Materiały źródłowe

• #1 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, 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. […] 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.

• #2 Prognosis of heart failure – UpToDate

  https://www.uptodate.com/contents/prognosis-of-heart-failure

  Prognosis of heart failure […] The prognosis of patients with HF with reduced ejection fraction (HFrEF) will be reviewed here. […] Morbidity and mortality rates after the onset of symptomatic HF are high, although variable mortality rates have been reported which likely reflect differences in demographics, disease severity, and the use of appropriate medical therapy. […] The need for hospitalization is an important marker for poor prognosis. […] Mortality rate was increased after HF hospitalizations, even after adjustment for baseline predictors of death (hazard ratio [HR] 3.2; 95% CI 2.8-3.5). […] Outcome with a cardiomyopathy is related to the etiology. […] Mortality and hospitalizations in heart failure show a seasonal variation. […] ACE inhibitor improves survival in advanced HF. […] ACE inhibitor improves survival in moderate HF. […] Enalapril improves outcome in asymptomatic LV dysfunction.

• #3 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 hots pots 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.

• #4 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. […] 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. […] 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.

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

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

  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. […] Newer techniques such as LGE on CMR and genetic sequencing are gaining ground in the work-up of DCM, which should be incorporated in future risk scores for DCM as they may improve ICD candidate selection even further.

• #6 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. […] In this study, we have developed a nomogram that can predict the risk of death within 1 year in patients with non-ischemic dilated cardiomyopathy. This will be useful in the early identification of patients in the terminal stages for better individualized clinical decisions. […] 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.

• #7 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

  Several of these factors are of high predictive value for the prognosis of NIDCM. […] The poor prognosis for most NIDCM patients puts a huge financial burden on individuals, families, and society. […] 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. […] To help with clinical decision-making in NIDCM patients, a specific 1-year mortality risk prediction model became an urgent need. […] 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.

• #8 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 studies of Li et al. and Chen et al. both support the value of LA strain in predicting the adverse clinical outcome of DCM. […] The research hotspots for further studies include the stratification of ventricular arrhythmia, myocardial fibrosis, prognosis prediction of CRT, and classification of DCM subtypes.

• #9

  https://link.springer.com/article/10.1007/s11845-020-02477-z

  Heart failure (HF) is the only cardiovascular disease with an ever increasing incidence. […] The main challenge in the treatment of HF is the availability of reliable prognostic models that would allow patients and doctors to develop realistic expectations about the prognosis and to choose the appropriate therapy and monitoring method. […] Prognosis assessment plays a special role in patients qualified for implantable device therapy or surgical treatment (including heart transplantation). […] However, no single risk factor is sufficient to predict prognosis in HF. Results of a few markers must be interpreted together. […] Still it is important to find the most important and valuable panel of a few predictors and there are still ongoing studies assessing potential new ones. […] The 2016 European Society of Cardiology (ESC) guidelines on HF named over 70 predictors in HF patients. […] Despite the identification of many markers and models of poor prognosis, clinical decisions and guidelines in HF are still based mainly on several basic parameters, such as the presence of HF symptoms (NYHA class), LVEF, and the duration and morphology of the QRS complex.

• #10 Progression of familial and non-familial dilated cardiomyopathy: long term follow up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1767723/

  It has been suggested that familial disease indicates a worse prognosis, and that this should be considered when planning the timing of heart transplantation. […] Five year survival was 55% for non-familial and 51% for familial patients (NS). The main predictor of mortality was an LVEF of 30%. Familial status did not predict mortality. […] Five year survival is not significantly different in the familial and non-familial forms of dilated cardiomyopathy. […] It has been suggested that patients with familial disease have a worse prognosis than those with non-familial disease. […] The five year survival rate was 54.4% similar to the mortality reported in other series of patients with dilated cardiomyopathy. There was no significant difference in survival between familial (51.5%) and non-familial patients (55.7%). […] We conclude that there is no significant difference in the clinical course of patients with familial and non-familial dilated cardiomyopathy. Familial status should not be the main consideration in timing heart transplantation in most cases.

• #11 Survival and prognostic factors in hypertrophic cardiomyopathy: a meta-analysis | Scientific Reports

  https://www.nature.com/articles/s41598-017-12289-4

  Hypertrophic cardiomyopathy (HCM) is a clinically and genetically heterogeneous disorder but data on survival rates are still conflicting and have not so far been quantitatively reviewed. The aim of this study is to conduct a meta-analysis of cohort studies to assess pooled survival rates and prognostic factors for survival in patients with HCM. Nineteen studies were included representing 12,146 HCM patients. The pooled 1-, 3-, 5- and 10-year survival rates were 98.0%, 94.3%, 82.2% and 75.0%, respectively. Among patients with HCM, age, NYHA functional class, family history of sudden death (FHSD), syncope, atrial fibrillation, non-sustained ventricular tachycardia (nsVT), maximum left ventricular wall thickness and obstruction were significant prognostic factors for cardiovascular death. For sudden cardiac death, FHSD, nsVT, and obstruction showed significant predictive values. Moreover, estimation of population attributable risk (PAR) suggested that nsVT was the strongest predictor for cardiovascular death (13.02%, 95% CI 3.6025.91%), while left ventricular outflow tract obstruction/mid-ventricular obstruction (LVO/MVO) was the strongest predictor for all-cause death and sudden cardiac death (10.09%, 95% CI 4.7220.42% and 16.44%, 95% CI 7.4531.55%, respectively). These risk factors may thus be useful for identifying HCM patients who might benefit from early diagnosis and therapeutic interventions.

• #12 Survival and prognostic factors in hypertrophic cardiomyopathy: a meta-analysis | Scientific Reports

  https://www.nature.com/articles/s41598-017-12289-4

  Prognostic factors for survival in HCM patients have also been assessed in several studies and were recently reviewed. It is important to identify such factors in order to ensure optimal care and facilitate appropriate monitoring. However, there are marked discrepancies among studies focused on HCM, and there is a lack of consensus on factors that best predict mortality in idiopathic HCM. […] The prognostic factors for survival and pooled hazard ratios are summarized. Among patients with HCM, average age, NYHA functional class, non-sustained ventricular tachycardia (nsVT), family history of sudden death, syncope, atrial fibrillation, maximum left ventricular wall thickness and obstruction were significant prognostic factors for cardiovascular death. Conversely, for all-cause death, average age and FHSD were not significant prognostic factors. For sudden cardiac death (SCD), nsVT, FHSD, and obstruction showed significant predictive values. Furthermore, NYHA class III/IV was the most important risk factor for cardiovascular death; MLVWT was the most important risk factor for sudden cardiac death whereas NYHA class III/IV showed the strongest prognostic value for all-cause death.

• #13 Survival and prognostic factors in hypertrophic cardiomyopathy: a meta-analysis | Scientific Reports

  https://www.nature.com/articles/s41598-017-12289-4

  Family history of sudden death accounted for 11.31% and 11.89% of the PAR for cardiovascular death and sudden cardiac death, whereas no significant association was observed for all cause death. nsVT showed the highest PAR in cardiovascular death, and conversely, obstruction showed the highest PAR for all-cause death and sudden cardiac death. […] Our findings indicate that known traditional HCM risk factors, such as FHSD and AF, have prognostic relevance, as expected. Judged by PAR, nsVT and LVO/MVO, respectively, are the most important risk factors for cardiovascular death and sudden cardiac death in HCM confirmed in our meta-analysis, and FHSD is also a highly important prognostic marker.

• #14 Association between programmed electrical stimulation inducibility and arrhythmic risk in hypertrophic cardiomyopathy: a systematic review and meta-analysis | Heart

  https://heart.bmj.com/content/early/2025/04/16/heartjnl-2024-325043

  PES inducibility is associated with a 10 times higher risk of MAE in patients with HCM. […] This systematic review and meta-analysis demonstrate that PES inducibility is strongly associated with a more than 10-times increased risk of major arrhythmic events in patients with HCM. […] These findings suggest that PES could have a role in refining risk stratification strategies for HCM, particularly for intermediate-risk patients. […] Our analysis provides evidence of an association between PES inducibility in patients with HCM and the occurrence of arrhythmic events in primary prevention, with an estimated 10 times higher risk.

• #15 Application of the ALBI Scoring System for Mortality Outcome Prediction in Patients with Hypertrophic Cardiomyopathy | Global Heart

  https://globalheartjournal.com/articles/10.5334/gh.1163

  Higher ALBI score is a strong independent predictor of HCM-related death in patients with HCM. […] Overall, there was a significant positive association between ALBI score and HCM-related death (adjusted hazard ratio [HR]: 1.79 per one standard deviation [SD] increment, 95% confidence interval [CI]: 1.362.35). […] The study has several limitations. Firstly, this was a single center, retrospective study, and those patients were from China, lack of region diversification and race comparison. […] In summary, higher ALBI score is a strong independent predictor of HCM-related death, and the patients with higher ALBI score may warrant closer follow-up and more aggressive therapy with hopefully prevention of events in the future.

• #16 The Predictive Role of Cardiac Troponin Elevation Ratio Combined With Heart Function Index Model in the Prognosis of Non-ST-Segment Elevation Myocardial Infarction Patients | Hu | Cardiology Research

  https://www.cardiologyres.org/index.php/Cardiologyres/article/view/1639

  Conclusions: In NSTEMI patients, a 36.25-fold increase in cTnI/ULN, coupled with NYHA class III and IV, independently predicted prognosis. We developed a nomogram model integrating cTnI/ULN and cardiac function indices, aiding clinicians in assessing risk and implementing early interventions for improved outcomes.

• #17 The Predictive Role of Cardiac Troponin Elevation Ratio Combined With Heart Function Index Model in the Prognosis of Non-ST-Segment Elevation Myocardial Infarction Patients | Hu | Cardiology Research

  https://www.cardiologyres.org/index.php/Cardiologyres/article/view/1639

  The Predictive Role of Cardiac Troponin Elevation Ratio Combined With Heart Function Index Model in the Prognosis of Non-ST-Segment Elevation Myocardial Infarction Patients […] Background: Non-ST-segment elevation myocardial infarction (NSTEMI) is a common form of coronary artery disease, and its prognosis is influenced by multiple factors. This study aimed to analyze the predictive role of the combined application of cardiac troponin and cardiac function indices in NSTEMI patients prognosis. […] Results: A total of 252 NSTEMI patients were included. Female gender, elevated high-sensitivity C-reactive protein (H-CRP), left ventricular ejection fraction (LVEF) 50%, NYHA class III and IV, and cTnI/ULN elevation by 36.25-fold were significantly independently associated with mortality outcomes. Multifactorial logistic analysis indicated that these indices remained associated with mortality. A nomogram model predicting NSTEMI patient mortality was constructed using these indices, with an area under the curve (AUC) of 0.911, sensitivity of 97.5%, and specificity of 72.8%. This predictive model outperformed the Grace score (AUC = 0.840).

• #18 Frontiers | Inherited transthyretin cardiac amyloidosis presenting with diastolic heart failure and gastrointestinal symptoms: a case report and literature review

  https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2025.1588291/full

  Inherited transthyretin cardiac amyloidosis (hATTR-CA) is rising with an aging population and more mutation carriers. Its symptoms often resemble other heart diseases, leading to delayed diagnosis and affecting prognosis. […] With early diagnosis and active treatment, the patient’s prognosis improved, symptoms of heart failure were controlled, and quality of life significantly improved. […] Timely identification of CA allows for effective treatment, improving prognosis. […] This case emphasizes the importance of early diagnosis and accurate treatment of hATTR-CA, particularly the role of imaging in diagnosing myocardial infiltration. […] After three months of treatment, follow-up visits showed significant improvement in the patient’s cardiac function, with a marked reduction in symptoms such as chest tightness and shortness of breath, leading to a substantial improvement in quality of life.

• #19 Frontiers | Inherited transthyretin cardiac amyloidosis presenting with diastolic heart failure and gastrointestinal symptoms: a case report and literature review

  https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2025.1588291/full

  This case report underscores the importance of an integrated diagnostic approach, particularly the use of multimodal imaging, in the early identification of ATTR-type myocardial amyloidosis. By leveraging multidisciplinary collaboration and advanced diagnostic tools, timely recognition and appropriate treatment can be initiated, significantly improving the patient’s quality of life.

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