or length of ICU or hospital stay. The results of this review suggest a need for well-designed randomized controlled trials with clinically relevant endpoints in more homogeneous patient populations.
10. Blume ED, Naftel DC, Bastardi HJ, et al; Pediatric Heart Transplant Study Investigators: Outcomes of children bridged to heart transplantation with ventricular assist devices: A multi-institutional study. Circulation 2006; 113:2313–2319
Using the Pediatric Heart Transplant Study database, 99 patients younger than 18 years old listed for transplantation between 1993 and 2003 were compared against 2,276 patients listed without ventricular assist device (VAD) implantation. Of the patients supported with VADs, median age at transplantation was 13.3 years, and 21 had CHD. Over the study period, the number of patients with VAD support at time of listing and transplantation increased, as did the use of long-term support devices. Seventy-seven patients were successfully bridged to transplantation and 17 died awaiting transplantation. In patients who died awaiting transplant with VAD support, 65% suffered strokes, and the hazard function was greatest immediately following implantation. Mean length of support was 57 days, and survival after transplantation was not different when compared with status 1 patients not supported with VADs. Multivariate analysis demonstrated female gender, CHD, and earlier year of implantation as risk factors for death while awaiting transplantation.
11. Bohn D, Macrae D, Chang AC: Acute viral myocarditis: Mechanical circulatory support. Pediatr Crit Care Med 2006; 7(Suppl):S21–S24
This meta-analysis identified 39 manuscripts describing the use of mechanical circulatory support for children younger than 18 years old with acute myocarditis. Patients were divided into two groups based on mechanical circulatory support received, cannulation method, and outcomes. Analysis of the literature demonstrated survival to discharge as high as 60% in some reports. Although outcomes are worse for patients placed on mechanical support during cardiac arrest, most series support the use of mechanical circulatory support in these circumstances for 10–14 days. The growing body of literature supports mechanical circulatory support as a primary therapy for patients with acute viral myocarditis, allowing time for recovery before listing for heart transplantation.
12. Booth KL, Roth SJ, Perry SB, et al: Cardiac catheterization of patients supported by extracorporeal membrane oxygenation. J Am Coll Cardiol 2002; 40:1681–1686
This single-center retrospective review investigated the indications and outcomes of 192 patients supported by 216 ECMO courses. The median age at cardiac catheterization was 22 days, and the median weight was 4.3 kg; a total of 60 catheterizations were performed with only two complications (both myocardial perforations controlled with a pericardial drain). Over half of the catheterizations were performed for low cardiac output, and the median time to catheterization was 1 day. Thirty-five percent of catheterizations were performed to assess operative results. Findings at catheterization led to subsequent intervention in 83% of cases, especially in those placed on ECMO in the postoperative period. Thirty-nine patients (72%) were successfully decannulated and 48% survived to discharge. The authors concluded that cardiac catheterization can be safely performed on patients supported with ECMO and can provide important diagnostic information which guides necessary interventions.
13. Booth KL, Roth SJ, Thiagarajan RR, et al: Extracorporeal membrane oxygenation support of the Fontan and bidirectional Glenn circulations. Ann Thorac Surg 2004; 77:1341–1348
This single-center retrospective review of 20 patients with cavopulmonary connections supported with ECMO shows variable outcomes. Of the 20 patients, 14 were Fontan patients and six were bidirectional Glenn (BDG) patients. Ten patients were decannulated and eight survived to discharge with an overall survival at a median follow up of 35 months of 30%. All four adult Fontan patients were withdrawn from support, and the only BDG survivor received a heart transplant from ECMO. Severe neurologic injury occurred in 25% of patients. The authors suggest early initiation of ECMO support and early decompression of the superior vena cava with optimization of venous drainage to maximize survival and neurologic outcomes.
14. Bradley SM, Simsic JM, Mulvihill DM: Hypoventilation improves oxygenation after bidirectional superior cavo-pulmonary connection. J Thorac Cardiovasc Surg 2003; 126:1033–1039
This study in 15 infants who underwent bidirectional cavopulmonary anastomosis showed that hypoventilation to a mean PaCO2 of 58 mm Hg increased both arterial PaO2 from 50 to 61 mm Hg and systemic oxygen saturation from 86% to 90%. Hypoventilation also increased cerebral blood flow, increased blood flow velocity, and decreased arteriovenous oxygen saturation difference across the upper body. This maneuver led to only a small increase in transpulmonary gradient from 6 to 8 mm Hg.
15. Braudis NJ, Curley MA, Beaupre K, et al: Enteral feeding algorithm for infants with hypoplastic left heart syndrome poststage I palliation. Pediatr Crit Care Med 2009; 10:460–466
This study evaluates the impact of a standardized enteral feeding algorithm following stage I palliation for HLHS. Thirty-six neonates older than 35 weeks’ gestation and more than 2 kg were prospectively enrolled and compared with a historical cohort of 27 matched infants. All infants with a history of fetal intervention, necrotizing enterocolitis (NEC), ECMO, or death were excluded. Infants fed with a standardized algorithm had statistically fewer days of total parenteral nutrition and achieved the recommended daily allowance of calories in shorter time. There were no episodes of NEC in the study group compared with three episodes in the historical controls.
16. Bridges ND, Mayer JE Jr, Lock JE, et al: Effect of baffle fenestration on outcome of the modified Fontan operation. Circulation 1992; 86:1762–1769
This study assessed the effect of baffle fenestration on outcome in 91 patients versus 56 patients who did not have fenestrations. The results showed that durations of pleural effusions and hospitalization were shorter and systemic venous pressures were lower (12.6 vs 14.3 mm Hg) in the fenestration group, despite the fenestrated group having significantly higher preoperative risks.
17. Bronicki RA, Backer CL, Baden HP, et al: Dexamethasone reduces the inflammatory response to cardiopulmonary bypass in children. Ann Thorac Surg 2000; 69:1490–1495
This randomized, double-blind, placebo-controlled trial of 29 patients undergoing cardiopulmonary bypass evaluated postoperative inflammatory markers and clinical data to determine anti-inflammatory benefits of preoperative dexamethasone use. Patients were randomized to receive 1 mg/kg dexamethasone versus placebo approximately 1 hour prior to the initiation of cardiopulmonary bypass. Complement levels were not significantly different between the two groups. Patients who received preoperative dexamethasone had decreased length of cardiac ICU stay, ventilator days, and fluid requirements.
18. Bronicki RA, Chang AC: Management of the postoperative pediatric cardiac surgical patient. Crit Care Med 2011; 39:1974–1984
This comprehensive review of the postoperative care of the pediatric postoperative cardiac patient focuses on maintenance of cardiac output as surgical and patient complexity continue to increase.
19. Brown ML, Dearani JA, Danielson GK, et al; Mayo Clinic Congenital Heart Center: The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 2008; 135:1120–1136, 1136.e1–1136.e7
This comprehensive review of nearly 600 Ebstein patients operated on in a single institution between 1972 and 2006 sought to define determinants of operative and long-term mortality and factors for reoperation and to compare outcomes of tricuspid valve repair versus valve replacement. The mean age of this patient population was 24.1 years, and most patients had a tricuspid valve replacement performed. Multiple preoperative and postoperative factors contributed to increased mortality, whereas ablation of accessory pathways and sinus rhythm at discharge portended decreased mortality. Late survival was associated with preoperative sinus rhythm and accessory pathway ablation. Male sex, right ventricular outflow tract obstruction, more severe forms of disease, and cyanosis were all associated with worse outcomes. Early mortality was 5.9% and late survival at 10 years was 84.7%.
20. Chang AC, Zucker HA, Hickey PR, et al: Pulmonary vascular resistance in infants after cardiac surgery: Role of carbon dioxide and hydrogen ion. Crit Care Med 1995; 23:568–574
This is a clinical investigation to define the roles of carbon dioxide and hydrogen ion in pulmonary vascular resistance. The study demonstrated that increasing arterial pH with administration of sodium bicarbonate lowered pulmonary arterial pressure and increased cardiac output, thus lowering pulmonary vascular resistance from 6.0 to 3.1 WU.
21. Charpie JR, Dekeon MK, Goldberg CS, et al: Serial blood lactate measurements predict early outcome after neonatal repair or palliation for complex congenital heart disease. J Thorac Cardiovasc Surg 2000; 120:73–80
This prospective study of 46 neonates who had complex cardiac surgery showed that patients with poor outcome had a greater initial mean lactate level of 9.4 versus 5.6 mmol/L and that a rise in serum lactate level of 0.75 mmol/L/hr was associated with a poor outcome.
22. Cholette JM, Rubenstein JS, Alfieris GM, et al: Elevated risk of thrombosis in neonates undergoing initial palliative cardiac surgery. Ann Thorac Surg 2007; 84:1320–1325
This single-center study evaluated the frequency of thrombosis in 22 neonates with single-ventricle physiology undergoing palliative procedures. The majority of patients underwent a Norwood operation with right ventricle-to-pulmonary artery (RVPA) conduit as the procedure evaluated. Thrombus was identified in five patients; eight infants died and thrombus was identified in only one infant.
23. Cournand A, Motley HL: Physiological studies of the effects of intermittent positive pressure breathing on cardiac output in man. Am J Physiol 1948; 152:162–174
This landmark study describes the impact of positive pressure ventilation on right heart filling, pressures, and cardiac output. Using Swan-Ganz catheters and invasive arterial pressure monitoring, 29 healthy adults were studied with three types of intermittent positive pressure ventilators, and 33 measurements demonstrated reliable and reproducible decreases in cardiac output which correlated with changes in right ventricular pressures. The physiologic alterations described are important interactions which need to be well understood in the care of children with heart disease.
24. Cullen S, Shore D, Redington A: Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot. Restrictive physiology predicts slow postoperative recovery. Circulation 1995; 91:1782–1789
This prospective Doppler study in 35 patients after repair for tetralogy of Fallot showed that 17 of 35 patients demonstrated restrictive right ventricular physiology characterized by pulmonary arterial antegrade flow during diastole and that this flow was abolished or greatly diminished during the inspiratory phase.
25. de Leeuw M, Williams JM, Freedom RM, et al: Impact of diaphragmatic paralysis after cardiothoracic surgery in children. J Thorac Cardiovasc Surg 1999; 118:510–517
This is a retrospective study of 160 children who had diaphragmatic paralysis after cardiac surgery. There was an incidence of 1.6% of bilateral hemidiaphragmatic paralysis requiring mechanical ventilation, especially in smaller patients. This may represent a higher risk subgroup.
26. Dent CL, Spaeth JP, Jones BV, et al: Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion. J Thorac Cardiovasc Surg 2005; 130:1523–1530
This study evaluates the early postoperative neurologic impact of low-flow regional cerebral perfusion for patients undergoing the Norwood operation. Most patients had a preoperative brain MRI and EEG followed by the same studies in the early postoperative period when stable. The majority of patients underwent a Norwood with Sano modification. Preoperative pulmonary overcirculation was managed with hypoxic gas mixtures. Preoperative brain MRI revealed ischemic injury in 5 of 22 patients (23%); none had anatomic abnormalities, and all patients had normal neurologic examinations and EEG results. Fifteen patients underwent postoperative MRI, and 11 of 15 patients (73%) showed new or worsened lesions, including all of whom had abnormal preoperative MRI studies. On analysis for risk factors for new postoperative lesions, only cerebral oximetry of less than 45% for longer than 180 minutes demonstrated statistical significance.
27. Duncan BW, Desai S: Pulmonary arteriovenous malformations after cavopulmonary anastomosis. Ann Thorac Surg 2003; 76:1759–1766
This is an extensive review of the proposed pathophysiologic mechanisms for formation of pulmonary arteriovenous malformations in single-ventricle patients. Multiple animal and human studies in patients with and without cyanotic CHD are reviewed.
28. Flori HR, Johnson LD, Hanley FL, et al: Transthoracic intracardiac catheters in pediatric patients recovering from congenital heart defect surgery: Associated complications and outcomes. Crit Care Med 2000; 28:2997–3001
In this descriptive study of 523 intracardiac catheters, younger age (age < 3 mo), catheter location (left atrial and pulmonary artery), and platelet count of less than 50,000 were identified as risk factors for complications after multivariate logistic regression analysis.
29. Fogel MA, Durning S, Wernovsky G, et al: Brain versus lung: Hierarchy of feedback loops in single-ventricle patients with superior cavopulmonary connection. Circulation 2004; 110(11 Suppl 1):II147–II152
This velocity mapping study of 12 infants after bidirectional cavopulmonary anastomosis showed that with hypercarbia, flow to the brain and lungs increased from 1.5 to 2.7 L/min/m2, PaO2 improved from 48 to 60 mm Hg, and cardiac index (CI) increased from 4.3 to 5.4 L/min/m2, all indicating that cerebral feedback loop overrides the pulmonary control system.
30. Gentles TL, Mayer JE Jr, Gauvreau K, et al: Fontan operation in five hundred consecutive patients: Factors influencing early and late outcome. J Thorac Cardiovasc Surg1997; 114:376–391
This is a single-center retrospective review of the first 500 Fontan operations performed between 1973 and 1991. Although surgical techniques, operative care, and postoperative ICU care have evolved significantly since this time period, these results provide a foundation for much of our current risk stratification. The primary outcome measure was survival with intact Fontan circuit; failure was classified as Fontan takedown, death, or transplantation. Age at operation and prevalence of early failure decreased, whereas complexity of anatomic variants increased over the time period studied. Multivariate analysis demonstrated a significant association between early failure and a mean preoperative pulmonary artery pressure of 19 mm Hg or more, age less than 4 years at the time of the Fontan operation, a diagnosis of heterotaxy syndrome, a right-sided tricuspid valve as the only systemic atrioventricular valve, and pulmonary artery distortion. A baffle fenestration was associated with a reduced probability of failure.
31. Giglia TM, Bronicki R, Checchia PA, et al: Critical treatment strategies for acute pulmonary hypertension in infants and children: Pediatric Cardiac Intensive Care Society Scientific Statement. Pediatr Crit Care Med 2010; 11(2 Suppl):S3
This comprehensive review of current pulmonary hypertension therapies in pediatrics is the work of the PCICS following presentations at the Seventh International Conference of the Society. All therapies are graded on scientific level of evidence; all adult and pediatric studies are reviewed; and management recommendations are made by an international panel of experts.
32. Goldberg CS, Bove EL, Devaney EJ, et al: A randomized clinical trial of regional cerebral perfusion versus deep hypothermic circulatory arrest: Outcomes for infants with functional single ventricle. J Thorac Cardiovasc Surg 2007; 133:880–887
This prospective single-center study randomized 77 patients undergoing the Norwood operation to receive regional cerebral perfusion versus deep hypothermic circulatory arrest. Patients less than 32 weeks’ gestation, less than 2 kg in weight, and those with genetic syndromes were excluded. Neurodevelopmental assessments were performed before the stage 2 operation and at 1 year of age. Overall hospital survival was 88%, with a 1-year survival of 75% for the entire cohort, which was not significantly different between the two groups. All patients had delayed psychomotor and mental development at both intervals studied. There was no statistically significant difference between the two groups studied.
33. Hoffman GM, Tweddell JS, Ghanayem NS, et al: Alteration of the critical arteriovenous oxygen saturation relationship by sustained afterload reduction after the Norwood procedure. J Thorac Cardiovasc Surg 2004; 127:738–745
This study evaluated 62 patients who received phenoxybenzamine on cardiopulmonary bypass and nine control patients who did not receive phenoxybenzamine. Patients placed on ECMO were excluded. All patients had a Norwood operation with modified Blalock-Taussig (MBT) shunt. The patients who received phenoxybenzamine had decreased arteriovenous oxygen saturation difference and a linear increase in superior vena cava and arterial oxygen saturation across a wide range of increasing systemic oxygen saturations. Arterial oxygen saturation was a poor predictor of periods of hemodynamic risk.
34. Hoffman TM, Bush DM, Wernovsky G, et al: Postoperative junctional ectopic tachycardia in children: Incidence, risk factors, and treatment. Ann Thorac Surg 2002; 74:1607–1611
This is a nested case cohort analysis of 33 patients with postoperative junctional ectopic tachycardia (JET) from a group of 594 consecutive patients undergoing cardiac surgery. In 19 of 33 patients, JET occurred within 24 hours following the operation, and JET occurred within 5 days in 30 of 33 patients. In four patients who died, JET had resolved prior to death. Multivariate analysis demonstrated age less than 6 months and dopamine use in the first 72 hours following surgery as risk factors for the development of JET.
35. Hoffman TM, Wernovsky G, Atz AM, et al: Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 2003; 107:996–1002
This double-blind, placebo-controlled, multicenter study of 227 children undergoing biventricular repair demonstrated a 64% risk reduction of low cardiac output syndrome (LCOS) with prophylactic use of high-dose milrinone (0.75 μg/kg/min) compared with the placebo group (in which the prevalence of LCOS was 25.9%).
36. Hoskote A, Li J, Hickey C, et al: The effects of carbon dioxide on oxygenation and systemic, cerebral, and pulmonary vascular hemodynamics after the bidirectional superior cavopulmonary anastomosis. J Am Coll Cardiol 2004; 44:1501–1509
This study of nine infants after bidirectional cavopulmonary anastomosis using inspired CO2showed that the PaO2increased from 36 to 44 mm Hg and then to 50 mm Hg at PaCO2levels of 35, 45, and 55 mm Hg, respectively, and this increased oxygenation was accompanied by increased pulmonary and cerebral blood flow.
37. Janousek J, Gebauer RA, Abdul-Khaliq H, et al; Working Group for Cardiac Dysrhythmias and Electrophysiology of the Association for European Paediatric Cardiology: Cardiac resynchronisation therapy in paediatric and congenital heart disease: Differential effects in various anatomical and functional substrates. Heart 2009; 95:1165–1171
This retrospective study of 109 patients evaluated the impact of cardiac resynchronization therapy in patients with and without CHD. The primary endpoint was improvement in echocardiographic variables of systemic ventricular function with a median follow-up of 7.5 months. The majority of patients (69) had a systemic left ventricle, and 75% had undergone prior cardiac surgery. Echocardiographic variables of function and New York Heart Association (NYHA) class both showed statistically significant improvement (p < 0.001 for both). Multivariable analysis predicted the presence of primary dilated cardiomyopathy and poor NYHA class as risk factors for nonresponse to therapy.
38. Khongphatthanayothin A, Wong PC, Samara Y, et al: Impact of respiratory syncytial virus infection on surgery for congenital heart disease: Postoperative course and outcome. Crit Care Med 1999; 27:1974–1981
This retrospective study of 25 children with CHD who had cardiac surgery within 6 months of respiratory syncytial virus (RSV) infection showed higher morbidity and mortality and longer postoperative ventilatory support (10.5 vs 1.2 d) in patients who had cardiac surgery during the same admission as the RSV infection compared with those who had surgery electively after RSV infection.
39. Kogon BE, Plattner C, Leong T, et al: Adult congenital heart surgery: Adult or pediatric facility? Adult or pediatric surgeon? Ann Thorac Surg 2009; 87:833–840
This single-center analysis of 303 operations on adults with CHD analyzed risk factors for survival by comparing those patients operated on in an adult hospital or by an adult cardiothoracic surgeon with patients operated on by pediatric surgeons in an adult or pediatric hospital. The majority of operations were repeat sternotomies, and the most common primary operative diagnosis was valve repair or replacement. Risk factors for mortality on multivariate regression analysis included age at operation, surgery performed by an adult surgeon, and operation performed at a pediatric institution. Neither prior operation nor Risk Adjustment for Congenital Heart Surgery score impacted mortality. The authors suggest that adults with CHD should be operated on by pediatric cardiothoracic surgeons in adult hospitals in part due to the availability of subspecialists to manage common comorbidities and because of the familiarity of pediatric surgeons with the complex anatomy and surgical procedures required by these patients.
40. Kolovos NS, Bratton SL, Moler FW, et al: Outcome of pediatric patients treated with extracorporeal life support after cardiac surgery. Ann Thorac Surg 2003; 76:1435–1441; discussion 1441–1442
This single-center retrospective review of 74 postsurgical patients supported with extracorporeal life support analyzed the risk factors associated with survival and outcomes. Patients supported with ECMO preoperatively and those who died during surgery were excluded. Total hospital mortality was 50%. Patients undergoing CPR at time of cannulation and those needing hemofiltration on ECMO were more likely to die. Renal function prior to cannulation and peak lactate on ECMO were not associated with survival. Patients with a favorable two-ventricle repair were more likely to survive than those with single-ventricle physiology.
41. Kussman BD, Wypij D, Laussen PC, et al: Relationship of intraoperative cerebral oxygen saturation to neurodevelopmental outcome and brain magnetic resonance imaging at 1 year of age in infants undergoing biventricular repair. Circulation 2010; 122:245–254
This study of 84 patients undergoing biventricular repair of CHD sought to determine whether intraoperative cerebral oxygenation impacts neurodevelopment at 1 year of age. Eightyfour patients were enrolled; patients with associated congenital anomalies, low birth weight, arch reconstruction, and reoperation following the index case were excluded. When grouped according to area under the curve for rSO2 less than or equal to 45%, lower psychomotor development scores were associated with lower rSO2 during the 60 minutes following cardiopulmonary bypass (CPB) among other postoperative time points. Hemosiderin foci on brain MRI were associated with lower average rSO2 from postinduction to 60 minutes post-CPB and lower minimum during rewarming and during the 60-minute period following CPB.
42. Langley SM, Winlaw DS, Stumper O, et al: Midterm results after restoration of the morphologically left ventricle to the systemic circulation in patients with congenitally corrected transposition of the great arteries. J Thorac Cardiovasc Surg 2003; 125:1229–1241
This report describes 54 patients after anatomic repair of congenitally corrected transposition or atrioventricular discordance with a double outlet right ventricle. The authors report good short-term and long-term survival (94% at 1 yr and 90% at 10 yr). Importantly, continued monitoring of LV function, aortic valve insufficiency, and conduit sufficiency is necessary.
43. Li J, Bush A, Schulze-Neick I, et al: Measured versus estimated oxygen consumption in ventilated patients with congenital heart disease: The validity of predictive equations. Crit Care Med 2003; 31:1235–1240
This is a prospective study of 126 patients with CHD in both the catheterization laboratory and the ICU using respiratory mass spectrometry. The authors concluded that poor agreement existed between measured values and the accepted four published predictive equations.
44. Li J, Zhang G, Holtby H, et al: Adverse effects of dopamine on systemic hemodynamic status and oxygen transport in neonates after the Norwood procedure. J Am Coll Cardiol2006; 48:1859–1864
This is a prospective study of 13 neonates after the Norwood procedure. Oxygen consumption and delivery were measured before and after dopamine administration. Dopamine increased oxygen consumption, and termination of dopamine was associated with an improved balance of oxygen consumption to delivery.
45. Luciani GB, Nichani S, Chang AC, et al: Continuous versus intermittent furosemide infusion in critically ill infants after open heart operations. Ann Thorac Surg 1997; 64:1133–1139
This prospective randomized study in 26 infants comparing continuous versus intermittent furosemide infusion demonstrated that a continuous infusion of furosemide resulted in higher urinary output per dose of drug (1.0 vs 0.5 mL/kg/hr/mg) and lower fluid replacement needs.
46. Mackie AS, Booth KL, Newburger JW, et al: A randomized, double-blind, placebo-controlled pilot trial of triiodothyronine in neonatal heart surgery. J Thorac Cardiovasc Surg2005; 130:810–816
This randomized, double-blind, placebo-controlled trial of neonates undergoing aortic arch reconstruction demonstrated a more rapid negative fluid balance in the group supplemented with triiodothyronine (T3) compared with placebo. The authors report a more favorable composite clinical outcome score in the T3 group (based on time to negative fluid balance, time to sternal closure, and time to first extubation).
47. Mahle WT, Cuadrado AR, Kirshbom PM, et al: Nesiritide in infants and children with congestive heart failure. Pediatr Crit Care Med 2005; 6:543–546
This is a single-center retrospective review of nesiritide use in children with congestive heart failure treated with diuretics and inotropes. Thirty children received nesiritide, including patients with single-ventricle and two-ventricle anatomy, patients following heart transplantation, and patients with dilated cardiomyopathy. The use of nesiritide dosed between 0.005 and 0.02 μg/kg/min resulted in negative fluid balance and a trend toward decrease in right atrial pressures. Therapy was discontinued in two patients due to drug-related side effects.
48. Mahle WT, Tavani F, Zimmerman RA, et al: An MRI study of neurological injury before and after congenital heart surgery. Circulation 2002; 106(12 Suppl 1):I109–II114
This is a prospective study of a cohort of neonates undergoing open-heart surgery that revealed mild ischemic lesions primarily in the form of periventricular leukomalacia (PVL) in more than 50% of neonates postoperatively. These lesions commonly resolved 4–6 months after surgery.
49. Main E, Elliott MJ, Schindler M, et al: Effect of delayed sternal closure after cardiac surgery on respiratory function in ventilated infants. Crit Care Med 2001; 29:1798–1802
This clinical investigation demonstrated that respiratory compliance decreased by a mean of 19% after delayed sternal closure in infants following cardiac surgery.
50. McCarthy RE 3rd, Boehmer JP, Hruban RH, et al: Longterm outcome of fulminant myocarditis as compared with acute (nonfulminant) myocarditis. N Engl J Med 2000; 342:690–695
This adult study demonstrated excellent long-term prognosis in patients with fulminant myocarditis (93% 11-yr survival compared with 45% in acute myocarditis) and therefore provided the basis for the concept of aggressive hemodynamic support in these profoundly ill patients.
51. McElhinney DB, Hedrick HL, Bush DM, et al: Necrotizing enterocolitis in neonates with congenital heart disease: Risk factors and outcomes. Pediatrics 2000; 106:1080–1087
This study revealed an overall NEC incidence of 3.3% in neonates with CHD, with elevated risk in HLHS and truncus arteriosus (odds ratios of 3.8 and 6.3, respectively), as well as factors such as prematurity, and low cardiac output or clinical shock.
52. McElhinney DB, Salvin JW, Colan SD, et al: Improving outcomes in fetuses and neonates with congenital displacement (Ebstein’s malformation) or dysplasia of the tricuspid valve. Am J Cardiol 2005; 96:582–586
This is a review of 66 fetuses and neonates diagnosed with Ebstein’s malformation of the tricuspid valve; 33 were diagnosed with fetal echocardiography and 33 diagnosed postnatally. Only 49% of the patients diagnosed in the fetal period survived to birth, and 21% survived past the neonatal period. Factors associated with poor outcome are described including echocardiographic
measurements (e.g., right atrium area index) and absence of antegrade flow across the pulmonary valve.
53. McGrath E, Wypij D, Rappaport LA, et al: Prediction of IQ and achievement at age 8 years from neurodevelopmental status at age 1 year in children with D-transposition of the great arteries. Pediatrics 2004; 114:e572–e576
The authors of this study examined the developmental outcomes of the cohort from the Boston Circulatory Arrest Study; 135 patients with a history of surgical repair of d-TGA were included. Using developmental studies at age 1 and 8 years, the authors concluded that children with low scores at age 1 continue to demonstrate low scores at age 8 and require close developmental surveillance. Importantly, children with normal developmental scores at age 1 may also have abnormal scores, be at significant developmental risk, and require continued close monitoring at age 8.
54. Migliavacca F, Pennati G, Dubini G, et al: Modeling of the Norwood circulation: Effects of shunt size, vascular resistances, and heart rate. Am J Physiol Heart Circ Physiol 2001; 280:H2076–H2086
This study uses a computational model of the Norwood circulation constructed on the basis of compartmental analysis and showed the following: 1) larger shunts decreased oxygen delivery, 2) systemic rather than pulmonary vascular resistance exerted more effect on hemodynamics, 3) systemic arterial oxygenation was minimally influenced by heart rate, 4) better correlation was found between venous rather than arterial oxygen saturation and oxygen delivery, and 5) pulmonary-tosystemic shunt ratio of 1.0 resulted in optimal oxygen delivery.
55. Miller OI, Tang SF, Keech A, et al: Inhaled nitric oxide and prevention of pulmonary hypertension after congenital heart surgery: A randomised double-blind study. Lancet 2000; 356:1464–1469
This study randomized 124 infants with high shunt flow lesions and a median age of 3 months to either low-dose nitric oxide or placebo and demonstrated that routine use of nitric oxide resulted in fewer pulmonary hypertensive crises and shortened postoperative course time to extubation (87 vs 117 hr) without incurring toxicity.
56. Miller SP, McQuillen PS, Hamrick S, et al: Abnormal brain development in newborns with congenital heart disease. N Engl J Med 2007; 357:1928–1938
This study used brain MRI with magnetic resonance spectroscopy and diffusion tensor imaging to examine 41 term newborns with CHD (d-TGA and HLHS). When compared with control newborns, significant and widespread brain abnormalities were found in the preoperative newborns with CHD.
57. Mou SS, Giroir BP, Molitor-Kirsch EA, et al: Fresh whole blood versus reconstituted blood for pump priming in heart surgery in infants. N Engl J Med 2004; 351:1635–1644 This single-center, randomized, double-blind, controlled trial in infants undergoing cardiac surgery showed that the use of whole blood versus reconstituted blood for bypass circuit priming not only conferred no advantages regarding chest tube output, blood product transfusion requirements, levels of serum mediators of inflammation such as interleukin (IL)-6 and C3a, and cardiac troponin I but also was associated with an increased length of ICU stay (97 vs 70.5 hr) and increased perioperative fluid overload (28.8 vs –6.9 mL/kg).
58. Namachivayam P, Theilen U, Butt WW, et al: Sildenafil prevents rebound pulmonary hypertension after withdrawal of nitric oxide in children. Am J Respir Crit Care Med 2006; 174:1042–1047
The authors of this prospective, randomized, placebo-controlled trial investigated if a single dose of oral sildenafil could prevent rebound pulmonary hypertension after withdrawal of inhaled nitric oxide. In 29 randomized children, sildenafil prevented rebound pulmonary hypertension and reduced length of tracheal intubation and ICU unit length of stay. Of note, patients in both treatment arms were managed with the authors’ standard protocol for weaning inhaled nitric oxide.
59. Nelson DP, Andropoulos DB, Fraser CD Jr: Perioperative neuroprotective strategies. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2008; 11:49–56
This review summarizes the long-term neurodevelopmental impairment commonly found in newborns and infants undergoing congenital heart surgery. In-depth summaries of the etiology/mechanism of neurologic injury (including preoperative, intraoperative, and postoperative management), the neurologic monitoring of congenital heart patients, and perioperative neuroprotective strategies are all discussed.
60. Norwood WI, Lang P, Hansen DD: Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med 1983; 308:23–26
This seminal paper describes the first patient with physiologically corrective surgery for HLHS. The case report describes follow-up of 6 months after single-stage atriopulmonary Fontan in a child with HLHS who received a stage I Norwood palliation at birth.
61. Odegard KC, Zurakowski D, DiNardo JA, et al: Prospective longitudinal study of coagulation profiles in children with hypoplastic left heart syndrome from stage I through Fontan completion. J Thorac Cardiovasc Surg 2009; 137:934–941
This longitudinal prospective study of patients with HLHS investigated the coagulation profile from stage I palliation through Fontan completion. Levels of both procoagulant and anticoagulant proteins were significantly decreased through the time to completion of the Fontan, but most important is the acquired elevation of factor VIII levels after Fontan completion.
62. Ohye RG, Sleeper LA, Mahony L, et al; Pediatric Heart Network Investigators: Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 2010; 362:1980–1992
This randomized controlled trial sponsored by the Pediatric Heart Network compared the RVPA shunt versus MBT shunt for stage I palliation (Norwood) of HLHS. In 555 randomized children who received a stage I palliation, transplantation-free survival at 12 months was improved with the RVPA shunt compared with the MBT shunt; however, after 12 months, this survival advantage did not persist, and no significant difference in transplantation-free survival was found.
63. Parr GV, Blackstone EH, Kirklin JW: Cardiac performance and mortality early after intracardiac surgery in infants and young children. Circulation 1975; 51:867–874
This early study of 139 children in the immediate postoperative period evaluated cardiac performance using mixed venous partial pressure of oxygen (Pvo2) and CI via indicator-dilution technique. Death was predicted by a Pvo2 less than 30 mm Hg and a CI less than 2.0 L/min/m2, suggesting that vasodilator therapy should be instituted in these circumstances. Note that this publication predated milrinone therapy and the Prophylactic Intravenous Use of Milrinone After Cardiac Operation in Pediatrics (PRIMACORP) study (see reference [35] earlier).
64. Perry JC, Fenrich AL, Hulse JE, et al: Pediatric use of intravenous amiodarone: Efficacy and safety in critically ill patients from a multicenter protocol. J Am Coll Cardiol 1996; 27:1246–1250
This is a multicenter study of 40 patients with various tachyarrhythmias who failed standard antiarrhythmic therapy. The majority responded to therapy with IV amiodarone at a mean loading dose of 6.3 mg/kg and a continuous infusion when necessary.
65. Petit CJ, Rome JJ, Wernovsky G, et al: Preoperative brain injury in transposition of the great arteries is associated with oxygenation and time to surgery, not balloon atrial septostomy. Circulation 2009; 119:709–716
The authors aimed to determine the relationship between preoperative brain injury and BAS in neonates with d-TGA. This retrospective study (using MRI data from another concurrent prospective study) found that 38% of patients had PVL unrelated to balloon septostomy. However, PVL was related to lower preoperative saturation and longer time to surgery.
66. Petrucci O, O’Brien SM, Jacobs ML, et al: Risk factors for mortality and morbidity after the neonatal Blalock-Taussig shunt procedure. Ann Thorac Surg 2011; 92:642–651; discussion 651–652
The authors abstracted data on 1,273 neonates who received a MBT shunt from the Society of Thoracic Surgeons database. Discharge mortality was 7.2% and composite morbidity (ECMO, LCOS, and unplanned reoperation) was 13.1%. One third of deaths occurred within 24 hours postoperatively, and infants with pulmonary atresia with intact ventricular septum and weighing less than 3 kg were at highest risk.
67. Pickard SS, Feinstein JA, Popat RA, et al: Short- and longterm outcomes of necrotizing enterocolitis in infants with congenital heart disease. Pediatrics 2009; 123:e901–e906
In this retrospective study, 126 neonates had NEC without CHD and 76 had NEC with CHD. Cardiac comorbidity was found to be the main determinant of outcome in NEC in the cardiac population, and short- and long-term outcomes are better for CHD-related NEC. The authors suggest that decreased mesenteric flow as the etiology of NEC in the cardiac population (in contrast to intestinal immaturity in premature neonates).
68. Polito A, Thiagarajan RR, Laussen PC, et al: Association between intraoperative and early postoperative glucose levels and adverse outcomes after complex congenital heart surgery. Circulation 2008; 118:2235–2242
This retrospective study evaluated the associations between perioperative glucose exposure, length of hospital stay, and morbidity/mortality. Intraoperatively, glucose levels less than 75 mg/dL were associated with adverse outcomes, whereas postoperative levels between 110 and 126 mg/dL may be optimal. This retrospective study provided the foundation for the subsequent randomized controlled trial by Agus et al (N Engl J Med 2012; 367:1208–1219).
69. Rajagopal SK, Almond CS, Laussen PC, et al: Extracorporeal membrane oxygenation for the support of infants, children, and young adults with acute myocarditis: A review of the Extracorporeal Life Support Organization registry. Crit Care Med 2010; 38:382–387
This retrospective review from the ELSO registry describes the survival outcomes for children who require ECMO for myocarditis and identifies female gender, arrhythmia on ECMO, and need for dialysis during ECMO as independent risk factors for mortality. Overall mortality in these 255 patients was 40%.
70. Ramamoorthy C, Tabbutt S, Kurth CD, et al: Effects of inspired hypoxic and hypercapnic gas mixtures on cerebral oxygen saturation in neonates with univentricular heart defects. Anesthesiology 2002; 96:283–288
This randomized crossover trial of neonates with singleventricle physiology examined the effects of 17% inspired oxygen versus 3% inspired carbon dioxide and demonstrated that 3% inspired carbon dioxide improved cerebral oxygenation from 56% to 68% and raised mean arterial pressure from 45 to 50 mm Hg, whereas 17% inspired oxygen did not have similar hemodynamic effects.
71. Raymond TT, Cunnyngham CB, Thompson MT, et al; American Heart Association National Registry of CPR Investigators: Outcomes among neonates, infants, and children after extracorporeal cardiopulmonary resuscitation for refractory inhospital pediatric cardiac arrest: A report from the National Registry of Cardiopulmonary Resuscitation. Pediatr Crit Care Med 2010; 11:362–371
The authors used the American Heart Association National Registry of Cardiopulmonary Resuscitation to investigate outcomes in children treated with extracorporeal CPR. Of 199 cases, 87 (43.7%) survived to hospital discharge; 95% of survivors with recorded neurologic outcomes had “favorable” outcomes. Cardiac illness category was associated with likelihood of survival (lower category with higher survival rate).
72. Reddy VM, Liddicoat JR, McElhinney DB, et al: Hemodynamic effects of epinephrine, bicarbonate and calcium in the early postnatal period in a lamb model of single-ventricle physiology created in utero. J Am Coll Cardiol 1996; 28:1877–1883
This is a fetal animal model of single-ventricle physiology created to examine the effects of resuscitative agents. The authors found that while epinephrine infusion and calcium and bicarbonate boluses increased total cardiac output without affecting Qp/Qs balance, epinephrine boluses were detrimental by increasing Qp/Qs 584% as a result of increased systemic vascular resistance.
73. Redlin M, Koster A, Huebler M, et al: Regional differences in tissue oxygenation during cardiopulmonary bypass for correction of congenital heart disease in neonates and small infants: Relevance of near-infrared spectroscopy. J Thorac Cardiovasc Surg 2008; 136:962–967
In this study of 20 neonates and small infants, venous oxygen saturation was lower and lactate level higher from the superior vena cava compared with the inferior vena cava. Cerebral tissue oxygenation was analyzed by near-infrared spectroscopy. Based on these measurements, the authors concluded that mixed venous saturation alone is inadequate to evaluate regional cerebral saturation.
74. Riordan CJ, Randsbaek F, Storey JH, et al: Inotropes in the hypoplastic left heart syndrome: Effects in an animal model. Ann Thorac Surg 1996; 62:83–90
This study of an animal model of single ventricle showed that although dopamine, dobutamine, and epinephrine all increased total cardiac output, only epinephrine at 0.1 μg/kg/min decreased the Qp/Qs ratio (1.23–0.8) and increased oxygen delivery (from 40 to 56 mL/min).
75. Rosenzweig EB, Starc TJ, Chen JM, et al: Intravenous arginine-vasopressin in children with vasodilatory shock after cardiac surgery. Circulation 1999; 100(19Suppl):II182–II186
This study showed that children who have catecholamineresistant hypotension after cardiac surgery responded to arginine vasopressin at a range of 0.0003–0.002 U/kg/min with an improvement in systolic blood pressure from 65 to 87 mm Hg. The authors suggested, however, that this vasoactive agent should probably be avoided in the presence of severe left ventricular dysfunction.
76. Schroeder AR, Axelrod DM, Silverman NH, et al: A continuous heparin infusion does not prevent catheter-related thrombosis in infants after cardiac surgery. Pediatr Crit Care Med 2010; 11:489–495
This randomized, double blind, placebo-controlled trial in postoperative patients less than 1 year concluded that a continuous heparin infusion at 10 U/kg/hr did not reduce catheterrelated thrombus formation, with the treatment and placebo groups exhibiting 15% and 16% thrombus rates, respectively. However, central venous catheters in place for 7 days or more had a greater risk of thrombus formation and malfunction.
77. Schroeder VA, Pearl JM, Schwartz SM, et al: Combined steroid treatment for congenital heart surgery improves oxygen delivery and reduces postbypass inflammatory mediator expression. Circulation 2003; 107:2823–2828
This study showed that compared with intraoperative use of steroid alone (methylprednisolone at 30 mg/kg), the combined preoperative and intraoperative use of steroid at the same dosage was associated with attenuated inflammatory mediator expression (reduced myocardial messenger RNA expression for IL-6, monocyte chemotactic protein-1, and intercellular adhesion molecule-1), as well as reduced fluid requirements, lower body temperature, and lower arteriovenous oxygen difference for the first 24 hours after surgery.
78. Schulze-Neick I, Ho SY, Bush A, et al: Severe airflow limitation after the unifocalization procedure: Clinical and morphological correlates. Circulation 2000; 102(19 Suppl 3):III142–III147
This clinical study elucidated the mechanism of severe airflow limitation in children with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (MAPCAs) as interruption of the tracheobronchial blood supply during mobilization of MAPCAs that leads to airway ischemia.
79. Schwartz SM, Dent CL, Musa NL, et al: Single-ventricle physiology. Crit Care Clin 2003; 19:393–411
The authors provide a thorough review of single-ventricle physiology, including a description of preoperative management and each surgical palliation.
80. Seale AN, Uemura H, Webber SA, et al; British Congenital Cardiac Association: Total anomalous pulmonary venous connection: Morphology and outcome from an international population-based study. Circulation 2010; 122:2718–2726
This retrospective, international, population-based study described the morphological spectrum of total anomalous pulmonary venous repair in the United Kingdom, Ireland, and Sweden. Risk factors for recurrent pulmonary venous obstruction (PVO) and death were assessed; an overall mortality of almost 18% was found. Forty percent of patients with postoperative PVO died by 3 years, and nearly 15% of patients required reintervention for recurrent PVO. Risk factors for recurrent PVO and death are discussed, and these provide prognostic data for clinicians.
81. Shekerdemian L, Bohn D: Cardiovascular effects of mechanical ventilation. Arch Dis Child 1999; 80:475–480
This review article focuses on the influence of ventilation on cardiac function, cardiovascular effects of changes in intrathoracic pressure and in lung volume, and heart-lung interactions in the intensive care setting.
82. Shekerdemian LS, Bush A, Lincoln C, et al: Cardiopulmonary interactions in healthy children and children after simple cardiac surgery: The effects of positive and negative pressure ventilation. Heart 1997; 78:587–593
This study examined the effects of positive versus negative pressure ventilation and showed that cuirass negative pressure ventilation led to a 28% increase in cardiac output in postoperative patients following cardiopulmonary bypass and that this increase was greater than that observed in patients who had patent ductus arteriosus closure.
83. Shekerdemian LS, Bush A, Shore DF, et al: Cardiopulmonary interactions after Fontan operations: Augmentation of cardiac output using negative pressure ventilation. Circulation 1997; 96:3934–3942
This investigation of 18 patients after the Fontan procedure assessed the effects of negative pressure ventilation. In nine patients immediately after Fontan and nine other patients in the convalescent phase, negative pressure ventilation increased pulmonary blood flow (and cardiac output) by 42%. The increased blood flow was due to a significant increase in ventricular stroke volume; these findings were most significant in Fontan patients with higher pulmonary artery pressures.
84. Shekerdemian LS, Bush A, Shore DF, et al: Cardiorespiratory responses to negative pressure ventilation after tetralogy of Fallot repair: A hemodynamic tool for patients with a low-output state. J Am Coll Cardiol 1999; 33:549–555
This investigation of 23 patients after tetralogy of Fallot repair assessed the effects of negative pressure ventilation. A brief period (15 min) of negative pressure ventilation increased pulmonary blood flow by 39% and a prolonged (45 min) period increased pulmonary blood flow by 67%. Patients with restrictive right ventricular physiology had a delayed but more significant increase in pulmonary blood flow of 84%.
85. Shore S, Nelson DP, Pearl JM, et al: Usefulness of corticosteroid therapy in decreasing epinephrine requirements in critically ill infants with congenital heart disease. Am J Cardiol 2001; 88:591–594
This retrospective review of infants who received more than or equal to 0.15 μg/kg/min of epinephrine showed that they responded to steroids even at low dose (< 100 mg/m2/d of hydrocortisone equivalents) with a decrease in inotrope requirement and a concomitant 15% increase in mean arterial blood pressure.
86. Simsic JM, Bradley SM, Stroud MR, et al: Risk factors for interstage death after the Norwood procedure. Pediatr Cardiol 2005; 26:400–403
This retrospective study of 50 patients after the Norwood procedure evaluated the risk factors for interstage mortality. Eight patients (16%) died during the interstage period; by multivariate analysis, the presence of an arrhythmia in the postoperative period and decreased ventricular function at hospital discharge were identified as independent risk factors.
87. Simsic JM, Bradley SM, Mulvihill DM: Sodium nitroprusside infusion after bidirectional superior cavopulmonary connection: Preserved cerebral blood flow velocity and systemic oxygenation. J Thorac Cardiovasc Surg 2003; 126:186–190
This is a prospective study of nine infants undergoing bidirectional cavopulmonary anastomosis who received nitroprusside in a dose range 1–4 μg/kg/min that showed a decrease in mean systemic blood pressure from 69 to 58 mm Hg without accompanying change in cerebral blood flow or oxygen saturation.
88. Suominen PK, Dickerson HA, Moffett BS, et al: Hemodynamic effects of rescue protocol hydrocortisone in neonates with low cardiac output syndrome after cardiac surgery. Pediatr Crit Care Med 2005; 6:655–659
This small retrospective study of 12 term neonates with LCOS after cardiac surgery describes the use of hydrocortisone for hemodynamic support. Low-dose hydrocortisone (starting dose 100 mg/m2/d) was associated with less inotrope requirement, higher blood pressure, lower heart rate, and no significant side effects. Proposed mechanisms of action of hydrocortisone include reversing down-regulation of adrenergic receptors, treating adrenal suppression, and decreasing capillary leak.
89. Tabbutt S, Duncan BW, McLaughlin D, et al: Delayed sternal closure after cardiac operations in a pediatric population. J Thorac Cardiovasc Surg 1997; 113:886–893
This retrospective review of 178 cardiac surgical patients who had delayed sternal closure showed significant increases in left and right atrial pressure upon sternal closure (7.7–9.8 mm Hg and 8.0–10.1 mm Hg, respectively) with concomitant small increases in ventilatory variables, including peak inspiratory pressure, delivered breaths/min, and Fio2.
90. Tabbutt S, Ramamoorthy C, Montenegro LM, et al: Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled ventilation. Circulation 2001; 104(12 Suppl 1):I159–I164
This is a prospective randomized crossover trial in 10 neonates with HLHS comparing hypoxia (17% FIO2) and hypercarbia (2.7% FICO2). It showed oxygen delivery calculated as
Sao2/(Sao2 – Svo2) is unchanged during hypoxia but increased
from 3.64 to 6.14 during hypercarbia.
91. Taeed R, Schwartz SM, Pearl JM, et al: Unrecognized pulmonary venous desaturation early after Norwood palliation confounds Gp:Gs assessment and compromises oxygen delivery. Circulation 2001; 103:2699–2704
This clinical study in 12 neonates after the Norwood operation measuring simultaneous arterial, superior vena cava, and pulmonary venous oximetry showed that pulmonary venous desaturation less than 95% occurred in 30% of patients and that higher pulmonary venous saturation to improve oxygen delivery can be achieved with judicious use of inspired oxygen and positive end-expiratory pressure.
92. Tanoue Y, Sese A, Ueno Y, et al: Bidirectional Glenn procedure improves the mechanical efficiency of a total cavopulmonary connection in high-risk Fontan candidates. Circulation 2001; 103:2176–2180
In this catheterization study of 18 patients who underwent a BDG procedure before the Fontan operation versus 29 patients who had the Fontan operation without a preceding BDG operation, the patients who had the interim BDG operation had afterload mismatch corrected and improved ventricular energetics.
93. Teele SA, Allan CK, Laussen PC, et al: Management and outcomes in pediatric patients presenting with acute fulminant myocarditis. J Pediatr 2011; 158:638–643.e1
This retrospective review of 20 patients with acute fulminant myocarditis (AFM) describes one institution’s management and outcomes in this patient population over a 12-year period. Seventeen patients (85%) survived to hospital discharge, with one of those survivors receiving a heart transplant; 10 patients (50%) required ECMO. Management strategies including cardiac biopsy, treatment with IV immunoglobulin, and the decision to advance to ECMO/VAD support or transplantation are all discussed. The authors comment on the improving outcomes with ECMO in AFM because of the short duration of mechanical support and good prognosis for cardiac recovery.
94. Tweddell JS, Ghanayem NS, Mussatto KA, et al: Mixed venous oxygen saturation monitoring after stage 1 palliation for hypoplastic left heart syndrome. Ann Thorac Surg 2007; 84:1301–1310; discussion 1310–1311
This study describes the outcomes of 116 patients with HLHS managed postoperatively with continuous mixed venous oxygen saturation (Svo2) data. Continuously measured Svo2 (approximated from the superior vena cava) differentiated patients who survived without complications, those who survived with complications, and those who died. A postoperative increase in Svo2 was demonstrated in survivors, whereas patients in the early mortality group had decreased Svo2 after ICU admission. The authors suggest that elevated lactate levels demonstrate that anaerobic metabolism is already occurring and that Svo2 monitoring is an earlier indicator of acute increase in systemic vascular resistance and low systemic cardiac output. These findings led them to initiate phenoxybenzamine treatment routinely in their HLHS patients postoperatively.
95. Tweddell JS, Hoffman GM, Mussatto KA, et al: Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: Lessons learned from 115 consecutive patients. Circulation 2002; 106(12 Suppl 1):I82–I89
This analysis of a consecutive series of 115 neonates after the Norwood palliation for HLHS showed improved survival with a combined strategy of early identification of decreased systemic output with continuous Svo2 monitoring, use of afterload reduction with phenoxybenzamine, and use of anti-inflammatory therapies such as aprotinin and modified ultrafiltration.
96. Walsh EP, Saul JP, Sholler GF, et al: Evaluation of a staged treatment protocol for rapid automatic junctional tachycardia after operation for congenital heart disease. J Am Coll Cardiol 1997; 29:1046–1053
This staged-therapy protocol study for JET in 71 patients showed that this postoperative tachyarrhythmia was associated with younger age and ventricular septal defect closure and that combined hypothermia and procainamide was most efficacious in resistant cases.
97. Wernovsky G, Wypij D, Jonas RA, et al: Postoperative course and hemodynamic profile after the arterial switch operation in neonates and infants. A comparison of lowflow cardiopulmonary bypass and circulatory arrest. Circulation 1995; 92:2226–2235
This study focused on the postoperative course in 122 neonates after the arterial switch operation who had complete hemodynamic profiles. It showed that the nadir in cardiac output was at 9–12 hours after surgery with a mean decrease in CI of 32.1%, including 23.8% of patients who had CI less than 2.0 L/min/m2.
98. Wernovsky G, Kuijpers M, Van Rossem MC, et al: Postoperative course in the cardiac intensive care unit following the first stage of Norwood reconstruction. Cardiol Young 2007; 17:652–665
This retrospective review of 99 consecutive patients at a single-center systematically categorizes the ICU course for this difficult patient population. Hospital mortality for the entire group was 15.2% and 7.3% for the standard (lower) risk patients. The postoperative ICU course, lasting a median of 11 days, is remarkable for significant morbidity including the cardiac, pulmonary, and CNSs. This article serves as a reference for the “typical” postoperative course in a Norwood patient.
99. Wessel DL, Adatia I, Giglia TM, et al: Use of inhaled nitric oxide and acetylcholine in the evaluation of pulmonary hypertension and endothelial function after cardiopulmonary bypass. Circulation 1993; 88(5 Pt 1):2128–2138
This clinical investigation studied postoperative endothelial dysfunction and showed that plasma cyclic guanosine monophosphate (cGMP) levels were unchanged after infusion of acetylcholine but increased more than three-fold during use of inhaled nitric oxide, which also decreased pulmonary vascular resistance by 33%. In contrast, acetylcholine had little effect, thus supporting the purported role of cGMP as the second messenger affecting smooth muscle relaxation.
100. Zahn EM, Dobrolet NC, Nykanen DG, et al: Interventional catheterization performed in the early postoperative period after congenital heart surgery in children. J Am Coll Cardiol 2004; 43:1264–1269
This retrospective study reviewed the experience of 62 patients at median age 4 months who underwent catheterizations within 6 weeks of surgery at a median of postoperative day 9. The report includes 30 procedures involving angioplasty or stent implantation without suture disruption or transmural vascular tear, with an overall survival of 83%.
ACKNOWLEDGMENTS
We thank Zhuoming Xu, Linhua Tan, Luca di Chiara, and Denise R. Adamy.
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Copyright
1
Department of Pediatrics (Cardiology), Lucile Packard Children’s Hospital, Stanford University Medical Center, Palo Alto, CA.
2
Division of Critical Care Medicine, Division of Cardiology, Children’s National Medical Center, Washington, DC.
3
Division of Cardiology, The Heart Institute, Children’s Hospital of Orange County, University of California Irvine School of Medicine, Orange, CA.
4
Department of Pediatrics (Cardiology), Texas Children’s Hospital, Baylor College of Medicine, Houston, TX.
Drs. Axelrod and Klugman are co-first authors.
The Board Members of the Pediatric Cardiac Intensive Care Society
The authors have disclosed that they do not have any potential conflicts of interest.
For information regarding this article, E-mail: daxelrod@stanford.edu
© 2013 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
DOI: 10.1097/01.PCC.0000434621.25332.71