GALATI, GIUSEPPE;
Germanova, Olga;
SABOURET, Pierre;
Aspromonte, Nadia;
Dalla Vecchia, Laura A;
Vranic, Ivana;
SENNI, MICHELE;
Metra, Marco;
Margonato, Alberto
Abstract 12790: Classification of the Different Types of Hypertrophic Cardiomyopathy Responsible for Advanced Heart Failure: From Clinical, Morphofunctional Characteristics to Prognosis and Potential New Therapies
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Medientyp:
E-Artikel
Titel:
Abstract 12790: Classification of the Different Types of Hypertrophic Cardiomyopathy Responsible for Advanced Heart Failure: From Clinical, Morphofunctional Characteristics to Prognosis and Potential New Therapies
Beteiligte:
GALATI, GIUSEPPE;
Germanova, Olga;
SABOURET, Pierre;
Aspromonte, Nadia;
Dalla Vecchia, Laura A;
Vranic, Ivana;
SENNI, MICHELE;
Metra, Marco;
Margonato, Alberto
Beschreibung:
<jats:p>
<jats:bold>Introduction:</jats:bold>
Advanced heart failure (AdvHF) in HCM was overlooked.
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<jats:p>
<jats:bold>Hypothesis:</jats:bold>
Based on a systematic revision, we described prevalence & outcomes of the main HCM phenotypes responsible for AdvHF, heart transplantation (HTx), LVAD implantation & HF-death.
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<jats:p>
<jats:bold>Methods:</jats:bold>
We classified the HCM profiles responsible for AdvHF in 1) End-stage HCM (ES-HCM) defined by an LVEF≤50%; 2) LVOTO despite optimal medical therapy (OMT) (Refractory HOCM); 3) Nonobstructive HCM with preserved EF (HNOCMpEF). The study screened 130 manuscripts in MEDLINE and EMBASE on AdvHF in HCM patients (pts) published from 2000 till January 2020, in adults. 8 manuscripts were eligible for the analysis. AdvHF was defined by the presence of severe NYHA symptoms (III & IV) because in all the manuscripts this definition was used. Mean follow-up = 7.6 years.
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<jats:p>
<jats:bold>Results:</jats:bold>
Figure 1: Prevalence of phenotypes responsible for AdvHF&HTx/LVAD implantation/HF-Death. Of 205 HCM pts, 119 (58%) underwent HTx, LVAD implantation, or died for HF. Figure 2: Outcome per each clinical phenotype.
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<jats:p>
<jats:bold>Conclusions:</jats:bold>
AdvHF in HCM has an ominous prognosis (58% of pts met HTx/LVAD implantation/HF-death) but with significant differences among the 3 phenotypes. HOCM has a very good outcome, indeed only few cases developed a Refractory HOCM & AdvHF & only 1 died for HF. 29.7% of AdvHF were caused by HNOCMpEF due to massive hypertrophy & restrictive physiology & only 16.4% of them died due to HF. ES-HCM represents the main cause of AdvHF in HCM & the major determinant for poor outcomes among all HCM pts (Only 1/3 of them were event-free). This reflects poor attention and portrays an unmet need for ES-HCM pts. Recently it was demonstrated that an extensive amount of myocardial fibrosis (MF) is the major determinant for ES-evolution. Taken together these findings show the importance of a strict follow-up for HCM pts to timely identify ES-HCM & early start HF OMT. Developing specific therapies to lower the burden of MF & stop adverse remodeling it's crucial.
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