National Fontan Operation short-term outcomes at or below 2-years-of-age compared to older than 2-years-of-age.

INTRODUCTION: Opinion is divided about optimal early timing of the Fontan Operation (FO). While some studies have suggested 3 years-of-age, others have shown good outcomes below 2 years-of-age. We analyzed the impact of age ≤2 years as compared age >2 years on short-term outcome of the FO using a large national database. METHODS: A retrospective analysis of the Kids Inpatient Database (2009-16) for the FO was done. The groups were divided into those who underwent FO at age ≤2 years (Early FO [EF]) as compared to age >2 years (Late FO [LF]). The data was abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. RESULTS: A total of 3381 patients underwent FO during this period of which 1482 (44%) were EF. The mean ages of the EF and LF were 1.6 and 4.3, respectively (p < .001). LF were more likely to be non-White, female, and have Heterotaxy syndrome. HLHS was more common in EF. There was no difference in the discharge mortality, length of stay, disposition (majority went home), and mean total charges incurred. The overall discharge mortality was low at 0.7% (24/3381). In multivariate analysis: cardiac arrest, acute kidney injury, mechanical ventilation >96 h, endocardial cushion defect and non-White ethnicity were predictors of a mortality and not age. CONCLUSION: Contemporary outcomes for FO are excellent with equivalent short-term outcomes in both the age groups. Occurrence of postoperative complications, non-White ethnicity and endocardial cushion defect diagnosis were predictive of a negative outcome.

Heart Transplantation from Biventricular Support in Infant with Novel SMYD1 Mutation.

SET and MYND domain-containing protein 1 (SMYD1) has been shown to be responsible for the development of fast twitch and cardiac muscle. Mutations in SMYD1 have been shown to be uniformly fatal in laboratory studies, and not previously described in living humans. We describe here the care of an infant suffering from cardiac failure due to an SMYD1 mutation requiring biventricular assist devices as a bridge to successful heart transplantation. The patient is now doing well 2 years post-transplant and represents a known survivor of a suspected uniformly fatal genetic mutation.

Survival and Long-Term Functional Outcomes for Children With Cardiac Arrest Treated With Extracorporeal Cardiopulmonary Resuscitation.

OBJECTIVES: To identify patient- and disease-related factors related to survival and favorable outcomes for children who underwent extracorporeal cardiopulmonary resuscitation after a refractory cardiac arrest. DESIGN: Retrospective observational study with prospective assessment of long-term functional outcome. PATIENTS: Fifty-six consecutive children undergoing extracorporeal cardiopulmonary resuscitation at our institution from 2007 to 2015. Median age at arrest was 3.5 months (interquartile range, 1-53). SETTING: Tertiary pediatric university hospital with a referral heart center. INTERVENTIONS: Health-related quality of life and family functioning assessment with the Pediatric Quality of Life Inventory and the McMaster Family Assessment Device. MEASUREMENTS AND MAIN RESULTS: Fifty-eight consecutive extracorporeal cardiopulmonary resuscitation episodes were included, with 46 (79.3%) related to primary cardiac conditions. Initial cannulation site was central in 19 (32.8%) and peripheral in 39 (67.2%). Survival to decannulation was 77.6% with survival at hospital discharge and at the end of the follow-up period being 65.5% and 62.1%, respectively. Time to follow-up was 38 months (interquartile range, 19-52). Patients who survived tended to be younger (3.5 mo [1 mo to 2 yr] vs 7 mo [1.25 mo to 17 yr]; p = 0.3) with decreased extracorporeal cardiopulmonary resuscitation times (28 min [15-47 min] vs 37.5 min [28.5-55 min]; p = 0.04). Those who received therapeutic hypothermia tended to have higher hospital survival (21/28 [75%] vs 16/29 [55%]; p = 0.08). Follow-up assessments of survivors demonstrated good quality of life and family functioning (Pediatric Quality of Life Inventory, 84 [76-89.5]; McMaster Family Assessment Device, 1.62 [1.33-1.83]). CONCLUSIONS: In this series, extracorporeal cardiopulmonary resuscitation was associated with relatively high survival rates and a good health-related quality of life and family functioning. Larger series are needed to assess whether this technique should be more broadly available in the pediatric critical care community.

Telemedicine in Pediatric Critical Care: A Retrospective Study in an International Extracorporeal Membrane Oxygenation Program.

INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) is an invaluable rescue technique for critically ill children with imminent or present cardiopulmonary collapse. However, medical team expertise to optimize results and decrease complications is scarce. Telemedicine can be used to enhance the delivery of quality interventions. MATERIALS AND METHODS: This is a retrospective review of pediatric patients assisted with ECMO in the cardiac intensive care unit (CICU) at Fundación Cardiovascular de Colombia from July 2011 to June 2015 (telemedicine) compared with similar patients from a previous period (pretelemedicine). Collected information included demographic data, cardiac diagnosis, risk adjustment for congenital heart surgery (RACHS-1), hospital mortality, CICU and hospital length of stay (LOS), ECMO type, and ECMO run hours as well as specific telemedicine information. RESULTS: Fifty-seven patients in the pretelemedicine and 109 in the telemedicine periods were included in the analysis. Forty-nine teleconsulted patients received 218 teleconsultations, with a recommendation for diagnostic or interventional catheterization in 38 patients (77.5%). A surgical procedure for significant residual lesions was recommended in 30 patients (61.2%). Patients in the telemedicine period were older (4.7 months vs. 1.6 months, p = 0.006), more likely to receive operating room ECMO (43.1% vs. 24.6%, p = 0.02), and had a higher proportion of patients with two-ventricle physiology (73.4% vs. 54.4%, p = 0.013). Hospital survival was higher during the telemedicine period (54.1% vs. 29.8%, p = 0.002), with a longer hospital LOS (67 days vs. 28 days, p < 0.001). CONCLUSION: The implementation of telemedicine-assisted interventions in a pediatric ECMO program delivered valuable diagnostic and therapeutic advice, was associated with significant changes in selection criteria and model of care, and an increased hospital survival.

Report of the 2022 Society of Thoracic Surgeons Congenital Heart Surgery Practice Survey.

BACKGROUND: The Society of Thoracic Surgeons Workforce on Congenital Surgery performed a practice survey to analyze contemporary data. METHODS: An electronic survey was sent to congenital heart surgeons in North America. Details on demographics, training paradigm, clinical practice, and work satisfaction were queried, tabulated, and analyzed. RESULTS: Of 312 unique contacts, 201 (64.4%) responded. Of these, 178 (89%) were practicing. The median age was 52 years (interquartile range, 43, 59 years), and 157 (88%) were male. The number of female respondents increased from 12 (7%) in 2015 to 18 (11%) at present. Practice composition was predominantly mixed pediatric and adult (141; 79%), although 15 (8%) surgeons practiced exclusively pediatric surgery. Most surgeons (154; 87%) reported performing the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 4 and 5 procedures. One-fourth (42; 24%) reported performing fewer than 50 pediatric cases per year, and 18 (10%) stated that their primary role was as a surgical assistant. Individual surgeon case volume was most commonly 100 to 149 total cases (29%). Although one-half (91; 51%) reported their volume as being "just right," 74 (42%) reported that their case volume was "too small." Seventy-six (43%) reported too many surgeons in their region. Of the 201 practicing surgeons, 30 (14.9%) plan retirement in the next 5 years. Most described career satisfaction, with 102 (57%) being very satisfied and 48 (27%) somewhat satisfied. CONCLUSIONS: Although most congenital heart surgeons in North America are satisfied with their careers, more than 40% believe that their caseload is inadequate and that there are too many surgeons in their region. Further analysis is warranted regarding career dissatisfaction and diversity.

Ventricular assist device support in children and adolescents with heart failure: the Children's Medical Center of Dallas experience.

Children with heart failure unresponsive to medical therapy are left with few options for survival. Ventricular assist devices (VADs) are life-saving options for such patients, allowing for bridge to transplantation or cardiac recovery. Retrospective review of cases from May 2006 to October 2010 was undertaken. Fourteen patients underwent implantation of VADs for refractory heart failure. Mean age was 9 years (range 1-17 years), and weight was 41 kg (range 9.7-71 kg). Indications for support: end-stage cardiomyopathy (n = 8), myocarditis (n = 3), univentricular failure (n = 2), and congenital heart disease/postcardiotomy (n = 1). Level of limitation at time of implant included critical cardiogenic shock in six (43%) and progressive decline in eight (57%). Extracorporeal membrane oxygenation was used as a bridge to VAD in five (36%) patients. Preimplant variables: 86% of patients requiring mechanical ventilation (mean 10.3 days), hyperbilirubinemia in 75%, and acute renal insufficiency in 79%. Device selection was systemic VAD in 11 (79%) and biventricular assist device in three (21%). Berlin Heart EXCOR was used in eight patients, while six patients received a Thoratec implantable VAD or paracorporeal VAD. Mean duration of support was 68 days (range 8-363 days). Overall survival was 79%. Ten patients (71%) were successfully bridged to transplantation, three (21%) died while on a device, one remains on support, and no patients were weaned from VAD. Children supported for single ventricle heart failure had a 50% survival with none currently bridged to transplantation. Complications included bleeding requiring reoperation in 21% (n = 3), stroke in 29% (n = 4), and driveline infections in 7% (n = 1). In two patients, a total of six pump exchanges were performed for thrombus formation. Survival for pediatric patients of all ages is excellent using current device technology with a majority of patients being successfully bridged to transplantation. Morbidity is acceptably low considering the severity of illness. Significant challenges exist with long-term extracorporeal support due to lack of donor availability and the high incidence of preformed alloantibodies especially in the failing single ventricle.

Multimodality delineation of a fistulous ruptured sinus of Valsalva aneurysm: a teaching case report.

Background: Ruptured sinus of Valsalva (SOV) is a rare cardiac anomaly with poor prognosis if untreated. Early diagnosis with accurate delineation of its anatomy is critical for timely treatment and choice of surgical vs. percutaneous intervention. Here we report a case of fistulous rupture of SOV; the preoperative multimodality studies including echocardiography, cardiac magnetic resonance and cardiac catheterization provided teaching and learning points. Case summary: A 48-year-old man with history of heart murmur and hypertension presented with a 5-day history of shortness of breath and peripheral oedema. He was diagnosed with rapid atrial flutter. The transthoracic and transesophageal echocardiography showed severe biventricular systolic dysfunction with a left-to-right shunt from ruptured SOV. The colour Doppler by transthoracic and transesophageal echocardiography and cardiac magnetic resonance revealed a swaying shunt flow exiting in direction to the right atrium (RA) and basal right ventricle (RV) during systole and diastole with no myocardial scaring. The left and right heart catheterization showed elevated right-sided pressures, pulmonary capillary wedge pressure, and left ventricular end-diastolic pressure. There was no difference in O2 saturation between venae cavae and RA but a misleading step-up in O2 saturation between RA and RV. Owing to rupture anatomy with uncertainty, the patient underwent surgical intervention. The ruptured SOV tunnelled through the base of tricuspid annulus to the RA very close to the basal RV. Discussion: Even with multimodality studies it can still be challenging to delineate the anatomy of a ruptured SOV without uncertainty preoperatively.

Beyond the Syndrome: Extensive Congenital Abnormalities in an Infant With Trisomy 21.

Herein we discuss the clinical course and subsequent autopsy of a female infant with trisomy 21 with balanced Rastelli Type "C" complete atrioventricular septal defect (AVSD), tetralogy of Fallot and right aortic arch with mirror image branching pattern who underwent a palliative right modified Blalock-Taussig-Thomas shunt (mBTTS) for hypoxemia from progressive right ventricular outflow tract obstruction. The baby was found to have multiple concomitant pathologic findings not typically seen with this constellation of cardiac anatomy. Autopsy revealed significant abdominal adhesions with near-complete stenosis of the transverse colon. In addition, the infant was found to have significantly elongated villi within the small and large bowel and a relatively large collagenous polyp in the small bowel. The decedent also had an abnormal tracheal bronchus, characterized by an additional superior right-sided bronchus, which is an extremely rare abnormality. Her clinical course was complicated by severe pulmonary hypertensive arteriolar changes out of proportion to what would be typical for her age, trisomy 21 status, and degree of left to right intracardiac shunting. Furthermore, she had refractory anasarca and recurrent chylous pleural effusions without gross lymphatic abnormalities that may have been secondary to systemic capillary leak syndrome (SCLS) versus severe pulmonary hypertension. Due to the aforementioned findings, the family elected for comfort care and the baby expired shortly after extubation. Overall, the infant had multiple, rare coexisting congenital abnormalities that likely represents an extreme phenotype of trisomy 21 that has not been described in the literature to date.

Orthotopic Heart Transplantation in a Patient With Gitelman Syndrome and Dilated Cardiomyopathy.

Gitelman syndrome (GS) is a rare hereditary tubulopathy affecting the distal tubule leading to significant electrolyte disturbances.1 Although generally a benign condition, rare associations with arrhythmias and sudden cardiac death have been reported.1 A paucity of literature exists associating GS with cardiomyopathy. We present a child with dilated cardiomyopathy and GS who was successfully treated with orthotopic heart transplantation.

Impact of Circuit Size on Coagulation and Hemolysis Complications in Pediatric Extracorporeal Membrane Oxygenation.

Extracorporeal membrane oxygenation (ECMO) circuit volume, patient size, and blood flow may influence coagulation and hemolysis complications. We performed a single-center retrospective analysis of ECMO patients over a 6.5 year period. In 299 ECMO runs, 13% required coagulation-associated circuit changes. Respiratory ECMO was associated with coagulation-associated circuit changes [odds ratio (O/R) 2.8, p < 0.05] and developed severe (plasma-free hemoglobin [pfHb] > 100 mg/dl) hemolysis (O/R 2.3, p < 0.05). Severe hemolysis and component changes were associated with hospital mortality (O/R 2.3 and 2.5, respectively, p < 0.05). The activated partial thromboplastin time (aPTT) to residence time (RT) ratio (aPTT/RT) was used as a surrogate for coagulation risk. We found that aPTT/RT > 2.5 more than doubled time to circuit change (3-8 days, p < 0.05), but aPTT/RT > 3 increased bleeding risks and hospital mortality (O/R 1.8; p < 0.1). Hemolysis was associated with patient weight and circuit to patient volume ratio (CPVR) (p < 0.05), but not pump type. Hemolysis slightly increased with transfusion (p = 0.08), and transfusion requirements increased for CPVR >50% (p < 0.1).Our data suggest that pediatric respiratory ECMO patients are more likely to develop coagulation and hemolysis complications, which are associated with increased mortality. This may result from higher inflammatory processes, which affect coagulation and red cell fragility. Minimizing circuit volume, inflammation, and red cell stress may help to reduce these two complications and their associated mortality.

Pipeline Embolization of an Infectious Basilar Artery Aneurysm in a 2-Year-Old Child: Case Report, Discussion of the Literature and Perioperative Considerations.

BACKGROUND AND IMPORTANCE: Flow diversion of intracranial aneurysms has been rarely described in the pediatric population. Here we discuss the technical and perioperative complexities inherent in the flow diversion of an infectious basilar apex aneurysm in a 2-yr-old child with significant medical comorbidities. CLINICAL PRESENTATION: Following judicious oral administration of dual anti-platelet agents and intra-arterial administration of calcium channel blockers to treat vasospasm, standard endovascular procedures were used to place a flow diverting stent across the neck of a rapidly enlarging infectious aneurysm of the basilar apex.Following the uncomplicated procedure, the patient demonstrated progressive thrombosis of the previously noted basilar apex aneurysm over a 3-mo period. The patient was therefore felt to be safe to proceed with, and eventually underwent, uncomplicated orthotopic heart transplant. CONCLUSION: Flow diversion of complex intracranial aneurysms in pediatric patients with significant medical comorbidities is feasible and safe; however, considerations have to be made in the pre- and perioperative care of these patients given the propensity for low-weight and complicated systemic disease processes.

Total Artificial Heart as Rescue Therapy for Primary Graft Failure in an Infant.

An infant unable to be weaned from cardiopulmonary bypass after orthotopic heart transplantation was cannulated for extracorporeal membrane oxygenation. During the next 3 days, allograft failure and intracardiac thrombosis necessitated cardiectomy. To provide acute mechanical circulatory support, artificial atrial chambers were constructed with Gore-Tex conduits and PediMag centrifugal pumps were connected to each by Berlin Heart EXCOR cannulae. The PediMag pumps were subsequently exchanged for 10-mL Berlin Heart EXCOR pumps. After 60 days of support by total artificial heart, the patient was bridged successfully to a second heart transplant.

Polytetrafluoroethylene conduits versus homografts for right ventricular outflow tract reconstruction in infants and young children: An institutional experience.

OBJECTIVE: Our institution uses a valved polytetrafluoroethylene conduit as an alternative to homografts. The objective of this study was to investigate the performance of bicuspid valved polytetrafluoroethylene conduits used for right ventricular outflow tract reconstruction in children aged less than 2 years and to evaluate risk factors for earlier conduit explant. METHODS: We performed an Institutional Review Board-approved retrospective chart review of all patients aged less than 2 years who underwent surgical right ventricular outflow tract reconstruction with a bicuspid valved polytetrafluoroethylene conduit or homograft conduit from July 2004 to December 2014. The end points of the study were defined as conduit explant, conduit explant or reintervention, conduit stenosis, and conduit insufficiency. RESULTS: Fifty-four patients underwent 65 right ventricular outflow tract reconstructions with a bicuspid valved polytetrafluoroethylene conduit (n = 39) or a homograft conduit (n = 26, 23 pulmonary, 3 aortic). The majority of diagnoses were truncus arteriosus (n = 28) and tetralogy of Fallot with pulmonary atresia (n = 19). Median age of patients at surgery was 134 (8-323) days and 128 (7-384) days in the PTFE and homograft groups, respectively. There was no difference in demographic data between the 2 groups. Time-to-event analysis demonstrated no difference in time to explant (P = .474) or time to explant or reintervention (P = .206) between the 2 conduit types. Younger age at surgery was the only independent risk factor for conduit explant (subdistribution hazard ratio 1.104 per 30 days younger, P < .001). There was no significant influence of conduit type on the development of moderate conduit stenosis (P = .931) or severe conduit insufficiency (P = .880). Larger conduit z score was protective for the development of moderate conduit stenosis (subdistribution hazard ratio, 0.46; P = .001). CONCLUSIONS: Bicuspid valved polytetrafluoroethylene conduits are a satisfactory choice for right ventricular outflow tract reconstruction in patients aged less than 2 years. Their availability, low cost, and lack of potential sensitization make them an appealing alternative to homograft conduits.

Predictors of Poor Outcomes in Pediatric Venoarterial Extracorporeal Membrane Oxygenation.

BACKGROUND: Venoarterial extracorporeal membrane oxygenation (VA-ECMO) provides respiratory and hemodynamic support to pediatric patients in severe cardiac failure. We aim to identify risk factors associated with poorer outcomes in this population. METHODS: A retrospective chart review was conducted of pediatric patients requiring VA-ECMO support for cardiac indications at our institution from 2004 to 2015. Data were collected on demographics, indication, markers of cardiac output, ventricular assist device (VAD) insertion, heart transplantation, or left atrial (LA) decompression. Univariate Cox proportional hazards models were used to calculate hazard ratios (HRs) for variables associated with the composite primary outcome of transplant-free survival (TFS). RESULTS: Of the 68 reviewed patients, 65% were male, 84% were white, 38% had a prior surgery, 13% had a prior transplant, 10% had a prior ECMO support, and 87.5% required vasoactive support within six hours of cannulation. The ECMO indications included congenital heart disease repaired >30 days prior (12%), cardiomyopathy (41%), posttransplant rejection (7%), and cardiorespiratory failure (40%). The TFS was 54.5% at discharge and 47.7% at one year. Predictors of transplant and/or death include epinephrine use (hazard ratio [HR] = 2.269, P = .041), elevated lactate (HR = 1.081, P = 0005), and elevated creatinine (HR = 1.081, P = .005) within six hours prior to cannulation. Sixteen (23.6%) patients underwent LA decompression. Placement of VAD occurred in 16 (23.5%) patients, for which nonwhite race (HR = 2.94, P = .034) and prior ECMO (HR = 3.42, P = .053) were the only identified risk factors. CONCLUSIONS: Need for VA-ECMO for cardiac support carries high inpatient morbidity and mortality. Epinephrine use and elevated lactate and creatinine were associated with especially poor outcomes. Patients who survived to discharge had good short-term follow-up results.

Use of Therapeutic Plasma Exchange during Extracorporeal Life Support in Critically Ill Cardiac Children with Thrombocytopenia-Associated Multi-Organ Failure.

BACKGROUND: Thrombocytopenia-associated multi-organ failure (TAMOF) in children is a well-described factor for increased hospital mortality. Low cardiac output syndrome (LCOS) and the effects of cardiopulmonary bypass may manifest with several adverse physiologic and immunologic effects, with varying degrees of thrombocytopenia and multi-organ dysfunction, sometimes very similar to TAMOF. LCOS is a common occurrence in children with critical heart disease, presenting in as much as 23.8% of infants postoperative of congenital heart surgery. Therapeutic plasma exchange (TPE) has been offered as a promising therapy for TAMOF; however, the therapeutic implications of this modality in children with critical heart disease and a clinical diagnosis of TAMOF are unknown. OBJECTIVES: We describe our institutional experience with TPE as an adjuvant rescue therapy for children with critical heart disease and a clinical diagnosis of TAMOF, while supported by extracorporeal membrane oxygenation (ECMO). METHODS: Single-center retrospective analysis of children with critical heart disease admitted to the CICU and supported by ECMO, undergoing TPE for a clinical diagnosis of TAMOF between January 2006 and June 2015. RESULTS: Forty-one patients were included for analysis. Median age and weight of patients was 0.6 years (range 0.0-17.2) and 8.5 kg (range 1.5-80.0). TPE was initiated at a median of 1 day (0-13) after initiation of ECMO. Modified organ failure index (MOFI) and platelet count improved after TPE start (p < 0.001). Patients with early TPE initiation after ECMO cannulation (<1 day) showed more improvement in MOFI and platelet counts than patients with late TPE initiation (p < 0.001 for each). Overall survival to hospital discharge was 53.7%. The within-groups hospital survival was 73.3% for patients with heart failure, 34.8% for patients with congenital heart disease, and 100% for those with other cardiac disease (p = 0.016). CONCLUSION: In children with critical cardiac disease and clinical diagnosis of TAMOF necessitating ECMO for hemodynamic support, concurrent TPE may be associated with an improvement in organ failure and platelet count, particularly when started early. Further studies are warranted to establish the most effective use of TPE and its effect on survival in this population.

Pulsatile paracorporeal assist devices in children and adolescents with biventricular failure.

Children with heart failure unresponsive to medical therapy are left with few options for survival. Pulsatile paracorporeal ventricular assist devices are life-saving options for such patients, allowing for bridge to transplantation or cardiac recovery. From March 1997 to July 2004, 12 patients underwent implantation of Thoratec biventricular assist devices (BVADs) for refractory heart failure. Mean age was 14.9 (range 7-20) and mean BSA was 1.7 (range 1.1-1.9). Indications for support included end-stage cardiomyopathy (n=10), myocarditis (n=1), and postcardiotomy heart failure (n=1). Preimplant variables included 50% of patients requiring mechanical ventilation (mean 4.2 days), hyperbilirubinemia in 58%, and acute renal failure in 50%. Mean duration of support was 64.5 (range 2-175) days. Overall survival was 83%, with nine patients successfully bridged to transplantation (75%). One patient exhibited recovery allowing for device explantation, and two patients died while on BVADs. Complications included bleeding requiring reoperation in 25% (n=3), stroke in 8% (n=1), driveline infections in 17% (n=2), and device malfunction in one patient. Pulsatile paracorporeal BVADs can be used successfully in children and adolescents with heart failure. These results warrant consideration of using available miniaturized technology in the United States for the support of smaller children with intractable biventricular failure.