Pediatric COVID-19 extracorporeal membrane oxygenation transport during the pandemic.

INTRODUCTION: ExtraCorporeal Membrane Oxygenation (ECMO) in pediatric patients with COVID-19 has a survival rate similar to adults. Occasionally, patients may need to be cannulated by an ECMO team in a referring hospital and transported to an ECMO center. The ECMO transport of a COVID-19 patient has additional risks than normal pediatric ECMO transport for the possible COVID-19 transmissibility to the ECMO team and the reduction of the ECMO team performance due to the need of wearing full personal protective equipment. Since pediatric data on ECMO transport of COVID-19 patients are lacking, we explored the outcomes of the pediatric COVID-19 ECMO transports collected in the EuroECMO COVID_Neo/Ped Survey. METHODS: We reported five European consecutive ECMO transports of COVID-19 pediatric patients collected in the EuroECMO COVID_Neo/Ped Survey including 52 European neonatal and/or pediatric ECMO centers and endorsed by the EuroELSO from March 2020 till September 2021. RESULTS: The ECMO transports were performed for two indications, pediatric ARDS and myocarditis associated to the multisystem inflammatory syndrome related to COVID-19. Cannulation strategies differed among patients according to the age of the patients, transport distance varied between 8 and 390 km with a total transport duration between 5 to 15 h. In all five cases, the ECMO transports were successfully performed without major adverse events. One patient reported a harlequin syndrome and another patient a cannula displacement both without major clinical consequences. Hospital survival was 60% with one patient reporting neurological sequelae. No ECMO team member developed COVID-19 symptoms after the transport. CONCLUSION: Five transports of pediatric patients with COVID-19 supported with ECMO were reported in the EuroECMO COVID_Neo/Ped Survey. All transports were performed by an experienced multidisciplinary ECMO team and were feasible and safe for both the patient and the ECMO team. Further experiences are needed to better characterize these transports and draw insightful conclusions.

Mediastinitis After Congenital Heart Surgery: Epidemiology, Clinical Features and Outcomes.

Mediastinitis after surgery for congenital heart disease has a great impact on morbidity and mortality. However, there are scarce studies focused on the epidemiology of postsurgical mediastinitis in pediatric patients. In this 18-year period retrospective study, the cumulative incidence of mediastinitis was low: 0.64%, (95% confidence interval: 0.36-1.1). Gram-negative bacilli were common (35%). The mortality rate was 7.1%, associated with fungal infection.

Primary neonatal and pediatric ECMO transport: First experience in Spain.

INTRODUCTION: The organization of primary Extracorporeal membrane oxygenation (ECMO) transport is highly variable. METHODS: To present the experience of the first mobile pediatric ECMO program in Spain, we designed a prospective descriptive study of all primary neonatal and pediatric (0-16 years) ECMO transports carried out over 10 years. The main variables recorded include demographic information, patient background, clinical data, ECMO indications, adverse events, and main outcomes. RESULTS: 39 primary ECMO transports were carried out with a 66.7% survival to hospital discharge. The median age was 1.24 months[IQR: 0.09-96]. Cannulation was mostly peripheral venoarterial (33/39). The mean response time from the call from the sending center to the departure of the ECMO team was 4 h[2.2-8]. The median inotropic score at the time of cannulation was 70[17.2-206.5], with a median oxygenation index of 40.5[29-65]. In 10% of the cases, ECMO-CPR was performed. Adverse events occurred in 56.4%, mostly related to the means of transport (40% overall). On arrival at the ECMO center, 44% of the patients underwent interventions. The median PICU stay was 20.5 days[11-32]. 5 patients developed neurological sequels. Statistically significant differences between survivors and deceased patients were not found. CONCLUSIONS: A good survival rate, with a low prevalence of serious adverse events, suggests a clear benefit of primary ECMO transport when conventional therapeutic measures are exhausted and the patient is too unstable to undergo conventional transport. A nationwide primary ECMO-transport program must therefore be offered to all patients regardless of their location.

Common Arterial Trunk in Fetal Life: Accuracy of Diagnosis and Prediction of Outcome.

INTRODUCTION: Common arterial trunk (CAT) is a congenital heart disease with significant perinatal mortality in which diagnostic agreement remains low. METHODS: We conducted a retrospective cohort study on fetuses with suspected CAT. Diagnostic accuracy was tested considering gold-standard postnatal ultrasound or necropsy. Prenatal sonographic markers were evaluated by logistic regression for perinatal survival. RESULTS: There were 79 fetuses with suspected CAT, and 55 cases had available necropsy/postnatal ultrasound confirming the study population. The diagnostic accuracy was 90.9% for CAT and 83.6% at a subtype level. In those with a confirmed diagnosis and intention-to-treat (n = 28) composite early mortality (intrauterine, presurgical, and early surgery) was of 25%, 10.5% in isolated cases. The OR for early-mortality of isolated cases was 0.13 (95% confidence interval [CI]: 0.03-0.75). After adjusting for isolated cases, the only prognostic prenatal markers of perinatal mortality were a dysplastic truncal valve OR 7.78 (95% CI: 1.23-49.13) and a stenotic flow OR 8.48 (95% CI: 1.40-51.10). CONCLUSION: CAT is a condition that if evaluated by experts, can be diagnosed with a high degree of accuracy. It remains an entity with high perinatal mortality. Its most important prognostic factor is its association with other anomalies. In isolated cases, the presence of a dysplastic truncal valve and a stenotic flow increase the chances of perinatal death.

Vocal cord paralysis after cardiovascular surgery in children: incidence, risk factors and diagnostic options.

OBJECTIVES: The aim of this study was to assess the incidence of vocal cord paralysis (VCP) in children after cardiovascular surgery. The secondary aims were to identify the factors potentially associated with VCP and to assess the diagnostic utility of laryngeal ultrasound (US). METHODS: This study is a retrospective review of patients who underwent aortic repair, patent ductus arteriosus ligation and left pulmonary artery surgeries from 2007 to 2017. The following data were collected: patient demographics, gestational age, weight and age at surgery, comorbidities, cardiovascular anomaly and type of procedure, laryngoscopic and US evaluation results. Univariable and multivariable logistic regression models were used to identify the variables associated with VCP. RESULTS: Two hundred and six patients were included in the study. Seventy-two patients (35%) were preterm and 32.5% showed comorbidities. At surgery, median age and weight were 0.6 months [interquartile range (IQR) 0.3-2.1] and 3.0 kg (IQR 1.3-4.0), respectively. Postoperatively, symptomatic patients underwent endoscopic evaluation and VCP was detected in 25 cases (12.1%). Laryngeal US was performed in 8 of these showing an excellent diagnostic relationship. On univariable analysis, factors significantly associated with VCP were prematurity, young age and lower weight at surgery and the presence of comorbidities. The presence of comorbidities and weight at surgery exhibited a significant risk of developing VCP postoperatively on multivariable analysis. CONCLUSIONS: VCP is not an unusual complication of cardiovascular surgery. Certain factors were associated with VCP development but only the presence of comorbidities and weight at surgery were statistically significant on multivariable analysis. Flexible laryngoscopy is the standard diagnostic technique and laryngeal US appears to be a reliable complement.

Atrioventricular valves dysplasia in a newborn.

A newborn with prenatally diagnosed dysplasia of both atrioventricular valves presented after birth with signs and symptoms of low cardiac output, severe regurgitation of both mitral and tricuspid valves. This combination is as rare as challenging, since it regards both the timing and management of this complex cardiac malformation. We report an early surgical repair of both atrioventricular valves in a symptomatic newborn, which improved his clinical status and, so far, delayed valve replacement.

Fetal Aortic Valvuloplasty: Experience and Results of Two Tertiary Centers in Spain.

OBJECTIVE: Fetal aortic valvuloplasty (FAV) may avoid progression of critical aortic stenosis (CAS) to hypoplastic left ventricle, improving the options for biventricular circulation (BVC). We describe the results of FAV in 2 referral centers in Spain. METHODS: We analyzed all FAVs performed in the period 2007-2015. The selection of candidates, the technique, and postnatal management were made following an agreed protocol. A descriptive analysis of survival, type of circulation after birth, and complications was made, considering all deaths in the first 48 h after FAV as FAV-related. RESULTS: FAV was performed in 28 fetuses at a median gestational age (GA) of 23 weeks (range, 20-32). FAV was technically successful in 22 (78.6%), of whom 11 were born alive and with intention to treat. Eight (72.7%) resulted in BVC and 3 (27.3%) in univentricular circulation. The rate of FAV-related deaths was 32%. These patients underwent FAV earlier than live-born fetuses (median GA at FAV 22 weeks [range, 20.0-25.0] vs. 24.5 weeks [range, 21.0-32.0], respectively, p = 0.031). CONCLUSIONS: A significant proportion of fetuses with CAS who undergo technically successful FAV have BVC postnatally. However, FAV implies a high risk of fetal death, which highly depends on the GA at which this intervention is required.

End-stage ischemic heart failure and Williams-Beuren syndrome: A unique scenario for pediatric heart transplantation.

WBS is a rare disorder caused by mutations in the chromosomal sub-band 7q11.23 involving the elastin gene. The clinical features (craniofacial, developmental, and cardiovascular abnormalities) are variable. The association with cardiac anomalies is a well-recognized feature, and SVAS is the most common cardiac defect found. End-stage ischemic heart disease is unusual in this setting but when it occurs, OHT remains the final therapeutic option. This decision can be difficult to determine, and it must be tailored to the individual patient based on the clinical status and concomitant cardiovascular and multisystem lesions. To date, no cases of OHT in patients with WBS have been described. We present a 14-month-old patient with WBS who developed severe LV dysfunction secondary to ischemia following a complex staged surgery for SVAS repair. He underwent successful OHT with no post-operative complications, and at three-month follow-up, he remains asymptomatic on standard immunosuppressive therapy. This case constitutes the first demonstration that OHT may be indicated for extended survival in selected children with WBS and we discuss the basic principles for extending the indication for OHT to this scenario as well as the particularities for post-transplant care.

Left arm underdevelopment secondary to an isolated left subclavian artery in tetralogy of Fallot.

The anomalous origin of the left subclavian artery is known to be associated with right aortic arch and tetralogy of Fallot. In our case, the left subclavian artery arose from the left pulmonary artery. Therefore, the left arm was perfused by poorly oxygenated blood from pulmonary arteries and some retrograde vertebral artery flow. Thus, the left arm was cyanotic and less developed than the right one. The patient underwent surgical repair with complete correction of tetralogy of Fallot and reimplantation of the left subclavian artery to the left carotid artery.