Electrocardiographic abnormalities in patients with cardiomyopathies

Abnormalities in impulse generation and transmission are among the first signs of cardiac remodeling in cardiomyopathies. Accordingly, 12-lead electrocardiogram (ECG) of patients with cardiomyopathies may show multiple abnormalities. Some findings are suggestive of specific disorders, such as the discrepancy between QRS voltages and left ventricular (LV) mass for cardiac amyloidosis or the inverted T waves in the right precordial leads for arrhythmogenic cardiomyopathy. Other findings are less sensitive and/or specific, but may orient toward a specific diagnosis in a patient with a specific phenotype, such as an increased LV wall thickness or a dilated LV. A “cardiomyopathy-oriented” mindset to ECG reading is important to detect the possible signs of an underlying cardiomyopathy and to interpret correctly the meaning of these alterations, which differs in patients with cardiomyopathies or other conditions

Use of Intravascular Ultrasound to Improve Diagnosis and Treatment of Transjugular Intrahepatic Portosystemic Shunt Dysfunction in Patients in the Long-term Follow-up

Aim: To evaluate the efficacy of intravascular ultrasound (IVUS) in transjugular intrahepatic portosystemic shunt (TIPS) revision associated with phlebography and invasive pressure measurement in patients with clinical or radiological signs of TIPS malfunction. Background: Four patients underwent TIPS revision between February and August 2021. Right internal jugular vein access was achieved under ultrasonographic guidance, a catheter was advanced to achieve the Inferior Vena Cava (IVC) and afterward the Portal vein through the TIPS. Once the Portal vein was achieved, a phlebography was performed, followed by invasive pressure measurement and IVUS exam over the guidewire. Based on the combination of phlebography, invasive pressure measurement, and IVUS evaluations, TIPS dysfunction was treated either with angioplasty or stent apposition. Case description: In all patients, we obtained the reduction of porto-systemic gradient. In three patients, angioplasty with a 10 mm diameter balloon catheter was performed. Anticoagulation therapy was added to one patient. In one patient, the Viatorr's proximal extremity in the suprahepatic vein wall was dislocated, so it was lengthened with a "Viabahn" covered stent. None of the patients developed hepatic encephalopathy after both TIPS placement and TIPS revision. No complications related to the procedure were observed during the follow-up. Clinical improvement in the immediate follow-up period was observed in all patients. In two patients, the abdominal ascites resolved. In another one, the abdominal pain disappeared, and a reduction of the longitudinal spleen diameter was recorded at 3 months follow-up. Conclusion: The use of IVUS allowed us to correctly visualize the organic cause of TIPS malfunction and to obtain direct visualization of the results of endovascular treatment. How to cite this article: Morosetti D, Lenci I, Argirò R, et al. Use of Intravascular Ultrasound to Improve Diagnosis and Treatment of Transjugular Intrahepatic Portosystemic Shunt Dysfunction in Patients in the Long-term Follow-up. Euroasian J Hepato-Gastroenterol 2022;12(1):50-56

Vertebral morphometry by dual-energy X-ray absorptiometry (DXA) for osteoporotic vertebral fractures assessment (VFA)

This study was done to evaluate the diagnostic accuracy of dual-energy X-ray absorptiometry (DXA) compared with conventional radiography for identifying vertebral fractures. A total of 930 postmenopausal women underwent conventional radiography and DXA imaging of the spine. The images were evaluated by two expert skeletal radiologists using the semiquantitative (SQ) method for conventional radiography and the morphometric vertebral fracture assessment (VFA) for DXA. The SQ method for radiography (SQ-Rx) analysed 99.1% of vertebrae, identifying 442 vertebral fractures; VFA analysed 97.5% vertebrae, detecting 420 vertebral fractures. Agreement between SQ-Rx and VFA reached 98.76%, and the kappa-score was 0.96 [95% confidence interval (CI), 0.95-0.98]. Assessing the grading of vertebral fractures, agreement reached 97.5% and the kappa-score was 0.841 (95% CI, 0.821-0.891). Considering SQ-Rx method as "gold standard", VFA had a sensitivity of 97.85 % and a specificity of 99.81%. The negative (NPV) and positive (PPV) predictive value for VFA were 99.83 % and 98.15%, respectively. Fractures were identified in 251 (27 %) and 242 (26 %) of patients on SQ-Rx and VFA, respectively. On a per-patient basis, the agreement between the two methods was 97% and the kappa-score was 0.95 (95% CI, 0.920-0.968). The diagnostic parameters for VFA were 97.23% sensitivity, 98.86% specificity, 97.60% PPV and 98.84% NPV. This study demonstrated that VFA with DXA may reach a high level of accuracy for diagnosing vertebral fractures, suggesting that VFA should be introduced in the screening of individuals with a risk of osteoporosis and in the follow-up of osteoporotic patients receiving treatment

Lymph Node Metastases from Differentiated Thyroid Carcinoma: does Radioiodine still play a role?

Ultrasonography and surgery have now become the elective diagnostic and therapeutic tools for neck lymph node (LN) metastases from differentiated thyroid carcinoma (DTC), reserving radioiodine therapy (RAI) for surgery failures. Aim of the present retrospective study was to evaluate results of RAI in cases of LN metastases displaying 131I uptake over a long-term observation period and its possible role today. From a series of 1276 patients who had undergone surgery for DTC, 130 cases were selected showing 131I uptaking LN metastases, detected during follow-up scans and who were then submitted to surgery and/or RAI. Patients were divided into groups according both to extent of surgery, with/without lymphectomy, and to following treatment and outcome. The initial surgical approach does not seem to significantly influence the outcome. 131I therapy alone, sometimes at low doses, can be very effective in the management of LN metastases detected at Whole Body Scan, but multiple doses are often needed. The age at diagnosis is confirmed as a negative prognostic factor. Considered radioprotection questions, RAI may solve 131I uptaking LN metastases, above all if < 10 mm. For larger LN metastases and in the case of failure of RAI surgical excision is mandatory, while a subsequent therapeutic dose of 131I could be useful to reveal incomplete excision

Liver-Specific Contrast-Enhanced Magnetic Resonance Cholangio-Pancreatography (Ce-MRCP) in Non-Invasive Diagnosis of Iatrogenic Biliary Leakage

Current non-invasive diagnostic modalities of iatrogenic bile leak (BL) are not particularly sensitive and often fail to localise the BL origin. Percutaneous transhepatic cholangiography (PTC) and endoscopic retrograde cholangiopancreatography (ERCP) are considered the gold standard, yet are invasive studies with potential complications. Ce-MRCP has been not comprehensively studied in this setting but may prove particularly helpful given its non-invasive nature and the anatomical dynamic detail. This paper reports a monocentric retrospective study of BL patients referred between January 2018 and November 2022 submitted to Ce-MRCP followed by PTC. The primary outcome was the accuracy of Ce-MRCP in detecting and localising BL compared to PTC and ERCP. Blood tests, coexisting cholangitis features and time for leak resolution were also investigated. Thirty-nine patients were included. Liver-specific contrast-enhanced MRCP detected BL in 69% of cases. The BL localisation was 100% accurate. Total bilirubin above 4 mg/dL was significantly associated with false negative results of Ce-MRCP. Ce-MRCP is highly accurate in detecting and localising BL, but sensitivity is significantly reduced by a high bilirubin level. Ce-MRCP may be very useful in early BL diagnosis and in accurate pre-treatment planning, but can only be reliably used in selected patients with TB &lt; 4 mg/dL. Non-surgical techniques, both radiological and endoscopic, are proven to be effective in terms of leak resolution

Prevalence of transthyretin-related amyloidosis in Tuscany: Data from the regional population-based registry

: The limited available data regarding the prevalence of transthyretin amyloidosis, both for wild-type (ATTRwt) and hereditary form (ATTRv), is inferred from highly selected patients and subsequent extrapolations that limit the comprehension of the clinical disease impact. The Tuscan healthcare system in 2006 developed a web-based rare disease registry, to monitor and profile patients affected by rare diseases. Clinicians belonging to regional validated healthcare data centres can register patients at the diagnosis, with a rigorous approach and distinguishing the types of amyloidosis, i.e., ATTRwt versus ATTRv. Thanks to this data collection method, available from July 2006 and extended with electronic therapy plans related to a diagnosis since May 2017, we analysed prevalence and incidence of ATTR and its subtypes. On November 30th 2022, ATTRwt prevalence in Tuscany is 90.3 per 1,000,000 persons and ATTRv prevalence is 9.5 per 1,000,000 persons, whereas the annual incidence ranges from 14.4 to 26.7 per 1,000,000 persons and from 0.8 to 2.7 per 1,000,000 persons, respectively. The male gender is predominant in both forms. All except one patient showed evidence of cardiomyopathy. This epidemiological data requires attention, not only to increase the effort for the clinical management and earlier diagnosis, but also to underline the need for the disease-specific treatments

Incidence and risk factors for pacemaker implantation in light-chain and transthyretin cardiac amyloidosis

The incidence and risk factors of pacemaker (PM) implantation in patients with cardiac amyloidosis (CA) are largely unexplored. We sought to characterize the trends in the incidence of permanent PM and to identify baseline predictors of future PM implantation in light-chain (AL) and transthyretin (ATTR) CA

Diagnostic pathways to wild-type transthyretin amyloid cardiomyopathy: a multicentre network study

Aim Epidemiology of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) remains poorly defined. A better characterization of pathways leading to ATTRwt-CA diagnosis is of key importance, and potentially informative of disease course and prognosis. The aim of this study was to describe the characteristics of contemporary pathways leading to ATTRwt-CA diagnosis, and their potential association with survival.Methods and results This was a retrospective study of patients diagnosed with ATTRwt-CA at 17 Italian referral centres for CA. Patients were categorized into different 'pathways' according to the medical reason that triggered the diagnosis of ATTRwt-CA (hypertrophic cardiomyopathy [HCM] pathway, heart failure [HF] pathway, incidental imaging or incidental clinical pathway). Prognosis was investigated with all-cause mortality as endpoint. Overall, 1281 ATTRwt-CA patients were included in the study. The diagnostic pathway leading to ATTRwt-CA diagnosis was HCM in 7% of patients, HF in 51%, incidental imaging in 23%, incidental clinical in 19%. Patients in the HF pathway, as compared to the others, were older and had a greater prevalence of New York Heart Association (NYHA) class III-IV and chronic kidney disease. Survival was significantly worse in the HF versus other pathways, but similar among the three others. In multivariate model, older age at diagnosis, NYHA class III-IV and some comorbidities but not the HF pathway were independently associated with worse survival.Conclusions Half of contemporary ATTRwt-CA diagnoses occur in a HF setting. These patients had worse clinical profile and outcome than those diagnosed either due to suspected HCM or incidentally, although prognosis remained primarily related to age, NYHA functional class and comorbidities rather than the diagnostic pathway itself