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Heart and cardiomyopathies
Original research article
The systolic paradox in hypertrophic cardiomyopathy
  1. Trine F Haland1,2,
  2. Nina E Hasselberg1,2,
  3. Vibeke Marie Almaas1,2,
  4. Lars A Dejgaard1,2,
  5. Jørg Saberniak1,2,
  6. Ida S Leren1,2,
  7. Knut Erik Berge3,
  8. Kristina H Haugaa1,2 and
  9. Thor Edvardsen1,2
  1. 1 Department of Cardiology, Institute for Surgical Research and Center for Cardiological Innovation, Oslo University Hospital, Rikshospitalet, Oslo, Norway
  2. 2 University of Oslo, Norway
  3. 3 Department of Medical Genetics, Unit for Cardiac and Cardiovascular Genetics, Oslo University Hospital, Ullevål, Oslo, Norway
  1. Correspondence to Dr Thor Edvardsen; thor.edvardsen{at}medisin.uio.no

Abstract

Objective We explored cardiac volumes and the effects on systolic function in hypertrophic cardiomyopathy (HCM) patients with left ventricular hypertrophy (HCM LVH+) and genotype-positive patients without left ventricular hypertrophy (HCM LVH−).

Methods We included 180 HCM LVH+, 100 HCM LVH− patients and 80 healthy individuals. End-Diastolic Volume Index (EDVI), End-Systolic Volume Index (ESVI) and ejection fraction (EF) were assessed by echocardiography. Left ventricular (LV) global longitudinal strain (GLS) was measured by speckle tracking echocardiography.

Results EDVI and ESVI were significantly smaller in HCM LVH+ compared with HCM LVH− patients (41±14 mL/m2 vs 49±13 mL/m2 and 16±7 mL/m2 vs 19±6 mL/m2, respectively, both p<0.001) and in healthy individuals (41±14 mL/m2 vs 57±14 mL/m2 and 16±7 mL/m2 vs 23±9 mL/m2, respectively, both p<0.001). HCM LVH− patients had significantly lower EDVI and ESVI compared with healthy individuals (49±13 mL/m2 vs 57±14 mL/m2 and 19±6 mL/m2 vs 23±9 mL/m2, both p<0.001). EF was similar (61%±7% vs 60%±8% vs 61%±6%, p=0.43) in the HCM LVH+, HCM LVH– and healthy individuals, despite significantly worse GLS in the HCM LVH+ (−16.4%±3.7% vs −21.3%±2.4% vs −22.3%±3.7%, p<0.001). GLS was worse in the HCM LVH− compared with healthy individuals in pairwise comparison (p=0.001). Decrease in ESVI was closely related to EF in HCM LVH+ and HCM LVH− (R=0.45, p<0.001 and R=0.43, p<0.001) as expected, but there was no relationship with GLS (R=0.02, p=0.77 and R=0.11, p=0.31). Increased maximal wall thickness (MWT) correlated significantly with worse GLS (R=0.58, p<0.001), but not with EF (R=0.018, p=0.30) in the HCM LVH+ patients.

Conclusion HCM LVH+ had smaller cardiac volumes that could explain the preserved EF, despite worse GLS that was closely related to MWT. HCM LVH− had reduced cardiac volumes and subtle changes in GLS compared with healthy individuals, indicating a continuum of both volumetric and systolic changes present before increased MWT.

  • Hypertrophic cardiomyopathy
  • echocardiography and heart failure with preserved ejection fraction

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/openhrt-2016-000571

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    Footnotes

    • Contributors All authors have read and approved the manuscript.

    • Funding This work was supported by the Center for Cardiological Innovation, funded by the Research Council of Norway.

    • Competing interests None declared.

    • Ethics approval The study complied with the Declaration of Helsinki and was approved by the Regional Committees for Medical Research Ethics.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data sharing statement No available unpublished data.