Purpose
Hyponatremia occurs in about 30% of patients with pneumonia, including those with SARS-CoV-2 (COVID-19) infection. Hyponatremia predicts a worse outcome in several pathologic conditions and ...in COVID-19 has been associated with a higher risk of non-invasive ventilation, ICU transfer and death. The main objective of this study was to determine whether early hyponatremia is also a predictor of long-term sequelae at follow-up.
Methods
In this observational study, we collected 6-month follow-up data from 189 laboratory-confirmed COVID-19 patients previously admitted to a University Hospital. About 25% of the patients (
n
= 47) had hyponatremia at the time of hospital admission.
Results
Serum Na
+
was significantly increased in the whole group of 189 patients at 6 months, compared to the value at hospital admission (141.4 ± 2.2 vs 137 ± 3.5 mEq/L,
p
< 0.001). In addition, IL-6 levels decreased and the PaO
2
/FiO
2
increased. Accordingly, pulmonary involvement, evaluated at the chest X-ray by the RALE score, decreased. However, in patients with hyponatremia at hospital admission, higher levels of LDH, fibrinogen, troponin T and NT-ProBNP were detected at follow-up, compared to patients with normonatremia at admission. In addition, hyponatremia at admission was associated with worse echocardiography parameters related to right ventricular function, together with a higher RALE score.
Conclusion
These results suggest that early hyponatremia in COVID-19 patients is associated with the presence of laboratory and imaging parameters indicating a greater pulmonary and right-sided heart involvement at follow-up.
Abstract
Background
Anderson-Fabry disease (AFD) is a rare genetic lysosomal storage disorder which often goes unnoticed until symptom onset requires aggressive treatment. Prompt diagnosis remains ...crucial. Dedicated multidisciplinary centres are a remarkable opportunity to develop early detection strategies and appropriate management.
Purpose
To describe the long-term outcomes of patients diagnosed with AFD followed at a Cardiomyopathy Referral Centre according to baseline phenotype (clinical involvement vs sub-clinical involvement).
Methods
All consecutive patients with AFD visited at our Cardiomyopathy Unit from 1989 to 2020 with >1-year follow-up were retrospectively reviewed. Clinical involvement was defined by one among left ventricular hypertrophy (LVH)>15mm, presence of conduction blocks or cardiac implantable electronic devices (CIED), atrial fibrillation, kidney disease (CKD), stroke or transient ischemic attack (TIA). The primary outcome was disease progression, defined as de novo CIED implantation, increased or de novo LVH, de novo Stroke/TIA, or progression of CKD. Of 110 patients diagnosed with AFD, 86 (78%) with >1-year follow-up were selected.
Results
Clinical involvement was present in 60 (70%) patients. Age at diagnosis was similar between patients with clinical and subclinical phenotype (42+17 vs 39+15, p=0.277). Patients with clinical involvement were more frequently men (N=25 42% vs 4 15%, p=0.025) and probands (p=0.01). Overall, 1-organ involvement was present in 31 (52%) patients, 2-organ involvement in 24 (40%) patients and 3-organ in 5 (8%). A total of 46 (77%) patients were referred to enzyme replacement therapy (ERT): 52% received agalsidase α, 26% agalsidase β, and 22% migalastat. Among those with a clinical involvement not on ERT, 9 (15%) were scheduled for ERT initiation, 3 (5%) were considered old for ERT, 1 (1.5%) refused ERT and 1 (1.5%) had an allergic reaction to ERT.
At 7 3–12 years follow up, both study cohorts manifested signs and symptoms of disease progression: N=28 (47%) vs N=4 (15%), p=0.01, in patients with vs. without baseline clinical involvement, respectively. The main causes for diseases progression were increase (28%) or de novo LVH (13%), CKD (7%) and CIED implantation (5%). At Cox multivariable analysis, however, after adjustment for age at diagnosis, late onset phenotype and gender, the impact of clinical vs subliclinical involvement on disease progression was lost (Hazard Ratio 0.92, 95% C.I. 0.30–2.81).
Conclusion
Clinical involvement in AFD is frequent, irrespective of age at diagnosis, being present in more than 1-in-2 patients at baseline. Prompt referral to dedicated centres is warranted for appropriate care as the disease may progress in both patients with and without initial overt clinical phenotype despite optimal medical management.
Funding Acknowledgement
Type of funding sources: None.
Abstract
Background
Excess force generation during myocardial contraction represents a cardinal feature of hypertrophic cardiomyopathy (HCM).
Purpose
To evaluate the anatomical, functional and ...prognostic correlates of left ventricular (LV) force in HCM.
Methods
We prospectively recruited a consecutive sample of 408 HCM patients with LV ejection fraction (EF) >50%, referred for baseline transthoracic echocardiography in 2 primary HCM centers in Hungary and Italy between 1999 and 2021. LV force was calculated as LV outflow tract gradient+systolic blood pressure/LV end-systolic volume. Patients were followed for a median of 107 months (IQ range, 58–158 months), the study endpoint was all-cause mortality.
Results
Mean LV force was 6.0±4.6 mm Hg/ml. Receiver-operating characteristic analysis identified 7.5 mm Hg/ml the best cut-off value to predict mortality. LV force >7.5 mm Hg/ml was present in 86 patients (21%), more frequently in women (58 vs 27%, p<0.0001), more often in patients with diabetes (16 vs 6%, p=0.003), beta-blocker (81 vs 58%, p<0.0001), calcium channel-blocker (19 vs 9%, p=0.012) and diuretic therapy (19 vs 8%, p=0.004), compared to patients with LV force ≤7.5 mm Hg/ml. Patients with excess LV force had more advanced NYHA class (1.8±0.7 vs 1.4±0.7, p=0.0001), greater left atrial diameter (LAd), 46.7±6.6 vs 43.0±7.2 mm, <0.0001, LV maximal wall thickness (23.2±5.5 vs 21.6±5.5 mm p=0.01), LV EF (74.6±6.4 vs 67.0±6.8%, <0.0001), mitral regurgitation grade (1.2±0.7 vs 0.7±0.7, p<0.0001) and E/e' (10.3±5.0 vs 7.8±5.0, p=0.005). During follow-up 43 deaths occurred. All-cause death was more frequent in patients with excess LV force (21 vs 8%, p<0.0001). At multivariable Cox regression analysis, excess LV force was an independent predictor of mortality (HR 2.9, 95% CI 1.14–7.26, p=0.025) independent of age (HR 1.03, 95% CI 1.00–1.05, p=0.022) and LAd (HR 1.07, 95% CI 1.02–1.14, p=0.005).
Conclusion
LV force with a threshold of 7.5 mm Hg/ml, independently predicts adverse outcome in patients with HCM and preserved systolic function. Excess LV force generation is associated with female sex, diabetes, NYHA class, medications, LAd, LV wall thickness, EF, mitral regurgitation grade and E/e'.
Funding Acknowledgement
Type of funding sources: None.
Abstract
Background
Myocardial fibrosis (MF) is a common endpoint in the majority of pathological mechanisms affecting cardiac muscle, ultimately resulting in cardiac failure. Cardiovascular magnetic ...resonance (CMR) with gadolinium-based contrast agent and T1 mapping technique is able to deliver a non-invasive quantification of the extracellular volume (ECV) expansion. Recently, ECV estimation with cardiac computed tomography (ECV-CCT) has been validated in the setting of cardiac amyloidosis, showing good agreement with ECV-CMR. However, no evidence is available with last generation single source single energy CT scanner in the clinical context of newly diagnosed left ventricular dysfunction. Therefore, the aim of this study is to test the diagnostic accuracy of ECV-CCT for the detection of MF in patients with recent diagnosis of reduced left ventricle ejection fraction (LVEF), having ECV-CMR as reference technique.
Methods
A consecutive cohort of 41 patients with newly diagnosed left ventricle dysfunction (LVEF < 50%) was enrolled. Time interval of maximum 30 days was present between CCT and CMR. Myocardial segment evaluability with each technique, agreement between ECV-CMR and ECV-CCT, regression analysis and Bland-Altman analysis were performed.
Results
Age of enrolled patients was 62 ± 11 years, and mean LVEF at CMR was 35.2 ± 10.7%. Mean total amount of administrated iodinated contrast was 112.6 ± 22.2 ml, while overall radiation exposure for ECV estimation was2.1 ± 1.1 mSv. Out of 656 myocardial segments available, 656 (100%) segments were evaluable at CCT while 640 (97.6%) were evaluable at CMR. ECV-CCT demonstrated slightly lower values compared to ECV-CMR both at global and region-based comparison (all segments, 33.1 ± 8.5% vs 35.5 ± 10.7%, p < 0.001). At regression analysis, strong correlations were described both at global and region-based analysis (all segments, r = 0.877, 95% CI: 0.840 to 0.914). On Bland-Altman analysis, bias between ECV-CMR and ECV-CCT for global analysis was 2.4 (95% CI: -7.8 to 12.7).
Conclusions
ECV estimation with last-generation single source single energy CT scanner is feasible and accurate. Integration of ECV measurement in comprehensive CCT evaluation of patients with newly diagnosed left ventricular dysfunction can be performed with a small increase in overall radiation exposure.
Abstract
Background
Non-invasive techniques have evolved as "gatekeepers" to invasive coronary angiography (ICA) for symptomatic patients with suspected or known coronary artery disease (CAD). ...Evaluation of myocardial ischemia with functional tests represents a milestone in CAD detection with proved diagnostic and prognostic power. Coronary computed tomography angiography (CCTA), a non-invasive anatomical assessment, intrinsically lacks physiologic data to categorize the downstream hemodynamic significance of lesions. Stress CT perfusion (stress-CTP) is a recently evolved imaging modality able to assess inducible myocardial perfusion defects. The aim of this study is to compare resources and outcomes Impact of combined CCTA+stress-CTP versus stress cardiovascular magnetic resonance (stress-CMR) in consecutive symptomatic patients with suspected CAD and intermediate to high pre-test likelihood of disease or known CAD or previous history of revascularization.
Methods
624 symptomatic patients with intermediate to high risk pre-test likelihood for CAD or previous history of revascularization referred to our hospital for clinically indicated CCTA+stress-CTP or stress-CMR were enrolled. Stress-CTP scans were performed in 223 patients using 256-row whole heart-coverage scanner, static protocol acquisition and vasodilation induced by adenosine. 401 patients with clinically indicated stress-CMR were evaluated in a 1.5-T scanner after vasodilatation induced with dipyridamole. Patient follow-up was performed at 1 year after index test performance. Endpoints were as follow: 1) all cardiac events as a combined endpoint of revascularization, non-fatal MI and death; 2) hard cardiac events as combined endpoint of non-fatal MI and death.
Results
Stress-CMR group showed lower age, higher prevalence of male gender and higher prevalence of previous revascularization, while CCTA+CTP group showed higher prevalence of family history of CAD and statin use. CCTA was defined positive for obstructive disease in 62% of patients while the addition of CTP on top of CCTA reduced the number of positive patients to 46%. Stress-CMR resulted positive in 23% of subjects. Patients who underwent CCTA+CTP underwent more revascularization (29% versus 7%, p: 0.001) while no differences were found in terms of non-fatal MI and death between the two strategies. According to the predefined endpoints, CCTA+CTP group showed higher rate of all cardiac events (29% vs 8%, p: 0.001) and lower rate of hard cardiac events (0.4% vs 3%, p: 0.033), respectively. Stratifying the baseline characteristics by all cardiac events and hard cardiac events, left ventricle volume and index test strategy predicted all cardiac events while only index test strategy predicted hard cardiac events.
Conclusions
The use of CCTA+CTP strategy was associated with higher referral to revascularization but with a protective trend in terms of hard cardiac events as compared to the usual strategy with lone functional evaluation.
Abstract
Background
Hypertrophic Cardiomyopathy (HCM) is the most common genetic cardiomyopathy. However, few studies have systematically investigated the clinical course of pregnancy in HCM.
Purpose
...To determine whether pregnancy is well tolerated in HCM.
Methods
Women consecutively referred to our Tertiary Clinic for Cardiomyopathies from 1969 to
2019 were retrospectively reviewed. Only women with complete data regarding pregnancy and with a follow up (FU)>1 year were included in the study. Overall, of the 647 women followed at our center, 378 (58%) fulfilled our inclusion criteria. Demographic, clinical and instrumental records were retrieved. The peripartum period was defined as the timeframe from −1 to 6 months after delivery.
Results
There were 433 pregnancies in 239 (63%) women with 132 (62%) having >1 pregnancy. By contrast, 139 (37%) reported no pregnancy or miscarriages: in 6 cases pregnancy was discouraged due to advanced disease stage. Twenty-eight (12%) women had 39 pregnancies after HCM diagnosis and were followed by the obstetrics department: this subset was significantly younger at diagnosis (age at diagnosis: 21 13–29 vs 56 47–66 vs 45 24–62 years, p<0.001, in women with a pregnancy after diagnosis vs women diagnosed after the pregnancy vs women with no pregnancy, respectively). Instrumental characteristics were comparable among women. Thirty percent presented with obstructive physiology at baseline. Among the 39 pregnancies in women who had a pregnancy after the diagnosis, there were 3 reported episodes of paroxysmal atrial fibrillation, one sustained ventricular tachycardia with pulse and three episodes of non-sustained ventricular tachycardia in the peripartum period. In this cohort, prevalence of intra-uterine growth delay and miscarriage was 8%. Only 3 women experienced a worsening clinical profile requiring hospitalization during the peripartum period: 2 were hospitalized for acute heart failure (AHF) and 1 was experienced a resuscitated cardiac arrest. Of note, 2/3 of patients were carriers of a (likely)pathogenic troponin mutation.
Long-term (FU: 5±3 years), nulligravida women were more symptomatic at last evaluation (NYHA III/IV: 25 vs 17, p<0.05), reported a higher incidence of ICD appropriate shocks (26 vs 12%p=0.02) but had similar rates of heart transplant (2.1 vs 0.5%, p=0.143) and episodes of AHF (12 vs 14%, p=0.193). Eighteen patients (8.2%) died: incidence of cardiovascular mortality was 4.8%, with a lower rate in patients who reported a pregnancy (0.8%/year vs 2.8%/year, p=0.01).
Conclusions
Women with HCM tolerate pregnancy well. Rare complications occurred in the peripartum period which were manageable. In the long-term, pregnancy, even when multiple, did not influence the long-term course of the disease nor its outcome. Strategies to support appropriate counselling and antenatal care should be implemented to identify those at greater risk of disease progression.
Funding Acknowledgement
Type of funding sources: None.
Abstract
Funding Acknowledgements
Type of funding sources: None.
Background
Aortic valve neocuspidalization (Av-Neo) aims to replace the three aortic cusps with autologous pericardium pre-treated ...with glutaraldehyde and is becoming a valid alternative to traditional aortic valve replacement (AVR). 4D-flow cardiac magnetic resonance (4D-CMR) imaging of patients allows the derivation of shear stress on the aortic wall (WSS). Different studies support the presence of a link between increased WSS at 4D-CMR and the presence of histological alterations of the aortic wall.
Purpose
To compare the WSS of the ascending aorta in patients who underwent Av-Neo vs AVR with biological prostheses.
Methods
We performed 4D-flow CMR on 20 patients who underwent AV-Neo (Group A) or AVR (Group B), 10 patients for each group.
Results
The WSS in the Ozaki group was significantly lower in the proximal and distal aortic outer curvature compared to AVR patients (p 0.0179 and 0.0412, respectively, Figure 1 and 2). WSS levels remained significantly lower at the proximal aorta’s outer curvature segment in the Av-Neo population, also after adjusting the WSS for the ejection fraction and the LVOT-Aorta angle (p 0.02).
Conclusions
Av-neo hemodynamical features showed to be non-inferior to the ones provided by the commercially available bioprosthetic valves. Av-Neo procedures may be considered in patients affected by collagenopathy and other predisposing risk factors for the development of ascending aortic aneurism and aortic dissection other than classical AVR.
Abstract
Background
Patients with hypertrophic cardiomyopathy (HCM) are at increased risk of atrial fibrillation (AF) and stroke, especially at an advanced disease stage. To date, however, the ...incidence and factors associated with cardioembolic events in HCM patients without AF remain unresolved.
Purpose
To determine the incidence of stroke in HCM patients in whom cardiac rhythm was monitored with a cardiac implantable electronic device (CIED). The association of stroke with left atrial (LA) enlargement was also examined.
Methods
Retrospective cohort study in an outpatient clinic in a tertiary HCM Referral Center.
All consecutive patients diagnosed with HCM and referred for CIED implantation with >16 years at diagnosis and >1 year follow-up post CIED implantation were reviewed. Severe LA dilatation was defined as a LA dimension (LAD) of >48mm at echocardiogram. Based on CIED monitoring, patients were classified as: Pre-existing AF (diagnosed with AF prior to CIED); De novo AF (diagnosed with AF after CIED implantation); Sinus Rhythm (SR): no episodes of AF. Incidence of stroke after CIED implantation was the primary outcome.
Results
A total of 185 patients (57% men, age: 54±17 years) were implanted with a CIED and were included. Pre-existing AF was present in 72 (36%) patients and de novo AF in 24 (13%); whereas 89 (48%) remained in SR. After 5 2–9 years, stroke was reported in 19 (10.3%) patients: 7 occurred in patients with pre-existing AF (1.1%/year), 3 in patients with de novo AF (2.2%/year), and 9 in patients with SR (2.3%/year). No difference was captured by CHA2DS2-VASc score among rhythm categories. Patients with AF had larger LAD at baseline. Among patients in SR, those with a LAD>48mm had the greatest risk of stroke (4.8%/year vs 0.5%/year, p<0.01; Hazard Ratio HR: 8.56, 95% C.I. 2.03–36.15). At Cox multivariable regression analysis, LA (HR: 1.104, 95%C.I. 1.039–1.173, p=0.001) and AF (HR: 0.310, 95% C.I. 0.102–0.939, p=0.038) were associated with incident stroke.
Conclusions
In HCM patients with CIED long-term monitoring and no prior history of AF, stroke rates were similar in those with de novo AF or stable sinus rhythm. CHA2DS2-VASc considerably underestimated risk, whereas severe LA dilatation was a powerful predictor of risk, irrespective of AF.
Funding Acknowledgement
Type of funding sources: None.
Abstract Background Cardiac amyloidosis (CA) is an increasingly diagnosed disease sharing several phenotypical features with aortic stenosis (AS). Purpose As diagnosing the two diseases has crucial ...prognostic and therapeutic implications, this study aims to identify a set Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation of stable and discriminative radiomic features derived from cardiac computed tomography (CT) to differentiate them. Methods 21 patients with cardiac amyloidosis (CA) and 44 with aortic stenosis (AS) were randomly extracted from our database. All patients underwent CT scan. After image preprocessing, 107 radiomics features pertaining to shape and size, first-order statistics and textural classes, were extracted on the LV wall 3D-volume. Radiomic feature selection was based on stability, non-redundancy and relevance based on the Wilcoxon test followed by LASSO algorithm. All these steps were performed on the training set and then applied on the validation and test set. A nested cross-validation, composed of a 7-fold outer cross-validation and a 7-fold inner cross-validation, was performed, using five classifiers (k-nearest neighbors, support vector classifier, decision tree, logistic regression and gradient boosting). Four additional ML models were implemented including: I) age and sex; II) age, sex, BMI, LVEDVi and LVESVi, LV EF and IVS thickness; III) radiomic features, age, and sex; IV) radiomic features and variables included in II). Results Feature selection steps reduced the number of features to 10. As logistic regression showed the best performances in the validation set, it was selected for test set prediction obtaining an AUC of 0.92, sensitivity and specificity of 0.857 and 0.864 respectively. ROC curves for the test set, were computed using radiomic features (Figure 1a) and/or clinical variables (Figure 1b). The greatest performances were observed in model IV (AUC=0.96). Conclusions The application of radiomics shows promising results in distinguishing left ventricle hypertrophy caused by CA from AS and might be used as a noninvasive tool able to support clinical decision making.
Abstract Objective Mortality rates are 2- to 4-fold higher in people with type 1 diabetes mellitus (T1DM) than in the general population. Cardiovascular disease (CVD) is the main cause of such excess ...mortality, with CVD events occurring over a decade earlier than expected. However, while epidemiological studies on CVD and type 2 diabetes mellitus are numerous, less information is available on the prevalence of CVD in patients with T1DM. The primary aim of the study was to evaluate the prevalence of CAD at coronary CT angiography (CCTA) in consecutive T1DM patients referred to our diabetic center and to assess the characteristics of coronary plaques in this population. Materials and Methods We prospectively enrolled 109 consecutive T1DM patients referred to our diabetic unit center in CARDT1 study. Patients underwent a non-contrast CT for calculation of Calcium Score and a CCTA. Patients were stratified into three risk groups according to the STENO T1 RISK ENGINE and the ESC score. After the finding of CCTA patients were also divided into three groups: normal (no coronary plaques), non-obstructive CAD (lesions ,50%), and obstructive CAD (lesions >50%). The number of segments with any obstructive plaque also was recorded. Each coronary segment was evaluated for the presence of calcified and mixed plaques. Results The analytic study population consisted of 88 patients. Fourty-seven were male (53%) and the mean age was 46.42±13.09 years old with a mean duration of diabetes of 24 years. According to the Steno Type 1 Risk Engine, the mean estimated risk of CVD events over 5 years was 5.1% and 10% over 10 years. Thirty-five patients (48%) and 15 (20%) were classified as low and high Steno Risk, respectively. Moreover 45 (51%) patients have completely normal coronary arteries, 32 (36%) non obstructive CAD and 11 (12%) obstructive CAD. In patients with obstructive CAD the duration of diabetes was significantly higher than in those with no CAD and not obstructive CAD (40 years vs 17 years and 27 years, respectively, p<0.0001). Eighty-three% of low risk Steno Score T1DM have normal coronary arteries while 21% of high risk Steno score patients had obstructive CAD and 14% of high Steno risk have no plaque at CTCA. The number of coronary segments diseased was 1.5 (median) in non-obstructive CAD patients and 4 in obstructive CAD patients (p<0.0001). Among 118 coronary segments diseased, in 51 segments there was a calcified disease and in 65 there was a non prevalent calcified atherosclerotic disease Conclusions CCTA is able to restratify the risk of CAD in T1DM according to the presence or absence of CAD. In contrast with T2 diabetic patients who have a prevalent calcified and diffuse disease, T1DM patients have a more focal and fibrolipidic pattern of CAD.
