Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. Nevertheless, correct execution and interpretation require in-depth knowledge of all technical and clinical aspects of CHD, a careful assessment of risks and benefits before each exam, proper imaging protocols to maximize diagnostic information, minimizing harm. This position paper, written by experts from the Working Group of the Italian Society of Pediatric Cardiology and from the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, is intended as a practical guide for applying CCT and CMR in children and adults with CHD, wishing to support Radiologists, Pediatricians, Cardiologists and Cardiac Surgeons in the multimodality diagnostic approach to these patients. The first part provides a review of the most relevant literature in the field, describes each modality's advantage and drawback, making considerations on the main applications, image quality, and safety issues. The second part focuses on clinical indications and appropriateness criteria for CMR and CCT, considering the level of CHD complexity, the clinical and logistic setting and the operator expertise.
MIS-C is a multisystem inflammatory syndrome that is characterized by multi-organ failure and cardiac involvement. The aim of this study was to describe the long-term cardiovascular outcome in a cohort of MIS-C pediatric patients, who were admitted to two Italian Pediatric Referral Centers. Sixty-seven patients (mean age 8.7 ± 4.7 years, male 60%) were included; 65 (97%) of them showed cardiac involvement. All of the patients completed one month of the follow-up, and 47% completed 1 year of it. ECG abnormalities were present in 65% of them, arrhythmias were present in 9% of them during an acute phase and it disappeared at the point of discharge or later. Pericarditis were detected in 66% of them and disappeared after 6 months. Coronaritis was observed in 35% of the children during an acute phase, and there were no more instances at the 1-year point. An LV dysfunction was present in 65% of the patients at the beginning of the study, with them having a full recovery at the point of discharge and thereafter. Elevated values of the NTproBNP and hsTp were initially detected, which progressively decreased and normalized at the points of discharge and FU. The CMR at the point of FU, there was a presence of long-term myocardial scars in 50% of the patients that were tested. No deaths that were caused by MIS-C during the FU were recorded. Cardiac involvement in MIS-C patients is almost the rule, but the patients’ clinical course was satisfactory, and no additional events or sequelae were observed apart from there being long-term myocardial scars in 50% of the patients that underwent CMR.
Congenital bronchopulmonary malformations encompass a wide spectrum of pathologies involving the lungs, trachea and bronchi, pulmonary vessels, and oesophagus. These developmental lesions are often isolated, but the association of two or more anomalies is not infrequent. Contrast-enhanced multidetector computed tomography (MDCT), thanks to multiplanar and 3D reconstructions, allows for detailed studies of these malformations, achieving better accuracy compared with conventional techniques such as chest X-ray, fluoroscopy, ventilation and perfusion scintigraphy and ultrasonography. MDCT is characterised by fast data acquisition and does not require sedation in the majority of cases. The main drawbacks of MDCT are the use of ionising radiation and - in many cases -contrast media. Recently, improved CT scanners and optimised CT protocols have made available to children all the benefits of MDCT, thanks to a significant reduction in radiation dose and an improved risk-benefit ratio. The aim of our paper was to evaluate MDCT in children with bronchopulmonary malformations by reporting our experience (about 2,400 studies in 30 months with a 64-slice MDCT scanner) and comparing it with the available literature.
The diagnostic accuracy of 64-row CTCA for ruling out the presence of significant coronary stenoses in patients undergoing valve surgery is excellent and allows CTCA to be used as a gatekeeper for invasive CCA in these patients. MDCT is a necessary preoperative examination that provides useful information for identifying potential operative complications of surgical procedures.
Introduction Jeune's syndrome, or asphyxiating thoracic dystrophy (ATD), is a rare autosomal recessive disorder characterized by skeletal dysplasia. Ribs are typically short and horizontal resulting—in lethal variant—in severe lung hypoplasia, progressive respiratory failure, and death. Lateral thoracic expansion (LTE) consists in staggered bilateral ribs osteotomy leading to chest expansion and lung development. Studies on LTE in ATD patients report encouraging data, but the rarity of ATD implies the lack of a standardized surgical path. The aim of this report is to present our experience with LTE, the technical modification we adopted, and patients' clinical outcome. Materials and Methods We retrospectively reviewed data of 11 LTE performed in 7 ATD patients with lethal variant. Information regarding pre- and postoperative clinical conditions and surgical details was collected. We adopted a single-stage or a two-stage approach based on patient clinical condition. Computed tomography (CT) scan was performed before and after surgery and lung volume was calculated. Results Five patients are alive, while two died in intensive care unit for other than respiratory cause (sepsis). Most patients experienced clinical improvement in terms of decreased respiratory infections rate, need for ventilation, and improved exercise tolerance. Postoperative CT scan demonstrated a median lung volume increase of 88%. Conclusion Mortality in ADT patients is high. However, LTE is a feasible and safe surgical approach, which could improve clinical conditions and survival rate. Survived patients showed postoperatively less oxygen requirement and improved clinical conditions.
Objectives: The aim of our study is to report a case of a child with subglottic thymus that was suspected during diagnostic work-up for severe airway obstruction, excised surgically and confirmed with final histopathological examination. Moreover, we performed a narrative literature review to outline clinical and diagnostic features of this rare condition and to report suggestions for the management of subglottic masses. Methods: We report the case of a 7-month-old boy who was admitted to our Pediatric Airway Team Unit due to a history of worsening biphasic stridor and recurrent episodes of upper airway obstruction. The successful diagnostic work-up and a narrative literature of analogous cases of subglottic thymus were reported. Results: Ectopic thymus is a very rare condition in which thymic tissue is found outside the normal pathway of its embryonic migration. It usually presents as a cystic or, more rarely, solid mass, showing an indolent course toward spontaneous involution. In some cases, however, it becomes symptomatic exerting compression on surrounding vital structures. Due to its rarity, the initial diagnosis is normally mistaken with inflammatory diseases or malignancies and the definitive diagnosis is only achieved after histological examination of the excised specimen. To our knowledge, only four other cases of subglottic ectopic thymic tissue have been reported in the English literature so far and the diagnosis has never been suspected preoperatively. Conclusion: It is mandatory to consider ectopic thymic tissue in the differential diagnosis in children presenting with airways obstruction in order to prevent unnecessary, extensive, and exploratory surgery.
Extraosseous Ewing sarcoma of primary cardiac origin is an extremely rare variety among pediatric cardiac neoplasms. We report a case of extraosseous Ewing sarcoma of primary cardiac origin in a 9-year-old girl, treated with debulking surgery, adjuvant chemotherapy, and radiotherapy.
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