Paediatric aortic valve replacement using decellularized allografts: a multicentre update following 143 implantations and five-year mean follow-up.

OBJECTIVES: Decellularized aortic homografts (DAH) were introduced in 2008 as a further option for paediatric aortic valve replacement (AVR). METHODS: Prospective, multicentre follow-up of all paediatric patients receiving DAH for AVR in 8 European centres. RESULTS: A total of 143 DAH were implanted between February 2008 and February 2023 in 137 children (106 male, 74%) with a median age of 10.8 years (interquartile range 6.6-14.6). Eighty-four (59%) had undergone previous cardiac operations and 24 (17%) had undergone previous AVR. The median implanted DAH diameter was 21 mm (interquartile range 19-23). The median operation duration was 348 min (227-439) with a median cardiopulmonary bypass time of 212 min (171-257) and a median cross-clamp time of 135 min (113-164). After a median follow-up of 5.3 years (3.3-7.2, max. 15.2 years), the primary efficacy end-points peak gradient (median 14 mmHg, 9-28) and regurgitation (median 0.5, interquartile range 0-1, grade 0-3) showed good results but an increase over time. Freedom from death/explantation/endocarditis/bleeding/thromboembolism at 5 years were 97.8 ± 1.2/88.7 ± 3.3/99.1 ± 0.9/100 and 99.2 ± 0.8%, respectively. Freedom from death/explantation/endocarditis/bleeding/thromboembolism at 10 years were 96.3 ± 1.9/67.1 ± 8.0/93.6 ± 3.9/98.6 ± 1.4 and 86.9 ± 11.6%, respectively. In total, 21 DAH were explanted. Seven were replaced by a mechanical AVR, 1 Ross operation was performed and a re-do DAH was implanted in 13 patients with no redo mortality. The calculated expected adverse events were lower for DAH compared to cryopreserved homograft patients (mean age 8.4 years), and in the same range as for Ross patients (9.2 years) and mechanical AVR (13.0 years). CONCLUSIONS: This large-scale prospective analysis demonstrates excellent mid-term survival using DAH with adverse event rates comparable to paediatric Ross procedures.

Five-year results from a prospective, single-arm European trial on decellularized allografts for aortic valve replacement-the ARISE Study and ARISE Registry Data.

OBJECTIVES: Decellularized aortic homografts (DAH) were introduced as a new option for aortic valve replacement for young patients. METHODS: A prospective, EU-funded, single-arm, multicentre study in 8 centres evaluating non-cryopreserved DAH for aortic valve replacement. RESULTS: A total of 144 patients (99 male) were prospectively enrolled in the ARISE Trial between October 2015 and October 2018 with a median age of 30.4 years [interquartile range (IQR) 15.9-55.1]; 45% had undergone previous cardiac operations, with 19% having 2 or more previous procedures. The mean implanted DAH diameter was 22.6 mm (standard deviation 2.4). The median operation duration was 312 min (IQR 234-417), the median cardiopulmonary bypass time was 154 min (IQR 118-212) and the median cross-clamp time 121 min (IQR 93-150). No postoperative bypass grafting or renal replacement therapy were required. Two early deaths occurred, 1 due to a LCA thrombus on day 3 and 1 due ventricular arrhythmia 5 h postoperation. There were 3 late deaths, 1 death due to endocarditis 4 months postoperatively and 2 unrelated deaths after 5 and 7 years due to cancer and Morbus Wegener resulting in a total mortality of 3.47%. After a median follow-up of 5.9 years [IQR 5.1-6.4, mean 5.5 years. (standard deviation 1.3) max. 7.6 years], the primary efficacy end-points peak gradient with median 11.0 mmHg (IQR 7.8-17.6) and regurgitation of median 0.5 (IQR 0-0.5) of grade 0-3 were excellent. At 5 years, freedom from death/reoperation/endocarditis/bleeding/thromboembolism were 97.9%/93.5%/96.4%/99.2%/99.3%, respectively. CONCLUSIONS: The 5-year results of the prospective multicentre ARISE trial continue to show DAH to be safe for aortic valve replacement with excellent haemodynamics.

How to counteract the lack of donor tissue in cardiac surgery? Initial experiences with a newly established homograft procurement program.

Homograft heart valves may have significant advantages and are preferred for the repair of congenital valve malformations, especially in young women of childbearing age, athletes and in patients with active endocarditis. A growing problem, however, is the mismatch between tissue donation and the increasing demand. The aim of this paper is to describe the initiation process of a homograft procurement program to attenuate the shortage of organs. A comprehensive description of the infrastructure and procedural steps required to initiate a cardiac and vascular tissue donation program combined with a prospective follow-up of all homografts explanted at our institution. Between January 2020 and May 2022, 28 hearts and 12 pulmonary bifurcations were harvested at our institution and delivered to the European homograft bank. Twenty-seven valves (19 pulmonary valves, 8 aortic valves) were processed and allocated for implantation. The reasons for discarding a graft were either contamination (n = 14), or morphology (n = 13) or leaflet damage (n = 2). Five homografts (3 PV, 2 AV) have been cryopreserved and stored while awaiting allocation. One pulmonary homograft with a leaflet cut was retrieved by bicuspidization technique and awaits allocation, as a highly requested small diameter graft. The implementation of a tissue donation program in cooperation with a homograft bank can be achieved with reasonable additional efforts at a transplant center with an in-house cardiac surgery department. Challenging situations with a potential risk of tissue injury during procurement include re-operation, harvesting by a non-specialist surgeon and prior central cannulation for mechanical circulatory support.

The Congenital Cardiology Cloud - optimizing long-term care by connecting ambulatory and hospital medical attendance via telemedicine.

BACKGROUND: Patients with complex congenital heart disease frequently undergo a life-long ambulatory therapy with the need for repeated hospital interventions. To optimize this manifold interplay, we designed and implemented a tele-medical service, the Congenital Cardiology Cloud (CCC). This study aims to analyse the requirements for its implementation through the comprehensive assessment of design, installation and impact on patient´s care. METHODS: CCC's development comprised the analysis of historically raised admission and discharge management and the definition of technical and organizational requirements. Elaboration of procedural flow charts, description of data formats and technical processes as well as distribution of patient structure formed part of this process. RESULTS: Analysis of existing workflows uncovered a need for the rebuilding of admission and discharge process and decision making for further treatment. The CCC reduces conference-meetings in general and repetitive meetings up to less than a third. Real-time dispatch of discharge documents guarantees an instantaneous access to patient-related data. Comparative analyses show a more complex patient group to be involved in tele-medical services. CONCLUSIONS: The CCC enables the sharing of complex clinical information by overcoming sectoral barriers and improves mutual patient advice. Implementation of a tele-medical network requires willingness, perseverance and professional engagement. Future application analysis and possible introduction of refinancing concepts will show its long-term feasibility.

Long-Term Effects of Child Early Surgical Ventricular Septal Defect Repair on Maternal Stress.

The ventricular septal defect (VSD) represents the most common congenital heart defect (CHD). The diagnosis of and cardiac surgery for their child's VSD are highly stressful experiences for parents; especially mothers, who are at risk of developing long-lasting stress-related symptoms. This study examined long-term alterations in maternal stress including self-reported psychological and biophysiological stress levels in a case-control design. We investigated 24 mothers of children with an isolated, surgically corrected VSD compared to non-affected controls. Maternal self-reports on psychopathology, everyday stress, parenting stress and hair cortisol concentrations (HCC) were measured during children's primary school age (6-9 years, t1) and early adolescence (10-14 years, t2). In maternal self-reports, psychopathology and stress symptoms in the VSD-group and controls were comparable at t1, whereas at t2, mothers in the VSD-group even showed a decrease in psychopathology. Maternal HCC levels in the VSD-group were significantly lower (hypocortisolism) than HCC levels of controls at t1. This effect was no longer observed at t2 reflecting an approximation of HCC levels in the VSD-group to controls' levels. This study highlights the potential for improved stress hormone balance and psychological well-being in mothers following their child's surgical VSD repair. However, the need for parent-centered interventions is discussed, particularly during peri-operative phases and in early child developmental stages.

XCR1 expression distinguishes human conventional dendritic cell type 1 with full effector functions from their immediate precursors.

Dendritic cells (DCs) are major regulators of innate and adaptive immune responses. DCs can be classified into plasmacytoid DCs and conventional DCs (cDCs) type 1 and 2. Murine and human cDC1 share the mRNA expression of XCR1. Murine studies indicated a specific role of the XCR1-XCL1 axis in the induction of immune responses. Here, we describe that human cDC1 can be distinguished into XCR1- and XCR1+ cDC1 in lymphoid as well as nonlymphoid tissues. Steady-state XCR1+ cDC1 display a preactivated phenotype compared to XCR1- cDC1. Upon stimulation, XCR1+ cDC1, but not XCR1- cDC1, secreted high levels of inflammatory cytokines as well as chemokines. This was associated with enhanced activation of NK cells mediated by XCR1+ cDC1. Moreover, XCR1+ cDC1 excelled in inhibiting replication of Influenza A virus. Further, under DC differentiation conditions, XCR1- cDC1 developed into XCR1+ cDC1. After acquisition of XCR1 expression, XCR1- cDC1 secreted comparable level of inflammatory cytokines. Thus, XCR1 is a marker of terminally differentiated cDC1 that licenses the antiviral effector functions of human cDC1, while XCR1- cDC1 seem to represent a late immediate precursor of cDC1.

Risk Factors and Outcome of Pulmonary Artery Stenting After Bidirectional Cavopulmonary Connection (BDCPC) in Single Ventricle Circulation.

After bidirectional cavopulmonary connection (BDCPC) central pulmonary arteries (PAs) of single ventricle (SV) patients can be affected by stenosis or even closure. Aim of this study is to compare SV patients with and without PA-stent implantation post-BDCPC regarding risk factors for stent implantation and outcome. Single center, retrospective (2006-2021) study of 136 SV consecutive patients with and without PA-stent implantation post-BDCPC. Patient characteristics, risk factors for PA-stent implantation and PA growth were assessed comparing angiographic data pre-BDCPC and pre-TCPC. A total of 40/136 (29%) patients underwent PA-stent implantation at median (IQR) 14 (1.1-39.0) days post-BDCPC. 37/40 (92.5%) underwent LPA-stenting. Multiple regression analysis showed single LV patients to receive less likely PA-stents than single RV patients (OR 0.41; p = 0.05). Reduced LPA/BSA (mm/m2) and larger diameter of neo-ascending aorta pre-BDCPC were associated with an increased likelihood of PA-stent implantation post-BDCPC (OR 0.89, p = 0.03; OR 1.05, p = 0.001). Stent re-dilatation was performed in 36/40 (89%) after 1 (0.8-1.5) year. Pulmonary artery diameters pre-BDCPC were lower in the PA-stent group: McGoon (p < 0.001), Nakata (p < 0.001). Indexed pulmonary artery diameters increased equally in both groups but remained lower pre-TCPC in the PA-stent group: McGoon (p < 0.001), Nakata (p = 0.009), and Lower Lobe Index (p = 0.003). LPA and RPA grew symmetrically in both groups. Single RV, larger neo-ascending aorta, and small LPA pre- BDCPC are independent risk factors for PA-stent implantation post-BDCPC. Pulmonary artery diameters after PA-stent implantation and stent re-dilatation showed significant growth together with the contralateral side, but the PA-system remained symmetrically smaller in the stent group.

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.

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.

Anomalous Origin of Left Circumflex Coronary Artery From Right Pulmonary Artery: An Unusual Cause of Sudden Cardiac Arrest.

Anomalous origin of the left circumflex coronary artery from the right pulmonary artery is an extremely rare coronary anomaly out of the group of anomalous coronary arteries arising from the pulmonary artery. We present the case of a 27-year-old male, in whom the diagnosis of an anomalous left circumflex coronary artery from the pulmonary artery was made after sudden cardiac arrest. The diagnosis was confirmed by multimodal imaging and the patient underwent successful surgical correction. Abnormal origins of a coronary artery may become symptomatic later in life and may occur as an isolated cardiac malformation. Due to a potentially unfavorable clinical course, surgical correction should be considered as soon as a diagnosis is made.

Exceptional case of aortic atresia associated with atypical double aortic arch.

We present the unique case of atretic aortic valve associated with hypoplastic ascending aorta and double aortic arch of unusual configuration supplying retrograde blood flow to the coronary arteries.

Safety issues with an inter-hospital transport of a patient with a Berlin Heart Excor biventircular assist device.

BACKGROUND: Interhospital transfers of pediatric patients on the Berlin Heart Excor have been published on an occasional basis. METHODS: Nowadays medicine evolves away from just feasibility towards quality and safety issues. Management tools like risk analysis have found their way into clinical practice. RESULTS: Exemplary, we present a case of a 20 months old boy on a Berlin Heart BiVAD Excor who underwent a 224 km ground transport. After a systematic review of the published literature, we describe our safety management with the aim was to provide highest quality of care for the transport. CONCLUSION: Besides a risk analysis, we also describe our training and simulation protocol.

Becoming a Teenager after Early Surgical Ventricular Septal Defect (VSD) Repair: Longitudinal Biopsychological Data on Mental Health and Maternal Involvement.

Beside somatic strains of congenital heart diseases (CHD), affected children often show developmental impairments in the long term. Ventricular septal defect (VSD) is the most common congenital heart defect and early surgical repair is associated with positive somatic outcomes. However, psychological adjustment is of lifelong relevance. We investigated 24 children with a surgically-corrected isolated VSD and their mothers from primary school (6-9 years) to adolescence (10-14 years) and compared them to controls. Both times, mothers reported child internalizing/externalizing problems, mothers and children rated child quality of life, and children performed neurodevelopmental tests. Adolescents also rated internalizing/externalizing problems themselves, and their hair cortisol levels were analyzed. Maternal anxiety and proactive parenting behavior were considered as moderators. Results revealed no group differences in child neurodevelopment (language, cognition), externalizing problems, and cortisol levels at any time. In reports from mothers, internalizing problems (depression, anxiety) were elevated in children with a VSD at both times-when mothers reported anxiety symptoms themselves. In adolescent reports, VSD patients' quality of life was increased and internalizing problems were decreased-proactive parenting behavior went along with decreased symptoms in VSD-affected adolescents and with increased symptoms in controls. The findings pronounce the crucial role of parenting behavior and the influence of maternal anxieties on child mental health after surgical VSD repair and might highlight the need for parent-centered interventions.

Significant adhesion reduction and time saving in pediatric heart surgery with 4DryField PH: A retrospective, controlled study.

Adhesions formation after surgery for congenital heart defects can complicate follow-up procedures due to bleeding from detached adhesion bands, injury to cardiac structures or large vessels, all of which do prolong operation times. The problem is enhanced by the fact that detached adhesions are predilection sites for new adhesions setting off a downward spiral. 4DryField® PH gel barrier has demonstrated high efficacy in reducing postoperative adhesions in general surgical and gynecological studies. This retrospective controlled study of 22 patients evaluates whether these positive results can be confirmed in pediatric cardiac surgery. Adhesions were scored from photographs of follow-up interventions by an independent cardiac surgeon blinded to group assignment. The publication provides not only score numbers but also original photographs of all sites for better traceability and transparency. In addition, timesaving due to reduced adhesions was evaluated. Results show a significantly reduced adhesion score for the 4DryField® group. Importantly, this resulted in a significantly shorter period between skin incision and start of cardiopulmonary bypass. In addition, timesaving due reduced adhesion formation was evaluated. The use of 4DryField® was safe, although higher doses per kg were used than in adults.

Surgical correction of partial anomalous left superior pulmonary venous connection to the innominate vein.

Partial anomalous pulmonary venous connection is reported to occur in 0.4 to 0.7% of children. Only 3% of these cases involve an anomalous left-sided pulmonary venous connection to the innominate vein [1]. In this video tutorial, we present the technique of reimplantation of the partial anomalous left superior pulmonary venous connection to the left atrial appendage through a median sternotomy and with a cardioplegic heart. We debate the merits of our strategy with respect to our ultimate goal of achieving long-term patency.

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.

Long-Term Results after Fallot Repair.

BACKGROUND: The aim of this study was to evaluate the long-term outcome and freedom from pulmonary valve replacement (PVR) after initial repair of tetralogy of Fallot (TOF). PATIENTS AND METHODS: The cohort of 306 patients treated between 1980 and 2017 was divided into anatomical subgroups according to the diagnosis of TOF-pulmonary stenosis, TOF-pulmonary atresia and TOF-double outlet right ventricle. Patients were treated with transannular patch (TAP), valve sparing repair (VSR), or conduits from the right ventricle to the pulmonary arteries (RVPA conduits). RESULTS: There were 21 deaths (6.9%), 14 being hospital deaths (4.6%) after primary correction and four deaths (1.3%) occurred after PVR. One patient died after a non-cardiac operation (0.3%). There were two late deaths (0.7%). During the past 12 years no early mortality has been observed. Ninety-one patients (30.4%) received PVR after a median of 12.1 ± 7.0 years with an early mortality of 4.4% (n = 4) and no late mortality. A significant difference in freedom from reoperation after TAP, VSR, and RVPA-conduits could be identified. Multivariate analysis displayed transannular repair (p = 0.016), primary palliation (p <0.001), the presence of major aortopulmonary collateral arteries (MAPCA; p = 0.023), and pulmonary valve Z-scores < - 4.0 (p = 0.040) as significant risk factors for PVR. CONCLUSION: TOF repair has a beneficial long-term prognosis with low morbidity and mortality. Pulmonary valve Z-scores < - 4.0, transannular repair, and presence of MAPCAs are associated with earlier PVR. Non-VSRs and TOF-pulmonary atresia lead to earlier reoperation but have no negative impact on survival.

Repair of a remote pericommissural anomalous left coronary artery from the pulmonary artery.

We demonstrate a classic trapdoor technique to correct a late-presenting remote pericommissural anomalous left coronary artery from the pulmonary artery. The merits of the technique compared to alternative techniques are explained. The impact of late diagnosis on left ventricular function as well as on papillary muscle ischemia-induced mitral regurgitation is evident. The child's recovery without the need for a temporary ventricular assist device supports the efficacy of  the technique, despite the residual mitral regurgitation.

Transfontanellar Contrast-enhanced US for Intraoperative Imaging of Cerebral Perfusion during Neonatal Arterial Switch Operation.

Background Brain injury and subsequent neurodevelopmental disorders are major determinants for later-life outcomes in neonates with transposition of the great arteries (TGA). Purpose To quantitatively assess cerebral perfusion in neonates with TGA undergoing arterial switch operation (ASO) using transfontanellar contrast-enhanced US (T-CEUS). Materials and Methods In a prospective single-center cross-sectional diagnostic study, neonates with TGA scheduled for ASO were recruited from February 2018 to February 2020. Measurements were performed at five time points before, during, and after surgery (T1-T5), and 11 perfusion parameters were derived per cerebral hemisphere. Neonate clinical characteristics, heart rate, mean arterial pressure, central venous pressure, near-infrared spectroscopy, blood gas analyses, ventilation time, time spent in the pediatric intensive care unit, and time in hospital were correlated with imaging parameters. Analysis of variance or a mixed-effects model were used for groupwise comparisons. Results A total of 12 neonates (mean gestational age, 39 6/7 weeks ± 1/7 [SD]) were included and underwent ASO a mean of 6.9 days ± 3.4 after birth. When compared with baseline values, T-CEUS revealed a longer mean time-to-peak (right hemisphere, 4.3 seconds ± 2.1 vs 17 seconds ± 6.4 [P < .001]; left hemisphere, 4.0 seconds ± 2.3 vs 21 seconds ± 8.7 [P < .001]) and rise time (right hemisphere, 3.5 seconds ± 1.7 vs 11 seconds ± 5.1 [P = .002]; left hemisphere, 3.4 seconds ± 2.0 vs 22 seconds ± 7.8 [P = .004]) in both cerebral hemispheres during low-flow cardiopulmonary bypass and hypothermia (T4) for all neonates. Neonate age at surgery negatively correlated with T-CEUS parameters during ASO, as calculated with the area under the flow curve (AUC) during wash-in (R = -0.60, P = .020), washout (R = -0.82, P = .002), and both wash-in and washout (R = -0.79, P = .004). Mean AUC values were lower in neonates older than 7 days compared with younger neonates during wash-in ([87 arbitrary units {au} ± 77] × 102 vs [270 au ± 164] × 102, P = .049]), washout ([15 au ± 11] × 103 vs [65 au ± 38] × 103, P = .020]) and both wash-in and washout ([24 au ± 18] × 103 vs [92 au ± 53] × 103, P = .023). Conclusion Low-flow hypothermic conditions resulted in reduced cerebral perfusion, as measured with transfontanellar contrast-enhanced US, which inversely correlated with age at surgery. Clinical trial registration no. NCT03215628 © RSNA, 2022 Online supplemental material is available for this article.

A classification of abdominal lymphatic perfusion patterns after Fontan surgery.

OBJECTIVES: Complications after Fontan surgery have been associated with arise and classification of abnormal thoracic lymphatic perfusion pattern. This study compiles abnormal abdominal lymphatic perfusion patterns and investigates their impact on serum protein readings. METHODS: We performed a retrospective analysis of patients who underwent magnetic resonance imaging with T2-weighted lymphatic imaging and serum protein measurements 6 months after having Fontan surgery. The abdominal lymphatic images were classified according to the anatomical lymphatic drainage patterns into 2 categories: (1) para-aortic (types 1-4); (2) portal-venous (types 1-3). Thoracic lymphatic images were classified (types 1-4) as described earlier. RESULTS: A total of 71 patients were included in the study. Para-aortic lymphatic perfusion patterns were classified as type 1 in 4, type 2 in 13, type 3 in 37 and type 4 in16 out of 71 patients. Portal-venous lymphatic perfusion patterns were classified as type 1 in 20, type 2 in 10 and type 3 in 41 patients. Thoracic lymphatic perfusion patterns were classified as type 1 in 8, type 2 in 11, type 3 in 39 and type 4 in 13 patients. The serum protein level was 66 (interquartile range: 7.5) g/l (< standard value in 37%). Higher-grade para-aortic (p = 0.0062), portal-venous (p = 0.022) and thoracic (p = 0.011) lymphatic abnormalities were correlated with lower total serum protein levels. Higher ratings of para-aortic lymphatic abnormalities were significantly associated with higher ratings of portal-venous abnormalities (p < 0.0001). Ratings of para-aortic and portal-venous classifications were correlated with the thoracic classification (p < 0.001). CONCLUSIONS: Abnormal abdominal lymphatic perfusion patterns can be classified according to anatomical structures with increasing severity. Higher grade abdominal and thoracic lymphatic perfusion patterns are associated with lower serum protein values.