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The need for urgent or emergent cardiovascular imaging in children is rare when compared to adults. Patients may present from the neonatal period up to adolescence, and may require imaging for both traumatic and non-traumatic causes. In children, coronary pathology is rarely the cause of an emergency unlike in adults where it is the main cause. Radiology, including chest radiography and computed tomography in conjunction with echocardiography, often plays the most important role in the acute management of these patients. Magnetic resonance imaging can occasionally be useful and may be suitable in more subacute cases. Radiologists' knowledge of how to manage and interpret these acute conditions including knowing which imaging technique to use is fundamental to appropriate care. In this review, we will concentrate on the most common cardiovascular emergencies in the thoracic region, including thoracic traumatic and non-traumatic emergencies and pulmonary vascular emergencies, as well as acute clinical disorders as a consequence of primary and postoperative congenital heart disease. This review will cover situations where cardiovascular imaging may be acutely needed, and not strictly emergencies only. Imaging recommendations will be discussed according to the different clinical presentations and underlying pathology.
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Background: In children with congenital heart disease (CHD), lung scintigraphy is the reference standard for evaluation of pulmonary perfusion. 4D flow CMR offers a non-ionizing alternative. Due to the intrinsic limitation in the spatial resolution, however, 4D flow may display clinically unacceptable differences compared to the reference standard. This case study aims to highlight the importance of correcting for such partial volume errors to accurately evaluate pulmonary perfusion in small pulmonary arteries. Methods: Children with CHD, mainly those with transposition of the great arteries or tetralogy-of-Fallot, referred to CMR from 2020 to 2022 at our clinic, were retrospectively reviewed; n = 37. All patients had been examined with a free breathing, motion-corrected 4D flow protocol. Comparison in pulmonary perfusion (PPR: relative flow through right and left pulmonary arteries) with scintigraphy were performed both for 4D flow before and after partial volume correction. Results: Patients with large pulmonary arteries, 76%, displayed small differences in PPR between modalities (<20%), while patients with arteries of only a few pixels, 24%, displayed differences up to 178%, depending on the relative difference in size between the right and left pulmonary artery. Differences were effectively reduced after partial volume correction (<21%). Conclusion: The present report shows that 4D flow is a promising tool to accurately evaluate the pulmonary perfusion in children with CHD, but that partial volume correction is warranted to overcome its limitation in the spatial resolution. Without such correction, lung scintigraphy is still recommended to ensure high diagnostic certainty in children with small pulmonary arteries.
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The potential association between endurance exercise and myocardial fibrosis is controversial. Data on exercise exposure and diffuse myocardial fibrosis in endurance athletes are scarce and conflicting. We aimed to investigate the association between exercise exposure and markers of diffuse myocardial fibrosis by cardiovascular magnetic resonance imaging (CMR) in endurance athletes. We examined 27 healthy adult male competitive endurance athletes aged 41 ± 9 years and 16 healthy controls in a cross sectional study using 3 Tesla CMR including late gadolinium enhancement and T1 mapping. Athletes reported detailed exercise history from 12 years of age. Left ventricular total mass, cellular mass and extracellular mass were higher in athletes than controls (86 vs. 58 g/m2, 67 vs. 44 g/m2 and 19 vs. 13 g/m2, all p < 0.01). Extracellular volume (ECV) was lower (21.5% vs. 23.8%, p = 0.03) and native T1 time was shorter (1214 ms vs. 1268 ms, p < 0.01) in the athletes. Increasing exercise dose was independently associated with shorter native T1 time (regression coefficient - 24.1, p < 0.05), but expressed no association with ECV. Our results indicate that diffuse myocardial fibrosis has a low prevalence in healthy male endurance athletes and do not indicate an adverse dose-response relationship between exercise and diffuse myocardial fibrosis in healthy athletes.
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Cardiomiopatias , Meios de Contraste , Adulto , Humanos , Masculino , Criança , Estudos Transversais , Gadolínio , Miocárdio/patologia , Cardiomiopatias/patologia , Fibrose , Atletas , Imagem Cinética por Ressonância Magnética , Valor Preditivo dos Testes , Função Ventricular Esquerda , Volume SistólicoRESUMO
Univentricular heart anomalies represent a group of severe congenital heart defects necessitating early surgical intervention in infancy. The Fontan procedure, the final stage of single-ventricle palliation, establishes a serial connection between systemic and pulmonary circulation by channeling venous return to the lungs. The absence of the subpulmonary ventricle in this peculiar circulation progressively eventuates in failure, primarily due to chronic elevation in inferior vena cava (IVC) pressure. This study experimentally validates the effectiveness of an intracorporeally-powered venous ejector pump (VEP) in reducing IVC pressure in Fontan patients. The VEP exploits a fraction of aortic flow to create a jet-venturi effect for the IVC, negating the external power requirement and driveline infections. An invitro Fontan mock-up circulation loop is developed and the impact of VEP design parameters and physiological conditions is assessed using both idealized and patient-specific total cavopulmonary connection (TCPC) phantoms. The VEP performance in reducing IVC pressure exhibited an inverse relationship with the cardiac output and extra-cardiac conduit (ECC) size and a proportional relationship with the transpulmonary pressure gradient (TPG) and mean arterial pressure (MAP). The ideal VEP with fail-safe features provided an IVC pressure drop of 1.82 ± 0.49, 2.45 ± 0.54, and 3.12 ± 0.43 mm Hg for TPG values of 6, 8, and 10 mm Hg, respectively, averaged over all ECC sizes and cardiac outputs. Furthermore, the arterial oxygen saturation was consistently maintained above 85% during full-assist mode. These results emphasize the potential utility of the VEP to mitigate elevated venous pressure in Fontan patients.
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Técnica de Fontan , Cardiopatias Congênitas , Coração Univentricular , Humanos , Hemodinâmica , Artéria Pulmonar , Ventrículos do Coração , Cardiopatias Congênitas/cirurgia , Modelos CardiovascularesRESUMO
BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of intraoperative and postoperative complications. So far, only limited data regarding the best radiological approach to monitor children during liver transplantation is available. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra- and postoperative phase. This paper reports the responses related to intraoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: Intraoperative ultrasound (US) is used by all sites to assess the quality of the vascular anastomosis in order to ensure optimal perfusion of the liver transplant. Vessel depiction is commonly achieved using color Doppler (95.3%). Additional US-based techniques are employed by fewer centers (power angio mode, 28.6%; B-flow, 19%; contrast-enhanced US, 14.3%). Most centers prefer a collaborative approach, with surgeons responsible for probe handling, while radiologists operate the US machine (47.6%). Less commonly, the intraoperative US is performed by the surgeon alone (28.6%) or by the radiologist alone (23.8%). Timing of US, imaging frequency, and documentation practices vary among centers. CONCLUSION: Intraoperative US is consistently utilized across all sites during pediatric liver transplantation. However, considerable variations were observed in terms of the US setup, technique preferences, timing of controls, and documentation practices. These differences provide valuable insights for future optimization and harmonization studies.
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Transplante de Fígado , Radiologia , Criança , Humanos , Ultrassonografia , Radiografia , Complicações Pós-Operatórias/diagnóstico por imagemRESUMO
BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of postoperative complications. So far, limited data is available regarding the best radiologic approach to monitor children after liver transplantation. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phases. This paper reports the responses related to postoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: All sites commence ultrasound (US) monitoring within 24 h after liver transplantation. Monitoring frequency varies across sites, ranging from every 8 h to 72 h in early, and from daily to sporadic use in late postoperative phases. Predefined US protocols are used by 73% of sites. This commonly includes gray scale, color Doppler, and quantitative flow assessment. Alternative flow imaging techniques, contrast-enhanced US, and elastography are applied at 31.8%, 18.2%, and 63.6% of sites, respectively. Computed tomography is performed at 86.4% of sites when clarification is needed. Magnetic resonance imaging is used for selected cases at 36.4% of sites, mainly for assessment of biliary abnormalities or when blood tests are abnormal. CONCLUSION: Diagnostic imaging is extensively used for postoperative surveillance of children after liver transplantation. While US is generally prioritized, substantial differences were noted in US protocol, timing, and monitoring frequency. The study highlights potential areas for future optimization and standardization of imaging, essential for conducting multicenter studies.
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Transplante de Fígado , Radiologia , Criança , Humanos , Ultrassonografia , Imageamento por Ressonância Magnética/métodos , Ultrassonografia Doppler , Complicações Pós-Operatórias/diagnóstico por imagemRESUMO
BACKGROUND: Liver transplantation is the state-of-the-art curative treatment in end-stage liver disease. Imaging is a key element for successful organ-transplantation to assist surgical planning. So far, only limited data regarding the best radiological approach to prepare children for liver transplantation is available. OBJECTIVES: In an attempt to harmonize imaging surrounding pediatric liver transplantation, the European Society of Pediatric Radiology (ESPR) Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phase. This paper reports the responses on preoperative imaging. MATERIAL AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted and 22 institutions from 11 countries returned the survey. From 2018 to 2020, the participating centers collectively conducted 1,524 transplantations, with a median of 20 transplantations per center per annum (range, 8-60). RESULTS: Most sites (64%) consider ultrasound their preferred modality to define anatomy and to plan surgery in children before liver transplantation, and additional cross-sectional imaging is only used to answer specific questions (computed tomography [CT], 90.9%; magnetic resonance imaging [MRI], 54.5%). One-third of centers (31.8%) rely primarily on CT for pre-transplant evaluation. Imaging protocols differed substantially regarding applied CT scan ranges, number of contrast phases (range 1-4 phases), and applied MRI techniques. CONCLUSION: Diagnostic imaging is generally used in the work-up of children before liver transplantation. Substantial differences were noted regarding choice of modalities and protocols. We have identified starting points for future optimization and harmonization of the imaging approach to multicenter studies.
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Transplante de Fígado , Radiologia , Criança , Humanos , Ultrassonografia , Tomografia Computadorizada por Raios X , Imageamento por Ressonância Magnética/métodosRESUMO
Fontan-type surgery is the final step in the sequential palliative surgical treatment of infants born with a univentricular heart. The resulting long-term haemodynamic changes promote liver damage, leading to Fontan-associated liver disease (FALD), in virtually all patients with Fontan circulation. Owing to the lack of a uniform definition of FALD and the competitive risk of other complications developed by Fontan patients, the impact of FALD on the prognosis of these patients is currently debatable. However, based on the increasing number of adult Fontan patients and recent research interest, the European Association for The Study of the Liver and the European Reference Network on Rare Liver Diseases thought a position paper timely. The aims of the current paper are: (1) to provide a clear definition and description of FALD, including clinical, analytical, radiological, haemodynamic, and histological features; (2) to facilitate guidance for staging the liver disease; and (3) to provide evidence- and experience-based recommendations for the management of different clinical scenarios.
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Técnicas de Imagem por Elasticidade , Técnica de Fontan , Hepatopatias , Adulto , Lactente , Humanos , Técnica de Fontan/efeitos adversos , Hepatopatias/diagnóstico , Hepatopatias/etiologia , Hepatopatias/cirurgia , Prognóstico , Técnicas de Imagem por Elasticidade/métodosRESUMO
BACKGROUND: It is unknown how shear wave dispersion (SWD) is displayed in pediatric liver transplant recipients and not fully elucidated how ultrasound shear wave elastography (2D-SWE) display within this cohort, which is important to determine to improve noninvasive surveillance of these patients. The study aimed to compare SWE and SWD values with histopathology in pediatric liver recipients. METHODS: Forty-eight pediatric liver recipients were examined with SWE in conjunction with an elective liver biopsy (clinically without complication). Additionally, SWD values were measured in 21 children. SWE and SWD values were compared to histologically determined fibrosis graded as none-to-mild (F0-1) and moderate-to-severe (F2-4), and inflammation graded as low (grade 0-1) and high (grade 2-4). RESULTS: Two children were excluded due to SWE IQR/median > 30% kPa. The mean age across 46 included patients was 10.9 years (range 1.4-18). The number of patients and median (range) SWE value (kPa) for each stage of fibrosis were: F0-1 [n = 23; 5.8 (3.2-16.1)], F2 [n = 22; 6.0 (4.5-25.9)], F3 [n = 1; 33.3], and F4 [n = 0]. Significantly higher SWE values and greater variability were registered in F2-4 vs. F0-1 (p = .05). Grade of fibrosis correlated weakly to SWE values (r = .3; p = .05), but not to SWD values (r = .2; p = .27). In patients with low-grade inflammation, median SWD was 13.7 m/s KHz (10.7-17.6). Only one patient had high-grade inflammation. CONCLUSIONS: Uncomplicated transplanted liver grafts in a small pediatric cohort revealed slightly increased SWE and SWD values compared to previously reported values in healthy children. This likely reflect both the fibrotic and inflammatory elements in the grafts; however, other confounders impacting the liver's viscoelastic properties are also probable factors.
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PURPOSE: The Fontan circulation carries a dismal prognosis in the long term due to its peculiar physiology and lack of a subpulmonic ventricle. Although it is multifactorial, elevated IVC pressure is accepted to be the primary cause of Fontan's high mortality and morbidity. This study presents a self-powered venous ejector pump (VEP) that can be used to lower the high IVC venous pressure in single-ventricle patients. METHODS: A self-powered venous assist device that exploits the high-energy aortic flow to lower IVC pressure is designed. The proposed design is clinically feasible, simple in structure, and is powered intracorporeally. The device's performance in reducing IVC pressure is assessed by conducting comprehensive computational fluid dynamics simulations in idealized total cavopulmonary connections with different offsets. The device was finally applied to complex 3D reconstructed patient-specific TCPC models to validate its performance. RESULTS: The assist device provided a significant IVC pressure drop of more than 3.2 mm Hg in both idealized and patient-specific geometries, while maintaining a high systemic oxygen saturation of more than 90%. The simulations revealed no significant caval pressure rise (< 0.1 mm Hg) and sufficient systemic oxygen saturation (> 84%) in the event of device failure, demonstrating its fail-safe feature. CONCLUSIONS: A self-powered venous assist with promising in silico performance in improving Fontan hemodynamics is proposed. Due to its passive nature, the device has the potential to provide palliation for the growing population of patients with failing Fontan.
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Técnica de Fontan , Cardiopatias Congênitas , Coração Auxiliar , Humanos , Técnica de Fontan/efeitos adversos , Artéria Pulmonar/cirurgia , Hemodinâmica , Veia Cava Inferior , Ventrículos do Coração/cirurgia , Modelos Cardiovasculares , Cardiopatias Congênitas/cirurgiaRESUMO
The Fontan operation is a lifesaving procedure for patients with functional single-ventricle congenital heart disease, where hypoplastic left heart syndrome is the most frequent anomaly. Hemodynamic changes following Fontan circulation creation are now increasingly recognized to cause multiorgan affection, where the development of a chronic liver disease, Fontan-associated liver disease (FALD), is one of the most important morbidities. Virtually, all patients with a Fontan circulation develop liver congestion, resulting in fibrosis and cirrhosis, and most patients experience childhood onset. FALD is a distinctive type of congestive hepatopathy, and its pathogenesis is thought to be a multifactorial process driven by increased nonpulsatile central venous pressure and decreased cardiac output, both of which are inherent in the Fontan circulation. In the advanced stage of liver injury, complications of portal hypertension often occur, and there is a risk of developing secondary liver cancer, reported at young age. However, FALD develops with few clinical symptoms, a surprisingly variable degree of severity in liver disease, and with little relation to poor cardiac function. The disease mechanisms and modifying factors of its development are still not fully understood. As one of the more important noncardiac complications of the Fontan circulation, FALD needs to be diagnosed in a timely manner with a structured monitoring scheme of disease development, early detection of malignancy, and determination of the optimal time point for transplantation. There is also a clear need for consensus on the best surveillance strategy for FALD. In this regard, imaging plays an important role together with clinical scoring systems, biochemical workups, and histology. Patients operated on with a Fontan circulation are generally followed up in cardiology units. Ultimately, the resulting multiorgan affection requires a multidisciplinary team of healthcare personnel to address the different organ complications. This article discusses the current concepts, diagnosis, and management of FALD, with special emphasis on the role of different imaging techniques in the diagnosis and monitoring of disease progression, as well as current recommendations for liver disease surveillance.
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Ecologically valid research and wearable brain imaging are increasingly important in cognitive neuroscience as they enable researchers to measure neural mechanisms of complex social behaviors in real-world environments. This article presents a proof of principle study that aims to push the limits of what wearable brain imaging can capture and find new ways to explore the neuroscience of acting. Specifically, we focus on how to build an interdisciplinary paradigm to investigate the effects of taking on a role on an actor's sense of self and present methods to quantify interpersonal coordination at different levels (brain, physiology, behavior) as pairs of actors rehearse an extract of a play prepared for live performance. Participants were six actors from Flute Theatre, rehearsing an extract from Shakespeare's A Midsummer Night's Dream. Sense of self was measured in terms of the response of the pFC to hearing one's own name (compared with another person's name). Interpersonal coordination was measured using wavelet coherence analysis of brain signals, heartbeats, breathing, and behavior. Findings show that it is possible to capture an actor's pFC response to their own name and that this response is suppressed when an actor rehearses a segment of the play. In addition, we found that it is possible to measure interpersonal synchrony across three modalities simultaneously. These methods open the way to new studies that can use wearable neuroimaging and hyperscanning to understand the neuroscience of social interaction and the complex social-emotional processes involved in theatrical training and performing theater.
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Relações Interpessoais , Dispositivos Eletrônicos Vestíveis , Humanos , Espectroscopia de Luz Próxima ao Infravermelho , Comportamento Social , Mapeamento EncefálicoRESUMO
Aim: To evaluate the feasibility of multiple ultrasound markers for the non-invasive characterization of fibrosis, inflammation and steatosis in the liver in pediatric patients. Materials and methods: The quantitative ultrasound measures shear wave elastography (SWE), shear wave dispersion (SWD) and attenuation imaging (ATI) were compared and correlated with percutaneous liver biopsies and corresponding measures in a control cohort. Results: The median age of the 32 patients was 12.1 years (range 0.1−17.9), and that of the 15 controls was 11.8 years (range: 2.6−16.6). Results: There was a significant difference in SWE values between histologic grades of fibrosis (p = 0.003), with a positive correlation according to the grade (r = 0.7; p < 0.0001). Overall, a difference in SWD values between grades of inflammation was found (p = 0.009) but with a lack of correlation (r = 0.1; p = 0.67). Comparing inflammation grades 0−1 (median:13.6 m/s kHz [min; max; 8.4; 17.5]) versus grades 2−3 (16.3 m/s kHz [14.6; 24.2]) showed significant differences between the groups (p = 0.003). In the 30 individuals with a steatosis score of 0, ATI was measured in 23 cases with a median value of 0.56 dB/cm/MHz. Conclusion: Comprehensive ultrasound analysis was feasible to apply in children and has the potential to reflect the various components of liver affection non-invasively. Larger studies are necessary to conclude to what extent these image-based markers can classify the grade of fibrosis, inflammation and steatosis.
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To evaluate the diagnostic accuracy of ultrasound shear wave elastography (SWE) prospectively and to determine cutoff value for nonfibrotic liver tissue in children with suspected or established liver disease. Methods: In 90 consecutive pediatric patients, standardized 2D-SWE was performed during general anesthesia and free breathing. Liver stiffness was estimated with SWE followed by a percutaneous biopsy from the corresponding area. SWE values were compared with histology with fibrosis scored according to Batts & Ludwig classification (grade 0-4 = F0-F4) and to hepatic biomarkers. Results: Four patients with SWE interquartile range (IQR)/median ≥ 30% kPa were excluded. The remaining 86 children (59% males) had a mean age = 10.2 years (0.1-18). The distribution of individuals with median (min;max) SWE values (kPa) within each fibrosis grade were; F0[n = 10; 5.0(3.4;6.3)], F1[n = 24; 5.0(3.6;8.7)], F2[n = 32; 5.8(3.5;13.4)], F3[n = 12, 7.5(4.0;14.4)], and F4[n = 8; 12.5(6.6;21.0)]. There was a significant difference between fibrosis grades (0.03 > P < 0.002) except between F0 and F1 respectively between F1 and F2. The AuROC differentiating F0-1 from F2-4 was 0.77(95% CI: 0.67-0.87). A cutoff SWE value of ≤4.5 kPa yielded 90% sensitivity and 68% specificity to rule out significant fibrosis (F2-F4). Out of the 18 children (21%) with SWE value ≤4.5 kPa, 12 had grade F0-1 and 6 had F2, although including some confounders for increased SWE measurements as steatosis/hepatitis/cholestasis. Conclusions: 2D-SWE ultrasound can reliably distinguish no/mild (F0/F1) from moderate/severe (F2-F4) fibrosis in children with suspected/established liver disease with good sensitivity and acceptable specificity. Our results show that in pediatric patients, when the indication for biopsy is to rule out significant fibrosis, SWE can be considered an alternative.
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BACKGROUND: The extent and significance in of cardiac remodeling in Fontan patients are unclear and were the subject of this study. METHODS: This retrospective cohort study compared cardiovascular magnetic resonance (CMR) imaging markers of cardiac function, myocardial fibrosis, and hemodynamics in young Fontan patients to controls. RESULTS: Fifty-five Fontan patients and 44 healthy controls were included (median age 14 years (range 7-17 years) vs 13 years (range 4-14 years), p = 0.057). Fontan patients had a higher indexed end-diastolic ventricular volume (EDVI 129 ml/m2 vs 93 ml/m2, p < 0.001), and lower ejection fraction (EF 45% vs 58%, p < 0.001), circumferential (CS - 23.5% vs - 30.8%, p < 0.001), radial (6.4% vs 8.2%, p < 0.001), and longitudinal strain (- 13.3% vs - 24.8%, p < 0.001). Compared to healthy controls, Fontan patients had higher extracellular volume fraction (ECV) (26.3% vs 20.6%, p < 0.001) and native T1 (1041 ms vs 986 ms, p < 0.001). Patients with a dominant right ventricle demonstrated larger ventricles (EDVI 146 ml/m2 vs 120 ml/m2, p = 0.03), lower EF (41% vs 47%, p = 0.008), worse CS (- 20.1% vs - 25.6%, p = 0.003), and a trend towards higher ECV (28.3% versus 24.1%, p = 0.09). Worse EF and CS correlated with longer cumulative bypass (R = - 0.36, p = 0.003 and R = 0.46, p < 0.001), cross-clamp (R = - 0.41, p = 0.001 and R = 0.40, p = 0.003) and circulatory arrest times (R = - 0.42, p < 0.001 and R = 0.27, p = 0.03). T1 correlated with aortopulmonary collateral (APC) flow (R = 0.36, p = 0.009) which, in the linear regression model, was independent of ventricular morphology (p = 0.9) and EDVI (p = 0.2). The composite outcome (cardiac readmission, cardiac reintervention, Fontan failure or any clinically significant arrhythmia) was associated with increased native T1 (1063 ms vs 1026 ms, p = 0.029) and EDVI (146 ml/m2 vs 118 ml/m2, p = 0.013), as well as decreased EF (42% vs 46%, p = 0.045) and worse CS (- 22% vs - 25%, p = 0.029). APC flow (HR 5.5 CI 1.9-16.2, p = 0.002) was independently associated with the composite outcome, independent of ventricular morphology (HR 0.71 CI 0.30-1.69 p = 0.44) and T1 (HR1.006 CI 1.0-1.13, p = 0.07). CONCLUSIONS: Pediatric Fontan patients have ventricular dysfunction, altered myocardial mechanics and increased fibrotic remodeling. Cumulative exposure to cardiopulmonary bypass and increased aortopulmonary collateral flow are associated with myocardial dysfunction and fibrosis. Cardiac dysfunction, fibrosis, and collateral flow are associated with adverse outcomes.
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Ventrículos do Coração , Imagem Cinética por Ressonância Magnética , Adolescente , Criança , Fibrose , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Ventrículos do Coração/cirurgia , Humanos , Valor Preditivo dos Testes , Estudos Retrospectivos , Função Ventricular EsquerdaRESUMO
Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.
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Técnica de Fontan , Cardiopatias Congênitas , Hepatopatias , Radiologia , Adulto , Criança , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Humanos , Fígado/patologia , Cirrose Hepática/patologia , Hepatopatias/patologia , Complicações Pós-Operatórias/patologiaRESUMO
Fontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.
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Carcinoma Hepatocelular , Técnica de Fontan , Cardiopatias Congênitas , Neoplasias Hepáticas , Radiologia , Criança , Consenso , Seguimentos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Humanos , Recém-Nascido , Cirrose Hepática , Neoplasias Hepáticas/diagnóstico por imagem , Inquéritos e QuestionáriosRESUMO
OBJECTIVE: Adults operated for tetralogy of Fallot (TOF) have high risk of ventricular arrhythmias (VA). QRS duration >180 ms is an established risk factor for VA. We aimed to investigate heart function, prevalence of arrhythmias and sex differences in patients with TOF at long-term follow-up. METHODS: We included TOF-operated patients≥18 years from our centre's registry. We reviewed medical records and the most recent echocardiographic exam. VA was recorded on ECGs, 24-hour Holter registrations and from implantable cardioverter defibrillator. RESULTS: We included 148 patients (age 37±10 years). Left ventricular global longitudinal strain (LV GLS, -15.8±3.1% vs -18.8±3.2%, p=0.001) and right ventricular (RV) GLS (-15.8±3.9% vs -19.1±4.1%, p=0.001) were lower in men at all ages compared with women. Higher RV D1 (4.3±0.5 cm vs 4.6±0.6 cm, p=0.01), lower ejection fraction (55%±8% vs 50%±9%, p=0.02), lower RV GLS (-18.1±4.0 ms vs -16.1±4.8 ms, p=0.04) and N-terminal pro-brain natriuretic peptide (NT-proBNP) over reference range (n=27 (23%) vs n=8 (77%), p<0.001) were associated with higher incidence of VA. QRS duration was longer in men (151±30 ms vs 128±25 ms, p<0.001). No patients had QRS duration >180 ms. QRS duration did not differ in those with and without VA (143±32 ms vs 137±28 ms, p=0.06). CONCLUSIONS: Our results confirmed reduced RV function in adults operated for TOF. Male patients had impaired LV and RV function expressed by lower LV and RV GLS values at all ages. Reduced cardiac function and elevated NT-proBNP were associated with higher incidence of VA and may be important in risk assessment.
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Procedimentos Cirúrgicos Cardíacos/métodos , Tetralogia de Fallot/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Ecocardiografia , Eletrocardiografia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Morbidade/tendências , Noruega/epidemiologia , Estudos Retrospectivos , Distribuição por Sexo , Fatores Sexuais , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/cirurgia , Fatores de Tempo , Adulto JovemRESUMO
OBJECTIVES: To assess the impact of pregnancy on cardiac function and fibrosis by cardiovascular magnetic resonance (CMR) in patients with repaired Tetralogy of Fallot (rToF). BACKGROUND: CMR T1 mapping can assess diffuse myocardial fibrosis which is associated to adverse clinical outcomes. Right ventricular (RV) accelerated remodeling is reported in rToF women with experienced pregnancy. METHODS: We included rToF women from the national registry of congenital heart disease to perform CMR, assessing functional data, T1 mapping/ extracellular volume fraction (ECV). The results including clinical data were compared between women with experienced pregnancy vs non-experienced pregnancy and healthy individuals. RESULTS: Fifty rToF women performed CMR, median age 36 (range 21-67) years. Fifteen were nulliparous. T1 mapping was compared to 30 controls, (14 women) median age 42 (24-64) years. In the left ventricle (LV), T1 times and ECV in all rToF women vs female controls were 1248 ± 61 ms/ 25.8 ± 2.9% vs 1255 ± 40 ms/ 26.8 ± 3.1%, p = 0.7 and p = 0.3, respectively. In rToF, RV T1 times was 1385 ± 124 ms and ECV 37.7 ± 5.4%. There was no association to parity or age in rToF LV T1/ ECV, p = 0.9 for both, or RV T1/ECV, p = 0.4 and p = 0.6, respectively. Indexed LV mass was higher in the rToF pregnancy group, 43 ± 10 vs 38 ± 6 g/m2, p = 0.03 while RV ejection fraction was lower, 49 ± 7% vs 53 ± 6%, p = 0.04. CONCLUSION: Women with rTOF showed evidence of increased RV CMR markers suggestive of diffuse fibrosis while LV CMR markers were within normal values. Having experienced pregnancy might affect RV function, however without association to CMR biomarkers.