Impact of hard lockdown on interventional cardiology procedures in congenital heart disease: a survey on behalf of the Italian Society of Congenital Heart Disease.

The Coronavirus disease 2019 (COVID-19) pandemic has thoroughly and deeply affected the provision of healthcare services worldwide. In order to limit the in-hospital infections and to redistribute the healthcare professionals, cardiac percutaneous intervention in Pediatric and Adult Congenital Heart Disease (ACHD) patients were limited to urgent or emergency ones. The aim of this article is to describe the impact of the COVID-19 pandemic on Pediatric and ACHD cath laboratory activity during the so-called 'hard lockdown' in Italy. Eleven out of 12 Italian institutions with a dedicated Invasive Cardiology Unit in Congenital Heart Disease actively participated in the survey. The interventional cardiology activity was reduced by more than 50% in 6 out of 11 centers. Adolescent and ACHD patients suffered the highest rate of reduction. There was an evident discrepancy in the management of the hard lockdown, irrespective of the number of COVID-19 positive cases registered, with a higher reduction in Southern Italy compared with the most affected regions (Lombardy, Piedmont, Veneto and Emilia Romagna). Although the pandemic was brilliantly addressed in most cases, we recognize the necessity for planning new, and hopefully homogeneous, strategies in order to be prepared for an upcoming new outbreak.

Catheter closure of a patent vertical vein after repair of an unusual total anomalous variant of pulmonary venous drainage.

Total anomalous pulmonary venous drainage (TAPVD) encompasses a wide spectrum of anatomical variants. The infradiaphragmatic type is almost always obstructive owing to the interposition of intrahepatic resistances; since almost invariable, the vertical vein inserts on the portal venous system. On correction of this variant, the vertical vein might not be ligated to avoid postoperative pulmonary hypertension. We hereby describe an unusual case of infradiaphragmatic TAPVD, with a vertical vein connected to ductus venosus. Since vertical vein was not ligated, it realized an unrestrictive pathway between the left atrium and the suprahepatic veins which resulted in persistent chylous peritoneal drainage. The patient successfully underwent catheter occlusion of the vertical vein which led to complete resolution of the clinical picture.

Patterns of myocardial involvement in children during COVID-19 pandemic: Early experience from northern Italy.

There is limited information about coronavirus disease 2019 (COVID-19) in the pediatric population. Preliminary data suggest a not insignificant prevalence of cardiac involvement. Here, we report our early experience with COVID-19 in the pediatric population. These patients display exceptionally high levels of acute-phase reactants. The clinical syndrome in these patients is somewhat similar to Kawasaki disease with or without myocardial involvement. In some cases, the presentation mimics typical myocarditis. Severe myocardial involvement is associated with transient electrocardiographic and echocardiographic abnormalities. These findings may be due to the cardiotropic nature of the virus or may be the result of an immunologic response to the infection.

COVID-19 in adult patients with CHD: a matter of anatomy or comorbidities?

Little is know about COVID-19 outcome in specific populations such as Adult congenital heart disease (ACHD) patients. We report three cases of adult patients with similar underlying disease with completely different clinical severity at the time of COVID-19 infection. The patient with the most severe clinical course was obese and diabetic, suggesting that COVID-19 mortality and morbidity in Adult congenital heart disease patients might be independent of anatomic complexity.

An outbreak of severe Kawasaki-like disease at the Italian epicentre of the SARS-CoV-2 epidemic: an observational cohort study.

BACKGROUND: The Bergamo province, which is extensively affected by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) epidemic, is a natural observatory of virus manifestations in the general population. In the past month we recorded an outbreak of Kawasaki disease; we aimed to evaluate incidence and features of patients with Kawasaki-like disease diagnosed during the SARS-CoV-2 epidemic. METHODS: All patients diagnosed with a Kawasaki-like disease at our centre in the past 5 years were divided according to symptomatic presentation before (group 1) or after (group 2) the beginning of the SARS-CoV-2 epidemic. Kawasaki- like presentations were managed as Kawasaki disease according to the American Heart Association indications. Kawasaki disease shock syndrome (KDSS) was defined by presence of circulatory dysfunction, and macrophage activation syndrome (MAS) by the Paediatric Rheumatology International Trials Organisation criteria. Current or previous infection was sought by reverse-transcriptase quantitative PCR in nasopharyngeal and oropharyngeal swabs, and by serological qualitative test detecting SARS-CoV-2 IgM and IgG, respectively. FINDINGS: Group 1 comprised 19 patients (seven boys, 12 girls; aged 3·0 years [SD 2·5]) diagnosed between Jan 1, 2015, and Feb 17, 2020. Group 2 included ten patients (seven boys, three girls; aged 7·5 years [SD 3·5]) diagnosed between Feb 18 and April 20, 2020; eight of ten were positive for IgG or IgM, or both. The two groups differed in disease incidence (group 1 vs group 2, 0·3 vs ten per month), mean age (3·0 vs 7·5 years), cardiac involvement (two of 19 vs six of ten), KDSS (zero of 19 vs five of ten), MAS (zero of 19 vs five of ten), and need for adjunctive steroid treatment (three of 19 vs eight of ten; all p<0·01). INTERPRETATION: In the past month we found a 30-fold increased incidence of Kawasaki-like disease. Children diagnosed after the SARS-CoV-2 epidemic began showed evidence of immune response to the virus, were older, had a higher rate of cardiac involvement, and features of MAS. The SARS-CoV-2 epidemic was associated with high incidence of a severe form of Kawasaki disease. A similar outbreak of Kawasaki-like disease is expected in countries involved in the SARS-CoV-2 epidemic. FUNDING: None.

Coronary pathology of inherited generalized arterial calcification of infancy: a case report.

Generalized arterial calcification of infancy (GACI), or idiopathic infantile arterial calcification, is a rare autosomal-recessive disease recognized aAs an inherited disorder characterized by severe pathologic calcification of large- and medium-sized arteries accompanied by smooth muscle cell (SMC) hyperplasia leading to vascular obstruction [1]. The prognosis is extremely poor, with 85% of affected infants dying within the first 6 months of life. Loss-of-function mutations in the ectonucleotide pyrophosphatase phosphodiesterase 1 (ENPP1) gene is recognized as the main defect associated with GACI [1]. The underlying pathogenesis of osteogenic transition leading to calcification and severe stenosis in GACI, however, is poorly understood. Herein, we present a case of a GACI patient with cardiac complications who exhibited extensive vascular disease at autopsy.

Optimus covered stent: Advanced covered stent technology for complex congenital heart disease.

AIM: To assess the acute results of the first human use of the Optimus covered stent in complex coarctation of the aorta. METHODS AND RESULTS: We successfully implanted the Optimus covered stent in eight cases in patients whose preprocedural anatomy looked challenging for currently available covered stents. Six of the patients had native coarctation with one recoarctation following surgical repair. There were no significant complications with reduction in the mean invasive gradient from 22 to 1 mm Hg. The length of stents used ranged from 33 to 57 mm with a median shortening after expansion of 13%. Postprocedural follow-up with magnetic resonance imaging or computed tomography has not shown evidence of fracture or migration or renarrowing. The median duration of follow-up is 10 months. CONCLUSIONS: Preliminary results show that the Optimus covered stent is safe and efficacious for use in patients with coarctation of challenging morphology. A systemic trial will be required to evaluate this stent for more widespread practice.

The unnatural history of failing univentricular hearts: outcomes up to 25 years after heart transplantation.

OBJECTIVES: Heart transplantation (HTx) in children with a univentricular physiology is a challenge. In this study, we aimed to investigate the early and late survival as well as the causes of death of HTx recipients at different stages of univentricular palliation. METHODS: Between January 1987 and September 2015, 40 orthotropic cardiac transplants were performed in 38 children with univentricular hearts at our institution. Outcomes were reviewed using medical records and transplant databases. RESULTS: For the purposes of this analysis, patients were divided into 3 subgroups according to their stage of palliation: Stage 1 (n = 10, 26%), Stage 2 (n = 5, 13%) and Fontan (n = 23, 61%). The median age at HTx was 15.2 years (range 0-38). The median follow-up time after transplantation was 8.7 years (range 0-25.4). Indications for transplant were ventricular dysfunction in 25 (66%) patients, protein losing enteropathy in 10 patients transplanted in Fontan (26%) and refractory arrhythmias in 3 (8%) patients with an atriopulmonary connection. Total mortality was 42% (4.84/100 patient-years), and total early mortality was 21%. Overall survival at 1, 10 and 20 years was 73% (95% confidence interval 56-84%), 58% (95% confidence interval 40-72%) and 49% (95% confidence interval 30-65%), respectively. CONCLUSIONS: HTx is a feasible option for patients with failing univentricular circulation, and although the mortality rate is high, this rate is still comparable to that in patients undergoing HTx for other congenital and non-congenital heart diseases.

Sagittal Normal Limits of Lumbosacral Spine in a Large Adult Population: A Quantitative Magnetic Resonance Imaging Analysis.

OBJECTIVES: The objective of this study was to determine, using magnetic resonance imaging (MRI) of the lumbosacral spine from L1 to S1, the values of the normal sagittal diameter of the spinal canal (SCD), sagittal diameter of the dural sac (DSD), and the normal values of dural sac ratio (DSR) in a large nonsymptomatic adult population and to discriminate whether a vertebral canal is pathological or nonpathological for dural ectasia and/or stenosis. MATERIALS AND METHODS: Six hundred and four patients were prospectively enrolled. All measurements were performed on MRI sagittal T1- and T2-weighted images. The 95% confidence interval (95% CI), defined as mean ± 1.96 standard deviation, was determined for each metric. The upper limit of 95% CI was considered the cutoff value for the normal DSR; the lower limit of 95% CI was considered the cutoff value for the normal SCD. RESULTS: SCD cutoff values from L1 to S1 ranged from 14.5-10.1 mm (males) to 15.0-9.9 mm (females). DSD ratios at S1 and L4 level show a significant difference in male and female groups: 11% of S1/L4 values exceeded 1 in male group while only 4% of S1/L4 values exceeded 1 in female group. Mean DSR at each level was significantly higher in female patients than in male patients (P < 0.001), ranging from 0.70 to 0.56 (male) and from 0.82 to 0.63 (female). CONCLUSIONS: We determined the cutoff values for the normal DSR and for the normal SCD. Our findings show the relevant discrepancies with respect to literature data for diagnosis of lumbar stenosis and/or dural ectasia.

Coronary Artery Involvement of Williams Syndrome in Infants and Surgical Revascularization Strategy.

Williams syndrome (WS) is a genetic disorder due to deficiency of elastin gene expression. It is characterized by typical somatic abnormalities and a wide range of cardiovascular malformations. Coronary artery involvement is a frequent finding of the syndrome, particularly in those patients with severe supravalvular aortic stenosis. We present the case of an 11-month-old infant affected by WS who developed severe coronary artery disease 2 months after the surgical repair of supravalvular aortic stenosis. The clinical picture and successful surgical revascularization strategy is also described.

On the cutting edge of intensity modulated radiotherapy and simultaneous integrated boost (IMRT-SIB): The case of a patient with 8 brain metastases.

BACKGROUND: Patients with multiple brain metastases, especially those with more than 3 lesions, usually undergo to palliative whole brain (WB) radiotherapy (RT). METHODS: A breast cancer patient with 8 brain metastases was treated on the brain by a radical RT regimen. Prescription doses were according to the simultaneous integrated boost-intensity modulated radiation therapy (SIB-IMRT) technique with all lesions as well brain irradiated simultaneously in 20 daily fractions. Doses of 40.0 Gy (2.0 Gy/fraction) and 50.0 Gy (2.5 Gy/fraction) were prescribed to the whole brain and to eight individual metastases, respectively. RESULTS: Mean volume of the eight metastases was 8.1 cc (range: 3.8-10.1 cc). For all lesions, the volume receiving 95% of prescribed dose was 100% and dose homogeneity was within 3%. Moreover, maximum doses were less than 105% of prescribed dose, while average mean dose to lesions was 50.6 Gy (range: 49.7-51.5 Gy). Whole brain mean dose was 45.2 Gy. Maximum doses to brainstem and optic chiasma were limited to 44.5 Gy and 42.9 Gy, respectively, while maximum doses to eyes, lens and optic nerves were limited to 9.2 Gy, 4.9 Gy and 41.0 Gy, respectively. From a clinical point of view, subsequent MRI brain controls showed a complete clinical response. Forty months after treatment the patient is disease free and shows no late brain and skin toxicities. CONCLUSION: This case demonstrates the technical feasibility of a SIB-IMRT treatment in patients with more than 3 brain metastases.

[Patent ductus arteriosus]. / Pervietà del dotto arterioso.

The ductus arteriosus is a physiological structure allowing shunt of blood from the pulmonary artery to the descending aorta during fetal life (unventilated lung). The incidence of isolated patent ductus arteriosus (PDA) in children born at term is about 1 per 2000 live births, accounting for 5-10% of all congenital heart defects. The pathophysiological significance and management of PDA vary according to the clinical context (isolated anomaly or as part of complex congenital heart disease), magnitude of the shunt, and stadium within the natural history. This review summarizes the embryogenetic, pathophysiological, clinical and therapeutic basic concepts for an updated approach to this particular heart disease commonly seen even by the cardiologist of the adult.

Integrated imaging of breast hamartoma: two case reports.

Hamartoma of the breast is an uncommon, benign, slow-growing mass usually diagnosed in women in the fourth and fifth decade of life undergoing mammography (MX). Here we report two cases of hamartoma of the breast assessed by integrated MX, ultrasonography and magnetic resonance imaging (MRI) examination. Case 1 was an asymptomatic 47-year-old woman who had never been screened previously. A 90 mm mass was found in her left breast on MX. Case 2 was a 35-year-old woman with pain in her right breast where a 50 mm mass was found on MX. Both patients underwent MRI examination. Breast MRI is an adjunct to MX that can confirm hamartoma diagnosis and exclude rare malignant transformation.

Complete rightward cardiac luxation caused due to left tension pneumothorax.

We present a 4-year-old patient with severe rightward cardiac dislocation due to left tension pneumothorax after surgical closure of a perimembranous ventricular septal defect. The chest roentgenograms showed the "dextrocardia-like" appearance of the cardiac silhouette. Given the persistent severe cardiac dislocation despite resolution of the pneumothorax, and considering the patient's ongoing hemodynamic instability, we decided to reopen the chest to return the heart to its natural position. The procedure was uneventful, and the patient was discharged at home on postoperative day 5.

Osteosarcoma-like divergent differentiation in sarcomatoid renal cell tumor. A rare entity whose diagnosis may be difficult.

A sarcomatoid component can occur in all histologic subtypes of renal cell carcinoma (RCC) and indicates an aggressive tumor. It is reported in literature that the sarcomatoid component can resemble fibrosarcoma, malignant fibrous histiocytoma or rhabdomyosarcoma. We describe the case of a 59-years old patient diagnosed with a chromophobe renal cell carcinoma, presenting a very rare osteosarcoma-like differentiation of the sarcomatoid component. The differential diagnosis with retroperitoneal calcifying masses is also discussed.

MRI findings of large low-grade angiosarcoma of the breast with subsequent bone metastases: a case report.

We describe the magnetic resonance imaging (MRI) findings of 13 cm-sized low-grade angiosarcoma of the breast that occurred in a 23-year-old woman. Magnetic resonance examination revealed an ill-defined mass with marked high-signal intensity on T2-weighted images and persistent heterogeneous enhancement. Thirty months later she developed bone metastases, incidentally found on an MRI performed to evaluate the pelvis. There were well-defined bone lesions with high-signal intensity on T2-weighted images and persistent contrast enhancement on delayed phases. The metastases were not detected on previous computed tomography and fluoro-deoxyglucose positron emission tomography scans because the lesions were subtle osteoblastic type with a low proliferative index.

Carcinoma in situ (CIS) of the testis.

Abstract. Testicular cancer is the most common malignancy in 20- to 34-years-old males. It has been stated that testicular cancer derives from a precocious lesion, the carcinoma in situ of the testis, also known as Intratubular Germ Cell Neoplasia (IGCN) or Testicular Intraepithelial Neoplasia (TIN). This lesion deserves great attention, because the diagnosis of CIS may lead to a precocious diagnosis of testicular cancer. Generally, the diagnosis of CIS is incidental. Every physician should know the management of this precocious lesion, as the correct management of CIS can lead to a decrease of the incidence of overt testicular cancer (the most frequent malignancy in young men). Moreover, the correct diagnosis and management of CIS can shorten the hospital stay, reduce the cost, and improve the social impact of the testicular cancer.

Postoperative analgesia by epidural infusion of ropivacaine and fentanyl versus intravenous administration of morphine in patients undergoing radical retropubic prostatectomy: results of a prospective study.

OBJECTIVES: Radical prostatectomy is followed by postoperative pain, that can be linked to potential morbidity. Aim of this study is to compare efficacy of epidural infusion of ropivacaine and fentanyl versus intravenous administration of morphine in terms of reduction of pain and possible complications. MATERIALS AND METHODS: We have considered 73 consecutive patients who underwent a retropubic radical prostatectomy. For analgesia control we have considered the use of epidural infusion of ropivacaine and fentanyl (Group R) in 48 patients, compared to continuous intravenous administration of morphine (Group M) in 25 patients. Evaluation criteria have been: blood pressure, heart and breath rate, sedation state, nausea and vomit, entity of pain with VRS scale (Verbal Rating Score) at rest and in movement in the first 72 hours, canalization and need of additional analgesia. RESULTS: VRS values were lower in Group R especially during movement, and need of additional analgesia was reported in one patient of Group R, and in three patients of Group M; assumption of morphine was interrupted in three patients. Epidural catheter was removed for malfunction in three patients, and seven patients complained for leg paresthesia. CONCLUSION: Sedation resulted higher in group M. Mobilization in second day occurred with fewer difficulties for patients of group R, while canalization resulted delayed in group M. Results underlined a good analgesic cover in both groups. Advantages of epidural analgesia are represented by the use of smaller doses of opioids, fewer cardiocirculatory and respiratory side effects, lower incidence of nausea and vomit, early canalization and post-operative mobilization.