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.

Annuloplasty for Aortic Regurgitation in Univentricular Heart on Ventricular Assist Support.

Progressive aortic regurgitation is a common complication in patients supported with continuous flow ventricular assist devices. The risk of neoaortic regurgitation is likely to be particularly high in patients with univentricular hearts owing to the lack of fibrous support for the neoaortic valve. Previously described techniques for addressing neoaortic regurgitation in this setting have required cardiopulmonary bypass and cross clamping, or ligation of the neoaortic root. We present a simple technique of external partial annuloplasty of the neoaortic valve, which can be performed without the need for cardiopulmonary bypass or cross clamping.

Evaluación de la capacidad funcional en escolares con fisiología de corazón univentricular / Assessment of functional capacity among students with univentricular heart

Se realizó un estudio transversal en escolares con corazón univentricular en estadio pos-bypass total de ventrículo derecho con el objetivo de determinar la capacidad funcional basal mediante el test de marcha en 6 minutos e identificar posibles factores determinantes. Participaron 30 pacientes con una mediana de edad de 12 años. Dieciocho pacientes fueron de sexo masculino. La mediana de distancia recorrida fue de 551,3 metros, un 84 % de la distancia teórica para población pediátrica sana. Las variables talla, presión arterial sistólica pretest y saturación arterial de oxígeno de reposo se asociaron significativamente con la distancia recorrida en el modelo de regresión lineal múltiple. No hubo asociación significativa en los metros caminados respecto de las variables sexo, estado nutricional, dignóstico cardiológico inicial, número de cirugías previas y edad al momento del bypass total de ventrículo derecho

A cross-sectional study was done in students with univentricular heart after undergoing total cavopulmonary connection (Fontan procedure) to establish their baseline functional capacity based on the six-minute walk test and identify potential determining factors. Thirty patients were included; their median age was 12 years old. Eighteen patients were males. The median distance walked was 551.3 meters, 84 % of the theoretical distance for a healthy pediatric population. Height, pre-test systolic blood pressure, and resting arterial oxygen saturation showed a significant association with the distance walked in the multiple linear regression model. No significant association was observed in the meters walked in terms of the following outcome measures: sex, nutritional status, baseline cardiological diagnosis, number of prior surgeries, and age at the time of Fontan procedure

A case of COVID-19 in a patient with a univentricular heart post total cavopulmonary connection (Fontan) surgery.

Coronavirus disease 2019 (COVID-19) has caused a global pandemic which has affected patients and healthcare systems around the world. Patients with underlying health conditions seem to be more severely affected. There are limited reports of patients with univentricular circulations and COVID 19; thus, we report a case of COVID-19 in a patient with a univentricular circulation.

Differences in Pulmonary and Systemic Flow Measurements by Cardiac Magnetic Resonance vs Cardiac Catheterization and Relation to Collateral Flow in Single Ventricle Patients.

Both cardiac magnetic resonance (CMR) and cardiac catheterization (cath) may assess patients with single ventricle physiology prior to stage II or Fontan palliation. However, development of significant aortopulmonary collaterals may invalidate assumptions of the Fick method. We compared CMR and cath flow measurements and evaluated the relation to collateral flow. This single-center study included all pre-stage II and pre-Fontan patients between 2010 and 2017 with CMR and cath within 1 month. Pulmonary (Qp) and systemic flow (Qs) by cath were calculated by Fick method. CMR Qp was calculated by total pulmonary venous flow, and Qs by total vena caval flow. Collateral flow by CMR was the difference of pulmonary vein and pulmonary artery flow. In 26 studies (16 pre-stage II and 10 pre-Fontan) in 21 patients, collateral flow was higher in pre-Fontan patients (1.8 ± 0.6 vs 0.9 ± 0.8 L/min/m2, p = 0.01). Overall, CMR and cath had good agreement for Qs and Qp:Qs, with moderate correlation (r = 0.44, p = 0.02 for Qs, r = 0.48, p = 0.02 for Qp:Qs). In pre-Fontan but not in pre-stage II patients, CMR had higher Qp (mean difference - 1.71 L/min/m2) and Qp:Qs (mean difference - 0.36). The underestimation of cath Qp correlated with amount of collateral flow (r = - 0.47, p = 0.02). Neither cath nor CMR flow measurements correlated with outcomes in this small cohort. In conclusion, collaterals lead to systematically higher Qp and Qp:Qs measurements by CMR vs cath in single ventricle patients. Measurements may not be used interchangeably, with potential clinical significance in estimating pulmonary vascular resistance. Further study is necessary to evaluate possible relation to clinical outcomes.

Left ventricular non-compaction in patients with single ventricle heart disease.

OBJECTIVE: Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function. METHODS: Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data. RESULTS: The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71). CONCLUSIONS: Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.

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.

Thromboembolic Risk After Atriopulmonary, Lateral Tunnel, and Extracardiac Conduit Fontan Surgery.

BACKGROUND: Thromboembolic events contribute greatly to morbidity and mortality following Fontan surgery for univentricular hearts. OBJECTIVES: This study sought to evaluate the effect of type of Fontan surgery on thromboembolic risk. METHODS: A North American multicenter retrospective cohort study enrolled 522 patients with Fontan palliation consisting of an atriopulmonary connection (APC) (21.4%), lateral tunnel (LT) (41.8%), or extracardiac conduit (EC) (36.8%). Thromboembolic complications and new-onset atrial arrhythmia were reviewed and classified by a blinded adjudicating committee. Thromboembolic risk across surgical techniques was assessed by multivariable competing-risk survival regression. RESULTS: Over a median follow-up of 11.6 years, 10- and 20-year freedom from Fontan conversion, transplantation, or death was 94.7% and 78.9%, respectively. New-onset atrial arrhythmias occurred in 4.4, 1.2, and 1.0 cases per 100 person-years with APC, LT, and EC, respectively. APC was associated with a 2.82-fold higher risk of developing atrial arrhythmias (p < 0.001), with no difference between LT and EC (p = 0.95). A total of 71 thromboembolic events, 32 systemic and 39 venous, occurred in 12.8% of subjects, for an overall incidence of 1.1%/year. In multivariable analyses, EC was independently associated with a lower risk of systemic (hazard ratio [HR]: 0.20 vs. LT; 95% confidence interval [CI]: 0.04 to 0.97) and combined (HR: 0.34 vs. LT; 95% CI: 0.13 to 0.91) thromboembolic events. A lower incidence of combined thromboembolic events was also observed with antiplatelet agents (HR: 0.54; 95% CI: 0.32 to 0.92) but not anticoagulation (p = 0.53). CONCLUSIONS: The EC Fontan was independently associated with a lower thromboembolic risk after controlling for time-varying effects of atrial arrhythmias and thromboprophylaxis.