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Background: Bidirectional Glenn shunt (BDG) failure carries high morbidity and mortality but the clinical factors associated with failure and the optimal management strategy are understudied. Methods: A total of 217 patients undergoing BDG at our institution between 1989 and 2020 were retrospectively reviewed and categorized as success or failure. Failure was defined as the need for reoperation (BDG takedown, reoperation for correction of cardiac defect, and/or transplantation) at any time postoperatively; operative mortality (death attributable to BDG malfunction occurring during the index hospitalization for BDG or within 30 days of discharge); or late mortality (death directly attributable to BDG malfunction occurring prior to Fontan or next-stage palliation). Univariate and binary logistic regression analyses were performed. Results: BDG failure occurred in 14 (6.5%) patients. Univariate predictors were: hypoplastic left heart syndrome ( P  = .037), right ventricular (RV) dominance ( P  = .010), greater pre-BDG pulmonary vascular resistance (PVR) ( P  = .012), concomitant atrioventricular valve repair ( P  = .020), prolonged pleural drainage ( P  = .001), intensive care unit ( P <.001) and hospital ( P  = .002) stays, and extracorporeal membrane oxygenation (ECMO) requirement ( P <.001). Multivariate predictors were: RV dominance ( P  = .002), greater PVR ( P  = .041), ICU ( P <.001) and hospital ( P  = .020) stays, and need for ECMO ( P <.001). As many as 10 of 14 (71%) patients with BDG failure died. Reoperation was performed for 10 patients with BDG failure. Five reoperation patients survived until discharge, with four patients alive at last follow-up (mean 7.9 years). Survivors underwent reoperation earlier than nonsurvivors (36 vs. 94 days). Conclusions: BDG failure carries high mortality, but preoperative predictors and postoperative indicators of failure exist. Early BDG takedown and insertion of aorta-pulmonary shunt may allow survival.

The objective of this study was to determine medium-term morbidity and mortality of patients who have undergone device closure of an extracardiac Fontan fenestration with an Amplatzer Vascular Plug II (AVPII) or Septal Occluder (ASO). A secondary objective was to compare medium-term morbidity and mortality between these patients and other fenestrated Fontan patients. A retrospective chart review was performed on patients who underwent an extracardiac fenestrated Fontan procedure between 1992 and 2015 at Cardinal Glennon Children's Medical Center. Procedural and follow-up data were obtained and compared between those who underwent fenestration closure and those who did not. Additional outcome measures included whether the fenestration had spontaneously closed, morbidity and mortality, oxygen saturations, and hemodynamics pre- and post-closure. Fifty-nine of 118 patients (50%) with a fenestrated Fontan underwent 60 device closures of the fenestration. Thirty-two (53%) of these were with the AVPII and 28 (47%) with the ASO. There was one device embolization. At a median follow-up of 3.9 years, five patients suffered morbidity, including 2 with arrhythmias, 1 with plastic bronchitis, 1 with protein losing enteropathy, and 1 with stroke. There were no cardiopulmonary deaths in this group. Twenty-three of 118 patients (19%) had spontaneous closure. There was no difference in morbidity and mortality between patients who underwent percutaneous fenestration closure and those who either had spontaneous closure or a persistently patent fenestration. Device closure of Fontan fenestrations is a safe and effective procedure with minimal morbidity and mortality comparable to other patients with fenestrations.

An increased incidence of CHD has been noted in twin gestations and in infants conceived using assisted reproductive technologies. However, CHD in these populations remains understudied and the mechanisms underlying these phenomena remain unclear. We present the case of twins conceived via in vitro fertilisation both with Tetralogy of Fallot and additional cardiac and extracardiac malformations.

We describe a case of type A aortic dissection in a 25-year-old woman who had undergone a Ross procedure for aortic valve endocarditis 13 years previously. She was pregnant and noted to have significant enlargement of the aortic root during the latter portion of the third trimester of her pregnancy. An echocardiogram after delivery demonstrated new aortic valve insufficiency in addition to an aortic root diameter of 6.5 cm. A computed tomography angiogram demonstrated a type A aortic dissection involving only the very proximal portion of the ascending aorta. At operation, the dissection was found to be limited to the pulmonary autograft. This was repaired using a valve-sparing technique. Her postoperative course was uneventful, and the aortic valve has shown only trace insufficiency at 3 years of follow-up., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Goel N, Huddleston CB, Fiore AC. A novel mutation of the MYH7 gene in a patient with hypertrophic cardiomyopathy. Turk J Pediatr 2018; 60: 315-318. Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterized by asymmetric cardiac hypertrophy due to inherited mutations in genes that encode sarcomeric proteins. MYH7, which encodes β-myosin heavy chain, is among the most commonly mutated genes in patients affected by HCM. We aimed to identify the specific mutation responsible for HCM in a six-month old Caucasian patient. NextGen DNA sequencing revealed a novel p.Ala1328Thr (A1328T) mutation of MYH7 in the affected patient as well as his asymptomatic father and asymptomatic brother. The clinical details of this mutation are described for the first time in this report. The genetic variant affects a residue that is highly conserved across species. Theoretical analysis suggests that A1328T is very likely deleterious to β-myosin heavy chain protein structure and function. Furthermore, this novel mutation was not observed with any significant frequency in approximately 6,500 healthy individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, underlining the potential pathogenicity of this variant.

Patients with childhood heart disease are living longer and entering adulthood, and may undergo implantable cardioverter-defibrillator (ICD) implantation to reduce the risk of sudden death. We evaluated the characteristics of adult patients with congenital heart disease or left ventricular noncompaction cardiomyopathy (LVNC) in the National Cardiovascular Disease Registry ICD Registry and determined ICD-related in-hospital complications. Patients with LVNC or transposition of the great arteries, tetralogy of Fallot, Ebstein's anomaly, atrial septal defect, ventricular septal defect, or common ventricle were identified in the registry. In-hospital complications were compared among different diagnoses using the chi-square test for categorical variables and the F-test in analyses of variance for continuous variables. A total of 3,077 patients were identified. The mean age was 48.0 ± 16.0 years, and 39.9% were female. Single-chamber ICDs were implanted in 25.2%, dual chamber in 41.9%, and cardiac resynchronization in 30.8%. Intraprocedural or postprocedural complications occurred in 70 patients (2.3%); there were 6 in-hospital deaths (0.2%). The most frequent complications were acute lead dislodgments, pneumothorax, and hematomas. Patients with Ebstein's anomaly had the greatest complication rate (8.3%, p = 0.03). The complication rate was 1.55% in single-chamber devices, 1.86% in dual chamber, and 3.5% in cardiac resynchronization (p < 0.001). For initial implants, the complication rate was 2.55%, 1.62% in generator replacements, and 8.77% in lead revisions (p = 0.001). In conclusion, in this large contemporary adult cohort of congenital heart disease and LVNC patients who underwent ICD implant procedures, periprocedural complication rates were low. Lead-related risks predominated., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Heart transplantation in children with intellectual disability (ID) is an issue of debate due to the shortage of available donor organs. We sought to perform the first large-scale retrospective cohort study describing the prevalence and outcomes of heart transplantation in this population. The United Network of Organ Sharing database was queried from 2008 to 2015 for pediatric patients (age <19 years) receiving first, isolated heart transplant. Recipients were divided into three subgroups: definite ID, probable ID, and no ID. The chi-square test was used to compare patients' baseline characteristics. Kaplan-Meier and Cox proportional hazard regression analyses were used to estimate the association between ID and death-censored graft failure and patient survival. Over the study period, 565 pediatric patients with definite (131) or probable (434) ID received first heart transplant, accounting for 22.4% of all first pediatric heart transplants (n=2524). Recipients with definite ID did not significantly differ from those without ID in terms of gender, ethnicity, ischemia time, severity of pretransplant condition (waitlist status, mechanical ventilation, inotrope dependence, ECMO, VAD, PVRI, infection prior to transplant), or incidents of acute rejection within the first year. ID was associated with prolonged waitlist time (P<.001). Graft and patient survival at 3 years was equivalent between children with and without ID (P=.811 and .578, respectively). We conclude that intellectual disability is prevalent in children receiving heart transplants, with 22.4% of recipients over the study period having definite or probable ID. ID does not appear to negatively affect transplantation outcomes. Future studies are needed to assess long-term outcomes of transplantation in this population., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

The management of decompensating critically ill children with severe PH is extremely challenging and requires a multidisciplinary approach. Unfortunately, even with optimal care, these children might continue to deteriorate and develop inadequate systemic perfusion and at times cardiac arrest secondary to a pulmonary hypertensive crisis. Tools to support these children are limited, and at times, the team should proceed with offering extracorporeal support, especially in newly diagnosed patients who have not benefitted from medical therapy prior to their acute deterioration, in patients with severe pulmonary venous disease and in patients with alveolar capillary dysplasia. Currently, the only approved mode for extracorporeal support in pediatric patients with PH eligible for lung transplantation is ECMO. To decrease the risks associated with ECMO, and offer potential for increased duration of support, extubation, and rehabilitation, we transitioned four small children with refractory PH from ECMO to a device comprising an oxygenator interposed between the PA and LA. This work describes in great detail our experience with this mode of support with emphasis on exclusion criteria, the implantation procedure, and the post-implantation management., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Heart-lung transplantation itself is not a particularly difficult operation technically. It is the setting in which this procedure is performed which is difficult. The three issues of importance in a successful outcome are appropriate harvest of the heart-lung bloc from the donor, careful explant of the heart and lungs of the recipient, and finally the implant of the heart-lung bloc into the recipient. None of this requires extraordinary technical skill, but does require careful coordination and planning as well as adhering to some fundamental principles. One of the major pitfalls encountered is bleeding related to the explant procedure. Another is graft failure related to harvest and/or the implant procedure. The third is injury to either the phrenic nerve(s) or the left recurrent laryngeal nerve related to the explant procedure. Heart-lung transplantation is a major investment in resources of all sorts including financial, personnel, as well as the organs themselves. It is absolutely imperative that this procedure be performed only by experienced surgeons in centers with established expertise.

Left ventricle to aortic conduits were used for the treatment of complex left ventricular outflow tract obstruction in the pediatric population in the mid-1970s. Although this technique has been largely replaced by the Ross-Konno procedure, many patients still have functioning apicoaortic conduits in place today. Few clinical reports or case series exist in pediatric cohorts documenting the natural history or potential long-term complications of this prosthesis. In this report, we describe our experience managing a patient with Shone's syndrome and an apical aortic porcine-valved conduit remnant that became infected 17 years postconduit valve excision for valvular insufficiency., (© The Author(s) 2014.)

Objective: Two different strategies have emerged in the initial palliation for hypoplastic left heart syndrome, the conventional Norwood operation and the so-called hybrid procedure. We have used each of these at our center. The purpose of the present study was to compare the outcomes of both procedures., Methods: From 2007 to 2012, 40 patients presented to the Cardinal Glennon Children's Medical Center with hypoplastic left heart syndrome or 1 of its variants. Of the 40 patients, 24 underwent a hybrid procedure and 16 a Norwood procedure for initial palliation. The medical records, echocardiograms, and cardiac catheterization data were retrospectively reviewed. Standard statistical analysis was performed., Results: The patients who underwent the hybrid procedure weighed less than those who underwent the Norwood procedure. Overall unadjusted survival was better in the Norwood group, although this did not reach statistical significance. Overall hospital resource usage was similar in both cohorts, taking into account both first and second palliation stages., Conclusions: In our review, we found no statistically significant difference in survival or resource usage between those patients undergoing the Norwood procedure and those undergoing a hybrid procedure as initial palliation for hypoplastic left heart syndrome., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: The purpose of this study is to compare morbidity and mortality between fenestrated (F, 61 patients) and nonfenestrated (NF, 54 patients) extracardiac Fontan patients during two eras from July 1995 to December 2010: era 1(1995 to 2004) and era 2 (2005 to 2010)., Methods: Variables evaluated included morphology, hemodynamics, chest tube volume and duration, intensive care and hospital stay, oxygen saturation, neurologic events, rhythm, and readmissions for chylous effusions. Follow-up in 114 hospital survivors was longer in the nonfenestrated cohort (F, 5.0 ± 3.3 years; NF, 7.1 ± 4.6 years; p < 0.005)., Results: Cohorts were similar in body size, morphology, and hemodynamics. Fenestration in hypoplastic left heart syndrome was appreciatively higher in era 2. Bypass time (F, 69 ± 27 minutes; NF, 57 ± 21 minutes) and conduit size (F, 18.8 mm; NF, 19.1 mm) were similar. There was 1 early nonfenestrated Fontan death (1 of 54; 2%) and 4 late deaths (F, 2 of 61, 5%; NF, 2 of 53, 4%; p = 0.86). Room air saturation was higher in NF patients (F, 89%; NF, 94%; p < 0.05). Total chest tube volume was similar, but fenestration was associated with greater chest tube drainage among hypoplastic left heart patients (HLHS, 5,582 ± 3,286 mL; non-HLHS, 3,405 ± 2,533 mL; p = 0.06; odds ratio; 2.0). Readmission to treat chylous effusions, loss of sinus rhythm, actuarial freedom from death, all neurologic events, pacemaker insertion, and Fontan takedown were similar in both cohorts., Conclusions: Fenestration was associated with lower discharge oxygen saturations, but late outcomes in fenestrated and nonfenestrated patients are equivalent., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Primary graft failure is the major cause of mortality in infant HTx. The aim of this study was to characterize the indication and outcomes of infants requiring ECMO support due to primary graft failure after HTx. We performed a retrospective review of all infants (<1 yr) who underwent Htx from three institutions. From 1999 to 2008, 92 infants (<1 yr) received Htx. Sixteen children (17%) required ECMO after Htx due to low cardiac output syndrome. Eleven (69%) infants were successfully weaned off ECMO, and 9 (56%) infants were discharged with a mean follow-up of 2.3 ± 2.5 yr. Mean duration of ECMO in survivors was 5.4 days (2-7 days) compared with eight days (2-10 days) in non-survivors (p = NS). The five-yr survival rate for all patients was 75%; however, the five-yr survival rate was 40% in the ECMO cohort vs. 80% in the non-ECMO cohort (p = 0.0001). Graft function within one month post-Htx was similar and normal between ECMO and non-ECMO groups (shortening fraction = 42 ± 3 vs. 40 ± 2, p = NS). For infants, ECMO support for primary graft failure had a lower short-term and long-term survival rate vs. non-ECMO patients. Duration of ECMO did not adversely impact graft function and is an acceptable therapy for infants after HTx for low cardiac output syndrome., (© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: To evaluate paracorporeal lung assist devices to treat neonates and children with decompensated respiratory failure as a bridge to recovery or lung transplantation., Methods: One neonate (23 days old) and 3 young children (aged 2, 9, and 23 months) presented with primary lung disease with pulmonary hypertension, including alveolar capillary dysplasia in 2 and right pulmonary hypoplasia and primary pulmonary hypertension in 1. The patients were listed for lung transplantation but decompensated and required extracorporeal membrane oxygenation (ECMO). The patients were transitioned from ECMO to a pumpless paracorporeal lung assist device (Maquet Quadrox-iD oxygenator in 3, Novalung in 1) with inflow from the pulmonary artery and return to the left atrium., Results: The patients were weaned from ECMO and supported by the device for 44 ± 29 days (range, 5-74). Three patients were extubated while supported by the device (after 9, 15, and 72 days). One patient was bridged to lung transplant (9 months old, with alveolar capillary dysplasia, supported 5 days). One patient was bridged to recovery with maximal medical therapy (23 months old, with primary pulmonary hypertension, supported 23 days). Two patients died while awaiting a suitable lung donor after a support time of 54 and 72 days., Conclusions: Pediatric patients bridged from ECMO to lung transplantation have poor results. An alternative method for longer term respiratory support was necessary as a bridge for these patients. The use of a paracorporeal lung assist device successfully supported 4 patients to recovery, lung transplantation, or past the average wait time for pediatric donor lungs (27 days). This therapy has the potential to bridge children with decompensated respiratory failure to lung transplantation., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Cardiac surgery requiring cardiopulmonary bypass and cardioplegic arrest leads to myocardial ischemic and reperfusion injury. Gaseous nitric oxide has been demonstrated to have a myocardial protective effect following ischemia-reperfusion. We hypothesized that gaseous nitric oxide administered during cardiopulmonary bypass would have similar beneficial effects., Methods: In a prospective, randomized, blinded, placebo-controlled study, children undergoing repair of tetralogy of Fallot received either 20 ppm of gaseous nitric oxide or placebo delivered to the membrane oxygenator during cardiopulmonary bypass., Results: A total of 16 children were randomized into 2 equal groups once their parents or guardians had given written informed consent. No differences were found in age, crossclamp time, cardiopulmonary bypass time, or methemoglobin between the 2 groups. The group receiving gaseous nitric oxide had a significantly shortened duration of mechanical ventilation (8.4 ± 7.6 vs 16.3 ± 6.5 hours; P < .05) and intensive care unit length of stay (53.8 ± 19.7 vs 79.4 ± 37.7 hours; P < .05) compared with the placebo group. The patients had significantly lower troponin levels at 12, 24, and 48 hours (P < .05) and lower B-type natriuretic peptide levels at 12 and 24 hours (P < .05). A trend was found toward a less positive fluid balance, with significantly less diuretic usage. The study patients had a greater mean hemoglobin at 48 hours, despite the absence of differences in chest tube output, packed red blood cell transfusion, platelet counts or transfusion requirements, fresh frozen plasma transfusion, or prothrombin time/partial thromboplastin time in the first 48 hours., Conclusions: The delivery of gaseous nitric oxide to the cardiopulmonary bypass circuit for children undergoing cardiac surgery results in myocardial protection, improved fluid balance, and an improved postoperative intensive care unit course., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Congenital pulmonary vein stenosis (PVS) is associated with high mortality because surgical repair is usually not feasible or is ineffective. In addition, acquired PVS after repair of congenital heart disease is a potential complication that occurs in 5% to 10% of patients and carries a poor prognosis. Lung transplantation has been proposed as a viable option. However, long-term outcomes after lung transplant in these patients remain unknown., Methods: This was a retrospective review of prospectively maintained database., Results: Between 1990 and 2010, 20 patients (12 girls, 8 boys) with PVS underwent transplantation. Of these, 8 had acquired stenosis from prior repair for total anomalous pulmonary venous return and 1 from atrioventricular canal repair. The median waiting time was 26 days. The mean age at transplant was 1.1 ± 0.89 years, and 16 of the 20 patients were white. All patients received bilateral lung transplants on cardiopulmonary bypass. Four patients (20%) were receiving extracorporeal membrane oxygenation (ECMO) support before transplant, and 3 (15%) required ECMO after transplant due to graft dysfunction. The mean intensive care unit stay was 33.5 ± 29.1 days, and the mean hospital stay was 58.7 ± 43.5 days. The 30-day mortality was 10%. ECMO support in the peri-operative period was the main predictor of 30-day and 1-year mortality (hazard ratio, 3.6; p = 0.01). The overall 5-year survival of the entire cohort was 59.8% (67.3% congenital vs 50.7% acquired). The predominant cause of long-term mortality was bronchiolitis obliterans. The 5-year bronchiolitis obliterans-free survival was 48% (57.2% congenital vs 41% acquired)., Conclusion: Lung transplant is a viable treatment option for PVS, particularly for patients with diffuse disease or failed surgical correction., (Copyright © 2013 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Neonates who experience respiratory failure despite maximal ventilatory support have only extracorporeal membrane oxygenation as a rescue therapy, but it has very poor outcomes as a bridge to transplantation. A pumpless lung-assist device has been used in adults as a bridge to lung transplantation. An alternative membrane oxygenator, the Quadrox iD, is a suitable size for neonatal blood flow. Here we report the use of the Quadrox iD membrane oxygenator with central cannulation as a paracorporeal respiratory support therapy for a neonate with alveolar capillary dysplasia awaiting lung transplantation., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Heart transplantation is the only viable treatment for children with end-stage heart failure due to congenital heart disease (CHD) or cardiomyopathy. This study reviewed the trends in the indications for transplant and survival after transplant during the past 24 years., Methods: A retrospective review was performed of the 307 heart transplants performed at our center since 1986. To analyze the trends in the indications for transplant as well as operative death and late-survival, the data were divided into three periods in 8-year increments: 1986 to 1993 (50 patients), 1994 to 2001 (116 patients), and 2002 to 2009 (141 patients)., Results: The indications for transplantation were 39% cardiomyopathy, 57% CHD, and 4% retransplant. Of the 173 with CHD, 139 (80%) had single-ventricle (SV) anomalies. In the CHD group, transplantation for failed SV palliation, including Fontan procedure, became the predominant indication in the last 8-year interval of our program. Survival after transplant was the best in patients with cardiomyopathy and the worst in patients with failed palliations for SV anomalies, including failed Fontan procedures., Conclusions: Transplantation for heart failure related to failed SV palliation has become the most common indication for patients with CHD. The high-risk nature of these transplants will have significant implications for heart transplant programs as more infants with SV anomalies survive palliative procedures performed during infancy., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The natural history of idiopathic pulmonary arterial hypertension (IPAH) in patients of all ages is one of relentless progression. For those who fail medical therapy, lung transplantation remains the ultimate palliation. In the USA, IPAH is the second leading indication for lung transplantation in children and first for children 1 to 5 years of age. In this study, we report our 18-year experience with lung transplantation in children with IPAH., Methods: We performed a retrospective chart review of children with IPAH listed for lung transplant at our center between 1991 and 2009. Our data reflect a total of 26 children ranging in age from 1.6 to 18.9 years. Nineteen were transplanted and 7 died while waiting (27%). The impact of a number of pre-transplant variables on survival was evaluated., Results: Median survival for those transplanted was 5.8 years, with 1- and 5-year survival rates of 95% and 61%, respectively. Survival was independent of pre-transplant considerations such as age, weight, need for intravenous (IV) inotropes, use of IV pulmonary vasodilators, year of transplant and severity of right-sided cardiac pressures. There was 1 hospital death. Compared with the transplanted group, children who died waiting had a significantly higher incidence of supra-systemic right heart pressures (p = 0.02) and hemoptysis (p = 0.01)., Conclusions: Our study is the largest to date to look at outcomes for lung transplantation in children with IPAH. Their median survival compares favorably with that of all pediatric lung transplant recipients, 5.8 years vs 4.5 years, respectively. We did not identify any pre-transplant variables that presaged a poorer outcome. Thus, survival seemed more related to factors that influence long-term outcomes in all transplant recipients such as rejection and infection. Lung transplantation remains a viable option for children with IPAH, especially for those with supra-systemic right heart pressures despite maximal medical therapy., (Copyright © 2011 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

A variety of complex congenital heart defects in the pediatric population involve placement of a right ventricle to pulmonary artery conduit as part of surgical repair. With the advent of percutaneous pulmonary valve implantation (PPVI), patients may avoid the risks of serial surgical reinterventions as the PPVI acts to prolong the life of a previously placed conduit. As the experience with PPVI is growing, new challenges arise from complicated anatomy and severe conduit stenosis. We present a case of a 16-year-old male who underwent successful pulmonary valve placement with a Melody valve via a subxyphoid hybrid approach after an unsuccessful attempt at percutaneous placement., (Copyright © 2011 Wiley-Liss, Inc.)