Transannular bovine jugular vein monocusp for the reconstruction of severe right ventricular outflow tract obstruction.

Objective: Severe right ventricular outflow tract obstruction in tetralogy of Fallot and variants necessitates the use of transannular patch in a significant proportion of children undergoing repair. We have used a Contegra monocusp together with delamination of native leaflet tissue in order to create a functioning pulmonary valve. Methods: In total, 18 (2017-2022) consecutive Contegra monocusp implantations were included. Median age and weight were 3.65 [2.00; 9.43] months and 6.12 [4.30; 8.22] kg, respectively. Nine of 18 patients had undergone palliation. Native pulmonary leaflet tissue was recruited to create a single posterior cusp. Contegra monocusp selection was based on the goal to achieve a neoannulus of Z value ≈ 0. Monocusp sizes implanted were 16 [14; 18] mm. Patch plasty of left pulmonary artery (LPA) (9), right pulmonary artery (RPA) (2), and both LPA-RPA (5) were often performed. Results: All patients survived the operation and were discharged home in good health. Median ventilation time and hospital stay were 2 [1; 9] and 12.5 [9; 54] days, respectively. Follow-up duration was 30.68 [3.47; 60.47] months and 100% complete. One patient with well-corrected right ventricular outflow tract died 9.4 months postoperatively, possibly of aspiration. One child with membranous pulmonary atresia needed reoperation (conduit insertion) at 3.5 months of follow-up. Five needed catheter interventions: supravalvar stent (2), LPA stent (3), and RPA stent (1), most of them in the earlier half of the experience. Pulmonary annulus changed from preoperative -3.91 [-5.98; -2.23] to -0.10 [-1.44; 1.92] at discharge; growing proportionally to -0.13 [-3.52; 2.73] at follow-up. Kaplan-Meier freedom from composite dysfunction was 79.25 (95% confidence interval, +13.68%, -31.44%) at 36 months. Conclusions: Recruitment of native leaflets, optimal Contegra monocusp, and commissuroplasty provide an easily replicable technique for achieving a competent, proportionally growing neopulmonary valve. Longer follow-up is needed to determine its impact on delaying a pulmonary valve replacement.

Risk Factors and Outcomes of Children with Congenital Heart Disease on Extracorporeal Membrane Oxygenation-A Ten-Year Single-Center Report.

For children born with congenital heart defects (CHDs), extracorporeal life support may be necessary. This retrospective single-center study aimed to investigate the outcomes of children with CHDs on extracorporeal membrane oxygenation (ECMO), focusing on various risk factors. Among the 88 patients, 36 (41%) had a single-ventricle heart defect, while 52 (59%) had a biventricular defect. In total, 25 (28%) survived, with 7 (8%) in the first group and 18 (20%) in the latter. A p-value of 0.19 indicated no significant difference in survival rates. Children with biventricular hearts had shorter ECMO durations but longer stays in the intensive care unit. The overall rate of complications on ECMO was higher in children with a single ventricle (odds ratio [OR] 1.57, 95% confidence interval [CI] 0.67-3.7); bleeding was the most common complication in both groups. The occurrence of a second ECMO run was more frequent in patients with a single ventricle (22% vs. 9.6%). ECMO can be effective for children with congenital heart defects, including single-ventricle patients. Bleeding remains a serious complication associated with worse outcomes. Patients requiring a second ECMO run within 30 days have lower survival rates.

Bridging with Veno-Arterial Extracorporeal Membrane Oxygenation in Children: A 10-Year Single-Center Experience.

Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) is frequently used in children with and without congenital heart disease (CHD). This study, of a single-center and retrospective design, evaluated the use and timing of V-A ECMO in a pediatric cohort who underwent V-A ECMO implantation between January 2009 and December 2019. The patients were divided into a pre-/non-surgical group and a post-surgical group. Among the investigated variables were age, gender, weight, duration of ECMO, ECMO indication, and ventricular physiology, with only the latter being statistically relevant between the two groups. A total of 111 children (58 male/53 female), with a median age of 87 days (IQR: 7-623) were supported using V-A ECMO. The pre-/non-surgical group consisted of 59 patients and the post-surgical group of 52 patients. Survival at discharge was 49% for the pre-/non-surgical group and 21% for the surgical group (p = 0.04). Single-ventricle physiology was significant for a worse outcome (p = 0.0193). Heart anatomy still has the biggest role in the outcomes of children on ECMO. Nevertheless, children with CHD can be successfully bridged with ECMO to cardiac operation.

Norwood-I in a hypoplastic left heart variant and right aortic arch.

Even though preoperative diagnostics have improved significantly, intraoperative surprises may still occur especially in the case of complex congenital heart disease. An instance of such a complex congenital heart disease is a hypoplastic left heart syndrome with a right-sided aortic arch. In this case report, we present 1 patient with such a complex and unexpected anatomy, as well as a possible way to overcome the obstacles.

Dysphagia and an aberrant subclavian artery: more than just a coincidence.

Aberrant subclavian artery (arteria lusoria) is the most common congenital malformation of the aortic arch in the general population. As patients are more often asymptomatic, indications for correcting this abnormality, even in those having dysphagia, are very vague. While presenting a case of a 10-year-old girl who suffered from dysphagia lusoria for 2 years before being cured by a simple surgical translocation, we review the literature and debate various aspects of its management from diagnosis to surgery and cosmesis.

Two-stage repair of interrupted aortic arch type A: Arch reconstruction and pulmonary artery banding without CPB through a left thoracotomy.

Treatment of interrupted aortic arch (IAA) is inherently challenging. Single-stage complete repair has become the norm in contemporary practice.  While palliation, in general, has fallen out of favor, a 2-stage approach to correcting selected type A IAA appears to be a productive, safe, and effective option, considering the neonatal age at repair. In this video tutorial, we demonstrate our preferred technique for neonatal aortic arch repair and pulmonary artery banding, which constitute the first stage of the 2-stage repair. Key steps of stage 1 repair are demonstrated and the arguments supporting our approach are presented. This approach significantly reduces morbidity and results in a well-palliated ventricular septal defect (VSD).  We also briefly discuss stage 2 of the repair, which is a straightforward VSD closure and pulmonary artery debanding procedure when the child is older and more resilient.

Serum lactate at 24 hours is associated with outcome in children requiring extracorporeal membrane oxygenation for pulmonary causes - a retrospective, observational study.

OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) has become an essential life-saving tool. Being resource-intensive, judicious use and optimising the outcomes of this precious resource is important. This retrospective, explanatory, observational study aimed to quantify associations between factors and outcome after pulmonary ECMO in children. METHODS: This study included 39 consecutive ECMO runs in 38 children performed for pulmonary indications at our hospital from 2008 to 2018. Indications included acute respiratory distress syndrome, infection, drowning, meconium aspiration and pulmonary hypertension, among others. Depending on the need for haemodynamic support, 21 patients (53.8%) received veno-venous ECMO, while 18 (46.2%) received veno-arterial ECMO. We sought to compare the 11 non-survivors with the 27 survivors with respect to time-independent and time-dependent variables. Logistic regression models and Cox proportional hazards models were used. Threshold analysis was done using the “minimum p-value approach”. RESULTS: 27/39 (69%) ECMO runs could be weaned; 27/38 (71%) patients were discharged. 20/27 (74%) survivors had unremarkable neurological status, six (22%) had mild findings (convulsions, muscular hypotony, neuropathy) and one (4%) had a hemi-syndrome at discharge. Univariate analyses showed a hazard ratio (HR) of 0.48 for log(pH) (95% confidence interval [CI] 0.22 to 1.02, p = 0.055) and an HR of 4.48 for log(lactate) (95% CI 1.92 to 10.48, p = 0.0005). Multivariate models showed an HR of 0.99 for log(pH) (95% CI 0.43 to 2.26, p = 0.98) and an HR of 4.44 for log(lactate) (95% CI 1.65 to 11.95, p = 0.003). Threshold analysis showed lactate >4.1 to be associated with mortality, with an HR of 32.7 (95% CI 4.8 to 221.7, p = 0.0002). This threshold should, however, be interpreted very cautiously. Evidence of an association between serum lactate at 24 hours and mortality was found (difference between survivors and non-survivors: −2.78, 95% CI −5.36 to −0.20, p = 0.037). CONCLUSIONS: The results of ECMO for pulmonary indications are very good. Serum lactate may be an early prognostic indicator.

Extracorporeal membrane oxygenation support in pediatrics.

Extracorporeal membrane oxygenation (ECMO) is a general term that describes the short- or long-term support of the heart and/or lungs in neonates, children and adults. Due to favorable results and a steady decline in absolute contraindications, its use is increasing worldwide. Indications in children differ from those in adults. The ECMO circuit as well as cannulation strategies also are individualized, considering their implications in children. The aim of this article is to review the clinical indications, different circuits, and cannulation strategies for ECMO. We also present our institutional experience with 92 pediatric ECMO patients (34 neonates, 58 pediatric) with the majority (80%) of veno-arterial placements between 2014 until 2018. We further to also highlight ECMO use in the setting of cardiac arrest [extracorporeal cardiopulmonary resuscitation (CPR) or eCPR].