Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force

Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.

International Survey on the Use of Arginine Vasopressin in the Postoperative Management of Single Ventricle Patients

Management of patients with single ventricle physiology after surgical palliation is challenging. Arginine vasopressin has gained popularity in recent years as a non-catecholamine vasoactive medication due to its unique properties. However, data regarding its use in the pediatric population is limited. Therefore, we designed a survey to explore whether and how clinicians use this medication in intensive care units for the postoperative management of single ventricle patients. This international survey aimed to assess usage, practices, and concepts related to arginine vasopressin in pediatric intensive care units worldwide. Directors of pediatric intensive care units who are members of the following international professional societies: European Society of Pediatric Neonatal Intensive Care, Association for European Pediatric and Congenital Cardiology, and Pediatric Cardiac Intensive Care Society were invited to participate in this survey. Of the 62 intensive care unit directors who responded, nearly half use arginine vasopressin in the postoperative management of neonatal single ventricle patients, and 90% also use the drug in subsequent surgical palliation. The primary indications are vasoplegia, hemodynamic instability, and refractory shock, although it is still considered a second-line medication. Conceptual benefits include improved hemodynamics and end-organ perfusion and decreased incidence of low cardiac output syndrome. Those practitioners who do not use arginine vasopressin cite lack of availability, fear of potential adverse effects, unclear indication for use, and lack of evidence suggesting improved outcomes. Both users and non-users described increased myocardial afterload and extreme vasoconstriction as potential disadvantages of the medication. Despite the lack of conclusive data demonstrating enhanced clinical outcomes, our study found arginine vasopressin is used widely in the care of infants and children with single ventricle physiology after the first stage and subsequent palliative surgeries. While many intensive care units use this medication, few had protocols, offering an area for further growth and development.

Right Ventricular Outflow Tract Stenting as Palliation of Critical Tetralogy of Fallot: Techniques and Results

Background. Despite current trends toward early primary repair, the surgical systemic-to-pulmonary shunt is still considered the first-choice palliation in patients with critical tetralogy of Fallot (TOF) and duct-dependent pulmonary circulation unsuitable for primary repair. However, stenting of the right ventricular outflow tract (RVOT) is nowadays emerging as an effective alternative to surgical palliation in selected patients. Methods and results. RVOT stenting is usually performed from a venous route, either femoral or, in selected cases, the right internal jugular vein. Less frequently, mostly in pulmonary infundibular/valvar atresia, this procedure can be performed using a hybrid surgical/interventional approach by surgical exposure of the RVOT, puncture of the atretic valve, and stent deployment under direct vision. The size and type of the most appropriate stent may be chosen, based on ultrasound measurements of the RVOT, to cover the right ventricular infundibulum completely and, at the same time, sparing the pulmonary valve, unless significant pulmonary valve annulus hypoplasia and/or supra-valvular stenosis is a significant component of the obstruction. In the large series so far published, early mortality of RVOT stenting is less than 2%, comparing favourably with either Blalock-Thomas-Taussig shunt or early primary repair. In addition, morbidity and clinical sequelae of this approach do not significantly differ from surgical palliation, even if RVOT stenting shows lesser durability and a higher rate of trans-catheter re-interventions over a mid-term follow-up. Finally, similar but more balanced pulmonary artery growth than surgical palliation following RVOT stenting is reported over a mid-term follow-up. Conclusions. RVOT stenting is a technically feasible, well-tolerated, and effective palliation in critical TOF. This approach is cost-effective with respect to surgical palliation either in high-risk neonates or whenever a short-term pulmonary blood flow source is anticipated due to the early surgical repair. It effectively increases pulmonary blood flow, improves arterial saturation, and promotes balanced pulmonary artery growth over a mid-term follow-up.

Surgical palliation for an infant with hypoplastic left heart syndrome and congenital lobar emphysema: a case report

Hypoplastic left heart syndrome is a form of congenital heart disease that is associated with significant lifelong morbidity and mortality. Although rarely reported together, the prognosis for patients with hypoplastic left heart syndrome and congenital anomalies of the airway or lung, such as congenital diaphragmatic hernia, has been shown to be very poor. The outcomes for patients with hypoplastic left heart syndrome and congenital lobar emphysema specifically is currently unknown. Reported herein is the first case of a child with a rare combination of hypoplastic left heart syndrome and congenital lobar emphysema, and a discussion of his presentation, diagnosis, management and outcome to date.

Abstract 14356: Initial Experience With Telemedicine for Interstage Monitoring in Infants With Palliated Congenital Heart Disease

Introduction: Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality. We have previously described the mediated risk for this patient population through close home monitoring. Given high rates of intercurrent illness, geographic and sociodemographic disparities in access to care, and significant family stressors, we instituted a telecardiology (TC) program in 2019. Methods: All consecutive patients discharged home were monitored from neonatal operation or intervention to subsequent surgical palliation. All had home scales and pulse oximeters. TC visits (TCVs) were held monthly, and increased to bimonthly during the COVID-19 pandemic. TCVs were launched through the electronic medical record (EMR) through a mobile device, with a full-version EMR available for access to full records and efficient documentation. We tracked how TCVs identified clinical concerns, prevented unnecessary emergency room visits (ERV) and expedited in-person assessment. Results: Between 8/2019-5/2020, we conducted 60 TCV for 29 patients (16 had stage 1 palliation, 6 with aortopulmonary shunt or stent and 7 with biventricular physiology with shunt or stents. Initial median weight was 3.4 kg (range 2.3-5.4 kg). Median monitoring time was 199 days (75-264). Median visits per patient were 2 (1-5 V) with average length per visit of 20 minutes. Most scheduled visits were completed (90%); 5% declined, and 5% canceled. Parents reported satisfaction for 97% of visits. In 6 visits (10%) significant clinical findings were identified which avoided ERV or hospital admissions, 4 TCVs (7%) visits led to expedited outpatient assessments, 1 required hospitalization. There were no missed events nor deaths. Additional issues identified included changes to medication (23%), feedings (15%) and equipment malfunction (5%). Frequency of EDV decreased from median 0.4 (0-3.7) V/patient/month the prior year to 0 (0-2.5), p=0.0004]. Conclusions: TC in these high-risk patients is feasible and effective to identify clinical concerns, no missed events and decrease in ERV per patient. TC was particularly effective during the COVID-19 pandemic allowing for easy adaptation of care to ensure patient safety in this fragile cohort.