Clinical Characteristics and Natural History of Hypertrophic Cardiomyopathy With Midventricular Obstruction

Georgios K. Efthimiadis; Efstathios D. Pagourelias; Despoina Parcharidou; Thomas Gossios; Vasileios Kamperidis; Efstratios K. Theofilogiannakos; Zoi Pappa; Soultana Meditskou; Stavros Hadjimiltiades; Christodoulos Pliakos; Haralampos Karvounis; Ioannis H. Styliadis

doi:10.1253/circj.CJ-12-1561

Myocardial Disease

Clinical Characteristics and Natural History of Hypertrophic Cardiomyopathy With Midventricular Obstruction

Georgios K. Efthimiadis, Efstathios D. Pagourelias, Despoina Parcharidou, Thomas Gossios, Vasileios Kamperidis, Efstratios K. Theofilogiannakos, Zoi Pappa, Soultana Meditskou, Stavros Hadjimiltiades, Christodoulos Pliakos, Haralampos Karvounis, Ioannis H. Styliadis

Author information

Georgios K. Efthimiadis
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Efstathios D. Pagourelias
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Despoina Parcharidou
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Thomas Gossios
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Vasileios Kamperidis
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Efstratios K. Theofilogiannakos
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Zoi Pappa
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Soultana Meditskou
Laboratory of Histology and Embryology, Medical School, Aristotle University of Thessaloniki
Stavros Hadjimiltiades
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Christodoulos Pliakos
First Department of Internal Medicine, AHEPA University Hospital
Haralampos Karvounis
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital
Ioannis H. Styliadis
Cardiomyopathies Center, First Cardiology Department, AHEPA University Hospital

Keywords: Heart failure, Hypertrophic cardiomyopathy, Midventricular obstruction, Sudden death

JOURNAL FREE ACCESS

2013 Volume 77 Issue 9 Pages 2366-2374

DOI https://doi.org/10.1253/circj.CJ-12-1561

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Abstract

Background: The prevalence, clinical characteristics and natural history of patients with hypertrophic cardiomyopathy (HCM) and midventricular obstruction (MVO) have not been adequately studied. Methods and Results: A single-center cohort consisting of 423 patients (mean age, 49.3±17.2 years; 66.2% male) was thoroughly followed up for a median of 84 months (7 years; range, 6–480 months). MVO, characterized by the echocardiographic appearance of midventricular muscular apposition with a simultaneous mid-cavitary gradient ≥30mmHg, was identified in 34 patients (8%). Patients with MVO tended to be more symptomatic during their initial evaluation (>90% presented with NYHA class ≥II) compared to the rest of the HCM cohort. Apical aneurysm formation was identified in more than one-fourth of patients with MVO (26.5%), being a characteristic of the group. On multivariate Cox regression hazard analysis, presence of MVO strongly predicted progression to end-stage (burnt out) HCM and related heart failure (HF) deaths (hazard ratio, [HR], 2.62; 95% confidence interval [CI]: 1.2–8.8; P=0.047), as well as sudden death and associated lethal arrhythmic events (HR, 3.3; 95% CI: 1.26–8.85; P=0.016). Conclusions: MVO is a distinct phenotype of HCM associated with unfavorable prognosis in terms of end-stage HCM, sudden death and lethal arrhythmic events. The high adverse outcome rate necessitates early recognition of MVO and appropriate therapeutic interventions. (Circ J 2013; 77: 2366–2374)

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