Pediatric Anesthesia ISSN 1155-5645
ORIGINAL ARTICLE
Patients with single ventricle physiology undergoing noncardiac surgery are at high risk for adverse events Morgan L. Brown, James A. DiNardo & Kirsten C. Odegard Division of Cardiac Anesthesia, Department of Anesthesiology, Boston Children’s Hospital, Boston, MA, USA
What is already known
• Patients with single ventricle physiology undergoing noncardiac surgery are at high risk for adverse events, although the risk is not well quantified.
What this article adds
• The risk of a major adverse event in the intraoperative or perioperative period was 11.8%. • There was no early perioperative mortality associated with noncardiac surgery in patients with single ventricle physiology.
Implications for translation
• Noncardiac surgery in patients with single ventricle physiology has a high rate of adverse events at a large tertiary pediatric hospital.
• Further research using multi-institutional databases may be able to define specific subgroups of patients with single ventricle physiology who are at especially high risk for adverse events and ways to mitigate this risk.
Keywords anesthesia; Norwood procedures; heart defects; congenital; intraoperative complications; postoperative complications; surgical procedure; operative Correspondence Dr. Morgan L. Brown, Division of Cardiac Anesthesiology, Department of Anesthesiology, Boston Children’s Hospital, Bader 3, 300 Longwood Ave., Boston, MA 02115, USA Email: [email protected]. edu Section Editor: Greg Hammer Accepted 10 April 2015 doi:10.1111/pan.12685
846
Summary Background: Patients with single ventricle physiology are at increased anesthetic risk when undergoing noncardiac surgery. Objective: To review the outcomes of anesthetics for patients with single ventricle physiology undergoing noncardiac surgery. Methods: This study is a retrospective chart review of all patients who underwent a palliative procedure for single ventricle physiology between January 1, 2007 and January 31, 2014. Anesthetic and surgical records were reviewed for noncardiac operations that required sedation or general anesthesia. Any noncardiac operation occurring prior to completion of a bidirectional Glenn procedure was included. Diagnostic procedures, including cardiac catheterization, insertion of permanent pacemaker, and procedures performed in the ICU, were excluded. Results: During the review period, 417 patients with single ventricle physiology had initial palliation. Of these, 70 patients (16.7%) underwent 102 anesthetics for 121 noncardiac procedures. The noncardiac procedures included line insertion (n = 23); minor surgical procedures such as percutaneous endoscopic gastrostomy or airway surgery (n = 38); or major surgical procedures including intra-abdominal and thoracic operations (n = 41). These interventions occurred on median day 60 of life (1–233 days). The procedures occurred most commonly in the operating room (n = 79, 77.5%). Patients’ median weight was 3.4 kg (2.4–15 kg) at time of noncardiac intervention. In 102 anesthetics, 26 patients had an endotracheal tube or tracheostomy in situ, 57 patients underwent endotracheal intubation, and 19 patients had a natural © 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
M.L. Brown et al.
Single ventricle physiology undergoing noncardiac surgery
or mask airway. An intravenous induction was performed in 77 anesthetics, an inhalational induction in 17, and a combination technique in 8. The median total anesthetic time was 126 min (14–594 min). In 22 anesthetics (21.6%), patients were on inotropic support upon arrival; an additional 24 patients required inotropic support (23.5%), of which dopamine was the most common medication. There were 10 intraoperative adverse events (9.8%) including: arrhythmias requiring treatment (n = 4), conversion from sedation to a general anesthetic (n = 2), difficult airway (n = 1), inadvertent extubation with desaturation and bradycardia (n = 1), hypotension and desaturation (n = 1), and cardiac arrest (n = 1). Postoperative events (20% of baseline values, arrhythmias requiring treatment (medical or electrical), new inotropic support (hemodynamic instability), and any episodes of cardiac arrest requiring chest compressions were recorded. Additional adverse outcomes including transfer to a higher acuity care setting or death were recorded for the first 48 h following the anesthetic.
© 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
847
M.L. Brown et al.
Single ventricle physiology undergoing noncardiac surgery
For the purposes of analysis, procedure types were grouped into three categories. Line insertion included both peripherally inserted central catheter (PICC) placement, as well as other types of long-term central access devices. Minor surgical procedures included circumcisions, airway procedures, percutaneous endoscopic gastrostomy (PEG) tube insertion, and other endoscopic procedures. Major surgical procedures included laparoscopic gastric (G) tube insertion and any intra-abdominal or thoracic procedure. Procedures which involved more than one intervention were grouped into the highest category (e.g. PICC and laparoscopic G tube were considered as a major surgical procedure). Data are reported as means and standard deviations or medians and interquartile ranges as appropriate. Due to small sample size, comparisons were made using the Fisher’s exact test, Mann–Whitney U-test, or Kruskal– Wallis test, as appropriate. The statistical program JMP version 11 (Cary, NC, USA) was used for all analyses. Results We identified 417 patients with single ventricle physiology who had initial palliation during the study period. Of these, 70 (16.7%) patients underwent 102 anesthetics for 121 noncardiac procedures. Patients’ median weight was 3.4 kg (range 2.4–15 kg) at time of noncardiac intervention. Fifty-five (78.6%) of patients had a systemic right ventricle, 5 patients (7.1%) had moderate or severe ventricular dysfunction, and 12 (17.1%) patients had moderate or severe atrioventricular valvular regurgitation. Initial palliative procedures included a Sano shunt (n = 33, 47.1%), a BT shunt (n = 24, 34.3%), a hybrid technique consisting of a balloon atrial septostomy and a ductus arteriosus stent (n = 8, 11.4%), a pulmonary artery band (n = 3, 4.3%), and a central shunt (n = 1, 1.4%), Table 1. One patient had noncardiac surgery prior to any cardiac intervention. Twenty patients were admitted from the cardiac intensive care unit (CICU), 49 patients were inpatients, and 1 patient was a
Table 1 Type of single ventricle palliative procedure in patients for noncardiac surgery Palliative procedure
N, %
Stage I, with Sano shunt Stage I, with Blalock–Taussig shunt Blalock–Taussig shunt Hybrid—balloon atrial septostomy, ductus arteriosus stent Pulmonary artery band Stage I, with Central shunt
33, 47.1 12, 17.1 12, 17.1 8, 11.4
848
3, 4.3 1, 1.4
Table 2 Type of noncardiac procedure Noncardiac procedures
n = 121
Peripherally inserted central catheter line or central line Percutaneous endoscopic gastrostomy tube Laparascopic gastrostomy tube Airway procedures (including tracheostomy) Open gastrostomy Laparotomy Other Ladd’s procedure Diaphragmatic plication Circumcision Endoscopic gastroscopy and biopsy Gastrostomy tube in interventional radiology
25 22 22 12 9 7 7 5 4 3 3 2
same-day admission at the time of the noncardiac surgery. A total of 121 procedures (Table 2) were performed under 102 separate anesthetics. In 77 anesthetics (75.5%), an intravenous (IV) induction was performed; in 17 anesthetics (16.7%), an inhaled induction was performed; and in 8 anesthetics (7.8%), a combination of intravenous (IV) agents and inhaled anesthetics were utilized for induction. In 102 anesthetics, 26 patients had an endotracheal tube or tracheostomy in situ, 57 patients underwent endotracheal intubation, and 19 patients had a natural or mask airway. In 25 anesthetics, patients had an arterial line; 13 were placed at the time of the noncardiac procedure. In 28 anesthetics, patients had either a central line or a PICC line. The median total anesthesia time was 126 min (range 14–594 min). Eighty-five of 102 anesthetics (83.3%) were for procedures that were considered elective and were performed during normal working hours. At the time of noncardiac surgery, patients were directly transferred from the CICU in 40 anesthetics (39.2%). Of the remaining 62 anesthetics, an additional 22 patients (35.5%) went to the cardiac CICU postoperatively. After the remainder of anesthetics (40 anesthetics, 39.2%), patients were admitted to the cardiology floor after the procedure. There were no deaths at 48 h. In 22 anesthetics (21.6%), patients were on inotropic support already on arrival to the operating room. An additional 24 patients (23.5%) required inotropic support during the procedure, of which dopamine was the most common medication. There were 10 intraoperative and 2 postoperative adverse events (n = 12, 11.8%). Intraoperative arrhythmias encountered included bradycardia requiring atropine (n = 3) and supraventricular tachycardia requiring adenosine (n = 1). One patient had significant intraoperative hypotension and desaturation of which the cause was unclear. It resolved with administration of volume, inotropic support, and vigorous © 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
M.L. Brown et al.
Single ventricle physiology undergoing noncardiac surgery
Table 3 Differences between patients who did or did not experience an adverse event Median age at noncardiac surgery (days) Male gender Median weight at noncardiac surgery (kg) Systemic right ventricle Sano shunt vs other Elective vs urgent/emergent Moderate or severe ventricular dysfunction Preoperative inotrope Preoperative admission location—ICU Location of procedure operating room vs other Median duration of anesthesia (h, IQR) General anesthesia (vs sedation) IV induction vs other Procedure type Line insertion Minor Major
No event n = 90
Adverse event n = 12
P value
72.5 (40.3–117.0) 56 (62.2%) 3.5 (3.1–4.2) 60 (66.7%) 38 (42.2%) 75 (83.3%) 7 (7.8%)
67.5 (31.8–131.5) 10 (83.3%) 3.3 (2.9–3.7) 8 (66.7%) 3 (25.0%) 10 (83.3%) 0 (0%)
0.25 0.21 0.15 1.0 0.33 1.0 1.0
18 (20.0%) 34 (37.8%) 72 (80.0%)
4 (33.3%) 6 (50.0%) 7 (58.3%)
0.28 0.53 0.14
2.0 (1.3–2.8) 76 (84.4%) 67 (74.4%)
2.4 (1.7–2.9) 10 (83.3%) 10 (83.3%)
0.27 0.72 0.73
21 (23.3%) 32 (35.6%) 37 (40.7%)
2 (16.7%) 6 (50%) 4 (33.3%)
0.62
An adverse event was defined as any major anesthetic complication including conversion from sedation to a general anesthetic, difficult airway, extubation requiring reintubation, desaturation or bradycardia >20% of baseline variables, arrhythmias requiring treatment, new inotropic support, cardiac arrest, transfer to a higher acuity care setting, or death within the first 48 h after the anesthetic.
hand-ventilation. One patient experienced ST segment changes within 24-h postanesthetic. This child was transferred to the CICU and underwent cardiac catheterization the following day, which revealed a residual aortic arch gradient. The patient underwent successful balloon dilation. Difficulties with airway management were encountered in six patients; two patients who initially were sedated with IV midazolam and ketamine during spontaneous respiration for PICC placement required conversion into a general anesthetic. One patient was receiving supplemental oxygen before the procedure and one was receiving continuous positive pressure airway (CPAP) support. Both experienced arterial desaturation which led to urgent, noncomplicated endotracheal intubation. The remainder of both anesthetics was uneventful. One patient was a known difficult airway requiring three attempts at intubation, placement of a laryngeal mask airway, and intubation through the laryngeal mask airway. One patient was inadvertently extubated during positioning in the lateral position and required reintubation. One patient scheduled for gastrostomy tube insertion experienced cardiac arrest requiring cardiac compressions and inotropic support following a failure to promptly intubate and ventilate the patient after induction. This patient went to the cardiac catheterization following the procedure to assess the status of © 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
the Sano shunt after chest compressions. Due to the positioning of the Sano shunt directly underneath the sternum, these patients are predisposed to compression of the shunt during a cardiac arrest. Cardiac catheterization revealed that the Sano shunt was patent, but narrowed. A stent was placed and the atrial septal defect was dilated due to a residual gradient. Lastly, one patient who underwent insertion of a PEG tube experienced a cardiorespiratory arrest within 24 h of the anesthetic while in the CICU. The etiology of the arrest was thought to be pulmonary overcirculation with poor coronary perfusion and low cardiac output. The child was successfully placed on an extracorporeal membrane oxygenator (ECMO) circuit and survived. Age, patient weight, time after initial cardiac surgery, gender, type of cardiac palliation, patient admission location, procedure location, and type of procedure were not significantly different between patients who did or did not experience an adverse outcome (Table 3). The longest procedure (594 min) was in an 8-monthold patient with hypoplastic left heart syndrome, who underwent a Stage I repair with a BT shunt, which was subsequently revised to a central shunt. He underwent a revision of a laparoscopic Nissen procedure which was complicated by difficult exposure and dense adhesions. This patient had a PICC line in situ, and an arterial line was placed for the procedure. The patient required 849
Single ventricle physiology undergoing noncardiac surgery
inotropic support for the majority of the procedure, but there were no adverse events. The patient was taken to the CICU postoperatively. Discussion In this single-center review of patients with single ventricle physiology undergoing noncardiac procedures, we demonstrate a high rate (11.8%) of intraoperative and early postoperative adverse events in this patient population. This is consistent with the literature which consistently identifies patients with single ventricle physiology to be at high risk for perioperative complications (8–10). At Boston Children’s Hospital, a review of cardiac arrests in children with congenital heart disease undergoing cardiac surgery, revealed an arrest frequency of 0.79% (2). Nine of these 41 arrests (22%) were in patients with single ventricle physiology. Similarly, the Pediatric POCA Registry reported that 34% of 373 cases of anesthesia-related cardiac arrest were in patients with congenital heart disease. Of this group, 13% (17 of 127) of these arrests in noncardiac procedures were in patients with single ventricle physiology prior to a second-stage repair, such as a bidirectional Glenn or hemi-Fontan procedure (8). In our group of patients, we experienced one case of intraoperative cardiac arrest, with a frequency of 0.98%. Our patient most likely experienced a cardiac arrest due to failure to promptly intubate and adequately ventilate. This clearly demonstrates the fragility of these patients’ physiology; hypercarbia and hypoxia during airway management will cause decreased pulmonary blood flow, coronary ischemia, low cardiac output, and arrest. An examination of the National Inpatient Sample from 1988 to 1997, revealed hospital mortality rates among children with hypoplastic left heart syndrome for noncardiac surgery to be 17–22% (11). Similarly, in a review of 30-day mortality in Virginia in 2000, demonstrated that patients with congenital heart disease had an increased mortality when compared to patients with normal hearts (OR 3.5 [95% CI 3.2–3.9]) (12). This increased mortality effect was magnified in both neonates and infants. It is unclear as to whether congenital heart disease is a surrogate for other comorbidities or if mortality is related to heart disease itself. Both studies were based on registry data with limited individual patient data. No patients in our study died within 48 h after the noncardiac procedure. Our study was not designed to look at specific causes of overall mortality in these patients but rather to examine the risk of each anesthetic for a noncardiac procedure. Noncardiac surgery in patients with major cardiovascular disease has become more common as the prevalence 850
M.L. Brown et al.
of children living with cardiovascular disease increases. Almost 17% of the single ventricle patients in our study underwent noncardiac surgery prior to completion of a bidirectional Glenn. It is our institutional practice to delay any elective operation or procedure until after the bidirectional Glenn is complete, unless medically necessary. A recent study from Vanderbilt, identified patients prior to Stage II palliation (bidirectional Glenn) having the highest risk of hemodynamic instability, defined as >25% change in blood pressure from baseline for more than 10 min or significant rhythm change or major arrhythmia (10). Instability was also related to case complexity and preoperative use of angiotensin-converting enzyme inhibitors. At our institution, the current practice is to hold angiotensin-converting enzyme inhibitors the night before and morning of the procedure if possible. Christensen from Ann Arbor reported five procedures in patients who had undergone a Stage I palliation, and two of the five (40%) experienced respiratory instability (9). Of patients with single ventricle physiology, those who have undergone Stage I palliation with a systemic to pulmonary artery shunt are at very high risk of gastrointestinal complications in the perioperative period (13). In a study of 117 patients with hypoplastic left heart syndrome, who had undergone Stage I palliation with a BT shunt, gastrointestinal complications occurred in 41% including 18 patients who required either a gastrostomy or jejunostomy feeding tube and 7 patients who underwent a surgical antireflux procedure. These children often have multifactorial causes for failure-to-thrive, and it is very common to require noncardiac surgery to address feeding issues. Although our study was not designed to assess the risk of requiring a noncardiac procedure in patients with a single ventricle, our results are consistent with the above findings, as the most common noncardiac procedures performed were related to the gastrointestinal tract (n = 70, 57.8%). Our study was unable to identify risk factors to predict adverse events in this patient group. Age, weight, gender, urgency of procedure type of shunt, systemic ventricle, ventricular function, preoperative inotrope, type of operation, duration of procedure, location of the procedure, induction type, and anesthetic type were not significantly different between patient groups. This may be due to the small sample size and low frequency of adverse events. Using multi-institutional anesthesia databases to track outcomes would be helpful in this regard. The rate of early postoperative complications was 2 of 102 anesthetics (2%). All patients who came from the CICU returned to the CICU postoperatively. An additional 36% went to the CICU for either continued mechanical ventilation or close observation. At Boston © 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
M.L. Brown et al.
Single ventricle physiology undergoing noncardiac surgery
Children’s Hospital, patients with single ventricle physiology generally have a ‘backup’ bed available in the CICU, but may return to the cardiology floor at the discretion of the treating anesthesiologist. The model at Boston Children’s Hospital is one where noncardiac pediatric anesthesiologists are the primary anesthesiologists for all noncardiac surgery. The pediatric cardiac anesthesiologists provide consultation on all cases, and are available to help out in an emergency, but do not administer or manage the anesthetics directly. As such, we are unable to ascertain if differences in the type of anesthesiologist would make any difference to the rate of adverse events. We did not include procedures performed in the ICU because sedation is administered by the attending intensivist. We also did not include noninvasive procedures, which may require anesthesia, such as magnetic resonance imaging of the brain, echocardiography, or cardiac catheterization. Our intent was to examine in detail noncardiac operations performed in the noncardiac operating rooms and not diagnostic procedures. Limitations of this study relate primarily to the retrospective nature of this review. It is often difficult to determine the cause of adverse events. As well, we were relying on anesthetic records which have limitations previously identified (14–17). Our study is also limited by the single-center design, as we may not have been
adequately powered to detect whether significant differences in anesthetic technique influenced outcome. However, this single-center design does allow for detailed chart review and a granularity which is not possible from administrative or billing type database studies. Our study demonstrates an increased incidence of adverse advents in patients with single ventricle physiology undergoing noncardiac procedures. This increased risk supports the notion that these patients ought to be managed by an experienced and dedicated team of pediatric anesthesiologists in a tertiary medical center where cardiac surgeons, invasive cardiologists, and pediatric intensivists are readily available to promptly manage and prevent critical events. Ethics approval Institutional review board approval was obtained for this study (IRB-P00006493). Funding The study received no external funding. Disclosures The authors report no conflict of interest.
References 1 Jonas RA. Comprehensive Surgical Management of Congenital Heart Disease, 2nd edn. Boca Raton: CRC Press, 2014: 66. 2 Odegard KC, DiNardo JA, Kussman BD et al. The frequency of anesthesia-related cardiac arrests in patients with congenital heart disease undergoing cardiac surgery. Anesth Analg 2007; 105: 335–343. 3 White MC, Peyton JM. Anaesthetic management of children with congenital heart disease for non-cardiac surgery. Contin Educ Anaesth Crit Care Pain 2012; 12: 17–22. 4 Gottlieb EA, Andropoulos DB. Anesthesia for the patient with congenital heart disease presenting for noncardiac surgery. Curr Opin Anaesthesiol 2014; 26: 318–326. 5 Petruscak J, Smith R, Breslin P. Mortality related to ophthalmologic surgery. Surg Anesthesiol 1974; 18: 87–95. 6 Romano P. General anesthesia morbidity and mortality in eye surgery at children’s hospitals. J Pediatr Ophthalmol Strabismus 1981; 18: 17–21. 7 Odegard KC, Bergersen L, Thiagarajan R et al. The frequency of cardiac arrests in
© 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
8
9
10
11
patients with congenital heart disease undergoing cardiac catheterization. Anesth Analg 2014; 118: 175–182. Ramamoorthy C, Haberkern CM, Bhananker SM et al. Anesthesia-related cardiac arrest in children with heart disease: data from the Pediatric Perioperative Cardiac Arrest (POCA) registry. Anesth Analg 2010; 11: 1376– 1382. Christensen RE, Gholami AS, Reynolds PI et al. Anaesthetic management and outcomes after noncardiac surgery in patients with hypoplastic left heart syndrome: a retrospective review. Eur J Anaesthesiol 2012; 29: 425–430. Watkins SC, McNew BS, Donahue BS. Risks of noncardiac operations and other procedures in children with complex congenital heart disease. Ann Thorac Surg 2013; 95: 204–211. Torres A Jr, DiLiberti J, Pearl RH et al. Noncardiac surgery in children with hypoplastic left heart syndrome. J Pediatr Surg 2002; 37: 1399–1403.
12 Baum VC, Barton DM, Gutgesell HP. Influence of congenital heart disease on mortality after noncardiac surgery in hospitalized children. Pediatrics 2000; 105: 332–335. 13 Jeffries HE, Wells WJ, Starnes VA et al. Gastrointestinal morbidity after Norwood palliation for hypoplastic left heart syndrome. Ann Thorac Surg 2006; 81: 982–987. 14 Devitt JH, Rapanos T, Kurrek M et al. The anesthetic record: accuracy and completeness. Can J Anaesth 1999; 46: 122–128. 15 Thrush DN. Are automated anesthesia records better? J Clin Anesth 1992; 4: 386– 389. 16 Lerou JG, Dirksen R, VanDaele M et al. Automated charting of physiological variables in anesthesia: a quantitative comparison of automated versus handwritten anesthesia records. J Clin Monit 1988; 4: 37–47. 17 Sandberg WS, Sandberg EH, Seim AR et al. Real-time checking of electronic anesthesia records for documentation errors and automatically text message clinicians improves quality of documentation. Anesth Analg 2008; 106: 192–201.
851
ORIGINAL ARTICLE
Patients with single ventricle physiology undergoing noncardiac surgery are at high risk for adverse events Morgan L. Brown, James A. DiNardo & Kirsten C. Odegard Division of Cardiac Anesthesia, Department of Anesthesiology, Boston Children’s Hospital, Boston, MA, USA
What is already known
• Patients with single ventricle physiology undergoing noncardiac surgery are at high risk for adverse events, although the risk is not well quantified.
What this article adds
• The risk of a major adverse event in the intraoperative or perioperative period was 11.8%. • There was no early perioperative mortality associated with noncardiac surgery in patients with single ventricle physiology.
Implications for translation
• Noncardiac surgery in patients with single ventricle physiology has a high rate of adverse events at a large tertiary pediatric hospital.
• Further research using multi-institutional databases may be able to define specific subgroups of patients with single ventricle physiology who are at especially high risk for adverse events and ways to mitigate this risk.
Keywords anesthesia; Norwood procedures; heart defects; congenital; intraoperative complications; postoperative complications; surgical procedure; operative Correspondence Dr. Morgan L. Brown, Division of Cardiac Anesthesiology, Department of Anesthesiology, Boston Children’s Hospital, Bader 3, 300 Longwood Ave., Boston, MA 02115, USA Email: [email protected]. edu Section Editor: Greg Hammer Accepted 10 April 2015 doi:10.1111/pan.12685
846
Summary Background: Patients with single ventricle physiology are at increased anesthetic risk when undergoing noncardiac surgery. Objective: To review the outcomes of anesthetics for patients with single ventricle physiology undergoing noncardiac surgery. Methods: This study is a retrospective chart review of all patients who underwent a palliative procedure for single ventricle physiology between January 1, 2007 and January 31, 2014. Anesthetic and surgical records were reviewed for noncardiac operations that required sedation or general anesthesia. Any noncardiac operation occurring prior to completion of a bidirectional Glenn procedure was included. Diagnostic procedures, including cardiac catheterization, insertion of permanent pacemaker, and procedures performed in the ICU, were excluded. Results: During the review period, 417 patients with single ventricle physiology had initial palliation. Of these, 70 patients (16.7%) underwent 102 anesthetics for 121 noncardiac procedures. The noncardiac procedures included line insertion (n = 23); minor surgical procedures such as percutaneous endoscopic gastrostomy or airway surgery (n = 38); or major surgical procedures including intra-abdominal and thoracic operations (n = 41). These interventions occurred on median day 60 of life (1–233 days). The procedures occurred most commonly in the operating room (n = 79, 77.5%). Patients’ median weight was 3.4 kg (2.4–15 kg) at time of noncardiac intervention. In 102 anesthetics, 26 patients had an endotracheal tube or tracheostomy in situ, 57 patients underwent endotracheal intubation, and 19 patients had a natural © 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
M.L. Brown et al.
Single ventricle physiology undergoing noncardiac surgery
or mask airway. An intravenous induction was performed in 77 anesthetics, an inhalational induction in 17, and a combination technique in 8. The median total anesthetic time was 126 min (14–594 min). In 22 anesthetics (21.6%), patients were on inotropic support upon arrival; an additional 24 patients required inotropic support (23.5%), of which dopamine was the most common medication. There were 10 intraoperative adverse events (9.8%) including: arrhythmias requiring treatment (n = 4), conversion from sedation to a general anesthetic (n = 2), difficult airway (n = 1), inadvertent extubation with desaturation and bradycardia (n = 1), hypotension and desaturation (n = 1), and cardiac arrest (n = 1). Postoperative events (20% of baseline values, arrhythmias requiring treatment (medical or electrical), new inotropic support (hemodynamic instability), and any episodes of cardiac arrest requiring chest compressions were recorded. Additional adverse outcomes including transfer to a higher acuity care setting or death were recorded for the first 48 h following the anesthetic.
© 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
847
M.L. Brown et al.
Single ventricle physiology undergoing noncardiac surgery
For the purposes of analysis, procedure types were grouped into three categories. Line insertion included both peripherally inserted central catheter (PICC) placement, as well as other types of long-term central access devices. Minor surgical procedures included circumcisions, airway procedures, percutaneous endoscopic gastrostomy (PEG) tube insertion, and other endoscopic procedures. Major surgical procedures included laparoscopic gastric (G) tube insertion and any intra-abdominal or thoracic procedure. Procedures which involved more than one intervention were grouped into the highest category (e.g. PICC and laparoscopic G tube were considered as a major surgical procedure). Data are reported as means and standard deviations or medians and interquartile ranges as appropriate. Due to small sample size, comparisons were made using the Fisher’s exact test, Mann–Whitney U-test, or Kruskal– Wallis test, as appropriate. The statistical program JMP version 11 (Cary, NC, USA) was used for all analyses. Results We identified 417 patients with single ventricle physiology who had initial palliation during the study period. Of these, 70 (16.7%) patients underwent 102 anesthetics for 121 noncardiac procedures. Patients’ median weight was 3.4 kg (range 2.4–15 kg) at time of noncardiac intervention. Fifty-five (78.6%) of patients had a systemic right ventricle, 5 patients (7.1%) had moderate or severe ventricular dysfunction, and 12 (17.1%) patients had moderate or severe atrioventricular valvular regurgitation. Initial palliative procedures included a Sano shunt (n = 33, 47.1%), a BT shunt (n = 24, 34.3%), a hybrid technique consisting of a balloon atrial septostomy and a ductus arteriosus stent (n = 8, 11.4%), a pulmonary artery band (n = 3, 4.3%), and a central shunt (n = 1, 1.4%), Table 1. One patient had noncardiac surgery prior to any cardiac intervention. Twenty patients were admitted from the cardiac intensive care unit (CICU), 49 patients were inpatients, and 1 patient was a
Table 1 Type of single ventricle palliative procedure in patients for noncardiac surgery Palliative procedure
N, %
Stage I, with Sano shunt Stage I, with Blalock–Taussig shunt Blalock–Taussig shunt Hybrid—balloon atrial septostomy, ductus arteriosus stent Pulmonary artery band Stage I, with Central shunt
33, 47.1 12, 17.1 12, 17.1 8, 11.4
848
3, 4.3 1, 1.4
Table 2 Type of noncardiac procedure Noncardiac procedures
n = 121
Peripherally inserted central catheter line or central line Percutaneous endoscopic gastrostomy tube Laparascopic gastrostomy tube Airway procedures (including tracheostomy) Open gastrostomy Laparotomy Other Ladd’s procedure Diaphragmatic plication Circumcision Endoscopic gastroscopy and biopsy Gastrostomy tube in interventional radiology
25 22 22 12 9 7 7 5 4 3 3 2
same-day admission at the time of the noncardiac surgery. A total of 121 procedures (Table 2) were performed under 102 separate anesthetics. In 77 anesthetics (75.5%), an intravenous (IV) induction was performed; in 17 anesthetics (16.7%), an inhaled induction was performed; and in 8 anesthetics (7.8%), a combination of intravenous (IV) agents and inhaled anesthetics were utilized for induction. In 102 anesthetics, 26 patients had an endotracheal tube or tracheostomy in situ, 57 patients underwent endotracheal intubation, and 19 patients had a natural or mask airway. In 25 anesthetics, patients had an arterial line; 13 were placed at the time of the noncardiac procedure. In 28 anesthetics, patients had either a central line or a PICC line. The median total anesthesia time was 126 min (range 14–594 min). Eighty-five of 102 anesthetics (83.3%) were for procedures that were considered elective and were performed during normal working hours. At the time of noncardiac surgery, patients were directly transferred from the CICU in 40 anesthetics (39.2%). Of the remaining 62 anesthetics, an additional 22 patients (35.5%) went to the cardiac CICU postoperatively. After the remainder of anesthetics (40 anesthetics, 39.2%), patients were admitted to the cardiology floor after the procedure. There were no deaths at 48 h. In 22 anesthetics (21.6%), patients were on inotropic support already on arrival to the operating room. An additional 24 patients (23.5%) required inotropic support during the procedure, of which dopamine was the most common medication. There were 10 intraoperative and 2 postoperative adverse events (n = 12, 11.8%). Intraoperative arrhythmias encountered included bradycardia requiring atropine (n = 3) and supraventricular tachycardia requiring adenosine (n = 1). One patient had significant intraoperative hypotension and desaturation of which the cause was unclear. It resolved with administration of volume, inotropic support, and vigorous © 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
M.L. Brown et al.
Single ventricle physiology undergoing noncardiac surgery
Table 3 Differences between patients who did or did not experience an adverse event Median age at noncardiac surgery (days) Male gender Median weight at noncardiac surgery (kg) Systemic right ventricle Sano shunt vs other Elective vs urgent/emergent Moderate or severe ventricular dysfunction Preoperative inotrope Preoperative admission location—ICU Location of procedure operating room vs other Median duration of anesthesia (h, IQR) General anesthesia (vs sedation) IV induction vs other Procedure type Line insertion Minor Major
No event n = 90
Adverse event n = 12
P value
72.5 (40.3–117.0) 56 (62.2%) 3.5 (3.1–4.2) 60 (66.7%) 38 (42.2%) 75 (83.3%) 7 (7.8%)
67.5 (31.8–131.5) 10 (83.3%) 3.3 (2.9–3.7) 8 (66.7%) 3 (25.0%) 10 (83.3%) 0 (0%)
0.25 0.21 0.15 1.0 0.33 1.0 1.0
18 (20.0%) 34 (37.8%) 72 (80.0%)
4 (33.3%) 6 (50.0%) 7 (58.3%)
0.28 0.53 0.14
2.0 (1.3–2.8) 76 (84.4%) 67 (74.4%)
2.4 (1.7–2.9) 10 (83.3%) 10 (83.3%)
0.27 0.72 0.73
21 (23.3%) 32 (35.6%) 37 (40.7%)
2 (16.7%) 6 (50%) 4 (33.3%)
0.62
An adverse event was defined as any major anesthetic complication including conversion from sedation to a general anesthetic, difficult airway, extubation requiring reintubation, desaturation or bradycardia >20% of baseline variables, arrhythmias requiring treatment, new inotropic support, cardiac arrest, transfer to a higher acuity care setting, or death within the first 48 h after the anesthetic.
hand-ventilation. One patient experienced ST segment changes within 24-h postanesthetic. This child was transferred to the CICU and underwent cardiac catheterization the following day, which revealed a residual aortic arch gradient. The patient underwent successful balloon dilation. Difficulties with airway management were encountered in six patients; two patients who initially were sedated with IV midazolam and ketamine during spontaneous respiration for PICC placement required conversion into a general anesthetic. One patient was receiving supplemental oxygen before the procedure and one was receiving continuous positive pressure airway (CPAP) support. Both experienced arterial desaturation which led to urgent, noncomplicated endotracheal intubation. The remainder of both anesthetics was uneventful. One patient was a known difficult airway requiring three attempts at intubation, placement of a laryngeal mask airway, and intubation through the laryngeal mask airway. One patient was inadvertently extubated during positioning in the lateral position and required reintubation. One patient scheduled for gastrostomy tube insertion experienced cardiac arrest requiring cardiac compressions and inotropic support following a failure to promptly intubate and ventilate the patient after induction. This patient went to the cardiac catheterization following the procedure to assess the status of © 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
the Sano shunt after chest compressions. Due to the positioning of the Sano shunt directly underneath the sternum, these patients are predisposed to compression of the shunt during a cardiac arrest. Cardiac catheterization revealed that the Sano shunt was patent, but narrowed. A stent was placed and the atrial septal defect was dilated due to a residual gradient. Lastly, one patient who underwent insertion of a PEG tube experienced a cardiorespiratory arrest within 24 h of the anesthetic while in the CICU. The etiology of the arrest was thought to be pulmonary overcirculation with poor coronary perfusion and low cardiac output. The child was successfully placed on an extracorporeal membrane oxygenator (ECMO) circuit and survived. Age, patient weight, time after initial cardiac surgery, gender, type of cardiac palliation, patient admission location, procedure location, and type of procedure were not significantly different between patients who did or did not experience an adverse outcome (Table 3). The longest procedure (594 min) was in an 8-monthold patient with hypoplastic left heart syndrome, who underwent a Stage I repair with a BT shunt, which was subsequently revised to a central shunt. He underwent a revision of a laparoscopic Nissen procedure which was complicated by difficult exposure and dense adhesions. This patient had a PICC line in situ, and an arterial line was placed for the procedure. The patient required 849
Single ventricle physiology undergoing noncardiac surgery
inotropic support for the majority of the procedure, but there were no adverse events. The patient was taken to the CICU postoperatively. Discussion In this single-center review of patients with single ventricle physiology undergoing noncardiac procedures, we demonstrate a high rate (11.8%) of intraoperative and early postoperative adverse events in this patient population. This is consistent with the literature which consistently identifies patients with single ventricle physiology to be at high risk for perioperative complications (8–10). At Boston Children’s Hospital, a review of cardiac arrests in children with congenital heart disease undergoing cardiac surgery, revealed an arrest frequency of 0.79% (2). Nine of these 41 arrests (22%) were in patients with single ventricle physiology. Similarly, the Pediatric POCA Registry reported that 34% of 373 cases of anesthesia-related cardiac arrest were in patients with congenital heart disease. Of this group, 13% (17 of 127) of these arrests in noncardiac procedures were in patients with single ventricle physiology prior to a second-stage repair, such as a bidirectional Glenn or hemi-Fontan procedure (8). In our group of patients, we experienced one case of intraoperative cardiac arrest, with a frequency of 0.98%. Our patient most likely experienced a cardiac arrest due to failure to promptly intubate and adequately ventilate. This clearly demonstrates the fragility of these patients’ physiology; hypercarbia and hypoxia during airway management will cause decreased pulmonary blood flow, coronary ischemia, low cardiac output, and arrest. An examination of the National Inpatient Sample from 1988 to 1997, revealed hospital mortality rates among children with hypoplastic left heart syndrome for noncardiac surgery to be 17–22% (11). Similarly, in a review of 30-day mortality in Virginia in 2000, demonstrated that patients with congenital heart disease had an increased mortality when compared to patients with normal hearts (OR 3.5 [95% CI 3.2–3.9]) (12). This increased mortality effect was magnified in both neonates and infants. It is unclear as to whether congenital heart disease is a surrogate for other comorbidities or if mortality is related to heart disease itself. Both studies were based on registry data with limited individual patient data. No patients in our study died within 48 h after the noncardiac procedure. Our study was not designed to look at specific causes of overall mortality in these patients but rather to examine the risk of each anesthetic for a noncardiac procedure. Noncardiac surgery in patients with major cardiovascular disease has become more common as the prevalence 850
M.L. Brown et al.
of children living with cardiovascular disease increases. Almost 17% of the single ventricle patients in our study underwent noncardiac surgery prior to completion of a bidirectional Glenn. It is our institutional practice to delay any elective operation or procedure until after the bidirectional Glenn is complete, unless medically necessary. A recent study from Vanderbilt, identified patients prior to Stage II palliation (bidirectional Glenn) having the highest risk of hemodynamic instability, defined as >25% change in blood pressure from baseline for more than 10 min or significant rhythm change or major arrhythmia (10). Instability was also related to case complexity and preoperative use of angiotensin-converting enzyme inhibitors. At our institution, the current practice is to hold angiotensin-converting enzyme inhibitors the night before and morning of the procedure if possible. Christensen from Ann Arbor reported five procedures in patients who had undergone a Stage I palliation, and two of the five (40%) experienced respiratory instability (9). Of patients with single ventricle physiology, those who have undergone Stage I palliation with a systemic to pulmonary artery shunt are at very high risk of gastrointestinal complications in the perioperative period (13). In a study of 117 patients with hypoplastic left heart syndrome, who had undergone Stage I palliation with a BT shunt, gastrointestinal complications occurred in 41% including 18 patients who required either a gastrostomy or jejunostomy feeding tube and 7 patients who underwent a surgical antireflux procedure. These children often have multifactorial causes for failure-to-thrive, and it is very common to require noncardiac surgery to address feeding issues. Although our study was not designed to assess the risk of requiring a noncardiac procedure in patients with a single ventricle, our results are consistent with the above findings, as the most common noncardiac procedures performed were related to the gastrointestinal tract (n = 70, 57.8%). Our study was unable to identify risk factors to predict adverse events in this patient group. Age, weight, gender, urgency of procedure type of shunt, systemic ventricle, ventricular function, preoperative inotrope, type of operation, duration of procedure, location of the procedure, induction type, and anesthetic type were not significantly different between patient groups. This may be due to the small sample size and low frequency of adverse events. Using multi-institutional anesthesia databases to track outcomes would be helpful in this regard. The rate of early postoperative complications was 2 of 102 anesthetics (2%). All patients who came from the CICU returned to the CICU postoperatively. An additional 36% went to the CICU for either continued mechanical ventilation or close observation. At Boston © 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
M.L. Brown et al.
Single ventricle physiology undergoing noncardiac surgery
Children’s Hospital, patients with single ventricle physiology generally have a ‘backup’ bed available in the CICU, but may return to the cardiology floor at the discretion of the treating anesthesiologist. The model at Boston Children’s Hospital is one where noncardiac pediatric anesthesiologists are the primary anesthesiologists for all noncardiac surgery. The pediatric cardiac anesthesiologists provide consultation on all cases, and are available to help out in an emergency, but do not administer or manage the anesthetics directly. As such, we are unable to ascertain if differences in the type of anesthesiologist would make any difference to the rate of adverse events. We did not include procedures performed in the ICU because sedation is administered by the attending intensivist. We also did not include noninvasive procedures, which may require anesthesia, such as magnetic resonance imaging of the brain, echocardiography, or cardiac catheterization. Our intent was to examine in detail noncardiac operations performed in the noncardiac operating rooms and not diagnostic procedures. Limitations of this study relate primarily to the retrospective nature of this review. It is often difficult to determine the cause of adverse events. As well, we were relying on anesthetic records which have limitations previously identified (14–17). Our study is also limited by the single-center design, as we may not have been
adequately powered to detect whether significant differences in anesthetic technique influenced outcome. However, this single-center design does allow for detailed chart review and a granularity which is not possible from administrative or billing type database studies. Our study demonstrates an increased incidence of adverse advents in patients with single ventricle physiology undergoing noncardiac procedures. This increased risk supports the notion that these patients ought to be managed by an experienced and dedicated team of pediatric anesthesiologists in a tertiary medical center where cardiac surgeons, invasive cardiologists, and pediatric intensivists are readily available to promptly manage and prevent critical events. Ethics approval Institutional review board approval was obtained for this study (IRB-P00006493). Funding The study received no external funding. Disclosures The authors report no conflict of interest.
References 1 Jonas RA. Comprehensive Surgical Management of Congenital Heart Disease, 2nd edn. Boca Raton: CRC Press, 2014: 66. 2 Odegard KC, DiNardo JA, Kussman BD et al. The frequency of anesthesia-related cardiac arrests in patients with congenital heart disease undergoing cardiac surgery. Anesth Analg 2007; 105: 335–343. 3 White MC, Peyton JM. Anaesthetic management of children with congenital heart disease for non-cardiac surgery. Contin Educ Anaesth Crit Care Pain 2012; 12: 17–22. 4 Gottlieb EA, Andropoulos DB. Anesthesia for the patient with congenital heart disease presenting for noncardiac surgery. Curr Opin Anaesthesiol 2014; 26: 318–326. 5 Petruscak J, Smith R, Breslin P. Mortality related to ophthalmologic surgery. Surg Anesthesiol 1974; 18: 87–95. 6 Romano P. General anesthesia morbidity and mortality in eye surgery at children’s hospitals. J Pediatr Ophthalmol Strabismus 1981; 18: 17–21. 7 Odegard KC, Bergersen L, Thiagarajan R et al. The frequency of cardiac arrests in
© 2015 John Wiley & Sons Ltd Pediatric Anesthesia 25 (2015) 846–851
8
9
10
11
patients with congenital heart disease undergoing cardiac catheterization. Anesth Analg 2014; 118: 175–182. Ramamoorthy C, Haberkern CM, Bhananker SM et al. Anesthesia-related cardiac arrest in children with heart disease: data from the Pediatric Perioperative Cardiac Arrest (POCA) registry. Anesth Analg 2010; 11: 1376– 1382. Christensen RE, Gholami AS, Reynolds PI et al. Anaesthetic management and outcomes after noncardiac surgery in patients with hypoplastic left heart syndrome: a retrospective review. Eur J Anaesthesiol 2012; 29: 425–430. Watkins SC, McNew BS, Donahue BS. Risks of noncardiac operations and other procedures in children with complex congenital heart disease. Ann Thorac Surg 2013; 95: 204–211. Torres A Jr, DiLiberti J, Pearl RH et al. Noncardiac surgery in children with hypoplastic left heart syndrome. J Pediatr Surg 2002; 37: 1399–1403.
12 Baum VC, Barton DM, Gutgesell HP. Influence of congenital heart disease on mortality after noncardiac surgery in hospitalized children. Pediatrics 2000; 105: 332–335. 13 Jeffries HE, Wells WJ, Starnes VA et al. Gastrointestinal morbidity after Norwood palliation for hypoplastic left heart syndrome. Ann Thorac Surg 2006; 81: 982–987. 14 Devitt JH, Rapanos T, Kurrek M et al. The anesthetic record: accuracy and completeness. Can J Anaesth 1999; 46: 122–128. 15 Thrush DN. Are automated anesthesia records better? J Clin Anesth 1992; 4: 386– 389. 16 Lerou JG, Dirksen R, VanDaele M et al. Automated charting of physiological variables in anesthesia: a quantitative comparison of automated versus handwritten anesthesia records. J Clin Monit 1988; 4: 37–47. 17 Sandberg WS, Sandberg EH, Seim AR et al. Real-time checking of electronic anesthesia records for documentation errors and automatically text message clinicians improves quality of documentation. Anesth Analg 2008; 106: 192–201.
851
