Single ventricle ventilation

Children with cardiac disease are at a higher risk of cardiac arrest as compared to healthy children. Delivering adequate cardiopulmonary resuscitation (CPR) can be challenging due to anatomic characteristics, risk profiles, and physiologies. We aimed to review the physiological aspects of resuscitation in different cardiac physiologies, summarize the current recommendations, provide un update of current literature, and highlight knowledge gaps to guide research efforts. We specifically reviewed current knowledge on resuscitation strategies for high-risk categories of patients including patients with single-ventricle physiology, right-sided lesions, right ventricle restrictive physiology, left-sided lesions, myocarditis, cardiomyopathy, pulmonary arterial hypertension, and arrhythmias...

BACKGROUND: Data on intraoperative three-dimensionally derived right ventricular free-wall strain (3D-RV FWS) is sparse. OBJECTIVES: We sought to evaluate the normal range of intraoperative 3D-RV FWS in patients scheduled for coronary artery bypass graft (CABG) surgery and compared to conventional echocardiographic parameters. Prospective observational study. METHODS: A total of 150 patients with preserved left and right ventricular (RV) function and sinus rhythm, without significant heart valve disease or pulmonary hypertension undergoing isolated on-pump CABG surgery, with an uneventful, complication-free intraoperative course...

OBJECTIVES: To define the incidence of definitive necrotising enterocolitis in term infants with CHD and identify risk factors for morbidity/mortality. METHODS: We performed a 20-year (2000-2020) single-institution retrospective cohort study of term infants with CHD admitted to the Boston Children's Hospital cardiac ICU with necrotising enterocolitis (Bell's stage ≥ II). The primary outcome was a composite of in-hospital mortality and post-necrotising enterocolitis morbidity (need for extracorporeal membrane oxygenation, multisystem organ failure based on the paediatric sequential organ failure assessment score, and/or need for acute gastrointestinal intervention)...

BACKGROUND: Right ventricle dysfunction is common after corrective surgery for tetralogy of Fallot and is associated with significant morbidity and mortality. We aimed to determine whether an increased portal vein pulsatility fraction (PVPF) was associated with worse clinical outcomes. METHODS: In a prospective, observational, single-centre study, PVPF and other commonly used parameters of right ventricle function were assessed in patients of all ages undergoing corrective surgery for tetralogy of Fallot intraoperatively, with transesophageal echocardiography, before and after bypass, and post-operatively, with transthoracic echocardiography, at days 1, 2, at extubation, and at ICU discharge...

BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) is a fatal congenital cardiac anomaly that requires urgent surgical intervention. The development of postoperative pulmonary vein obstruction (PVO) negatively impacts long-term survival. OBJECTIVES: The present study aimed to evaluate the surgical outcomes of TAPVC repair and risk factors associated with postoperative PVO. METHODS: Patients who underwent primary TAPVC repair at our institute between 2004 and 2022 were retrospectively enrolled, and those with right atrial isomerism and single ventricle physiology were excluded...

BACKGROUND AND PURPOSE: Deep inspiration breath-hold (DIBH) protects critical organs-at-risk (OARs) for adjuvant breast radiotherapy. Guidance systems e.g. surface guided radiation therapy (SGRT) improve the positional breast reproducibility and stability during DIBH. In parallel, OARs sparing with DIBH is enhanced through different techniques e.g. prone position, continuous positive airway pressure (CPAP). By inducing repeated DIBH with the same level of positive pressure, mechanically-assisted and non-invasive ventilation (MANIV) could potentially combine these DIBH optimizations...

Patients with pulmonary vein stenosis (PVS) often require frequent transcatheter pulmonary vein (PV) interventions for management of restenosis. Predictors of serious adverse events (AEs) and need for high-level cardiorespiratory support (mechanical ventilation, vasoactive support, and/or extracorporeal membrane oxygenation) 48 h after transcatheter PV interventions have not been reported. This is a single-center retrospective cohort analysis of patients with PVS who underwent transcatheter PV interventions from 3/1/2014 to 12/31/2021...

BACKGROUND: Neonates with congenital diaphragmatic hernia (CDH) have varying degrees of pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac dysfunction. These neonates frequently require vasoactive support and are at high risk for mortality and morbidity, including prolonged ventilator support, need for extracorporeal membrane oxygenation (ECMO), prolonged length of stay, and need for tracheostomy. However, identifying which infants are at increased risk can be challenging. In this study, we sought to investigate the utility of the inotropic score (IS) and vasoactive inotropic score (VIS) as tools to predict significant clinical outcomes and overall survival in patients with CDH...

BACKGROUND: Right ventricular (RV) function is increasingly being recognised as an important factor influencing outcomes in ST elevation myocardial infarction (STEMI) complicated by cardiogenic shock (CS). In this study, we investigated RV echocardiographic parameters' association with 28-day mortality in patients admitted to intensive care with STEMI complicated by CS with reduced left ventricle ejection fraction (LVEF). METHOD: We performed a retrospective analysis of patients admitted to intensive care unit (ICU) in a single tertiary cardiac centre over a 34-month period with STEMI complicated by CS and LVEF < 40%...

BACKGROUND: The aim of this study is to present our experience regarding the creation of an aortopulmonary window as the initial palliative procedure. METHODS: Between February 2016 and February 2021, a total of eight patients (3 males, 5 females; median age: 2 months; range, 0.7 to 6 months) who underwent aortopulmonary window creation were retrospectively analyzed. Data collection was performed by review from our institution. RESULTS: There was no occurrence of early or late mortality in any patient...

OBJECTIVE: Neonatal presentation of Ebstein anomaly (EA) represents the most severe form of this condition. Despite significant advances, operative mortality remains high and management decisions represent a formidable challenge. We used a strategy aimed to match anatomy and physiology with type and time of intervention to increase survival. METHODS: We performed a review of all patients with fetal or neonatal diagnosis of EA managed at a single center between 2007 and 2020...

BACKGROUND: Right ventricular (RV) dysfunction has been commonly reported in patients with Coronavirus disease 2019 (COVID-19), and is associated with mortality in mixed cohorts of patients requiring and not requiring invasive mechanical ventilation (IMV). Using RV-speckle tracking echocardiography (STE) strain analysis, we aimed to identify the prevalence of RV dysfunction (diagnosed by abnormal RV-STE) in patients with COVID-19 that are exclusively undergoing IMV, and assess association between RV dysfunction and 30 day mortality...

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...

BACKGROUND AND AIMS: Postoperative cardiac outcomes after intracardiac repair (ICR) are determined by numerous factors whereas right ventricle (RV) dysfunction is considered essential for them, as only few studies attempted to evaluate it postsurgically. RV's function is supposed to be the strong prognostic factor for patients diagnosed with congenital heart defects; therefore, assessing it is the main objective of the study. METHODS: This is a prospective single-centered cohort study performed on 50 pediatric patients with congenital heart disease (CHD) who underwent ICR between January 2019 and January 2022...

OBJECTIVES: There is an ongoing discussion on the optimal right to left (RV/LV) diameter ratio threshold and the best definition of RV dysfunction on computed tomography pulmonary angiography (CTPA) for risk assessment of pulmonary embolism (PE). METHODS: On routine diagnostic CTPA, volumetric and diameter measurements (axial and reconstructed views) of the ventricles and reflux of contrast medium into the inferior vena cava (IVC) and hepatic veins were assessed in consecutive PE patients enrolled in a prospective single-center registry...

BACKGROUND: Heart transplantation (HT) is the gold standard for managing end-stage heart failure. Multiple quality metrics, including length of stay (LOS), have been used in solid organ transplantation. However, limited data are available regarding trends and factors influencing LOS after pediatric HT. We hypothesized that various donor, peri-transplant and recipient factors affect LOS after pediatric HT. METHODS: We analyzed patients <18years at time of HT from January 2005 to December 2018 in the Pediatric Heart Transplant Society database, and examined LOS trends, defined prolonged LOS (PLOS = LOS>30days after HT), identified factors associated with PLOS and assessed outcomes...

BACKGROUND: Tracheal agenesis (TA) is a rare congenital defect that consists of a complete or partial absence of the trachea below the larynx, with or without tracheoesophageal fistula (TEF). It is a severe congenital defect with a very high mortality rate. The recommended surgical approach is esophageal ligation and gastrostomy. Despite the progress in reconstructive surgical techniques, the outcome of the anomaly is still very poor. We described a case of TA with a TEF in a female newborn with a hemivertebra, single ventricle, single atrioventricular valve, single atrium, and cardiac left isomerization...

The number of adults with congenital heart disease (CHD) requiring cardiovascular (CV) surgery is increasing rapidly in today's era. We hypothesized that exposure to perioperative blood products is associated with worse outcomes in adults. All adults (≥ 18 years old) undergoing CV surgery with Cardio-Pulmonary Bypass (CPB) between 2015 and 2020 were reviewed retrospectively. Associations between transfusion and outcomes were studied by univariable logistic regression and Wilcoxon rank sum tests. Cox/ logistic regression was used to assess (a) postoperative ventilation time and length of stay, and (b) major complications, respectively...

Study objectives: To assess whether echocardiography, systematically performed, could help in risk stratifying patients with acute respiratory distress syndrome (ARDS) due to SARS-CoV2 (COVID) infection for non invasive ventilation (NIV) failure. Design: Observational single center investigation. Setting: Intensive care unit. Interventions: Echocardiography. Outcome measures: NIV failure. Main results: Seventy-five patients were included in our study...

BACKGROUND: Endoscopic procedures are rarely performed in children with congenital heart disease (CHD); therefore, the associated complications are unknown. We report an abrupt change in circulatory and respiratory condition during endoscopic injection sclerotherapy for esophageal varices. CASE PRESENTATION: A 9-year-old boy with a history of total anomalous pulmonary venous connection (TAPVC) repair and Fontan procedure for asplenia and a single ventricle with TAPVC underwent endoscopic injection sclerotherapy under general anesthesia for esophageal varices...