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Comparison of Clinical Course and Outcomes between Dilated and Hypokinetic Non-Dilated Cardiomyopathy
Ewa Dziewięcka1, Mateusz Winiarczyk1, Sylwia Wiśniowska-Śmiałek1,2
1Department of Cardiac and Vascular Diseases, Jagiellonian University Collegium Medicum, John Paul II Hospital, Krakow, Poland.
Insights
Hypokinetic non-dilated cardiomyopathy (HNDC) and classic dilated cardiomyopathy (DCM) share similar mortality outcomes, despite HNDC patients presenting with less severe heart failure symptoms and cardiac remodeling. This suggests HNDC may be an underdiagnosed entity within the spectrum of DCM.
Area of Science:
- Cardiology
- Internal Medicine
- Clinical Research
Background:
- Dilated cardiomyopathy (DCM) is defined by left ventricular (LV) enlargement and systolic dysfunction.
- Hypokinetic non-dilated cardiomyopathy (HNDC) is a newer classification characterized by LV systolic dysfunction without LV dilatation.
- The clinical course and outcomes of HNDC compared to classic DCM remain largely unknown.
Discussion:
- This retrospective analysis compared 785 patients diagnosed with either classic DCM or HNDC.
- Patients were assessed for clinical parameters, cardiac remodeling, and heart failure severity.
- Outcomes including all-cause mortality, cardiovascular mortality, heart transplant, and LVAD implantation were evaluated over a median follow-up of 47 months.
Key Insights:
- Patients with classic DCM exhibited more severe heart failure symptoms, larger cardiac chambers, and lower ejection fraction compared to HNDC patients.
- Despite these differences, both classic DCM and HNDC groups showed no significant disparities in all-cause mortality, cardiovascular mortality, or composite endpoints.
- HNDC patients required lower diuretic dosages and had less advanced cardiac remodeling.
Outlook:
- The findings suggest that HNDC is an important clinical entity within the spectrum of DCM, often presenting with less severe symptoms but similar long-term prognoses.
- Further research is warranted to understand the underlying mechanisms and optimize management strategies for HNDC.
- Increased recognition and diagnosis of HNDC by cardiologists may refine patient stratification and treatment approaches.
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.
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