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Patients with ccTGA or d-TGA managed via atrial switch (Mustard or Senning operations) have biventricular circulations with a sub-aortic right ventricle (2V-RV). Other than in a tiny percentage of ccTGA patients, premature heart failure (HF) is common, driven by chronic RV dilatation and dysfunction and/or tricuspid regurgitation. These patients are different from the general HF population in that they are younger, more heterogeneous, are predisposed to pulmonary hypertension and present unique and complex surgical challenges. Despite their young age, they experience disproportionately poor access to advanced therapies and are often disqualified for transplant by pulmonary hypertension, HLA sensitization, program risk-tolerance and psychosocial issues. Mechanical support of the subaortic RV with ventricular assist device (subaortic RVAD, also known as SVAD), although technically challenging, can be an effective alternative to palliative care and offers high likelihood of bridging patients to heart transplant candidacy. In addition, temporary trans-catheter SVAD Impella support has been advantageous for stabilization of decompensated 2V-RV patients or as bridge to durable SVAD support. Improved awareness of and access to specialist ACHD-HF teams offering mechanical support (and transplantation) for 2V-RV patients is increasingly urgent for this aging population, and will improve options and outcomes for these patients as HF emerges.

Learning objectives While coronary artery anomalies (CAAs) are relatively rare overall, they are one of the most common types of congenital heart disease.1 2 Multiple classification schemes have been proposed, typically taking into account abnormalities of vessel origin, course, number and ventricular myocardium supply.3 4 These anomalies can occur in otherwise structurally normal hearts or associated with other types of congenital heart disease. While many CAAs are not associated with untoward outcomes, some may have a more concerning clinical course resulting in presentation with sudden cardiac death (SCD).5 6 Unfortunately, this dramatic presentation may be the first presenting symptom, though patients may have had prior symptoms that were not linked to the underlying potentially fatal diagnosis.5 This review will focus on the evaluation and management of CAA among adolescents and young adults with otherwise structurally normal hearts, primarily focusing on anomalous aortic origin of a coronary artery (AAOCA) at or above the opposite sinus of Valsalva, the second leading cause of SCD in the young.6 AAOCA may involve the right coronary artery (AAORCA) originating from the left (opposite anterior) sinus of Valsalva, the left coronary originating from the right (opposite anterior) sinus of Valsalva (AAOLCA), or, more rarely, the origin of a coronary artery from or near the ‘non-coronary’ (posterior) sinus.7 They are typically also categorised into the following groups based on their course: interarterial, subpulmonic (also called intraseptal or intraconal), pre-pulmonic, retroaortic and retrocardiac (figure 1). Based on this anatomical definition, lesions have historically been classified as normal variants without increased risk of mortality (typically including those variants without an …

Mapping and ablation of atrial tachycardia (AT) is commonly performed in lateral tunnel Fontan (LTF) patients, yet there is little information on the need of baffle puncture to access the pulmonary venous atrium (PVA). This study aimed to evaluate the most common chamber location of critical sites for majority of AT in LTF patients. Consecutive LTF patients underwent catheter-based high-density mapping and ablation of AT from Nov. 2015 to Mar. 2019. Critical sites were identified by a combination of activation and entrainment mapping. Acute procedural success was defined as AT termination with ablation and non-inducibility of any AT. Predictors for ablation failure were evaluated in retrospect. Fifteen catheter ablation procedures were performed in 9 patients. A total of 15 clinical ATs (mean TCL 369 ± 91 ms) were mapped. The mechanism was macro re-entry in 11 (73%) and micro re-entry in 2. In 11 ATs (73%), 94 ± 5% of tachycardia cycle length (TCL) were mapped inside the tunnel. The commonest site of successful ablation in the tunnel was on the lateral wall (60%). Trans-baffle access was obtained during 5 of 15 procedures (33%). Overall, procedural success was achieved in 9 of 15 procedures (60%). There were no complications. Recurrence of AT was 42% over a follow-up period of 4.3 ± 3.2 years. Faster TCL of 200–300 ms showed a trend towards ablation failure, (OR 17, 95% CI 0.7 to 423, p = 0.08). Catheter ablation can be performed effectively for ATs in LTF patients usually from inside the tunnel. ATs with critical sites in the PVA are uncommon. This information will help plan ablation in LTF patients without resorting to initial trans-baffle access.

Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients.A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates.A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029).Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort.

Purpose End-stage heart failure is a leading cause of morbidity and mortality in adult congenital heart disease (ACHD) patients; however, advanced therapies such as ventricular assist devices (VADs) are rarely implemented in this population. The aim of this study was to examine the outcomes of ACHD patients supported by durable continuous-flow VADs. Methods We performed a retrospective review of patients with congenital heart disease >18 years of age at time of VAD implantation at Texas Children's Hospital and Baylor St. Luke's Medical Center from 2011-2020. Results In total, 13 patients (median age 34 years [IQR, 24-47]) received a VAD (Table). All devices were implanted in the subaortic ventricle; 2 patients (15%) and 11 patients (85%) had moderate and great complexity congenital heart disease, respectively. Prior to implantation, 11 patients (85%) required inotropic support and 5 patients (38%) required temporary mechanical circulatory support. 12 patients (92%) survived to hospital discharge with a median postoperative LOS of 36 days (IQR, 27-50). Among those who survived to hospital discharge, all (100%) were NYHA class I or II at follow-up and the median duration of support was 816 days (IQR, 160-1629). Patients experienced device-related complications including neurologic events (n = 5; n = 2 disabling stroke), driveline infections (n = 3), presumed device infections requiring chronic antibiotic therapy (n = 4; n = 1 associated pump exchange), GI bleeds (n = 6), and presumed pump thromboses (n = 5; n = 2 associated pump exchanges). Overall survival (heart transplant censored) at 1, 3, and 5 years was 82%, 70%, and 32%, respectively. 2 patients (50%) were successfully bridged to transplant and 1 patient initially implanted as destination therapy eventually received a transplant. Conclusion Our data demonstrate that durable VADs can be used effectively in ACHD patients with end-stage heart failure and support efforts to more readily utilize these therapies in this population.

Purpose Adult congenital heart disease (ACHD) patients are disproportionally affected by heart failure and cardiogenic shock compared to the general population. Despite a growing and aging cohort, experience with percutaneous continuous flow ventricular assist device (pVAD) use in ACHD patients is rarely described in the literature. Methods Retrospective chart review at Texas Children's Hospital and Baylor St. Luke's Medical Center from 1/2016 to 8/2020 for patients with congenital heart disease who were adult age at time of pVAD placement. Results Six patients with ACHD received 7 pVAD devices with a median age at placement of 34 [IQR 23- 38] years (Table). Five (83%) patients were male and 5 (83%) had complex congenital heart disease. Three (50%) patients had Fontan circulation. Devices included: 4 (57%) Impella CP®, 1 (14%) Impella 5.0® and 2 (29%) Impella 5.5®. Four (57%) devices were placed in patients with INTERMACS profile score 1. Four (57%) devices were placed in patients with acute decompensated heart failure in the setting of chronic heart failure and 3 (43%) devices were placed due to peri-procedural hemodynamic compromise. Three devices (43%) were placed via hybrid-surgical method with axillary artery grafts and 4 (57%) Impella CP® devices were placed percutaneously in the femoral artery. Median duration of patient support was 20 [IQR 3-44] days. Major pVAD-related complications included severe hemolysis requiring replacement of Impella CP® with Impella 5.0 device® and aortic valve replacement at time of Impella® 5.0 removal and durable ventricular assist device implantation in a neo-aortic valve with pre-existing regurgitation. There were no significant limb injuries/ischemia, stroke or bleeding events. Two (33%) patients were bridged to a durable ventricular assist device; all 6 (100%) patients survived to hospital discharge. Conclusion Percutaneous continuous flow ventricular assist devices can be successfully used to hemodynamically support ACHD patients in cardiogenic shock.

Hypoplastic left heart syndrome (HLHS) is a high-risk condition among the wider spectrum of single-ventricle physiology lesions, and few survivors have reached adulthood.1 As HLHS is one of the most common diagnoses for children undergoing Fontan palliation,2 identification of risk factors for adverse outcomes in this emerging population is of importance. We report results from a multicenter observational study that characterizes adults with Fontan palliation for underlying HLHS at entry into adulthood and defines factors associated with adverse outcomes. Adults (≥18 years by July 2014) with HLHS were studied. The diagnosis of HLHS was restricted to patients with mitral stenosis or atresia, aortic stenosis or atresia, and a diminutive left ventricle. Excluded were patients with other forms of dominant right ventricular lesions with univentricular physiology managed with a Norwood procedure (ie, unbalanced atrioventricular septal defect or double-outlet right ventricle) or those with heart transplantation. Available pediatric and adult cardiovascular data were reviewed. The primary end point (Fontan-specific major adverse cardiovascular event) was defined as death, transplantation listing, hospitalization (>24 hours) for heart failure requiring intravenous diuretics, sustained ventricular …

Introduction: In addition to understanding causes of morbidity and mortality following tetralogy of Fallot repair (rTOF), the importance of patient-reported outcomes (PROs) such as quality of life (QOL) is increasingly being recognized. Hypothesis: We hypothesized that PROs in rTOF would be associated with selected sociodemographic factors, functional status, and/or clinical variables. Methods: As part of a prospective study of patients ≥12 years with rTOF and significant pulmonary regurgitation, participants completed PRO surveys: SF-12 Health Status (physical component summary [PCS] and mental component summary [MCS] t-scores), EQ-5D (5 dimensions: mobility, self-care, usual activities, pain/discomfort, and anxiety/depression), and a 0-100 QOL linear analogue scale (QOL-LAS). With multivariable regression analysis, we determined the association between PROs and selected predictors. Results: We studied n=627 participants (55% male, 26±13 years, 75% adult) at 14 sites (North America, Europe and Asia). Median age at repair was 1.6 years (IQR 0.6, 4.2). In general, scores on the SF-12 and QOL-LAS suggested good outcomes: SF-12 PCS Conclusions: Age and NYHA functional class at enrollment were found to be predictors of PROs in rTOF. While global measures of health status and QOL (SF-12 and QOL-LAS) suggest relatively good PROs, this study demonstrates the importance of inquiring about specific health problems (EQ-5D) to achieve a richer understanding of PROs in order to potentially maximize patient well-being.

Introduction: Late complications after tetralogy of Fallot repair (rTOF) often arise from chronic pulmonary regurgitation (PR). Prospective studies of outcomes in those with rTOF and significant PR are lacking. Hypothesis: We hypothesized that cardiovascular magnetic resonance imaging (CMR) imaging measurements would be associated with poor outcomes in an “at risk” population with rTOF and ≥moderate PR. Methods: This was a prospective, international study (North America, Europe and Asia) of children and adults. Inclusion criteria were rTOF in childhood, age ≥12 years, ≥moderate PR, and CMR within 18 months of enrollment. All CMR analysis occurred in a central core laboratory. Patient reported outcomes were collected using standardized tools. The primary clinical outcome was death or sustained (>30 seconds) ventricular tachycardia (VT) recorded at 3 year follow-up. Results: We enrolled 629 patients (55% male) at 29±14 years (2013-2017). Primary rTOF was done at 1.6 years (IQR 0.6,4.2) with a transannular patch in 55%. By standardized symptom assessment, 78% were asymptomatic (NYHA 1). Baseline CMR findings included PR fraction 38±14%, right ventricular (RV) end-diastolic volume indexed (EDVi) 155±41mL/m2, RV ejection fraction (EF) 44±7%, RV mass indexed 59±16 g/m2, left ventricular (LV) EDVi 85±18mL/m2, LVEF 55±7% and LV mass indexed 88±27 g/m2. Outcomes at 3 years were recorded (available in n=101). During follow-up, surgical pulmonary valve replacement (PVR) occurred in 38 patients (38%). In the PVR group compared with the non-PVR group, baseline RVEDVi was larger (189 vs. 152 mL/m2, p Conclusions: Despite significant PR, most patients with rTOF were asymptomatic. At 3 year follow-up, PVR occurred in more than one third of patients. Death or VT was observed in a small number of patients who had higher RV and LV mass on baseline CMR.

The use of venoarterial extracorporeal membrane oxygenation (VA-ECMO) for the support of critically ill patients with cardiogenic shock is rapidly increasing. Intracardiac thrombus formation is a well-recognized complication. We present 3 cases of dramatic intracardiac thrombosis after the initiation of VA-ECMO. A 64-year-old man presented to a community hospital 3 days after the onset of chest pain with ECG evidence of anterior ST-elevation–myocardial infarction and clinical findings of cardiogenic shock. Physical examination was notable for a pansystolic murmur suggestive of a ventricular septal defect. Coronary angiography showed an occluded left anterior descending artery. Echocardiography confirmed an ischemic ventricular septal defect. He was taken urgently to the operating room for aortocoronary bypass and ventricular septal defect patch repair. Attempts to wean off cardiopulmonary bypass were unsuccessful, and the patient was placed on central VA-ECMO as a bridge to decision, with the chest left open. Forty-eight hours after the initiation of ECMO, the circuit was converted to a peripheral set-up to facilitate chest closure. Transesophageal echocardiogram performed 24 hours after chest closure showed complete thrombosis of the right ventricle despite full anticoagulation (Figure [A]; Movie I in the Data Supplement). He was taken back …

Background and Purpose— To assess whether prophylactic postoperative intraaortic balloon counterpulsation (IABC) reduces the risk of poor outcome because of vasospasm following aneurysmal subarachnoid haemorrhage relative to conventional hypervolemic therapy (HT). Methods— This was a single-center, parallel group randomized controlled trial. Patients suffering a subarachnoid hemorrhage at high risk of vasospasm were eligible. Patients were randomly allocated to receive prophylactic IABC (n=35) or HT (n=36). The primary end point was Glasgow Outcome and SF-36 scores assessed at 6 months by a blinded and independent observer and analyzed by intention to treat. Secondary analysis of physiological parameters was by treatment performed. Results— Twenty-seven patients in each arm had a good outcome ( P =0.55). There was no statistical difference in mean SF-36 score (t=0.39, P =0.70). There were no long-term complications secondary to IABC. There were no differences in preload (pulmonary artery wedge pressure, P =0.97) or afterload (mean arterial pressure, P =0.97). IABC was associated with a lower cardiac output ( P =0.002) and higher systemic vascular resistance ( P =0.005), although for both groups mean cardiac output was >6 L/min. Cerebral blood flow was not different between groups: HT=41.5 (SD 7.2), IABP=44.9 (SD 8.6) mL/100 g/min ( P =0.14). Conclusions— In this study, prophylactic IABC did not improve perfusion indices or confer any clinical benefit following subarachnoid haemorrhage in patients with normal cardiac function. The study was small, however, and cannot be extrapolated to patients with cardiac failure and medically refractory symptomatic cerebral vasospasm. Clinical Trial Registration— This trial was not registered because enrolment began prior to July 1, 2005.

Background: We have shown that giving a low dose of lipopolysaccharide (LPS) to P7 rat pups will result in 90% reduction in ischemia-induced brain damage. This dramatic LPS neuroprotection was observed in P7rat pups which correspond in terms of brain development to human term newborn but not in P3 or P5 pups which correspond to human premature infant. LPS is a known Toll-like receptor 4 (TLR-4) ligand. We hypothesized that TLRs other than TLR-4 may mediate preconditioning against cerebral ischemic injury in the developing brain Methods: Brains from P5 and P7 rats were removed, fixed in 10% formalin, embedded in paraffin and cut in 5μ m coronal sections. TLR-3 and TLR-4 expression was assessed by immunohistochemistry. In subsequent experiment, P5 Rat pups were randomly assigned to TLR-3 specific agonist, poly I:C, treated group (20μ g; IP) or saline treated group. Forty eight hours after the injections, hypoxic-ischemic injury was induced by using the Rice-Vannucci model. Unilateral internal carotid artery ligation in rat pups followed by exposure to 8% hypoxia for approximately 65min will cause a reproducible unilateral infarct ipsilateral to the ligated artery involving caudate, putamen, hippocampus and cortex. Body temperature will be maintained at 37-37.5 0 C during hypoxia using an incubator. Brains were removed 1 week after HI injury, fixed, embedded in paraffin and cut in 5μ m coronal sections. Brain sections were then stained with Hematoxylin and Eosin and infarct volumes were compared between the two groups using Nikon NIS-Element Basic Research Image analysis software system Results: TLR-4 was highly expressed in brains of P7 but not in P5 rat pups. The number of TLR-4 positive cells was significantly lower in P5 rat brains compared to P7 pups ( p < 0.05). TLR-3 was predominately expressed in P5 ( p < 0.05). There was a significant reduction in infarct volume ( p = 0.01) in poly I:C P5 treated pups (6.6mm 3 + SEM 3) compared to saline treated P5 pups (26mm 3 + SEM 6). Conclusion: This is the first study to show that TLR-3 is expressed in the immature brain and mediates preconditioning against ischemic injury. LPS-mediated TLR-4 activation is necessary for inducing preconditioning in P7 rats whereas poly I:C is effective in protecting P5 pups where TLR-3 expression predominates. Therefore, the role of TLRs in protecting the brain from ischemic injury is developmentally determined.