A systematic approach to echocardiography in hypertrophic cardiomyopathy: a guideline protocol from the British Society of Echocardiography

in Echo Research and Practice

Hypertrophic cardiomyopathy (HCM) is a relatively common inherited cardiac condition with a prevalence of approximately one in 500. It results in otherwise unexplained hypertrophy of the myocardium and predisposes the patient to a variety of disease-related complications including sudden cardiac death. Echocardiography is of vital importance in the diagnosis, assessment and follow-up of patients with known or suspected HCM. The British Society of Echocardiography (BSE) has previously published a minimum dataset for transthoracic echocardiography, providing the core parameters necessary when performing a standard echocardiographic study. However, for patients with known or suspected HCM, additional views and measurements are necessary. These additional views allow more subtle abnormalities to be detected or may provide important information in order to identify patients with an adverse prognosis. The aim of this Guideline is to outline the additional images and measurements that should be obtained when performing a study on a patient with known or suspected HCM.

Abstract

Hypertrophic cardiomyopathy (HCM) is a relatively common inherited cardiac condition with a prevalence of approximately one in 500. It results in otherwise unexplained hypertrophy of the myocardium and predisposes the patient to a variety of disease-related complications including sudden cardiac death. Echocardiography is of vital importance in the diagnosis, assessment and follow-up of patients with known or suspected HCM. The British Society of Echocardiography (BSE) has previously published a minimum dataset for transthoracic echocardiography, providing the core parameters necessary when performing a standard echocardiographic study. However, for patients with known or suspected HCM, additional views and measurements are necessary. These additional views allow more subtle abnormalities to be detected or may provide important information in order to identify patients with an adverse prognosis. The aim of this Guideline is to outline the additional images and measurements that should be obtained when performing a study on a patient with known or suspected HCM.

Introduction

  1. The British Society of Echocardiography (BSE) Education Committee has previously published a minimum dataset for a standard adult transthoracic echocardiogram (1). This Guideline specifically states that the minimum dataset is usually sufficient only when the echocardiographic study is entirely normal. The aim of the BSE Education Committee is to publish a series of appendices to cover specific pathologies supporting this minimum dataset.

  2. The intended benefits of such supplementary recommendations are to:

    • Support cardiologists and echocardiographers to develop local protocols and quality control programs for adult transthoracic study.

    • Promote quality by defining a set of descriptive terms and measurements, in conjunction with a systematic approach to performing and reporting a study in specific disease states.

    • Facilitate the accurate comparison of serial echocardiograms performed in patients at the same or different sites.

  3. This Guideline gives recommendations for the image and analysis dataset required in patients either being assessed for, or with a known diagnosis of hypertrophic cardiomyopathy (HCM). The views and measurements are supplementary to those outlined in the minimum dataset and are given assuming that a full study will be performed in all patients.

  4. When the condition or acoustic windows of the patient prevent the acquisition of one or more components of the supplementary dataset, or when measurements result in misleading information (e.g. off-axis measurements), this should be stated.

  5. This document is a guideline for echocardiography in HCM and will be updated in accordance with changes directed by publications or changes in practice (Table 1).

    Table 1

    Additional views and measurements to be obtained in patients with known or suspected hypertrophic cardiomyopathy

    View (modality)MeasurementExplanatory noteImage
    PLAX (2D/MM)IVSdIVSd measure >3 cm is a key marker of increased risk (2)

    Demonstrate if ASH is present

    Measure RV wall thickness if on axis
    PLAX (2D/MM)LA sizeMeasure LA size (anterior–posterior diameter). LA diameter is one of the criteria used to estimate risk of sudden cardiac death (3)
    PLAX (MM and CFM)MV leaflet tips and AV leaflet tipsDemonstrate if SAM is present on M-Mode and for colour flow turbulence within the LVOT

    Demonstrate if early closure of the AV
    PSAX MV (2D)Frozen 2D image: obtain wall thickness measurements from level of the basal LV. Measure at four points, using clock face references (12, 3, 6, 9 o'clock)To assess for asymmetric and symmetric segmental LV hypertrophy

    Segmental hypertrophy >1.5 cm (2) with normal or small LV internal cavity dimensions is strongly suggestive of HCM (in absence of other pathologies such as hypertension)
    PSAX PM (2D)2D frozen image at the mid-LV level. Measure at four points, using clock face references (12, 3, 6, 9 o'clock)Avoid off-axis measurements, papillary muscle and trabeculations
    PSAX Apex (2D)Apical-level measure at two points (12 and 6 o'clock)Apical hypertrophy may be present if apical/basal lateral ratio is >1.5. Consideration should be given to use of LV opacification contrast
    Modified PSAX (2D and PW/CW)RV wall thickness and RVOT forward flow velocitiesModify both the RV inflow and outflow to assess for RVH and RVOT obstruction. RVH present if >0.5 cm
    Modified A4C (2D)RV wall thicknessIf clear images can be obtained, measure RV wall thickness. Otherwise measurement from PLAX and subcostal views is preferred. RVH present if >0.5 cm
    A4C and A2C (2D)LA volumeIndex LA volume to BSA (4)
    A4C (CFM)Aetiology and severity of mitral regurgitationIf SAM is present, MR may be eccentric and is usually mid/late systolic
    A4C (PW TDI)Systolic (s'), early (e') and atrial (a') relaxation velocities at anterolateral LV annulusReduction in s' or e' velocities below normal range for age and sex (5)

    Assess for elevated LVEDp by measuring E/e'. Average septal and lateral velocities for e'. Abnormal if >10 (4)
    A4C (PW TDI)Systolic (s'), early (e') and atrial (a') relaxation velocities at inferoseptal LV annulusReduction in Sa or Ea velocities below normal range for age and sex (5)

    Assess for elevated LVEDp by measuring E/e'. Average septal and lateral velocities for e'. Abnormal if >10 (4)
    A5C and A3C (CFM)Locate turbulent flow both within the LV cavity and the LVOT
    A5C and A3C (PW/CW)Quantify LVOT/LV intracavity dynamic flow gradientSample PW Doppler throughout the LV cavity, paying particular attention to areas with turbulent flow. HPRF/CW Doppler may be appropriate if aliasing occurs. Take care not to include MR jet in sample volume. A Valsalva manoeuvre should be performed in the sitting and semi-supine position (and then on standing if no gradient is produced) to assess dynamic LVOT gradients. The peak gradient (rest or Valsalva) should be recorded. In addition, exercise stress echocardiography should be considered in patients with LVOT gradients <50 mmHg at rest (with or without Valsalva) (3)
    A2C (PW TDI)Systolic (s'), early (e') and atrial (a') relaxation velocities at inferior LV annulusReduction in s' or e' velocities below normal range for age and sex (5)
    A2C (PW TDI)Systolic (s'), early (e') and atrial (a') relaxation velocities at anterior LV annulusReduction in s' or e' velocities below normal range for age and sex (5)

Abbreviations

Views
 A2CApical two chamber
 A4CApical four chamber
 A5CApical five chamber
 A3CApical three chamber or apical long axis
 PLAXParasternal long axis
 PSAXParasternal short axis
 SCSubcostal
 SSNSuprasternal
Modality
 CFMColour flow Doppler
 CWContinuous-wave Doppler
 PWPulse wave Doppler
 TDITissue Doppler imaging
Measurement and explanatory text
 a'Lateral and/or septal late annular relaxation velocity
 AoAorta
 ASHAsymmetrical septal hypertrophy
 AVAortic valve
 BSABody surface area
 DTDeceleration time
 e'Lateral and/or septal early annular relaxation velocity
 HCMHypertrophic cardiomyopathy
 HPRFHigh pulse repetition frequency
 IVCInferior vena cava
 IVSdInterventricular septal width in diastole
 LALeft atrium
 LLPVLeft lower pulmonary vein
 LPALeft pulmonary artery
 LUPVLeft upper pulmonary vein
 LVLeft ventricle
 LVEDpLeft ventricular end-diastolic pressure
 LVIDd/sLeft ventricular internal dimension in diastole and systole
 LVOTLeft ventricular outflow tract
 LVPWdLeft ventricular posterior wall width in diastole
 MAPSEMitral annular plane systolic excursion
 MRMitral regurgitation
 MVMitral valve
 PAPulmonary artery
 PAPPulmonary artery pressure
 PHTPressure half-time
 PRPulmonary regurgitation
 PSPulmonary stenosis
 PVPulmonary valve
 RARight atrium
 RLPVRight lower pulmonary vein
 RUPVRight upper pulmonary vein
 RVRight ventricle
 RVHRight ventricular hypertrophy
 RVIDdRight ventricular cavity diameter in diastole
 RWMARegional wall motion abnormality
 RVOTRight ventricular outflow tract
 RVOTdRight ventricular outflow tract dimension
 s'Lateral and/or septal systolic annular velocity
 SAMSystolic anterior motion
 STJSinotubular junction
 SVolStroke volume
 TAPSETricuspid annular plane systolic excursion
 TRTricuspid regurgitation
 TVTricuspid valve
 VmaxMaximum velocity
 VSDVentricular septal defect
 VTIVelocity time integral

Declaration of interest

This manuscript was prepared by the British Society of Echocardiography Education Committee. The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of this guideline.

Funding

This guideline did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.

References

  • 1

    WhartonGSteedsRAllenJPhillipsHJonesRKanagalaPLloydGMasaniNMathewTOxboroughD 2015 A minimum dataset for a standard adult transthoracic echocardiogram: a guideline protocol from the British Society of Echocardiography. Echo Research and Practice 2 G9G24. doi:10.1530/ERP-14-0079

  • 2

    MaronBJMcKennaWJDanielsonGKKappenbergerLJKuhnHJSeidmanCEShahPMSpencerWHIIISpiritoPTen CateFJ 2003 American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. Journal of the American College of Cardiology 42 16871713. doi:10.1016/S0735-1097(03)00941-0

  • 3

    ElliottPMAnastasakisABorgerMABorggrefeMCecchiFCharronPHagegeAALafontALimongelliGMahrholdtH 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). European Heart Journal 35 27332779. doi:10.1093/eurheartj/ehu199

  • 4

    Masani N Wharton G Allen J Chambers J Graham J Jones R Rana B 2011 British Society of Echocardiography Guidelines for Chamber Quantification; http://www.bsecho.org/media/40506/chamber-final-2011_2_.pdf

  • 5

    NaguehSFAppletonCPGillebertTCMarinoPNOhJKSmisethOAWaggonerADFlachskampfFAPellikkaPAEvangelistaA 2009 Recommendations for the evaluation of left ventricular diastolic function by echocardiography. Journal of the American Society of Echocardiography 22 107133. doi:10.1016/j.echo.2008.11.023

(N Smith is the lead author)

(R Steeds is the Guidelines Chair)

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References

1

WhartonGSteedsRAllenJPhillipsHJonesRKanagalaPLloydGMasaniNMathewTOxboroughD 2015 A minimum dataset for a standard adult transthoracic echocardiogram: a guideline protocol from the British Society of Echocardiography. Echo Research and Practice 2 G9G24. doi:10.1530/ERP-14-0079

2

MaronBJMcKennaWJDanielsonGKKappenbergerLJKuhnHJSeidmanCEShahPMSpencerWHIIISpiritoPTen CateFJ 2003 American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. Journal of the American College of Cardiology 42 16871713. doi:10.1016/S0735-1097(03)00941-0

3

ElliottPMAnastasakisABorgerMABorggrefeMCecchiFCharronPHagegeAALafontALimongelliGMahrholdtH 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). European Heart Journal 35 27332779. doi:10.1093/eurheartj/ehu199

4

Masani N Wharton G Allen J Chambers J Graham J Jones R Rana B 2011 British Society of Echocardiography Guidelines for Chamber Quantification; http://www.bsecho.org/media/40506/chamber-final-2011_2_.pdf

5

NaguehSFAppletonCPGillebertTCMarinoPNOhJKSmisethOAWaggonerADFlachskampfFAPellikkaPAEvangelistaA 2009 Recommendations for the evaluation of left ventricular diastolic function by echocardiography. Journal of the American Society of Echocardiography 22 107133. doi:10.1016/j.echo.2008.11.023

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