Single ventricle ventilation

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) universally develop in patients with single ventricle congenital heart disease (CHD). Single ventricle PAVMs have been recognized for over 50 years, yet they are poorly understood, and we lack any medical therapies. To improve our understanding of single ventricle PAVM initiation and progression, we developed a surgical rat model of Glenn circulation and characterized PAVM physiology over multiple time points. METHODS: Using adult rats, we performed a left thoracotomy and end-to-end anastomosis of the left superior vena cava to the left pulmonary artery (unilateral Glenn), or sham surgical control...

Guidelines advocate for integrating palliative care into the management of heart failure (HF) and of children with life-limiting disease. The potential impact of palliative care integration into pediatric HF on patient-centered outcomes is poorly understood. The present study sought to assess the association of programmatic implementation of palliative care into the heart transplant evaluation process with hospital-free days (HFD) and end of life (EOL) treatment choices. The study included patients less than 19 years of age who underwent a heart transplant evaluation between February 2012 and April 2020 at a single center...

BACKGROUND: A subset of patients with COVID-19 acute respiratory distress syndrome (ARDS) require extracorporeal membrane oxygenation (ECMO). Veno-pulmonary (VP) ECMO provides support to the right ventricle and decreased risk of recirculation. METHODS: A retrospective analysis of patients with COVID-19 ARDS and VP ECMO was performed. Patients were separated into groups by indication (1) "right ventricular (RV) failure," (2) "refractory hypoxemia," and (3) "recurrent suck-down events (SDEs)...

BACKGROUND: Poor weight gain has been identified as an independent risk factor for increased surgical morbidity and mortality for patients with single-ventricle physiology undergoing staged surgical palliation. Conversely, excessive weight gain has also emerged as an independent risk factor predicting increased morbidity and mortality in a single-center study. Given this novel single-center concept, we investigated the impact of excessive weight on patients with single-ventricle physiology undergoing bidirectional Glenn palliation in a multicenter study model...

BACKGROUND: Staged repair is common for complex conotruncal defects, often involving bidirectional Glenn (BDG) procedure. Following the cavopulmonary shunt, both Fontan completion and biventricular conversion (BiVC) serve as definitive approaches. The optimal strategy remains controversial. METHODS: The baseline, perioperative and follow-up data were obtained for all paediatric patients with conotruncal defects who underwent BDG procedure as palliation in Fuwai Hospital from 2013 to 2022...

Patent ductus arteriosus stenting (PDAS) for ductal-dependent pulmonary blood flow (DDPBF) provides a new paradigm for managing neonates with single ventricles (SV). Currently, sparse data exist regarding outcomes for subsequent palliation. We describe our experience with inter-stage care and stage 2 (S2P) conversion with PDAS in comparison to a prior era of patients who received surgical aorto-pulmonary shunts (APS). Retrospective review of 18 consecutive DDPBF SV patients treated with PDAS between 2016 and 2021 was done and compared with 9 who underwent APS from 2010 to 2016...

Background: To develop a more holistic measure of congenital heart center performance beyond mortality, we created a composite "textbook outcome" (TO) for the Glenn operation. We hypothesized that meeting TO would have a positive prognostic and financial impact. Methods: This was a single center retrospective study of patients undergoing superior cavopulmonary connection (bidirectional Glenn or Kawashima ± concomitant procedures) from 2005 to 2021. Textbook outcome was defined as freedom from operative mortality, reintervention, 30-day readmission, extracorporeal membrane oxygenation, major thrombotic complication, length of stay (LOS) >75th percentile (17d), and mechanical ventilation duration >75th percentile (2d)...

BACKGROUND: Anomalous origin of pulmonary artery (AOPA) is a rare congenital cardiac anomaly. It requires early surgical intervention (<6 months) to prevent irreversible pulmonary vaso-occlusive disease. This study was conducted to determine the surgical outcomes of this rare and intriguing anomaly. METHODS: From January 2015 to 2022, we have studied, 20 patients who underwent surgical correction for this anomaly with a mean age of 6.25 ± 3...

Background: Advancements in palliative surgery of patients with single ventricle physiology have led to an increase in the need for deep sedation protocols for painful procedures. However, positive pressure ventilation during anesthesia can result in unfavorable cardiopulmonary interactions. This patient population may benefit from sedation from these painful procedures. Methods: This study aims to demonstrate the safety and efficacy of deep sedation by pediatric intensivists outside the operating room for children with single ventricle physiology...

OBJECTIVES:  Repair of congenital heart defects involving right ventricular outflow tract (RVOT) may require pulmonary valve replacement at time of primary repair or reoperation. This study compares the outcomes of cryopreserved homografts with bovine jugular vein conduits (BJVC) in children < 2 years of age with RVOT reconstruction. METHODS:  Retrospective, single-center review of 70 conduits implanted in 63 patients undergoing right ventricle-to-pulmonary artery reconstruction with valved conduit from 2002 to 2022...

PURPOSE: There is evidence that COVID-19 can have a clinically significant effect on the right ventricle (RV). Our objective was to enhance the efficiency of assessing RV dilation for diagnosing ACP by utilizing both linear measurements and qualitative assessment and its usefulness as an independent predictor of mortality. METHODS: This is an observational, retrospective and single-center study of the Intensive Care Unit of the Sanatorio de Los Arcos in Buenos Aires, Argentina from March 2020 to January 2022...

INTRODUCTION: Pulmonary hypertension in children is associated with high rates of adverse events under anesthesia. In children who have failed medical therapy, a posttricuspid shunt such as a Potts shunt can offload the right ventricle and possibly delay or replace the need for lung transplantation. Intraoperative management of this procedure, during which an anastomosis between the pulmonary artery and the descending aorta is created, is complex and requires a deep understanding of the pathophysiology of acute and chronic right ventricular failure...

It is well appreciated that the Fontan circulation perturbs central venous hemodynamics, with elevated pressure being the clearest change associated with Fontan comorbidities, such as Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE). Our group has better quantity of these venous perturbations through single- and multi-location analyses of flow waveforms obtained from magnetic resonance imaging of Fontan patients. Here, we determine if such analyses, which yield principal components (PC) that describe flow features, are associated with Fontan survival...

BACKGROUND: Recent large-scale sequencing efforts have shed light on the genetic contribution to the etiology of congenital heart defects (CHD); however, the relative impact of genetics on clinical outcomes remains less understood. Outcomes analyses using genetics are complicated by the intrinsic severity of the CHD lesion and interactions with conditionally dependent clinical variables. METHODS: Bayesian Networks were applied to describe the intertwined relationships between clinical variables, demography, and genetics in a cohort of children with single ventricle CHD...

Therapies to support small infants in decompensated heart failure that are failing medical management are limited. We have used the hybrid approach, classically reserved for high-risk infants with single ventricle physiology, in patients with biventricular physiology with left ventricular failure. This approach secures systemic circulation, relieves left atrial hypertension, protects the pulmonary vasculature, and allows the right ventricle to support cardiac output. This approach can be used as a bridge to transplantation in select individuals...

OBJECTIVES: To describe associations between the timing of tracheostomy and patient characteristics or outcomes in the cardiac ICU (CICU). DESIGN: Single-institution retrospective cohort study. SETTING: Freestanding academic children's hospital. PATIENTS: CICU patients with tracheostomy placed between July 1, 2011, and July 1, 2020. INTERVENTIONS: We compared patient characteristics and outcomes between early and late tracheostomy based on the duration of positive pressure ventilation (PPV) before tracheostomy placement, fitting a receiver operating characteristic curve for current survival to define a cutoff...

BACKGROUND: The impact of antegrade pulmonary blood flow (APBF) during single-ventricle (SV) palliation continues to be debated. We sought to assess its impact on the hemodynamic profile on the short- and long-term outcomes on patients progressing through stages of SV-palliation. METHODS: Retrospective single-center study of SV-patients who underwent surgery between January/2010 and December/2020. Patients with APBF were matched to those with no-APBF using a propensity score based on BSA, sex, and type of systemic-ventricle...

Currently, several imaging techniques are being used for a comprehensive evaluation of patients with suspected pulmonary hypertension (PH), in order to provide information that may clarify the presence and identify the aetiology of this complex pathology. The current paper is focused on recent updates regarding the importance of comprehensive imaging techniques for patients with suspected PH. Transthoracic echocardiography that can mainly detect right ventricle pressure overload and dysfunction is the cornerstone of imaging evaluation, while right heart catheterisation remains the gold standard assessment method...

OBJECTIVES: In infants with single-ventricle congenital heart disease, prematurity and low weight at the time of the Norwood operation are risk factors for mortality. Reports assessing outcomes (including neurodevelopment) post Norwood palliation in infants ≤2.5 kg are limited. METHODS: All infants who underwent a Norwood-Sano procedure between 2004 and 2019 were identified. Infants ≤2.5 kg at the time of the operation (cases) were matched 3:1 with infants >3...

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is associated with high mortality rates and significant pulmonary morbidities. The objective of this study was to delineate the histopathological features observed in necropsies of CDH patients and correlate these with their clinical manifestations. METHODS: We retrospectively reviewed the postmortem findings and corresponding clinical characteristics in eight CDH cases from 2017 to July 2022. RESULTS: The median survival time was 46 (8-624) hours...