EFFECTS OF HYPOKINTEIC TRANSFORMATION ON CLINICAL PROFILE AND FUNCTIONAL CAPACITY IN HYPERTROPHIC CARDIOMYOPATHY - The 67th Annual Conference of the Israel Heart Society

Edward Itelman ^1,2 Yishay Wasserstrum ^1,2 Roberto Barriales-Villa ^3,4 Xusto Fernández-Fernández ^4,5 Yehuda Adler ^1,2 Dor Lotan ^1,2 Yael Peled ^1,2 Robert Klempfner ^1,2 Nir Shlomo ^1,2 Avi Sabbag ^1,2 Dov Freimark ^1,2 Lorenzo Monserrat ³ Michael Arad ^1,2

¹Leviev Heart Center, Sheba Medical Center, Israel
²Sackler School of Medicine, Tel Aviv University, Israel
³Inherited Cardiovascular Diseases Unit, Cardiology Service, Complexo Hospitalario Universitario A Coruña, Servizo Galego de Sau´de (SERGAS), Universidade da Coruña, A Coruña, Spain
⁴A Coruña, Instituto de Investigacio´n Biome´dica de A Coruña (INIBIC), Spain
⁵Cardiology Department, Health in Code S.L., Spain

Background: Advanced hypertrophic cardiomyopathy (HCM) may be complicated by a dilated hypokinetic transformation. Reduced left ventricular ejection fraction (HFrEF) has been described in terms of specific risks of morbidity and mortality, and specifically in terms of increased risk for fatal arrhythmias. Nevertheless, recent publications have casted doubt regarding the role of arrhythmia in non-ischemic HFrEF and questioned the role of primary prevention strategies in these cases.

Methods: We`ve reviewed clinical characteristics of 883 patients age ≥40, diagnosed with HCM who were evaluated in the cardiomyopathy clinic in two tertiary medical centers in Israel and Spain.

Results: Forty-five patients (5%) suffered from hypokinetic transformation. They were younger at diagnosis (median 44 [IQR 26-61] vs. 54 [44-64], p=0.002) and incurred more from strokes (27% vs 8%, p>0.001). They had lower had a lower NYHA class (p=0.004) and lower exercise capacity (6.6 [4.1-10.0] vs. 9.0 [6.0-11.0] METS, p=0.05). These patients had a higher prevalence of cardiovascular risk factors These patients had higher rates of pacemaker and implanted defibrillator (ICD) implantations (40% vs 11%, p<0.001) and (36% vs 10%, p<0.001) respectively. These patients had a higher incidence of ventricular tachyarrhythmias (16% vs 2%, p<0.001). Among patients who had an ICD, patients suffering from hypokinetic transformation had received more appropriate ICD therapy (44% vs 9%, p<0.001). These patients received more heart transplantations (13% vs 1%, p<0.001), and had higher instance rate of Sudden cardiac death (9% vs 2% p=0.018) and higher 5-year mortality rates.

Conclusions: HCM patients suffering from hypokinetic transformation have a lower functional capacity, are more likely to suffer from ventricular tachyarrhythmias and experience appropriate ICD therapy, and undergo heart transplantation. They also have a significantly lower 5-year survival.