The development of knowledge about anomalies of systemic venous connection (ASVC) is best illustrated by the history of its most common representative, the left superior caval vein. In one large series, 100 cases of ASVC were found among 5127 patients undergoing open-heart surgery between 1955 and 1974. Embryologically, left superior caval vein to the coronary sinus is thought to be due to the persistence of the left anterior and common cardinal veins. Unroofed coronary sinus syndrome (UCSS) is a spectrum of congenital cardiac anomalies consisting of partial or complete absence of the partition between the coronary sinus and the left atrium. UCSS can consist of either complete or partial unroofing. Indirect inferior caval vein connections exist when the inferior caval vein does not connect to the atrium from below, but rather “continues” as an extension of either the azygos or hemiazygos veins.

Extracorporeal Membrane Oxygenation (ECMO) for cardiac support has largely been limited to pediatric patients. Adults with cardiac failure have other options including ventricular assist devices and the intraaortic balloon pump. ECMO is best performed by an organized and established team. Impromptu ECMO, often run with local or proprietary perfusionists with only a limited background in ECMO support may occasionally be successful but more often than not is a recipe for disappointment. ECMO support is usually initiated in the intensive care unit except for those patients placed on ECMO in the operating room following open heart surgery. The most widely used form of ECMO is venoarterial ECMO which involves cannulation of both the right common carotid artery and internal jugular vein. Most neonates considered for ECMO support have respiratory failure with pulmonary hypertension of the newborn often being the underlying mechanism of respiratory distress, irrespective of the primary insult. Periodic neurological checks are necessary for all patients on ECMO support.

Background. Limited data exist about neurobehavioral outcomes of children treated with open-chest cardiopulmonary resuscitation (CPR). Our objective was to describe neurobehavioral outcomes 1 year after arrest among children who received open-chest CPR during in-hospital cardiac arrest and to explore factors associated with 1 year survival and survival with good neurobehavioral outcome. Methods. The study is a secondary analysis of the Therapeutic Hypothermia after Pediatric Cardiac Arrest In-Hospital Trial. Fifty-six children who received openchest CPR for in-hospital cardiac arrest were included. Neurobehavioral status was assessed using the Vineland Adaptive Behavior Scales, Second Edition (VABS-II) at baseline before arrest and 12 months after arrest. Norms for VABS-II are 100 +/- 15 points. Outcomes included 12-month survival, 12-month survival with VABS-II decreased by no more than 15 points from baseline, and 12-month survival with VABS-II of 70 or more points. Results. Of 56 children receiving open-chest CPR, 49 (88%) were after cardiac surgery and 43 (77%) were younger than 1 year. Forty-four children (79%) were cannulated for extracorporeal membrane oxygenation (ECMO) during CPR or within 6 hours of return of spontaneous circulation. Thirty-three children (59%) survived to 12 months, 22 (41%) survived to 12 months with VABS-II decreased by no more than 15 points from baseline, and of the children with baseline VABS-II of 70 or more points 23 (51%) survived to 12 months with VABS-II of 70 or more points. On multivariable analyses, use of ECMO, renal replacement therapy, and higher maximum international normalized ratio were independently associated with lower 12-month survival with VABS-II of 70 or more points. Conclusions. Approximately one-half of children survived with good neurobehavioral outcome 1 year after open-chest CPR for in-hospital cardiac arrest. Use of ECMO and postarrest renal or hepatic dysfunction may be associated with worse neurobehavioral outcomes. (C) 2019 by The Society of Thoracic Surgeons

Case Report A 14 year old presented for an episode of transient ischemic event consistent with left side paresis and left facial droop, resolved spontaneously in 15 min. There were no preceding warning symptoms. One week prior to this episode, the patient complained of exertional chest pain and shortness of breath while dancing. Her physical examination was unremarkable except for systolic ejection murmur best heard at the left upper sternal border. Neurological examination was unremarkable. The vital signs were normal for age. Chest radiograph showed normal cardiac silhouette with normal lung fields. EKG showed normal sinus with T …

Background: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion that usually involves the head and neck or extremities. Involvement of the coronary arterial system is unreported. Case: A 1-month-old patient born with hypoplastic left heart syndrome died from a massive myocardial infarction shortly after first stage palliation with Norwood/Sano. Autopsy demonstrated a massive univentricular hemorrhagic infarction with complete occlusion of the left main coronary artery and its intramural branches by intraluminal papillary endothelial hyperplasia (IPEH). Immunostaining with CD34 and CD31 confirmed the diagnosis. The inferior and superior mesenteric artery branches also had IPEH. Conclusion: IPEH can involve the coronary vasculature, can be multifocal and can occur in the newborn.

We present the case of a 7-week-old male infant diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) who underwent repair by left coronary artery reimplantation, followed by an eventful postoperative period including need for venous arterial extracorporeal membrane oxygenation and mitral valve replacement due to mitral calcification and severe insufficiency. He also required heart transplant due to severe rapidly progressive biventricular hypertrophy. The pathology examination of the explanted heart showed massive cardiomegaly. Subsequently, the infant's cardiomyopathy panel was positive for RAF1 mutation, consistent with diagnosis of a rare form of Noonan syndrome. To our knowledge, this autosomal dominant condition in association with ALCAPA has not been previously reported in the literature.

Pseudoaneurysm (PSA) is a known but rare complication of the right ventricle to pulmonary artery (RV-PA) conduits. The patient's clinical presentation can be variable ranging from asymptomatic to potential rupture. We describe an unusual case of a massive PSA in an infant who underwent RV-PA pulmonary homograft placement after relief of right ventricular outflow tract obstruction.

Amiodarone is a class III antiarrhythmic medication known for its efficacy in treating tachyarrhythmias. The pulmonary complications including acute lung toxicity are well documented in the adult population, however much less so in pediatrics. We describe two pediatric congenital heart disease patients with suspected amiodarone induced pulmonary toxicity in the post-operative period. Case 1 presented with acute respiratory distress syndrome accompanied by periods of pulmonary hemorrhage post-operatively; after multiple courses of methylprednisolone and discontinuation of amiodarone, she showed significant clinical improvement with normalization of her CXR. Case 2 presented as hypoxemic with acute respiratory distress syndrome, and had notable clinical improvement after cessation of amiodarone with pulse dose methylprednisolone and inhaled budesonide.

Arterial switch operation has become the standard of care for d-transposition of great arteries and has excellent short- and long-term outcomes. We report the case of a newborn with a diagnosis of d-transposition of great arteries with intact ventricular septum and a low-risk coronary artery anatomy who developed coronary artery vasospasm while coming off bypass following arterial switch operation in the operating room. The coronary artery spasm led to severe biventricular dysfunction and need for extracorporeal membranous oxygenation support. Despite extracorporeal membranous oxygenation and inotropic support, there was no improvement in the left ventricular function, and cardiac transplantation was performed after 8 days. The explanted heart showed extensive infarction of both ventricles. Both the coronary ostei were patent with no evidence of thrombus, suggesting coronary artery vasospasm rather than embolus or thrombus formation. This is the first case of coronary artery vasospasm in a neonate with d-transposition of great arteries leading to cardiac transplantation. We speculate that early identification of patients who are at a high risk for coronary vasospasm and prophylactic or timely infusion of papaverine directly into the coronary arteries may be beneficial in this condition.

Central MessageCarefully selected patients with a borderline left ventricle may be candidates for left ventricular recruitment and 2-ventricle circulation, providing left ventricular end-diastolic pressure < 13 mm Hg.See Article page 572. Carefully selected patients with a borderline left ventricle may be candidates for left ventricular recruitment and 2-ventricle circulation, providing left ventricular end-diastolic pressure < 13 mm Hg. See Article page 572. Approximately 20 years ago, when I was a senior registrar at Great Ormond Street Hospital in London, Marc de Leval performed a bidirectional Glenn anastomosis in a child with double-outlet right ventricle and a remote ventricular septal defect. The child did beautifully, and was on her way home to Israel in less than a week. This case sparked an interest in looking up the Great Ormond Street Hospital experience with single-ventricle palliation in patients with 2 ventricles. Mr de Leval put a finer point on it, and reduced it to a basic question: Is a complicated 2 ventricle repair better than single ventricle palliation?1Delius R.E. Rademecker M.A. de Leval M.R. Elliott M.J. Stark J. Is a high risk biventricular repair always preferable to conversion to a single ventricle repair?.J Thorac Cardiovasc Surg. 1996; 112: 1561-1569Abstract Full Text Full Text PDF PubMed Scopus (95) Google Scholar Two decades later, the question remains unanswered. There have been some studies suggesting that pushing a 2-ventricle repair in patients with a marginal left ventricle increases mortality compared with single-ventricle palliation.2Hickey E.J. Caldarone C.A. Blackstone E.H. Lofland G.K. Yeh Jr., T. Pizarro C. et al.Critical left ventricular outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases.J Thorac Cardiovasc Surg. 2007; 134: 1429-1437Abstract Full Text Full Text PDF PubMed Scopus (125) Google Scholar The long-term outcomes of laminar flow-type Fontans, either later tunnel or external conduit, are just now coming to light, but clearly seem to provide better long-term palliation than atriopulmonary-type Fontans.3Poh C.L. Zannino D. Weintraub R.G. Winlaw D.S. Grigg L.E. Cordina R. et al.Three decades later: the fate of the population of patients who underwent the atriopulmonary Fontan procedure.Int J Cardiol. 2017; 231: 99-104Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar, 4Pundi K.N. Johnson J.N. Dearani J.A. Pundi K.N. Li Z. Hinck C.A. et al.40-year follow-up after the Fontan operation: long term outcomes of 1,052 patients.J Am Coll Cardiol. 2015; 66: 1700-1710Crossref PubMed Scopus (372) Google Scholar, 5d'Udekem Y. Iyengar A.J. Galati J.C. Forsdick V. Weintraub R.G. Wheaton G.R. 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-S38Crossref PubMed Scopus (398) Google Scholar Nevertheless, in virtually all busy practices there are patients with seemingly perfect Fontans who experience protein-losing enteropathy or plastic bronchitis, as well as other sequelae of single-ventricle palliation. The group at Children's Hospital of Boston has clearly come down on the side of 2 ventricles being preferable, and have provided another excellent article on this topic.6Herrin M.A. Zurakowski D. Baird C.W. Banka P. Esch J.J. del Nido P.J. et al.Hemodynamic parameters predict adverse outcomes following biventricular conversion with single-ventricle palliation takedown.J Thorac Cardiovasc Surg. 2017; 154: 572-582Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar This report delivers some clearly defined messages: an elevated left ventricular end-diastolic pressure ≥ 13 mm Hg is a risk factor for a suboptimal outcome when pursuing a 2-ventricle strategy and is a better discriminator than anatomic measurements. This is not surprising—anatomy and physiology usually work in tandem—but when they diverge, physiology usually wins the argument. The article also suggests that a 2-ventricle recruitment strategy has worse outcomes in patients with borderline hypoplastic left heart syndrome than in patients with unbalanced atrioventricular canal.6Herrin M.A. Zurakowski D. Baird C.W. Banka P. Esch J.J. del Nido P.J. et al.Hemodynamic parameters predict adverse outcomes following biventricular conversion with single-ventricle palliation takedown.J Thorac Cardiovasc Surg. 2017; 154: 572-582Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar The inference is that using a series of operations to coax left ventricular growth can work, but the keyhole these patients have to pass through is relatively small. As with any research, one can quibble about some findings. The authors suggest that 75% of patients are not candidates for single-ventricle palliation, but they do not elucidate what those factors are in a given patient.6Herrin M.A. Zurakowski D. Baird C.W. Banka P. Esch J.J. del Nido P.J. et al.Hemodynamic parameters predict adverse outcomes following biventricular conversion with single-ventricle palliation takedown.J Thorac Cardiovasc Surg. 2017; 154: 572-582Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar In a bit of cognitive dissonance, they in turn suggest that single-ventricle palliation is a fallback plan if left ventricular recruitment maneuvers are not successful. In any event, the article is very well done and clinically useful. I applaud the authors for pushing the envelope on this matter. Their approach is bold, creative, and innovative. In this era of public reporting and hospital administrators wringing their hands over US News and World Report rankings and 3-star ratings, new ideas that entail a degree of risk are often discouraged. One wonders if the conversion from atrial to arterial switch would ever happen in the modern era, given that Mustard/Senning mortality at the time was ∼ 1% versus a 15% mortality in early arterial switch series. Despite these prevailing headwinds, the Boston Children's Hospital group has forged ahead and applied a thoughtful and novel approach to this cohort of patients. Hemodynamic parameters predict adverse outcomes following biventricular conversion with single-ventricle palliation takedownThe Journal of Thoracic and Cardiovascular SurgeryVol. 154Issue 2PreviewPatients with a borderline left ventricular hypoplasia in the hypoplastic left heart syndrome variant or an unbalanced atrioventricular canal who undergo initial single-ventricle palliation may be candidates for biventricular (BiV) conversion following left ventricle (LV) recruitment procedures. We investigated associations among preoperative parameters and postoperative outcomes in patients undergoing BiV conversion. 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BACKGROUND:We sought to further validate the novel vasoactive-ventilation-renal (VVR) score in a prospective study of a heterogeneous cohort of children undergoing cardiac surgery that includes patients with single-ventricle anatomy and residual mixing lesions.METHODS:We prospectively performed an observational study of all children less than 18 years of age who underwent surgery for congenital heart disease at our center from November 2013 to June 2014. We calculated VVR score as follows: vasoactive-inotrope score + ventilation index + (change in serum creatinine from baseline × 10). Admission, peak, and 48-hour measurements were recorded. Outcomes of interest were prolonged duration of mechanical ventilation and intensive care unit and hospital stays, represented by the upper 25% for all patients. Areas under the receiver-operating characteristic curves (AUC) were determined for all study timepoints and outcome variables.RESULTS:Ninety-two patients were analyzed; their median age was 0.65 (range, 3 days to 17.9 years), and 17 (18%) had single-ventricle anatomy. The VVR measurements outperformed vasoactive-inotrope scores in isolation at all timepoints, with higher AUC values for all outcomes. Of the three timepoints assessed, the 48-hour VVR score most consistently predicted poor outcome, especially with regard to prolonged duration of mechanical ventilation (AUC 0.980) and prolonged intensive care unit stay (AUC 0.919).CONCLUSIONS:In a heterogeneous population of children undergoing cardiac surgery, the 48-hour VVR score was a very strong predictor of outcomes, and outperformed the more traditional vasoactive-inotrope score. The VVR score, therefore, represents a novel and potentially powerful means of predicting clinical outcomes relatively early in the hospital course of these patients.

Extracorporeal membrane oxygenation (ECMO) is a widely used form of mechanical circulatory support for infants and children with refractory cardiac and respiratory failure. The use of ECMO in infant and pediatric cardiac failure continues to increase over the last decade with 51% survival to discharge. Despite improvements in short term survival, ECMO remains an invasive and expensive therapy with significant associated complications and both short and long term sequelae. The focus of this review is to provide clinicians with a better understanding of the evolving indications, implementation, complications, outcomes and utilization in unique subgroups of pediatric cardiac ECMO patients.

We read with interest the report of Delius and colleagues [1Delius R.E. Walters III, H.L. Bondarenko I. An unusual complication of three-patch repair of supravalvar aortic stenosis.Ann Thorac Surg. 2014; 98: 1449-1451Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar] describing coronary ischemia after the three-patch repair for supravalvar aortic stenosis. They encountered kinking of the origin of the left anterior descending coronary artery that was subsequently managed successfully by reimplanting the left coronary artery into the left sinus patch. The three-patch technique, originally described by Brom, is now our preferred surgical option for repair of supravalvar aortic stenosis. As with any operation on the aortic root, compromise of the coronary arteries is a risk after the three-patch repair. We have managed several patients who experienced severe right coronary ischemia secondary to a redundant right sinus patch after a Brom operation (Fig 1). A few strategically placed horizontal mattress sutures were used in each case to reduce kinking of the proximal right coronary artery, thereby restoring coronary perfusion. There was an immediate improvement in all patients in right ventricular color and contractility. This allowed us to avoid a further period of myocardial ischemia. We have used a similar technique in the setting of the arterial switch operation to reduce coronary compression by a redundant pericardial patch used for neopulmonary artery reconstruction. Coronary complications must be recognized and managed early to avoid morbidity resulting from prolonged myocardial ischemia. It is very important to constantly observe myocardial perfusion and contractility after release of the aortic cross-clamp, especially when preparing to come off cardiopulmonary bypass and soon after separating the patient from cardiopulmonary bypass. Because the right ventricle is anterior, it was a simple matter in our cases to suspect and diagnose a right coronary problem by visual assessment of the myocardium alone. In contrast, left ventricular ischemia secondary to left coronary artery kinking or compression is more difficult to diagnose and requires a high index of suspicion. Electrocardiographic and echocardiographic data should be supplemented, whenever possible, with visual inspection of left ventricular perfusion and contractility to identify left coronary artery distortion. We congratulate Delius and colleagues [1Delius R.E. Walters III, H.L. Bondarenko I. An unusual complication of three-patch repair of supravalvar aortic stenosis.Ann Thorac Surg. 2014; 98: 1449-1451Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar] on the successful outcome of their challenging case. We acknowledge the help of Rebekah Dodson for the illustrations used in this report. An Unusual Complication of Three-Patch Repair of Supravalvar Aortic StenosisThe Annals of Thoracic SurgeryVol. 98Issue 4PreviewThree-patch repair of supravalvar aortic stenosis is a widely accepted surgical approach for this congenital heart lesion. We describe an unusual complication of this approach, which resulted in ischemia in the left anterior coronary artery distribution. Subtle oversizing of the left sinus of Valsalva patch led to kinking of the origin of the left anterior descending artery; the circumflex artery was not affected. Sinus of Valsalva reconstruction and reimplantation of the left coronary button restored normal coronary perfusion. Full-Text PDF ReplyThe Annals of Thoracic SurgeryVol. 99Issue 6PreviewWe would like to thank Drs Ramakrishnan and Jonas [1] for their interesting comments about our case report [2]. It is gratifying in a schadenfreude sort of way to know that we have not been the only ones to be confronted with this issue! The authors provided a suggested solution that is also applicable to an arterial switch, and they have nicely illustrated their technique. We have also used this technique in the setting of an arterial switch, with good effect. It is useful to have both techniques available in your surgical quiver when confronted by a patient with unanticipated ischemia following sinus of Valsalva patching. Full-Text PDF

OBJECTIVE:Extubation failure after neonatal cardiac surgery has been associated with considerable postoperative morbidity, although data identifying risk factors for its occurrence are sparse. We aimed to determine risk factors for extubation failure in our neonatal cardiac surgical population.DESIGN:Retrospective chart review.SETTING:Urban tertiary care free-standing children's hospital.PATIENTS:Neonates (0-30 d) who underwent cardiac surgery at our institution between January 2009 and December 2012 was performed.INTERVENTIONS:Extubation failure was defined as reintubation within 72 hours after extubation from mechanical ventilation. Multivariate logistic regression analysis was performed to determine independent risk factors for extubation failure.MEASUREMENTS AND MAIN RESULTS:We included 120 neonates, of whom 21 (17.5%) experienced extubation failure. On univariate analysis, patients who failed extubation were more likely to have genetic abnormalities (24% vs 6%; p = 0.023), hypoplastic left heart (43% vs 17%; p = 0.009), delayed sternal closure (38% vs 12%; p = 0.004), postoperative infection prior to extubation (38% vs 11%; p = 0.002), and longer duration of mechanical ventilation (median, 142 vs 58 hr; p = 0.009]. On multivariate analysis, genetic abnormalities, hypoplastic left heart, and postoperative infection remained independently associated with extubation failure. Furthermore, patients with infection who failed extubation tended to receive fewer days of antibiotics prior to their first extubation attempt when compared with patients with infection who did not fail extubation (4.9 ± 2.6 vs 7.3 ± 3; p = 0.073).CONCLUSIONS:Neonates with underlying genetic abnormalities, hypoplastic left heart, or postoperative infection were at increased risk for extubation failure. A more conservative approach in these patients, including longer pre-extubation duration of antibiotic therapy for postoperative infections, may be warranted.

OBJECTIVE:The effect of Hybrid stage 1 palliation for hypoplastic left heart syndrome on right ventricular function is unknown. We sought to compare right ventricular function in normal neonates and those with hypoplastic left heart syndrome before Hybrid palliation and to assess the effect of Hybrid palliation on right ventricular function, using the right ventricular myocardial performance index and the ratio of systolic and diastolic durations. METHODS:We carried out a retrospective review of echocardiographic data on 23 infants with hypoplastic left heart syndrome who underwent Hybrid palliation and 35 normal controls. Data were acquired before Hybrid and after Hybrid palliation - post 1, 0-4 days; post 2, 1 week; post 3, 2-3 weeks; post 4, 1-1.5 months following Hybrid palliation. RESULTS:Myocardial performance index and ratio of systolic and diastolic durations were higher in the pre-Hybrid hypoplastic left heart syndrome group (n=23) - 0.47±0.16 versus 0.25±0.07, p<0.001; 1.59±0.44 versus 1.09±0.14, p<0.0001 - compared with controls (n=35). There was no significant change in the myocardial performance index at any of the post-Hybrid time points. Ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid - post 3: 2.08±0.62 and post 4: 2.21±0.45 versus pre: 1.59±0.44, p=0.043 and 0.003. There were no significant differences in parameters between sub-groups of infants who died (n=10) and survivors (n=13). CONCLUSIONS:Right ventricular myocardial performance index and ratio of systolic and diastolic durations were significantly higher in infants with hypoplastic left heart syndrome before intervention compared with controls. The ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid palliation. Our data suggest that infants with hypoplastic left heart syndrome have right ventricular dysfunction before intervention, which worsens over 2 weeks after Hybrid palliation.

Objective To determine the prevalence of and risk factors for extrathoracic upper-airway obstruction after pediatric cardiac surgery. Study design A retrospective chart review was performed on 213 patients younger than 18 years of age who recovered from cardiac surgery in our multidisciplinary intensive care unit in 2012. Clinically significant upper-airway obstruction was defined as postextubation stridor with at least one of the following: receiving more than 2 corticosteroid doses, receiving helium-oxygen therapy, or reintubation. Multivariate logistic regression analysis was performed to determine independent risk factors for this complication. Results Thirty-five patients (16%) with extrathoracic upper-airway obstruction were identified. On bivariate analysis, patients with upper-airway obstruction had greater surgical complexity, greater vasoactive medication requirements, and longer postoperative durations of endotracheal intubation. They also were more difficult to calm while on mechanical ventilation, as indicated by greater infusion doses of narcotics and greater likelihood to receive dexmedetomidine or vecuronium. On multivariable analysis, adjunctive use of dexmedetomedine or vecuronium (OR 3.4, 95% CI 1.4-8) remained independently associated with upper-airway obstruction. Conclusion Extrathoracic upper-airway obstruction is relatively common after pediatric cardiac surgery, especially in children who are difficult to calm during endotracheal intubation. Postoperative upper-airway obstruction could be an important outcome measure in future studies of sedation practices in this patient population.

Our aim was to evaluate the Vasoactive Inotropic Score (VIS) as a prognostic marker in adolescents following surgery for congenital heart disease. This single-center retrospective chart review included patients 10-18 years of age, who underwent cardiac surgery from 2009 to 2014. Hourly VIS was calculated for the initial 48 postoperative hours using standard formulae and incorporating doses of six pressors. The composite adverse outcome was defined as any one of death, resuscitation or mechanical support, arrhythmia, infection requiring antibacterial therapy, acute kidney injury or neurologic injury. Surgeries were risk-stratified by the type of surgical repair using the validated STAT score. Statistical analysis (SPSS 19.0) included Mann-Whitney U test, Chi-square test, ROC curves, and binary regression analysis. Our cohort (n = 149) had a mean (SD) age of 13.9 (2.4) years and included 97 (65.1 %) males. Maximal VIS at 24 and 48 h following surgery was significantly higher in subjects (n = 27) who suffered an adverse outcome. Subjects with adverse outcome had longer bypass and cross-clamp times, durations of stay in the hospital, and a higher rate of acute kidney injury, compared to those (n = 122) without postoperative adverse outcomes. The area under the ROC for maximum VIS at 24-48 h after surgery was 0.76, with sensitivity, specificity, and positive and negative predictive values with 95 % CI of 67 (48-82) %, 74 (70-77) %, and 36 (26-44) % and 91 (86-95) %, respectively, at a cutoff >4.75. On binary logistic regression, maximum VIS on second postoperative day remained significantly associated with adverse outcome (OR 1.35; 95 % CI> 1.12-1.64, p = 0.002). Maximal VIS at 24 and 48 h correlated significantly with length of stay and time to extubation. Maximal VIS on the second postoperative day predicts adverse outcome in adolescents following cardiac surgery. This simple yet robust prognostic indicator may aid in risk stratification and targeted interventions in this population.