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AIMS: The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up (FU) in ECC. METHODS AND RESULTS: All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum FU 5 years). Of 353 consecutive patients, 303 [57.8% males, aging 8-50 (median 20) years at last FU] were considered and divided into two groups depending on left (194 in Group 1) or right (109 in Group 2) ventricular morphology. Eighty-five (28%) experienced ≥1 arrhythmic complications, with early and late arrhythmias in 17 (5.6%) and 73 (24.1%) patients, respectively. Notably, late bradyarrhythmias occurred after 6 years in 21 (11%) patients in Group 1, and in 15 (13.8%) in Group 2 [P = 0.48]. Late tachyarrhythmias occurred in 55 (18.2%) patients after 12 years: 33 (17%) in Group 1 and 22 (20.2%) patients in Group 2 [P = 0.5]. Ventricular tachycardias (VT) were documented after 12.5 years in 14 (7.2%) patients of Group 1 and 15 (13.8%) of Group 2 [P = 0.06] with a higher incidence in Group 2 during the FU [P = 0.005]. CONCLUSION: Extracardiac conduit is related to a significant arrhythmic risk in the long-term FU, higher than previously reported. Bradyarrhythmias occur earlier but are less frequent than tachyarrhythmias. Interestingly, patients with systemic right ventricle have a significantly higher incidence of VT, especially in a very long FU.
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Arritmias Cardíacas , Técnica de Fontan , Ventrículos do Coração , Humanos , Masculino , Feminino , Técnica de Fontan/efeitos adversos , Incidência , Criança , Adolescente , Adulto , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/diagnóstico , Pessoa de Meia-Idade , Adulto Jovem , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/epidemiologia , Estudos Retrospectivos , Fatores de Tempo , Coração Univentricular/cirurgia , Coração Univentricular/epidemiologia , Coração Univentricular/fisiopatologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Fatores de RiscoRESUMO
BACKGROUND: Lymphopenia, particularly when restricted to the T-cell compartment, has been described as one of the major clinical hallmarks in patients with coronavirus disease 2019 (COVID-19) and proposed as an indicator of disease severity. Although several mechanisms fostering COVID-19-related lymphopenia have been described, including cell apoptosis and tissue homing, the underlying causes of the decline in T-cell count and function are still not completely understood. OBJECTIVE: Given that viral infections can directly target thymic microenvironment and impair the process of T-cell generation, we sought to investigate the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) on thymic function. METHODS: We performed molecular quantification of T-cell receptor excision circles and κ-deleting recombination excision circles to assess, respectively, T- and B-cell neogenesis in SARS-CoV-2-infected patients. We developed a system for in vitro culture of primary human thymic epithelial cells (TECs) to mechanistically investigate the impact of SARS-CoV-2 on TEC function. RESULTS: We showed that patients with COVID-19 had reduced thymic function that was inversely associated with the severity of the disease. We found that angiotensin-converting enzyme 2, through which SARS-CoV-2 enters the host cells, was expressed by thymic epithelium, and in particular by medullary TECs. We also demonstrated that SARS-CoV-2 can target TECs and downregulate critical genes and pathways associated with epithelial cell adhesion and survival. CONCLUSIONS: Our data demonstrate that the human thymus is a target of SARS-CoV-2 and thymic function is altered following infection. These findings expand our current knowledge of the effects of SARS-CoV-2 infection on T-cell homeostasis and suggest that monitoring thymic activity may be a useful marker to predict disease severity and progression.
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COVID-19 , Linfopenia , Humanos , COVID-19/metabolismo , SARS-CoV-2 , Timo , Linfopenia/genética , Gravidade do PacienteRESUMO
Adult congenital heart disease (ACHD) patients represent a growing population with increasing use of acute emergency department (ED) care. Providing comprehensive ED care necessitates an understanding of the most common clinical scenarios to improve morbidity and mortality in this population. The aim of this position document is to provide a consensus regarding the management of the most common clinical scenarios of ACHD patients presenting to the ED.
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Medicina de Emergência , Cardiopatias Congênitas , Cirurgia Torácica , Adulto , Consenso , Serviço Hospitalar de Emergência , Cardiopatias Congênitas/cirurgia , HumanosRESUMO
BACKGROUND: Tracheal trifurcation is an uncommon and often unknown type of anomalous tracheobronchial arborization, characterized by three main bronchi originating at the level of the carina. Diagnosis is important due to its clinical implications. OBJECTIVE: To highlight the anatomical, clinical and diagnostic aspects of tracheal trifurcation by reporting our experience and reviewing the literature. MATERIALS AND METHODS: We retrospectively evaluated pediatric patients referred to our institution from January 2018 to May 2020 with a diagnosis of tracheal trifurcation. All patients underwent chest radiographs, computed tomography (CT) (with/without dynamic airway scanning) and bronchoscopy. Clinical and anatomical data were collected. RESULTS: Three patients with tracheal trifurcation were identified (1 female, median age: 7.6±0.4 months). All had associated anomalies: two had tetralogy of Fallot, one with esophageal atresia/tracheoesophageal fistula and one with an atrioventricular septal defect, and the other had skeletal ciliopathy. Chest radiographs were not diagnostic for tracheal trifurcation. Bronchoscopy could not fully evaluate the trachea and main bronchi in two cases. CT detected tracheal trifurcation in all of the patients and also demonstrated other lung parenchymal and congenital anomalies. Two of the three main bronchi were directed to the right lung in all cases. Ostial stenosis of either the central (two patients) or right upper bronchus (one patient) was present. No signs of tracheobronchomalacia were found. CONCLUSION: Tracheal trifurcation is rare and often associated with additional disorders, such as other tracheobronchial anomalies, cardiovascular defects or esophageal malformations, responsible for clinical manifestations and earlier detection. Bronchoscopy is often used for diagnosis, but is invasive and may be incomplete or inconclusive, while CT allows for a noninvasive and correct diagnosis, while also highlighting additional findings in the thorax.
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Traqueia , Fístula Traqueoesofágica , Brônquios , Broncoscopia , Criança , Feminino , Humanos , Lactente , Estudos Retrospectivos , Traqueia/diagnóstico por imagemRESUMO
We present a case of palliative arterial switch with aortic arch reconstruction performed as the first stage of anatomical correction in a patient with congenitally corrected transposition of great arteries (ccTGA), ventricular septal defect (VSD), duct-dependent aortic coartation, and hypoplasia of the ascending aorta and subaortic tract.
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Anormalidades Múltiplas/cirurgia , Aorta Torácica/cirurgia , Aorta/anormalidades , Coartação Aórtica/cirurgia , Procedimentos Cirúrgicos Cardiovasculares/métodos , Comunicação Interventricular/cirurgia , Cuidados Paliativos , Transposição dos Grandes Vasos/cirurgia , Coartação Aórtica/complicações , Comunicação Interventricular/complicações , Humanos , Recém-Nascido , Masculino , Transposição dos Grandes Vasos/complicaçõesRESUMO
The CardioMEMS HF System (Abbott, Abbott Park, IL) is the first FDA- and CE-Mark-approved device for monitoring patients with heart failure, significantly reducing hospitalizations and improving the quality of life for NYHA class III non-congenital adult patients. This device, implanted percutaneously, allows the direct monitoring of pulmonary arterial pressure with the wireless transfer of pressure data to the clinician, who can adjust the therapy remotely. Limited experience exists regarding its use in patients with congenital heart disease (CHD). CardioMEMS device implantation is feasible and safe in selected adults and children with CHD. The potential of the device to reduce heart failure hospitalizations in this population is enormous, but further multi-center studies are needed to demonstrate its efficacy.
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BACKGROUND: Cardiac Magnetic Resonance (CMR) parametric mapping is underexplored in cardiac tumors. OBJECTIVES: To evaluate the contribution of mapping sequences on the characterization of pediatric tumors. METHODS: All pediatric patients referred for cardiac tumors at Bambino Gesù Children's Hospital from June 2017 to November 2023, who underwent CMR with mapping sequences, were included. The diagnosis of tumor type was performed according to signal characteristics on different sequences. Mass parametric mapping for each subtype and interobserver variability was assessed. RESULTS: Sixteen patients were enrolled. The mean age at CMR was 7 ± 5 years. "Traditional" mass-type assessment diagnosed hemangioma (Group A) in 3 patients (19%), fibroma (Group B) in 4 patients (25%), rhabdomyoma (Group C) in 6 patients (37%), and lipoma (Group D) in 3 patients (19%). The ANOVA analysis revealed significant differences in mass native T1 and mass extracellular volume (ECV) values among the four subgroups (p<0.001 for both comparisons). The mean native T1 and ECV values were respectively 1465 ± 158 msec and 54 ± 4% for Group A, 860 ± 118 msec and 93 ± 4% for Group B, 1007 ± 57 msec and 23 ± 5% for Group C, and 215 ± 13 msec and 0 ± 0% for Group D. CONCLUSIONS: Mass mapping analysis is feasible and reproducible in children. ECV values provide the most accurate differentiation. Mass ECV consistently resembles normal myocardium in rhabdomyoma, is extremely high (approaching 100%) in fibroma, equals to zero in lipoma, and matches blood pool ECV (1-Hct) in hemangioma.
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[This corrects the article DOI: 10.3389/fped.2023.1215928.].
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Marfan syndrome (MIM: # 154700; MFS) is an autosomal dominant disease representing the most common form of heritable connective tissue disorder. The condition presents variable multiorgan expression, typically involving a triad of cardiovascular, eye, and skeletal manifestations. Other multisystemic features are often underdiagnosed. Moreover, the disease is characterized by age related penetrance. Diagnosis and management of MFS in the adult population are well-described in literature. Few studies are focused on MFS in the pediatric population, making the clinical approach (cardiac and multiorgan) to these cases challenging both in terms of diagnosis and serial follow-up. In this review, we provide an overview of MFS manifestations in children, with extensive revision of major organ involvement (cardiovascular ocular and skeletal). We attempt to shed light on minor aspects of MFS that can have a significant progressive impact on the health of affected children. MFS is an example of a syndrome where an early personalized approach to address a dynamic, genetically determined condition can make a difference in outcome. Applying an early multidisciplinary clinical approach to MFS cases can prevent acute and chronic complications, offer tailored management, and improve the quality of life of patients.
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Procedimentos Cirúrgicos Cardíacos/métodos , Doenças Fetais/diagnóstico por imagem , Doenças Fetais/cirurgia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Teratoma/diagnóstico por imagem , Teratoma/cirurgia , Tamponamento Cardíaco/etiologia , Ecocardiografia , Emergências , Feminino , Doenças Fetais/patologia , Idade Gestacional , Neoplasias Cardíacas/embriologia , Neoplasias Cardíacas/patologia , Humanos , Recém-Nascido , Pericárdio/diagnóstico por imagem , Pericárdio/patologia , Pericárdio/cirurgia , Derrame Pleural/diagnóstico por imagem , Derrame Pleural/embriologia , Derrame Pleural/cirurgia , Radiografia Torácica , Teratoma/embriologia , Teratoma/patologia , Resultado do TratamentoRESUMO
The ideal aortic valve substitute for young and middle-aged adults with aortic valve disease is still debated. The Ross procedure (pulmonary autograft replacement) is the only operation that allows replacement of the diseased aortic valve with a living substitute. During the years, the use of this procedure has declined significantly due to concerns over increased intraoperative risk and especially potential long-term failure of the operation. However, several recent publications have shown that, after appropriate learning curve and in specialized centers, the Ross procedure can be performed safely in appropriately selected patients. Moreover, comparative studies suggest that the Ross procedure is associated with better long-term outcomes compared to conventional aortic valve replacement in young and middle-aged adults.
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Insuficiência da Valva Aórtica , Próteses Valvulares Cardíacas , Valva Pulmonar , Adulto , Pessoa de Meia-Idade , Humanos , Autoenxertos , Resultado do Tratamento , Transplante Autólogo , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Valva Pulmonar/cirurgia , Reoperação , Estudos Retrospectivos , SeguimentosRESUMO
Although not completely devoid of risk, pregnancy can be managed in virtually all patients affected by even the most complex forms of congenital heart disease. It is not however advisable in patients with any form of pulmonary arterial hypertension. Pregnancy is even manageable in patients with univentricular heart converted to Fontan circulation. A personalised risk stratification should be performed, and patients affected by advanced NYHA functional class appropriately warned of the potential risks. In this setting, metabolomics might represent a novel tool for use in conducting personalised risk stratification. All pregnancies, particularly those at higher risk, should be managed in a tertiary care centre capable of providing the necessary assistance to both the mother and infant. With a few rare exceptions, vaginal delivery is to be preferred over caesarean section due to the lower degree of maternal and foetal complications. The desire for motherhood, at times extreme in women with congenital heart disease, may often be accomplished, thus providing a ray of hope in the lives of these patients.
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Constrictive pericarditis is a chronic inflammatory process that can lead to heart failure if not diagnosed and treated correctly. Although Epstein-Barr virus (EBV)-related pericarditis is a very rare condition, it should still be considered for a differential diagnosis. We report the case of an 18-year-old male, who was surgically treated for constrictive pericarditis, in which in situ hybridization to Epstein-Barr virus-encoded RNA (EBER) probe of the excised pericardium led to the subsequent etiological diagnosis of chronic pericarditis caused by EBV.
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BACKGROUND: Adults with congenital heart disease (ACHD) are a growing population needing ongoing care. The aim of this study was to investigate if a dedicated ACHD team impacted the timing and indication of invasive cardiology procedures in these patients at our hospital. METHODS: Our retrospective single-center study enrolled adult patients with moderate or complex congenital heart disease and with at least one cardiac catheterization between January 2010 and December 2021. According to the period, procedures were labeled as group A (2010 to 2015) or group B (2016 to 2021) and further divided into diagnostic (DCC) and interventional cardiac catheterizations (ICC). RESULTS: 594 patients were eligible for the study. Both DCC (p < 0.05) and ICC increased between groups A and B (p < 0.05). In group B: Fontan patients accounted for the majority of DCC (p < 0.001), while DCC decreased in arterial switch repair (p < 0.001). In Fontan patients, conduit stenting was prevalent (p < 0.001), while fenestration closures dropped (p < 0.01). In patients with tetralogy of Fallot and native outflow tract, percutaneous pulmonary valve implantations (PPVI) increased, with a concurrent reduction in pulmonary valve replacements (p < 0.001 vs. surgical series). In right ventricular conduits, ICC increased (p < 0.01), mainly due to PPVI. Among Mustard/Senning patients, baffle stenting increased from Group A to Group B (p < 0.001). In patients with pulmonary atresia and biventricular repair, ICC often increased for pulmonary artery stenting. CONCLUSIONS: A dedicated working group could improve ACHD patients' indications for interventional procedures, leading to tailored treatment, better risk stratification and optimizing time until heart transplantation.
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Aortic reconstruction at the time of the comprehensive stage II (CSII) procedure can be complicated by compression within the aortopulmonary space resulting in airway or pulmonary artery narrowing. We describe our experience with 2 patients with hypoplastic left heart syndrome and pulmonary artery stenosis after the CSII procedure. Both patients underwent an aortic extension with a Hemashield interposition graft to open up the aortopulmonary space. The patients were discharged from the hospital. In all cases the aortopulmonary space was enlarged, and the pulmonary arteries and airway were free from compression. Aortic extension is an option to be considered in children with pulmonary artery compression who previously had a CSII procedure.
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Síndrome do Coração Esquerdo Hipoplásico , Aorta/diagnóstico por imagem , Aorta/cirurgia , Criança , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Cuidados Paliativos/métodos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos VascularesRESUMO
Objectives: To evaluate the incidence of pericardial effusion (PE) after surgical atrial septal defect (ASD) closure and to investigate the presence of predictive risk factors for its development. Methods: We collected data from 203 patients followed at Bambino Gesù Children's Hospital of Rome who underwent cardiac surgery for ASD repair between January 2015 and September 2019. Results: A total of 200/203 patients with different types of ASD were included. Patients were divided into two groups: Group 1) 38 (19%) who developed PE and Group 2) 162 (81%) without PE. No differences were noted between the two groups with regard to gender or age at the surgery. Fever in the 48 h after surgery was significantly more frequent in group 1 than in group 2 (23.7 vs. 2.5%; p < 0.0001). ECG at discharge showed significant ST-segment elevation in children who developed PE, 24.3 vs. 2.0% in those who did not (p < 0.0001). Group 1 patients were divided into two subgroups on the basis of the severity of PE, namely, 31 (81.6%) with mild and 7 (18.4%) with moderate/severe PE. Patients with moderate/severe PE had a significantly higher BMI value (median 19.1 Kg/m2) (range 15.9-23.4, p = 0.004). Conclusion: The presence of fever and ST-segment elevation after surgery predicts subsequent development of PE suggesting a closer follow-up for these categories of patients. A higher BMI appears to be associated with a higher risk of moderate/severe PE.
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The Fontan operation represents the final stage of a series of palliative surgical procedures for children born with complex congenital heart disease, where a "usual" biventricular physiology cannot be restored. The palliation results in the direct connection of the systemic venous returns to the pulmonary arterial circulation without an interposed ventricle. In this unique physiology, systemic venous hypertension and intrathoracic pressures changes due to respiratory mechanics play the main role for propelling blood through the pulmonary vasculature. Although the Fontan operation has dramatically improved survival in patients with a single ventricle congenital heart disease, significant morbidity is still a concern. Patients with Fontan physiology are in fact suffering from a multitude of complications mainly due to the increased systemic venous pressure. Consequently, these patients need close clinical and imaging monitoring, where cardiac exams play a key role. In this article, we review the main cardiac imaging modalities available, summarizing their main strengths and limitations in this peculiar setting. The main purpose is to provide a practical approach for all clinicians involved in the care of these patients, even for those less experienced in cardiac imaging.
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Myxomas are slowly growing benign neoplasms which are rare in children. Up to 80% can be located in the left atrium and generate symptoms such as embolism, cardiac failure, fever and weight loss. Rarely, myxomas can be detected in the right ventricle outflow tract, causing arrhythmias, pulmonary emboli and sudden death. We report the case of a 13-year-old healthy child brought to the Emergency Department (ED) of the Children's Hospital Bambino Gesù, Rome, for recent dyspnea, chest pain on exertion and new onset cardiac murmur. Patient underwent medical examination and echocardiogram with the finding of a rounded and lobulated voluminous mass in the right ventricle outflow tract (RVOT) which caused severe obstruction. The contrast computed tomography (CT) scan confirmed the presence of a heterogeneously enhancing soft-tissue mass occupying the RVOT with no evidence of pulmonary embolization. The mass was surgically excised, and the pathologic examination confirmed our suspicion of myxoma. Our experience suggests that myxoma can have mild clinical symptoms, the presentation may be non-specific, and diagnosis can be a challenge Careful examination and a diagnostic imaging workup, primarily with the transthoracic echocardiogram, are needful to make a rapid differential diagnosis and to better manage surgical treatment and follow-up.
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Neoplasias Cardíacas , Mixoma , Adolescente , Criança , Dispneia/etiologia , Sopros Cardíacos/etiologia , Sopros Cardíacos/patologia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/cirurgia , Ventrículos do Coração , Humanos , Mixoma/complicações , Mixoma/diagnóstico , Mixoma/cirurgiaRESUMO
Patients with repaired Tetralogy of Fallot (rToF) typically report having preserved subjective exercise tolerance. Chronic pulmonary regurgitation (PR) with varying degrees of right ventricular (RV) dilation as assessed by cardiac magnetic resonance imaging (MRI) is prevalent in rToF and may contribute to clinical compromise. Cardiopulmonary exercise testing (CPET) provides an objective assessment of functional capacity, and the International Physical Activity Questionnaire (IPAQ) can provide additional data on physical activity (PA) achieved. Our aim was to assess the association between CPET values, IPAQ measures, and MRI parameters. All rToF patients who had both an MRI and CPET performed within one year between March 2019 and June 2021 were selected. Clinical data were extracted from electronic records (including demographic, surgical history, New York Heart Association (NYHA) functional class, QRS duration, arrhythmia, MRI parameters, and CPET data). PA level, based on the IPAQ, was assessed at the time of CPET. Eighty-four patients (22.8 ± 8.4 years) showed a reduction in exercise capacity (median peak VO2 30 mL/kg/min (range 25-33); median percent predicted peak VO2 68% (range 61-78)). Peak VO2, correlated with biventricular stroke volumes (RVSV: ß = 6.11 (95%CI, 2.38 to 9.85), p = 0.002; LVSV: ß = 15.69 (95% CI 10.16 to 21.21), p < 0.0001) and LVEDVi (ß = 8.74 (95%CI, 0.66 to 16.83), p = 0.04) on multivariate analysis adjusted for age, gender, and PA level. Other parameters which correlated with stroke volumes included oxygen uptake efficiency slope (OUES) (RVSV: ß = 6.88 (95%CI, 1.93 to 11.84), p = 0.008; LVSV: ß = 17.86 (95% CI 10.31 to 25.42), p < 0.0001) and peak O2 pulse (RVSV: ß = 0.03 (95%CI, 0.01 to 0.05), p = 0.007; LVSV: ß = 0.08 (95% CI 0.05 to 0.11), p < 0.0001). On multivariate analysis adjusted for age and gender, PA level correlated significantly with peak VO2/kg (ß = 0.02, 95% CI 0.003 to 0.04; p = 0.019). We observed a reduction in objective exercise tolerance in rToF patients. Biventricular stroke volumes and LVEDVi were associated with peak VO2 irrespective of RV size. OUES and peak O2 pulse were also associated with biventricular stroke volumes. While PA level was associated with peak VO2, the incremental value of this parameter should be the focus of future studies.
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Background: The aim of this study is to identify echocardiographic predictors of transient left ventricle dysfunction after pulmonary valve balloon dilatation (PVBD), in neonates with pulmonary valve stenosis (PVS) and atresia with intact septum (PAIVS) at birth. Methods: The study includes patients admitted at the Bambino Gesù Children Hospital from January 2012 to January 2017. Clinical, echocardiographic and cardiac catheterization data before and after PVBD were retrospectively analyzed. Results: Twenty-nine infants were included in the study (21 male and eight female). The median age was 5.8 ± 7.1 days. Eight patients developed transient LV dysfunction (three PAIVS and five PVS) and comparing data before and after the procedure, there was no difference in right ventricle geometrical and functional parameters except for evidence of at least moderate pulmonary valve regurgitation after PVBD. Conclusion: Moderate to severe degree pulmonary valve regurgitation was significant associated to LV dysfunction (p < 0.05) in PVS and PAIVS patients.