Clinical Presentation and Therapy of Tricuspid Atresia and Univentricular Heart.

Although the terms "single ventricle" and "univentricular heart" are frequently used to describe a variety of complex congenital heart defects, in fact, nearly all hearts have two ventricles, although one of the two may be too small to be functional. A better term for these hearts would therefore be "functional single ventricle."

Ability of Video Telemetry to Predict Unplanned Hospital Admissions for Single Ventricle Infants.

Background Our Cardiac High Acuity Monitoring Program (CHAMP) uses home video telemetry (HVT) as an adjunct to monitor infants with single ventricle during the interstage period. This study describes the development of an objective early warning score using HVT, for identification of infants with single ventricle at risk for clinical deterioration and unplanned hospital admissions (UHA). Methods and Results Six candidate scoring parameters were selected to develop a pragmatic score for routine evaluation of HVT during the interstage period. We evaluated the individual and combined ability of these parameters to predict UHA. All infants with single ventricle monitored at home by CHAMP between March 2014 and March 2018 were included. Videos obtained within 48 hours before UHA were compared with videos obtained at baseline. We used binary logistic regression models and receiver operating characteristic curves to evaluate the parameters' performance in discriminating the outcome of interest. Thirty-nine subjects with 64 UHA were included. We compared 64 pre-admission videos to 64 paired baseline videos. Scoring was feasible for a mean of 91.6% (83.6%-98%) of all observations. Three different HVT score models were proposed, and a final model composed of respiratory rate, respiratory effort, color, and behavior exhibited an excellent discriminatory capability with an area under the receiver operating characteristic curve of 93% (89%-98%). HVT score of 5 was associated with specificity of 93.8% and sensitivity of 88.7% in predicting UHA. Conclusions We developed a feasible and reproducible HVT score that can serve as a tool to predict UHA in infants with single ventricle. Future directions involve prospective, multicenter validation of this tool.

Integrated Multimodality Telemedicine to Enhance In-Home Care of Infants During the Interstage Period.

Performing interstage home monitoring using digital platforms (teleIHM) is becoming commonplace but, when used alone, may still require frequent travel for in-person care. We evaluated the acceptability, feasibility, and added value of integrating teleIHM with synchronous telemedicine video visits (VVs) and asynchronous video/photo sharing (V/P) during the interstage period. We conducted a descriptive program evaluation of patient-families receiving integrated multimodality telemedicine (teleIHM + VV + V/P) interstage care from 7/15/2018 to 05/15/2020. First, provider focus groups were conducted to develop a program logic model. Second, patient characteristics and clinical course were reviewed and analyzed with univariate statistics. Third, semi-structured qualitative interviews of family caregivers' experiences were assessed using applied thematic analysis. Within the study period, 41 patients received teleIHM + VV + V/P care, of which 6 were still interstage and 4 died. About half (51%) of patients were female and 54% were a racial/ethnic minority. Median age was 42 days old (IQR 25, 58) at interstage start, with a median of 113 total days (IQR 72, 151). A total of 551 VVs were conducted with a median 12 VVs (IQR 7, 18) per patient. Parents sent a median 2 pictures (IQR 0-3, range 0-82). Qualitatively, families reported an adjustment period to teleIHM, but engaged favorably with telemedicine overall. Families felt reassured by the oversight routine telemedicine provided and identified logistical and clinical value to VVs above teleIHM alone, while acknowledging trade-offs with in-person care. Integration of multimodality telemedicine is a feasible and acceptable approach to enhance in-home care during the interstage period.

Characterization of Extracorporeal Membrane Oxygenation Support for Single Ventricle Patients.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) can provide crucial support for single ventricle (SV) patients at various stages of palliation. However, characterization of the utilization and outcomes of ECMO in these unique patients remains incompletely studied. METHODS: We performed a single-center retrospective review of SV patients between 2010 and 2017 who underwent ECMO cannulation with primary end point of survival to discharge and secondary end point of survival to decannulation or orthotopic heart transplantation (OHT). Multivariate analysis was performed for factors predictive of survival to discharge and survival to decannulation. RESULTS: Forty SV patients with a median age of one month (range: 3 days to 15 years) received ECMO support. The incidence of ECMO was 14% for stage I, 3% for stage II, and 4% for stage III. Twenty-seven (68%) patients survived to decannulation, and 21 (53%) patients survived to discharge, with seven survivors to discharge undergoing OHT. Complications included infection (40%), bleeding (40%), thrombosis (33%), and radiographic stroke (45%). Factors associated with survival to decannulation included pre-ECMO lactate (hazard ratio [HR]: 0.61, 95% confidence interval [CI]: 0.41-0.90, P = .013) and post-ECMO bicarbonate (HR: 1.24, 95% CI: 1.0-1.5, P = .018). Factors associated with survival to discharge included central cannulation (HR: 40.0, 95% CI: 3.1-500.0, P = .005) and lack of thrombotic complications (HR: 28.7, 95% CI: 2.1-382.9, P = .011). CONCLUSIONS: Extracorporeal membrane oxygenation can be useful to rescue SV patients with approximately half surviving to discharge, although complications are frequent. Early recognition of the role of heart transplant is imperative. Further study is required to identify areas for improvement in this population.

Bridge to Transplant with Ventricular Assist Device Support in Pediatric Patients with Single Ventricle Heart Disease.

Ventricular assist device (VAD) support for children with single ventricle (SV) heart disease remains challenging. We performed a single-center retrospective review of SV patients on VAD support and examined survival to transplant using the Kaplan-Meier method. Patients transplanted were compared with those who died on support. Between 2009 and 2017, there were 14 SV patients with 1,112 patient-days of VAD support. Stages of palliation included pre-Glenn (n = 5), Glenn (n = 5), and Fontan (n = 4). Eight patients (57%) were successfully bridged to transplant at a median 107 days. Deaths occurred early (n = 6, median 16 days) and in smaller patients (10.1 vs. 28.3 kg, P = 0.04). All Fontan patients survived to transplant, whereas only 20% of Glenn patients survived to transplant. Adverse events occurred in 79% (n = 11). Five patients met hospital discharge criteria, with two patients (one pre-Glenn, one Glenn) discharged and transplanted after 219 and 174 days of VAD support. All transplanted patients were discharged at a median 21 days posttransplant. SV patients in various stages of palliation can be successfully bridged to transplant with VAD support. With use of intracorporeal continuous-flow devices, longer-term support and hospital discharge are possible.

Current evaluation and management of plastic bronchitis in the pediatric population.

OBJECTIVE: To describe a multidisciplinary approach for the treatment of plastic bronchitis (PB) in children. METHODS: Retrospective chart review of children with PB between 1997 and 2017. Data regarding clinical presentation, diagnosis, management, and outcomes were analyzed. RESULTS: Of 34 patients presenting with PB, 24 had single ventricle (SV) heart disease, 9 had pulmonary disease, and one had no underlying disease. Median (IQR: interquartile range) age at the time of PB diagnosis was 5.5 years (IQR: 9.0). Presenting symptoms included cough productive of casts (n = 27, 79%), wheezing (n = 5, 15%), dyspnea (n = 18, 53%), hypoxia (n = 31, 91%), and respiratory failure (n = 9, 26%). Diagnosis was made based on clinical evaluation, bronchoscopy findings, and/or pathology of casts. Treatment methods included bronchoscopy for cast removal (25% of SV patients, 91% of non-SV patients), chest physiotherapy (SV: 92%, non-SV: 45%), albuterol (SV: 79%, non-SV: 73%), inhaled steroids (SV: 75%, non-SV: 18%), nebulized hypertonic saline (SV: 29%, non-SV: 9%), nebulized heparin (SV: 8%, non-SV: 55%), nebulized tissue plasminogen activator (tPA; SV: 33%, non-SV: 9%), inhaled Dornase Alfa (SV: 54%, non-SV: 9%), antibiotics (SV: 46%, non-SV: 45%), systemic steroids (SV: 13%, non-SV: 45%), and lymphatic embolization (SV: 8%, non-SV: 45%). Of SV patients, 11 had no recurrence, 5 underwent heart transplantation, one awaits transplant, and 3 died due to cardiac disease. Three patients with respiratory disease had recurrent PB and one died from MRSA pneumonia. CONCLUSION: PB is a highly morbid disease with limited treatment options. Bronchoscopy and chest physiotherapy for airway clearance are among the most-utilized therapies.

Early Integration of Palliative Care in Families of Children with Single Ventricle Congenital Heart Defects: A Quality Improvement Project to Enhance Family Support.

Children with single ventricle congenital heart defects (SVCHD) experience a significant risk of early mortality throughout their lifespan, particularly during their first year of life. Due to the intense care needed for these children and families, pediatric palliative care (PPC) team consults should be routine; however, medical staff are often reluctant to broach the idea of PPC to families. The involvement of PPC for many carries with it an association to end-of-life (EOL) care. Setting the standard of PPC involvement from the time of admission for the first palliative surgery led to increased family support, decreased days to consult, improved acceptance and communication. The purpose of this article is to describe a quality improvement project of early integration of PPC with families of children with SVCHD. Lessons learned will be presented, including the resources needed and the barriers encountered in assimilating PPC into the standard of care for all patients with SVCHD. The single ventricle (SV) and PPC teams collaborated to enhance the support given to SV families. Education was initiated with cardiology and PPC providers to understand the goal of consistent PPC consults beginning after birth for patients with SVCHD. Parents were educated during fetal consultation regarding the involvement of the PPC team. The SV team ensured compliance with the PPC initiative by identifying eligible patients and requesting consult orders from the primary providers. PPC consultation increased significantly over the 40 month study period to nearly 100% compliance for children with SVCHD who are undergoing pre-Fontan surgery. In addition, mean days to consult decreased dramatically during the study to a current average of 3 days into the patient's hospitalization; the data likely suggest that more PPC consults were routinely ordered versus urgently placed for unexpected complications. Data indicate that patients are being followed by the PPC team at an earlier age and stage in their SV journey which allows for more opportunity to provide meaningful support to these patients and families. The early involvement of the PPC team for children with SV physiology was operationally feasible and was accepted by families, thus allowing PPC providers to establish a therapeutic relationship early in the disease trajectory with the family. It allowed more continuity throughout the SV journey in a proactive fashion rather than a reactive manner.

Economic implications of outpatient cardiac catheterisation in infants with single ventricle congenital heart disease.

BACKGROUND: Resource utilisation for infants with single ventricle CHD remains high without well-studied ways to decrease economic burden. Same-day discharge following cardiac catheterisation has been shown to be safe and effective in children with CHD, but those with single ventricle physiology are commonly excluded. The purpose of this study was to investigate the economic implications of planned same-day discharge following cardiac catheterisation versus universal overnight hospital admission in infants with single ventricle CHD. METHODS AND RESULTS: A probabilistic decision-tree analysis with sensitivity analyses was performed. All included patients were categorised into four possible outcomes; discharge, readmission following discharge (within 48 hours), observation and prolonged hospitalisation. Baseline probabilities of each node of the tree were then combined with the cost data to evaluate the comparative dominance of one decision (immediately discharge) versus the other decision (routinely admit). Patients discharged on the same day as the procedure accrued the lowest attributed hospital cost ($5469), while patients readmitted to the hospital had the highest attributed cost ($11,851). Currently, no other studies have assessed the cost of hospitalisation following cardiac catheterisation in this population. Thus, we allowed for a wide range of cost variation, but same-day discharge dominated the decision outcome with a lower economic burden. CONCLUSION: Same-day discharge following routine cardiac catheterisation in patients with single ventricle physiology is less costly compared to universal overnight admission. This demonstrates an important cost-limiting step in a complex population of patients who have high resource utilisation.

Outcome of patients with functional single ventricular heart after pacemaker implantation: What makes it poor, and what can we do?

BACKGROUND: Pacemaker implantation in patients with single ventricle is associated with poor outcomes. OBJECTIVE: The purpose of this study was to determine the reasons for the poor outcomes of pacemaker implantation. METHODS: We performed a retrospective chart review of patients with single ventricle who had undergone permanent pacemaker implantation. Patients were categorized into 3 groups based on the site of pacing and the proportion of ventricular pacing (VP) as follows: (1) atrial pacing group with atrial pacing only (n = 11); (2) low VP group with low daily VP proportion (<50%; n = 12); and (3) high VP group with high daily VP proportion (≥50%; n = 15). Pacing leads were placed at the epicardium in all patients. RESULTS: No patients in the atrial pacing or low VP groups died, whereas the survival rate in the high VP group was 58.9% and 39.3% at 10 and 20 years, respectively, after pacemaker implantation. Among the post-Fontan patients, plasma brain natriuretic peptide (BNP) levels significantly increased with the proportion of VP: 11.7, 20.3, and 28.4 pg/mL in the atrial pacing, low VP, and high VP groups, respectively (P = 0.04). In the high VP group, the plasma BNP level was significantly lower in patients with an apical pacing lead than in those with a nonapical pacing lead (27.0 pg/mL vs 82.8 pg/mL, respectively; P = .03). CONCLUSION: A higher proportion of VP was associated with poor outcome and higher plasma BNP levels, probably due to ventricular dyssynchrony. In epicardial ventricular pacing, apical pacing is better to avoid the increase in ventricular stress and plasma BNP level.

Early prediction of critical events for infants with single-ventricle physiology in critical care using routinely collected data.

OBJECTIVE: Critical events are common and difficult to predict among infants with congenital heart disease and are associated with mortality and long-term sequelae. We aimed to achieve early prediction of critical events, that is, cardiopulmonary resuscitation, emergency endotracheal intubation, and extracorporeal membrane oxygenation in infants with single-ventricle physiology before second-stage surgery. We hypothesized that naïve Bayesian models learned from expert knowledge and clinical data can predict critical events early and accurately. METHODS: We collected 93 patients with single-ventricle physiology admitted to intensive care units in a single tertiary pediatric hospital between 2014 and 2017. Using knowledge elicited from experienced cardiac-intensive-care-unit providers and machine-learning techniques, we developed and evaluated the Cardiac-intensive-care Warning INdex (C-WIN) system, consisting of a set of naïve Bayesian models that leverage routinely collected data. We evaluated predictive performance using the area under the receiver operating characteristic curve, sensitivity, and specificity. We performed the evaluation at 5 different prediction horizons: 1, 2, 4, 6, and 8 hours before the onset of critical events. RESULTS: The area under the receiver operating characteristic curves of the C-WIN models ranged between 0.73 and 0.88 at different prediction horizons. At 1 hour before critical events, C-WIN was able to detect events with an area under the receiver operating characteristic curve of 0.88 (95% confidence interval, 0.84-0.92) and a sensitivity of 84% at the 81% specificity level. CONCLUSIONS: Predictive models may enhance clinicians' ability to identify infants with single-ventricle physiology at high risk of critical events. Early prediction of critical events may indicate the need to perform timely interventions, potentially reducing morbidity, mortality, and health care costs.