Extracorporeal membrane oxygenation in paediatric cardiac surgery: 5-year single centre experience.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) has become an integral part of paediatric cardiac surgery. We report the experience of a well-established ECMO service over 5 years. METHODS: This retrospective study analysed all paediatric patients who required ECMO support following cardiac surgery from April 2015 to March 2020. Inclusion criteria were age less than 18 years and post-operative ECMO support. Patients were analysed dividing into groups according to the urgency for ECMO support (extracorporeal cardiopulmonary resuscitation (ECPR) and cardiac ECMO) and according to age (neonatal and paediatric ECMO groups). They were followed for 30-day, 6-month mortality, long-term survival, postoperative morbidity and the need for reintervention. RESULTS: Forty-six patients were included who had a total of venoarterial (VA) 8 ECMO runs. The 5-year incidence of the need for VA ECMO after cardiac surgery was 3.3% (48 of the overall 1441 cases recorded). The median follow-up period was 3.5 (interquartile ranges, 0.8-4.7) years. Thirty-day, 6-month and follow-up survival rate was 85%, 65% and 52% respectively. At the 6-month follow-up, the ECPR group showed a trend towards worse survival compared with the cardiac ECMO group (47% vs. 55%) but with no statistical significance (p = 0.35). Furthermore, the survival rates between paediatric (60%) and neonatal (46%) ECMO groups were similar, with no statistical significance (p = 0.45). The rate of acute neurological events was 27% (13/48). CONCLUSION: ECPR and neonatal ECMO groups had higher mortality. VA ECMO 30-day and 6-month survival rates were 85% and 65% respectively. Major neurological injury resulting in ECMO termination occurred in 3 patients. Accumulated experiences and protocols in ECMO management can improve mortality and morbidity.

Outcome monitoring and risk stratification after cardiac procedure in neonates, infants, children and young adults born with congenital heart disease: protocol for a multicentre prospective cohort study (Children OMACp).

INTRODUCTION: Congenital heart disease (CHD) represents the most common birth defect, affecting from 0.4% to 1.2% of children born in developed countries. The survival of these patients has increased significantly, but CHD remains one of the major causes of neonatal and childhood death. The aetiology of CHD is complex, with some evidence of both genetic and environmental causes. However, there is still lack of knowledge regarding modifiable risk factors and molecular and genetic mechanisms underlying the development of CHD. This study aims to develop a prospective cohort of patients undergoing cardiac procedures that will bring together routinely collected clinical data and biological samples from patients and their biological mothers, in order to investigate risk factors and predictors of postoperative-outcomes, as well as better understanding the effect of the surgical intervention on the early and long-term outcomes. METHODS AND ANALYSIS: Children OMACp (OMACp, outcome monitoring after cardiac procedure in congenital heart disease) is a multicentre, prospective cohort study recruiting children with CHD undergoing a cardiac procedure. The study aims to recruit 3000 participants over 5 years (2019-2024) across multiple UK sites. Routine clinical data will be collected, as well as participant questionnaires collecting sociodemographic, NHS resource use and quality of life data. Biological samples (blood, urine and surgical waste tissue from patients, and blood and urine samples from biological mothers) will be collected where consent has been obtained. Follow-up outcome and questionnaire data will be collected for 5 years. ETHICS AND DISSEMINATION: The study was approved by the London-Brent Research Ethics Committee on 30 July 2019 (19/SW/0113). Participants (or their parent/guardian if under 16 years of age) must provide informed consent prior to being recruited into the study. Mothers who wish to take part must also provide informed consent prior to being recruited. The study is sponsored by University Hospitals Bristol and Weston Foundation Trust and is managed by the University of Bristol. Children OMACp is adopted onto the National Institute for Health Research Clinical Research Network portfolio. Findings will be disseminated through peer-reviewed publications, presentation at conference, meetings and through patient organisations and newsletters. TRIAL REGISTRATION NUMBER: ISRCTN17650644.

Transposition With Unobstructed Right Aortic Arch and Bicuspid Aortic Valve.

In patients with transposition, a right aortic arch has rarely been reported among the associated malformations. When present, the arch has been obstructed because of the presence of aortic coarctation and/or arch hypoplasia. A bicuspid aortic valve is also very rare in the presence of transposition. We report here the unusual association of transposition, an unobstructed right aortic arch, and a bicuspid aortic valve in a neonate who underwent a successful arterial switch operation.

Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study.

OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events. RESULTS: Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively). CONCLUSIONS: Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present.

Left-Sided Reoperations After Arterial Switch Operation: A European Multicenter Study.

BACKGROUND: We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type. METHODS: Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.4%) between January 1975 and December 2010. Original diagnoses included D-TGA (n = 99) and DORV TGA-type (n = 12). Main indications for LSR were neoaortic valve insufficiency (n = 52 [47%]) and coronary artery problems (CAPs) (n = 21 [19%]). RESULTS: Median age at reoperation was 8.2 years (interquartile range [IQR], 2.9-14 years). Seven patients died early after LSRs (6.3%); 4 patients with D-TGA (5.9%) and 3 patients with DORV TGA-type (25%) (p = 0.02). Median age at last follow-up was 16.1 years (IQR, 9.9-21.8 years). Seventeen patients (16%) required another reoperation, which was more frequent in patients with DORV- TGA type (4 of 9 [45%]) than in patients with D-TGA (13 of 95 [14%]). Late death occurred in 4 patients (4 of 104 [3.8%]). The majority of survivors were asymptomatic at last clinical examination (84 of 100 [84%]). CONCLUSIONS: Reoperations for residual LSRs are infrequent but may become necessary late after an ASO, predominantly for neoaortic valve insufficiency and CAPs. Risk at reoperation is not negligible, and DORV TGA-type anatomy, as well as procedures on the coronary arteries, were significantly associated with a higher morbidity and a lower overall survival. Recurrent reoperations after LSRs may be required.

Management of complex CHD at the National Cardiothoracic Center of Excellence, University of Nigeria Teaching Hospital, Enugu: the role of foreign cardiac missions in 3.5 years.

BACKGROUND: CHD is defined as structural defect(s) in the heart and proximal blood vessels present at birth. The National Cardiothoracic Center of Excellence, University of Nigeria Teaching Hospital (UNTH), Enugu, through the aid of visiting Cardiac Missions has managed a significant number of patients within the last 3.5 years. Aim/Objective The objective of this study was to review surgical options and outcome of complex CHD among patients attending UNTH, Enugu, Enugu. Materials and Method During the period of 3.5 years (March, 2013 to June, 2016), a total of 20 cases of complex CHD were managed by cardiac missions that visited UNTH, Enugu. Their case notes and operating register were retrieved, reviewed, and analysed using SPSS version 19 (Chicago). RESULTS: There were eight females and 12 males, with a ratio of 2:5. The age range was from 5 months to 34 years with a mean of 1.7. Among all, five patients died giving a mortality rate of 25%. The operative procedures ranged from palliative shunts to complete repair. The outcome was relatively good. CONCLUSION: Complex CHD are present in our environment. Their surgical management in our centre is being made possible by periodic visits of foreign cardiac missions.

Single-stage repair of Taussig-Bing anomaly and interrupted aortic arch-type A.

The surgical repair of Taussig-Bing anomaly and associated lesions has evolved over the years from palliative procedures to complete repairs-either in two stages or in one single stage. We present a video illustrating our preferred surgical option in the treatment of Taussig-Bing anomaly, in this case, with an associated type A interrupted aortic arch.

Cor triatriatum dexter and coarctation of the aorta--a rare association in a 7-year-old child with type 1 neurofibromatosis.

Cor triatriatum dexter is an extremely rare congenital anomaly of the right atrium. It occurs because of the persistence of the right sinus venosus valve, resulting in partitioning of the right atrium. Most of the described cases of cor triatriatum dexter in the literature were incidental findings on echocardiogram or at necropsy. We present a case report of a 7-year-old girl who was referred to us for further assessment, with a possible diagnosis of coarctation of the aorta. Initial investigations confirmed not only the presence of a long segment coarctation of the aorta, but also a large obstructive membrane in the right atrium. A catheter intervention was performed to stent the coarctation segment, and the fibro-muscular shelf in the right atrium was surgically resected.

Aortic arch thrombectomy in a 2.8 kilogram neonate--a case report and review of the literature.

Aortic arch thrombus is a rare occurrence in neonates. In the few described cases, this has mainly been associated with sepsis or early postnatal interventions, such as insertion of umbilical arterial line. We describe a case of occlusive aortic arch thrombus in a neonate who presented with signs of critical coarctation and successfully underwent surgical thrombectomy on deep hypothermic circulatory arrest. We also present a review of the most recently published cases of aortic arch thrombus in neonates and the treatment options employed.

Hypoplastic left heart syndrome with anomalous origin of left coronary artery from the right pulmonary artery: successful surgical treatment in a neonate.

Anomalous origin of the left coronary artery from the right pulmonary artery in association with hypoplastic left heart syndrome is a rare congenital anomaly. We describe a successful simultaneous surgery for both anomalies during the first stage palliation in a neonate.