Curr Treat Options Cardio Med (2015) 17:3 DOI 10.1007/s11936-014-0362-x
Adult Congenital Heart Disease (A Bhatt and K Niwa, Section Editors)
Critical Care Management of the Adult Patient with Congenital Heart Disease: Focus on Postoperative Management and Outcomes Nathalie Roy, MD, FRCSC Address Division of Cardiac Surgery, Massachusetts General Hospital, 55 Fruit St, Blake 872, Boston, MA 02114, USA Email: [email protected] 2 Harvard Medical School, Boston, MA, USA
* Springer Science+Business Media New York 2015
This article is part of the Topical Collection on Adult Congenital Heart Disease Keywords Adult congenital heart disease I Critical care I Cardiac surgery I Outcomes I Arrhythmia I Heart failure I Death I Acute kidney injury I Liver failure I Coagulopathy I Thrombosis I Obesity I Delirium
Opinion statement Advances in surgical techniques and in the medical management of children with congenital heart disease has increased survival into adulthood, resulting in a population of adults with congenital heart disease now surpassing the pediatric population in numbers. Furthermore, many of the patients will require repeat surgical, catheter-based, procedures and/or obstetrical care in their adult lives, and understanding the specific cardiopulmonary physiology and the involvement of other organ systems is critical to successful intervention. A team approach, with consultants from medical specialties in the setting of an established adult congenital heart center, is the optimal setting for superior outcomes. In this review, we discuss critical care management of the adult congenital heart disease patient in the perioperative period.
Introduction In recent decades, the number of patients with adult congenital heart disease (ACHD) has surpassed the number of children [1]. Indeed, approximately 85 % of patients born with congenital heart disease now
survive into adulthood, and the population with ACHD is now estimated at over 1 M in the United States and over 1.2 M in Europe [2]. Furthermore, the incidence of newly diagnosed conditions in
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adulthood is approximately equal to the number of patients needing a reoperation following palliation or corrective surgery [3]. The development of new surgical techniques for neonates with congenital heart disease (CHD), improvement in anesthesia and critical care, and catheter-based interventions all account for the improved survival of these patients [4]. However, many patients will need further surgical or catheter-based reintervention, or admission for heart failure or for noncardiac perioperative or peripartum management. In the recent years, certain centers have reviewed surgical outcomes in ACHD [5, 6]. Price et al [7•] looked at critical care outcomes of ACHD patients prospectively. They reported an overall mortality of 4.4 %. Mortality was 0 % in the patients with simple
lesions, but the mortality was 10.6 % in patients with lesions of moderate to severe complexity. In this study, the standard severity-of-illness scores were poorly predictive of perioperative mortality. Finally, the patients required high resource utilization in the intensive care unit (ICU) for diagnostics, as well as special therapies such as renal replacement therapy. There is growing interest in critical care management of these unique patients, as evidenced by a number of recent reviews [8–12]. However, there is a paucity of evidence-based practices due, in part to disease variability and small number of patients. The purpose of the present review is to address current trends in postoperative critical care management of adult patients with congenital heart disease, focusing on postoperative management and outcomes.
Critical care management of the adult patient with congenital heart disease Cardiac surgery for the patient with ACHD is performed for primary repair or reoperation [3]. Fortunately, patients with previously undiagnosed lesions amenable to primary repair in adulthood often fall in the noncomplex category. Conversely, patients requiring reoperations for ACHD often present with complex physiology, reduction in ventricular function and, as a result, chronic changes in many organ systems can complicate their management. A multidisciplinary approach to patient selection and to perioperative management should include the cardiac surgeon, adult congenital cardiologist, anesthesiologist and intensivist, along with specialist consultants from renal, hematology, hepatology, and neuropsychiatry.
Cardiovascular system Arrhythmias Arrhythmias are very prevalent in adult patients with congenital heart defects [13•], and are a frequent occurrence after cardiac surgery for adult congenital disease [14•]. Certain patients have an anatomical substrate predisposing them to arrhythmias (eg, heterotaxy, CCTGA). In most cases, it is the result of previous intervention, palliation, or longstanding pathophysiology such as atrioventricular valve regurgitation, baffle obstruction, atrial and ventricular dilatation, myocardial hypertrophy, and heart failure [11]. It is estimated that up to one-third of patients with ACHD have supraventricular arrhythmias [15, 16], leading to major morbidity and sudden death [17]. Nevertheless, the leading cause of sudden death in ACHD patients is ventricular arrhythmias, often the result of ventricular dysfunction [16]. Ventricular arrhythmias have been reported most commonly with systemic right ventricles (such as in transposition of the great arteries (D-TGA) with atrial-level
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repair or corrected transposition (L-TGA)), Eisenmenger syndrome, tetralogy of Fallot, and in patients with severe left-sided ventricular obstruction. Khairy et al [18] described a risk score for sudden death in patients with tetralogy of Fallot in order to determine guidelines for preventive implantable cardioverterdefibrillator (ICD) implantation in this population, but data lacks in other physiologies. ICDs should be considered in the setting of sustained ventricular tachycardia or cardiac arrest, but even the use of established heart failure guidelines for ICD [19] are often not observed in the ACHD patient population because of lack of evidence. Furthermore, anatomical and technical issues, such as baffles, presence of right-sided artificial valves, right-to-left shunts, and history of (or the risk of thrombosis associated with) intravascular leads, complicate the insertion of ICDs. Koyak et al [14•] reviewed the incidence of postoperative arrhythmia in ACHD. The multicenter retrospective study found an incidence of postoperative arrhythmia of 32 %, the majority occurring in the first 3 days after surgery. Twenty-four percent (24 %) of patients developed supraventricular arrhythmias, 11 % experienced bradyarrhythmias, and ventricular tachycardia was seen in 5 % of the patients. Arrhythmias were more frequent with intracardiac repair, vs surgical repair involving the aortic root. All types of arrhythmias were hemodynamically significant, resulting in a twofold increase in ICU stay, increased likelihood of pacemaker implantation (5 % of the patients) especially in the patients experiencing bradyarrhythmias, and increase in adverse events, including heart failure and death. In a multivariate analysis, significant risk factors for postoperative arrhythmia were older age, New York Heart Association (NYHA) class, moderate or more atrioventricular valve regurgitation of the subpulmonary ventricle, cardiopulmonary bypass (CPB) time, and increased level of the cardiac enzyme (CK-MB) postoperatively. Supraventricular arrhythmias were treated most frequently with beta-blockers and amiodarone, and approximately one-third of the patients required long-term antiarrhythmic therapy. Conversely to surgery for acquired disease [20], there are no data on the prophylactic use of beta-blockers to prevent the incidence of postoperative arrhythmias. The reluctance in adopting a prophylactic use of these agents may be related to their negative inotropic effect in a patient population with depressed ventricular function at baseline. Patients presenting for surgery with an ICD device should have the defibrillation function turned off at the time of induction. Furthermore, it is important to recognize that leads may be displaced, and intraoperative interrogation at the end of the procedure is critical. The defibrillation function should be turned back on before the patient transfers from the intensive care unit to the stepdown unit.
Ventricular function, heart failure, and support Patients with ACHD often have a decline in ventricular function and may be admitted to the ICU as a result of heart failure for tailored assessment of their physiology and medical management of heart failure. The use of preoperative and intraoperative echo is critical to evaluate the repair, but also to determine the presence of residual shunts, gradients, and valvular regurgitation, as well as the ventricular function. Central and intracardiac pressure monitoring is useful to determine right and left ventricular preload, pulmonary pressures, and allow
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mixed venous saturation measurement. Certain patients have anatomical reasons that make the use of flow-directed PA catheters difficult, and transcutaneous left atrial line monitoring may be helpful in postoperative management. Continuous transesophageal echocardiography monitoring with the use of disposable miniaturized probes may be helpful in assessing preload, ventricular function, and response to interventions in the critically ill patient [21]. Factors associated with postoperative adverse events in ACHD patients [5] are increased NYHA class and longer CBP times. Holst et al reviewed their experience in patients undergoing multivalve operations for ACHD [6], and found that the risk factors for early mortality included nonelective surgery, increased CPB and ischemic times, lower hematocrit, increased creatinine, and mechanical ventilation greater than 5 days. The development of right ventricular failure is associated with high mortality in the postoperative period. In the study by Schuuring et al [22], the postoperative incidence of right ventricular failure was 4.4 %. Patients with repairs involving the systemic circulation and the subaortic ventricle, and repairs involving both ventricles, have a greater risk than patients having surgery involving the subpulmonary ventricle alone. Other risk factors of postoperative right ventricular failure are increased NYHA class (≥2), preoperative supraventricular arrhythmia, longer CPB times (9150 m) and decreased creatinine clearance. The challenge is prompt recognition and providing adequate support. Right ventricular failure manifests by an increase in jugular venous pressure, associated with systemic hypotension, renal and hepatic dysfunction, and ascites. If left untreated, it can lead to gut ischemia from low cardiac output, superimposed with increased venous pressure. The management includes inotropic support of the right ventricle with phosphodiesterase inhibitors and adrenaline, aggressive management of pulmonary hypertension with inhaled agents (NO, inhaled prostacyclin), as well as systemic support of the blood pressure. Fluid removal with diuresis or early continuous venovenous hemofiltration (CVVH) is important to prevent end-organ damage. Temporary right-sided mechanical support can be performed using a Centrimag pump (Thoratec Corporation, Pleasantville, CA).
Mechanical circulatory support Published reports of extracorporeal membrane oxygenation (ECMO) for congenital heart disease include mostly pediatric patients, and few publications include adult patients. ECMO in children is mostly used as a salvage therapy postcardiotomy, or for end stage heart failure, or periprocedure in the setting of high-risk catheter-based intervention [23]. In ACHD, the existence of multiorgan dysfunction often leads to renal and hepatic failure, neurologic injury and bleeding complications in the setting of ECMO, and contributes to poor results in this population. Furthermore, vascular access is often compromized and limits the success of this therapy. Everitt et al [19] reviewed the United Network for Organ Sharing database for ACHD patients listed for heart transplantation from 2005–2009. They identified only five patients supported on ECMO out of 314 patients with ACHD. Furthermore, only 16 patients were supported by a ventricular assist device, emphasizing the underutilization of this therapy in ACHD. In recent
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years, a few case reports of ventricular assist device (VAD) implantation in the setting of ACHD were published, including a three-case series [24] of patients with D-TGA and L-TGA. The paper focused on implantation technique in patients with systemic right ventricles. A publication by Shah et al [25] reported six (6) ACHD patients supported on a VAD: they reported one early (G30 days) and one late mortality (262 days), two patients supported on DT-VADs for over 1 year, and two patients successfully transplanted. One of the patients was supported for 988 days prior to receiving his organ. The perioperative morbidity was significant. Adults with complex congenital heart disease experience earlier deaths [1], mostly because of heart failure and multi end-organ dysfunction, as a result of longstanding cardiac pathophysiology. Unfortunately, despite a growing patient population, mechanical circulatory support and heart transplantation remains fairly uncommon in ACHD, with longer times awaiting organ allocation [19]. This is related, in part, to prior sensitization [9], and to secondary organ dysfunction and the need for dual organ transplantation.
Respiratory system Cardiopulmonary interactions and mechanical ventilation Cardiopulmonary interactions are important in the patient with complex congenital heart disease [26]. These patients are likely to have impaired right ventricular function, and the negative impact of positive pressure ventilation and positive end-expiratory pressure (PEEP) can lead to decreased left ventricular filling and low cardiac output. The goal should be early extubation, especially in patients with good hemodynamics and low inotrope scores. However, in the setting of marginal left ventricular function, PEEP decreases left ventricular wall stress, and premature extubation in the setting of fluid overload can lead to respiratory failure. Extubation to noninvasive positive pressure ventilation may be beneficial [27], especially in patients with a history of sleep apnea. The use of low dose dexmedetomidine may be helpful to reduce the need for narcotic and anxiolytics. Weissmann et al [28] looked at the practice of extubating ACHD patients in the operating room. They found that low Risk Adjusted classification for Congenital Heart Surgery score, and low body mass index in the setting of increased disease complexity correlated with early extubation. For patients who remain sedated for hemodynamic reasons, keeping the mean airway pressure low and avoiding pulmonary vasoconstriction is important. Thus, targeting a normal acid-base balance and PCO2, and preventing hypoxemia.
Acute kidney injury and fluid control Fifty percent (50 %) of patients with congenital heart disease have some degree of chronic kidney disease [29], and 9 % have moderate to severe reductions in glomerular filtration rate (GFR). Contributing factors include previous exposure to cardiopulmonary bypass, cyanosis [30], and the effects of years of pathologic circulation with marginal systemic cardiac output and elevated venous pressures. There is a three-fold increase in mortality at baseline in the ACHD
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Curr Treat Options Cardio Med (2015) 17:3 patients with moderate and severe renal dysfunction [29]. Furthermore, there is increased perioperative mortality in ACHD patients with renal disease [7]. The goal of perioperative management of ACHD patients with renal dysfunction undergoing cardiac surgery is to maintain an adequate organ perfusion pressure (ie, MAP – CVP or intra-abdominal pressure), and cardiac output. Early postoperative diuresis is important in order to optimize right ventricular preload in the setting of right ventricular dysfunction. Early fluid removal with continuous veno-veno hemofiltration (CVVH) can be helpful in managing fluid overload and increased right-sided venous pressure. It should be initiated promptly in the setting of oliguria and decreasing GFR. A retrospective study [31] looking at the timing for initiation of CVVH in acute renal failure postcardiotomy suggested that early CVVH (G1 day) conferred a survival advantage over late initiation of CVVH. A clinical trial evaluating this strategy is currently underway. In addition, glucose control has been associated with a reduction in the need for renal replacement therapy [32]. A meta-analysis looking at the use of fenoldopam after cardiac surgery also found benefits in preventing renal replacement therapy [33], but this strategy is not widely adopted. Many clinical trials are currently underway to evaluate different renoprotective drugs in the setting of cardiac surgery (clinicaltrial.gov). However, there is no published data in ACHD, and this population is often specifically excluded from clinical trials.
GI system Hepatic dysfunction Hepatic dysfunction of varying degrees is very common in ACHD patients [34]. It often results from chronic venous congestion associated with single ventricular repair and Fontan physiology, or in the context of right ventricular dysfunction. Other contributors to hepatic dysfunction include marginal systemic cardiac output, steel from systemic-to-pulmonary shunts, and the added hepatic insults from cardiopulmonary bypass, multiple transfusions with their inherent infectious risks, and hepatotoxic drugs. Over time, cardiac cirrhosis may develop, resulting in portal hypertension with ascites and porto-systemic varices. The metabolic anomalies associated with passive congestion in the liver are often increased bilirubin and prolonged Prothrombine Time (PT/INR). Low cardiac output and liver ischemia tend to cause an elevation in transaminases, but low transaminase levels are also possible as a result of chronic ischemia and fibrosis. Acute liver dysfunction is likely to be associated with encephalopathy and jaundice. The patient most at risk for liver dysfunction in the CHD population is the adult with a failing Fontan physiology. The risk of developing hepatic complications increases over time, and cirrhosis is seen as early as 10 years after Fontan completion [35]. On histology, there is sinusoidal fibrosis in the space of Disse and sinusoidal dilatation from zone 3 to the portal tract [36]. These patients present with ascites, hyperbilirubinemia, and coagulopathy, and can also present with bleeding varices. Minimizing pulmonary pressures by avoidance of intubation and by drainage of pleural effusions and ascites is essential. Optimizing cardiac output may require inotropes, reduction of venous pressure with aggressive diuresis or CVVH, and drainage of ascites. Management also involves nutritional support, correction of coagulopathy, and monitoring for hepatic encephalopathy.
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Liver dysfunction has a significant impact on perioperative mortality [7•]. Indeed, nonliver transplantation surgery in patients with cirrhosis is associated with significant mortality. In patients undergoing adult cardiac surgery, liver disease greater than Childs-A is associated with high morbidity and mortality [37]. The Model for End-Stage Liver Disease score is predictive of outcomes after cardiac surgery [38] and was recently found to be predictive of sudden cardiac death, death from congestive heart failure, and death after cardiac transplantation in the Fontan population [39]. In regards to heart transplantation, it remains unknown whether early heart transplantation may avoid the need for liver transplantation in the setting of congenital heart disease and liver dysfunction. Isolated heart transplantation for failing Fontan has significantly lower 1- and 5-yeat survival; 63 % and 57 %, respectively [40]. Early deaths were related to bleeding, sepsis and multiorgan failure. Regarding dual organ transplantation, cardiac cirrhosis represents only 11 % of the combined heart-liver transplants, with congenital heart disease representing only 1 % (13 % of patients with cardiac cirrhosis). The most common diagnosis for heart-liver transplantation is amyloidosis.
Abdominal compartment syndrome In the setting of right ventricular dysfunction and low postoperative cardiac output, ascites may develop, causing an increase in intra-abdominal pressure and abdominal compartment syndrome [41]. Abdominal compartment syndrome is associated with acute kidney injury, gut ischemia, acute lung injury from high airway pressures, and circulatory collapse. The diagnosis is confirmed by measuring the bladder pressure (920 Torr) and requires an emergent laparotomy for relief of abdominal pressure.
Hematologic considerations Patients with ACHD are at risk of bleeding and thrombosis [42]. From a technical perspective, re-entry into the chest after multiple surgeries can lead to significant blood loss, especially because many patients have chest wall and mediastinal collaterals. Recent evidence by Jensen et al suggests that cyanotic patients have impaired hemostasis [43, 44]. Patients with Fontan physiology have lower levels of procoagulant and anticoagulant factors [45], and the clinical significance is uncertain. Another consideration is the presence of foreign material, especially in the venous circulation where stenosis and stasis promote thrombosis. Venous thromboembolism prophylaxis should be considered in children with congenital heart disease postoperatively [46]. Although there is no consensus in ACHD, venous thromboembolism prophylaxis is suggested postoperatively for the reasons aforementioned. Management of the bleeding and thrombotic risks is complex in this population, and because of the paucity of evidence, it should be individualized [42].
Endocrine system Glucose control Glucose control can significantly improve morbidity and mortality after cardiac surgery in the adult patient. The landmark trial by Van den Berghe in 2001 [32]
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Curr Treat Options Cardio Med (2015) 17:3 showed a reduction in mortality, and morbidity in patients treated with tight glucose control. The findings demonstrated a reduced renal replacement therapy and lower risk for hyperbilirubinemia, earlier wean from mechanical ventilation, and earlier discharge from the hospital and the ICU. A subanalysis of the high-risk cardiac surgical patients in this study [47] showed maintenance of the survival benefit seen with intensive insulin therapy at 4 years after discharge. However, controversy still persists regarding how tight glucose control should be, with one study suggesting that moderate is superior to tight control [48]. At the first international consensus conference on mortality reduction in cardiac surgical anesthesia and critical care [49], perioperative insulin therapy during cardiac surgery was highly ranked as a possible lifesaving ancillary technique in cardiac anesthesia and intensive care. In fact, the benefits of insulin administration in cardiac surgery appear to extend beyond the avoidance of hyperglycemia. Normoglycemia achieved through administration of glucose, insulin, and potassium (GIK) appears cardioprotective [50–53]. The caveat is whether these findings can be extrapolated to the ACHD patient population, who are typically younger and free of significant coronary artery disease. A recent randomized trial in pediatric cardiac surgical patients [54] found that tight glycemic control did not significantly impact the infection rate, mortality, length of stay, or measures of organ failure, compared with standard care. Hypoglycemia infrequent (3 %) in this population and was not associated with adverse events. The current guidelines for the use of insulin for glucose management in critically ill patients [55] suggest that patients undergoing cardiac surgery should maintain moderate glucose control using a reliable insulin infusion protocol and a target blood glucose of less than 150 mg/dL.
Obesity As opposed to children with congenital heart disease, who tend to have failure to thrive and malnutrition, the prevalence of obesity in ACHD patients ranges from 10 %–30 % [56, 57]. This may be partly related to sedentary lifestyle. In two recent studies [58, 59•], obesity was not associated with worse surgical outcomes in patients with ACHD.
Thyroid function In the study by Price et al [7•], preoperative abnormal thyroid function was predictive of mortality postcardiac surgery and in patients admitted to the ICU for medical or surgical complications. A thyroid-stimulating hormone (TSH) screening is recommended.
Sedation and delirium prevention Two-thirds of patients with ACHD have been subject to previous surgery and anesthesia, and tolerance to analgesics and sedatives in not uncommon. No specific data exist in this population, but newer agents such as dexmedetomidine appear to be safe in adults [60] and children [61]. Furthermore, dexmedetomidine has favorable sedative and anxiolytic properties and limited effects on hemodynamic and respiratory function. As a result, there is growing interest in the drug’s use for the pediatric patient with congenital heart disease during mechanical ventilation, for prevention of procedure-related
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anxiety, to treat delirium, withdrawal, and for shivering after general anesthesia [61]. A retrospective review of adult patients postcardiac surgery suggested lower early and 1-year mortality in patients receiving dexmedetomidine, as well as a lower incidence of delirium and overall complications [62].
Conclusions Patients with ACHD either have a simple defect detected at an older age, which may be complicated by the effects of volume and pressure load over years, or they have complex physiology with previous palliation and/or may have had multiple interventions. As a result, they require specialized care by a multidisciplinary team including cardiologists, cardiac surgeons, anesthesiologists, critical care physicians, and nurses with training and experience in congenital heart disease. Care of these patients often further requires the input of many other adult specialties, such as nephrologists, hepatologists, hematologists, neuropsychatrists, and the involvement of social workers. This is best accomplished in dedicated centers for ACHD that see a large number of patients annually. These ACHD centers may be based in adult or pediatric hospitals, and examples of successful programs exist in both settings. It is remarkable that despite the growing number of publications in the field of ACHD, there is still a paucity of evidence-based practice in critical care, postoperative management, and outcomes. This is, in part, a consequence of the varied presentations of congenital heart disease, and the relative low number of patients, even in large programs. Multi-institutional collaborations for research and a common database are greatly needed.
Acknowledgments The author would like to thank Tomas E. MacGillivray for comments on the manuscript.
Compliance with Ethics Guidelines Conflict of Interest Nathalie Roy declares that she has no conflict of interest. Human and Animal Rights and Informed Consent This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading Papers of particular interest, published recently, have been highlighted as: • Of importance 1.
Mackie AS, Pilote L, Ionescu-Ittu R, Rahme E, Marelli AJ. Health care resource utilization in adults with congenital heart disease. Am J Cardiol. 2007;99:839–43.
2.
van der Bom T, Bouma BJ, Meijboom FJ, Zwinderman AH, Mulder BJ. The prevalence of adult congenital heart disease, results from a systematic review and
3
Page 10 of 11
evidence based calculation. Am Heart J. 2012;164:568–75. 3. Putman LM, van Gameren M, Meijboom FJ, et al. Seventeen years of adult congenital heart surgery: a single centre experience. Eur J Cardiothorac Surg. 2009;36:96–104. 4. Khairy P, Ionescu-Ittu R, Mackie AS, Abrahamowicz M, Pilote L, Marelli AJ. Changing mortality in congenital heart disease. J Am Coll Cardiol. 2010;56:1149–57. 5. Kogon B, Grudziak J, Sahu A, et al. Surgery in adults with congenital heart disease: risk factors for morbidity and mortality. Ann Thorac Surg. 2013;95:1377–82. discussion 82. 6. Holst KA, Dearani JA, Burkhart HM, et al. Reoperative multivalve surgery in adult congenital heart disease. Ann Thorac Surg. 2013;95:1383–9. 7.• Price S, Jaggar SI, Jordan S, et al. Adult congenital heart disease: intensive care management and outcome prediction. Intensive Care Med. 2007;33:652–9. This publication looks at ICU management, resource utilization and predictors of mortality in critically ill ACHD patients. 8. Roche SL, Redington AN. The failing right ventricle in congenital heart disease. Can J Cardiol. 2013;29:768– 78. 9. VanderPluym C, Urschel S, Buchholz H. Advanced therapies for congenital heart disease: ventricular assist devices and heart transplantation. Can J Cardiol. 2013;29:796–802. 10. Mondesert B, Marcotte F, Mongeon FP, et al. Fontan circulation: success or failure? Can J Cardiol. 2013;29:811–20. 11. Escudero C, Khairy P, Sanatani S. Electrophysiologic considerations in congenital heart disease and their relationship to heart failure. Can J Cardiol. 2013;29:821–9. 12. Allan CK. Intensive care of the adult patient with congenital heart disease. Prog Cardiovasc Dis. 2011;53:274–80. 13.• Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation. 2007;115:534–45. This review addresses the major arrhythmias associated with ACHD, and the current management. 14.• Koyak Z, Achterbergh RC, de Groot JR, et al. Postoperative arrhythmias in adults with congenital heart disease: incidence and risk factors. Int J Cardiol. 2013;169:139–44. A multicenter study of the clinical impact of arrhythmias after surgery for congenital heart disease in adults. 15. Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122:868–75. 16. Koyak Z, Harris L, de Groot JR, et al. Sudden cardiac death in adult congenital heart disease. Circulation. 2012;126:1944–54. 17. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. The emerging burden of hospital admissions of adults with congenital heart disease. Heart. 2010;96:872–8.
Curr Treat Options Cardio Med (2015) 17:3 18. 19.
20.
21.
22.
23.
24.
25.
26. 27.
28.
29.
30.
31.
32.
Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation. 2008;117:363–70. Everitt MD, Donaldson AE, Stehlik J, et al. Would access to device therapies improve transplant outcomes for adults with congenital heart disease? Analysis of the United Network for Organ Sharing (UNOS). J Heart Lung Transplant. 2011;30:395–401. Connolly SJ, Cybulsky I, Lamy A, et al. Double-blind, placebo-controlled, randomized trial of prophylactic metoprolol for reduction of hospital length of stay after heart surgery: the beta-Blocker Length Of Stay (BLOS) study. Am Heart J. 2003;145:226–32. Cioccari L, Baur HR, Berger D, Wiegand J, Takala J, Merz TM. Hemodynamic assessment of critically ill patients using a miniaturized transesophageal echocardiography probe. Crit Care. 2013;17:R121. Schuuring MJ, van Gulik EC, Koolbergen DR, et al. Determinants of clinical right ventricular failure after congenital heart surgery in adults. J Cardiothorac Vasc Anesth. 2013;27:723–7. Booth KL, Roth SJ, Perry SB, del Nido PJ, Wessel DL, Laussen PC. Cardiac catheterization of patients supported by extracorporeal membrane oxygenation. J Am Coll Cardiol. 2002;40:1681–6. Joyce DL, Crow SS, John R, et al. Mechanical circulatory support in patients with heart failure secondary to transposition of the great arteries. J Heart Lung Transplant. 2010;29:1302–5. Shah NR, Lam WW, Rodriguez 3rd FH, et al. Clinical outcomes after ventricular assist device implantation in adults with complex congenital heart disease. J Heart Lung Transplant. 2013;32:615–20. Pick RA, Handler JB, Murata GH, Friedman AS. The cardiovascular effect of positive end-expiratory pressure. Chest. 1982;82:345–50. Gupta P, Kuperstock JE, Hashmi S, et al. Efficacy and predictors of success of noninvasive ventilation for prevention of extubation failure in critically ill children with heart disease. Pediatr Cardiol. 2013;34:964–77. Weismann CG, Yang SF, Bodian C, Hollinger I, Nguyen K, Mittnacht AJ. Early extubation in adults undergoing surgery for congenital heart disease. J Cardiothorac Vasc Anesth. 2012;26:773–6. Dimopoulos K, Diller GP, Koltsida E, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320–8. Inatomi J, Matsuoka K, Fujimaru R, Nakagawa A, Iijima K. Mechanisms of development and progression of cyanotic nephropathy. Pediatr Nephrol. 2006;21:1440–5. Elahi MM, Lim MY, Joseph RN, Dhannapuneni RR, Spyt TJ. Early hemofiltration improves survival in postcardiotomy patients with acute renal failure. Eur J Cardiothorac Surg. 2004;26:1027–31. van den Berghe G, Wouters P, Weekers F, et al. Intensive insulin therapy in critically ill patients. N Engl J Med. 2001;345:1359–67.
Curr Treat Options Cardio Med (2015) 17:3 33.
34. 35. 36. 37.
38. 39. 40. 41. 42. 43.
44.
45.
46.
47.
48.
Landoni G, Biondi-Zoccai GG, Marino G, et al. Fenoldopam reduces the need for renal replacement therapy and in-hospital death in cardiovascular surgery: a meta-analysis. J Cardiothorac Vasc Anesth. 2008;22:27–33. Asrani SK, Asrani NS, Freese DK, et al. Congenital heart disease and the liver. Hepatology. 2012;56:1160–9. Pike NA, Evangelista LS, Doering LV, Koniak-Griffin D, Lewis AB, Child JS. Clinical profile of the adolescent/ adult Fontan survivor. Congenit Heart Dis. 2011;6:9–17. Kendall TJ, Stedman B, Hacking N, et al. Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study. J Clin Pathol. 2008;61:504–8. Gundling F, Seidl H, Gansera L, et al. Early and late outcomes of cardiac operations in patients with cirrhosis: a retrospective survival-rate analysis of 47 patients over 8 years. Eur J Gastroenterol Hepatol. 2010;22:1466–73. Teh SH, Nagorney DM, Stevens SR, et al. Risk factors for mortality after surgery in patients with cirrhosis. Gastroenterology. 2007;132:1261–9. Assenza GE, Graham DA, Landzberg MJ, et al. MELD-XI score and cardiac mortality or transplantation in patients after Fontan surgery. Heart. 2013;99:491–6. Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, et al. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol. 2004;44:2065–72. Dalfino L, Sicolo A, Paparella D, Mongelli M, Rubino G, Brienza N. Intra-abdominal hypertension in cardiac surgery. Interact Cardiovasc Thorac Surg. 2013;17:644–51. Oechslin E. Hematological management of the cyanotic adult with congenital heart disease. Int J Cardiol. 2004;97 Suppl 1:109–15. Jensen AS, Johansson PI, Bochsen L, et al. Fibrinogen function is impaired in whole blood from patients with cyanotic congenital heart disease. Int J Cardiol. 2013;167:2210–4. Jensen AS, Johansson PI, Idorn L, et al. The haematocrit–an important factor causing impaired haemostasis in patients with cyanotic congenital heart disease. Int J Cardiol. 2013;167:1317–21. Odegard KC, McGowan Jr FX, Zurakowski D, et al. Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor VIII may predispose to thrombosis. J Thorac Cardiovasc Surg. 2003;125:1260–7. Monagle P, Chalmers E, Chan A, et al. Antithrombotic therapy in neonates and children: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest. 2008;133:887S–968. Ingels C, Debaveye Y, Milants I, et al. Strict blood glucose control with insulin during intensive care after cardiac surgery: impact on 4-year survival, dependency on medical care, and quality-of-life. Eur Heart J. 2006;27:2716–24. Bhamidipati CM, LaPar DJ, Stukenborg GJ, et al. Superiority of moderate control of hyperglycemia to tight control in patients undergoing coronary artery bypass grafting. J Thorac Cardiovasc Surg. 2011;141:543–51.
Page 11 of 11 3 49.
Landoni G, Augoustides JG, Guarracino F, et al. Mortality reduction in cardiac anesthesia and intensive care: results of the first International Consensus Conference. HSR Proc Intensiv Care Cardiovasc Anesth. 2011;3:9–19. 50. Van den Berghe G. Coronary bypass surgery: protective effects of insulin or of prevention of hyperglycemia, or both? J Clin Endocrinol Metab. 2011;96:1272–5. 51. Carvalho G, Pelletier P, Albacker T, et al. Cardioprotective effects of glucose and insulin administration while maintaining normoglycemia (GIN therapy) in patients undergoing coronary artery bypass grafting. J Clin Endocrinol Metab. 2011;96:1469–77. 52. Howell NJ, Ashrafian H, Drury NE, et al. Glucoseinsulin-potassium reduces the incidence of low cardiac output episodes after aortic valve replacement for aortic stenosis in patients with left ventricular hypertrophy: results from the Hypertrophy, Insulin, Glucose, and Electrolytes (HINGE) trial. Circulation. 2011;123:170–7. 53. Fan Y, Zhang AM, Xiao YB, Weng YG, Hetzer R. Glucose-insulin-potassium therapy in adult patients undergoing cardiac surgery: a meta-analysis. Eur J Cardiothorac Surg. 2011;40:192–9. 54. Agus MS, Steil GM, Wypij D, et al. Tight glycemic control versus standard care after pediatric cardiac surgery. N Engl J Med. 2012;367:1208–19. 55. Jacobi J, Bircher N, Krinsley J, et al. Guidelines for the use of an insulin infusion for the management of hyperglycemia in critically ill patients. Crit Care Med. 2012;40:3251–76. 56. Zomer AC, Vaartjes I, Uiterwaal CS, et al. Social burden and lifestyle in adults with congenital heart disease. Am J Cardiol. 2012;109:1657–63. 57. Moons P, Van Deyk K, Dedroog D, Troost E, Budts W. Prevalence of cardiovascular risk factors in adults with congenital heart disease. Eur J Cardiovasc Prev Rehabil. 2006;13:612–6. 58. Zaidi AN, Bauer JA, Michalsky MP, et al. The impact of obesity on early postoperative outcomes in adults with congenital heart disease. Congenit Heart Dis. 2011;6:241–6. 59.• Holst KA, Dearani JA, Burkhart HM, et al. Risk factors and early outcomes of multiple reoperations in adults with congenital heart disease. Ann Thorac Surg. 2011;92:122–8. A retrospective study that sought factors for cardiac injury and early death in ACHD patients undergoing repeat cardiac surgery. 60. Chorney SR, Gooch ME, Oberdier MT, Keating D, Stahl RF. The safety and efficacy of dexmedetomidine for postoperative sedation in the cardiac surgery intensive care unit. HSR Proc Intensiv Care Cardiovasc Anesth. 2013;5:17–24. 61. Tobias JD, Gupta P, Naguib A, Yates AR. Dexmedetomidine: applications for the pediatric patient with congenital heart disease. Pediatr Cardiol. 2011;32:1075–87. 62. Ji F, Li Z, Nguyen H, et al. Perioperative dexmedetomidine improves outcomes of cardiac surgery. Circulation. 2013;127:1576–84.
Adult Congenital Heart Disease (A Bhatt and K Niwa, Section Editors)
Critical Care Management of the Adult Patient with Congenital Heart Disease: Focus on Postoperative Management and Outcomes Nathalie Roy, MD, FRCSC Address Division of Cardiac Surgery, Massachusetts General Hospital, 55 Fruit St, Blake 872, Boston, MA 02114, USA Email: [email protected] 2 Harvard Medical School, Boston, MA, USA
* Springer Science+Business Media New York 2015
This article is part of the Topical Collection on Adult Congenital Heart Disease Keywords Adult congenital heart disease I Critical care I Cardiac surgery I Outcomes I Arrhythmia I Heart failure I Death I Acute kidney injury I Liver failure I Coagulopathy I Thrombosis I Obesity I Delirium
Opinion statement Advances in surgical techniques and in the medical management of children with congenital heart disease has increased survival into adulthood, resulting in a population of adults with congenital heart disease now surpassing the pediatric population in numbers. Furthermore, many of the patients will require repeat surgical, catheter-based, procedures and/or obstetrical care in their adult lives, and understanding the specific cardiopulmonary physiology and the involvement of other organ systems is critical to successful intervention. A team approach, with consultants from medical specialties in the setting of an established adult congenital heart center, is the optimal setting for superior outcomes. In this review, we discuss critical care management of the adult congenital heart disease patient in the perioperative period.
Introduction In recent decades, the number of patients with adult congenital heart disease (ACHD) has surpassed the number of children [1]. Indeed, approximately 85 % of patients born with congenital heart disease now
survive into adulthood, and the population with ACHD is now estimated at over 1 M in the United States and over 1.2 M in Europe [2]. Furthermore, the incidence of newly diagnosed conditions in
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adulthood is approximately equal to the number of patients needing a reoperation following palliation or corrective surgery [3]. The development of new surgical techniques for neonates with congenital heart disease (CHD), improvement in anesthesia and critical care, and catheter-based interventions all account for the improved survival of these patients [4]. However, many patients will need further surgical or catheter-based reintervention, or admission for heart failure or for noncardiac perioperative or peripartum management. In the recent years, certain centers have reviewed surgical outcomes in ACHD [5, 6]. Price et al [7•] looked at critical care outcomes of ACHD patients prospectively. They reported an overall mortality of 4.4 %. Mortality was 0 % in the patients with simple
lesions, but the mortality was 10.6 % in patients with lesions of moderate to severe complexity. In this study, the standard severity-of-illness scores were poorly predictive of perioperative mortality. Finally, the patients required high resource utilization in the intensive care unit (ICU) for diagnostics, as well as special therapies such as renal replacement therapy. There is growing interest in critical care management of these unique patients, as evidenced by a number of recent reviews [8–12]. However, there is a paucity of evidence-based practices due, in part to disease variability and small number of patients. The purpose of the present review is to address current trends in postoperative critical care management of adult patients with congenital heart disease, focusing on postoperative management and outcomes.
Critical care management of the adult patient with congenital heart disease Cardiac surgery for the patient with ACHD is performed for primary repair or reoperation [3]. Fortunately, patients with previously undiagnosed lesions amenable to primary repair in adulthood often fall in the noncomplex category. Conversely, patients requiring reoperations for ACHD often present with complex physiology, reduction in ventricular function and, as a result, chronic changes in many organ systems can complicate their management. A multidisciplinary approach to patient selection and to perioperative management should include the cardiac surgeon, adult congenital cardiologist, anesthesiologist and intensivist, along with specialist consultants from renal, hematology, hepatology, and neuropsychiatry.
Cardiovascular system Arrhythmias Arrhythmias are very prevalent in adult patients with congenital heart defects [13•], and are a frequent occurrence after cardiac surgery for adult congenital disease [14•]. Certain patients have an anatomical substrate predisposing them to arrhythmias (eg, heterotaxy, CCTGA). In most cases, it is the result of previous intervention, palliation, or longstanding pathophysiology such as atrioventricular valve regurgitation, baffle obstruction, atrial and ventricular dilatation, myocardial hypertrophy, and heart failure [11]. It is estimated that up to one-third of patients with ACHD have supraventricular arrhythmias [15, 16], leading to major morbidity and sudden death [17]. Nevertheless, the leading cause of sudden death in ACHD patients is ventricular arrhythmias, often the result of ventricular dysfunction [16]. Ventricular arrhythmias have been reported most commonly with systemic right ventricles (such as in transposition of the great arteries (D-TGA) with atrial-level
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repair or corrected transposition (L-TGA)), Eisenmenger syndrome, tetralogy of Fallot, and in patients with severe left-sided ventricular obstruction. Khairy et al [18] described a risk score for sudden death in patients with tetralogy of Fallot in order to determine guidelines for preventive implantable cardioverterdefibrillator (ICD) implantation in this population, but data lacks in other physiologies. ICDs should be considered in the setting of sustained ventricular tachycardia or cardiac arrest, but even the use of established heart failure guidelines for ICD [19] are often not observed in the ACHD patient population because of lack of evidence. Furthermore, anatomical and technical issues, such as baffles, presence of right-sided artificial valves, right-to-left shunts, and history of (or the risk of thrombosis associated with) intravascular leads, complicate the insertion of ICDs. Koyak et al [14•] reviewed the incidence of postoperative arrhythmia in ACHD. The multicenter retrospective study found an incidence of postoperative arrhythmia of 32 %, the majority occurring in the first 3 days after surgery. Twenty-four percent (24 %) of patients developed supraventricular arrhythmias, 11 % experienced bradyarrhythmias, and ventricular tachycardia was seen in 5 % of the patients. Arrhythmias were more frequent with intracardiac repair, vs surgical repair involving the aortic root. All types of arrhythmias were hemodynamically significant, resulting in a twofold increase in ICU stay, increased likelihood of pacemaker implantation (5 % of the patients) especially in the patients experiencing bradyarrhythmias, and increase in adverse events, including heart failure and death. In a multivariate analysis, significant risk factors for postoperative arrhythmia were older age, New York Heart Association (NYHA) class, moderate or more atrioventricular valve regurgitation of the subpulmonary ventricle, cardiopulmonary bypass (CPB) time, and increased level of the cardiac enzyme (CK-MB) postoperatively. Supraventricular arrhythmias were treated most frequently with beta-blockers and amiodarone, and approximately one-third of the patients required long-term antiarrhythmic therapy. Conversely to surgery for acquired disease [20], there are no data on the prophylactic use of beta-blockers to prevent the incidence of postoperative arrhythmias. The reluctance in adopting a prophylactic use of these agents may be related to their negative inotropic effect in a patient population with depressed ventricular function at baseline. Patients presenting for surgery with an ICD device should have the defibrillation function turned off at the time of induction. Furthermore, it is important to recognize that leads may be displaced, and intraoperative interrogation at the end of the procedure is critical. The defibrillation function should be turned back on before the patient transfers from the intensive care unit to the stepdown unit.
Ventricular function, heart failure, and support Patients with ACHD often have a decline in ventricular function and may be admitted to the ICU as a result of heart failure for tailored assessment of their physiology and medical management of heart failure. The use of preoperative and intraoperative echo is critical to evaluate the repair, but also to determine the presence of residual shunts, gradients, and valvular regurgitation, as well as the ventricular function. Central and intracardiac pressure monitoring is useful to determine right and left ventricular preload, pulmonary pressures, and allow
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mixed venous saturation measurement. Certain patients have anatomical reasons that make the use of flow-directed PA catheters difficult, and transcutaneous left atrial line monitoring may be helpful in postoperative management. Continuous transesophageal echocardiography monitoring with the use of disposable miniaturized probes may be helpful in assessing preload, ventricular function, and response to interventions in the critically ill patient [21]. Factors associated with postoperative adverse events in ACHD patients [5] are increased NYHA class and longer CBP times. Holst et al reviewed their experience in patients undergoing multivalve operations for ACHD [6], and found that the risk factors for early mortality included nonelective surgery, increased CPB and ischemic times, lower hematocrit, increased creatinine, and mechanical ventilation greater than 5 days. The development of right ventricular failure is associated with high mortality in the postoperative period. In the study by Schuuring et al [22], the postoperative incidence of right ventricular failure was 4.4 %. Patients with repairs involving the systemic circulation and the subaortic ventricle, and repairs involving both ventricles, have a greater risk than patients having surgery involving the subpulmonary ventricle alone. Other risk factors of postoperative right ventricular failure are increased NYHA class (≥2), preoperative supraventricular arrhythmia, longer CPB times (9150 m) and decreased creatinine clearance. The challenge is prompt recognition and providing adequate support. Right ventricular failure manifests by an increase in jugular venous pressure, associated with systemic hypotension, renal and hepatic dysfunction, and ascites. If left untreated, it can lead to gut ischemia from low cardiac output, superimposed with increased venous pressure. The management includes inotropic support of the right ventricle with phosphodiesterase inhibitors and adrenaline, aggressive management of pulmonary hypertension with inhaled agents (NO, inhaled prostacyclin), as well as systemic support of the blood pressure. Fluid removal with diuresis or early continuous venovenous hemofiltration (CVVH) is important to prevent end-organ damage. Temporary right-sided mechanical support can be performed using a Centrimag pump (Thoratec Corporation, Pleasantville, CA).
Mechanical circulatory support Published reports of extracorporeal membrane oxygenation (ECMO) for congenital heart disease include mostly pediatric patients, and few publications include adult patients. ECMO in children is mostly used as a salvage therapy postcardiotomy, or for end stage heart failure, or periprocedure in the setting of high-risk catheter-based intervention [23]. In ACHD, the existence of multiorgan dysfunction often leads to renal and hepatic failure, neurologic injury and bleeding complications in the setting of ECMO, and contributes to poor results in this population. Furthermore, vascular access is often compromized and limits the success of this therapy. Everitt et al [19] reviewed the United Network for Organ Sharing database for ACHD patients listed for heart transplantation from 2005–2009. They identified only five patients supported on ECMO out of 314 patients with ACHD. Furthermore, only 16 patients were supported by a ventricular assist device, emphasizing the underutilization of this therapy in ACHD. In recent
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years, a few case reports of ventricular assist device (VAD) implantation in the setting of ACHD were published, including a three-case series [24] of patients with D-TGA and L-TGA. The paper focused on implantation technique in patients with systemic right ventricles. A publication by Shah et al [25] reported six (6) ACHD patients supported on a VAD: they reported one early (G30 days) and one late mortality (262 days), two patients supported on DT-VADs for over 1 year, and two patients successfully transplanted. One of the patients was supported for 988 days prior to receiving his organ. The perioperative morbidity was significant. Adults with complex congenital heart disease experience earlier deaths [1], mostly because of heart failure and multi end-organ dysfunction, as a result of longstanding cardiac pathophysiology. Unfortunately, despite a growing patient population, mechanical circulatory support and heart transplantation remains fairly uncommon in ACHD, with longer times awaiting organ allocation [19]. This is related, in part, to prior sensitization [9], and to secondary organ dysfunction and the need for dual organ transplantation.
Respiratory system Cardiopulmonary interactions and mechanical ventilation Cardiopulmonary interactions are important in the patient with complex congenital heart disease [26]. These patients are likely to have impaired right ventricular function, and the negative impact of positive pressure ventilation and positive end-expiratory pressure (PEEP) can lead to decreased left ventricular filling and low cardiac output. The goal should be early extubation, especially in patients with good hemodynamics and low inotrope scores. However, in the setting of marginal left ventricular function, PEEP decreases left ventricular wall stress, and premature extubation in the setting of fluid overload can lead to respiratory failure. Extubation to noninvasive positive pressure ventilation may be beneficial [27], especially in patients with a history of sleep apnea. The use of low dose dexmedetomidine may be helpful to reduce the need for narcotic and anxiolytics. Weissmann et al [28] looked at the practice of extubating ACHD patients in the operating room. They found that low Risk Adjusted classification for Congenital Heart Surgery score, and low body mass index in the setting of increased disease complexity correlated with early extubation. For patients who remain sedated for hemodynamic reasons, keeping the mean airway pressure low and avoiding pulmonary vasoconstriction is important. Thus, targeting a normal acid-base balance and PCO2, and preventing hypoxemia.
Acute kidney injury and fluid control Fifty percent (50 %) of patients with congenital heart disease have some degree of chronic kidney disease [29], and 9 % have moderate to severe reductions in glomerular filtration rate (GFR). Contributing factors include previous exposure to cardiopulmonary bypass, cyanosis [30], and the effects of years of pathologic circulation with marginal systemic cardiac output and elevated venous pressures. There is a three-fold increase in mortality at baseline in the ACHD
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Curr Treat Options Cardio Med (2015) 17:3 patients with moderate and severe renal dysfunction [29]. Furthermore, there is increased perioperative mortality in ACHD patients with renal disease [7]. The goal of perioperative management of ACHD patients with renal dysfunction undergoing cardiac surgery is to maintain an adequate organ perfusion pressure (ie, MAP – CVP or intra-abdominal pressure), and cardiac output. Early postoperative diuresis is important in order to optimize right ventricular preload in the setting of right ventricular dysfunction. Early fluid removal with continuous veno-veno hemofiltration (CVVH) can be helpful in managing fluid overload and increased right-sided venous pressure. It should be initiated promptly in the setting of oliguria and decreasing GFR. A retrospective study [31] looking at the timing for initiation of CVVH in acute renal failure postcardiotomy suggested that early CVVH (G1 day) conferred a survival advantage over late initiation of CVVH. A clinical trial evaluating this strategy is currently underway. In addition, glucose control has been associated with a reduction in the need for renal replacement therapy [32]. A meta-analysis looking at the use of fenoldopam after cardiac surgery also found benefits in preventing renal replacement therapy [33], but this strategy is not widely adopted. Many clinical trials are currently underway to evaluate different renoprotective drugs in the setting of cardiac surgery (clinicaltrial.gov). However, there is no published data in ACHD, and this population is often specifically excluded from clinical trials.
GI system Hepatic dysfunction Hepatic dysfunction of varying degrees is very common in ACHD patients [34]. It often results from chronic venous congestion associated with single ventricular repair and Fontan physiology, or in the context of right ventricular dysfunction. Other contributors to hepatic dysfunction include marginal systemic cardiac output, steel from systemic-to-pulmonary shunts, and the added hepatic insults from cardiopulmonary bypass, multiple transfusions with their inherent infectious risks, and hepatotoxic drugs. Over time, cardiac cirrhosis may develop, resulting in portal hypertension with ascites and porto-systemic varices. The metabolic anomalies associated with passive congestion in the liver are often increased bilirubin and prolonged Prothrombine Time (PT/INR). Low cardiac output and liver ischemia tend to cause an elevation in transaminases, but low transaminase levels are also possible as a result of chronic ischemia and fibrosis. Acute liver dysfunction is likely to be associated with encephalopathy and jaundice. The patient most at risk for liver dysfunction in the CHD population is the adult with a failing Fontan physiology. The risk of developing hepatic complications increases over time, and cirrhosis is seen as early as 10 years after Fontan completion [35]. On histology, there is sinusoidal fibrosis in the space of Disse and sinusoidal dilatation from zone 3 to the portal tract [36]. These patients present with ascites, hyperbilirubinemia, and coagulopathy, and can also present with bleeding varices. Minimizing pulmonary pressures by avoidance of intubation and by drainage of pleural effusions and ascites is essential. Optimizing cardiac output may require inotropes, reduction of venous pressure with aggressive diuresis or CVVH, and drainage of ascites. Management also involves nutritional support, correction of coagulopathy, and monitoring for hepatic encephalopathy.
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Liver dysfunction has a significant impact on perioperative mortality [7•]. Indeed, nonliver transplantation surgery in patients with cirrhosis is associated with significant mortality. In patients undergoing adult cardiac surgery, liver disease greater than Childs-A is associated with high morbidity and mortality [37]. The Model for End-Stage Liver Disease score is predictive of outcomes after cardiac surgery [38] and was recently found to be predictive of sudden cardiac death, death from congestive heart failure, and death after cardiac transplantation in the Fontan population [39]. In regards to heart transplantation, it remains unknown whether early heart transplantation may avoid the need for liver transplantation in the setting of congenital heart disease and liver dysfunction. Isolated heart transplantation for failing Fontan has significantly lower 1- and 5-yeat survival; 63 % and 57 %, respectively [40]. Early deaths were related to bleeding, sepsis and multiorgan failure. Regarding dual organ transplantation, cardiac cirrhosis represents only 11 % of the combined heart-liver transplants, with congenital heart disease representing only 1 % (13 % of patients with cardiac cirrhosis). The most common diagnosis for heart-liver transplantation is amyloidosis.
Abdominal compartment syndrome In the setting of right ventricular dysfunction and low postoperative cardiac output, ascites may develop, causing an increase in intra-abdominal pressure and abdominal compartment syndrome [41]. Abdominal compartment syndrome is associated with acute kidney injury, gut ischemia, acute lung injury from high airway pressures, and circulatory collapse. The diagnosis is confirmed by measuring the bladder pressure (920 Torr) and requires an emergent laparotomy for relief of abdominal pressure.
Hematologic considerations Patients with ACHD are at risk of bleeding and thrombosis [42]. From a technical perspective, re-entry into the chest after multiple surgeries can lead to significant blood loss, especially because many patients have chest wall and mediastinal collaterals. Recent evidence by Jensen et al suggests that cyanotic patients have impaired hemostasis [43, 44]. Patients with Fontan physiology have lower levels of procoagulant and anticoagulant factors [45], and the clinical significance is uncertain. Another consideration is the presence of foreign material, especially in the venous circulation where stenosis and stasis promote thrombosis. Venous thromboembolism prophylaxis should be considered in children with congenital heart disease postoperatively [46]. Although there is no consensus in ACHD, venous thromboembolism prophylaxis is suggested postoperatively for the reasons aforementioned. Management of the bleeding and thrombotic risks is complex in this population, and because of the paucity of evidence, it should be individualized [42].
Endocrine system Glucose control Glucose control can significantly improve morbidity and mortality after cardiac surgery in the adult patient. The landmark trial by Van den Berghe in 2001 [32]
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Curr Treat Options Cardio Med (2015) 17:3 showed a reduction in mortality, and morbidity in patients treated with tight glucose control. The findings demonstrated a reduced renal replacement therapy and lower risk for hyperbilirubinemia, earlier wean from mechanical ventilation, and earlier discharge from the hospital and the ICU. A subanalysis of the high-risk cardiac surgical patients in this study [47] showed maintenance of the survival benefit seen with intensive insulin therapy at 4 years after discharge. However, controversy still persists regarding how tight glucose control should be, with one study suggesting that moderate is superior to tight control [48]. At the first international consensus conference on mortality reduction in cardiac surgical anesthesia and critical care [49], perioperative insulin therapy during cardiac surgery was highly ranked as a possible lifesaving ancillary technique in cardiac anesthesia and intensive care. In fact, the benefits of insulin administration in cardiac surgery appear to extend beyond the avoidance of hyperglycemia. Normoglycemia achieved through administration of glucose, insulin, and potassium (GIK) appears cardioprotective [50–53]. The caveat is whether these findings can be extrapolated to the ACHD patient population, who are typically younger and free of significant coronary artery disease. A recent randomized trial in pediatric cardiac surgical patients [54] found that tight glycemic control did not significantly impact the infection rate, mortality, length of stay, or measures of organ failure, compared with standard care. Hypoglycemia infrequent (3 %) in this population and was not associated with adverse events. The current guidelines for the use of insulin for glucose management in critically ill patients [55] suggest that patients undergoing cardiac surgery should maintain moderate glucose control using a reliable insulin infusion protocol and a target blood glucose of less than 150 mg/dL.
Obesity As opposed to children with congenital heart disease, who tend to have failure to thrive and malnutrition, the prevalence of obesity in ACHD patients ranges from 10 %–30 % [56, 57]. This may be partly related to sedentary lifestyle. In two recent studies [58, 59•], obesity was not associated with worse surgical outcomes in patients with ACHD.
Thyroid function In the study by Price et al [7•], preoperative abnormal thyroid function was predictive of mortality postcardiac surgery and in patients admitted to the ICU for medical or surgical complications. A thyroid-stimulating hormone (TSH) screening is recommended.
Sedation and delirium prevention Two-thirds of patients with ACHD have been subject to previous surgery and anesthesia, and tolerance to analgesics and sedatives in not uncommon. No specific data exist in this population, but newer agents such as dexmedetomidine appear to be safe in adults [60] and children [61]. Furthermore, dexmedetomidine has favorable sedative and anxiolytic properties and limited effects on hemodynamic and respiratory function. As a result, there is growing interest in the drug’s use for the pediatric patient with congenital heart disease during mechanical ventilation, for prevention of procedure-related
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anxiety, to treat delirium, withdrawal, and for shivering after general anesthesia [61]. A retrospective review of adult patients postcardiac surgery suggested lower early and 1-year mortality in patients receiving dexmedetomidine, as well as a lower incidence of delirium and overall complications [62].
Conclusions Patients with ACHD either have a simple defect detected at an older age, which may be complicated by the effects of volume and pressure load over years, or they have complex physiology with previous palliation and/or may have had multiple interventions. As a result, they require specialized care by a multidisciplinary team including cardiologists, cardiac surgeons, anesthesiologists, critical care physicians, and nurses with training and experience in congenital heart disease. Care of these patients often further requires the input of many other adult specialties, such as nephrologists, hepatologists, hematologists, neuropsychatrists, and the involvement of social workers. This is best accomplished in dedicated centers for ACHD that see a large number of patients annually. These ACHD centers may be based in adult or pediatric hospitals, and examples of successful programs exist in both settings. It is remarkable that despite the growing number of publications in the field of ACHD, there is still a paucity of evidence-based practice in critical care, postoperative management, and outcomes. This is, in part, a consequence of the varied presentations of congenital heart disease, and the relative low number of patients, even in large programs. Multi-institutional collaborations for research and a common database are greatly needed.
Acknowledgments The author would like to thank Tomas E. MacGillivray for comments on the manuscript.
Compliance with Ethics Guidelines Conflict of Interest Nathalie Roy declares that she has no conflict of interest. Human and Animal Rights and Informed Consent This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading Papers of particular interest, published recently, have been highlighted as: • Of importance 1.
Mackie AS, Pilote L, Ionescu-Ittu R, Rahme E, Marelli AJ. Health care resource utilization in adults with congenital heart disease. Am J Cardiol. 2007;99:839–43.
2.
van der Bom T, Bouma BJ, Meijboom FJ, Zwinderman AH, Mulder BJ. The prevalence of adult congenital heart disease, results from a systematic review and
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evidence based calculation. Am Heart J. 2012;164:568–75. 3. Putman LM, van Gameren M, Meijboom FJ, et al. Seventeen years of adult congenital heart surgery: a single centre experience. Eur J Cardiothorac Surg. 2009;36:96–104. 4. Khairy P, Ionescu-Ittu R, Mackie AS, Abrahamowicz M, Pilote L, Marelli AJ. Changing mortality in congenital heart disease. J Am Coll Cardiol. 2010;56:1149–57. 5. Kogon B, Grudziak J, Sahu A, et al. Surgery in adults with congenital heart disease: risk factors for morbidity and mortality. Ann Thorac Surg. 2013;95:1377–82. discussion 82. 6. Holst KA, Dearani JA, Burkhart HM, et al. Reoperative multivalve surgery in adult congenital heart disease. Ann Thorac Surg. 2013;95:1383–9. 7.• Price S, Jaggar SI, Jordan S, et al. Adult congenital heart disease: intensive care management and outcome prediction. Intensive Care Med. 2007;33:652–9. This publication looks at ICU management, resource utilization and predictors of mortality in critically ill ACHD patients. 8. Roche SL, Redington AN. The failing right ventricle in congenital heart disease. Can J Cardiol. 2013;29:768– 78. 9. VanderPluym C, Urschel S, Buchholz H. Advanced therapies for congenital heart disease: ventricular assist devices and heart transplantation. Can J Cardiol. 2013;29:796–802. 10. Mondesert B, Marcotte F, Mongeon FP, et al. Fontan circulation: success or failure? Can J Cardiol. 2013;29:811–20. 11. Escudero C, Khairy P, Sanatani S. Electrophysiologic considerations in congenital heart disease and their relationship to heart failure. Can J Cardiol. 2013;29:821–9. 12. Allan CK. Intensive care of the adult patient with congenital heart disease. Prog Cardiovasc Dis. 2011;53:274–80. 13.• Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation. 2007;115:534–45. This review addresses the major arrhythmias associated with ACHD, and the current management. 14.• Koyak Z, Achterbergh RC, de Groot JR, et al. Postoperative arrhythmias in adults with congenital heart disease: incidence and risk factors. Int J Cardiol. 2013;169:139–44. A multicenter study of the clinical impact of arrhythmias after surgery for congenital heart disease in adults. 15. Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122:868–75. 16. Koyak Z, Harris L, de Groot JR, et al. Sudden cardiac death in adult congenital heart disease. Circulation. 2012;126:1944–54. 17. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. The emerging burden of hospital admissions of adults with congenital heart disease. Heart. 2010;96:872–8.
Curr Treat Options Cardio Med (2015) 17:3 18. 19.
20.
21.
22.
23.
24.
25.
26. 27.
28.
29.
30.
31.
32.
Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation. 2008;117:363–70. Everitt MD, Donaldson AE, Stehlik J, et al. Would access to device therapies improve transplant outcomes for adults with congenital heart disease? Analysis of the United Network for Organ Sharing (UNOS). J Heart Lung Transplant. 2011;30:395–401. Connolly SJ, Cybulsky I, Lamy A, et al. Double-blind, placebo-controlled, randomized trial of prophylactic metoprolol for reduction of hospital length of stay after heart surgery: the beta-Blocker Length Of Stay (BLOS) study. Am Heart J. 2003;145:226–32. Cioccari L, Baur HR, Berger D, Wiegand J, Takala J, Merz TM. Hemodynamic assessment of critically ill patients using a miniaturized transesophageal echocardiography probe. Crit Care. 2013;17:R121. Schuuring MJ, van Gulik EC, Koolbergen DR, et al. Determinants of clinical right ventricular failure after congenital heart surgery in adults. J Cardiothorac Vasc Anesth. 2013;27:723–7. Booth KL, Roth SJ, Perry SB, del Nido PJ, Wessel DL, Laussen PC. Cardiac catheterization of patients supported by extracorporeal membrane oxygenation. J Am Coll Cardiol. 2002;40:1681–6. Joyce DL, Crow SS, John R, et al. Mechanical circulatory support in patients with heart failure secondary to transposition of the great arteries. J Heart Lung Transplant. 2010;29:1302–5. Shah NR, Lam WW, Rodriguez 3rd FH, et al. Clinical outcomes after ventricular assist device implantation in adults with complex congenital heart disease. J Heart Lung Transplant. 2013;32:615–20. Pick RA, Handler JB, Murata GH, Friedman AS. The cardiovascular effect of positive end-expiratory pressure. Chest. 1982;82:345–50. Gupta P, Kuperstock JE, Hashmi S, et al. Efficacy and predictors of success of noninvasive ventilation for prevention of extubation failure in critically ill children with heart disease. Pediatr Cardiol. 2013;34:964–77. Weismann CG, Yang SF, Bodian C, Hollinger I, Nguyen K, Mittnacht AJ. Early extubation in adults undergoing surgery for congenital heart disease. J Cardiothorac Vasc Anesth. 2012;26:773–6. Dimopoulos K, Diller GP, Koltsida E, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320–8. Inatomi J, Matsuoka K, Fujimaru R, Nakagawa A, Iijima K. Mechanisms of development and progression of cyanotic nephropathy. Pediatr Nephrol. 2006;21:1440–5. Elahi MM, Lim MY, Joseph RN, Dhannapuneni RR, Spyt TJ. Early hemofiltration improves survival in postcardiotomy patients with acute renal failure. Eur J Cardiothorac Surg. 2004;26:1027–31. van den Berghe G, Wouters P, Weekers F, et al. Intensive insulin therapy in critically ill patients. N Engl J Med. 2001;345:1359–67.
Curr Treat Options Cardio Med (2015) 17:3 33.
34. 35. 36. 37.
38. 39. 40. 41. 42. 43.
44.
45.
46.
47.
48.
Landoni G, Biondi-Zoccai GG, Marino G, et al. Fenoldopam reduces the need for renal replacement therapy and in-hospital death in cardiovascular surgery: a meta-analysis. J Cardiothorac Vasc Anesth. 2008;22:27–33. Asrani SK, Asrani NS, Freese DK, et al. Congenital heart disease and the liver. Hepatology. 2012;56:1160–9. Pike NA, Evangelista LS, Doering LV, Koniak-Griffin D, Lewis AB, Child JS. Clinical profile of the adolescent/ adult Fontan survivor. Congenit Heart Dis. 2011;6:9–17. Kendall TJ, Stedman B, Hacking N, et al. Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study. J Clin Pathol. 2008;61:504–8. Gundling F, Seidl H, Gansera L, et al. Early and late outcomes of cardiac operations in patients with cirrhosis: a retrospective survival-rate analysis of 47 patients over 8 years. Eur J Gastroenterol Hepatol. 2010;22:1466–73. Teh SH, Nagorney DM, Stevens SR, et al. Risk factors for mortality after surgery in patients with cirrhosis. Gastroenterology. 2007;132:1261–9. Assenza GE, Graham DA, Landzberg MJ, et al. MELD-XI score and cardiac mortality or transplantation in patients after Fontan surgery. Heart. 2013;99:491–6. Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, et al. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol. 2004;44:2065–72. Dalfino L, Sicolo A, Paparella D, Mongelli M, Rubino G, Brienza N. Intra-abdominal hypertension in cardiac surgery. Interact Cardiovasc Thorac Surg. 2013;17:644–51. Oechslin E. Hematological management of the cyanotic adult with congenital heart disease. Int J Cardiol. 2004;97 Suppl 1:109–15. Jensen AS, Johansson PI, Bochsen L, et al. Fibrinogen function is impaired in whole blood from patients with cyanotic congenital heart disease. Int J Cardiol. 2013;167:2210–4. Jensen AS, Johansson PI, Idorn L, et al. The haematocrit–an important factor causing impaired haemostasis in patients with cyanotic congenital heart disease. Int J Cardiol. 2013;167:1317–21. Odegard KC, McGowan Jr FX, Zurakowski D, et al. Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor VIII may predispose to thrombosis. J Thorac Cardiovasc Surg. 2003;125:1260–7. Monagle P, Chalmers E, Chan A, et al. Antithrombotic therapy in neonates and children: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest. 2008;133:887S–968. Ingels C, Debaveye Y, Milants I, et al. Strict blood glucose control with insulin during intensive care after cardiac surgery: impact on 4-year survival, dependency on medical care, and quality-of-life. Eur Heart J. 2006;27:2716–24. Bhamidipati CM, LaPar DJ, Stukenborg GJ, et al. Superiority of moderate control of hyperglycemia to tight control in patients undergoing coronary artery bypass grafting. J Thorac Cardiovasc Surg. 2011;141:543–51.
Page 11 of 11 3 49.
Landoni G, Augoustides JG, Guarracino F, et al. Mortality reduction in cardiac anesthesia and intensive care: results of the first International Consensus Conference. HSR Proc Intensiv Care Cardiovasc Anesth. 2011;3:9–19. 50. Van den Berghe G. Coronary bypass surgery: protective effects of insulin or of prevention of hyperglycemia, or both? J Clin Endocrinol Metab. 2011;96:1272–5. 51. Carvalho G, Pelletier P, Albacker T, et al. Cardioprotective effects of glucose and insulin administration while maintaining normoglycemia (GIN therapy) in patients undergoing coronary artery bypass grafting. J Clin Endocrinol Metab. 2011;96:1469–77. 52. Howell NJ, Ashrafian H, Drury NE, et al. Glucoseinsulin-potassium reduces the incidence of low cardiac output episodes after aortic valve replacement for aortic stenosis in patients with left ventricular hypertrophy: results from the Hypertrophy, Insulin, Glucose, and Electrolytes (HINGE) trial. Circulation. 2011;123:170–7. 53. Fan Y, Zhang AM, Xiao YB, Weng YG, Hetzer R. Glucose-insulin-potassium therapy in adult patients undergoing cardiac surgery: a meta-analysis. Eur J Cardiothorac Surg. 2011;40:192–9. 54. Agus MS, Steil GM, Wypij D, et al. Tight glycemic control versus standard care after pediatric cardiac surgery. N Engl J Med. 2012;367:1208–19. 55. Jacobi J, Bircher N, Krinsley J, et al. Guidelines for the use of an insulin infusion for the management of hyperglycemia in critically ill patients. Crit Care Med. 2012;40:3251–76. 56. Zomer AC, Vaartjes I, Uiterwaal CS, et al. Social burden and lifestyle in adults with congenital heart disease. Am J Cardiol. 2012;109:1657–63. 57. Moons P, Van Deyk K, Dedroog D, Troost E, Budts W. Prevalence of cardiovascular risk factors in adults with congenital heart disease. Eur J Cardiovasc Prev Rehabil. 2006;13:612–6. 58. Zaidi AN, Bauer JA, Michalsky MP, et al. The impact of obesity on early postoperative outcomes in adults with congenital heart disease. Congenit Heart Dis. 2011;6:241–6. 59.• Holst KA, Dearani JA, Burkhart HM, et al. Risk factors and early outcomes of multiple reoperations in adults with congenital heart disease. Ann Thorac Surg. 2011;92:122–8. A retrospective study that sought factors for cardiac injury and early death in ACHD patients undergoing repeat cardiac surgery. 60. Chorney SR, Gooch ME, Oberdier MT, Keating D, Stahl RF. The safety and efficacy of dexmedetomidine for postoperative sedation in the cardiac surgery intensive care unit. HSR Proc Intensiv Care Cardiovasc Anesth. 2013;5:17–24. 61. Tobias JD, Gupta P, Naguib A, Yates AR. Dexmedetomidine: applications for the pediatric patient with congenital heart disease. Pediatr Cardiol. 2011;32:1075–87. 62. Ji F, Li Z, Nguyen H, et al. Perioperative dexmedetomidine improves outcomes of cardiac surgery. Circulation. 2013;127:1576–84.
