Intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: a case series.

Apical hypertrophic cardiomyopathy (ApHCM) is an HCM variant, affecting frequently males in midlife. It is characterized by apical obliteration and persistent diastolic contraction, often resulting in microvascular ischaemia. We report five cases of ApHCM, with evidence of intramyocardial calcification on echocardiogram. On cardiac magnetic imaging (MRI), a hypointense component at early gadolinium enhancement (EGE) sequences, compatible with calcium, and a deep layer, with hyperintensity at late gadolinium enhancement (LGE) sequences, referable to fibrosis, suggest an endomyocardial fibrosis (EMF) diagnosis. EMF pathologic hallmark is endocardium and myocardium scarring, evolving to dystrophic calcification. It is found only in few ApHCM patients. Our series is the largest one described until now. Analysing patients' history, coexistent inflammatory triggers were evident in all of them, so their co-morbidities could represent a further cause of small vessel disease, in the context of ischaemic microvascular stress due to hypertrophy, leading to fibrosis and dystrophic calcification. This series could demonstrate the relation between apical fibrosis/calcification and microvascular ischaemia due to hypertrophy and inflammatory triggers.

Long-term failure after endovascular aneurysm sealing in a real-life, single-center experience with the Nellix endograft.

BACKGROUND: Endovascular aneurysm sealing (EVAS) is an innovative alternative to conventional endovascular aneurysm repair (EVAR). EVAS relies on sac anchoring without proximal fixation to achieve sealing and should have allowed for the treatment of a broader range of anatomic features compared with standard EVAR. Despite the encouraging early reports, the mid- and long-term follow-up data have shown increased rates of failure. To address the issue, the manufacturer introduced revised instructions for use (IFU) in 2016. The present study reports the outcomes of this system after a median follow-up of 45 months. METHODS: Data for all patients electively treated with EVAS at our institution were retrospectively collected. The patients were retrospectively reclassified according to the 2016 revised IFU of the device. All patients in the present series had undergone EVAS for the treatment of infrarenal abdominal aortic aneurysms (AAAs). The primary end point was therapeutic failure: graft migration >5 mm, sac expansion >5 mm, type IA endoleak (Is2 and Is3 using the Van den Ham classification), type Ib endoleak, and secondary rupture. The overall mortality, aortic-related mortality, and reintervention rates were also analyzed. RESULTS: A total of 101 patients had undergone elective treatment by EVAS from 2013 to 2018 for infrarenal AAAs. The median follow-up was 3.75 years. Therapeutic failure was observed in 31 of the 101 patients (30.7%), with no significant difference between the in-IFU and off-IFU 2016 subgroups. Failure occurred at a median interval of 34 months from the index procedure. Of the 101 patients, 6.9% had presented with secondary rupture. Freedom from aneurysm-related mortality was 96.9% at 1 and 2 years and 89.9% at 5 years. Freedom from reintervention decreased over time: 94.7% at 1 year, 77% at 4 years, and 52.1% at 6 years. Of the 101 patients, 14 (13.9%) had undergone emergent or elective graft explantation. CONCLUSIONS: EVAS performed worse than conventional endografts for several critical end points, regardless of any preoperative anatomic parameters. The incidence of therapeutic failures tended to increase over time, especially 4 years after the index procedure.

[Intravenous epinephrine for anaphylaxis: Kounis or takotsubo syndrome?] / Adrenalina endovenosa in corso di reazione anafilattica: sindrome di Kounis o takotsubo?

The presence of an acute coronary syndrome in patients with anaphylaxis is a challenging diagnostic conundrum for the cardiologist. Both Kounis syndrome and takotsubo syndrome must be taken into account. We present here the case of a 46-year-old woman suffering from ventricular fibrillation after adrenaline infusion for an anaphylactic reaction. The case report shows the important role of a clear diagnostic work-up and the role of cardiac magnetic resonance in this clinical scenario to reach the final diagnosis of reverse takotsubo cardiomyopathy.

Whole body MRI with qualitative and quantitative analysis of DWI for assessment of bone marrow involvement in lymphoma.

AIM: Our study aimed to investigate the role of qualitative and quantitative whole body MRI with DWI for assessment of bone marrow involvement (BMI) in newly diagnosed lymphoma using FDG PET-CT and bone marrow biopsy (BMB) as reference standard. MATERIALS AND METHODS: We retrospectively evaluated 56 patients with newly diagnosed lymphoma (21 Hodgkin's lymphoma and 35 non-Hodgkin's lymphoma) who underwent random unilateral BMB, FDG PET-CT and Wb-MRI-DWI for initial staging. In a patient-based analysis, results of Wb-MRI-DWI were compared with FDG PET-CT and BMB. For quantitative analysis, mean ADC values of posterior iliac crest were correlated with BMI and bone marrow cellularity. RESULTS: WB-MR-DWI obtained excellent concordance with FDG PET-CT both in HL (k = 1.000; 95% CI 1.000-1.000) and in DLBCL (k = 1.000; 95% CI 1.000-1.000). In other NHL, WB-MRI-DWI obtained a good correlation with BMB (k = 0.611; 95% CI 0.295-0.927) while FDG PET-CT had poor concordance (k = 0.067; 95% CI 0.372-0.505). WB-MR-DWI has no false negative errors but 4 false positive results consisting in focal lesions consensually reported by FDG PET-CT and resolved after therapy. No significant correlation between ADC mean value and BMI was found (p = 0.0586). CONCLUSION: Our data suggest that Wb-MRI-DWI is a valid technique for BMI assessment in lymphoma patients, thanks to its excellent concordance with FDG PET-CT and good concordance with BMB (superior than FDG PET-CT). If further investigations will confirm our results on larger patient groups, it could become a useful tool in the clinical workup.

Staging of colorectal liver metastases after preoperative chemotherapy. Diffusion-weighted imaging in combination with Gd-EOB-DTPA MRI sequences increases sensitivity and diagnostic accuracy.

OBJECTIVES: To compare the diagnostic accuracy and sensitivity of Gd-EOB-DTPA MRI and diffusion-weighted (DWI) imaging alone and in combination for detecting colorectal liver metastases in patients who had undergone preoperative chemotherapy. METHODS: Thirty-two consecutive patients with a total of 166 liver lesions were retrospectively enrolled. Of the lesions, 144 (86.8 %) were metastatic at pathology. Three image sets (1, Gd-EOB-DTPA; 2, DWI; 3, combined Gd-EOB-DTPA and DWI) were independently reviewed by two observers. Statistical analysis was performed on a per-lesion basis. RESULTS: Evaluation of image set 1 correctly identified 127/166 lesions (accuracy 76.5 %; 95 % CI 69.3-82.7) and 106/144 metastases (sensitivity 73.6 %, 95 % CI 65.6-80.6). Evaluation of image set 2 correctly identified 108/166 (accuracy 65.1 %, 95 % CI 57.3-72.3) and 87/144 metastases (sensitivity of 60.4 %, 95 % CI 51.9-68.5). Evaluation of image set 3 correctly identified 148/166 (accuracy 89.2 %, 95 % CI 83.4-93.4) and 131/144 metastases (sensitivity 91 %, 95 % CI 85.1-95.1). Differences were statistically significant (P < 0.001). Notably, similar results were obtained analysing only small lesions (<1 cm). CONCLUSIONS: The combination of DWI with Gd-EOB-DTPA-enhanced MRI imaging significantly increases the diagnostic accuracy and sensitivity in patients with colorectal liver metastases treated with preoperative chemotherapy, and it is particularly effective in the detection of small lesions.