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Echocardiography-based screening for rheumatic heart disease : What does borderline mean?
International Journal of Cardiology 203 (2016) 1003–1004
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Correspondence
Echocardiography-based screening for rheumatic heart disease : What does borderline mean? Raoul Bacquelin a, Muriel Tafflet a, Bernard Rouchon b, Nina Guillot b, Eloi Marijon a,c,d, Xavier Jouven a,c,d, Mariana Mirabel a,c,d,⁎ a
INSERM U970, Paris Cardiovascular Research Center PARCC, Paris, France Agence Sanitaire et Sociale de Nouvelle Calédonie, Nouméa, New Caledonia Université Paris Descartes, Sorbonne Paris Cité, Paris, France d Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France b c
a r t i c l e
i n f o
Article history: Received 9 November 2015 Accepted 16 November 2015 Available online 18 November 2015 Keywords: Rheumatic heart disease Acute rheumatic fever Echocardiography Indigenous health
Rheumatic heart disease (RHD) is a major challenge in developing countries and echocardiography-based screening is more sensitive than clinical screening to detect mild lesions [1]. Given the increasing number of publications in the field [2], an evidence-based consensus was published by the World Heart Federation (WHF) to standardize diagnostic criteria [3]. These criteria include subtle morphological features of RHD of the mitral and aortic valves and Doppler assessment of mitral stenosis, mitral regurgitation and aortic regurgitation. To fulfil pathological mitral valve morphology, two of the following criteria need to be met: anterior mitral valve thickening ≥3 mm (under 21 years of age); chordal thickening; restricted leaflet motion; and excessive leaflet tip motion during systole. Aimed at echocardiography-based RHD screening in endemic regions, three diagnoses are driven from the WHF criteria: “definite RHD”, “borderline RHD” and “no RHD” [3]. Definite RHD is most often diagnosed by the combination of mitral valve regurgitation and morphological criteria [4,5]. The reliability of the diagnosis of ‘borderline RHD' is however subject of debate, essentially due to the subjective assessment of some of the morphological features (e.g., mitral valve chordal thickening, mild leaflet motion abnormalities). Furthermore, the management of children with these very subtle changes has not been fully addressed [6,7].
⁎ Corresponding author at: Paris Cardiovascular Research Centre, Inserm U970, 56 rue Leblanc, 75737 Paris CEDEX 15, France. E-mail address: [email protected] (M. Mirabel).
http://dx.doi.org/10.1016/j.ijcard.2015.11.110 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved
The purpose of the present study was to assess the robustness of WHF echocardiographic criteria by studying the variability of interpretation of serial testing at short time intervals (b 12 months) in highrisk children initially screened positive for subclinical RHD. Rheumatic heart disease remains endemic in the Pacific [8,9]. A nationwide echobased screening programme was launched in New Caledonia, a South Pacific archipelago, in 2008 and a cohort of children was followed-up [4]. Two independent readers (RB, MM) blinded to each other's results and clinical data analysed serial standardized echocardiograms (VIVID I, General Electrics®) [5]. Ethical clearance was granted from the Bordeaux and French Overseas Protection Committee and the French Institute of Medical Research and Health. The study complies with the declaration of Helsinki. All children and their parents/guardians gave informed consent. Forty-seven children underwent two echocardiograms at a mean 5.5 (SD 2.4) months during follow-up clinics. Mean age was 12.5 (SD 1.5) years old and the sex ratio (M/F) was 1.04. Ninety-four echocardiograms were available for analysis. Inter-observer variability was overall good with kappa values of 0.83 (95% CI 0.74–0.93). No echocardiogram interpreted as “definite RHD” by one observer was read as “no RHD” by the other. Inter-observer disagreement was present between “borderline RHD” and “definite RHD”; and between “borderline RHD” and “no RHD”. Inter-observer agreement for the Doppler criteria fulfilling pathological mitral regurgitation was excellent: kappa 0.92 (95% CI 0.83–1.00). Inter-observer variability was good when focusing on morphological mitral valve changes with a kappa value of 0.83 (95% CI 0.72–0.94) for discrimination between normal and pathological mitral valve morphology. When taken individually, the inter-observer variability of the mitral valve morphological criteria was fair: kappa 0.53 (95% CI 0.35–0.70) for restricted leaflet motion; 0.62 (95% CI 0.46–0.78) for chordal thickening; kappa 0.60 (95% CI 0.43–0.77) for thickened anterior mitral leaflet. Among the 47 participants, 16 were diagnosed with “definite RHD”, 11 with ‘borderline RHD’ and twenty children with no RHD according to the one experienced reader (MM). Classification varied after a short period of time in the subset of children diagnosed with features of “borderline” RHD. At 5 months follow-up, among the 11 initial “borderline RHD”, 3/11 (27.3%) remained “borderline RHD”, 4/11 (36.4%) of them were classified as “definite RHD” and 4/11 (36.4%) reverted to “no
1004
Correspondence
Fig. Interpretation of follow-up echocardiograms in children diagnosed with “borderline” RHD by two independent readers. RHD indicates rheumatic heart disease, FU indicates follow-up.
RHD” according to the readings of one of the authors (MM). The variability was greater if a second cardiologist (RB) read the follow-up scans (Fig.). The inter-study variability was high in children initially diagnosed with “borderline” RHD with kappa values ranging from 0.47 (95% CI 0.27–0.67) to 0.56 (95% CI 0.38–0.74) according to the two readers. Timing intervals between the two studies did not influence variability (data not shown). Rheumatic heart disease has regained interest with the advent of echocardiography-based surveys in endemic countries [1]. Interpretation of very mild echocardiographic changes in asymptomatic highrisk children is however challenging [10]. We demonstrate here that inter-observer and inter-study variability is high when it comes to the diagnosis of “borderline” RHD. Our results should prompt caution in the field of RHD screening given the variability of “borderline RHD” in terms of echocardiographic diagnosis a few months apart and among readers. Further studies are warranted to assess the significance of “borderline RHD”. Conflict of interest The authors report no relationships that could be construed as a conflict of interest.
References [1] E. Marijon, M. Mirabel, D.S. Celermajer, X. Jouven, Rheumatic heart disease, Lancet 379 (2012) 953–964. [2] M. Rothenbuhler, C.J. O'Sullivan, S. Stortecky, et al., Active surveillance for rheumatic heart disease in endemic regions: a systematic review and meta-analysis of prevalence among children and adolescents, Lancet Glob. Health 2 (2014) e717–e726. [3] B. Remenyi, N. Wilson, A. Steer, et al., World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease—an evidence-based guideline, Nat. Rev. Cardiol. 9 (2012) 297–309. [4] M. Mirabel, T. Fauchier, R. Bacquelin, et al., Echocardiography screening to detect rheumatic heart disease: a cohort study of schoolchildren in French Pacific Islands, Int. J. Cardiol. 188 (2015) 89–95. [5] M. Mirabel, R. Bacquelin, M. Tafflet, et al., Screening for rheumatic heart disease: evaluation of a focused cardiac ultrasound approach, Circ. Cardiovasc. Imaging 8 (2015) http://dx.doi.org/10.1161/CIRCIMAGING.114.002324. [6] E. Marijon, D.S. Celermajer, X. Jouven, Management of patients with subclinical rheumatic heart disease, Int. J. Cardiol. 134 (2009) 295–296. [7] M. Remond, D. Atkinson, A. White, et al., Are minor echocardiographic changes associated with an increased risk of acute rheumatic fever or progression to rheumatic heart disease? Int. J. Cardiol. 198 (2015) 117–122. [8] T. Fauchier, M. Tafflet, G. Filitoga, et al., Acute rheumatic fever: a population-based study in wallis, a South Pacific Island, Int. J. Cardiol. 181 (2015) 30–31. [9] M. Mirabel, M. Tafflet, B. Noel, et al., Newly diagnosed rheumatic heart disease among indigenous populations in the Pacific, Heart 101 (2015) 1901–1906. [10] M. Mirabel, D.S. Celermajer, B. Ferreira, et al., Screening for rheumatic heart disease: evaluation of a simplified echocardiography-based approach, Eur. Heart J. Cardiovasc. Imaging 13 (2012) 1024–1029.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Correspondence
Echocardiography-based screening for rheumatic heart disease : What does borderline mean? Raoul Bacquelin a, Muriel Tafflet a, Bernard Rouchon b, Nina Guillot b, Eloi Marijon a,c,d, Xavier Jouven a,c,d, Mariana Mirabel a,c,d,⁎ a
INSERM U970, Paris Cardiovascular Research Center PARCC, Paris, France Agence Sanitaire et Sociale de Nouvelle Calédonie, Nouméa, New Caledonia Université Paris Descartes, Sorbonne Paris Cité, Paris, France d Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France b c
a r t i c l e
i n f o
Article history: Received 9 November 2015 Accepted 16 November 2015 Available online 18 November 2015 Keywords: Rheumatic heart disease Acute rheumatic fever Echocardiography Indigenous health
Rheumatic heart disease (RHD) is a major challenge in developing countries and echocardiography-based screening is more sensitive than clinical screening to detect mild lesions [1]. Given the increasing number of publications in the field [2], an evidence-based consensus was published by the World Heart Federation (WHF) to standardize diagnostic criteria [3]. These criteria include subtle morphological features of RHD of the mitral and aortic valves and Doppler assessment of mitral stenosis, mitral regurgitation and aortic regurgitation. To fulfil pathological mitral valve morphology, two of the following criteria need to be met: anterior mitral valve thickening ≥3 mm (under 21 years of age); chordal thickening; restricted leaflet motion; and excessive leaflet tip motion during systole. Aimed at echocardiography-based RHD screening in endemic regions, three diagnoses are driven from the WHF criteria: “definite RHD”, “borderline RHD” and “no RHD” [3]. Definite RHD is most often diagnosed by the combination of mitral valve regurgitation and morphological criteria [4,5]. The reliability of the diagnosis of ‘borderline RHD' is however subject of debate, essentially due to the subjective assessment of some of the morphological features (e.g., mitral valve chordal thickening, mild leaflet motion abnormalities). Furthermore, the management of children with these very subtle changes has not been fully addressed [6,7].
⁎ Corresponding author at: Paris Cardiovascular Research Centre, Inserm U970, 56 rue Leblanc, 75737 Paris CEDEX 15, France. E-mail address: [email protected] (M. Mirabel).
http://dx.doi.org/10.1016/j.ijcard.2015.11.110 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved
The purpose of the present study was to assess the robustness of WHF echocardiographic criteria by studying the variability of interpretation of serial testing at short time intervals (b 12 months) in highrisk children initially screened positive for subclinical RHD. Rheumatic heart disease remains endemic in the Pacific [8,9]. A nationwide echobased screening programme was launched in New Caledonia, a South Pacific archipelago, in 2008 and a cohort of children was followed-up [4]. Two independent readers (RB, MM) blinded to each other's results and clinical data analysed serial standardized echocardiograms (VIVID I, General Electrics®) [5]. Ethical clearance was granted from the Bordeaux and French Overseas Protection Committee and the French Institute of Medical Research and Health. The study complies with the declaration of Helsinki. All children and their parents/guardians gave informed consent. Forty-seven children underwent two echocardiograms at a mean 5.5 (SD 2.4) months during follow-up clinics. Mean age was 12.5 (SD 1.5) years old and the sex ratio (M/F) was 1.04. Ninety-four echocardiograms were available for analysis. Inter-observer variability was overall good with kappa values of 0.83 (95% CI 0.74–0.93). No echocardiogram interpreted as “definite RHD” by one observer was read as “no RHD” by the other. Inter-observer disagreement was present between “borderline RHD” and “definite RHD”; and between “borderline RHD” and “no RHD”. Inter-observer agreement for the Doppler criteria fulfilling pathological mitral regurgitation was excellent: kappa 0.92 (95% CI 0.83–1.00). Inter-observer variability was good when focusing on morphological mitral valve changes with a kappa value of 0.83 (95% CI 0.72–0.94) for discrimination between normal and pathological mitral valve morphology. When taken individually, the inter-observer variability of the mitral valve morphological criteria was fair: kappa 0.53 (95% CI 0.35–0.70) for restricted leaflet motion; 0.62 (95% CI 0.46–0.78) for chordal thickening; kappa 0.60 (95% CI 0.43–0.77) for thickened anterior mitral leaflet. Among the 47 participants, 16 were diagnosed with “definite RHD”, 11 with ‘borderline RHD’ and twenty children with no RHD according to the one experienced reader (MM). Classification varied after a short period of time in the subset of children diagnosed with features of “borderline” RHD. At 5 months follow-up, among the 11 initial “borderline RHD”, 3/11 (27.3%) remained “borderline RHD”, 4/11 (36.4%) of them were classified as “definite RHD” and 4/11 (36.4%) reverted to “no
1004
Correspondence
Fig. Interpretation of follow-up echocardiograms in children diagnosed with “borderline” RHD by two independent readers. RHD indicates rheumatic heart disease, FU indicates follow-up.
RHD” according to the readings of one of the authors (MM). The variability was greater if a second cardiologist (RB) read the follow-up scans (Fig.). The inter-study variability was high in children initially diagnosed with “borderline” RHD with kappa values ranging from 0.47 (95% CI 0.27–0.67) to 0.56 (95% CI 0.38–0.74) according to the two readers. Timing intervals between the two studies did not influence variability (data not shown). Rheumatic heart disease has regained interest with the advent of echocardiography-based surveys in endemic countries [1]. Interpretation of very mild echocardiographic changes in asymptomatic highrisk children is however challenging [10]. We demonstrate here that inter-observer and inter-study variability is high when it comes to the diagnosis of “borderline” RHD. Our results should prompt caution in the field of RHD screening given the variability of “borderline RHD” in terms of echocardiographic diagnosis a few months apart and among readers. Further studies are warranted to assess the significance of “borderline RHD”. Conflict of interest The authors report no relationships that could be construed as a conflict of interest.
References [1] E. Marijon, M. Mirabel, D.S. Celermajer, X. Jouven, Rheumatic heart disease, Lancet 379 (2012) 953–964. [2] M. Rothenbuhler, C.J. O'Sullivan, S. Stortecky, et al., Active surveillance for rheumatic heart disease in endemic regions: a systematic review and meta-analysis of prevalence among children and adolescents, Lancet Glob. Health 2 (2014) e717–e726. [3] B. Remenyi, N. Wilson, A. Steer, et al., World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease—an evidence-based guideline, Nat. Rev. Cardiol. 9 (2012) 297–309. [4] M. Mirabel, T. Fauchier, R. Bacquelin, et al., Echocardiography screening to detect rheumatic heart disease: a cohort study of schoolchildren in French Pacific Islands, Int. J. Cardiol. 188 (2015) 89–95. [5] M. Mirabel, R. Bacquelin, M. Tafflet, et al., Screening for rheumatic heart disease: evaluation of a focused cardiac ultrasound approach, Circ. Cardiovasc. Imaging 8 (2015) http://dx.doi.org/10.1161/CIRCIMAGING.114.002324. [6] E. Marijon, D.S. Celermajer, X. Jouven, Management of patients with subclinical rheumatic heart disease, Int. J. Cardiol. 134 (2009) 295–296. [7] M. Remond, D. Atkinson, A. White, et al., Are minor echocardiographic changes associated with an increased risk of acute rheumatic fever or progression to rheumatic heart disease? Int. J. Cardiol. 198 (2015) 117–122. [8] T. Fauchier, M. Tafflet, G. Filitoga, et al., Acute rheumatic fever: a population-based study in wallis, a South Pacific Island, Int. J. Cardiol. 181 (2015) 30–31. [9] M. Mirabel, M. Tafflet, B. Noel, et al., Newly diagnosed rheumatic heart disease among indigenous populations in the Pacific, Heart 101 (2015) 1901–1906. [10] M. Mirabel, D.S. Celermajer, B. Ferreira, et al., Screening for rheumatic heart disease: evaluation of a simplified echocardiography-based approach, Eur. Heart J. Cardiovasc. Imaging 13 (2012) 1024–1029.