A Rare Case of Left Ventricular Non-Compaction with Coronary Artery Anomaly Complicated by ST-Elevation Myocardial Infarction and Subcutaneous Defibrillator Implantation

Left ventricular non-compaction (LVNC) is a rare congenital cardiomyopathy caused by arrest of normal endomyocardial embryogenesis and characterized by the persistence of ventricular hypertrabeculation, isolated or associated to other congenital defects. A 33-year-old male, with family history of sudden cardiac death (SCD), presented to our ER with typical chest pain and was diagnosed with anterior STEMI. Coronary angiography showed an anomalous origin of the circumflex artery from the right coronary artery and a critical stenosis on the proximal left anterior descending artery, treated with primary percutaneous coronary intervention. The echocardiogram documented left ventricular severe dysfunction with lateral wall hypertrabeculation, strongly suggestive for non-compaction, confirmed by cardiac MRI. At 3 months follow up, for the persistence of the severely depressed EF (30%) and the family history for SCD, the patient underwent subcutaneous ICD (sICD) implantation for primary prevention. To the best of our knowledge, this is the first case of LVNC associated with anomalous coronary artery origin and STEMI reported in the literature. Arrhythmias are common in LVNC due to endocardial hypoperfusion and fibrosis. sICD overcomes the risks of transvenous ICD, and it is a valuable option when there is no need for pacing therapy for bradycardia, cardiac resynchronization therapy and anti-tachycardia pacing

ASSOCIATION BETWEEN GENOTYPE AND DISEASE PROGRESSION IN ITALIAN STARGARDT PATIENTS: A Retrospective Natural History Study

PURPOSE: To investigate the natural history of Stargardt disease over a multiyear follow-up. METHODS: We reviewed medical records of Stargardt disease patients, with clinical diagnosis of Stargardt disease at a single institution, which was also supported by molecular diagnosis. All patients underwent best-corrected visual acuity, fundus photography, optical coherence tomography, and full-field electroretinography. RESULTS: The study cohort consisted of 157 Stargardt disease patients aged 30.4 ± 1.1 years. Longitudinal analysis (mean follow-up: 3 years) showed a significant worsening of best-corrected visual acuity at an average rate of 1.5 Early Treatment Diabetic Retinopathy Study letters/year (P < 0.001), an enlargement of retinal pigment epithelium lesion area by optical coherence tomography at an average linear rate of 0.10 mm/year (P < 0.001), and a thinning of central macular thickness at a mean rate of -1.42 μm/year (P < 0.001). Survival analysis showed that patients with 2 alleles harboring likely-null variants, on average, reached most severe disease stage, i.e., legal blindness, alteration in both dark-adapted and light-adapted electroretinographic responses, and retinal pigment epithelium lesion area larger than 2.5 mm significantly earlier than patients with at least one allele harboring a missense variant. CONCLUSION: The current longitudinal study showed a significant genotype-phenotype correlation characterization, because patients harboring 2 likely-null alleles reach a severe disease stage about 10 years earlier than patients with at least one missense allele

Correction: Malizia et al. Response of a Radiology Department to the SARS-CoV-2 Pandemic: The Experience of the Hospital &ldquo;Policlinico Tor Vergata&rdquo; in Rome. Int. J. Environ. Res. Public Health 2021, 18, 5255

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Response of a Radiology Department to the SARS-CoV-2 Pandemic: The Experience of the Hospital “Policlinico Tor Vergata” in Rome

The dissemination of severe acute respiratory syndrome linked to the novel coronavirus, SARS-CoV-2, prompted all health services to provide adequate measures to limit new cases that could affect healthcare professionals. Due to the large number of suspected patients subjected to CT scans and the proximity of radiologists to the patient during exams, radiologists as well as the entire staff of the radiology department are particularly exposed to SARS-CoV-2. This article includes the emergency management procedures, the use of personal protective devices, and the rearrangement of exam rooms and of human resources in the department of radiology at “Policlinico Tor Vergata” in Rome performed during the SARS-CoV-2 pandemic. We introduce the management measures that our department has taken to cope with the influx of patients while still ensuring the proper management of other emergencies and time-sensitive exams

Radiological findings in Erdheim Chester disease: A very rare multisistemic disease

: Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis affecting multiple systems. There is limited knowledge on the imaging capabilities of this disease. We present an extremely rare case of Erdheim-Chester illness in a 67-year-old man with multisystem involvement, including the cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration) and the neurologic system. The involvement of the various organs was thoroughly assessed using multimodal imaging modalities such as computed tomography, magnetic resonance imaging, positron emission tomography and bone scintigraphy. Erdheim-Chester illness was revealed by a bone biopsy. Especially when there is cardiac and cerebral involvement, Erdheim-Chester illness is a rare condition with a poor prognosis. Knowing the imaging characteristics of Erdheim-Chester disease may be helpful in understanding the radiological results of many organs affected by the disease as described and discussed in the current case report

Transcatheter aortic valve implantation in patients with unruptured aortic root pseudoaneurysm: an observational study

Unruptured aortic root pseudoaneurysm (UARP) is a rare complication of aortic valve endocarditis. Infectious spread to the valvular annulus or myocardium can cause septic complications that manifest as wall thickening, and spontaneous abscess drainage leads to pseudoaneurysm formation. We report the first patient series in which transcatheter aortic valve implantation (TAVI) using a single valve-resolved aortic valvulopathy associated with UARP was performed

Lymphocytes, Interleukin 6 and D-dimer Cannot Predict Clinical Outcome in Coronavirus Cancer Patients: LyNC1.20 Study

Background/Aim: Knowledge of Coronavirus 19 (COVIDI9) pathogenetic mechanisms is necessary to provide new treatment strategies. This study aims to assess how oncological disease impacts on the clinical course of COVID-19 patients. Patients and Methods: From 1st March to 30th April 2020, 96 COVID-I9 patients were classified according to clinical outcome as severe (n=67) and moderate (n=29). Demographic data, medical history, admission lymphocytes, procalcitonin (PCT), c-reactive-protein (CRP), D-dieter, and Interleukin-6 (IL-6) were collected. Results: A statistically significant association was found between hypertension (p=0.007) and three or more comorbidities with severe outcomes (p.0.034). No statistical differences were found between the severe and moderate groups with regards to the rate of patients with past oncological history. However, no patient allocated in the moderate group had received oncological treatment within 12 months. Higher values of CRP, IL-6, D-Dimer and lower values of lymphocytes were reported in the severe group (p=0.0007, p=0.00386, p=0.041, and p=0.007, respectively). Using binary logistic regression, higher values of CRP (012.8.861; p=0.012) and PCT were associated with a higher risk of severe outcome (OR.21 .075; p=0.008). Within the oncological population, D-Dimer and IL-6 did not confirm their prognostic significance as in the general population (p&gt;0.05). Conclusion: Specific prognostic factors for oncological patients should be designed for COVID-19 clinical practice

Role of computed tomography in transcatheter replacement of 'other valves': a comprehensive review of preprocedural imaging

: Transcatheter procedures for heart valve repair or replacement represent a valid alternative for treating patients who are inoperable or at a high risk for open-heart surgery. The transcatheter approach has become predominant over surgical intervention for aortic valve disease, but it is also increasingly utilized for diseases of the 'other valves', that is the mitral and, to a lesser extent, tricuspid and pulmonary valve. Preprocedural imaging is essential for planning the transcatheter intervention and computed tomography has become the main imaging modality by providing information that can guide the type of treatment and choice of device as well as predict outcome and prevent complications. In particular, preprocedural computed tomography is useful for providing anatomic details and simulating the effects of device implantation using 3D models. Transcatheter mitral valve replacement is indicated for the treatment of mitral regurgitation, either primary or secondary, and computed tomography is crucial for the success of the procedure. It allows evaluating the mitral valve apparatus, the surrounding structures and the left heart chambers, identifying the best access route and the landing zone and myocardial shelf, and predicting obstruction of the left ventricular outflow tract, which is the most frequent postprocedural complication. Tricuspid valve regurgitation with or without stenosis and pulmonary valve stenosis and regurgitation can also be treated using a transcatheter approach. Computer tomography provides information on the tricuspid and pulmonary valve apparatus, the structures that are spatially related to it and may be affected by the procedure, the right heart chambers and the right ventricular outflow tract

Chest Computed Tomography Scoring in Patients With Novel Coronavirus-infected Pneumonia: Correlation With Clinical and Laboratory Features and Disease Outcome

This study investigated the correlation of chest computed tomography (CT), findings, graded using two different scoring methods, with clinical and laboratory features and disease outcome, including a novel clinical predictive score, in patients with novel coronavirus-infected pneumonia (NCIP)

Radiological findings in Erdheim Chester disease: A very rare multisistemic disease

Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis affecting multiple systems. There is limited knowledge on the imaging capabilities of this disease. We present an extremely rare case of Erdheim-Chester illness in a 67-year-old man with multisystem involvement, including the cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration) and the neurologic system. The involvement of the various organs was thoroughly assessed using multimodal imaging modalities such as computed tomography, magnetic resonance imaging, positron emission tomography and bone scintigraphy. Erdheim-Chester illness was revealed by a bone biopsy. Especially when there is cardiac and cerebral involvement, Erdheim-Chester illness is a rare condition with a poor prognosis. Knowing the imaging characteristics of Erdheim-Chester disease may be helpful in understanding the radiological results of many organs affected by the disease as described and discussed in the current case report