Recommendations for cardiovascular magnetic resonance and computed tomography in congenital heart disease: a consensus paper from the CMR/CCT working group of the Italian Society of Pediatric Cardiology (SICP) and the Italian College of Cardiac Radiology endorsed by the Italian Society of Medical and Interventional Radiology (SIRM) Part I.

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.

Organizational and welfare mode of breast centers network: a survey of Sicilian radiologists.

The European Society of Breast Cancer Specialists has created quality indicators for breast units to establish the minimum standards of care for patients. In Italy, each region differs, indeed, in terms of health care and services warranted to patients suffering from breast cancer. Since Sicilian Regional Administration today is still disregarding implementation of the provisions contained in the proposal of the Ministry of Health entitled "Guidelines on Organizational and Health Care Methods of Breast Centers Network" in November 2015 the Sicilian Regional Group of the Italian Society of Radiology (SIRM) decided to carry out a survey to see the position of the Sicilian Healthcare System and define the gap to bridge over. Sicilian breast imaging radiologists were asked to fill in a questionnaire concerning the type of job relationship (public or private sector), qualification (Manager, Department Manager, Freelancer) and years of experience on breast imaging. With regard to technological requirements, were answered the questions about the number, type, age and completeness of accessories of the equipment supplied in the Sicilian healthcare facilities. The data showed that over 64% of breast imagers in Sicilian centers work in breast units, whereas only 18% are involved in screening programs. A majority of radiologists (81%) working in the breast health care system is very experienced (more than 10 years of experience in the field). The result provided concerning the medical and technical staff demonstrates an uneven situation, but overall an inadequate value compared with the required guidelines especially in interventional procedures. The aim of this paper is to illustrate the method used and the results obtained. These data have to be shared with policy makers to enhance quality improvement in Sicilian Breast Center Network.

Mitomycin-based hepatic arterial infusion chemotherapy for solitary ampullary cancer liver metastasis: an unusual treatment for an uncommon disease.

Ampullary carcinoma is an uncommon gastrointestinal disease. Its natural history is often characterized by the occurrence of liver metastases. Among patients who undergo pancreatoduodenectomy, those presenting with lymph nodes involvement are more prone to early distant disease relapse. In this report, a patient previously diagnosed with ampullary carcinoma had been treated with curative surgery. After subsequent adjuvant gemcitabine, the patient developed significant myelotoxicity and suffered from a single liver metastasis a few months later. A hepatic intra-arterial mitomycin plus fluorouracil-based chemotherapy was administered in order to avoid any serious systemic toxicity. The treatment was well tolerated and no serious side effects occurred. Extra-hepatic cancer relapse, involving intra-thoracic and abdominal lymph nodes, was observed not long after the initial intra-hepatic almost complete response. In conclusion, the locoregional chemotherapy administration was effective in overcoming any systemic toxicities and showed activity against the liver metastasis but it did not prevent extra-hepatic cancer dissemination.

Magnetic Resonance Imaging in Preterm Infant: A Systematic Review on Clinical Procedure Safety.

BACKGROUND: Currently, there is no evidence that MRI produces harmful effects on premature newborns, as well as short-term and long-term safety issues regarding radiofrequency fields and loud acoustic environment, while the examination that is being performed has not been clearly investigated. MRI of the brain conducted on preterm infants should be part of the diagnostic workup, when necessary. This article is intended to evaluate the short-term safety of MRI performed in preterm infants, when required, by analyzing all vital parameters available before, during, and after the MRI procedures. METHODS: We conducted a systematic review of the literature on electronic medical databases (PubMed and ClinicalTrials.gov) following the Preferred Reported Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We included all preterm infants who underwent MRI whose clinical, hemodynamic, and respiratory parameters were reported. The quality of the included articles was assessed using QUADAS-2 (Quality Assessment of Diagnostic Accuracy Studies) tool. RESULTS: Six studies were included with a total of 311 preterm infants. No severe adverse event, such as death, occurred during MRI procedures. Vital signs remained stable in about two-thirds of all patients. CONCLUSIONS: Given the general clinical safety of MRI, we suggest it as a tool to be used in preterm infants in Neonatal Intensive Care Units, when necessary. We further suggest the development of standard protocols to guide the use of MRI in preterm infants to maximize the clinical safety of the procedure.

Transvenous retrieval of foreign objects lost during cardiac device implantation or revision: a 10-year experience.

BACKGROUND: Many techniques for the endovascular retrieval of lost or misplaced foreign objects have been developed, and the removal of almost every foreign object has become possible. In this paper, we report our experience in retrieving foreign objects lost during cardiac device implantations or previous extraction procedures. METHODS: This study was a retrospective analysis of the case records of all patients referred to our institution for transvenous retrieval of intravascular foreign objects. RESULTS: Over 10 years, 45 consecutive patients underwent procedures for the retrieval of intravascular foreign objects. These objects were: 25 distal portions of introducer sheaths, 18 pacing lead fragments, one guidewire, and one anchoring sleeve. The majority of fragments were located in the right ventricle and subclavian and caval veins. Some had migrated to the pulmonary artery or more distally. The median dwell time of the fragments was 3 months. Retrieval was most frequently achieved through the femoral veins, and was successful in 42 (93%) procedures. No procedure-related complications occurred in this series. CONCLUSIONS: In the present single-center experience, the endovascular approach to retrieving intravascular objects lost during cardiac device implantation or previous extraction procedures seemed effective with currently available tools and was associated with no complications.

MR imaging of primary benign cardiac tumors in the pediatric population.

Primary cardiac tumors are rare in all ages, especially in children, with a reported prevalence range of 0.0017-0.28% in autopsy series. Due to their rarity, the diagnostic and therapeutic pathways reserved to them are usually described by single case reports, leading to the point where a common diagnostic protocol is imperative to obtain a differential diagnosis. The first diagnostic approach is done with transthoracic echocardiogram (TTE), due to its wide availability, low cost, absence of ionizing radiations and non-invasiveness. Several tumors are discovered incidentally and, in many cases, TTE is helpful to determine location, size and anatomical features, playing a key role in the differential diagnosis. In the last few years, cardiac magnetic resonance imaging (CMR) has had an increased role in the diagnostic pathway of pediatric cardiac masses, due to its high accuracy in characterizing mass tissue properties (especially for soft tissue), and in detecting tumor size, extent, pericardial/pleural effusion, leading to the correct diagnosis, treatment and follow-up. Therefore, nowadays, several consensus statements consider CMR as a leading imaging technique, thanks to its non-invasive tissue characterization, without the use of ionizing radiation, in an unrestricted field of view. As suggested by the most recent literature, the pediatric protocol is not so different from the adult one, adapted to the size and cardiac frequency of the patient, sometimes requiring special conditions such as free-breathing sequences and/or sedation or general anesthesia in non-cooperating patients.

Imaging in a Rare Case of Neonatal Arterial Tortuosity Syndrome.

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive disorder that affects the connective tissue. The incidence of ATS is not well known and to date only 106 patients have been described in the literature. ATS affects medium and large size arteries, leading to widespread elongation and intensification of the average vessel tortuousness, responsible of several loops and kinks. Like other connective tissue disorders, ATS can present with joint laxity, hernias, pectus excavatum, scoliosis or other musculoskeletal abnormalities, and ocular defects. Due to the extreme variability of clinical symptoms and the fact that ATS has no curative management, prompt diagnosis is of tremendous importance to prevent disease-associated complications. In this situation, imaging techniques have a central role. In this study, we describe a rare case of a male newborn with tortuosity and lengthening of the main arterial and venous medium and large caliber branches with associated aortic coarctation who passed away prematurely. The finding of aortic coarctation in a newborn with ATS has rarely been described in the literature.

Role of advanced cardiovascular imaging in chemotherapy-induced cardiotoxicity.

The development of cardiotoxicity induced by cancer treatments has emerged as a significant clinical problem, both in the short run, as it may influence drug administration in chemotherapeutic protocols, and in the long run, because it may determine adverse cardiovascular outcomes in survivors of various malignant diseases. Therefore, early detection of anticancer drug-related cardiotoxicity is an important clinical target to improve prevention of adverse effects and patient care. Today, echocardiography is the first-line cardiac imaging techniques used for identifying cardiotoxicity. Cardiac dysfunction, clinical and subclinical, is generally diagnosed by the reduction of left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS). However, myocardial injury detected by echocardiography is preceded by other alterations, such as myocardial perfusion and mitochondrial and metabolic dysfunction, that can only be recognized by second-level imaging techniques, like cardiac magnetic resonance (CMR) and nuclear imaging, which, using targeted radiotracers, may help to provide information on the specific mechanisms of cardiotoxicity. In this review, we focus on the current and emerging role of CMR, as a critical diagnostic tool of cardiotoxicity in the very early phase, due to its availability and because it allows the contemporary detection of functional alterations, tissue alterations (mainly performed using T1, T2 mapping with the evaluation of extracellular volume-ECV) and perfusional alteration (evaluated with rest-stress perfusion) and, in the next future, even metabolic changes. Moreover, in the subsequent future, the use of Artificial Intelligence and big data on imaging parameters (CT, CMR) and oncoming molecular imaging datasets, including differences for gender and countries, may help predict cardiovascular toxicity at its earliest stages, avoiding its progression, with precise tailoring of patients' diagnostic and therapeutic pathways.

Anomalous venoatrial connections - CT and MRI assessment.

Abnormal venous atrial (VA) connections present a congenital heart disease (CHD) challenge for pediatric cardiologists. Fully anatomical evaluation is very difficult in prenatal and perinatal follow-up, but it has a profound impact on surgical correction and outcome. The echocardiogram is first-line imaging and represents the gold standard tool for simple abnormal VA connection. CT and MRI are mandatory for more complex heart disease and "nightmare cases". 3D post-processing of volumetric CT and MRI acquisition helps to clarify anatomical relationships and allows for the creation of 3D printing models that can become crucial in customizing surgical strategy. Our article describes a ten-year (2013-2022) tertiary referral CHD center of abnormal AV connections investigated with CT and MRI, illustrating most of these complex diseases with the help of volume rendering (VR) or multiplanar reconstructions (MPR). The nightmarish cases will also be addressed due to the complex cardiovascular arrangement that requires a challenging surgical solution for correction along with the post-surgical complications.

Low molecular weight heparin administration in cancer patients with hypercoagulability-related complications and carrying brain metastases: a case series study.

BACKGROUND: Venous thromboembolism (VTE) and brain metastases (MTS) are significant clinical problems in the cancer patient population. Brain MTS and deep vein thrombosis are life-threatening conditions because of the risk of fatal endocranic hypertension and pulmonary embolism. Low molecular weight heparin (LMWH) is a major treatment for cancer patients suffering from VTE with regard to the management of the acute phase and subsequent secondary prophylaxis. Treatment with anticoagulants is feared because of the risk of triggering a massive intracranial hemorrhage. METHODS: The medical records of patients with hypercoagulability-related complications and carrying brain MTS treated with LMWH, in a 10-year period, were scrutinized. The authors aimed to focus on the occurrence of intracranial hemorrhage in anticoagulated patients; furthermore, data were collected with regard to the characteristics of the administered LMWHs along with the duration and dosing of the anticoagulative treatment. RESULTS: A total of 38 patients (pts) carrying an intracranial metastatic tumor were administered LMWHs: calcium nadroparin (32 pts); enoxaparin (2 pts); reviparin (2 pts); parnaparin (2 pts). Reason for LMWH therapy: deep vein thrombosis and/or pulmonary embolism (15 pts); superficial thrombophlebitis (15 pts); intracardiac thrombus (1 pt); mild DIC (5 pts); acute DIC (1 pt); Raynaud phenomenon (1 pt); atrial fibrillation (1 pt). Median duration of LMWH therapy: 13 weeks (range 1-52). None of the patients developed clinical and/or radiographic findings imputable to intracranial hemorrhage. CONCLUSION: There is no standard medical approach for the management of patients who require anticoagulant treatment and are suffering from brain MTS. These patients as necessary, might be anticoagulated with LMWH and its dose reduction is to be considered.

[The future of the ultrasonography techniques in nephrology]. / Il futuro delle tecniche ultrasonografiche in nefrologia.

Thanks to the wide geographical diffusion, low cost and lack of ionizing radiation, ultrasound is now the most widely used imaging technique in clinical practice, second only to chest radiography. Recent technological innovations and introduction of ultrasound contrast agents, further expanded the fields of application of ultrasound, guarantying for the future to this technique an important role in imaging of the urinary tract. The nephrologist must be able to exploit the potential offered by technological innovations in ultrasound imaging for the study of the kidney. The proper management of equipment, in fact, allows to obtain ultrasound images in gray scale of the highest quality, to optimize the diagnostic accuracy of Doppler techniques and take full advantage of the tools offered by means of ultrasound contrast agents.

Diagnosis and Management of Button Battery Ingestion Complicated by Tracheo-Esophageal and Aorto-Esophageal Fistulas.

Button battery ingestion (BBI) is common in children and its prevalence has increased in the last decades. BBI can be responsible for very severe and potentially fatal complications if not promptly detected. We describe the successful management of two cases of BBI that occurred in two previously healthy infants. Both patients presented with vague symptoms and no witness of foreign body ingestion. The prolonged time of exposure to the corrosive effects of disk batteries was responsible for the development of tracheo-esophageal fistula (TEF) and aorto-esophageal fistula (AEF). We demonstrate how prompt diagnosis and management are crucial for the infants' survival.

A Rare Variant of Hypogenetic Lung Syndrome Mimicking Scimitar Vein.

Hypogenetic lung syndrome, also known as scimitar syndrome, is a rare and well-known congenital condition that includes hypoplastic right pulmonary artery and lung, right displacement of the heart, anomalous systemic arterial supply to the lung, and a characteristically curved anomalous right pulmonary vein draining into the inferior vena cava. In exceptional cases, the anomalous pulmonary vein may drain into left atrium. We here report a case of a 17-year-old girl with a rare variant of hypogenetic lung syndrome diagnosed by means of multimodality imaging and treated with percutaneous occlusion of the aortopulmonary collateral.

Hepatic intra-arterial interferon alpha 2b-based immunotherapy combined with 5-fluorouracil (5-FU)-based systemic chemotherapy for patients with hepatocellular carcinoma (HCC) not responsive and/or not eligible for conventional treatments: a pilot study.

BACKGROUND: Surgery (partial hepatic resection or orthotopic liver transplantation) remains the mainstay for treatment of hepatocellular carcinoma (HCC). Unfortunately, most patients have HCC that cannot be removed either as a result of its size, multiple tumors, location, proximity to major vessels or ducts within the liver, and comorbidity, such as a not well-compensated cirrhosis. For patients who cannot be treated surgically, systemic chemotherapy is frequently limited by unacceptable toxicity, poor response and low survival rates, so that locoregional approaches may be considered as alternatives. PATIENTS AND METHODS: Nine patients with HCC, not eligible for conventional treatments, were treated with interferon alpha 2b-based locoregional, hepatic intra-arterial, immunotherapy and concomitant 5-fluorouracil (5-FU)-based systemic chemotherapy. Interferon was administered at a starting dose of 2,000,000 IU, which could be escalated to 9,000,000 IU, adding 1,000,000 IU weekly, depending on toxicity. 5-Fluorouracil was infused continuously over 28 days, administered as an endovenous protracted infusion weekly at a dose of 250 mg/m2/day for 4 weeks followed by a 2-week break. Eight out of nine patients were evaluable for response and toxicity. The median patient age was 68 years (range 51-77 years). All patients were suffering from cirrhosis. RESULTS: A total of 29 cycles of treatment were administered with a median of 3.6 per patient (range 1-11 per patients). A partial response was observed in 3 out of 8 patients; 1 had stable and 4 progressive disease. The main toxicities were: grade 3 hepatic toxicity (1 patient), grade 3 flu-like syndrome (1 patient) and grade 3 abdominal pain (1 patient). Moreover, one patient developed fatal ischemic stroke and another a fatal central venous catheter infection. CONCLUSION: The preliminary data, show that an interferon-based hepatic intra-arterial immunotherapy combined with low doses of 5-fluorouracil (5-FU)-based systemic chemotherapy, can represent a tolerable combination to apply in the palliative treatment of patients with hepatocellular carcinoma.

Hamartoma of the spleen (splenoma) with calcifications in a child with beta-thalassemia: A case report.

Splenic hamartoma (or splenoma) is a rare, benign, vascular tumor, often incidentally found at imaging, surgery or autopsy. Albeit usually asymptomatic and rare in children, when it occurs in the pediatric population it is more commonly symptomatic. We report a case of a 15-year-old girl with iron-deficiency anemia and beta-thalassemia, who had a large (10 × 8 × 7 cm) splenic lesion with calcifications, incidentally found during follow-up for splenomegaly and histologically characterized as hamartoma with calcified areas. The association of hamartoma and hematological disorders is a very unusual condition in children.