Comparison of Clinical Course and Outcomes between Dilated and Hypokinetic Non-Dilated Cardiomyopathy.

BACKGROUND: By definition, dilated cardiomyopathy (DCM) is characterized by enlargement of the left ventricular (LV) cavity, and systolic dysfunction. However, in 2016 ESC introduced a new clinical entity - hypokinetic non-dilated cardiomyopathy (HNDC). HNDC is defined as LV systolic dysfunction without LV dilatation. However, the diagnosis of HNDC has so far rarely been made by a cardiologist, and it is unknown whether "classic" DCM differs from HNDC in terms of clinical course and outcomes. OBJECTIVES: Comparison of heart failure profiles and outcomes between patients with "classic" dilated (DCM) and HNDCs. METHOD: We retrospectively analysed 785 DCM patients, defined as impaired left ventricle (LV) systolic function (ejection fraction [LVEF] <45%) in the absence of coronary artery disease, valve disease, congenital heart disease, and severe arterial hypertension. "Classic" DCM was diagnosed when LV dilatation was present (LV end-diastolic diameter >52 mm/58 mm in women/men); otherwise, HNDC was diagnosed. After 47 ± 31 months, the all-cause mortality and composite endpoint (all-cause mortality, heart transplant - HTX, left ventricle assist device implantation - LVAD) were assessed. RESULTS: There were 617 (79%) patients with LV dilatation. Patients with "classic" DCM differed from HNDC in terms of clinically relevant parameters [hypertension (47% vs. 64%, p = 0.008), ventricular tachyarrhythmias (29% vs. 15%, p = 0.007), NYHA class (2.5 ± 0.9 vs. 2.2 ± 0.8, p = 0.003)], had lower cholesterol (LDL: 2.9 ± 1.0 vs. 3.2 ± 1.1 mmol/L, p = 0.049), and higher N-terminal pro-brain natriuretic peptide (3,351 ± 5,415 vs. 2,563 ± 8584 pg/mL, p = 0.0001) and required higher diuretics dosages (57.8 ± 89.5 vs. 33.7 ± 48.7 mg/day, p ≤ 0.0001). All of their chambers were larger (LVEDd: 68.3 ± 4.5 vs. 52.7 ± 3.5 mm, p < 0.0001) and they had lower LVEF (25.2 ± 9.4 vs. 36.6 ± 11.7%, p < 0.0001). During the follow-up, there were 145 (18%) composite endpoints ("classic" DCM vs. HNDC: 122 [20%] vs. 26 [18%], p = 0.22): deaths (97 [16%] vs. 24 [14%], p = 0.67), HTX (17 [4%] vs. 4 [4%], p = 0.97) and LVAD (19 [5%] vs. 0 [0%], p = 0.03). Both groups did not differ in terms of all-cause mortality (p = 0.70), cardiovascular (CV) mortality (p = 0.37) and composite endpoint (p = 0.26). CONCLUSIONS: LV dilatation was absent in more than one-fifth of DCM patients. HNDC patients had less severe heart failure symptoms, less advanced cardiac remodelling, and required lower diuretics dosages. On the other hand, "classic" DCM and HNDC patients did not differ in terms of all-cause mortality, CV mortality, and composite endpoint.

Clinical Utility and Validation of the Krakow DCM Risk Score-A Prognostic Model Dedicated to Dilated Cardiomyopathy.

BACKGROUND: One of the most common causes of heart failure is dilated cardiomyopathy (DCM). In DCM, the mortality risk is high and reaches approximately 20% in 5 years. A patient's prognosis should be established for appropriate HF management. However, so far, no validated tools have been available for the DCM population. METHODS: The study population consisted of 735 DCM patients: 406 from the derivation cohort (previously described) and 329 from the validation cohort (from 2009 to 2020, with outcome data after a mean of 42 months). For each DCM patient, the individual mortality risk was calculated based on the Krakow DCM Risk Score. RESULTS: During follow-up, 49 (15%) patients of the validation cohort died. They had shown significantly higher calculated 1-to-5-year mortality risks. The Krakow DCM Risk Score yielded good discrimination in terms of overall mortality risk, with an AUC of 0.704-0.765. Based on a 2-year mortality risk, patients were divided into non-high (≤6%) and high (>6%) mortality risk groups. The observed mortality rates were 8.3% (n = 44) vs. 42.6% (n = 75), respectively (HR 3.37; 95%CI 1.88-6.05; p < 0.0001). CONCLUSIONS: The Krakow DCM Risk Score was found to have good predictive accuracy. The 2-year mortality risk > 6% has good discrimination for the identification of high-risk patients and can be applied in everyday practice.

Lack of Relationship between Fibrosis-Related Biomarkers and Cardiac Magnetic Resonance-Assessed Replacement and Interstitial Fibrosis in Dilated Cardiomyopathy.

The relationship between circulating fibrosis-related molecules and magnetic resonance-assessed cardiac fibrosis in dilated cardiomyopathy (DCM) is poorly understood. To compare circulating biomarkers between DCM patients with high and low fibrosis burdens, we performed a prospective, single-center, observational study. The study population was composed of 100 DCM patients (87 male, mean age 45.2 ± 11.8 years, mean ejection fraction 29.7% ± 10.1%). Replacement fibrosis was quantified by means of late gadolinium enhancement (LGE), whereas interstitial fibrosis was assessed via extracellular volume (ECV). Plasma concentrations of cardiotrophin-1, growth differentiation factor-15, platelet-derived growth factor, procollagen I C-terminal propeptide, procollagen III N-terminal propeptide, and C-terminal telopeptide of type I collagen were measured. There were 44% patients with LGE and the median ECV was 27.7%. None of analyzed fibrosis serum biomarkers were associated with the LGE or ECV, whereas NT-proBNP was independently associated with both LGE and ECV, and troponin T was associated with ECV. None of the circulating fibrosis markers differentiated between DCM patients with and without replacement fibrosis, or patients stratified according to median ECV. However, cardiac-specific markers, such as NT-proBNP and hs-TnT, were associated with fibrosis. Levels of circulating markers of fibrosis seem to have no utility in the diagnosis and monitoring of cardiac fibrosis in DCM.