Background The empirically derived 2014 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model incorporates adjustment for procedure type and patient-specific factors. ...The purpose of this report is to describe this model and its application in the assessment of variation in outcomes across centers. Methods All index cardiac operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database (January 1, 2010, to December 31, 2013) were eligible for inclusion. Isolated patent ductus arteriosus closures in patients weighing less than or equal to 2.5 kg were excluded, as were centers with more than 10% missing data and patients with missing data for key variables. The model includes the following covariates: primary procedure, age, any prior cardiovascular operation, any noncardiac abnormality, any chromosomal abnormality or syndrome, important preoperative factors (mechanical circulatory support, shock persisting at time of operation, mechanical ventilation, renal failure requiring dialysis or renal dysfunction (or both), and neurological deficit), any other preoperative factor, prematurity (neonates and infants), and weight (neonates and infants). Variation across centers was assessed. Centers for which the 95% confidence interval for the observed-to-expected mortality ratio does not include unity are identified as lower-performing or higher-performing programs with respect to operative mortality. Results Included were 52,224 operations from 86 centers. Overall discharge mortality was 3.7% (1,931 of 52,224). Discharge mortality by age category was neonates, 10.1% (1,129 of 11,144); infants, 3.0% (564 of 18,554), children, 0.9% (167 of 18,407), and adults, 1.7% (71 of 4,119). For all patients, 12 of 86 centers (14%) were lower-performing programs, 67 (78%) were not outliers, and 7 (8%) were higher-performing programs. Conclusions The 2014 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model facilitates description of outcomes (mortality) adjusted for procedural and for patient-level factors. Identification of low-performing and high-performing programs may be useful in facilitating quality improvement efforts.
Beginning in 2000 and accelerating in 2004, the Berlin Heart EXCOR (Berlin Heart Inc Woodlands, TX) became the first pediatric-specific ventricular assist device (VAD) applied throughout North ...America for children of all sizes. This retrospective study analyzed the initial Berlin Heart EXCOR pediatric experience as a bridge to transplantation.
Between June 2000 and May 2007, 97 EXCOR VADs were implanted in North America at 29 different institutions. The analysis is limited to 73 patients (75%) from 17 institutions, for which retrospective data were available.
Median age and weight at VAD implant were 2.1 years (range, 12 days-17.8 years) and 11 kg (range, 3-87.6 kg), respectively. The primary diagnoses were dilated cardiomyopathy in 42 (58%), congenital heart disease in 19 (26%), myocarditis in 7 (10%), and other cardiomyopathies in 5 (7%). Pre-implant clinical condition was critical cardiogenic shock in 38 (52%), progressive decline in 33 (45%), or other in 2 (3%). Extracorporeal membrane oxygenation was used as a bridge to EXCOR in 22 patients (30%). Device selection was left VAD (LVAD) in 42 (57%) and biventricular assist devices (BiVAD) in 31 (43%). The EXCOR bridged 51 patients (70%) to transplant and 5 (7%) to recovery. Mortality on the EXCOR was 23% (n = 17) overall, including 35% (11 of 31) in BiVAD vs 14% (6 of 42) in LVAD patients (p = 0.003). Multivariate analysis showed younger age and BiVAD support were significant risk factors for death while on the EXCOR.
This limited but large preliminary North American experience with the Berlin Heart EXCOR VAD as a bridge to cardiac transplantation for children of all ages and sizes points to the feasibility of this approach. The prospective investigational device evaluation trial presently underway will further characterize the safety and efficacy of the EXCOR as a bridge to pediatric cardiac transplantation.
Background An update of evidence-based guidelines concerning liberation from mechanical ventilation is needed as new evidence has become available. The American College of Chest Physicians (CHEST) ...and the American Thoracic Society (ATS) have collaborated to provide recommendations to clinicians concerning liberation from the ventilator. Methods Comprehensive evidence syntheses, including meta-analyses, were performed to summarize all available evidence relevant to the guideline panel’s questions. The evidence was appraised using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach, and the results were summarized in evidence profiles. The evidence syntheses were discussed and recommendations developed and approved by a multidisciplinary committee of experts in mechanical ventilation. Results Recommendations for three population, intervention, comparator, outcome (PICO) questions concerning ventilator liberation are presented in this document. The guideline panel considered the balance of desirable (benefits) and undesirable (burdens, adverse effects, costs) consequences, quality of evidence, feasibility, and acceptability of various interventions with respect to the selected questions. Conditional (weak) recommendations were made to use inspiratory pressure augmentation in the initial spontaneous breathing trial (SBT) and to use protocols to minimize sedation for patients ventilated for more than 24 h. A strong recommendation was made to use preventive noninvasive ventilation (NIV) for high-risk patients ventilated for more than 24 h immediately after extubation to improve selected outcomes. The recommendations were limited by the quality of the available evidence. Conclusions The guideline panel provided recommendations for inspiratory pressure augmentation during an initial SBT, protocols minimizing sedation, and preventative NIV, in relation to ventilator liberation.
Hot Topics in Tetralogy of Fallot Villafañe, Juan, MD; Feinstein, Jeffrey A., MD; Jenkins, Kathy J., MD, MPH ...
Journal of the American College of Cardiology,
12/2013, Letnik:
62, Številka:
23
Journal Article
Recenzirano
Odprti dostop
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore “hot topics” to highlight areas of emerging science for clinicians and scientists in moving toward a better ...understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.
Abstract Objective To determine the incidence and risk factors for endocarditis and reintervention in patients undergoing placement of right ventricle-to-pulmonary artery valve conduits. Methods All ...right ventricle–to–pulmonary artery valved conduits placed between 1995 and 2014 were included. Freedom from endocarditis, reintervention, and replacement were analyzed using the Kaplan-Meier method and parametric survival regression models. Results A total of 586 patients underwent placement of a total of 792 valved conduits, including 289 (36%) pulmonary homografts, 121 (15%) aortic homografts, 245 (31%) bovine jugular grafts, and 137 (17%) porcine heterografts. There were 474 (60%) primary placements and 318 (40%) replacements. The median duration of conduit follow-up was 7 years; 23 conduits developed endocarditis at a median of 5 years after surgery. The use of bovine jugular grafts was the sole significant risk factor associated with endocarditis (hazard ratio, 9.05; 95% confidence interval, 2.6–31.8 compared with homografts). The hazard was greater for bovine jugular grafts compared with the other conduit types and increased with time; however, bovine jugular grafts were associated with a lower risk for reintervention ( P < .0001) and replacement ( P = .0002). Factors associated with greater risk of both reintervention and replacement were younger age and smaller conduit size. In addition, a diagnosis of truncus arteriosus was associated with a greater risk for replacement ( P = .03). Conclusions Bovine jugular grafts are associated with a significantly greater risk of late endocarditis but with lower reintervention rates compared with other valved conduits. The risk of endocarditis and durability must be balanced during conduit selection. Antibiotic prophylaxis and a high index of suspicion for endocarditis are warranted in patients with bovine jugular grafts.
The aim of this study was to show the clinical data of long-term (3-year) follow-up of 5 patients affected by Leber congenital amaurosis type 2 (LCA2) treated with a single unilateral injection of ...adeno-associated virus AAV2-hRPE65v2.
Clinical trial.
Five LCA2 patients with RPE65 gene mutations.
After informed consent and confirmation of trial eligibility criteria, the eye with worse visual function was selected for subretinal delivery of adeno-associated virus (AAV2-hRPE65v2). Subjects were evaluated before and after surgery at designated follow-up visits (1, 2, 3, 14, 30, 60, 90, 180, 270, and 365 days, 1.5 years, and 3 years) by complete ophthalmic examination. Efficacy for each subject was monitored with best-corrected visual acuity, kinetic visual field, nystagmus testing, and pupillary light reflex.
Best-corrected visual acuity, kinetic visual field, nystagmus testing, and pupillary light reflex.
The data showed a statistically significant improvement of best-corrected visual acuity between baseline and 3 years after treatment in the treated eye (P<0.001). In all patients, an enlargement of the area of visual field was observed that remained stable until 3 years after injection (average values: baseline, 1058 deg(2) vs. 3 years after treatment, 4630 deg(2)) and a reduction of the nystagmus frequency compared with baseline at the 3-year time point. Furthermore, a statistically significant difference was observed in the pupillary constriction of the treated eye (P<0.05) compared with the untreated eye in 3 patients at 1- and 3-year time points. No patients experienced serious adverse events related to the vector in the 3-year postinjection period.
The long-term follow-up data (3 years) on the 5-patient Italian cohort involved in the LCA2 gene therapy clinical trial clearly showed a stability of improvement in visual and retinal function that had been achieved a few months after treatment. Longitudinal data analysis showed that the maximum improvement was achieved within 6 months after treatment, and the visual improvement was stable up to the last observed time point.
Proprietary or commercial disclosure may be found after the references.
Background We sought to evaluate the impact of the evolution of a pediatric mechanical circulatory support (MCS) program on outcomes of children listed for heart transplantation at our institution. ...Methods All patients listed for isolated heart transplantation from 1995 to 2013 were included. The use of MCS while on the wait-list was recorded. Wait-list and posttransplant outcomes were compared before and after 2005, which was when we became capable of providing long-term MCS without size limitation. Results In total, 259 patients were listed for transplant and 201 (78%) reached transplant. The use of MCS was significantly increased between the eras (13% and 37%, p = 0.0001). Wait-list mortality was significantly decreased (25% and 11%, p = 0.0006). Among transplant recipients, the proportion of patients who underwent MCS was significantly increased (13% and 37%, p = 0.0002). Of these MCS patients, the use of long-term devices was significantly increased (50% and 98%, p = 0.0004). Median duration of MCS was significantly increased (12 and 78 days, p = 0.004). Kaplan-Meier estimates showed a trend ( p = 0.08) toward improved survival after bridge-to-transplant both at 1 year (70% in the early era and 88% in the late era) and at 5 years (60% and 78%, respectively). Conclusions Outcomes of pediatric heart transplantation have significantly improved over the last 2 decades. We believe such improvement is, at least in part, attributable to maturation of MCS strategy, characterized by avoiding the use of temporary devices such as extracorporeal membrane oxygenation as a bridge-to-transplant and a more aggressive use of long-term devices.
Background New intraparenchymal brain injury on magnetic resonance imaging is observed in 36% to 73% of neonates after cardiac surgery with cardiopulmonary bypass. Brain immaturity in this population ...is common. We performed brain magnetic resonance imaging before and after neonatal cardiac surgery, using a high-flow cardiopulmonary bypass protocol, hypothesizing that brain injury on magnetic resonance imaging would be associated with brain immaturity. Methods Cardiopulmonary bypass protocol included 150 mL · kg−1 · min−1 flows, pH stat management, hematocrit > 30%, and high-flow antegrade cerebral perfusion. Regional brain oxygen saturation was monitored, with a treatment protocol for regional brain oxygen saturation < 50%. Brain magnetic resonance imaging, consisting of T1-, T2-, and diffusion-weighted imaging, and magnetic resonance spectroscopy were performed preoperatively, 7 days postoperatively, and at age 3 to 6 months. Results Twenty-four of 67 patients (36%) had new postoperative white matter injury, infarction, or hemorrhage, and 16% had new white matter injury. Associations with preoperative brain injury included low brain maturity score ( P = .002). Postoperative white matter injury was associated with single-ventricle diagnosis ( P = .02), preoperative white matter injury ( P < .001), and low brain maturity score ( P = .05). Low brain maturity score was also associated with more severe postoperative brain injury ( P = .01). Forty-five patients had a third scan, with a 27% incidence of new minor lesions, but 58% of previous lesions had partially or completely resolved. Conclusions We observed a significant incidence of both pre- and postoperative magnetic resonance imaging abnormality and an association with brain immaturity. Many lesions resolved in the first 6 months after surgery. Timing of delivery and surgery with bypass could affect the risk of brain injury.
A unique form of lead failure has been described in the Riata (8-F) and Riata ST (7-F) silicone defibrillation lead degradation of the outer insulation, resulting in the externalization of conductor ...cables.
To assess rates of lead revision due to lead failure in Riata leads affected by the Riata advisory.
Nineteen implantable cardioverter-defibrillator implant and follow-up centers were surveyed.
As of March 1, 2012, there were 5043 known affected leads implanted in Canada. Data on 4358 (86.4%) leads were obtained; 65.3% of these were Riata (8-F) and 32.4% were Riata ST (7-F) leads. The median time from implant to last follow-up was 5 years. Electrical abnormalities were reported in 4.6% of the affected leads; 8.0% of these were found to have concomitant radiographic evidence of externalization. The rate of electrical failure was higher in the 8-F (5.2%) vs 7-F (3.3%) leads (P = .007). Oversensing with or without inappropriate shocks was reported in 39.8% of the leads with confirmed failure. Abnormally high or low impedance values (29.9%) and elevated pacing capture thresholds (43.8%) were frequently reported. One death (0.5%) attributed to lead failure was reported. Among the leads that were replaced, 21% were extracted. Two major complications (1.0%) were attributed to extraction of these leads.
The overall rate of lead failure in the Riata (8-F) and Riata ST (7-F) leads is higher than previously reported by using passive surveillance data. The impact of recent advisories related to these leads is not yet apparent.
Background Currently, there are no Food and Drug Administration–approved devices available that can provide long-term mechanical circulatory support to smaller children with severe heart failure as a ...bridge to heart transplant (HT). In recent years, the Berlin Heart EXCOR Pediatric ventricular assist device (VAD) has emerged as a potential treatment option. Systematic data on the safety and efficacy of the EXCOR are limited. Methods The Investigational Device Exemption (IDE) clinical study is designed to evaluate the safety and probable benefit of the EXCOR to support regulatory review of the device under the Humanitarian Device Exemption regulation. The study design and rationale are reviewed in light of the well-described challenges inherent in small population studies. Results The Berlin Heart EXCOR IDE clinical study is a prospective, multicenter, single-arm, clinical cohort study. Children aged 0 to 16 years with severe heart failure (Interagency Registry for Mechanically Assisted Circulatory Support profile 1 or 2) due to 2-ventricle heart disease and actively listed for HT comprise the primary study cohort. The control population is a propensity-matched retrospective cohort of children supported with extracorporeal membrane oxygenation, the only bridge device available to smaller children before the EXCOR. The primary efficacy end point is survival to heart transplantation or recovery. The primary safety end point is the incidence of serious adverse events as defined by pediatric Interagency Registry for Mechanically Assisted Circulatory Support criteria. The study will enroll a total of 48 subjects in 2 cohorts based on body surface area (cohort 1 <0.7 m2 , cohort 2 0.7-1.5 m2 ) and is powered to show safety superiority to a prespecified performance goal of 0.25 serious adverse events per day of support. Children ineligible for the primary cohort will still have access to the device in a third compassionate-use cohort where adverse event data will be collected for additional safety characterization of the device. Conclusion The Berlin Heart IDE clinical study will be the first bridge-to-HT VAD study designed exclusively for children. It is anticipated that the study will provide important information on the safety and efficacy of the Berlin Heart EXCOR Pediatric in children while providing valuable lessons into the design and conduct of future VAD studies in children.
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