Rajesh Beniwal and Maneesha Bhaya
Optimizing the Diagnosis of Subclinical Disease''
Echocardiography: The Inadequacy of World Health Organization Criteria for
Letter by Beniwal and Bhaya Regarding Article, ''Rheumatic Heart Disease Screening by
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Letter by Beniwal and Bhaya Regarding Article,
“Rheumatic Heart Disease Screening by
Echocardiography: The Inadequacy of World
Health Organization Criteria for Optimizing the
Diagnosis of Subclinical Disease”
To the Editor:
Marijon and colleagues1base their arguments on a phenomenon
not observed by them during their study on rheumatic heart disease
(RHD). They assume that “pathophysiologically, repeated rheumatic
carditis can result in subvalvular or valvular thickening before the
development of leaflet retraction and thereby regurgitation.” They
further state, “In adding the diagnostic criterion of morphological
valve changes, otherwise well defined in more advanced rheumatic
valve lesions, we have detected many children with subclinical RHD
but without significant valve regurgitation who would not be eligible
for secondary RHD prophylaxis under current international
A phenomenon of dissociation between mild- to moderate-grade
RHD-like valve abnormalities and significant regurgitation in about
a fifth of the cases has been reported recently by Bhaya et al.2They
had adopted a strategy of universal definitive echocardiography for
all of the enrolled schoolchildren without any intervening screening
round of auscultation or echocardiography to remove any chance of
selection bias while attempting to evaluate and improvise on the
World Health Organization criteria for probable RHD.
Marijon et al had adopted a strategy of an initial screening round
of echocardiography to identify cases with any regurgitation (phys-
iological/significant) of mitral and/or aortic valves followed by a
round of definitive echocardiography. Thus, they would have missed
a large fraction of cases having valvular abnormalities like coexisting
valve thickening without regurgitation during the screening round.
This, we think, was an inadvertent methodological error.
In our experience, mitral valve prolapse is diagnosed in ?1% to
4% of children screened for RHD by echocardiography.2,3For mitral
valve prolapse, significant regurgitation is a late feature, but it is not
unusual to find thickened valves quite early in the course of disease.
Thus, thickened valve leaflets in a child without significant regurgi-
tation is more likely to be of nonrheumatic origin in our opinion.
This fact is also corroborated by an autopsy study.4
In areas of low prevalence, the presentation of subclinical RHD
may be different,2and presentation and progression of the disease
process leading to RHD in a given age group can be variable in
different geographic areas because of differing environmental deter-
minants.5Consequently, the criteria proposed by Marijon et al may
be important for the surveyed population of schoolchildren in
Mozambique, but these criteria are difficult to adopt universally. If a
diagnosis of RHD were to be based on the combined criteria
proposed by Marijon et al, many cases with only significant
regurgitation as evidence of disease would be missed. Such cases
might be much greater in number as reported by Bhaya et al.2
Once acute rheumatic fever has been diagnosed, antibiotic therapy
is offered to prevent recurrences, but it is unlikely that antibiotic
therapy prevents the progression of the immune processes leading to
RHD.6The recent addition of statins to the possible options for
pharmacotherapy of RHD necessitates that a diagnostic criterion for
subclinical RHD should identify the patients in the state when
significant regurgitation is due to softening of valves secondary to
the disease process and well before the time when repeated rheu-
matic carditis results in chronicity of significant regurgitation due to
scarring and retraction of leaflets.
We feel that focusing on significant regurgitation and not valve
deformities would make echocardiographic criteria for diagnosis of
subclinical RHD more sensitive and universally applicable.
Rajesh Beniwal, MBBS, MD
National Institute of Occupational Health
Maneesha Bhaya, MBBS, MD
Sardar Patel Medical College
1. Marijon E, Celermajer DS, Tafflet M, El-Haou S, Jani DN, Ferreira B,
Mocumbi A, Paquet C, Sidi D, Jouven X. Rheumatic heart disease
screening by echocardiography: the inadequacy of World Health
Organization criteria for optimizing the diagnosis of subclinical
disease. Circulation. 2009;120:663–668.
2. Bhaya M, Panwar RB, Beniwal R, Panwar S. Echocardiographic evidence
of significant regurgitation can be the sole criterion for diagnosis of
probable rheumatic heart disease: experience from a large cross-sectional
survey. J Am Coll Cardiol. 2009;53:A409. Abstract.
3. Periwal KL, Gupta BK, Panwar RB, Khatri PC, Raja S, Gupta R. Prev-
alence of rheumatic heart disease in school children in Bikaner: an
echocardiographic study. J Assoc Physicians India. 2006;54:279–282.
4. Roberts WC, Ko JM. Some observations on mitral and aortic valve
disease. Proc (Bayl Univ Med Cent). 2008;21:282–299.
5. Carapetis JR, Currie BJ, Mathews JD. Cumulative incidence of rheumatic
fever in an endemic region: a guide to the susceptibility of the population?
Epidemiol Infect. 2000;124:239–244.
6. Ben-Yehuda O, DeMaria AN. Statins in rheumatic heart disease: taking
the bite out? J Am Coll Cardiol. 2009;53:1880–1882.
© 2010 American Heart Association, Inc.
Circulation is available at http://circ.ahajournals.orgDOI: 10.1161/CIRCULATIONAHA.109.908129
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