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Lung transplantation is the definitive surgical treatment for end-stage lung disease. However, infants comprise less than 5% of pediatric cases. This study sought to provide an overview of infant lung transplantation outcomes over the past 3 decades by using linked United Network for Organ Sharing (UNOS) and Pediatric Health Information System (PHIS) data.Infants undergoing lung transplantation from 1989 to 2020 in UNOS were reviewed. UNOS and PHIS records for patients who underwent lung transplantation from 1995 to 2020 were linked using date of birth, sex, and date of surgery ± 3 days. The study assessed underlying diagnoses, pretransplant and posttransplant extracorporeal membrane oxygenation support, retransplant-free survival to discharge, hospital experience (≥1 annual transplant for ≥4 years in a 5-year period), operative decade, bronchiolitis obliterans syndrome, long-term survival, and functional status at latest follow-up.A total of 112 lung transplants were performed in 109 infants over 31 years. Of these, 21 patients died before discharge, and 2 underwent repeat transplantation during the same admission. The study linked 80.6% (83 of 103) of UNOS and PHIS records. Hospital survival was lower for infants with idiopathic pulmonary hypertension and those who underwent transplant procedures at less experienced centers. All 7 infants requiring postoperative extracorporeal membrane oxygenation support died. Median freedom from bronchiolitis obliterans syndrome was 8.1 years (interquartile range, 4.6 to 11.6 years). After discharge, median survival was 10.3 years (interquartile range, 6.3 to 14.4 years), with improved 10-year survival for those patients who underwent transplantation from 2010 to 2020 (87.3%) vs 2000 to 2009 (52.4%; P = .098) and 1989 to 1999 (34.1%; P = .004). A total of 84.6% (33 of 39) of survivors had minor or no restrictions at latest follow-up.Carefully selected infants experience promising short- and long-term outcomes after lung transplantation.

Heart-lung transplantation has historically been used as a definitive treatment for children with end-stage cardiopulmonary failure, although the number performed has steadily decreased over time. In this review, we discuss current indications, preoperative risk factors, outcomes, and heart-lung transplantation in unique patient subsets, including infants, children with single-ventricle physiology, tetralogy of Fallot/major aortopulmonary collateral arteries, and prior Potts shunt palliation. We also describe the different surgical techniques utilized in pediatric heart-lung transplantation.

Only a handful of highly specialized transplant centers across the world are performing pediatric lung transplantation. Growing experience with sequential bilateral transplants has resulted in improved outcomes and it has mostly replaced the en bloc double lung transplant technique. In our institution, however, we preferentially adopted this technique for the infant population and we have noted favorable outcomes with this approach. Median sternotomy and dissection of the aorta, aortopulmonary window and right pulmonary artery is performed. The trachea is exposed between the aorta and the superior vena cava. The lungs are mobilized and the hilar structures are isolated extrapericardially. The pericardium is opened on both sides and pleuropericardial windows are developed. The recipient is cannulated at the ascending aorta, superior vena cava and IVC. Each lung is removed sequentially with the division of the pulmonary veins, pulmonary arteries, and main bronchi all extrapericardially. The donor right and the left lungs are prepared and passed into the respective hemithoraces through their respective pericardial windows. The donor distal trachea is then taken into the posterior mediastinum between the superior vena cava and aortic arch and suture anastomosed with recipient trachea. The recipient main pulmonary artery is divided just proximal to its bifurcation and main pulmonary artery to main pulmonary artery end to end anastomosis is carried out. An oblique atriotomy is made between the ligated stumps of the right and left pulmonary veins, and extended into the left atrial appendage. The donor left atrial cuff is anastomosed to this atriotomy. The lungs are slowly reinflated and ventilated.

Introduction Oxygenators are well known for their application in extracorporeal circulation. Berlin Heart silicone cannulae were specifically designed for use with ventricular assist devices. We describe a novel combination of these two devices for creation of a pumpless, paracorporeal system for bridging an infant to lung transplantation. Case Description A four-month-old baby, born at 36 weeks of gestation with refractory respiratory failure with components of both parenchymal lung disease and pulmonary hypertension, was referred from an outside institution. He had previously undergone ductal stenting at …

Since 2012, we have supported 18 children with single ventricle (SV) physiology on ventricular assist devices (VADs) as a bridge to decision, transplantation, or recovery. We provide a detailed report of our cumulative surgical experience and lessons learned from these patients.We reviewed all SV-VADs between March 2012 and April 2020. Implanted SV-VADs intended for short-term support were excluded. Demographic and clinical data included palliation stage at the time of VAD implantation, cannulation configuration, device type, duration of support, circuit and device interventions, postoperative support, anticoagulation strategy, complications, mortality, and 1-year survival postdischarge.Five SV newborns without prior surgical palliation, 8 infants post-Norwood/hybrid procedure, 4 infants post-Glenn, and 1 infant post-Fontan were initially supported with either continuous-flow (n = 13 of 18, 72%) or pulsatile-flow (n = 5 of 18, 28%) devices. Three (17%) of 18 transitioned to another device during support. Before VAD conversion, 9 (50%) of 18 were supported by extracorporeal membrane oxygenation. Outcomes included 7 (39%) of 18 who transplanted, 2 (11%) of 18 who recovered, and 9 (50%) of 18 who died before discharge. Of these deaths, 2 occurred after transplantation and 2 after explantation, and 5 had redirection of care while on support secondary to previously undiagnosed pulmonary venoocclusive disease (n = 2) or severe neurologic events (n = 3). Overall, 6 (33%) of 18 experienced neurologic injury. At last follow-up, 9 (50%) of 18 children were alive (median 1.2 [interquartile range, 0.8-4.3] years postexplantation/transplantation).Our experience shows that SV children, including newborns, can be successfully bridged to desired endpoints with proper patient selection and using specific cannulation strategies. Continuing utilization of this strategy is warranted for future children requiring VAD support.

Lung transplantation is a crucial component in the treatment of end-stage lung disease in infants. Traditionally, most lung transplants have been performed in older children and adults, resulting in a scarcity of data for infant patients. To address the challenges unique to this age group, novel strategies to provide the best preoperative, intraoperative, and postoperative care for these youngest patients are paramount. We review recent advances in bridge-to-transplantation therapy, including the use of a paracorporeal lung assist device, and differences in surgical technique, including bronchial artery revascularization, for incorporation into the overarching treatment strategy for infants undergoing lung transplantation.

While the relationship between maternal connective tissue diseases and neonatal rashes was described in the 1960s and congenital heart block in the 1970s, the "culprit" antibody reactivity to the SSA/Ro-SSB/La ribonucleoprotein complex was not identified until the 1980s. However, studies have shown that approximately 10-15% of cases of congenital heart block are not exposed to anti-SSA/Ro-SSB/La. Whether those cases represent a different disease entity or whether another antibody is associated has yet to be determined. Moreover, the cutaneous manifestations of neonatal lupus have also been identified in infants exposed only to anti-U1RNP antibodies. In this review, we describe what we believe to be the first case of congenital heart block exposed to maternal anti-U1RNP antibodies absent anti-SSA/Ro-SSB/La. The clinical and pathologic characteristics of this fetus are compared to those typically seen associated with SSA/Ro and SSB/La. Current guidelines for fetal surveillance are reviewed and the potential impact conferred by this case is evaluated.

Subcutaneous enoxaparin is the mainstay anticoagulant in critically ill pediatric patients although it poses several challenges in this patient population. Enoxaparin infused IV over 30 minutes represents an attractive alternative, but there is limited experience with this route of administration in children. In this study, we assess dosing, anticoagulation quality, safety, and clinical efficacy of IV enoxaparin compared to subcutaneous enoxaparin in critically ill infants and children.Retrospective single-center study comparing dosing, anticoagulation quality, safety, and clinical efficacy of two different routes of enoxaparin administration (IV vs subcutaneous) in critically ill infants and children. Key outcome measures included dose needed to achieve target antifactor Xa levels, time required to achieve target antifactor Xa levels, proportion of patients achieving target anticoagulation levels on initial dosing, number of dose adjustments, duration spent in the target antifactor Xa range, anticoagulation-related bleeding complications, anticoagulation failure, and radiologic response to anticoagulation.Tertiary care pediatric hospital.All children admitted to the cardiac ICU, PICU, or neonatal ICU who were prescribed enoxaparin between January 2014 and March 2016 were studied.One hundred ten patients were identified who had received IV or subcutaneous enoxaparin and had at least one postadministration peak antifactor Xa level documented.Of the 139 courses of enoxaparin administered, 96 were therapeutic dose courses (40 IV and 56 subcutaneous) and 43 were prophylactic dose courses (20 IV and 23 subcutaneous). Dosing, anticoagulation quality measurements, safety, and clinical efficacy were not significantly different between the two groups.Our study suggests that anticoagulation with IV enoxaparin infused over 30 minutes is a safe and an equally effective alternative to subcutaneous enoxaparin in critically ill infants and children.

Purpose Intraoperative extracorporeal membrane oxygenation (ECMO) as the primary means of tissue oxygenation during pediatric lung transplantation has been increasingly used as an alternative to cardiopulmonary bypass (CPB). Current literature in adults undergoing lung transplantation suggest a benefit of ECMO over CPB. However, there is limited data on the outcomes in children. We sought to determine outcomes of intraoperative ECMO versus CPB during pediatric lung transplantation at our institution. Methods We retrospectively reviewed the records of pediatric patients ( Results Of the 35 children who underwent lung transplantation over this period, 14 (40.0%) were transplanted on ECMO support while 21 (60.0%) were transplanted on CPB. There was no statistically significant difference in mortality at 30 days (0.0% ECMO vs 4.8 % CPB), 6 months (0.0% ECMO vs 11.1% CPB), or at 1 year post-transplant (14.3% ECMO vs 33.3% CPB). Other analyzed variables did not differ significantly (Table 1). Conclusion Although the use of ECMO as a primary means of tissue oxygenation in adult lung transplantation has shown a benefit when compared to CPB, this study shows no difference in outcomes within intraoperative and postoperative variables during pediatric lung transplantation. This data is representative of transplants within a single high-volume institution; data from additional institutions may strengthen these observations.

Background: Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit. Methods: Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children’s National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis. Results: Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support. All patients identified manifested persistent severe bleeding, despite appropriate management of anticoagulation and blood product administration based on the established protocols. Detailed hemostatic testing including quantitative von Willebrand factor (vWF) multimer analysis revealed decreased high-molecular-weight multimers (HMWMs) and absent ultra-HMWM, consistent with AvWS in all patients. Eight patients received treatment with vWF concentrate, one patient with desmopressin, and five recovered without specific treatment. Bleeding ceased in all but one patient. Conclusions: Acquired von Willebrand syndrome is an uncommon but important cause of bleeding in pediatric patients with cardiac disease. A high index of clinical suspicion with knowledge of the characteristic clinical scenario in addition to low levels of vWF multimers is required to manage and diagnose AvWS. Although the optimal management of AvWS in this patient population is unclear, vWF concentrates are available and appear to be efficacious for controlling life-threatening bleeding.

The availability of extracorporeal membrane oxygenation (ECMO) is the standard of care at most high-acuity congenital heart surgery programs. Extracorporeal membrane oxygenation is also an integral part of managing children with in-hospital cardiac arrest refractory to conventional cardiopulmonary resuscitation (extracorporeal cardiopulmonary resuscitation). In this article, we aim to provide a practical guide to initiate rapid ECMO in a nonoperating room setting.

Objective To elicit the perceptions of bedside critical care nurses toward continual in-house attending coverage and its effect on patient safety, communication, and nursing education. Design A 5-point Likert-type questionnaire was designed to evaluate the perception of bedside nurses in the pediatric cardiac intensive care unit (PCICU) toward the presence of a 24 hour in-house attending physician. Setting Single tertiary referral PCICU in Washington, DC Subjects The 46 PCICU nurses who participated in the study were separated into two groups based on exposure to the recent implementation of continual in-house attending coverage at our institution. Group one consisted of 14 nurses with only exposure to the new 24/7 in-house coverage while group two encompassed 32 nurses who had experienced both the new and old system (off-site on-demand attending physician). Measurements and Main Results. Surveys demonstrated that both groups found that the new system has a positive impact on nursing education (median score of 5) as well as a positive impact on the communication between multidisciplinary teams and between care team and families (median score of 5). Nurses who experienced only the new system scored one point lower (median score of 4) regarding the effect of this staffing model on patient outcomes than nurses who had experienced both systems (median score of 5, P = .016). Between 83% and 98% of all 46 nurses who participated indicated they agree or strongly agree with each of the questions regarding the benefit of 24 hour in-house attending coverage. Conclusion Our study suggests that regardless of differences in experience, pediatric cardiac nurses believe the presence of an on-site intensivist to be beneficial to both nursing and patients.

Background Epicardial implantable cardioverter-defibrillator (ICD) placement in infants, children, and patients with complex cardiac anatomy requires an open surgical thoracotomy and is associated with increased pain, longer length of stay, and higher cost. Objective The purpose of this study was to compare an open surgical epicardial placement approach with percutaneous pericardial placement of an ICD lead system in an infant piglet model. Methods Animals underwent either epicardial placement by direct suture fixation through a left thoracotomy or minimally invasive pericardial placement with thoracoscopic visualization. Initial lead testing and defibrillation threshold testing (DFT) were performed. After the 2-week survival period, repeat lead testing and DFT were performed before euthanasia. Results Minimally invasive placement was performed in 8 piglets and open surgical placement in 7 piglets without procedural morbidity or mortality. The mean initial DFT value was 10.5 J (range 3–28 J) in the minimally invasive group and 10.0 J (range 5–35 J) in the open surgical group ( P = .90). After the survival period, the mean DFT value was 12.0 J (range 3–20 J) in the minimally invasive group and 12.3 J (range 3–35 J) in the open surgical group ( P = .95). All lead and shock impedances, R-wave amplitudes, and ventricular pacing thresholds remained stable throughout the survival period. Conclusion Compared with open surgical epicardial ICD lead placement, minimally invasive pericardial placement demonstrates an equivalent ability to effectively defibrillate the heart and has demonstrated similar lead stability. With continued technical development and operator experience, the minimally invasive method may provide a viable alternative to epicardial ICD lead placement in infants, children, and adults at risk of sudden cardiac death.

Background: High-fidelity simulation using patient-specific three-dimensional (3D) models may be effective in facilitating pediatric cardiac intensive care unit (PCICU) provider training for clinical management of congenital cardiac surgery patients. Methods: The 3D-printed heart models were rendered from preoperative cross-sectional cardiac imaging for 10 patients undergoing congenital cardiac surgery. Immediately following surgical repair, a congenital cardiac surgeon and an intensive care physician conducted a simulation training session regarding postoperative care utilizing the patient-specific 3D model for the PCICU team. After the simulation, Likert-type 0 to 10 scale questionnaire assessed participant perception of impact of the training session. Results: Seventy clinicians participated in training sessions, including 22 physicians, 38 nurses, and 10 ancillary care providers. Average response to whether 3D models were more helpful than standard hand off was 8.4 of 10. Questions regarding enhancement of understanding and clinical ability received average responses of 9.0 or greater, and 90% of participants scored 8 of 10 or higher. Nurses scored significantly higher than other clinicians on self-reported familiarity with the surgery (7.1 vs 5.8; P = .04), clinical management ability (8.6 vs 7.7; P = .02), and ability enhancement (9.5 vs 8.7; P = .02). Compared to physicians, nurses and ancillary providers were more likely to consider 3D models more helpful than standard hand off (8.7 vs 7.7; P = .05). Higher case complexity predicted greater enhancement of understanding of surgery ( P = .04). Conclusion: The 3D heart models can be used to enhance congenital cardiac critical care via simulation training of multidisciplinary intensive care teams. Benefit may be dependent on provider type and case complexity.

ObjectiveInjury to the recurrent laryngeal nerve can lead to significant morbidity during congenital cardiac surgery. The objective is to expand on the limited understanding of the severity and recovery of this iatrogenic condition.DesignA six-year retrospective review of all congenital heart operations at a single institution from January 1, 2008 to December 31, 2013 was performed. All patients with documented vocal cord paralysis on laryngoscopic examination comprised the study cohort. Evaluation of time to vocal cord recovery and need for further surgical intervention was the primary focus.ResultsThe incidence of post-operative vocal cord paralysis was 1.1% (32 out of 3036 patients; 95% confidence interval: 0.7–1.5%). The majority were left-sided injuries (71%). Overall rate of recovery was 61% with a median time of 10months in those who recovered, and a total follow up of 46months. Due to feeding complications, 45% of patients required gastrostomy tube after the injury, and these patients were found to have longer duration of post-operative days of intubation (median 10 vs. 5days, p=0.03), ICU length of stay (50 vs. 8days, p=0.002), and hospital length of stay (92 vs. 41days, p=0.01). No pre-operative variables were identified as predictive of recovery or need for gastrostomy placement.ConclusionRecurrent laryngeal nerve injury is a serious complication of congenital heart surgery that impacts post-operative morbidity, in some cases leading to a need for further intervention, in particular, gastrostomy tube placement. A prospective, multi-center study is needed to fully evaluate factors that influence severity and time to recovery.

Background: Postoperative care delivered in the pediatric cardiac intensive care unit (CICU) relies on providers’ understanding of patients’ congenital heart defects (CHDs) and procedure performed. Novel, bedside use of virtual, three-dimensional (3D) heart models creates access to patients’ CHD to improve understanding. This study evaluates the impact of patient-specific virtual 3D heart models on CICU provider attitudes and care delivery. Methods: Virtual 3D heart models were created from standard preoperative cardiac imaging of ten patients with CHD undergoing repair and displayed on a bedside tablet in the CICU. Providers completed a Likert questionnaire evaluating the models’ value in understanding anatomy and improving care delivery. Responses were compared using two-tailed t test and Mann-Whitney U test and were also compared to previously collected CICU provider responses regarding use of printed 3D heart models. Results: Fifty-three clinicians (19 physicians, 34 nurses/trainees) participated; 49 (92%) of 53 and 44 (83%) of 53 reported at least moderate to high satisfaction with the virtual 3D heart’s ability to enhance understanding of anatomy and surgical repair, respectively. Seventy-one percent of participants felt strongly that virtual 3D models improved their ability to manage postoperative problems. The majority of both groups (63% physicians, 53% nurses) felt that virtual 3D heart models improved CICU handoffs. Virtual 3D heart models were as effective as printed models in improving understanding and care delivery, with a noted provider preference for printed 3D heart models. Conclusions: Virtual 3D heart models depicting patient-specific CHDs are perceived to improve understanding and postoperative care delivery in the CICU.

Background Shunt or conduit thrombosis in a single ventricle circuit is a life-threatening complication that requires prompt treatment to rapidly restore shunt/conduit patency. Transcatheter interventions represent an attractive alternative to systemic thrombolysis or open surgical procedures. We report our center's experience with catheter-based approaches in patients with palliated single ventricle who present with shunt/conduit thrombosis. Methods A retrospective review was performed of all patients with palliated single ventricle physiology who were diagnosed over a 5-year period with shunt/conduit thrombosis and received catheter-based interventions. Patients were followed up to hospital discharge. Results Thirteen patients were identified that were diagnosed with thrombosis of a modified Blalock-Taussig shunt (five patients), bidirectional cavopulmonary shunt (one patient), and total cavopulmonary pathway (seven patients). Shunt/conduit thrombosis occurred both early and late after palliation surgery. Catheter-based interventions included balloon angioplasty (one patient), stent implantation (12 patients), and mechanical thrombectomy (one patient). Thrombophilia was identified in seven patients. Technical and clinical success with restoration of normal shunt flow and improvement in clinical status was achieved in 12 patients. Reversible procedure-related complications occurred in three patients with no significant sequelae. Conclusions Our experience suggests that percutaneous catheter-based interventions are safe and effective in managing shunt/conduit thrombosis in infants and children with palliated single ventricle circulation.

Objective Gastrostomy tube (G-tube) placement during three-stage surgical palliation of single-ventricle cardiac physiology has been shown to improve weight gain in this population of infants who often suffer from inadequate feeding. The optimal timing of this intervention is unclear and requires further investigation. Design A retrospective review of all patients who underwent G-tube placement at any stage of surgical palliation of single-ventricle physiology from January 2005 to December 2012 was performed at a single congenital cardiac surgery center. Analysis of weight gain and survival was undertaken by comparing patients who received the G-tube either less than or greater than 90 days after the first surgical stage. Results Fifty-four patients were identified that met the criteria, 26 (48%) of which received the G-tube within 90 days of stage 1, while 28 (52%) patients received the tube at greater than 90 days. Percentage of weight gain at time of discharge from stage 1 was significantly higher for group B (A: median 9.9%, interquartile range [IQR] 4.9–29.8; B: median 29.0%, IQR 16.0–44.3; P = .05). However, total hospital length of stay was decreased for the patients who received G-tubes earlier (A: median 60 days, IQR 35–100; B: median 83, IQR 48–184) as was intensive care unit length of stay (A: median 27 days, IQR 13–69; B: median 48, IQR 16–119) by nearly half, although not statistically significant (P = .47). Survival to time of discharge from stage 1 surgery was not significantly different between earlier tube placements vs. later (92% vs. 100%, respectively; P = .14). Multivariable analysis found inclusion of fundoplication to predict weight gain (P = .006) at time of first discharge. Conclusion Earlier placement of G-tube may increase the rate of recovery from stage 1 of multistage palliative cardiac surgery for single-ventricle physiology. Fundoplication may improve perioperative weight gain when indicated.

Background With the advent of three-dimensional (3D) printers and high-resolution cardiac imaging, rapid prototype constructions of congenital cardiac defects are now possible. Typically, source images for these models derive from higher resolution, cross-sectional cardiac imaging, such as cardiac magnetic resonance imaging or computed tomography. These imaging methods may involve intravenous contrast, sedation, and ionizing radiation. New echocardiographic transducers and advanced software and hardware have optimized 3D echocardiographic images for this purpose. Thus, the objectives of this study were to confirm the feasibility of creating cardiac models from 3D echocardiographic data and to assess accuracy by comparing 3D model measurements with conventional two-dimensional (2D) echocardiographic measurements of cardiac defects. Methods Nine patients undergoing 3D echocardiography were identified (eight with ventricular septal defects, one with three periprosthetic aortic valve leaks). Raw echocardiographic image data were exported anonymously and converted to Digital Imaging and Communications in Medicine format. The image data were filtered for noise reduction, imported into segmentation software to create a 3D digital model, and printed. Measurements of the defects from the 3D model were compared with defect measurements from conventional 2D echocardiographic data. Meticulous care was taken to ensure identical measurement planes. Results Long- and short-axis measurements of eight ventricular septal defects and three perivalvar leaks were obtained. Mean ± SD values for the 3D model measurements and conventional 2D echocardiographic measurements were 7.5 ± 6.3 and 7.1 ± 6.2 mm respectively ( P = .20), indicating no significant differences between the standard 2D and 3D model measurements. The two groups were highly correlated, with a Pearson correlation coefficient of 0.988. The mean absolute error (2D − 3D) for each measurement was 0.4 ± 0.9 mm, indicating accuracy of the 3D model of Conclusions Three-dimensional printed models of echocardiographic data are technically feasible and may accurately reflect ventricular septal defect anatomy. Three-dimensional models derived from 3D echocardiographic data sets represent a new tool in procedural planning for children with congenital heart disease.

Background Extracorporeal membrane oxygenation (ECMO) is a life-saving measure for pediatric patients with cardiopulmonary failure. The option of cannulating neck vessels versus those of the groin exists for patients over 15 kg; however, each carries the risk for complications. We present a single-center experience comparing the risks and benefits of these alternate peripheral ECMO cannulation sites. Methods All pediatric patients supported by venoarterial (VA) ECMO via peripheral cannulation from January 2006 to May 2013 were retrospectively reviewed, excluding those weighing less than 15 kg. We compared complications of neck versus groin cannulation sites, including neurologic dysfunction, limb ischemia, and in-hospital mortality. Univariate analysis and multivariable logistic regression were performed to identify factors for complications including mortality. Results Thirty-six patients (median age 10.8 years, interquartile range: 6.0–15.8) were cannulated for VA ECMO. Forty-four percent were cannulated via neck vessels and 47% were cannulated via groin. Nine percent were cannulated at both sites. Overall survival was 72% at decannulation and 67% at hospital discharge. No statistically significant survival difference between groin and neck cannulation subgroups was found. Rate of neurologic injury was higher in neck (25%) versus groin (12%) cannulation, but this was not statistically significant (p = 0.52). Extremity ischemia occurred in five patients having groin cannulation (29%, p = 0.04). Conclusions Neck cannulation is a valuable alternative to groin vessels in patients above 15 kg. Risk of limb ischemia with groin cannulation is significant, and must be considered when choosing the cannulation site for ECMO support. doi: 10.1111/jocs.12538 (J Card Surg 2015;30:461–465)

There are limited data on the relationship between the administered dose of recombinant factor seven (rFVIIa) and the development of adverse clinical outcomes after congenital heart surgery. This single institution case series reports on dosing, adverse events, and blood product usage after the administration of rFVIIa in the congenital heart surgery patient population. A retrospective review identified 16 consecutive pediatric patients at an academic, free-standing, children’s hospital who received rFVIIa to curtail bleeding following congenital heart surgery between April 2004 and June 2012. Patients were assessed for survival to hospital discharge versus in-hospital mortality and the presence or absence of a major neurological event during inpatient hospitalization. The median age at surgery was 6.8months (range: 3days–42years). Seven patients (44%) survived to hospital discharge and nine patients (56%) died. The cause of mortality included major neurological events (44%), uncontrolled bleeding (33%), and sepsis (23%). Eight patients (50%) required extracorporeal membrane oxygenation support following congenital heart surgery. The median cumulative rFVIIa dose administered was 97mcg/kg, and the median cumulative amount of blood products administered was 452ml/kg. In conclusion, this case series underscores the need to prospectively evaluate the effect that rFVIIa has on patient survival and the incidence of adverse events, including thrombotic and major neurological events, in congenital heart surgery patients. Ideally, a randomized, multicenter study would provide the sufficient numbers of patients and events to test these relationships.

The following report describes the case of newborn girl with an asymptomatic systolic murmur, which on imaging revealed a nearly obstructive mass in the left-ventricular outflow tract. The mass was resected and found to be consistent with a rhabdomyoma. Here, we describe the pathologic and clinical characteristics of this tumor.

Symptoms of airway compression secondary to double aortic arch are relieved by division of one of the two aortic arches. However, in some cases inherent tracheomalacia and other factors may result in persistence of symptoms. We report one such occurrence in our experience and describe the use of aortopexy as a curative procedure to tackle this problem.

Ectopic atrial tachycardia (EAT) is common in surgically repaired congenital heart disease (CHD) and carries the potential for significant hemodynamic compromise. Our objective was to determine the incidence, and risk factors of EAT after CHD surgery. Prospective study of patients that underwent surgery for CHD from February to October 2016 was performed. Demographic, perioperative and electrophysiologic data were collected. Sustained EAT (> 30 s) was documented by telemetry or electrocardiogram and confirmed by a pediatric electrophysiologist. All patients were followed through index hospitalization. During the study period, 17/204 (8%) of patients developed EAT with median time-to-event of 14 days. 15/17 (88%) received anti-arrhythmic therapy for sustained EAT. By univariate analysis, younger age (5 vs. 284 days, P

Uhl’s anomaly, first reported in 1952, is an extremely rare congenital cardiac defect characterized by partial or complete loss of the right ventricular myocardium and unknown etiology. Fewer than 100 cases have been described. The response to medical management is poor and there is no known ideal surgical approach or timing for treatment. We report the case of a previously active adolescent male presenting with cardiac arrest, who underwent successful bidirectional cavopulmonary anastomosis (“Glenn” anastomosis) with right atrial reduction and right ventricular free wall plication.

Background Patent ductus arteriosus (PDA) is a common complication of prematurity and a risk factor for poor outcome. Infants undergoing surgical PDA ligation are at highest risk for neurodevelopmental injury. Autonomic dysfunction has been described in premature infants with PDA. Aim To interrogate the autonomic nervous system by analysis of advanced heart rate variability (HRV) metrics before and after surgical closure of the PDA. Study Design Prospective, observational study. Subjects Twenty-seven infants born before 28 weeks' gestation were included in this study. Methods Continuous electrocardiogram data were sampled at a rate of 125 Hz for a total of 6 hours before and 6 hours after 30 hours of surgical closure. HRV was determined by detrended fluctuation analysis to calculate the short and long root mean square (RMSL and RMSS) and α components at two time scales (long and short). Results Gestational age (GA) was positively associated with RMSL, RMSS, and αS and was negatively associated with αL. There was no difference between RMSs, RMSL, αS, or αL before and after surgery; however, median heart rate was lower after surgery (p Conclusion Advancing GA is highly associated with increasing HRV; however, surgical ligation does not affect HRV in the postoperative period.

Background: Congenital ion channel disorders, including congenital long QT syndrome (LQTS), cause significant morbidity in pediatric patients. When medication therapy does not control symptoms or arrhythmias, more invasive treatment strategies may be necessary. This study examines our institution’s clinical experience with surgical cardiac denervation therapy for management of these arrhythmogenic disorders in children. Methods: An institutional review board–approved retrospective review identified ten pediatric patients with congenital ion channelopathies who underwent surgical cardiac denervation therapy at a single institution between May 2011 and April 2014. Eight patients had a diagnosis of congenital LQTS, two patients were diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT). All patients underwent sympathectomy and partial stellate ganglionectomy via video-assisted thoracoscopic surgery (VATS). Results: Six of the ten patients had documented ventricular arrhythmias preoperatively, and 70% of the patients had preoperative syncope. The corrected QT interval decreased in 75% of patients with LQTS following sympathectomy. Postoperative arrhythmogenic symptoms were absent in 88% of congenital LQTS patients, but both patients with CPVT continued to have symptoms throughout the duration of follow-up. All patients were alive after a median follow-up period of 10 months. Conclusions: Surgical cardiac denervation therapy via VATS is a useful treatment strategy for congenital LQTS patients who fail medical management, and its potential benefit in the management of CPVT is unclear. A prospective comparison of the efficacy of surgical cardiac denervation therapy and implantable cardioverter-defibrillator use in congenital ion channelopathies is timely and crucial.

Background Extracorporeal membrane oxygenation (ECMO) support is often required in the management of perioperative congenital heart surgery (CHS) patients. However, 24-hour in-hospital congenital cardiac surgical coverage (24-CCSC) is not available at all institutions. The purpose of this study is to evaluate the effect of 24-CCSC on perioperative ECMO outcomes in CHS patients. Methods An institutional review board approved, retrospective review of 128 perioperative CHS ECMO patients at a single, quaternary care children's hospital between January 2003 and December 2012 was performed. Primary endpoints evaluated were mortality in children supported with ECMO after undergoing cardiac surgery and ECMO-related morbidity after initiation of 24-CCSC with advanced congenital cardiac surgical fellows. Patients were divided into 2 groups based on whether 24-CCSC was absent (cohort 1: January 2003 to July 2007) or present (cohort 2: August 2007 to December 2012) at the time of ECMO management. Results The surgical procedures performed were similar in both cohorts based on STAT Mortality Categories (5 Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Categories). The overall mortality rate in children supported with ECMO after undergoing cardiac surgery was 53%. This mortality was significantly reduced from 68% to 43% ( p = 0.007) with 24-CCSC. Multivariate logistic regression analysis revealed that 24-CCSC ( p = 0.009) and lower STAT Mortality Category ( p = 0.042) were independent predictors of operative survival. Cardiac arrhythmias (36% to 16%; p = 0.012) and pulmonary complications (32% to 8%; p Conclusions The presence of 24-CCSC significantly decreased the rate of mortality in children supported with ECMO after undergoing cardiac surgery, as well as cardiac arrhythmias and pulmonary complications for perioperative CHS patients receiving ECMO support. This study demonstrates that CHS programs would benefit from 24-CCSC in the care of this critically ill patient population.

To determine if development of an extracorporeal cardiopulmonary resuscitation simulation program reduced extracorporeal cardiopulmonary resuscitation times in real patients: Before-after study.Twenty-six bed pediatric cardiac ICU in a tertiary urban hospital.Forty-three cardiac patients (aged 1 d to 16 yr) who received extracorporeal cardiopulmonary resuscitation.An interdisciplinary team collaborated to define the roles and clarify responsibilities of each individual involved in extracorporeal cardiopulmonary resuscitation. An "ideal rapid deployment" was defined and tested using simulation sessions. This included a task analysis, role creation, and multidisciplinary simulations, including structured debriefings and video review and the creation of a master checklist.There were a total of 43 episodes of extracorporeal cardiopulmonary resuscitation during the study period, 16 (37%) of which occurred during the preintervention time period (from February 2009 to March 2010) and 27 (63%) during the postintervention time period (April 2010 to March 2013). The median deployment time in the preintervention time period was 51 minutes (interquartile range, 43-62 min), whereas the median deployment time in the postintervention time period was 40 minutes (interquartile range, 23-52 min) (p = 0.018).There are no standard guidelines of how a team should coordinate the efforts of nursing, physicians, extracorporeal membrane oxygenation specialists, surgeons, respiratory therapists, patient care technicians, and unit clerks to emergently execute this complex procedure. Because time is of the essence, it is essential to develop a highly functioning and well-coordinated team with a standardized method of the procedure, its documentation, and communication. Simulation accomplished this for our program. Following these simulation exercises, not only was there a subjectively observed improved coordination and smoother deployment of extracorporeal membrane oxygenation in real-life extracorporeal cardiopulmonary resuscitation, but we have also demonstrated a significantly faster deployment of extracorporeal membrane oxygenation as compared with the presimulation era.

Surgical repair of congenital heart disease during cardiopulmonary bypass is common, and performing these complicated procedures in the absence of blood transfusions is especially challenging. A case of a Jehovah's Witness child who underwent surgical repair of a ventricular septal defect utilizing a new tetrastarch for autologous normovolemic hemodilution is reported. A successful operative repair was achieved without the need for non-autologous blood transfusion.

Introduction Following congenital heart surgery, pediatric patients may experience persistent respiratory failure that requires tracheostomy placement. Currently, definitive knowledge of the optimal timing for tracheostomy placement in this patient population is lacking. Methods An 8-year retrospective review of 17 pediatric patients who underwent congenital heart surgery and subsequently required tracheostomy placement was performed. Patients were evaluated with regard to the timing of tracheostomy and mortality. Results The overall study mortality was 24%. The median duration of intubation prior to tracheostomy was 60 days (interquartile range: 19–90 days); there was no difference in the average time between intubation and tracheostomy for survivors compared with nonsurvivors (51 vs. 73 days, P = .37). No difference was observed in the overall duration of positive pressure ventilation when tracheostomy was performed within 30 days of intubation compared with greater than 30 days following intubation (481 vs. 451 days, P = .88). Overall, 18% of patients were successfully weaned from the ventilator after a median duration of positive pressure ventilation of 212 days. Conclusion The timing of tracheostomy placement may be an important factor in clinical outcomes for pediatric patients with persistent dependence on mechanical ventilatory support following congenital heart surgery. A larger, multi-institution study may help further elucidate our observed clinical findings in this patient population.

Background: Although there are considerable data regarding in-hospital results of congenital heart surgery (CHS) patients requiring postoperative extracorporeal membrane oxygenation (ECMO) support, there is limited information on intermediate-term outcomes. Methods: A single-institution retrospective review of 25 consecutive postoperative CHS patients who required ECMO and survived to hospital discharge between January 2003 and June 2008. Survival was estimated by the Kaplan-Meier method. Results: At a median follow-up of 3.3 years (interquartile range: 1.2-5.9 years), there was one death which occurred at six months postsurgery. Kaplan-Meier-estimated survival at three years was 95% (95% confidence interval: 90%-100%). Indications for ECMO included extracorporeal cardiopulmonary resuscitation (48%), systemic hypoxia (4%), postoperative low-cardiac output syndrome (28%), and intraoperative failure to wean off cardiopulmonary bypass (20%). Following ECMO support, 65% of patients had unplanned cardiac reinterventions (three requiring operative interventions, six requiring percutaneous interventions, and four requiring both), and 47% of patients required unplanned hospitalizations. In all, 29% of patients developed neurological deficits and 12% of patients developed chronic respiratory failure. No patients developed renal failure. Overall, systemic ventricular (SV) function normalized in 83% of patients, whereas 17% of patients had persistent mild-to-moderate SV dysfunction. Conclusions: Intermediate-term patient survival of ECMO following CHS is encouraging. However, neurological impairment and unplanned cardiac reinterventions remain significant concerns. Further delineation of risk factors to improve patient outcomes is warranted.

The use of extracorporeal membrane oxygenation (ECMO) in terminally ill pediatric patients who are not candidates for long-term mechanical circulatory support or heart transplantation requires careful deliberation. We present the case of a 16-year-old female with a relapse of acute lymphoid leukemia and acute-on-chronic cardiomyopathy who received short-term ECMO therapy. In addition, we highlight several ethical considerations that were crucial to this patient’s family-centered care and demonstrate that this therapy can be accomplished in a manner that respects patient autonomy and family wishes.

Purpose Lung re-transplantation (re-LTx) has increased in the last decade coinciding with implementation of the lung allocation score (LAS) and better survival outcomes in first time re-LTx patients. LAS adoption in 2005 likely led to a new patient population undergoing second re-LTx (three donor lungs in a lifetime). We performed the first study of second time re-LTx patients to characterize this unique population, describe factors influencing survival and compare outcomes with first time re-LTx. Methods The United Network for Organ Sharing database (1985-2018) was queried for second time re-LTx patients and control matched 1:3 with first time re-LTx patients by age, transplant year and diagnosis at first LTx. Descriptive analysis, Cox proportional hazards regression modeling and Kaplan-Meier survival analysis were performed, with death as the primary endpoint. Results Our cohort had 25 second time re-LTx patients with 15 male (60%) averaging 34.91±15.91 yrs. Cohort primarily consisted of CF (40%), Idiopathic Pulmonary Fibrosis (20%) and COPD (8%). Significantly reduced survival was found between second time re-LTx and controls (47% vs 67.4% at 1 year; 60% vs 42% at 2 years; 8.5% vs 34.1% at 5 years, p=0.015). Univariate Cox proportional hazard modeling indicated successive bilateral LTx (HR=0.362, p=0.057) improves survival while female donors decreased survival (HR=4.701, p=0.004). No difference was observed from bilateral LTx in the multivariate model (p=0.232), but female donor hazard remained significantly higher (HR=3.615, p=0.025). Female donors showed worse survival (median=4.9 months) compared to the male donors (median= 29.13 months) in second time re-LTx. Leading causes of death in the cohort were infection-related (28%), respiratory failure (12%) and graft failure-related (12%). Matched controls showed graft failure and infection related causes equally (16.5%). Conclusion Second time re-LTx patients show worse survival than matched first time re-LTx patients when age, LTx year and diagnosis). Second time re-LTx patients have grown rapidly since 2007, 80% of whom emerged in the last 11 years. Decreased survival in second time re-LTx and from female donors warrants further study on donor and recipient selection in second time re-LTx patients.

BACKGROUND Following second heart transplantation (HTx), some patients experience graft failure and require third-time heart transplantation. Little data exist to guide decision-making with regard to repeat retransplantation in older patients. METHODS We performed a retrospective cohort analysis of patients receiving a third HTx, as identified in the United Network for Organ Sharing (UNOS) database from 1985 to 2017. RESULTS The study cohort consisted of N = 60 patients, with an average age of 29 with a standard deviation of ±18 years. Overall survival for the cohort at 1, 5, and 10 years is 83%, 64%, and 44%, respectively. The rate of third-time HTxs has steadily increased in all age groups. Patients older than 50 years now account for 18.3% of all third-time HTxs. Although this group demonstrated longer average previous graft survival, after third HTx they demonstrate significantly poorer survival outcomes compared to third-time HTx recipients younger than 21 (P = 0.05). Age over 50, BMI over 30, and diabetes were all found to be independent risk factors for decreased survival following third HTx. CONCLUSIONS We describe trends in patients undergoing third HTx. We highlight subsets of such recipients who exhibit decreased survival.

Idiopathic pulmonary hemosiderosis is a rare disease defined by the triad of iron deficiency anemia, hemoptysis, and diffuse pulmonary infiltrates on chest radiograph. Idiopathic pulmonary hemosiderosis is known to cause dyspnea and, in some cases, acute onset of massive pulmonary hemorrhage which is traditionally treated with conventional mechanical ventilation or high-frequency oscillation in conjunction with immunosuppressive therapy. In this case report, we describe a 5-week-old infant presenting with hemoptysis, massive pulmonary hemorrhage, and significant hypercapnic respiratory failure. The patient failed conventional ventilation but responded well to extracorporeal life support that was initiated early in his course. Idiopathic pulmonary hemosiderosis was suspected in light of his response to high-dose steroids and was confirmed by subsequent lung biopsies. Conclusion: Patients with severe pulmonary hemorrhage secondary to idiopathic pulmonary hemosiderosis can be safely supported with extracorporeal life support when conventional therapies have been exhausted.

A full-term male neonate presented with cyanosis upon delivery and was subsequently diagnosed with d-transposition of the great arteries, ventricular septal defect, and restrictive atrial septal defect. Following initiation of intravenous prostaglandins and balloon atrial septostomy, an arterial switch operation was performed on day 3 of life. The postoperative course was complicated by intractable ventricular tachycardia that was refractory to lidocaine, amiodarone, esmolol, fosphenytoin, and mexiletine drug therapy. Ventricular tachycardia was suppressed with overdrive atrial pacing but recurred upon discontinuation. Seven weeks postoperatively, radiofrequency catheter ablation was performed due to hemodynamically compromising persistent ventricular tachycardia refractory to medical therapy. The ventricular tachycardia was localized to the inferior-lateral right ventricular outlet septum. The procedure was successful without complications or recurrence. Antiarrhythmics were discontinued after the ablation procedure. Seven days after the ablation, a different, slower fascicular rhythm was noted to compete with the infant's sinus rhythm. This was consistent with the preablation amiodarone having reached subtherapeutic levels given its very long half-life. The patient was restarted on oral beta blockers and amiodarone. The patient was subsequently discharged home in predominantly sinus rhythm with intermittent fascicular rhythm.

Background The IL-17 axis is implicated in pathogenesis of chronic rejection after human lung transplantation. Using a murine model of obliterative airway disease (OAD), we recently demonstrated that Abs to MHC class I antigens can induce immune responses to self-antigens that contributes to immunopathogenesis of chronic rejection. Using a murine model of OAD, we determined the role of IL-17 family members in induction of autoimmunity leading to OAD after ligation of MHC class I. Methods Anti-MHC class I or control antibodies (Abs) were administered intrabronchially to wild-type (WT) and IL-17a knock out (IL-17A-/-) C57BL/6. Results By day 30, anti-MHC I administered endobronchially in IL-17A-/- mice demonstrated significant reduction in cellular infiltration, a 36.8% reduction in CD4 T cells, 62.7% in CD11b macrophages, 37.5% in degree of fibrosis, 1.94 fold and 2.17 fold decrease in anti-KAT and anti-Col-V, respectively, when compared with wild-type mice. Analysis of lung infiltrating cells in anti-MHC I WT revealed increase in IL-17A (KAT:92+21,Col-V:103+19spm) and IL-17F (KAT:5.03%,Col-V:2.75%) secreting CD4+ T cells. However, administration of anti-MHC I in IL-17A-/- demonstrated increase only in IL-17F for KAT (13.70%) and Col-V (7.08%). Anti-IL-17(A-F) mAb administration after anti-MHC I abrogated OAD in both WT and IL-17A-/-. Conclusion Our findings indicate that IL-17A and IL-17F secreted by CD4+Th17 cells specific to lung self-antigens are critical mediators of autoimmunity leading to the pathogenesis of OAD.

To elicit the perceptions of bedside critical care nurses toward continual in-house attending coverage and its effect on patient safety, communication, and nursing education.A 5-point Likert-type questionnaire was designed to evaluate the perception of bedside nurses in the pediatric cardiac intensive care unit (PCICU) toward the presence of a 24 hour in-house attending physician.Single tertiary referral PCICU in Washington, DC SUBJECTS: The 46 PCICU nurses who participated in the study were separated into two groups based on exposure to the recent implementation of continual in-house attending coverage at our institution. Group one consisted of 14 nurses with only exposure to the new 24/7 in-house coverage while group two encompassed 32 nurses who had experienced both the new and old system (off-site on-demand attending physician).Surveys demonstrated that both groups found that the new system has a positive impact on nursing education (median score of 5) as well as a positive impact on the communication between multidisciplinary teams and between care team and families (median score of 5). Nurses who experienced only the new system scored one point lower (median score of 4) regarding the effect of this staffing model on patient outcomes than nurses who had experienced both systems (median score of 5, P = .016). Between 83% and 98% of all 46 nurses who participated indicated they agree or strongly agree with each of the questions regarding the benefit of 24 hour in-house attending coverage.Our study suggests that regardless of differences in experience, pediatric cardiac nurses believe the presence of an on-site intensivist to be beneficial to both nursing and patients.

Kaposiform hemangioendothelioma (KH) is a rare tumor of vascular origin that commonly affects the cutaneous tissues of the extremities. It can, however, affect the abdomen, thorax, head, or neck. Compared with juvenile hemangiomas, which tend to regress, KH tumors are locally aggressive and usually persist. Associated morbidity and mortality rates range from 12 to 30 % and typically are related to either compressive effects on surrounding vital structures or effects of the Kasabach-Merritt phenomenon [10, 11, 13]. To our knowledge, this report is the first to describe KH presenting as a primary intracardiac tumor.

BACKGROUND Presence of donor-specific antibodies (Abs) is detrimental to posttransplant allograft function. Some sensitized recipients have successfully undergone transplantation after pretransplant conditioning regimen using plasmapheresis and/or intravenous immunoglobulin therapy, but underlying mechanisms that confer such allograft protection are undefined. METHODS We developed a single human leukocyte antigen (HLA)-mismatched heterotopic murine heart transplant model (HLA-A2 into HLA-A2-sensitized-C57BL/6) to determine whether pretreatment of donors with low concentration of HLA class I (W6/32) or control Ab (C1.18.4) will confer protection. Expression levels of survival genes, Bcl-2 and heme oxygenase-1, were analyzed by gene array analysis and quantitative real-time polymerase chain reaction. Expression levels of cytokine panel were analyzed by Luminex. Role of Bcl-2 in the induction of allograft protection was analyzed by silencing the Bcl-2 expression in the donor hearts using a small hairpin (shRNA) specific for Bcl-2. RESULTS Control Ab-pretreated hearts were rejected in less than 5 days demonstrating hemorrhage, Ab, and C4 deposition. In contrast, W6/32-pretreated hearts were rejected at 15 days (P