Noonan syndrome associated with anomalous left coronary artery from the pulmonary artery in a patient with the rare RAF1 mutation: A case report and review of literature.

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.

Intravascular Papillary Endothelial Hyperplasia in the Coronary Artery: An Unusual Cause of Massive Myocardial Infarction in Hypoplastic Left Heart Syndrome.

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.

One-Year Survival and Neurologic Outcomes After Pediatric Open-Chest Cardiopulmonary Resuscitation.

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 open-chest 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.

Large pseudoaneurysm following right ventricle to pulmonary artery homograft placement in an infant.

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.

Acute kidney injury following first-stage palliation in hypoplastic left heart syndrome: hybrid versus Norwood palliation.

OBJECTIVE: The aim of this study was to evaluate the prevalence of acute kidney injury after first-stage surgical palliation in patients with a single ventricle and to explore associated risk factors and outcomes. Design and patients This single-centre retrospective study included neonates who underwent either Norwood or Hybrid procedure from 2008 to 2015 for a single ventricle. Postoperative acute kidney injury was defined using the paediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), criteria within 72 hours of the procedure. Main results Our cohort (n=48) underwent surgical palliation at a mean (SD) age of 12 (11) days. Postoperative acute kidney injury was diagnosed in 14 (29%) patients. The prevalence of acute kidney injury in the Hybrid group was 16% and 53% in the Norwood group. Infants who developed acute kidney injury underwent surgery at younger ages [6 (5-10) versus 10 (8-16) days, p=0.016], and had a higher peak lactate level in the initial 24 hours [5.9 (4.2-9.1) versus 3.4 (2.4-6.7), p=0.007]. Norwood procedure was significantly associated with acute kidney injury [odds ratio 11.7 (95% confidence interval 1.3-101.9), p=0.03]. ICU stay [38 (21-84) versus 16 (6-45) days, p=0.038] and time to extubation [204 (120-606) versus 72 (26-234) hours, p=0.014] were longer in those with acute kidney injury. The two patients who developed early postoperative renal failure as per pRIFLE died before discharge from associated comorbidities. CONCLUSIONS: Acute kidney injury occurs in a third of the patients with single ventricle after surgical palliation but is mostly transient. Norwood, compared with Hybrid procedure, is a risk factor for postoperative acute kidney injury, which, in turn, is associated with longer ICU stay and time to extubation.

Coronary artery spasm in a neonate with transposition of great arteries: a rare complication and reason for heart transplant.

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.

Prospective Validation of a Novel Vasoactive-Ventilation-Renal Score as a Predictor of Outcomes After Pediatric Cardiac Surgery.

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.

Effect of Hybrid stage 1 procedure on ventricular function in infants with hypoplastic left heart syndrome.

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.

Vasoactive Inotropic Score (VIS) as Biomarker of Short-Term Outcomes in Adolescents after Cardiothoracic Surgery.

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.

Risk Factors for Extubation Failure Following Neonatal Cardiac Surgery.

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.

Prevalence and risk factors for upper airway obstruction after pediatric cardiac surgery.

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.

Use of a novel vasoactive-ventilation-renal score to predict outcomes after paediatric cardiac surgery.

OBJECTIVES: Prior studies have established peak postoperative lactate and the vasoactive-inotrope score (VIS) as modest predictors of outcome following paediatric cardiac surgery. We developed a novel vasoactive-ventilation-renal (VVR) score and aimed to determine if this index, which incorporates postoperative respiratory, cardiovascular and renal function, would more consistently predict outcome in this patient population. METHODS: We performed an Institutional Review Board-approved retrospective analysis of 222 infants at our institution less than 365 days old who underwent surgery for congenital heart disease at our centre from January 2009 to April 2013. The VVR score was calculated as follows: vasoactive-inotrope score + ventilation index + (change in serum creatinine from baseline × 10). For all patients, peak lactate and admission, peak, and 48 h VIS and VVR were recorded. RESULTS: For all outcome measures, areas under the curve for 48-h VVR were greater than its corresponding admission and peak values, VIS alone at all three time points and peak lactate. On multivariate regression, 48-h VVR was strongly associated with prolonged intubation [odds ratio (OR): 39.13, P <0.0001], significantly more so than 48-h VIS (odds ratio: 6.18, P <0.0001) and peak lactate (odds ratio: 2.52, P = 0.017). The 48-h VVR was also more significantly associated with prolonged use of vasoactive infusions, chest tube drainage and ICU and hospital stay when compared with VIS alone and peak lactate. CONCLUSIONS: The novel 48-h VVR was a robust predictor of outcome following paediatric cardiac surgery and outperformed the VIS and peak postoperative lactate.

An unusual complication of three-patch repair of supravalvar aortic stenosis.

Three-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.

Peritoneal Drainage Versus Pleural Drainage After Pediatric Cardiac Surgery.

BACKGROUND: We aimed to determine whether infants undergoing cardiac surgery would more efficiently attain negative fluid balance postoperatively with passive peritoneal drainage as compared to traditional pleural drainage. METHODS: A prospective, randomized study including children undergoing repair of tetralogy of Fallot (TOF) or atrioventricular septal defect (AVSD) was completed between September 2011 and June 2013. Patients were randomized to intraoperative placement of peritoneal catheter or right pleural tube in addition to the requisite mediastinal tube. The primary outcome measure was fluid balance at 48 hours postoperatively. Variables were compared using t tests or Fisher exact tests as appropriate. RESULTS: A total of 24 patients were enrolled (14 TOF and 10 AVSD), with 12 patients in each study group. Mean fluid balance at 48 hours was not significantly different between study groups, -41 ± 53 mL/kg in patients with periteonal drainage and -9 ± 40 mL/kg in patients with pleural drainage (P = .10). At 72 hours however, postoperative fluid balance was significantly more negative with peritoneal drainage, -52.4 ± 71.6 versus +2.0 ± 50.6 (P = .04). On subset analysis, fluid balance at 48 hours in patients with AVSD was more negative with peritoneal drainage as compared to pleural, -82 ± 51 versus -1 ± 38 mL/kg, respectively (P = .02). Fluid balance at 48 hours in patients with TOF was not significantly different between study groups. CONCLUSION: Passive peritoneal drainage may more effectively facilitate negative fluid balance when compared to pleural drainage after pediatric cardiac surgery, although this benefit is not likely universal but rather dependent on the patient's underlying physiology.

Necessity of temporary epicardial pacing wires after surgery for congenital heart disease.

BACKGROUND: Temporary epicardial pacing wires are commonly placed in patients undergoing surgery for congenital heart disease. Though often helpful, these wires are not without risk. We aimed to identify characteristics that would obviate placement of temporary epicardial pacing wires in this patient population. METHODS: A prospective observational study was performed on patients admitted to the pediatric intensive care unit after surgery for congenital heart disease between October 2011 and October 2012. Logistic regression analysis was performed to identify independent predictors of patients in whom wires were not helpful postoperatively. RESULTS: Wires were placed in 213 of 249 patients. Wires were helpful in 50 patients; 23 for diagnostic purposes only, 17 for therapeutic purposes only, and 10 for both. On logistic regression analysis, absence of intraoperative arrhythmias (p < 0.01), lower arteriovenous O2 difference (p < 0.01), and shorter duration of cardiopulmonary bypass (p = 0.050) were significant predictors of patients in whom wires were not helpful postoperatively. Further, the predicted probability based on logistic regression model using these 3 variables correctly identified 93% of patients who did not need pacing wires. Four complications (1.9%) related to wires occurred, including 1 episode of life-threatening bleeding that was found, during emergent exploration, to be due to atrial perforation at the wire insertion site. CONCLUSIONS: Temporary epicardial pacing wires are not necessary in many patients recovering from surgery for congenital heart disease. A conservative approach to their use may therefore be warranted.

Hybrid procedure of bilateral pulmonary artery banding and bilateral ductal stenting in an infant with aortic atresia and interrupted aortic arch.

We report an infant with aortic valve atresia, interrupted aortic arch, ventricular septal defect, confluent pulmonary arteries, bilateral arterial ducts, absent common carotid arteries, and anomalous coronary arteries arising from main pulmonary artery. Hybrid procedure consisting of bilateral pulmonary artery banding and bilateral arterial duct stenting was performed at 4 weeks of age. Hybrid procedure can be an alternative palliative approach in an infant with this complex cardiac anatomy.

Safety and utility of passive peritoneal drainage following Fontan palliation.

OBJECTIVE: Placement of peritoneal drainage catheters intra-operatively has been shown to help prevent fluid overload in children recovering from surgery for two-ventricle heart disease. We aimed to determine whether this practice is also helpful in children recovering from Fontan palliation. MATERIAL AND METHODS: A retrospective review was performed on children with single-ventricle anatomy undergoing Fontan palliation at our institution from 2007 to 2011. Variables in those with peritoneal drainage were compared with those without using t-tests, Mann-Whitney U-tests, chi-square tests, or analysis of variance for repeated measures as appropriate. Data were represented as mean with standard deviation unless otherwise noted. RESULTS: A total of 43 children were reviewed, 21 (49%) with peritoneal drainage catheters. No complications from catheter placement occurred. The groups did not differ with regard to cardiopulmonary bypass duration, dominant ventricle, pre-operative haemodynamic data, fenestration use, and initial intensive care unit ventilation index. Central venous pressures, vasoactive medication use, and diuretic use during the first 48 hours were also not statistically different. At 48 hours, the median fluid balance was -9 (interquartile range : -50, +20) in those with peritoneal drainage and +77 cc/kg (interquartile range : +22, +96) in those without (p < 0.001), yet median duration of mechanical ventilation was 40 hours (range: 19-326) in those with peritoneal drainage and 23 hours (range: 9-92) in those without, p = 0.01. CONCLUSION: Patients with peritoneal drainage recovering from Fontan palliation achieved negative fluid balance as compared with those without peritoneal drainage, although this difference was associated with a longer duration of mechanical ventilation.