408 SECTION IV Pediatric Critical Care Cardiovascular After a Norwood operation, a pH of 7 4, Paco2 of 40 mm Hg, and Pao2 of 40 mm Hg in room air, with a mixed venous oxygen saturation of 60%, connote[.]
408 S E C T I O N I V Pediatric Critical Care: Cardiovascular After a Norwood operation, a pH of 7.4, Paco2 of 40 mm Hg, and Pao2 of 40 mm Hg in room air, with a mixed venous oxygen saturation of 60%, connote a well-balanced circulation Higher saturations can be achieved if the systemic circulation is well dilated without compromising perfusion pressure Frequent adjustments in mechanical ventilation settings and Fio2 may be necessary in the first few hours after surgery However, manipulations of Fio2 in the face of a restrictive 3.5-mm shunt may have less impact on pulmonary blood flow than systemic vasodilation.214 Leaving the sternum open after a Norwood operation may facilitate lower filling pressures, balanced circulation, and a stable ventilation pattern Deep sedation or even muscle paralysis and anesthesia often are continued after surgery to minimize the stress response until the patient has stable circulation and gas exchange Inotropic support with dopamine or epinephrine usually is required, titrated to systemic pressure and perfusion Afterload reduction with milrinone as a second-line agent is beneficial to reduce myocardial work and improve systemic perfusion Monitoring SVC oxygen saturations as a measure of Svo2 and cardiac output is useful in this assessment Volume replacement to maintain preload is essential, aiming for a common atrial pressure approximating to 10 mm Hg The type, diameter, length, and position of the shunt affect the balance of pulmonary and systemic flow Generally, a 3.5-mm modified BT shunt from the distal innominate artery provides adequate pulmonary blood flow without excessive steal from the systemic circulation for most full-term neonates Nevertheless, a shunt resulting in a low diastolic pressure (,30 mm Hg) in turn affects perfusion to other vascular beds, particularly the coronary, cerebral, renal, and splanchnic perfusion This may contribute to a prolonged and difficult postoperative course Overcirculation in the immediate postoperative period with an Sao2 greater than 90% may reflect a low PVR or increased flow across the shunt if the shunt size is too large or the perfusion pressure is increased from residual aortic arch obstruction distal to the shunt insertion site The increased volume load on the systemic ventricle results in CHF and progressive systemic hypoperfusion with cool extremities, oliguria, and metabolic acidosis Although manipulation of mechanical ventilation and inspired oxygen concentration may help limit pulmonary blood flow, surgical revision to reduce the shunt size may be necessary If there is significant diastolic runoff through a large shunt, coronary perfusion will be reduced and lead to ischemia, low output, arrhythmias, and cardiac arrest Rhythm disturbances are uncommon in the immediate postoperative period following a Norwood operation, and a sudden loss of sinus rhythm, particularly heart block or ventricular fibrillation, should increase the suspicion of myocardial ischemia and impending circulatory collapse In the immediate postoperative period, mild hypoxemia with an Sao2 of 70% to 75% and Pao2 of 30 to 35 mm Hg is preferable to an overcirculated state with high systemic oxygen saturations and falling mixed venous oxygen saturation Pulmonary blood flow often increases on the first postoperative day as ventricular function improves and PVR falls during recovery from CPB Pulmonary venous desaturation from parenchymal lung diseases—such as atelectasis, pleural effusions, and pneumothorax—requires aggressive management Persistent desaturation and hypotension reflect low cardiac output from poor ventricular function, thereby decreasing the perfusion pressure across the shunt Svo2 is low (often ,40%), and treatment is directed first at augmenting contractility with inotropic agents and subsequently reducing afterload with a vasodilator This is a serious clinical problem with high mortality after a Norwood operation The related myocardial ischemia and acidosis further impair myocardial function and systemic perfusion, leading to circulatory collapse Atrioventricular valve regurgitation and residual aortic arch obstruction are important causes of persistent low cardiac output and inability to wean from mechanical ventilation Echocardiography is useful for assessing valve and ventricular function but is less accurate for assessing the degree of residual arch obstruction Cardiac catheterization is sometimes necessary and will enable fine-tuning of hemodynamic support or balloon dilation of a hypoplastic segment of narrowed aorta Occasionally, surgical revision of the aortic arch or atrioventricular valve is necessary, although this is seen more commonly in the interval before the bidirectional cavopulmonary shunt The advocated Sano modification of the Norwood procedure involves placement of a conduit from the RV to the PA confluence (RV-PA shunt).220,221 The primary advantage of this procedure in the immediate postoperative period is improved diastolic perfusion without runoff across an aortopulmonary shunt Ventricular function is less likely to be compromised after surgery because the volume load to the ventricle is reduced from a lower Qp/Qs, along with a reduced risk for myocardial ischemia because of improved coronary perfusion Perfusion to cerebral, renal, and splanchnic circulations also is likely to be improved with the lack of diastolic runoff to the pulmonary circulation, which may enhance postoperative recovery Because pulmonary blood flow occurs only during ventricular systole across the RV to the pulmonary artery conduit, there may be a critical reduction in pulmonary blood flow and excessive hypoxemia, especially during periods of low cardiac output or if there is dynamic obstruction to flow at the ventricular insertion site Efforts to overcome this limitation by creating a larger RV incision run the longerterm risk of ventricular dysfunction, arrhythmias, or aneurysm formation A multicenter, randomized, controlled trial involving 549 neonates with HLHS demonstrated a significantly improved transplantation-free survival for patients randomized to receive an RV-PA shunt compared to those palliated with a modified BT shunt (74% vs 64%, P 01).222 The major benefit of the RV-PA shunt seems to be an increased survival rate in the early period after the procedure The short-term survival advantage of the Sano modification of the Norwood operation for centers where the mortality rate after the Norwood operation was already below 15% will be hard to demonstrate More recent long-term followup, however, demonstrates an elimination of this survival advantage at years of age with the RV-PA shunt group.223 In addition, this group had worse RV ejection fraction, validating concerns regarding the impact of an incision in the systemic ventricle.223 Orthotopic heart transplantation has gained acceptance as an alternative treatment for HLHS.224 Neonatal transplants appear to be well-tolerated, and some centers have avoided maintenance steroid therapy while achieving excellent midterm results using transplantation as the sole therapeutic option for this disease.225,226 Others have successfully advocated a combined approach using either transplantation or staged reconstruction, depending on the pathophysiologic state of the child and the availability of a donor heart However, the critical shortage of donor organs places a marked limitation on the correction of this common congenital heart lesion CHAPTER 36 Critical Care After Surgery for Congenital Cardiac Disease Many children have derived benefit from a completed, staged reconstruction or heart transplantation for this previously fatal illness They are often able to lead active, productive lives, and many develop normally.227,228 Both survival and developmental outcomes for this disease are improving worldwide However, the long-term prognosis for this evolving therapy will not be known for several years Hybrid Approach. An alternative to the stage I Norwood palliation that eliminates the insult associated with CPB in the fragile neonate with HLHS has been developed This approach combines interventional cardiac catheterization techniques with less-involved surgery and is referred to as the hybrid procedure.229,230 The goal is to replicate the physiologic state of the Norwood procedure by the combination of three interventions: (1) placement of bilateral pulmonary artery bands to limit flow to the lungs, (2) placement of an endovascular stent to maintain the long-term patency of the ductus arteriosus, and (3) balloon atrial septostomy with or without stenting of the atrial communication to ensure adequate mixing and unrestricted left-to-right atrial flow.229–232 This procedure is accomplished through a standard median sternotomy and does not require CPB The procedure requires superb coordination between the surgical and cardiac catheterization teams It is generally performed in a hybrid suite consisting of a large modern cardiac catheterization laboratory with enough room to accommodate the surgeons and supporting operating room staff in addition to the interventional cardiology team and anesthesiologists Outcomes have been encouraging, with early mortality rates comparable to those of standard protocols (about 15%–20%).233,234 Direct comparison of the two methods is complicated because, in most centers, the hybrid procedure generally has been reserved for patients regarded as high risk for bypass surgery (e.g., low birthweight, unstable hemodynamics, and poor ventricular function) Interstage mortality can be significant (15%–20%),233 and case-matched studies have shown no benefit over conventional surgery.232 One barrier to the widespread implementation of the hybrid procedure is that results of conventional surgery in the low-risk groups are now so good that many centers of excellence have been reluctant to undertake a new procedure with its attendant learning curve The hybrid procedure poses some unique technical challenges: the ductal stent position is crucial and, if patients have a diminutive ascending and transverse aorta, then the procedure does not address this impediment to coronary flow If the transverse aorta is small, the stent itself might distort or interfere with retrograde flow into the arch For this reason, most centers not recommend the hybrid approach in the setting of a small transverse arch Nevertheless, the hybrid approach undoubtedly has a role in the management of patients with HLHS With continued encouraging results235 beyond the centers of excellence that developed and advanced this technique, it has found a niche among high-risk patients or as a bridge to transplantation Up to 50% of patients who survive the stage I hybrid procedure require catheter reintervention due to stent migration or restrictive flow across the atrial communication.233 The stage II procedure becomes much more extensive than the conventional stage II because the aortic arch needs to be reconstructed (excising the ductal stent), the bands removed, and the pulmonary arteries repaired with a patch in addition to creating the cavopulmonary connection Consequently, stage II after the hybrid procedure carries higher operative mortality (10% to 15%) This needs to be taken into account when comparing it with conventional techniques.233,235 No absolute consensus exists on the future of the 409 hybrid procedure This innovative approach offers potential benefits that should be scrutinized through careful study and longterm follow-up Summary The cardiac ICU has become the epicenter of activity in large cardiovascular programs Nowhere are collaborative practices and multidisciplinary skills more valued or necessary A curriculum in cardiac intensive care is now formally incorporated into cardiology training Pediatric intensive care training programs have a mandate to include curricula and experience in the management of postoperative cardiac patients Additional cardiac intensive care training is offered in selected centers to pediatric intensive care specialists wishing to pursue a career in the cardiac ICU Specialists in this field must have in-depth training in pediatric intensive care and cardiology, as the scope of practice goes well beyond the cardiovascular system and requires expertise in complex respiratory physiology, diagnosis and management of multiorgan system dysfunction, and the various supportive techniques vital to the discipline of intensive care, to name a few Increased complexity of disease, advances in technology and applied research, shortened LOSs, and improved survival in contemporary series all describe the fast-paced specialized environment that has accompanied the development of this new specialty of pediatric cardiac intensive care The dramatic reduction in cardiac intensive care mortality has been gratifying It is attributable to many factors Achieving 100% survival with minimal morbidity remains our elusive goal It will challenge the next generation of practitioners Acknowledgment We gratefully acknowledge the contributions of Peter C Laussen and David L Wessel, who authored this chapter in previous editions Key References Agus MS, Steil GM, Wypij D, et al Tight glycemic control versus standard care after pediatric cardiac surgery N Engl J Med 2012;367:1208-1219 Almond CS, Singh TP, Gauvreau K, et al Extracorporeal membrane oxygenation for bridge to heart transplantation among children in the United States: analysis of data from the Organ Procurement and Transplant Network and Extracorporeal Life Support Organization Registry Circulation 2011;123:2975-2984 Atz AM, Wessel DL Sildenafil ameliorates effects of inhaled nitric oxide withdrawal Anesthesiology 1999;91:307-310 Booth KL, Roth SJ, Thiagarajan RR, Almodovar MC, del Nido PJ, Laussen PC Extracorporeal membrane oxygenation support of the Fontan and bidirectional Glenn circulations Ann Thorac Surg 2004;77:1341-1348 Chang AC, Hanley FL, Wernovsky G, et al Early bidirectional cavopulmonary shunt in young infants Postoperative course and early results Circulation 1993;88:II149- II58 d’Udekem Y, Iyengar AJ, Galati JC, et al Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand Circulation 2014;130:S32-S38 Galantowicz M, Cheatham JP Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome Pediatr Cardiol 2005;26:190-199 Hoffman GM, Tweddell JS, Ghanayem NS, et al Alteration of the critical arteriovenous oxygen saturation relationship by sustained 410 S E C T I O N I V Pediatric Critical Care: Cardiovascular afterload reduction after the Norwood procedure J Thorac Cardiovasc Surg 2004;127:738-745 Jatene AD, Fontes VF, Souza LC, Paulista PP, Neto CA, Sousa JE Anatomic correction of transposition of the great arteries J Thorac Cardiovasc Surg 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Harb Perspect Med 2012;2:a006445 76 Dori Y, Keller MS, Fogel MA, et al MRI of lymphatic abnormalities after functional single-ventricle palliation surgery AJR Am J Roentgenol 2014;203:426-431 77 Mittnacht AJ, Hollinger I Fast-tracking in pediatric cardiac surgery—the current standing Ann Card Anaesth 2010;13:92-101 78 Vricella LA, Dearani JA, Gundry SR, Razzouk AJ, Brauer SD, Bailey LL Ultra fast track in elective congenital cardiac surgery Ann Thorac Surg 2000;69:865-871 79 Limperopoulos C, Majnemer A, Shevell MI, Rosenblatt B, Rohlicek C, Tchervenkov C Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery J Pediatr 2000;137:638-645 80 Marino BS, Lipkin PH, Newburger JW, et al Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association Circulation 2012;126:1143-1172 81 Donofrio MT, Bremer YA, Schieken RM, et al Autoregulation of cerebral blood flow in fetuses with congenital heart disease: the brain sparing effect Pediatr Cardiol 2003;24:436-443 82 Licht DJ, Shera DM, Clancy RR, et al Brain maturation is delayed in infants with complex congenital heart defects J Thorac Cardiovasc Surg 2009;137:529-536; discussion 536-537 ... transplantation for this previously fatal illness They are often able to lead active, productive lives, and many develop normally.227,228 Both survival and developmental outcomes for this disease are... procedure does not address this impediment to coronary flow If the transverse aorta is small, the stent itself might distort or interfere with retrograde flow into the arch For this reason, most centers... mortality (10% to 15%) This needs to be taken into account when comparing it with conventional techniques.233,235 No absolute consensus exists on the future of the 409 hybrid procedure This innovative