ARVC


Lead Authors; Dr. David Oxborough, Dr Abbas Zaidi, Prof Sanjay Sharma, Prof John Somauroo Education Committee Authors, Dr Rick Steeds (Chair), Will Bradlow, Alison Carr, Richard Jones, Prathap Kanagala, Daniel Knight, Guy Lloyd, Thomas Mathew, Navroz Masani, Kevin O’Gallagher, Bushra Rana, Liam Ring, Julie Sandoval, Martin Stout, Gill Wharton, Richard Wheeler

Download ARVC protocol

Introduction

Published May 2013

Assessment of the right ventricle (RV) is often challenging and sometimes overlooked, however recent guideline documentationfrom the American Society of Echocardiography suggested a measure of RV structure and function should be mandatory in allclinical reports*. The BSE advocates RV assessment within the minimum dataset; however in certain conditions such asarrhythmogenic right ventricular cardiomyopathy (ARVC), pulmonary hypertension, pulmonary embolism, RV myocardial infarctionand athletic heart syndrome a more comprehensive assessment of the RV is required. RV assessment can be described in terms ofRV dimensions, structure and function and the assessment of ARVC utilises this approach. It is clear that with other RV pathologythe measurements are similar but their interpretation should be taken in the clinical context.

ARVC is one of the most common and under-diagnosed causes of cardiac sudden death in a young person and therefore anappropriate diagnosis is crucial. Echocardiography has variable sensitivity and specificity for the diagnosis of ARVC and thereforeonly forms a small part of the complete diagnosis. Corroborative investigations are key and include a comprehensive history, clinicalexamination, electrocardiogram, magnetic resonance imaging and genetic testing all contributing to the overall assessment.Echocardiographic criteria demonstrated in isolation should be interpreted with caution and therefore although this document is aprotocol for RV assessment per se, it should be used only as part of the assessment for ARVC.

 

Table 1- Echocardiographic criteria for ARVC (adapted from Marcus et al 2010)

MAJOR ECHOCARDIOGRAPHIC CRITERIA FOR ARVC
Regional RV Dyskinesia or Aneurysm
And one of the following

PLAX RVOT = 32mm (corrected for body size [PLAX/BSA] = 19mm/m2)

PSAX RVOT = 36mm (corrected for body size [PLAX/BSA] = 21mm/m2)

Or
Fractional Area Change = 33%

 

MINOR ECHOCARDIOGRAPHIC CRITERIA FOR ARVC
Regional RV Akinesia or Dyskinesia
And one of the following

PLAX RVOT = 29 to < 32mm (corrected for body size [PLAX/BSA] = 16 to < 19mm/m2)

PSAX RVOT = 32 to < 36mm (corrected for body size [PLAX/BSA] = 18 to 21mm/m2)

Or
Fractional Area Change > 33 to < 40%

 

ADDITIONAL NOTES

• These values should be interpreted with caution in the athletic population‡

• RV akinesia, dyskinesia or aneurysm are diagnostic criteria in the presence of RV dilatation or reduced RV fractional area change**

• Assess the LV in line with the BSE minimum dataset - LV involvement may occur early in the course of the disease†

 

*Rudski, L. G., Lai, W. W., Afilalo, J., Hua, L., Handschumacher, M. D., Chandrasekaran, K., Solomon, S. D., Louie, E. K. & Schiller, N. B. 2010. Guidelines forthe Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography: Endorsed by the European Association ofEchocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. Journal of the American Society ofEchocardiography, 23, 685-713.

** Marcus, F. I., Mckenna, W. J., Sherrill, D., Basso, C., Bauce, B., Bluemke, D. A., Calkins, H., Corrado, D., Cox, M. G. P. J., Daubert, J. P., Fontaine, G., Gear, K.,Hauer, R., Nava, A., Picard, M. H., Protonotarios, N., Saffitz, J. E., Sanborn, D. M. Y., Steinberg, J. S., Tandri, H., Thiene, G., Towbin, J. A., Tsatsopoulou, A.,Wichter, T. & Zareba, W. 2010. Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. Circulation, 121, 1533-1541.

† Sen-Chowdhry S, Syrris P, Prasad SK, Hughes SE, Merrifield R, Ward D, Pennell DJ, McKenna WJ. Left-dominant arrhythmogenic cardiomyopathy: an underrecognizedclinical entity. J Am Coll Cardiol. 2008;52:2175-2187.

‡ Oxborough D, Sharma S, Shave R, Whyte G, Birch K, Artis N, Batterham A, George K The right ventricle of the endurance athlete: the relationship betweenmorphology and deformation. J Am Soc Echocardiogr - 25(3):263-271

  VIEW MODALITY MEASUREMENTS EXPLANATORY NOTE IMAGE

PLAX

2D

RVOT PLAX

Qualitative regional wall motion analysis ofthe anterior wall of the RV

- end diastole*

- adjust depth and focal zone to visualise RVOT.

- for consistency, ideally, this measurement should be taken at a similar level to RVOT1 measurement of PSAX AV view. Hence RVOT PLAX should be a measurement perpendicular line from the RV anterior wall to the level of the aortic valve.

- all 2D measurements should be blood tissue interface to blood tissue interface

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RVOT PLAX = 32mm or = 19mm/m2 AND the presence of regional RV akinesia, dyskinesia or aneurysm is a major criterion**

RVOT PLAX = 29mm to < 32mm OR =16mm/m2 to <19mm/m2 AND the presence of regional RV akinesia or dyskinesia is a minor criterion**

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PLAX RV inflow

2D

Qualitative regional wall motion analysis of the anterior and inferior walls of the RV

- ensure the ventricular septum has been excluded and the true inferior wall is seen (diaphragm and liver in view)

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PLAX RV inflow

Colour Flow Doppler

Assess the severity of tricuspid regurgitation and estimate RV systolic pressure (for details see pulmonary hypertension dataset)

The presence of TR is not a sensitive or specific finding for ARVC however severe functional TR may occur in the presence of RV dilatation and dysfunction

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CW Doppler

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PSAX AV level

2D

Proximal RVOT (RVOT1)

Qualitative assessment of RV structure and function

Regional wall motion analysis of the outflow tract of the RV (infundibulum)

- at end diastole*

- measured from anterior aortic wall directly up to the RV free wall (at the level of the aortic valve)

- the PSAX view has been shown to be more reproducible than the measurement obtained from the PLAX orientation RVOT1 = 36mm or = 21mm/m2 in the presence of regional RV akinesia, dyskinesia or aneurysm is a major criterion**

RVOT1 = 32mm to < 36mm or = 18mm/m2to <21mm/m2 in the presence of regional RV akinesia or dyskinesia is a minor criterion**

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PSAX PV level

2D

Distal RVOT (RVOT2)

Qualitative assessment of RV structure and function

Regional wall motion analysis of the infundibulum of the RV

PA diameter

- end diastole*

- measured just proximal to PV

There are no specific values for diagnosis of ARVC however this should be used to demonstrate dilatation.

RVOT2 > 27mm is abnormal in other cardiac pathology*

- end diastole

- half way between pulmonary valve (PV) and bifurcation of main PA or 1cm distal to PV

Enlargement of the pulmonary artery makes the diagnosis of ARVC less likely (may be indicative of conditions causing pulmonary hypertension)

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PSAX Base

2D

Qualitative assessment of RV structure and function at basal level

Regional wall motion analysis of inferior, lateral, anterior and septal walls of RV in PSAX at base (mitral valve) level

Relative size of RV to LV should be assessed

There is disproportionate enlargement of the RV in ARVC

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PSAX Mid

2D

Qualitative assessment of RV structure and function at papillary muscle level

Regional wall motion analysis of inferior, lateral, anterior and septal walls of RV in PSAX at mid (papillary muscle) level

Relative size of RV to LV should be assessed

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PSAX Apex

Qualitative assessment of RV structure and function at the apex

Regional wall motion analysis of inferior, lateral and septal walls of RV in PSAX at apex level

Relative size of RV to LV should be assessed

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Apical 4Ch Focused RV

2D

RVD1 - Basal RV diameter (end diastole at the maximal value within the first third of the RV)*

RVD2 - Mid RV diameter (end diastole in the middle third of the RV at the level of the LV papillary muscles)

Focused RV 4CH view is obtained by ensuring:

  1. true apex is visualised, with scan plane positioned through the LV in the centre of the cavity
  2. RV is not foreshortened and LVOT is not opened
  3. largest RV dimensions are optimised while maintaining 'on axis' view, as described above (for further clarification see ASE RV guidelines*)

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RVD3 - RV length (end diastole from tricuspid annulus to the RV apex)

There are no specific values for diagnosis of ARVC however all RV measurements should be used to demonstrate dilatation.

RVD1 > 42mm,RVD2 > 35mm and RVD3 > 86mm are abnormal*

- trace around the endocardium of the RV lateral wall at end diastole and end systole.

- do not trace around individual trabeculations, which should be included within the cavity area.)

Click to enlarge

Fractional Area Change (FAC)

Qualitative assessment of RV structure and longitudinal function

FAC = 33% in the presence of regional RV akinesia, dyskinesia or aneurysm is a major criterion** even in the presence of normal RVOT size.

FAC > 33% to = 40% in the presence of regional RV akinesia or dyskinesia is a minor criterion** even in the presence of normal RVOT size.

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AP4CH

M-mode

Tricuspid Plane Systolic Excursion (TAPSE)

Ensure correct alignment of RV, such that RV base moves perpendicular to scan plane and is not oblique. The latter will cause a falsely reduced TAPSE value

There are no specific values for diagnosis of ARVC however TAPSE should be used to demonstrate longitudinal dysfunction. TAPSE < 16mm is abnormal*

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AP4CH

PW Doppler

E and A wave peak velocities for RV diastolic function using trans-tricuspid PW Doppler (optional)

There are no specific values for diagnosis of ARVC however diastolic dysfunction may indicate early changes in overall RV function. E < 0.35cm/s and E:A ratio < 0.8 may indicate impairment in diastolic filling*

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AP4CH

Tissue Doppler

Systolic (S'), early (E') and atrial (A') relaxation velocitiesat lateral TV annulus

There are no specific values for diagnosis of ARVC however TDI should be used to demonstrate longitudinal systolic and/or diastolic dysfunction. s' < 10cm/s, e' < 8cm/s and A' < 7cm/s are abnormal* .An E/e' of > 6 may be consistent with an elevated RA pressure.

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Modified AP4CH

(medial movement of the angle of the ultrasound beam)

Colour Flow Doppler

 

CW Doppler

Assess the severity of Triscuspid Regurgitation and estimate RV systolic pressure

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Useful additional parameters

in standard AP4CH

2D

Basal RV:LV ratio at end diastole

There are no specific values for diagnosis of ARVC however the measurement may be used to demonstrate RV dilatation.  RV:LV ration > 0.66 is abnormal*

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Qualitative assessment of RV structure and longitudinal function.

A thickened or echo-bright moderator band is not specific for ARVC bu may support the diagnosis in hte presence of other findings.

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Detection of regional RV dyskinesia or aneurysm formation is part of hte major echocardioggraphic criteria for ARVC

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RV area at ventricular end systole

There are no specific values for diagnosis of ARVC however the measurement should be used to demonstrate RA dilatation.  RA area > 18cm2 is abnormal*

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Apical 5CH

2D

Identify thickened moderator band

Outflow tract of the RV (infundibulum)/thickened moderator band is not specific for ARVC but may suppoert the diagnosis inthe presence of other findings

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Sub costal

2D

Qualitative assessment of TV structure and function

Regional wall motion analysis of inferior wall of RV

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RV wall thickness

- at end diastole

- ignore trabeculations and papillary muscles

- use reduced depth to improve resolution and measurment accuracy

 

There are no specific values for diagnosis of ARVC however the measurement should be used to demonstrate RV thinning <3mm.  RV wall thickness >5mm is consistent with RV hypertrophy.*

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IVC size and inspiratory collapse

Estimate of RA pressure to define RV end systolic pressure (see pulmonary hypertension protocols for details).

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Sub-costal

Colour Flow Doppler

Assess the severity of Tricuspid Regurgitation and estimate RV systolic pressure

The presence of TR is not a sensitive or specific finding for ARVC however significant funtional TR may occur in the presence of RV dilatation and dysfunction

Click to enlarge

Sub-costal

CW Doppler

May perform if good Doppler alignment of Triscuspid Regurgitation jet direction

Click to enlarge

Copyright © 2011 British Society of Echocardiography