De Novo and Progressive Pulmonary Vein Stenosis Following Pediatric Heart Transplantation: A Multicenter Retrospective Study.

BACKGROUND: Pulmonary vein stenosis (PVS) is a rare condition in which neointimal proliferation leads to venous and arterial hypertension. Little is known about PVS after heart transplant (HTx) in children. We sought to describe the characteristics and outcomes of children who develop PVS after HTx. METHODS: We performed a retrospective review of patients ≤18 years old who underwent HTx at two HTx centers between April 2012 and October 2023. Patients with PVS were identified via database queries. Cardiac diagnosis, PVS location and extent, and outcomes were recorded. RESULTS: Over 11.5 years, 422 patients underwent HTx across both centers. Nineteen patients with PVS (10 male) were identified, 15 with de novo PVS. Sixteen had underlying congenital heart disease (CHD), two with anomalous pulmonary venous return. PVS was diagnosed at a median of 2 months (range 2 weeks to 14 years) after HTx. At time of initial diagnosis, 13 patients had one-vessel PVS. At final follow-up, 7/19 (37%) had increases in the number of vessels involved. Six patients underwent surgery, and nine patients had stent or balloon angioplasty. Two patients were treated for pulmonary hypertension following PVS diagnosis. Three patients died from right heart failure secondary to PVS. CONCLUSIONS: This is the largest study to describe the characteristics of post-HTx PVS in children. PVS occurs in 4.5% of HTx, and underlying CHD is a strong risk factor. Multiple vessels can be involved and may require catheter-based or surgical intervention. Clinicians must be vigilant in monitoring the development of PVS in this population.

Histopathology, mRNA expression profile, and donor-derived cell-free DNA for assessment of rejection in pediatric heart transplantation.

BACKGROUND: The relationship between histopathologic and molecular ("MMDx"®) assessments of endomyocardial biopsy (EMB) and serum donor-derived cell-free DNA (ddcfDNA) in acute rejection (AR) assessment following pediatric heart transplantation (HT) is unknown. METHODS: EMB sent for MMDx and histopathology from November 2021 to September 2022 were reviewed. MMDx and histopathology results were compared. DdcfDNA obtained ≤1 week prior to EMB were compared with histopathology and MMDx results. The discrimination of ddcfDNA for AR was assessed using receiver-operating curves. FINDINGS: In this study, 177 EMBs were obtained for histopathology and MMDx, 101 had time-matched ddcfDNA values. MMDx and Histopathology displayed moderate agreement for T-cell-mediated rejection (TCMR, Kappa = 0.52, p < .001) and antibody-mediated rejection (ABMR, Kappa = 0.41, p < .001). Discordant results occurred in 24% of cases, most often with ABMR. Compared with no AR, ddcfDNA values were elevated in cases of AR diagnosed by both histopathology and MMDx (p < .01 for all). Additionally, ddcfDNA values were elevated in injury patterns on MMDx, even when AR was not present (p = .01). DdcfDNA displayed excellent discrimination (AUC 0.83) for AR by MMDx and/or histopathology. Using a threshold of ≥0.135%, ddcfDNA had a sensitivity of 90%, specificity of 63%, PPV of 52%, and NPV of 94%. CONCLUSIONS: Histopathology and MMDx displayed moderate agreement in diagnosing AR following pediatric HT, with most discrepancies noted in the presence of ABMR. DdcfDNA is elevated with AR, with excellent discrimination and high NPV particularly when utilizing MMDx. A combination of all three tests may be necessary in some cases.

Impact of center volume on outcomes after ventricular assist device implantation in pediatric patients: An analysis of the STS-Pedimacs database.

BACKGROUND: To date, no pediatric studies have highlighted the impact of center's ventricular assist device (VAD) volumes on post implant outcomes. METHODS: Children (age <19) enrolled in Pedimacs undergoing initial left ventricular assist device implantation from 2012 to 2020 were included. Center volume was analyzed as a continuous and categorical variable. For categorical analysis, center volumes were divided as: low volume (1-15 implants), medium volume (15-30 implants), and high volume (>30 implants) during our study period. Patient characteristics and outcomes were compared by center's VAD volumes. RESULTS: Of 44 centers, 16 (36.4%) were low, 11 (25%) were medium, and 17 (38.6%) were high-volume centers. Children at high-volume centers were least likely intubated, sedated, or paralyzed, and most likely ambulating preimplant (p < 0.05 for all). Center's VAD volumes were not a significant risk factor for mortality post implant when treated as a continuous or a categorical variable (p > 0.05). Compared to low volume, children at high-volume centers had fewer early neurological events. Compared to medium volume, those at high-volume centers had fewer late bleeding events (p < 0.05 for all). There were no significant differences in survival after an adverse event by hospital volumes (p > 0.05). CONCLUSIONS: Although hospital volume does not affect post-VAD implant mortality, pediatric centers with higher VAD volumes have fewer patients intubated, sedated, paralyzed pre implant, and have lower adverse events. Failure to rescue was not significantly different between low, medium, and high-volume VAD centers.

Clinical Outcomes of SARS-CoV-2 Infection in Pediatric Patients on Ventricular Assist Device Support: An ACTION Registry Analysis.

Adult patients on left ventricular assist device (LVAD) support have increased morbidity and mortality after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. There are no reported clinical data describing outcomes among pediatric patients on ventricular assist device (VAD) support infected with SARS-CoV-2. We conducted a retrospective study using the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry to evaluate patient characteristics and clinical outcomes after SARS-CoV-2 infection. A total of 22 children on VAD support (median age at infection 10.6 years) from 16 centers tested positive for SARS-CoV-2. Cardiomyopathy (59.1%) and congenital heart disease (40.9%) were the most common primary diagnoses. The type of support included LVAD in 19 (86.4%), biventricular assist device (BIVAD) in one (4.5%), and single ventricle VAD in two (9%) patients. At the time of infection, 50% were outpatients, 23% were inpatients on a general cardiology floor, and 27% were in the cardiac intensive care unit (CICU). Most patients (82%) were symptomatic at time of diagnosis, but only 13% required escalation of respiratory support, and 31% received SARS-CoV-2 therapies. Notably, no mortality occurred, and significant morbidity was rare after SARS-CoV-2 infection in pediatric patients on VAD support.

Contemporary Care and Outcomes of Critically-ill Children With Clinically Diagnosed Myocarditis.

PURPOSE: To describe contemporary management and outcomes in children with myocarditis who are admitted to a cardiac intensive care unit (CICU) and to identify the characteristics associated with mortality. METHODS: All patients in the Pediatric Cardiac Critical Care Consortium (PC4) registry between August 2014 and June 2021 who were diagnosed with myocarditis were included. Univariable analyses and multivariable logistic regression evaluated the factors associated with in-hospital mortality. RESULTS: There were 847 CICU admissions for myocarditis in 51 centers. The median age was 12 years (IQR 2.7-16). In-hospital mortality occurred in 53 patients (6.3%), and 60 (7.1%) had cardiac arrest during admission. Mechanical ventilation was required in 339 patients (40%), and mechanical circulatory support (MCS) in 177 (21%); extracorporeal membrane oxygenation (ECMO)-only in 142 (16.7%), ECMO-to-ventricular assist device (VAD) in 20 (2.4%), extracorporeal cardiac resuscitation in 43 (5%), and VAD-only in 15 (1.8%) patients. MCS was associated with in-hospital mortality; 20.3% receiving MCS died compared to 2.5% without MCS (P < 0.001). Mortality rates were similar in ECMO-only, ECMO-to-VAD and VAD-only groups. The median time from CICU admission to ECMO was 2.0 hours (IQR 0-9.4) and to VAD, it was 9.9 days (IQR 6.3-16.8). Time to MCS was not associated with mortality. In multivariable modeling of patients' characteristics, smaller body surface area (BSA) and low eGFR were independently associated with mortality, and after including critical therapies, mechanical ventilation and ECMO were independent predictors of mortality. CONCLUSION: This contemporary cohort of children admitted to CICUs with myocarditis commonly received high-resource therapies; however, most patients survived to hospital discharge and rarely received VAD. Smaller patient size, acute kidney injury and receipt of mechanical ventilation or ECMO were independently associated with mortality.

Seventh Annual Society of Thoracic Surgeons Pedimacs Report.

BACKGROUND: The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), supported by The Society of Thoracic Surgeons, provides detailed information on pediatric patients supported with ventricular assist devices (VADs). METHODS: From September 19, 2012, to December 31, 2022, 1463 devices in 1219 patients aged <19 years were reported to the registry from 40 North American hospitals. RESULTS: Cardiomyopathy remains the most common underlying etiology (59%), followed by congenital heart disease (26%) and myocarditis (8%). Implantable continuous devices were most common (39%) type, followed by paracorporeal pulsatile (28%) and paracorporeal continuous (27%) devices. At 6 months after VAD implantation, a favorable outcome (transplant, recovery, or alive on device) was achieved in 85% of patients, which was greatest among those on implantable continuous VADs (92%) and least for paracorporeal continuous VADs (68%), although the patient population supported on these devices is different. CONCLUSIONS: This Seventh Pedimacs Report demonstrates the continued importance of VADs in the treatment of children. With the complexity of cardiac physiologies and sizes of patients, multiple types of devices are used, including paracorporeal continuous, paracorporeal pulsatile, and implantable continuous devices. The preoperative risk factors and differences in patient populations may account for some of the differences in survival observed among these devices. This report, along with other collaborative work, continues to advance the care of this challenging and vulnerable population.

Outcomes after initial heart failure consultation in Fontan patients.

BACKGROUND: Patients with Fontan failure are high-risk candidates for heart transplantation and other advanced therapies. Understanding the outcomes following initial heart failure consultation can help define appropriate timing of referral for advanced heart failure care. METHODS: This is a survey study of heart failure providers seeing any Fontan patient for initial heart failure care. Part 1 of the survey captured data on clinical characteristics at the time of heart failure consultation, and Part 2, completed 30 days later, captured outcomes (death, transplant evaluation outcome, and other interventions). Patients were classified as "too late" (death or declined for transplant due to being too sick) and/or "care escalation" (ventricular assist device implanted, inotrope initiated, and/or listed for transplant), within 30 days. "Late referral" was defined as those referred too late and/or had care escalation. RESULTS: Between 7/2020 and 7/2022, 77 Fontan patients (52% inpatient) had an initial heart failure consultation. Ten per cent were referred too late (6 were too sick for heart transplantation with one subsequent death, and two others died without heart transplantation evaluation, within 30 days), and 36% had care escalation (21 listed ± 5 ventricular assist device implanted ± 6 inotrope initiated). Overall, 42% were late referrals. Heart failure consultation < 1 year after Fontan surgery was strongly associated with late referral (OR 6.2, 95% CI 1.8-21.5, p=0.004). CONCLUSIONS: Over 40% of Fontan patients seen for an initial heart failure consultation were late referrals, with 10% dying or being declined for transplant within a month of consultation. Earlier referral, particularly for those with heart failure soon after Fontan surgery, should be encouraged.

Prenatally Diagnosed Interventricular Septal Aneurysm with Associated Ventricular Dysfunction.

Congenital interventricular septal aneurysms (IVSA) of the muscular septum are rare and can be associated with other familial abnormalities of the ventricular septum, arrhythmias, additional congenital heart disease, and chromosomal abnormalities. IVSA is also linked to ventricular dysfunction and non-compaction, although there are limited reports of this association presenting in utero. We describe a case of fetal ventricular septal aneurysm associated with ventricular dysfunction and pericardial effusion.

Interventricular Septal Hematoma Complicating Ventricular Assist Device Placement in an Infant: Case Report and Review of the Literature.

Interventricular septal hematoma is a rare and life-threatening complication of pediatric cardiac surgery. Commonly seen following ventricular septal defect repair, it has also been associated with ventricular assist device (VAD) placement. Although conservative management is usually successful, operative drainage of interventricular septal hematoma occurring in pediatric patients undergoing VAD implantation should be considered.

Stroke Diagnosis Protocol for Children With Ventricular Assist Devices.

Ventricular assist devices (VADs) are increasingly used for end-stage heart failure in children. VAD-associated neurologic dysfunction, including stroke and intracranial hemorrhage, occurs in more than 20% of patients. Starting in 2019, we implemented a protocol to diagnose stroke in relation to VAD to facilitate treatment. A multidisciplinary approach was implemented including targeted education for providers. VAD goals, structured neurologic exam, and frequency of neuromonitoring were incorporated into daily rounds, tailored to patient's phase of recovery. A protocolized neurocritical team assessment was implemented. A VAD-specific stroke algorithm and order set were implemented to facilitate rapid neuroimaging. We performed a pre- and postimplementation analysis from 2015 to 2020. Forty-six patients had VADs placed, 25 preintervention, and 21 postintervention. We compared the number of patients evaluated for stroke, time to imaging, and documentation of last known normal exam. Preintervention, time to imaging was 7 hours, and documentation was inconsistent. Postintervention, time to imaging decreased to 2.8 hours ( p = 0.038) with universal documentation of last known normal ( p = 0.009). The use of head computerized tomographies decreased from 11 preintervention to three postintervention. Development of a VAD protocol decreased time to imaging for suspected stroke and reduced unnecessary imaging. Further studies are required to validate these data.

Waitlist and post-transplant outcomes for children with myocarditis listed for heart transplantation over 3 decades.

BACKGROUND: There is limited and conflicting information on waitlist and transplant outcomes for children with myocarditis. METHODS: Retrospective review included children with myocarditis and dilated cardiomyopathy (DCM) listed for HT from January 01, 1993 to December 31, 2019 in the Pediatric Heart Transplant Society database. Clinical characteristics, waitlist and post-HT outcomes (graft loss, rejection, cardiac allograft vasculopathy, infection and malignancy) for children listed from early (1993-2008) and current era (2009-2019) with myocarditis were evaluated and compared to those with DCM. RESULTS: Of 9755 children listed, 322 (3.3%) had myocarditis and 3178 (32.6%) DCM. Compared to DCM, children with myocarditis in the early and the current era were significantly more likely to be listed at higher urgency; be in intensive care unit; on mechanical ventilation; extracorporeal membrane oxygenation and ventricular assist device (p < 0.05 for all). While unadjusted analysis revealed lower transplant rates and higher waitlist mortality for children with myocarditis, in multivariable analysis, myocarditis was not a risk factor for waitlist mortality. Myocarditis, however, was a significant risk factor for early phase post-HT graft loss (HR 2.46; p = 0.003). Waitlist and post-HT survival for children with myocarditis were similar for those listed and transplanted in the early era to those listed and transplanted in the current era (p > 0.05 for both). CONCLUSIONS: Children with myocarditis have a higher acuity of illness at listing and at HT and have inferior post-HT survival compared to children with DCM. Outcomes for children with myocarditis have not improved over the 3 decades and efforts are needed to improve outcomes for this cohort.

Ventricular Assist Device Outcomes in Infants and Children With Stage 1 Single Ventricle Palliation.

Data on ventricular assist device (VAD) outcomes in infants with stage 1 single ventricle (SV) palliation are limited. We examined the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry for outcomes of pre/poststage 1 SV patients undergoing VAD implantation between March 2018 and October 2020. Data are collected from 32 centers and major adverse events undergo central adjudication. During the study period, 30 stage 1 SV patients underwent VAD implant with median age of 0.9 months (range 0.1-25) and weight 3.7 kg (2.4-17). Preimplant illness severity was high: ventilator support (90%), ECMO (30%), prior cerebral vascular accident (CVA, 23%), and dialysis (13%). Devices used included paracorporeal pulsatile (50%), paracorporeal continuous-flow (37%), and multiple devices (13%). Median support duration was 56 days (range 3-246). A positive clinical outcome (transplanted or weaned) was attained in 63% (63% transplanted, 37% mortality, 0% weaned). VAD adverse events included: major infection (43%), neurologic dysfunction (any = 30%; CVA = 20%), major bleeding (17%), renal dysfunction (13%), and device malfunction (3%). In conclusion, stage 1 SV patients undergoing VAD support have high preimplant illness severity and complexity, as well as significant morbidity and mortality postimplant. A variety of devices and strategies are employed by centers to support this challenging population.

Outcomes of infants with pulmonary atresia with intact ventricular septum listed for heart transplantation: A multi-institutional study.

BACKGROUND: Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs. other single ventricle (SV) anatomies listed for HT. METHODS: Data from the Pediatric Heart Transplant Society (1993-2018) were analyzed for survival and risk factors for mortality. RESULTS: Of 1617 SV infants, 300 had PA/IVS (19%) and 1317 had other SV (81%). Overall, 1-, 5-, and 10-year survival was higher among PA/IVS (74%, 65%, 61%) versus other SV infants (62%, 54%, 50%, p = .004). While waitlist mortality was similar between groups (p = .09), PA/IVS was an independent predictor of improved waitlist survival (HR 0.68, p = .03), and PA/IVS infants had higher incidence of waitlist removal (8% vs. 5.5%, p = .03), most commonly for being "too well." Post-transplant survival was superior among PA/IVS versus other SV infants (1- and 5-year survival 93% and 81% vs. 80% and 71%, p < .0001). Risk factors for PA/IVS waitlist mortality (2008-2018) included extracorporeal membrane oxygenation and mechanical ventilation. Prior aortopulmonary (AP) shunt among PA/IVS infants was associated with improved waitlist survival. CONCLUSIONS: Overall survival among PA/IVS infants listed for HT exceeds that of other SV infants with PA/IVS identified as an independent predictor of improved waitlist and post-transplant survival. Prior AP shunt among listed PA/IVS infants was associated with improved waitlist outcomes, though, which may reflect a listing selection bias.

Biventricular Assist Device Support for Intractable Arrhythmias From Histiocytoid Cardiomyopathy.

Histiocytoid cardiomyopathy (HICMP) is a rare mitochondrial cardiomyopathy associated with recurrent life-threatening arrhythmias and variable degrees of systolic dysfunction. Successful heart transplantation for HICMP has been described, but there has been no published experience with biventricular assist device (BiVAD) support for intractable arrhythmias in HICMP. We report a 13 month old girl with left ventricular noncompaction and preserved systolic function who presented in cardiogenic shock secondary to incessant ventricular arrhythmias. After failed attempts at chemical and electrical cardioversion, she underwent BiVAD implantation as bridge to transplantation. Her BiVAD course was complicated by mechanical inflow obstruction during sinus rhythm, necessitating left-sided cannulation revision from an apical to atrial inflow cannula. This maneuver resolved the obstruction and the patient was transitioned to Berlin EXCOR (Berlin Heart Inc, The Woodlands, TX) BiVADs. On Berlin pumps, she had intermittent pauses (no fill/no eject) while in sinus rhythm, felt to be due to competition from intrinsic ejection. Despite these pauses, the patient experienced an uneventful remainder of her BiVAD course (205 days total) with minimal fibrin deposition and no device-related complications. BiVAD can support pediatric patients with hemodynamically significant arrhythmias to transplantation. Atrial cannulation strategy may be preferred in cases of preserved systolic function, ventricular noncompaction, and frequent rhythm changes.

Patient and Device Selection in Pediatric MCS: A Review of Current Consensus and Unsettled Questions.

The field of pediatric ventricular assist device (VAD) support has expanded significantly over the past 20 years, with one third of pediatric heart transplant recipients currently being bridged to transplant with a VAD. Despite increased pediatric VAD utilization, however, there remains little formalized guidance for patient or device selection. The population of children with advanced heart failure is quite heterogeneous, and the available data suggest that VAD outcomes vary significantly based upon patient size, anatomy, level of illness, and type of device implanted. In an effort to better understand current practice patterns and identify populations for whom there does not appear to be a consensus approach to achieving optimal VAD outcomes, the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) has surveyed clinical decision-making among member sites and conducted a review of the available literature regarding patient population-specific VAD outcomes and center-specific practices related to patient and device selection. Rather than aiming to provide clinical guidelines, this document offers an overview of contemporary approaches to patient and device selection, highlighting specific populations for whom there is not a consensus approach to achieving reliably good VAD outcomes, as these populations may benefit most from future research and quality improvement efforts directed toward identifying best practice.

Pediatric Heart Transplant Recipients Bridged with Biventricular Assist Device Have Worse 1 Year Graft Survival.

There are little data on postheart transplant (HT) outcomes for pediatric patients that were supported to HT with biventricular assist device (BiVAD). The United Network for Organ Sharing database was queried for patients <18 years old at time of HT between January 2005 and March 2018, excluding patients bridged with total artificial hearts and right ventricular assist device (VAD). Of 4,904 pediatric HT recipients, patients were grouped by no VAD support (3,934; 80.2%), left ventricular assist device only (736; 15%), and BiVAD (234; 4.8%). Overall graft survival analysis indicates crossing hazard rates between groups over time with the BiVAD group having a significantly lower graft survival at 1 year post-HT. A Cox model adjusted for age, era, diagnosis, and time by group interaction demonstrated increased 1 year hazard ratio (HR) of 8.5 (95% confidence intervals [CI]: 6.15-11.79) comparing BiVAD to no VAD. Comparable hazard between BiVAD and no VAD groups were found at 5 years (HR 1.01; 95% CI: 0.67-1.51), while lower hazard for the BiVAD group was found at 10 years post-HT (HR 0.07; 95% CI: 0.03-0.18). Although pre-HT BiVAD support leads to worse graft survival 1 year post-HT, long-term survival is acceptable.

Right heart failure considerations in pediatric ventricular assist devices.

Right heart failure (RHF) is a vexing problem in children after left ventricular assist device (LVAD) implantation that can negatively impact transplant candidacy and survival. Anticipation, prevention, early identification and appropriate medical and device management of RHF are important to successful LVAD outcomes. However, there is limited pediatric evidence to guide practice. This pediatric-focused review summarizes the relevant literature and describes the harmonized approach to RHF from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION). This review seeks to improve RHF outcomes through the sharing of best practices and experience across the pediatric VAD community.

Berlin Heart EXCOR and ACTION post-approval surveillance study report.

BACKGROUND: The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported. This report summarizes the outcomes of patients supported with EXCOR from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. These data were part of the PMA surveillance study (PSS) required by the Food and Drug Administration. METHODS: ACTION is a learning collaborative of over 40 pediatric heart failure programs worldwide, which collects data for all VAD implantations as one of its initiatives. All patients in North America with EXCOR implants reported to ACTION from 2018 to 2020 (n = 72) who had met an outcome were included in the EXCOR PSS group. This was compared with a historical, previously reported Berlin Heart EXCOR study group (Berlin Heart study [BHS] group, n = 320, 2007‒2014). RESULTS: Patients in the PSS group were younger, were smaller in weight/body surface area, were more likely to have congenital heart disease, and were less likely to receive a bi-VAD than those in the BHS group. Patients in the PSS group were less likely to be in Interagency Registry for Mechanically Assisted Circulatory Support Profile 1 and were supported for a longer duration. The primary anticoagulation therapy for 92% of patients in the PSS group was bivalirudin. Success, defined as being transplanted, being weaned for recovery, or being alive on a device at 180 days after implantation, was 86% in the PSS group compared with 76% in the BHS group. Incidence of stroke was reduced by 44% and the frequency of pump exchange by 40% in the PSS group compared with those in the BHS group. Similarly, all other adverse events, including major bleeding, were reduced in the PSS group. CONCLUSIONS: The PSS data, collected through ACTION, highlight the improvement in outcomes for patients supported with EXCOR compared with the outcomes in a historical cohort. These findings may be the result of changes in patient care practices over time and collaborative learning.

Long-Term Patient Experience Following Acutely Successful Ablation of Supraventricular Tachycardia Substrate in Children.

Definitive treatment of supraventricular tachycardia (SVT) substrate involves catheter ablation. While objective success rates have been well established, long-term subjective patient experiences have not been well described. We quantify a subjective cure rate and characterize long-term patient experience after acutely successful ablation. A cross-sectional survey of pediatric patients with accessory pathways or atrioventricular nodal reentrant tachycardia who underwent acutely successful ablation from 2008 to 2012 was performed. Data were obtained from medical records and patient surveys. Patients with congenital heart disease other than patent ductus arteriosus, patent foramen ovale, or coronary artery abnormalities were excluded. Statistical analyses included Student's t-test and χ2 analysis for continuous and categorical variables, respectively. Surveys were sent to 153 patients of which 147 responded with median follow-up of 7.2 (IQR 6.1-8.5) years. Of the 147 responders, 124 (84%) patients reported cure with a male predominance. Symptoms were present in 130/147 (88%) patients pre-ablation and in 53/147 (36%) post-ablation. Among those with post-ablation symptoms, 50/53 (94%) reported symptomatic improvement. Recurrence occurred in 23/147 (16%) patients and was more prevalent following cryoablation. Ablation of SVT substrate can be curative with excellent long-term results and patient satisfaction. Long-term subjective cure rate is high and there is a substantial decrease in symptoms post-ablation. Many patients continue to have symptoms following ablation; however, the majority of these patients consider themselves cured and symptoms can be attributed to other etiologies. Recurrence is uncommon and occurs more frequently following cryoablation.