Materiały źródłowe

• #1 Fifteen-year mortality and prognostic factors in patients with dilated cardiomyopathy: persistent standardized application of drug therapy and strengthened management may bring about encouraging change in an aging society

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9170907/

  There is scarce data on the long-term mortality and associated prognostic factors in patients with dilated cardiomyopathy (DCM). […] The independent risk factors for the 5-year mortality were age 70 years old (OR = 5.45, P = 0.006), systolic blood pressure (SBP) 120 mmHg (OR = 3.63, P = 0.004), 6-minute walk distance (6MWD) 450 m (OR = 3.84, P = 0.001). […] The independent risk factors for 15-year mortality were age 70 years old (OR = 16.07, P = 0.009), LVEF 35% (OR = 5.69, P = 0.003), and SBP 120 mmHg (OR = 9.56, P 0.001). […] This study was the first to demonstrate the 15-year survival rate of 34% in DCM patients. […] Age 70 years, LVEF 35%, and SBP 120 mmHg were independent predictors of 15-year all-cause mortality. […] Patients with dilated cardiomyopathy have a poor prognosis, so more accurate risk stratification and personalized therapy may considerably improve outcomes.

• #2 Prediction of outcome in dilated cardiomyopathy. | Heart

  https://heart.bmj.com/content/58/4/393

  One hundred and sixty nine patients (mean age 39.1 years) with documented dilated cardiomyopathy were studied for a mean of 5.5 years. One hundred and four patients died during this period. The average (SD) interval from the onset of symptoms to death was 4.1 (3.7) years. One year and five year mortality rates were 27.8% and 57.4% respectively. Most of the deaths occurred within two years of diagnosis. The only difference between survivors and those who died was in the severity of left ventricular dysfunction at the time of referral. Significant differences between survivors and non-survivors were found for left ventricular end diastolic pressure (17.3 versus 23.4 mm Hg), left ventricular end systolic volume (87.4 versus 128.9 ml/m2), left ventricular end diastolic volume (130.7 versus 173.2 ml/m2), and ejection fraction (32.8 versus 25.4%). Patients with dilated cardiomyopathy have a high mortality irrespective of treatment. The only identifiable prognostic indicator was the severity of left ventricular impairment at referral.

• #3 Dilated Cardiomyopathy – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/cardiomyopathies/dilated-cardiomyopathy

  Prognosis for dilated cardiomyopathy generally has been poor, although prognosis has improved with current management regimens (eg, use of beta-blockers, angiotensin-converting enzyme [ACE] inhibitors, angiotensin II receptor blockers [ARB], angiotensin II receptor blocker and neprilysin inhibitor [ARNI], mineralocorticoid receptor antagonists, sodium glucose cotransporter 2 protein (SGLT2) inhibitors, implantable cardioverter-defibrillators, or cardiac resynchronization therapy). About 20% of patients die in the first year and then about 10%/year thereafter; about 40 to 50% of deaths are sudden, due to a malignant arrhythmia (1). […] Prognosis is better for females than for males, and people of African ancestry survive about half as long as White people (1). […] Prognosis is better if compensatory hypertrophy preserves ventricular wall thickness and is worse if ventricular walls thin markedly and the ventricle dilates. Patients whose dilated cardiomyopathy is well-compensated with treatment may be stable for many years.

• #4 Diagnosis and Risk Prediction of Dilated Cardiomyopathy in the Era of Big Data and Genomics

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7956169/

  New opportunities now lie in big data-minded clinical research. […] The performance of these models in DCM may, however, be suboptimal because their derivation also included patients with ischemic aetiology who are known to have a higher mortality risk than DCM patients. […] Interestingly, the risk models typically overestimated mortality risk in DCM patients. […] Moreover, there is a distinct subgroup of DCM patients who experience LV function recovery and have a subsequent mild clinical course during follow-up. […] Patients with DCM have a 4.5% annual risk of LTVA and therefore may benefit from ICD implantation. […] Several studies have evaluated risk factors for LTVA in DCM, which include reduced LVEF; LV dilatation; LGE on CMR; prior (non)sustained ventricular arrhythmia; and pathogenic variants in PLN, LMNA, FLNC, and TTNtv.

• #5 Diagnosis and Risk Prediction of Dilated Cardiomyopathy in the Era of Big Data and Genomics

  https://www.mdpi.com/2077-0383/10/5/921

  Several clinical prediction models have been constructed for heart failure in the general cardiology population in order to facilitate prognostic assessments. […] The performance of these models in DCM may, however, be suboptimal because their derivation also included patients with ischemic aetiology who are known to have a higher mortality risk than DCM patients. […] Interestingly, the risk models typically overestimated mortality risk in DCM patients. […] Patients with DCM have a ±4.5% annual risk of LTVA and therefore may benefit from ICD implantation. […] Current guidelines provide a Class IB recommendation for ICD implantation in symptomatic (NYHA ≥ II) DCM patients with an LVEF ≤35%, despite ≥3 months of optimal pharmacological therapy. […] Several studies have evaluated risk factors for LTVA in DCM, which include reduced LVEF; LV dilatation; LGE on CMR; prior (non)sustained ventricular arrhythmia; and pathogenic variants in PLN, LMNA, FLNC, and TTNtv. […] The significance of TTNtv, however, remains uncertain in unaffected relatives and individuals tested for a non-DCM disease. Long-term outcome studies in unaffected carriers are necessary to determine appropriate management recommendations in these subjects.

• #6 Epidemiology and Prognosis of Myocarditis and dilated Cardiomyopathy: Predictive Value of Clinical Parameters and Biopsy Findings | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-7643-8352-7_2

  The natural history of myocarditis varies based on its initial clinical presentation. […] Fulminant myocarditis appears to have an excellent, long-term prognosis with event-free survival rate exceeding 90% at 10 years if aggressive hemodynamic support is provided during the acute phase of the illness. […] Mortality for biopsy-verified lymphocytic myocarditis averages 20% at 1 year and over 50% at 4 years. […] The survival rates are similar to observational data for patients with idiopathic dilated cardiomyopathy. […] Survival with giant cell myocarditis is substantially lower with fewer than 20% of patients surviving 5 years. […] Important predictors of adverse prognosis with biopsy-proven myocarditis include giant cell histopathology, persistent viral genome on repeat endomyocardial biopsy, elevated myocardial or circulatory Fas and Fas ligand levels, extent of left ventricular enlargement and sphericity, pulmonary hypertension and bundle branch block.

• #7 Fifteen-year mortality and prognostic factors in patients with dilated cardiomyopathy: persistent standardized application of drug therapy and strengthened management may bring about encouraging change in an aging society

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9170907/

  Our findings also suggest that regular follow-up and management contribute to the prognosis of HF patients. […] The significantly improved 15-year survival of such patients is very encouraging. […] The predictors identified in the study affecting long-term mortality were consistent with the results of previous studies. […] Our results showed that LVEF 35% was associated with 15-year all-cause death. […] Our results showed that SBP 120 mmHg was an independent risk predictor for both 5- and 15-year mortality. […] It can be seen that although the first 5-year mortality rate of DCM patients was very high, the mortality rate decreased significantly after the standardized treatment and reached a plateau in the last 10 years, and the survival rate of DCM patients improved at the end. […] We suggest that more attention should be paid to the prognosis of older patients with DCM.

• #8 Diagnosis and Risk Prediction of Dilated Cardiomyopathy in the Era of Big Data and Genomics

  https://www.mdpi.com/2077-0383/10/5/921

  Dilated cardiomyopathy (DCM) is a leading cause of heart failure and life-threatening ventricular arrhythmias (LTVA). […] Throughout the last decade, improved genetic testing of patients has identified genotype–phenotype associations and enhanced evaluation of at-risk relatives leading to better patient prognosis. […] In general, patients with DCM are at risk of frequent hospitalisation and overt heart failure, for which left ventricular assist devices (LVAD) and orthotopic heart transplantation (HTx) are the effective last resort treatments. […] Independent predictors of progressive heart failure include low LVEF, RV dilatation, global segmental wall motion abnormalities, high New York Heart Association (NYHA) class, older age, male sex, the presence of conduction disorders, and LGE on CMR (i.e., fibrosis).

• #9 Development and validation of a nomogram to predict the risk of death within 1 year in patients with non-ischemic dilated cardiomyopathy: a retrospective cohort study | Scientific Reports

  https://www.nature.com/articles/s41598-022-12249-7

  Predicting the chances mortality within 1 year in non-ischemic dilated cardiomyopathy patients can be very useful in clinical decision-making. […] The main predictors included in the nomogram were pulse pressure, red blood cell count, left ventricular end-diastolic dimension, levels of N-terminal pro b-type natriuretic peptide, medical history, in-hospital worsening heart failure, and use of angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers. […] A decision curve analysis confirmed that our nomogram was clinically useful. […] The poor prognosis for most NIDCM patients puts a huge financial burden on individuals, families, and society. […] Many factors affect the prognosis of NIDCM; these include genetics and family history, aetiology, gender, age, and presence of comorbidities such as renal failure, chronic obstructive pulmonary disease, etc.

• #10 The application of radiology for dilated cardiomyopathy diagnosis, treatment, and prognosis prediction: a bibliometric analysis – Fang – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/117649/html

  Radiology plays a highly crucial role in the diagnosis, treatment, and prognosis prediction of dilated cardiomyopathy (DCM). […] The 5-year survival rate of patients with DCM is as low as 50%. […] Cardiac imaging is used for DCM diagnosis and prognosis prediction. […] Therefore, cardiac imaging plays an important role in the diagnosis and treatment of DCM. […] The current research hot spots are myocardial fibrosis, risk stratification of ventricular arrhythmia, the prognosis of cardiac resynchronization therapy (CRT) treatment, and subtype classification of DCM. […] The study on ventricular remodeling and long-term prognosis in patients with DCM is an area of intense clinical research interest. […] Studies have shown that LGE-CMR more accurately performs ventricular tachycardia (VT) stratification and better predicts the risk of sudden cardiac death (SCD) in DCM patients.

• #11 The application of radiology for dilated cardiomyopathy diagnosis, treatment, and prognosis prediction: a bibliometric analysis – Fang – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/117649/html

  Positive LGE results have been linked to poor prognosis, which helps in guiding clinical follow-up treatment. […] The factors influencing prognosis and myocardial remodeling in patients with DCM through use of endomyocardial biopsy have been explored at the cellular, gene, and protein levels, among others. […] The occurrence of LVRR indicates the effectiveness of the treatment and predicts a good DCM prognosis. […] The studies of Li et al. and Chen et al. both support the value of LA strain in predicting the adverse clinical outcome of DCM. […] In our bibliometric analysis, the top 3 keywords with the highest citation burst after 2018 were sudden cardiac death, cardiac magnetic resonance, and ventricular arrhythmia, with cardiac magnetic resonance being the most cited among them. […] This suggests that CMR imaging has long played an important role in DCM radiology. […] Risk stratification of ventricular fibrillation in patients with DCM and prediction of SCD have emerged to the forefront of DCM radiology research.

• #12 Diagnosis and Risk Prediction of Dilated Cardiomyopathy in the Era of Big Data and Genomics

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7956169/

  Dilated cardiomyopathy (DCM) is a leading cause of heart failure and life-threatening ventricular arrhythmias (LTVA). […] Throughout the last decade, improved genetic testing of patients has identified genotype-phenotype associations and enhanced evaluation of at-risk relatives leading to better patient prognosis. […] In addition, genetic risk scores derived from genome/exome-wide association studies can estimate an individual’s lifetime genetic risk of developing DCM. […] The large clinical heterogeneity of DCM results in many challenges in disease management. […] In addition, prognosis of DCM is difficult to predict, as exemplified by the fact that the majority of DCM patients who receive an implantable cardiac defibrillator (ICD) do not receive appropriate interventions during follow-up. This emphasises the need for better risk prediction models.

• #13 Diagnosis and Risk Prediction of Dilated Cardiomyopathy in the Era of Big Data and Genomics

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7956169/

  The significance of TTNtv, however, remains uncertain in unaffected relatives and individuals tested for a non-DCM disease. Long-term outcome studies in unaffected carriers are necessary to determine appropriate management recommendations in these subjects. […] Clinical management of DCM patients is challenging given the large heterogeneity in disease phenotype, genetic background, and progression of disease.

• #14 Epidemiology and Prognosis of Myocarditis and dilated Cardiomyopathy: Predictive Value of Clinical Parameters and Biopsy Findings | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-7643-8352-7_2

  Long-term outcome of fulminant myocarditis as compared with acute (nonfulminant) myocarditis. […] Survival in biopsy-proven myocarditis: A long-term retrospective analysis of the histopathologic, clinical, and hemodynamic predictors. […] Viral persistence in the myocardium is associated with progressive cardiac dysfunction.

• #15 Development and validation of a nomogram to predict the risk of death within 1 year in patients with non-ischemic dilated cardiomyopathy: a retrospective cohort study | Scientific Reports

  https://www.nature.com/articles/s41598-022-12249-7

  The 2017 AHA (American Heart Association)/ACC (American College of Cardiology)/HRS (Heart Rhythm Society) Guidelines clearly state that ICD implantation is applicable for patients with expected survival times of 1 year. […] Therefore, the assessment of the risk of death within 1 year for NIDCM patients is particularly important for weighing the benefits and risks of different life-saving procedures. […] We have created a nomogram for assessing the risk of mortality within 1 year for NIDCM patients by using several common predictors in clinical practice; this model has been verified as having a good predictive value. […] The nomogram that we have developed can be used to assess the 1-year mortality risk in NIDCM patients to allow for better individualized clinical decisions.

• #16 Dilated cardiomyopathy | Nature Reviews Disease Primers

  https://www.nature.com/articles/s41572-019-0084-1

  Dilated cardiomyopathy (DCM) is a clinical diagnosis characterized by left ventricular or biventricular dilation and impaired contraction that is not explained by abnormal loading conditions (for example, hypertension and valvular heart disease) or coronary artery disease. […] As DCM eventually leads to impaired contractility, standard approaches to prevent or treat heart failure are the first-line treatment for patients with DCM. […] In addition, identifying the probable cause of DCM helps tailor specific therapies to improve prognosis. […] An improved aetiology-driven personalized approach to clinical care will benefit patients with DCM, as will new diagnostic tools, such as serum biomarkers, that enable early diagnosis and treatment. […] This study compares the angiotensin receptor-neprilysin inhibitor LCZ696 with enalapril in patients who had heart failure with a reduced ejection fraction. LCZ696 was superior to enalapril in reducing the risks of death and of hospitalization for heart failure.

• #17 Dilated cardiomyopathy | Nature Reviews Disease Primers

  https://www.nature.com/articles/s41572-019-0084-1

  In this EMB-based analysis of the long-term disease course, perforin, a key predictor of poor outcome, was detected in the myocardium of a large cohort of patients with inflammatory cardiomyopathy. […] This review article aims to help bridge the widening gap between recent mechanistic insights and their potential impact on disease burden. The article provides an overview of the entire management of myocarditis, particularly with respect to aetiology-based therapy. […] This review article focuses on immune reactions in the myocardium, evoked by external triggers, that continue insidiously and lead to the process of cardiac remodelling with ventricular dilatation, systolic dysfunction and DCM.

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