[Analysis of long-term prognosis and risk factors in patients with dilated cardiomyopathy].

Objective: To investigate the risk factors and long-term prognosis of major adverse cardiovascular events(MACEs) in patients with dilated cardiomyopathy (DCM). Methods: This study was a single-center retrospective cohort study. Clinical information from 300 patients with DCM hospitalized in Peking Union Medical College Hospital from April 2013 to April 2023 was collected. Based on echocardiography results, the patients were divided into two groups: isolated DCM and DCM with left ventricular non-compaction cardiomyopathy (LVNC). The MACEs, including major heart failure events, severe ventricular arrhythmias, and cardiovascular death, were recorded by outpatient or telephone follow-up. Univariate and multivariate Cox proportional hazard regression models were used to analyze the risk factors affecting the prognosis of patients with DCM. Kaplan-Meier curve and log-rank were used for survival analysis to compare the difference in the incidence of cardiovascular events between the two groups. Results: The included 300 DCM patients were (47.8±16.8) years old, with 197 males (65.7%), of which 237 (79.0%) were isolated DCM and 63 (21.0%) were DCM with LVNC. The follow-up time was 4.0 (1.9, 6.2) years. A total of 142 (47.3%) MACEs occurred, including 117 (39.0%) major heart failure events, 20 (6.7%) severe ventricular arrhythmia events, and 53 (17.7%) cardiovascular death events. Multivariate Cox proportional hazard regression analysis showed that increased left ventricular end-diastolic diameter (HR=1.21, 95%CI: 1.01-1.44, P=0.042), moderate or severe mitral regurgitation (HR=1.71, 95%CI: 1.19-2.47, P=0.004), increased ln (N-terminal pro-B-type natriuretic peptide) (HR=1.30, 95%CI: 1.10-1.54, P=0.002) were independent risk factors for dverse cardiovascular events in DCM patients, and angiotensin-converting enzyme inhibitor (ACEI)/angiotensin receptor blocker (ARB)/angiotensin receptor neprilysin inhibitor (ARNI) treatment (HR=0.45, 95%CI: 0.26-0.78, P=0.004) was independent protective factor. Kaplan-Meier survival analysis found no significant difference in the risk of MACEs between isolated DCM and DCM with LVNC (P=0.22). Similarly, there were no significant differences in the incidence of major heart failure, severe ventricular arrhythmia, and cardiovascular death between the two groups (all P>0.05). Conclusion: An increase in left ventricular end-diastolic diameter, moderate or severe mitral regurgitation, elevated N-terminal pro-B-type natriuretic peptide, and non use of ACEI/ARB/ARNI are independent predictors of cardiovascular events in DCM patients. There was no significant risk of MACEs in patients with isolated DCM and DCM with LVNC, and suggested that LVNC may be a unique phenotype and should be accurately managed in combination with genetic background.

Dilated cardiomyopathy due to hypocalcaemia: a case report.

BACKGROUND: Hypocalcaemia is a rare, but reversible, cause of dilated cardiomyopathy causing heart failure. Several case reports have been reported on reversible cardiomyopathy secondary to hypocalcaemia. CASE PRESENTATION: We report a case of 54-year-old female Sri Lankan patient who presented with shortness of breath and was diagnosed with heart failure with reduced ejection fraction due to dilated cardiomyopathy. The etiology for dilated cardiomyopathy was identified as hypocalcemic cardiomyopathy, secondary to primary hypoparathyroidism, which was successfully treated with calcium and vitamin D replacement therapy. CONCLUSION: This adds to literature of this rare cause of reversible cardiomyopathy secondary to hypocalcemia reported from the South Asian region of the world. This case highlights the impact of proper treatment improving the heart failure in patients with hypocalcemic cardiomyopathy.

Use of cardiac contractility modulation combined with left bundle branch pacing CRT-P in a female with a 22-year history of non-ischemic dilated cardiomyopathy: A case report.

Cardiac contractility modulation (CCM) is a novel device-based therapy used to treat patients with heart failure with reduced ejection fraction (HFrEF). In both randomized clinical trials and real-life studies, CCM has been shown to improve exercise tolerance and quality of life, reverse left ventricular remodeling, and reduce hospitalization in patients with HFrEF. In this case report, we describe for the first time the use of CCM combined with left bundle branch pacing (LBBP) cardiac resynchronization therapy pacemaker (CRT-P) implantation therapy in a female with a 22-year history of non-ischemic dilated cardiomyopathy. With the optimal medical therapy and cardiac resynchronization therapy (CRT) strategies, the patient's quality of life initially recovered to some extent, but began to deteriorate in the past year. Additionally, heart transplantation was not considered due to economic reasons and late stage systolic heart failure. This is the first case of CCM implantation in Fujian Province and the first report of a combined CCM and left bundle branch pacing CRT-P implantation strategy in a patient with non-ischemic etiology dilated cardiomyopathy in China.

Echocardiographic quantification of mitral apparatus morphology and dynamics in patients with dilated cardiomyopathy.

Mitral regurgitation is among the most common valvular heart diseases. Mitral regurgitation in patients with dilated cardiomyopathy is a complex pathology involving annular dilatation, papillary muscle displacement, systolic leaflet tethering, and left ventricular remodeling. Quantification of mitral apparatus damage in these patients is essential for successful interventional and surgical therapy. Mitral regurgitation in the presence of dilated cardiomyopathy is classified as Carpentier type IIIB, with restricted leaflet mobility as a standard feature. Echocardiography allows accurate evaluation of the complex anatomy and function of the mitral apparatus. Updated guidelines recommend two-dimensional followed by systematic three-dimensional echocardiographic evaluation in patients with mitral regurgitation. New three-dimensional echocardiographic software packages provide many parameters that help identify the precise morphology and function of the various components of the mitral apparatus, helping to determine the etiology of mitral regurgitation and evaluate disease severity. This review provides the first point-by-point approach to the assessment of all old and new echocardiographic methods, from the simplest to the most complex, used to examine the components of the mitral valve apparatus in patients with dilated cardiomyopathy. Although these parameters are still under research, this information will be helpful for establishing therapeutic procedures in a disease with a poor prognosis.

Deep learning-based prediction of major arrhythmic events in dilated cardiomyopathy: A proof of concept study.

Prediction of major arrhythmic events (MAEs) in dilated cardiomyopathy represents an unmet clinical goal. Computational models and artificial intelligence (AI) are new technological tools that could offer a significant improvement in our ability to predict MAEs. In this proof-of-concept study, we propose a deep learning (DL)-based model, which we termed Deep ARrhythmic Prevention in dilated cardiomyopathy (DARP-D), built using multidimensional cardiac magnetic resonance data (cine videos and hypervideos and LGE images and hyperimages) and clinical covariates, aimed at predicting and tracking an individual patient's risk curve of MAEs (including sudden cardiac death, cardiac arrest due to ventricular fibrillation, sustained ventricular tachycardia lasting ≥30 s or causing haemodynamic collapse in <30 s, appropriate implantable cardiac defibrillator intervention) over time. The model was trained and validated in 70% of a sample of 154 patients with dilated cardiomyopathy and tested in the remaining 30%. DARP-D achieved a 95% CI in Harrell's C concordance indices of 0.12-0.68 on the test set. We demonstrate that our DL approach is feasible and represents a novelty in the field of arrhythmic risk prediction in dilated cardiomyopathy, able to analyze cardiac motion, tissue characteristics, and baseline covariates to predict an individual patient's risk curve of major arrhythmic events. However, the low number of patients, MAEs and epoch of training make the model a promising prototype but not ready for clinical usage. Further research is needed to improve, stabilize and validate the performance of the DARP-D to convert it from an AI experiment to a daily used tool.

The impact of type 2 diabetes mellitus on the clinical profile, myocardial fibrosis, and prognosis in non-ischemic dilated cardiomyopathy: a prospective cohort study.

BACKGROUND: The impact of the coexistence of type 2 diabetes mellitus (T2DM) in patients with non-ischemic dilated cardiomyopathy (DCM) on clinical profiles, myocardial fibrosis, and outcomes remain incompletely understood. METHOD: A total of 1152 patients diagnosed with non-ischemic DCM were prospectively enrolled from June 2012 to October 2021 and categorized into T2DM and non-T2DM groups. Clinical characteristics, cardiac function, and myocardial fibrosis evaluated by CMR were compared between the two groups. The primary endpoint included both all-cause mortality and heart transplantation. Cause of mortality was classified into heart failure death, sudden cardiac death, and non-cardiac death. Cox regression analysis and Kaplan-Meier analysis were performed to identify the association between T2DM and clinical outcomes. Propensity score matching (PSM) cohort including 438 patients was analyzed to reduce the bias from confounding covariates. RESULTS: Among the 1152 included DCM patients, 155 (13%) patients had T2DM. Patients with T2DM were older (55 ± 12 vs. 47 ± 14 years, P < 0.001), had higher New York Heart Association (NYHA) functional class (P = 0.003), higher prevalence of hypertension (37% vs. 21%, P < 0.001), atrial fibrillation (31% vs. 16%, P < 0.001), lower left ventricular (LV) ejection fraction (EF) (23 ± 9% vs. 27 ± 12%, P < 0.001), higher late gadolinium enhancement (LGE) presence (55% vs. 45%, P = 0.02), and significantly elevated native T1 (1323 ± 81ms vs. 1305 ± 73ms, P = 0.01) and extracellular volume fraction (ECV) (32.7 ± 6.3% vs. 31.3 ± 5.9%, P = 0.01) values. After a median follow-up of 38 months (interquartile range: 20-57 months), 239 patients reached primary endpoint. Kaplan-Meier analysis showed that patients with T2DM had worse clinical outcomes compared with those without T2DM in the overall cohort (annual events rate: 10.2% vs. 5.7%, P < 0.001). T2DM was independently associated with an increased risk of primary endpoint in the overall (Hazard ratio [HR]: 1.61, 95% CI: 1.13-2.33, P = 0.01) and PSM (HR: 1.54, 95% CI: 1.05-2.24, P = 0.02) cohorts. Furthermore, T2DM was associated with a higher risk of heart failure death (P = 0.006) and non-cardiac death (P = 0.02), but not sudden cardiac death (P = 0.16). CONCLUSIONS: Patients with T2DM represented a more severe clinical profile and experienced more adverse outcomes compared to those without T2DM in a large DCM cohort. TRIAL REGISTRATION: Trial registration number: ChiCTR1800017058; URL: https://www. CLINICALTRIALS: gov .

[Development and clinical value of programmed ventricular stimulation in coronary artery disease and dilated cardiomyopathy]. / Entwicklung und Bedeutung der programmierten Ventrikelstimulation bei der koronaren Herzerkrankung und dilatativen Kardiomyopathie.

Programmed ventricular stimulation (PVS), a clinical tool introduced in the early 1980s, aims to prove the electrical vulnerability of the heart and, independent of spontaneous arrhythmia variability, to trigger arrhythmias under controlled conditions. A specific response is the inducibility of monomorphic sustained ventricular tachycardia. This depends on the underlying heart disease, e.g., only for coronary artery disease but not for nonischemic diseases. The value of pharmacologic arrhythmia control as serial electrical testing is uncertain. Up to now there seems to be no prognostic value of PVS concerning sudden cardiac death. PVS is used as a tool to monitor the results of ventricular tachycardia (VT)-catheter ablation in patients who were primarily inducible.

Development and validation of a risk model for intracardiac thrombosis in patients with dilated cardiomyopathy: a retrospective study.

Intracardiac thrombosis is a severe complication in patients with non-ischemic dilated cardiomyopathy. This study aims to develop and validate an individualized nomogram to evaluate the risk of intracardiac thrombosis in patients with non-ischemic dilated cardiomyopathy. This retrospective study included patients diagnosed with dilated cardiomyopathy at first admission. Clinical baseline characteristics were acquired from electronic medical record systems. Multiple methods were applied to screen the key variables and generate multiple different variable combinations. Multivariable logistic regression was used to build the models, and the optimal model was chosen by comparing the discrimination. Then we checked the performance of the model in different thrombus subgroups. Finally, the model was presented using a nomogram and evaluated from the perspectives of discrimination, calibration, and clinical usefulness. Internal validation was performed by extracting different proportions of data for Bootstrapping. Ultimately, 564 eligible patients were enrolled, 67 of whom developed an intracardiac thrombosis. Risk factors included d-dimer, white blood cell count, high-sensitivity C-reactive protein, pulse pressure, history of stroke, hematocrit, and NT-proBNP in the optimal model. The model had good discrimination and calibration, and the area under the curve (AUC) was 0.833 (0.782-0.884), and the model's performance in each subgroup was stable. Clinical decision curve analysis showed that the model had clinical application value when the high-risk threshold was between 2% and 78%. The AUC of interval validation (30% and 70% data resampling) was 0.844 (0.765-0.924) and 0.833 (0.775-0.891), respectively. This novel intracardiac thrombosis nomogram could be conveniently applied to facilitate the individual intracardiac thrombosis risk assessment in patients with non-ischemic dilated cardiomyopathy.

Differences in underlying cardiac substrate among S-ICD recipients and its impact on long-term device-related outcomes: Real-world insights from the iSUSI registry.

BACKGROUND: Outcome comparisons among subcutaneous implantable cardioverter-defibrillator (S-ICD) recipients with nonischemic cardiomyopathies are scarce. OBJECTIVE: The aim of this study was to evaluate differences in device-related outcomes among S-ICD recipients with different structural substrates. METHODS: Patients enrolled in the i-SUSI (International SUbcutaneouS Implantable cardioverter defibrillator registry) project were grouped according to the underlying substrate (ischemic vs nonischemic) and subgrouped into dilated cardiomyopathy, hypertrophic cardiomyopathy, Brugada syndrome (BrS), arrhythmogenic right ventricular cardiomyopathy (ARVC). The main outcome of our study was to compare the rates of appropriate and inappropriate shocks and device-related complications. RESULTS: Among 1698 patients, the most common underlying substrate was ischemic (31.7%), followed by dilated cardiomyopathy (20.5%), BrS (10.8%), hypertrophic cardiomyopathy (8.5%), and ARVC (4.4%). S-ICD for primary prevention was more common in the nonischemic cohort (70.9% vs 65.4%; P = .037). Over a median (interquartile range) follow-up of 26.5 (12.6-42.8) months, no differences were observed in appropriate shocks between ischemic and nonischemic patients (4.8%/y vs 3.9%/y; log-rank, P = .282). ARVC (9.0%/y; hazard ratio [HR] 2.492; P = .001) and BrS (1.8%/y; HR 0.396; P = .008) constituted the groups with the highest and lowest rates of appropriate shocks, respectively. Device-related complications did not differ between groups (ischemic: 6.4%/y vs nonischemic: 6.1%/y; log-rank, P = .666), nor among underlying substrates (log-rank, P = .089). Nonischemic patients experienced higher rates of inappropriate shocks than did ischemic S-ICD recipients (4.4%/y vs 3.0%/y; log-rank, P = .043), with patients with ARVC (9.9%/y; P = .001) having the highest risk, even after controlling for confounders (adjusted HR 2.243; confidence interval 1.338-4.267; P = .002). CONCLUSION: Most S-ICD recipients were primary prevention nonischemic cardiomyopathy patients. Among those, patients with ARVC tend to receive the most frequent appropriate and inappropriate shocks and patients with BrS the least frequent appropriate shocks.

Venous-arterial extracorporeal membrane oxygenation for psittacosis pneumonia complicated with cardiogenic shock: case report and literature review.

INTRODUCTION: Dilated cardiomyopathy (DCM) is characterized by the enlargement of the left ventricle or biventricular, accompanied by myocardial systolic dysfunction. Chlamydia psittacosis (CP) is a zoonotic pathogen, which can cause severe pneumonia, respiratory failure, and acute organ dysfunction. The deterioration of DCM caused by CP infection is extremely rare, and few cases of successful management were reported. CASE PRESENTATION: We reported a 67-year-old male patient with DCM and chronic heart failure. Who was admitted to ICU with severe pneumonia, acute hypoxemic respiratory failure, acute decompensated heart failure, arrhythmia, and cardiogenic shock. Mechanical ventilation (MV) and venous-arterial extracorporeal membrane oxygenation (VA-ECMO) were established for respiratory and circulatory support. Broncho alveolar lavage fluid(BALF)was collected for culture and metagenomics next-generation sequencing (mNGS) test. Repeated mNGS tests indicated the high possibility of CP pneumonia, thereafter, moxifloxacin and doxycycline were prescribed. After targeted antibiotics and organ support treatment, pneumonia, respiratory and circulatory failure were gradually resolved, patient was successfully weaned from MV and VA-ECMO. Finally, the patient was recovered and discharged alive. CONCLUSIONS: Severe respiratory and circulatory failure caused by CP infection in DCM patients is a rare life-threatening clinical condition. Early accurate diagnosis, targeted antibiotic therapy, coupled with extracorporeal life support posed positive impact on the patient's disease course and outcome.

Identification of patients with nonischemic dilated cardiomyopathy at risk of malignant ventricular arrhythmias: insights from cardiac magnetic resonance feature tracking.

BACKGROUND: Patients with nonischemic dilated cardiomyopathy (NIDCM) are prone to arrhythmias, and the cause of mortality in these patients is either end-organ dysfunction due to pump failure or malignant arrhythmia-related death. However, the identification of patients with NIDCM at risk of malignant ventricular arrhythmias (VAs) is challenging in clinical practice. The aim of this study was to evaluate whether cardiovascular magnetic resonance feature tracking (CMR-FT) could help in the identification of patients with NIDCM at risk of malignant VAs. METHODS: A total of 263 NIDCM patients who underwent CMR, 24-hour Holter electrocardiography (ECG) and inpatient ECG were retrospectively evaluated. The patients with NIDCM were allocated to two subgroups: NIDCM with VAs and NIDCM without VAs. From CMR-FT, the global peak radial strain (GPRS), global longitudinal strain (GPLS), and global peak circumferential strain (GPCS) were calculated from the left ventricle (LV) model. We investigated the possible predictors of NIDCM combined with VAs by univariate and multivariate logistic regression analyses. RESULTS: The percent LGE (15.51 ± 3.30 vs. 9.62 ± 2.18, P < 0.001) was higher in NIDCM patients with VAs than in NIDCM patients without VAs. Furthermore, the NIDCM patients complicated with VAs had significantly lower GPCS than the NIDCM patients without VAs (- 5.38 (- 7.50, - 4.22) vs.-9.22 (- 10.73, - 8.19), P < 0.01). Subgroup analysis based on LGE negativity showed that NIDCM patients complicated with VAs had significantly lower GPRS, GPCS, and GPLS than NIDCM patients without VAs (P < 0.05 for all). Multivariate analysis showed that both GPCS and %LGE were independent predictors of NIDCM combined with VAs. CONCLUSIONS: CMR global strain can be used to identify NIDCM patients complicated with VAs early, specifically when LGE is not present. GPCS < - 13.19% and %LGE > 10.37% are independent predictors of NIDCM combined with VAs.

Diabetes mellitus is associated to high-risk late gadolinium enhancement and worse outcomes in patients with nonischemic dilated cardiomyopathy.

BACKGROUND: Diabetes mellitus (DM) is associated with a worse prognosis in patients with heart failure. Our aim was to analyze the clinical and imaging features of patients with DM and their association with outcomes in comparison to nondiabetic patients in a cohort of patients with nonischemic dilated cardiomyopathy (DCM). METHODS: This is a prospective cohort study of patients with DCM evaluated in a tertiary care center from 2018 to 2021. Transthoracic echocardiography and cardiac magnetic resonance findings were assessed. A high-risk late gadolinium enhancement (LGE) pattern was defined as epicardial, transmural, or septal plus free-wall. The primary outcome was a composite of heart failure hospitalizations and all-cause mortality. Multivariable analyses were performed to evaluate the impact of DM on outcomes. RESULTS: We studied 192 patients, of which 51 (26.6%) had DM. The median left ventricular ejection fraction was 30%, and 106 (55.2%) had LGE. No significant differences were found in systolic function parameters between patients with and without DM. E/e values were higher (15 vs. 11.9, p = 0.025), and both LGE (68.6% vs. 50.4%; p = 0.025) and a high-risk LGE pattern (31.4% vs. 18.5%; p = 0.047) were more frequently found in patients with DM. The primary outcome occurred more frequently in diabetic patients (41.2% vs. 23.6%, p = 0.017). DM was an independent predictor of outcomes (OR 2.01; p = 0.049) and of LGE presence (OR 2.15; p = 0.048) in the multivariable analysis. Patients with both DM and LGE had the highest risk of events (HR 3.1; p = 0.003). CONCLUSION: DM is related to a higher presence of LGE in DCM patients and is an independent predictor of outcomes. Patients with DM and LGE had a threefold risk of events. A multimodality imaging approach allows better risk stratification of these patients and may influence therapeutic options.

Iron deficiency and anemia in pediatric dilated cardiomyopathy are associated with clinical, biochemical, and hematological markers of severe disease and adverse outcomes.

BACKGROUND: There is limited evidence regarding the prevalence and impact of iron deficiency (ID) in children with dilated cardiomyopathy (DCM). METHODS: Retrospective single-center review of all children between 2010 and 2020 with a diagnosis of DCM and complete iron studies. ID was defined as ≥2 of ferritin <20 µg/liter, iron <9 µmol/liter, transferrin >3 g/liter, or transferrin saturation (TSat) <15%. Clinical and laboratory indices and freedom from a composite adverse event (CAE) of mechanical circulatory support (MCS), heart transplant, or death were compared between children with and without ID. RESULTS: Of 138 patients with DCM, 47 had available iron studies. Twenty-nine (62%) were iron deficient. Children with ID were more likely to be receiving inotropes (17, 59%, p = 0.005) or invasive/noninvasive ventilation (13, 45%, p = 0.016) than those who were iron replete. They had a higher incidence of anemia (22, 76%, p = 0.004) and higher NT-proBNP (1,590 pmol/liter, IQR 456-3,447, p = 0.001). Children with ID had significantly less freedom from the CAE at 1-year (54% ± 10%), 2-years (45 ± 10), and 5-years (37% ± 11%) than those without (p = 0.011). ID and anemia were the only significant predictors of the CAE on univariate Cox regression. CONCLUSIONS: ID is highly prevalent in children with DCM. Iron studies are undermeasured in clinical practice, but ID is associated with severe heart failure (HF) and an increased risk of the CAE. The need for iron replacement therapy should be considered in children who present in HF with DCM.