Diagnostic Performance of On-Site Computed Tomography Derived Fractional Flow Reserve on Non-Culprit Coronary Lesions in Patients with Acute Coronary Syndrome.

The role of coronary computed tomography angiography (CCTA) derived fractional flow reserve (CT-FFR) in the assessment of non-culprit lesions (NCL) in patients with acute coronary syndrome (ACS) is debated. In this prospective clinical study, a total of 68 ACS patients with 89 moderate (30−70% diameter stenosis) NCLs were enrolled to evaluate the diagnostic accuracy of on-site CT-FFR compared to invasive fractional flow reserve (FFRi) and dobutamine stress echocardiography (DSE) as reference standards. CT-FFR and FFRi values ≤0.80, as well as new or worsening wall motion abnormality in ≥2 contiguous segments on the supplying area of an NCL on DSE, were considered positive for ischemia. Sensitivity, specificity, positive, and negative predictive value of CT-FFR relative to FFRi and DSE were 51%, 89%, 75%, and 74% and 37%, 77%, 42%, and 74%, respectively. CT-FFR value (ß = 0.334, p < 0.001) and CT-FFR drop from proximal to distal measuring point [(CT-FFR drop), ß = −0.289, p = 0.002)] were independent predictors of FFRi value in multivariate linear regression analysis. Based on comparing their receiver operating characteristics area under the curve (AUC) values, CT-FFR value and CT-FFR drop provided better discriminatory power than CCTA-based minimal lumen diameter stenosis to distinguish between an NCL with positive and negative FFRi [0.77 (95% Confidence Intervals, CI: 0.67−0.86) and 0.77 (CI: 0.67−0.86) vs. 0.63 (CI: 0.52−0.73), p = 0.029 and p = 0.043, respectively]. Neither CT-FFR value nor CT-FFR drop was predictive of regional wall motion score index at peak stress (ß = −0.440, p = 0.441 and ß = 0.403, p = 0.494) or was able to confirm ischemia on the territory of an NCL revealed by DSE (AUC = 0.54, CI: 0.43−0.64 and AUC = 0.55, CI: 0.44−0.65, respectively). In conclusion, on-site CT-FFR is superior to conventional CCTA-based anatomical analysis in the assessment of moderate NCLs; however, its diagnostic capacity is not sufficient to make it a gatekeeper to invasive functional evaluation. Moreover, based on its comparison with DSE, CT-FFR might not yield any information on the microvascular dysfunction in the territory of an NCL.

Predictors and neurological consequences of periprocedural cerebrovascular events following transcatheter aortic valve implantation with self-expanding valves.

Aims: To evaluate the patient- and procedure-related predictors of transcatheter aortic-valve implantation (TAVI)-associated ischemic brain lesions and to assess the effect of silent cerebral ischemic lesions (SCIL) on neurocognitive function. Methods and results: We investigated 113 consecutive patients with severe aortic stenosis who underwent brain magnetic resonance imaging (MRI) within a week following TAVI. To assess periprocedural cerebral ischemic lesions, diffusion-weighted MRI was utilized. We used multivariate linear regression to identify the independent predictors of TAVI-related ischemic lesion volume (ILV) and periprocedural stroke. Neurocognitive evaluation was performed before and following TAVI at 6-month and one-year follow-up. Following TAVI, a total of 944 new cerebral ischemic lesions were detected in 104 patients (92%). The median ILV was 257 µl (interquartile range [IQR]:97.1-718.8µl) with a median lesion number of 6/patient [IQR:2-10]. The majority of ischemic lesions were clinically silent (95%), while 5% of the lesions induced a stroke, which was confirmed by MRI. Predilatation (ß = 1.13[95%CI:0.32-1.93], p = 0.01) and the number of valve positioning attempts during implantation (ß = 0.28[95%CI:0.06-0.50], p = 0.02) increased the log-transformed total ILV. Predilatation (OR = 12.04[95%CI:1.46-99.07], p = 0.02) and alternative access routes (OR = 7.84[95%CI:1.01-61.07], p = 0.02) were associated with stroke after adjustments for comorbidities and periprocedural factors. The presence of SCILs were not associated with a change in neurocognitive function that remained stable during the one-year follow-up. Conclusion: While periprocedural ischemic lesions are frequent, most of them are clinically silent and might not impact the patients' neurocognitive function. The number of valve positioning attempts, predilatation, and alternative access routes should be taken into consideration during TAVI to reduce the ILV and risk for stroke.

Subclinical leaflet thrombosis after transcatheter aortic valve implantation is associated with silent brain injury on brain magnetic resonance imaging.

AIMS: Whether hypoattenuated leaflet thickening (HALT) following transcatheter aortic valve implantation (TAVI) carries a risk of subclinical brain injury (SBI) is unknown. We investigated whether HALT is associated with SBI detected on magnetic resonance imaging (MRI), and whether post-TAVI SBI impacts the patients' cognition and outcome. METHODS AND RESULTS: We prospectively enrolled 153 patients (age: 78.1 ± 6.3 years; female 44%) who underwent TAVI. Brain MRI was performed shortly post-TAVI and 6 months later to assess the occurrence of acute silent cerebral ischaemic lesions (SCIL) and chronic white matter hyperintensities (WMH). HALT was screened by cardiac computed tomography (CT) angiography (CTA) 6 months post-TAVI. Neurocognitive evaluation was performed before, shortly after and 6 months following TAVI. At 6 months, 115 patients had diagnostic CTA and 10 had HALT. HALT status, baseline, and follow-up MRIs were available in 91 cases. At 6 months, new SCIL was evident in 16%, new WMH in 66%. New WMH was more frequent (100 vs. 62%; P = 0.047) with higher median volume (319 vs. 50 mm3; P = 0.039) among HALT-patients. In uni- and multivariate analysis, HALT was associated with new WMH volume (beta: 0.72; 95%CI: 0.2-1.39; P = 0.009). The patients' cognitive trajectory from pre-TAVI to 6 months showed significant association with the 6-month SCIL volume (beta: -4.69; 95%CI: -9.13 to 0.27; P = 0.038), but was not related to the presence or volume of new WMH. During a 3.1-year follow-up, neither HALT [hazard ratio (HR): 0.86; 95%CI: 0.202-3.687; P = 0.84], nor the related WMH burden (HR: 1.09; 95%CI: 0.701-1.680; P = 0.71) was related with increased mortality. CONCLUSIONS: At 6 months post-TAVI, HALT was linked with greater WMH burden, but did not carry an increased risk of cognitive decline or mortality over a 3.1-year follow-up (NCT02826200).

Microstructural alterations measured by diffusion tensor imaging following transcatheter aortic valve replacement and their association with cerebral ischemic injury and cognitive function - a prospective study.

PURPOSE: We assessed diffusion tensor imaging (DTI) metric changes of the corpus callosum and cingulum correlated to postprocedural ischemic lesion load (ILL) and cognitive performance in transcatheter aortic valve replacement (TAVR). METHODS: TAVR subjects had DTI post-TAVR (≤ 8 days) and at 6 months (78 participants, males 56%, age 78.8 years ± 6.3) and four neurocognitive tests (pre-TAVR, post-TAVR, 6 months, 1 year). DTI metrics (fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), radial diffusivity (RD)) were calculated for 7 regions: corpus callosum (genu, body, splenium) and cingulum (cingulate gyrus, parahippocampal cingulum bilaterally). DTI metrics post-TAVR and at 6 months were compared with Student's t-test (p < 0.0071) and ANOVA covarying for sex, ILL (p < 0.05) with post hoc analysis of ILL groups (p < 0.0167). Repeated-measures linear mixed-effect model (p < 0.05) was performed to investigate the effect of time and ILL on cognition. RESULTS: At 6 months, significant decrease of the following DTI metrics was detected: AD (genu, body, splenium, right parahippocampal cingulum: p ≤ 0.0046); MD (body, both cingulate gyri: p ≤ 0.0050); RD (left cingulate gyrus: p = 0.0021); FA (splenium: p < 0.0001). ANOVA confirmed significant effect of female sex on AD + MD reduction (body, right cingulate gyrus) and AD reduction (left cingulate gyrus) (p ≤ 0.0254). Significant negative effect of ILL on some DTI metric changes was found (AD + MD-body: p ≤ 0.0050; MD-left cingulate gyrus: p = 0.0087). Cognitive performance remained stable with significant negative correlation of ILL and retrograde memory and visual scores (p ≤ 0.0483). CONCLUSION: Significant effect of TAVR on cerebral microstructural integrity was found with reduced diffusivities opposite to the trends reported in various neurodegenerative conditions/ageing, notably in women and lower ILL, and with preserved/improved cognition. TRIAL REGISTRATION NUMBER: NCT02826200 at ClinicalTrials.gov; date of registration: 07. July 2016.

The Predictive Value of Left Atrial Strain Following Transcatheter Aortic Valve Implantation on Anatomical and Functional Reverse Remodeling in a Multi-Modality Study.

Introduction: Transcatheter aortic valve implantation (TAVI) can improve left ventricular (LV) mechanics and survival. Data on the predictive value of left atrial (LA) strain following TAVI are scarce. We aimed to evaluate the association of LA strain measured shortly post-TAVI with functional and anatomical reverse remodeling of the LA and LV, and its association with mortality. Methods: We prospectively investigated 90 patients who underwent TAVI. Transthoracic echocardiography including strain analysis was performed shortly after TAVI and repeated 6 months later. CT angiography (CTA) was performed for pre-TAVI planning and 6 months post-TAVI. Speckle tracking echocardiography was used to determine LA peak reservoir strain (LASr) and LV global longitudinal strain (LV-GL), LA volume index (LAVi) was measured by TTE. LV mass index (LVMi) was calculated using CTA images. LA reverse remodeling was based on LASr and LAVi changes, whereas LV reverse remodeling was defined as an improvement in LV-GLS or a reduction of LVMi. The association of severely reduced LASr (<20%) at baseline with changes (Δ) in LASr, LAVi, LV-GLS and LVMi were analyzed using linear regression, and Cox proportional hazard model for mortality. Results: Mean LASr and LV-GLS were 17.7 ± 8.4 and -15.3 ± 3.4% at baseline and 20.2 ± 10.2 and -16.6 ± 4.0% at follow-up (p = 0.024 and p < 0.001, respectively). Severely reduced LASr at baseline was associated with more pronounced ΔLASr (ß = 5.24, p = 0.025) and LVMi reduction on follow-up (ß = 5.78, p = 0.036), however, the majority of the patients had <20% LASr on follow-up (44.4%). Also, ΔLASr was associated with ΔLV-GLS (adjusted ß = 2.10, p < 0.001). No significant difference in survival was found between patients with baseline severely reduced LASr (<20%) and higher LASr (≥20%) (p = 0.054). Conclusion: LV reverse remodeling based on LVMi was present even in patients with severely reduced LASr following TAVI, although extensive LA damage based on LA strain was demonstrated by its limited improvement over time. Clinical Trial Registration: (ClinicalTrials.gov number: NCT02826200).

Multidisciplinary management of patients affected with pseudoxanthoma elasticum / Pseudoxanthoma elasticumban szenvedo betegek multidiszciplináris ellátása

Pseudoxanthoma elasticum (PXE, OMIM # 264800) is an autosomal recessive, multisystemic disorder, associated with mutations of the ABCC6 gene. Ectopic mineralization is in the background of the clinical manifestations of the disease. Calcium-salt crystals are deposited primarily in the skin, in the Bruch membrane of the eyes, and in the vascular endothelium. Thus, in addition to the skin lesions, visual impairment and cardiovascular involvement also occur. Clinical symptoms show varying severity and display heterogeneous appearance. The identification of the phenotype and care of the patients require a multidisciplinary perspective based on the collaboration of a dermatologist, ophthalmologist, cardiologist, and clinical geneticist. The aim of our work is to describe the development of symptoms of the disease, in order to facilitate the diagnosis. In addition, we aim to draw attention to the importance of early diagnosis of pseudoxanthoma elasticum, and to present modern diagnostic methods. Considering the development of severe systemic complications, the early diagnosis with the collaboration between related specialists is crucial to provide optimal clinical care and management of the patients.

"Turtle cage" method in the cardiac surgical treatment of constrictive pericarditis ­ our short-term results / "Turtle cage" módszer a pericarditis constrictiva szívsebészeti kezelésében - rövid távú eredményeink.

Összefoglaló. Bevezetés: A pericarditis constrictiva egy krónikus gyulladásos folyamat révén kialakuló betegség, melynek során a pericardium elveszíti rugalmasságát, gátolja a szív muködését, végso soron szívelégtelenséghez vezet. Egyetlen oki terápiája sebészi. A mutéti megoldásként legelterjedtebben alkalmazott teljes pericardiectomia hosszú idotartamú mutét, amely akár 18%-os mutéti kockázattal járhat, és amelyhez az esetek jelentos részében szívmotor alkalmazása szükséges. Célkituzés: Egy, az irodalomból már ismert, de csak ritkán és a legtöbbször csak a hagyományos pericardiectomia kiegészítéseként alkalmazott mutéti eljárás, a "turtle cage" pericardiectomia hatásosságának, eredményeinek, lehetséges elonyeinek vizsgálata. Módszer: 2008 és 2021 között Klinikánkon 33 "turtle cage" mutétet végeztünk pericarditis constrictiva miatt. A posztoperatív 30 napos idoszak eredményeit több, a nemzetközi irodalomban megjelent közlemény adataival hasonlítottuk össze. Eredmények: Az intraoperatív kép alapján minden esetben sikeres volt a beavatkozás, a 33 beteg egyikénél sem volt szükség szívmotor alkalmazására (0%), szemben a vizsgált közleményekkel. A 33 beavatkozás során 1 beteget veszítettünk el (3%), valamint 1 páciensnél volt szükség vérzés miatti reoperációra (3%), 4 betegnél dialízisre (12,1%). Ezen eredményeink összevethetok a nagy esetszámot felvonultató közleményekkel, és szignifikánsan jobbak az egyik megjelenített európai centrum eredményeinél. Következtetés: Az általunk alkalmazott "turtle cage" pericardiectomia önmagában is megfelelo eljárás a pericarditis constrictiva szívsebészeti kezelésére. Alkalmazásával minimalizálható a szívmotor használatának szükségessége, ezáltal a mutéti kockázat. Eredményeink a technikának köszönhetoen még a nagy esetszámú, sok tapasztalattal rendelkezo centrumok eredményeivel is összevethetok, azokkal megegyezok. Orv Hetil. 2022; 163(10): 393-399. INTRODUCTION: Constrictive pericarditis is a disease caused by a chronic inflammatory process, which is characterized by the pericardium's loss of flexibility, inhibiting the function of the heart, ultimately causing heart failure. The only definitive therapy is surgical. Total pericardiectomy, which is the most common surgical approach, is a lengthy procedure with up to 18% operative risk, and it often requires the use of cardiopulmonary bypass. OBJECTIVE: The evaluation of the effectiveness, results and possible advantages of a surgical technique, "turtle cage" pericardiectomy, which is described in the literature, although rarely used, mainly in addition to conventional pericardiectomy. METHOD: Between 2008 and 2021, we performed 33 "turtle cage" procedures on patients with constrictive pericarditis in our Institute. We compared the results of the 30-day postoperative period with internationally published data from multiple sources. RESULTS: Based on intraoperative findings, the procedure was successful in all cases, there were no instances when the use of cardiopulmonary bypass was required (0%). During the 33 procedures, we lost 1 patient (3%), reoperation was necessary for postoperative bleeding in 1 case (3%), and postoperative dialysis was necessary in 4 cases (12.1%). These results are comparable to those published by high-volume centres, and significantly better than those of one of the European centres published. CONCLUSION: The "turtle cage" pericardiectomy, as performed in our Institute, is suitable for the treatment of constrictive pericarditis on its own. With its use, we were able to minimize the use of cardiopulmonary bypass and the operative risk. Our results with this technique are comparable to those of the high-volume, highly experienced centres. Orv Hetil. 2022; 163(10): 393-399.

The extravascular implantable cardioverter-defibrillator: characterization of anatomical parameters impacting substernal implantation and defibrillation efficacy.

AIMS: The aim of this study is to provide a thorough, quantified assessment of the substernal space as the site of extravascular implantable cardioverter-defibrillator (ICD) lead placement using computed tomography (CT) scans and summarizing adverse events and defibrillation efficacy across anatomical findings. Subcutaneous ICDs are an alternative to transvenous defibrillators but have limitations related to ICD lead distance from the heart. An alternative extravascular system with substernal lead placement has the potential to provide defibrillation at lower energy and pacing therapies from a single device. METHODS AND RESULTS: A multi-centre, non-randomized, retrospective analysis of 45 patient CT scans quantitatively and qualitatively assessing bony, cardiac, vascular, and other organ structures from two human clinical studies with substernal lead placement. Univariate logistic regression was used to evaluate 15 anatomical parameters for impact on defibrillation outcome and adjusted for multiple comparisons. Adverse events were summarized. Substernal implantation was attempted or completed in 45 patients. Defibrillation testing was successful in 37 of 41 subjects (90%) using ≥10 J safety margin. There were two intra-procedural adverse events in one patient, including reaction to anaesthesia and an episode of transient atrial fibrillation during ventricular fibrillation induction. Anatomical factors associated with defibrillation failure included large rib cage width, myocardium extending very posteriorly, and a low heart position in the chest (P-values <0.05), though not significant adjusting for multiple comparisons. CONCLUSION: Retrospective analysis demonstrates the ability to implant within the substernal space with low intra-procedural adverse events and high defibrillation efficacy despite a wide range of anatomical variability.

Patients with Moderate Non-Culprit Coronary Lesions of Recent Acute Coronary Syndrome.

Fractional flow reserve (FFR) measurement was compared to dobutamine stress echocardiography (DSE) instable angina (SA) with stable coronary lesion (s) (SCL (s) ) in a few trials; however, similar comparisons in patients with acute coronary syndrome (ACS) with non-culprit lesion (s) (NCL (s) ) are lacking. Our objectives were to prospectively evaluate the diagnostic performance of FFR with two different cutoff values (< 0.80 and < 0.75) relative to DSE in moderate (30%-70% diameter stenosis) NCLs (ACS group) and to compare these observations with those measured in SCLs (SA group). One hundred seventy-five consecutive patients with SA (n = 86) and ACS (n = 89) with 225 coronary lesions (109 SCLs and 116 NCLs) were enrolled. In contrast to the ACS cohort in SA patients, normal DSE was associated with higher FFR values compared to those with abnormal DSE (P = 0.051 versus P = 0.006). In addition, in the SA group, a significant correlation was observed between DSE (regional wall motion score index at peak stress) and FFR (r = -0.290; P = 0.002), whereas a similar association was absent (r = -0.029; P = 0.760) among ACS patients. In the SA group, decreasing the FFR cutoff value (< 0.80 versus < 0.75) improved the concordance of FFR with DSE (70.6% versus 81.7%) without altering its discriminatory power (area under the curve; 0.68 versus 0.63; P = 0.369), whereas in the ACS group, concordance remained similar (69.0% versus 71.6%) and discriminatory power decreased (0.62 versus 0.51; P = 0.049), respectively. In conclusion, lesion-specific FFR assessment may have different relevance in patients with moderate NCLs than in patients with SCLs.

Quantification of hypo-attenuated leaflet thickening after transcatheter aortic valve implantation: clinical relevance of hypo-attenuated leaflet thickening volume.

AIMS: Our aim was to establish an objective, quantitative methodology for volumetric hypo-attenuated leaflet thickening (HALT) diagnosis and evaluate its clinical significance. METHODS AND RESULTS: We prospectively enrolled 144 patients who underwent transcatheter aortic valve implantation (TAVI) between 2011 and 2016. At inclusion, cardiac computed tomography angiography (CTA), transthoracic echocardiography, and brain magnetic resonance imaging (MRI) were performed. We quantified HALT on CTA datasets by segmenting the inner volume of TAVI frame at the level of leaflets and extracted voxels between a threshold of -200 to 200 HU based on prior recommendation. The median HALT volume was 72 [inter-quartile range (IQR): 1-154] mm3 (intra- and inter-reader agreement: intra-class correlation coefficient = 0.92 and 0.94, respectively) and 79% (n = 87/111) of the patients had HALT >0 mm3. In multivariate linear regression, oral anti-coagulation (ß: -0.32; 95% CI: -0.62 to -0.01; P = 0.004) and history of myocardial infarction (ß: 0.32; 95% CI: 0.01-0.63; P = 0.043) were associated with HALT quantity. Log-transformed HALT volume was associated with elevated (>13 mmHg) aortic mean gradient (AMG, OR: 12.85; 95% CI: 1.96-152.93; P = 0.021) and moderate-to-severe valvular degeneration (AMG ≥ 20 mmHg or ΔAMG ≥ 10 mmHg; OR: 10.56; 95% CI: 1.44-148.71; P = 0.046) but did not predict ischaemic brain lesions on MRI or all-cause death after a median follow-up of 29 (IQR: 11-29) months (all P > 0.05). CONCLUSION: Through systematic analysis of asymptomatic patients with TAVI, an objective and reproducible methodology was feasible for volumetric measurement of HALT. Anti-coagulation might have a protective effect against HALT. Ischaemic brain lesions and all-cause death were not associated with HALT; nevertheless, it might deteriorate prosthesis function due to its association with elevated AMG. CLINICAL TRIAL REGISTRATION: http//:www.ClinicalTrials.gov; NCT02826200.

Subclinical leaflet thrombosis is associated with impaired reverse remodelling after transcatheter aortic valve implantation.

AIMS: Cardiac CT is increasingly applied for planning and follow-up of transcatheter aortic valve implantation (TAVI). However, there are no data available on reverse remodelling after TAVI assessed by CT. Therefore, we aimed to evaluate the predictors and the prognostic value of left ventricular (LV) reverse remodelling following TAVI using CT angiography. METHODS AND RESULTS: We investigated 117 patients with severe, symptomatic aortic stenosis (AS) who underwent CT scanning before and after TAVI procedure with a mean follow-up time of 2.6 years after TAVI. We found a significant reduction in LV mass (LVM) and LVM indexed to body surface area comparing pre- vs. post-TAVI images: 180.5 ± 53.0 vs. 137.1 ± 44.8 g and 99.7 ± 25.4 vs. 75.4 ± 19.9 g/m2, respectively, both P < 0.001. Subclinical leaflet thrombosis (SLT) was detected in 25.6% (30/117) patients. More than 20% reduction in LVM was defined as reverse remodelling and was detected in 62.4% (73/117) of the patients. SLT, change in mean pressure gradient on echocardiography and prior myocardial infarction was independently associated with LV reverse remodelling after adjusting for age, gender, and traditional risk factors (hypertension, body mass index, diabetes mellitus, and hyperlipidaemia): OR = 0.27, P = 0.022 for SLT and OR = 0.22, P = 0.006 for prior myocardial infarction, OR = 1.51, P = 0.004 for 10 mmHg change in mean pressure gradient. Reverse remodelling was independently associated with favourable outcomes (HR = 0.23; P = 0.019). CONCLUSION: TAVI resulted in a significant LVM regression on CT. The presence of SLT showed an inverse association with LV reverse remodelling and thus it may hinder the beneficial LV structural changes. Reverse remodelling was associated with improved long-term prognosis.

Left ventricular and atrial strain imaging with cardiac computed tomography: Validation against echocardiography.

BACKGROUND: Data on left ventricular (LV) deformation imaging using CT angiography (CTA) are scarce and the feasibility of atrial deformation analysis by CT has not been addressed. We aimed to compare 2D echocardiographic and CT derived LV and left atrial (LA) global longitudinal strain (GLS) obtained by using a novel feature tracking algorithm in patients following transcatheter aortic valve implantation. METHODS: Twenty-eight patients were included who underwent retrospectively-gated 256-slice CTA and speckle-tracking echocardiography (STE) on the same day. CT datasets in 10% increments were reconstructed throughout the cardiac cycle. LV GLS and LA global peak reservoir strain (LA GS) was measured. RESULTS: Median absolute values for LV GLS were 19.9 [14.8-22.4] vs. 19.9 [16.8-24.7], as measured by CT vs STE, respectively (p = 0.017). We found good inter-modality correlation for LV GLS (ρ = 0.78, p < 0.05) with a mean bias of -1.6. Regarding atrial measurements, the median LA GS was 19.0 [13.5-27.3] for CT vs. 28.0 [17.5-32.6] for STE (p < 0.001) with a mean bias of -5.6 between CT and STE and a correlation coefficient of ρ = 0.87, p < 0.001. CT measurements were highly reproducible: intra-observer intra-class correlation coefficient was 0.96 for LV GLS and 0.95 for LA GS. CONCLUSION: We detected good correlation between CTA and echocardiography-based LV and LA longitudinal strain parameters. CTA provides accurate strain measurements with high reproducibility. Feature tracking-based deformation analysis could provide a clinically important addition to CT examinations by complementing anatomical information with functional data.

Coexistence of aortic valve stenosis and cardiac amyloidosis: echocardiographic and clinical significance.

BACKGROUND: Left ventricular hypertrophy and diastolic dysfunction are common echocardiographic features of both aortic valve stenosis (AS) and cardiac amyloidosis (CA). These two different entities therefore may mask each other. From recent years, there is a growing body of evidence about the relatively high incidence of wild-type transthyretin (wtTTR) amyloidosis in AS, but there are scarce data on the prevalence of AS in CA, particularly in AL-type amyloidosis. The echocardiographic approach to these patients is not obvious, and not evidence based. We aimed to study the prevalence, severity, and type of AS in patients with CA and also to evaluate the potential of echocardiography in the diagnostic process. METHODS: Between January 2009 and January 2019, we retrospectively analyzed the clinical and echocardiographic data, and the echocardiographic work up of 55 consecutive CA patients. RESULTS: 80% of our CA patients had AL amyloidosis. We identified 5 patients (9%) with moderate to severe AS: two with moderate AS and three with low-flow, low-grade AS (LFLG AS). Further analysis of the latter three patients with dobutamine stress echocardiography revealed pseudo-severe LFLG AS in two, and true-severe AS in one patient. CONCLUSION: The prevalence of moderate to severe AS is 9% in our population of CA patients, the majority of whom have AL amyloidosis. Dobutamine echocardiography seems to be appropriate for the further characterization of patients with LFLG AS, even with normal ejection fraction.

Contemporary Presentation and Management of Valvular Heart Disease: The EURObservational Research Programme Valvular Heart Disease II Survey.

BACKGROUND: Valvular heart disease (VHD) is an important cause of mortality and morbidity and has been subject to important changes in management. The VHD II survey was designed by the EURObservational Research Programme of the European Society of Cardiology to analyze actual management of VHD and to compare practice with guidelines. METHODS: Patients with severe native VHD or previous valvular intervention were enrolled prospectively across 28 countries over a 3-month period in 2017. Indications for intervention were considered concordant if the intervention was performed or scheduled in symptomatic patients, corresponding to Class I recommendations specified in the 2012 European Society of Cardiology and in the 2014 American Heart Association/American College of Cardiology VHD guidelines. RESULTS: A total of 7247 patients (4483 hospitalized, 2764 outpatients) were included in 222 centers. Median age was 71 years (interquartile range, 62-80 years); 1917 patients (26.5%) were ≥80 years; and 3416 were female (47.1%). Severe native VHD was present in 5219 patients (72.0%): aortic stenosis in 2152 (41.2% of native VHD), aortic regurgitation in 279 (5.3%), mitral stenosis in 234 (4.5%), mitral regurgitation in 1114 (21.3%; primary in 746 and secondary in 368), multiple left-sided VHD in 1297 (24.9%), and right-sided VHD in 143 (2.7%). Two thousand twenty-eight patients (28.0%) had undergone previous valvular intervention. Intervention was performed in 37.0% and scheduled in 26.8% of patients with native VHD. The decision for intervention was concordant with Class I recommendations in symptomatic patients with severe single left-sided native VHD in 79.4% (95% CI, 77.1-81.6) for aortic stenosis, 77.6% (95% CI, 69.9-84.0) for aortic regurgitation, 68.5% (95% CI, 60.8-75.4) for mitral stenosis, and 71.0% (95% CI, 66.4-75.3) for primary mitral regurgitation. Valvular interventions were performed in 2150 patients during the survey; of them, 47.8% of patients with single left-sided native VHD were in New York Heart Association class III or IV. Transcatheter procedures were performed in 38.7% of patients with aortic stenosis and 16.7% of those with mitral regurgitation. CONCLUSIONS: Despite good concordance between Class I recommendations and practice in patients with aortic VHD, the suboptimal number in mitral VHD and late referral for valvular interventions suggest the need to improve further guideline implementation.

Long-term single-centre large volume experience with transseptal endocardial left ventricular lead implantation.

AIMS: The aim of our study was to investigate the long-term efficacy and safety of transseptal endocardial left ventricular lead implantation (TELVLI). METHODS AND RESULTS: Transseptal endocardial left ventricular lead implantation was performed in 54 patients (44 men, median age 69, New York Heart Association III-IV stage) between 2007 and 2017 in a single centre. In 36 cases, the transseptal puncture (TP) was performed via the femoral vein, and in 18 cases, the TP and also the left ventricular (LV) lead placement were performed via the subclavian vein. An electrophysiological deflectable catheter was used to reach the LV wall through the dilated TP hole. The LV lead implantation was successful in all patients. A total of 54 patients were followed up for a median of 29 months [interquartile range (IQR) 8-40 months], the maximum follow-up time was 94 months. Significant improvement in the LV ejection fraction was observed at the 3-month visit, from the median of 27% (IQR 25-34%) to 33% (IQR 32-44%), P < 0.05. Early lead dislocation was observed in three cases (5%), reposition was performed using the original puncture site in all. The patients were maintained on anticoagulation therapy with a target international normalized ratio between 2.5 and 3.5. Four thromboembolic events were noticed during follow-up. A total of 27 patients died, with a median survival of 15 months (IQR 6-40). CONCLUSION: The TELVLI is an effective approach for cardiac resynchronization therapy (CRT) however it is associated with a substantial thromboembolic risk (7%).

The demanding grey zone: Sport indices by cardiac magnetic resonance imaging differentiate hypertrophic cardiomyopathy from athlete's heart.

BACKGROUND: We aimed to characterize gender specific left ventricular hypertrophy using a novel, accurate and less time demanding cardiac magnetic resonance (CMR) quantification method to differentiate physiological hypertrophy and hypertrophic cardiomyopathy based on a large population of highly trained athletes and hypertrophic cardiomyopathy patients. METHODS: Elite athletes (n = 150,>18 training hours/week), HCM patients (n = 194) and athletes with hypertrophic cardiomyopathy (n = 10) were examined by CMR. CMR based sport indices such as maximal end-diastolic wall thickness to left ventricular end-diastolic volume index ratio (EDWT/LVEDVi) and left ventricular mass to left ventricular end-diastolic volume ratio (LVM/LVEDV) were calculated, established using both conventional and threshold-based quantification method. RESULTS: Whereas 47.5% of male athletes, only 4.1% of female athletes were in the grey zone of hypertrophy (EDWT 13-16mm). EDWT/LVEDVi discriminated between physiological and pathological left ventricular hypertrophy with excellent diagnostic accuracy (AUCCQ:0.998, AUCTQ:0.999). Cut-off value for LVM/LVEDVCQ<0.82 mm×m2/ml and for EDWT/LVEDViTQ<1.27 discriminated between physiological and pathological left ventricular hypertrophy with a sensitivity of 77.8% and 89.2%, a specificity of 86.7% and 91.3%, respectively. LVM/LVEDV evaluated using threshold-based quantification performed significantly better than conventional quantification even in the male subgroup with EDWT between 13-16mm (p<0.001). CONCLUSIONS: Almost 50% of male highly trained athletes can reach EDWT of 13 mm. CMR based sport indices provide an important tool to distinguish hypertrophic cardiomyopathy from athlete's heart, especially in highly trained athletes in the grey zone of hypertrophy.

Pregnant intravenous drug user tricuspid valve infective endocarditis treated with a successful simultaneous valve replacement and Cesarean section.

A 30-year-old female patient known to be an intravenous drug user (IVDU) was admitted to Bajcsy-Zsilinszky Hospital Cardiology Intensive Care Unit at 29-week gestation with severe sepsis and right heart failure. She had methicillin-sensitive Staphylococcus aureus on blood culture. Echocardiography confirmed the diagnosis of tricuspid valve infective endocarditis (IE). She had acute deterioration and hemodynamic instability for which an emergency tricuspid valve replacement (TVR) with a simultaneous Cesarean section (CS) was performed simultaneously. Medical management is the standard treatment in IE of IVDU pregnant patients, but in case of life-threatening complications, emergency TVR and CS are to be considered. This is the first reported case of IVDU IE treated with simultaneous TVR and CS.

Quality of life measured with EuroQol-five dimensions questionnaire predicts long-term mortality, response, and reverse remodelling in cardiac resynchronization therapy patients.

Aims: There are previous studies on quality of life (QoL) in cardiac resynchronization therapy (CRT) patients; however, there are no data with the short EuroQol-five dimensions (EQ-5D) questionnaire predicting outcomes. We aimed to assess the predictive role of baseline QoL and QoL change at 6 months after CRT with EQ-5D on 5-year mortality and response. Methods and results: In our prospective follow-up study, 130 heart failure (HF) patients undergoing CRT were enrolled. Clinical evaluation, echocardiography, and EQ-5D were performed at baseline and at 6 months of follow-up, continued to 5 years. Primary endpoint was all-cause mortality at 5 years. Secondary endpoints were (i) clinical response with at least one class improvement in New York Heart Association without HF hospitalization and (ii) reverse remodelling with 15% reduction in left ventricular end-systolic volume at 6 months. Fifty-four (41.5%) patients died during 5 years, 85 (65.3%) clinical responders were identified, and 63 patients (48.5%) had reverse remodelling. Baseline issues with mobility were associated with lower response [odds ratio (OR) 0.36, 95% confidence interval (CI) 0.16-0.84; P = 0.018]. Lack of reverse remodelling correlated with self-care issues at baseline (OR 0.10, 95% CI 0.01-0.94; P = 0.04). Furthermore, self-care difficulties [hazard ratio (HR) 2.39, 95% CI 1.17-4.86; P = 0.01) or more anxiety (HR 1.51, 95% CI 1.00-2.26; P = 0.04) predicted worse long-term survival. At 6 months, mobility (HR 3.95, 95% CI 1.89-8.20; P < 0.001), self-care (HR 7.69, 95% CI 2.23-25.9; P = 0.001), or ≥ 10% visual analogue scale (VAS) (HR 2.24, 95% CI 1.27-3.94; P = 0.005) improvement anticipated better survival at 5 years. Conclusion: EuroQol-five dimension is a simple method assessing QoL in CRT population. Mobility issues at baseline are associated with lower clinical response, whereas self-care issues predict lack of reverse remodelling. Problems with mobility or anxiety before CRT and persistent issues with mobility, self-care, and VAS scale at 6 months predict adverse outcome.

The impact of cardiac resynchronization therapy on routine laboratory parameters.

BACKGROUND: Cardiac resynchronization therapy (CRT) in chronic heart failure has been shown to improve mortality and morbidity. However, comprehensive data are not available as concerns how circulating biomarkers reflecting different organ functions, such as serum uric acid, blood urea nitrogen (BUN), albumin, cholesterol, or various liver enzymes, change over time as a consequence of CRT. The aim of this prospective study was to overview these possible changes. METHODS: A total of 20 routine laboratory parameters were measured in 122 control subjects and in 129 patients with chronic heart failure before CRT, 6 months, and 2 years later. RESULTS: The levels of serum uric acid [before: 432 (331-516) mmol/L, 6-month: 372 (304-452) mmol/L, 2-year: 340 (290-433) mmol/L; p < 0.001] and BUN [8.3 (6.4-11.5) mmol/L, 8.0 (6.3-11.1) mmol/L, 6.8 (5.0-9.7) mmol/L; p < 0.001) reduced statistically significant. Total bilirubin underwent reduction [16 (11-23) µmol/L, 11 (7-14) µmol/L, 8 (7-13) µmol/L; p < 0.001], while albumin increased [45 (43-48) g/L, 46 (44-48) g/L, 46 (43-48) g/L; p = 0.04]. Cholesterol concentrations elevated [4.3 (3.6-5.0) mmol/L, 4.5 (3.8-5.1) mmol/L, 4.6 (3.8-5.4) mmol/L; p < 0.001] and glucose decreased [6.2 (5.6-7.2) mmol/L, 5.9 (5.1-6.7) mmol/L, 5.7 (5.1-6.8) mmol/L; p < 0.001]. CONCLUSIONS: CRT influences the levels of routinely used biomarkers suggesting improvements in renal function, liver capacity, and metabolic changes. These changes could mirror the multiorgan improvement after CRT.

Three-dimensional dynamic morphology of the mitral valve in different forms of mitral valve prolapse - potential implications for annuloplasty ring selection.

BACKGROUND: Real-time three-dimensional transesophageal echocardiography has increased our understanding of the distinct pathomechanisms underlying functional, ischaemic or degenerative mitral regurgitation. However, potential differences in dynamic morphology between the subtypes of degenerative mitral prolapse have scarcely been investigated. METHODS: In order to compare the dynamic behavior of the different phenotypes of degenerative mitral valve prolapse, real-time three-dimensional transesophageal echocardiography recordings of 77 subjects, 27 with Barlow disease (BD), 32 with Fibroelastic deficiency (FED) and 18 normal controls (NC) were analysed. RESULTS: Geometric annular and valvular parameters of the myxomatous patients were significantly larger compared to controls (BD vs. FED vs. NC 3D annular area: 15 ± 2.8 vs. 13.3 ± 2.4 vs. 10.6 ± 2.3cm(2), all p < 0.01). Beside similar ellipticity, BD annuli were significantly flatter compared to FED. Myxomatous annuli appeared less dynamic than normals, with decreased overall 3D area change, however only the BD group differed from NC significantly (BD vs. FED vs. NC normalized 3D area change 4.40 vs. 6.81 vs. 9.69 %; BD vs. NC p = 0.000; FED vs. NC p = not significant, BD vs. FED p = 0.025). CONCLUSION: BD and FED differ not only in terms of valve morphology, but also annular dynamics. Both pathologies are characterized by annular dilatation. However, in BD the annulus is remarkably flattened and hypodynamic, whereas in FED its saddle-shape and contractile function is relatively preserved. These features might influence the choice of repair technique and the selection of annuloplasty ring.