Temporal progression of replacement and interstitial fibrosis in optimally managed dilated cardiomyopathy patients: A prospective study.

BACKGROUND: To prospectively examine the dynamic evolution of fibrotic processes within a one-year in patients with dilated cardiomyopathy (DCM). METHODS: Between May 2019 and September 2020, 102 DCM patients (mean age 45.2 ± 11.8 years, EF 29.9 ± 11.6%) underwent cardiac magnetic resonance (CMR-1). After 13.9 ± 2.9 months, 92 of these patients underwent a follow-up CMR (CMR-2). Replacement fibrosis was assessed via late gadolinium enhancement (LGE), quantified in terms of LGE mass and extent. Interstitial fibrosis was evaluated via T1-mapping and expressed as extracellular volume fraction (ECV). This data, along with left ventricular (LV) mass, facilitated the calculation of LV matrix and cellular volumes. RESULTS: At CMR-1, LGE was present in 45 patients (48.9%), whereas at CMR-2 LGE was detected in 46 (50%) (p = 0.88). Although LGE mass remained stable, LGE extent increased from 2.18 ± 4.1% to 2.7 ± 4.6% (p < 0.01). Conversely, ECV remained unchanged [27.7% (25.5-31.3) vs. 26.7% (24.5-29.9); p = 0.19]; however, LV matrix and cell volumes exhibited a noteworthy regression. During a subsequent follow-up of 19.2 ± 9 months (spanning from CMR-2 to April 30th, 2023), the composite primary outcome (all-cause mortality, HTX, LVAD or heart failure worsening) was evident in 18 patients. Only the LV matrix volume index at follow-up was an independent predictor of outcome (OR 1.094; 95%CI 1.004-1.192; p < 0.05). CONCLUSIONS: In optimally managed DCM patients, both replacement and interstitial fibrosis remained stable over the course of one year. In contrast, LV matrix and cell volumes displayed significant regression. LV matrix volume index at 12-month follow-up was found to be an independent predictor of outcome in DCM.

Imaging modality-dependent carotid stenosis severity variations against intravascular ultrasound as a reference: Carotid Artery intravasculaR Ultrasound Study (CARUS).

PURPOSE: Different non-invasive and invasive imaging modalities are used to determine carotid artery stenosis severity that remains a principal parameter in clinical decision-making. We compared stenosis degree obtained with different modalities against vascular imaging gold standard, intravascular ultrasound, IVUS. METHODS: 300 consecutive patients (age 47-83 years, 192 men, 64% asymptomatic) with carotid artery stenosis of " ≥ 50%" referred for potential revascularization received as per study protocol (i) duplex ultrasound (DUS), (ii) computed tomography angiography (CTA), (iii) intraarterial quantitative angiography (iQA) and (iv) and (iv) IVUS. Correlation of measurements with IVUS (r), proportion of those concordant (within 10%) and proportion of under/overestimated were calculated along with recipient-operating-characteristics (ROC). RESULTS: For IVUS area stenosis (AS) and IVUS minimal lumen area (MLA), there was only a moderate correlation with DUS velocities (peak-systolic, PSV; end-diastolic, EDV; r values of 0.42-0.51, p < 0.001 for all). CTA systematically underestimated both reference area and MLA (80.4% and 92.3% cases) but CTA error was lesser for AS (proportion concordant-57.4%; CTA under/overestimation-12.5%/30.1%). iQA diameter stenosis (DS) was found concordant with IVUS in 41.1% measurements (iQA under/overestimation 7.9%/51.0%). By univariate model, PSV (ROC area-under-the-curve, AUC, 0.77, cutoff 2.6 m/s), EDV (AUC 0.72, cutoff 0.71 m/s) and CTA-DS (AUC 0.83, cutoff 59.6%) were predictors of ≥ 50% DS by IVUS (p < 0.001 for all). Best predictor, however, of ≥ 50% DS by IVUS was stenosis severity evaluation by automated contrast column density measurement on iQA (AUC 0.87, cutoff 68%, p < 0.001). Regarding non-invasive techniques, CTA was the only independent diagnostic modality against IVUS on multivariate model (p = 0.008). CONCLUSION: IVUS validation shows significant imaging modality-dependent variations in carotid stenosis severity determination.

Link between fibrosis-specific biomarkers and interstitial fibrosis in hypertrophic cardiomyopathy.

BACKGROUND: Cardiac fibrosis is a hallmark of hypertrophic cardiomyopathy (HCM) and has confirmed unfavorable clinical significance. Replacement fibrosis is better known and has already been studied on a larger scale, whereas interstitial fibrosis is less explored. AIMS: We aimed to analyze the relationship between serum biomarkers and interstitial fibrosis, as assessed with cardiac magnetic resonance (CMR) in HCM patients. METHODS: We performed 3T CMR scans in 50 HCM patients to assess interstitial fibrosis as expressed by extracellular volume (ECV). In all patients, we determined levels of serum cardiac-specific (troponin T [TnT], N-terminal prohormone of brain natriuretic peptide [NT-proBNP]) and fibrosis-specific (procollagen I C-terminal propeptide, procollagen III N-terminal propeptide, transforming growth factor ß1, galectin-3) biomarkers. Patients were divided based on their median value of ECV. RESULTS: The final study population included 49 patients. The median value of ECV in our cohort was 28.1%. Patients stratified according to median ECV differed in terms of several variables: body mass index, late gadolinium extent, NT-proBNP, and galectin-3 levels (all P <0.05). Cardiac biomarkers (TnT and NT-proBNP) and galectin-3 were significantly correlated with ECV (rS = 0.34; P = 0.02; rS = 0.39; P = 0.006; rS = 0.43; P = 0.002, respectively). Galectin-3 and body mass index were found to be independent predictors of ECV (odds ratio [OR], 2.29 [1.07-4.91]; P = 0.03; OR, 0.81 [0.68-0.97]; P = 0.02, respectively). CONCLUSIONS: Galectin-3 was an independent predictor of interstitial fibrosis in HCM patients expressed as elevated ECV values. The other measured fibrosis-specific biomarkers were not useful in detecting interstitial fibrosis in HCM. In addition, there was a positive correlation between classical cardiac biomarkers and interstitial fibrosis in HCM patients.

The Relationship between Cardiac Magnetic Resonance-Assessed Replacement and Interstitial Fibrosis and Ventricular Arrhythmias in Hypertrophic Cardiomyopathy.

Non-sustained ventricular tachycardia (nsVT) creates the electrical basis for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). We aimed to evaluate the relationship between interstitial fibrosis on cardiac magnetic resonance (CMR) and nsVT in HCM. A total of 50 HCM patients underwent CMR with a 3 T scanner to determine the presence of replacement fibrosis expressed by late gadolinium enhancement (LGE), and interstitial fibrosis expressed by native T1, post-contrast T1, and extracellular volume (ECV). The incidence of nsVT was assessed by Holter monitoring. We detected nsVT in 14 (28%) out of 50 HCM patients. Replacement fibrosis expressed by LGE was present in 37 (74%) patients and only showed a trend towards a differentiation between the groups with and without nsVT (p = 0.07). However, the extent of LGE was clearly higher in the nsVT group (3.8 ± 4.9% vs. 7.94 ± 4.5%, p = 0.002) and was an independent predictor of nsVT in a multivariable regression analysis (OR 1.2; 95%CI 1.02-1.4; p = 0.02). No relationship was observed between interstitial fibrosis and nsVT. To conclude, it was found that it is not the mere presence but the actual extent of LGE that determines the occurrence of nsVT in HCM patients; the role of interstitial fibrosis remains unclear.

Misclassification of carotid stenosis severity with area stenosis-based evaluation by computed tomography angiography: impact on erroneous indication to revascularization or patient (lesion) migration to a higher guideline recommendation class as per ESC/ESVS/ESO/SVS and CMS-FDA thresholds.

Intoduction: Despite a growing understanding of the role played by plaque morphology, the degree of carotid lumen reduction remains the principle parameter in decisions on revascularization in symptomatic and asymptomatic patients. Computed tomography angiography (CTA) is a widely used guideline-approved imaging modality, with "percent stenosis" commonly calculated as %area reduction (area stenosis - AS). Aim: We evaluated the impact of the non-linear relationship between diameter stenosis (DS) and AS (area = π • (diameter/2)2, so that in concentric lesions 51%AS is 30%DS and 75%AS is 50%DS) on stenosis severity misclassification using calculation of area reduction. Material and methods: CTA and catheter quantitative angiography (cQA) were performed in 300 consecutive patients referred to a tertiary vascular centre for potential carotid revascularization (age: 47-83 years, 33.7% symptomatic, 36% female; referral stenosis of ≥ "50%"). CTA-AS was determined by agreement of 2 experienced radiologists; cQA-DS (pivotal trials standard reference, NASCET method) was calculated by agreement of 2 corelab analysts. Results: For symptomatic lesion thresholds, CTA-AS-based calculation reclassified 76% of "< 50%" cQA-DS measurements to the "50-69%" group, and 58% of "50-69%" measurements to the "≥ 70%" group. For asymptomatic lesion thresholds, 78% of "< 60%" cQA-DS measurements were reclassified to the "60-79%" group, whereas 42% of "60-79%" cQA measurements crossed to the "≥ 80%" class. Overall, employing CTA-AS instead of cQA-DS enlarged the "60-79%" and "≥ 80%" lesion severity classes 1.6- and 5.8-fold, respectively, whereas the "≥ 70%" class increased 4.15-fold. Conclusions: Replacing the pivotal carotid trials reference standard cQA-DS "%stenosis" measurement with CTA-AS-based "%stenosis" results in a large-scale lesion/patient erroneous gain of an "indication" to revascularization or migration to a higher revascularization indication class. In consequence, unnecessary carotid procedures may be performed in the absence of cQA verification. Until guidelines rectify the "%stenosis" measurement methods with different guideline-approved imaging modalities (and, where needed, re-adjust decision thresholds), CTA-AS measurement should not be used as a basis for carotid revascularization.

Extracellular volume is an independent predictor of arrhythmic burden in dilated cardiomyopathy.

The current stratification of arrhythmic risk in dilated cardiomyopathy (DCM) is sub-optimal. Cardiac fibrosis is involved in the pathology of arrhythmias; however, the relationship between cardiovascular magnetic resonance (CMR) derived extracellular volume (ECV) and arrhythmic burden (AB) in DCM is unknown. This study sought to evaluate the presence and extent of replacement and interstitial fibrosis in DCM and to compare the degree of fibrosis between DCM patients with and without AB. This is a prospective, single-center, observational study. Between May 2019 and September 2020, 102 DCM patients underwent CMR T1 mapping. 99 DCM patients (88 male, mean age 45.2 ± 11.8 years, mean EF 29.7 ± 10%) composed study population. AB was defined as the presence of VT or a high burden of PVCs. There were 41 (41.4%) patients with AB and 58 (58.6%) without AB. Replacement fibrosis was assessed with late gadolinium enhancement (LGE), whereas interstitial fibrosis with ECV. Overall, LGE was identified in 41% of patients. There was a similar distribution of LGE (without AB 50% vs. with AB 53.7%; p = 0.8) and LGE extent (without AB 4.36 ± 5.77% vs. with AB 4.68 ± 3.98%; p = 0.27) in both groups. ECV at nearly all myocardial segments and a global ECV were higher in patients with AB (global ECV: 27.9 ± 4.9 vs. 30.3 ± 4.2; p < 0.02). Only indexed left ventricular end-diastolic diameter (HR 1.1, 95%CI 1.0-1.2; p < 0.02) and global ECV (HR 1.12, 95%CI 1.0-1.25; p < 0.02) were independently associated with AB. The global ECV cut-off value of 31.05% differentiated both groups (AUC 0.713; 95%CI 0.598-0.827; p < 0.001). Neither qualitative nor quantitative LGE-based assessment of replacement fibrosis allowed for the stratification of DCM patients into low or high AB. Interstitial fibrosis, expressed as ECV, was an independent predictor of AB in DCM. Incorporation of CMR parametric indices into decision-making processes may improve arrhythmic risk stratification in DCM.

Successful percutaneous treatment of late outflow graft failure of the left ventricular assist device: a long-term follow-up.

Continuous flow left ventricular assist device (LVAD) outflow graft stenosis constitutes a severe complication. Treatment options include surgical pump exchange, transcatheter procedures, or systemic thrombolysis. We present a case of a spontaneous mechanical twisting of the outflow graft at two distinct points, which was treated by a two-step percutaneous stent implantation. Self-expanding stents were used during the first procedure. We also ensured distal bilateral percutaneous neuroprotection against cerebrovascular embolism. During the second, previously unpredicted procedure, we used balloon-expandable bare-metal stents to overcome the torque of the graft, because of their higher radial force. It was assumed that the external, self-expanding layer of the stent might protect the graft from the bare-metal stents. The effects of the treatment were monitored both clinically and through computed tomography angiography. The check-up 12 months later revealed nothing of note. Interventional transcatheter procedures are a safe treatment option for outflow graft stenosis, with good long-term effects. Both self-expanding and balloon-expandable stents can be used for transcatheter intervention. Both the incidence of the graft twisting and the radial force of the implanted stent seem to be critical and robust predictors of the long-term result of percutaneous therapy.

Lack of Relationship between Fibrosis-Related Biomarkers and Cardiac Magnetic Resonance-Assessed Replacement and Interstitial Fibrosis in Dilated Cardiomyopathy.

The relationship between circulating fibrosis-related molecules and magnetic resonance-assessed cardiac fibrosis in dilated cardiomyopathy (DCM) is poorly understood. To compare circulating biomarkers between DCM patients with high and low fibrosis burdens, we performed a prospective, single-center, observational study. The study population was composed of 100 DCM patients (87 male, mean age 45.2 ± 11.8 years, mean ejection fraction 29.7% ± 10.1%). Replacement fibrosis was quantified by means of late gadolinium enhancement (LGE), whereas interstitial fibrosis was assessed via extracellular volume (ECV). Plasma concentrations of cardiotrophin-1, growth differentiation factor-15, platelet-derived growth factor, procollagen I C-terminal propeptide, procollagen III N-terminal propeptide, and C-terminal telopeptide of type I collagen were measured. There were 44% patients with LGE and the median ECV was 27.7%. None of analyzed fibrosis serum biomarkers were associated with the LGE or ECV, whereas NT-proBNP was independently associated with both LGE and ECV, and troponin T was associated with ECV. None of the circulating fibrosis markers differentiated between DCM patients with and without replacement fibrosis, or patients stratified according to median ECV. However, cardiac-specific markers, such as NT-proBNP and hs-TnT, were associated with fibrosis. Levels of circulating markers of fibrosis seem to have no utility in the diagnosis and monitoring of cardiac fibrosis in DCM.

CT evaluation of aberrant right subclavian artery: anatomy and clinical implications.

PurposeThe aim of the study was to perform CT angiography-based evaluation of aberrant right subclavian artery prevalence, anatomy, and its influence on clinical symptoms. METHODS: A total of 6833 patients who underwent 64-slice or dual-source CT angiography and those who revealed aberrant right subclavian artery underwent evaluation of its anatomy and were interviewed for the presence of clinical symptoms. RESULTS: Aberrant right subclavian artery was found in 32 (0.47%) patients consisting of 13 males and 19 females, with mean age of 60.8±13.4 years. Among the interviewed 30 (94%) patients, oesophageal compression was observed in 14 cases (47%) and tracheal compression in three cases (10%). None of the patients underwent surgery related to aberrant right subclavian artery. Dysphagia was the most common clinical symptom in nine cases (30%), and in those patients the median distance between aberrant right subclavian artery and trachea was lower (4 mm) than in individuals without dysphagia (7.5 mm) (p = 0.009). The median lumen area of the aberrant right subclavian artery at the level of oesophagus was higher in patients with dysphagia (208 mm2) compared with individuals without dysphagia (108 mm2) (p = 0.01). CONCLUSIONS: Aberrant right subclavian artery is a rare occurring abnormality in CT angiography. In the evaluated adult population, the most common symptom was dysphagia, which occurred in patients with decreased distance between aberrant right subclavian artery and trachea and increased lumen area of the aberrant artery at the level of compressed oesophagus.

Effect of ventricular function and volumes on exercise capacity in adults with repaired Tetralogy of Fallot.

OBJECTIVES: Investigate the effects of left and right ventricular function and severity of pulmonary valve regurgitation, quantified by cardiac magnetic resonance (CMR), on exercise tolerance in adult patients who underwent ToF repair at a young age. METHODS: This is a retrospective cohort study of 52 patients after ToF surgery and 33 age- and sex-matched healthy volunteers. CMR and cardiopulmonary exercise testing (CPET) were performed on all patients; CPET was performed on control subjects. RESULTS: The main finding of CPET was a severe decrease in oxygen uptake at peak exercise VO2peak in TOF patients. The patients were characterized also by lower pulse O2peak and heart rate at peak exercise. Ejection fraction of the right and left ventricles was correlated (r=0,32; p=0,03). Left ventricle ejection fraction was negatively correlated with right ventricular volumes (r=-0,34; p=0,01) and right ventricular mass (r=-046; p<0,00). Right ventricular mass was positively correlated with left ventricular variables (left ventricle end diastolic volume, r=0,43; p=0,002; left ventricle end systolic volume, r=0,54; p<0,00) as was VO2peak: LVEDV (r=0,38; p=0,01); LVESV (r=0,33; p=0,03) and LV mass (r=0,42; p=0,006). CONCLUSION: Exercise intolerance in adults with repaired ToF is markedly depressed. The decreased exercise capacity is correlated with impaired RV function and may be associated also with LV dysfunction, which suggests right-to-left ventricular interaction.

Noninvasive techniques for the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery in adult patients.

Background Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly which requires surgical treatment, which improves left ventricular function and clinical outcomes. The definitive diagnosis of ALCAPA is based on coronary angiography, however, noninvasive visualization of the origin of the coronary artery is often helpful in the diagnosis of ALCAPA. Objectives The purpose of this study was to evaluate noninvasive techniques for the diagnosis of ALCAPA, and present the clinical outcomes in adults after surgical correction of ALCAPA. Methods Medical charts of five patients with ALCAPA treated at the John Paul II Hospital in Cracow between 2004 and 2012 were analyzed retrospectively. Noninvasive imaging techniques were used to visualize coronary vessels preoperatively. Patients were followed one year after the operation with echocardiograms and assessment of the New York Heart Association functional class. Results Computed tomography showed ALCAPA in four patients. Magnetic resonance imaging showed abnormal left ventricle remodeling suggestive of ischemia of the anterior cardiac wall. Myocardial perfusion imaging revealed ischemia of the middle and periapical segments of the anterior and anterolateral wall of the left ventricle in two patients. Postoperatively, patients had symptomatic improvement, but there was no statistically significant difference in mean ejection fraction. Conclusions Noninvasive techniques can be used to visualize the origin of the coronary arteries and characterize their morphology. Long-term follow-up of adult patients after surgical correction of ALCAPA is not well documented in the literature, but surgical treatment revealed beneficial short-term effects and symptoms relief.

Synchronous extraskeletal Ewing's sarcoma/PNET and gallbladder carcinoma: a case report and literature review.

Ewing's sarcoma (ES) and primitive neuroectodermal tumour (PNET) are now considered to be the same tumour and usually occur in long bones. Extraskeletal Ewing's sarcoma is an extremely rare neoplasm, accounting for 1% of soft tissue sarcomas, with most common location in the thorax. Gallbladder cancer (GBC) represents the most common type among the biliary tract cancers with a poor prognosis even among patients undergoing aggressive therapy. We present study of extraskeletal ES/PNET found in the hilus of the liver of an elderly, diagnosed one month prior with GBC woman. The patient underwent two cycles of chemotherapy SAIME/SAVAC for ES and thereafter was operated. During three-year follow-up no recurrence of ES/PNET has been reported. However, two years after chemotherapy the patient suffered a relapse of adenocarcinoma of the gallbladder and thus received palliative chemotherapy of gemcitabine and cisplatin. After 16 months of recurrence she died. To the best of our knowledge, this is the first case of ES/PNET located in the hilus of the liver and as a synchronous neoplasm.

Adenoid cystic carcinoma of the external auditory canal with metastases to lymph nodes and lungs--problematic diagnosis and treatment based on a case report.

Adenoid cystic carcinoma (ACC) of the external auditory canal (EAC) is an exceedingly rare tumor. Despite the slow growth it is characterized by a high malignancy and infiltration of surrounding tissue. Differential diagnosis may be especially difficult if the tumor appears in an atypical localization as it can present with non-specific features. The optimal treatment of this malignancy has not been fully established. We present a case report of a 55-year-old man with ACC of EAC metastasizing to the lymph nodes, lungs and vertebrae, with intracranial involvement. The patient was initially diagnosed with basal cell carcinoma (BCC) of EAC based on MR imaging examinations and excisional biopsy from EAC. Current information about nomenclature, epidemiology, characteristic features and treatment possibilities of ACC are presented and difficulties in making diagnosis are discussed. ACC should be considered among the malignant tumors of EAC. Its natural behavior is probably more unpredictable than commonly thought. Similar cases are rare and our knowledge about the tumor's specificity and prognosis is limited.

Anomalous origin of the coronary artery from the wrong coronary sinus evaluated with computed tomography: "high-risk" anatomy and its clinical relevance.

OBJECTIVE: The aim of the study was to assess coronary arteries arising from the wrong coronary sinus, including CT-evaluated high-risk anatomic features, clinical symptoms and cardiac events during follow-up. METHODS: A total of 7,115 patients scheduled for 64-slice or dual-source cardiac CT were screened for the presence of isolated anomalous origin of the coronary artery from the wrong coronary sinus. RESULTS: Anomalous origin of the coronary artery was found in 54 (0.76 %) patients (29 men, 25 women, mean age 60.9 ± 11.6 years). Sixteen (30 %) patients with abnormal right coronary origin (ARCA) more commonly had a slit-like orifice (15 vs. 3; p < 0.001), intramural course (15 vs. 3; p < 0.001) and interarterial course (11 vs. 0; p < 0.001) than 22 (41 %) and 13 (24 %) individuals with abnormal circumflex artery (ALCx) and left coronary artery (ALCA) origin, respectively. Patients with ALCA presented less frequently with chest pain than subjects with ARCA and ALCx (25 vs. 3; p = 0.03). Patients with ARCA tended to show higher occurrence of cardiac events in the follow-up than individuals with ALCA and ALCx (5 vs. 4; p = NS). CONCLUSIONS: High-risk anatomy features are most common in patients with ARCA and these patients also have higher prevalence of chest pain and cardiac events in the follow-up than individuals with ALCA and ALCx. KEY POINTS: • Multislice computed tomography enables detection and evaluation of the coronary artery anomalies. • Anomalous anatomy of the coronary artery potentially influences the prevalence of adverse events. • Adverse events tend to be most common in anomalous right coronary arteries.

[Cystic lesion in the topography of occluded aortocoronary saphenous vein bypass]. / Zmiana torbielowata w topografii niedroinego, zylnego pomostu aortalno-wiencowego.

Article presents a case of a 66-year old manwith a pericardial cyst, localized in tlhe topography of an occluded aorto-ccoronary saphenous vein graft. The lesion was revealed in computed tomogrnaphy and also evaluated in the subsequently performed magnetic resonance.