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Professor Marc de Leval (16 April 1941 –26 June 2022) was a pioneer in the field of congenital cardiothoracic surgery and former chair of the British Congenital Cardiac Association [https://www.bcca-uk.org/]. Professor de Leval was appointed as a Consultant Cardiothoracic Surgeon at Great Ormond Street Hospital in 1974. Throughout his distinguished career at Great Ormond Street Hospital, he worked with dedication to improve outcomes for cardiothoracic surgery. His contribution to academic cardiothoracic surgery was significant and far-reaching with over 300 peer-reviewed publications. Of particular note was his work in improving the palliation of children with cyanotic congenital heart disease by the use of the modified Blalock-Thomas-Taussig shunt and his pioneering work over many years to improve understanding of the Fontan circulation using in vitro modelling and computational fluid dynamics that led to the development of the total cavopulmonary connection. His other significant contributions include the importance of analysing surgical failures, being the co-editor of the most readable textbook in our field, and so much more. During his career, he trained many eminent surgeons from around the world, and a Marc de Leval Fellowship of The American Association for Thoracic Surgery [https://www.aats.org/about-the-foundation/the-reach-of-our-programs/foundation-honoring-our-mentors-program/marc-r-de-leval-md] now allows the opportunity for a trainee to study congenital cardiac surgery in the UK or Europe. Marc is fondly remembered by the staff at Great Ormond Street Hospital. The nurses recall his arrival at the break of day on the intensive care unit, having already been for a swim, and the diligence with which he observed his patients following surgery. Former trainees remember a man who, despite his standing, always remained courteous, approachable, and kind. Professor de Leval will be remembered not only as a skilled and dedicated surgeon but also as a distinguished scholar, one with a quest for knowledge in the pursuit of excellence.
A 9 mm Occlutech septal occluder Flex II device was retrieved in a 4-year-old 22 kg child; A 6 Fr Cook® Flexor sheath (child) was inserted into a 9 Fr Occlutech® ASD Delivery Set (mother). Once the tip of the smaller sheath was close to the device, a 4 Fr right Judkins catheter was introduced with a snare. The right atrial hub was captured and withdrawn to the level of the 6 Fr sheath which was then withdrawn into the 9 Fr sheath before being removed completely. The “mother and child” technique offers a greater likelihood of slenderising and retrieving embolised devices.
We describe a cohort of children referred with multisystem inflammatory syndrome in children associated with severe acute respiratory syndrome coronavirus 2 and compare this cohort with a 2019 cohort of children with Kawasaki disease.
We conducted a retrospective cohort study of 2019 and 2020 referrals to the inflammatory cardiology service at Great Ormond Street Hospital for Children. We compared cardiac and inflammatory parameters of a sub-section of the 2020 cohort who presented with reduced left ventricular ejection fraction with the remainder of the cohort.
Referrals significantly increased between February and June 2020 compared to 2019 (19.8/30 days versus 3.9/30 days). Frequency of coronary artery aneurysms (11/79 (13.9%) versus 7/47 (14.9%)) or severe coronary artery aneurysms (6/79 (7.6%) versus 3/47 (6.4%)) was similar between 2020 and 2019, respectively. The 2020 cohort was older (median age 9.07 years versus 2.38 years), more likely to be of Black, Asian, or other minority ethnic group (60/76 (78.9%) versus 25/42 (59.5%)), and more likely to require inotropic support (22 (27.5%) versus 0 (0%)). Even children with significantly reduced left ventricular ejection fraction demonstrated complete recovery of cardiac function within 10 days (mean 5.25 days ± 2.7).
We observed complete recovery of myocardial dysfunction and an overall low rate of permanent coronary sequelae, indicating that the majority of children with multisystem inflammatory syndrome in children are unlikely to encounter long-term cardiac morbidity. Although the frequency of myocardial dysfunction and inotropic support requirement is not consistent with a diagnosis of Kawasaki disease, the frequency of coronary artery abnormalities and severe coronary artery abnormalities suggests a degree of phenotypic overlap.
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.
We determined the relationship between aortic arch anatomy in tetralogy of Fallot with pulmonary stenosis and chromosomal or genetic abnormality, by performing analysis of 257 consecutive patients undergoing surgical repair from January, 2003 to March, 2011. Chromosomal or genetic abnormality was identified in 49 of the 257 (19%) patients. These included trisomy 21 (n = 14); chromosome 22q11.2 deletion (n = 16); other chromosomal abnormalities (n = 9); CHARGE (n = 2); Pierre Robin (n = 2); and Kabuki, Alagille, Holt–Oram, Kaufman McKusick, Goldenhar, and PHACE (n = 1 each). Aortic anatomy was classified as left arch with normal branching, right arch with mirror image branching, left arch with aberrant right subclavian artery, or right arch with aberrant left subclavian artery. Associated syndromes occurred in 33 of 203 (16%) patients with left arch and normal branching (odds ratio 1); three of 36 (8%) patients with right arch and mirror image branching (odds ratio 0.4, 95% confidence interval 0.1–1.6); seven of eight (88%) patients with left arch and aberrant right subclavian artery (odds ratio 36, 95% confidence interval 4–302); and six of 10 (60%) patients with right arch and aberrant left subclavian artery (odds ratio 8, 95% confidence interval 2–26). Syndromes were present in 13 of 18 (72%) patients with either right or left aberrant subclavian artery (odds ratio 15, 95% confidence interval 4–45). Syndromes in patients with an aberrant subclavian artery included trisomy 21 (n = 4); chromosome 22q11.2 deletion (n = 5); and Holt–Oram, PHACE, CHARGE, and chromosome 18p deletion (n = 1 each). Aberrant right or left subclavian artery in tetralogy of Fallot with pulmonary stenosis is associated with an increased incidence of chromosomal or genetic abnormality, whereas right aortic arch with mirror image branching is not. The assessment of aortic arch anatomy at prenatal diagnosis can assist counselling.
To assess the cardiac catheterisation findings of all children in whom cardiac magnetic resonance imaging found great artery stenosis.
We conducted a retrospective analysis of all 45 consecutive children with congenital cardiac disease who were undergoing cardiac catheterisation for intervention on cardiac magnetic resonance-defined great vessel stenosis, between January, 2006 and August, 2008.
Following cardiac magnetic resonance, 60 significant great vessel stenoses were identified and referred to cardiac catheterisation for intervention. All patients were catheterised within a median and interquartile range of 84 and 4–149 days, respectively, of cardiac magnetic resonance. At cardiac catheterisation, the children were aged 11.5 years – with an interquartile range of 3.8–16.9 years – and weighed 34 kilograms – with an interquartile range of 15–56 kilograms. Comparing cardiac magnetic resonance and cardiac catheterisation findings, 53 (88%) findings were concordant and seven were discordant. In six of seven (86%) discordant observations, cardiac magnetic resonance defined moderate–severe great vessel stenosis – involving three branch pulmonary arteries and three aortas. This was not confirmed by cardiac catheterisation, which revealed mild stenoses and haemodynamic gradients insufficient for intervention. In one patient, a mild, proximal right pulmonary artery narrowing was found at cardiac catheterisation, which was not mentioned in the cardiac magnetic resonance report. There was no difference between discordant and concordant groups on the basis of patient age, weight, interval between cardiac magnetic resonance and cardiac catheterisation, or type of lesion.
Invasive assessment confirmed cardiac magnetic resonance-diagnosed great vessel stenosis in the majority of this cohort. The predominant discordant finding was lower catherisation gradient than predicted by morphologic and functional cardiac magnetic resonance assessment. Flow volume diversion – for example, unilateral pulmonary artery stenosis – and anaesthetic effects may account for some differences. Prospective refinement of cardiac magnetic resonance and interventional data may further improve the validity of non-invasive imaging thresholds for intervention.