Correspondence
Ria Novosti/Science Photo Library
rather than a specific phenotype. This discrepancy is highlighted by the fact that patients with mixed phenotypes seem to have a worse prognosis than do those with idiopathic hypertrophic cardiomyopathy or malformation syndromes, yet these phenotypes can occur in patients with various underlying disorders, including inherited errors of metabolism and neuromuscular disease, but also malformation syndromes and idiopathic disease. Furthermore, the new category in this study, mixed hypertrophic cardiomyopathy, includes some patients identified as having idiopathic hypertrophic cardiomyopathy or hypertrophic cardiomyopathy associated with inherited errors of metabolism, malformation syndromes, or neuromuscular disorders in the authors’ previous reports,4 in which attempts at risk stratification on the basis of phenotype were also made. Finally, to develop a specific riskstratification algorithm applicable to each phenotypic category is problematic, because of the small numbers in each group and, in particular, the heterogeneous and nonuniform nature of the hypertrophic cardiomyopathy groups compared.5 Ultimately, heart failure at presentation seems to be the main determinant of the outcome, particularly in infancy, irrespective of the phenotype. Paediatric cardiomyopathies are a very heterogeneous group of disorders, and early diagnosis and aggressive clinical management are needed in children with specific aetiologies and heart failure at presentation. Although this study is an important attempt to describe real clinical scenarios, we believe that the emphasis on the phenotype alone could be misleading and future studies should concentrate on determining the specific aetiology, which is likely to be a stronger determinant of the natural history and outcome of hypertrophic cardiomyopathy in the paediatric population. 782
We declare that we have no competing interests.
*Juan Pablo Kaski, Giuseppe Limongelli [email protected] Inherited Cardiovascular Diseases Unit, Department of Cardiology, Great Ormond Street Hospital, London WC1N 3JH, UK (JPK); Institute of Cardiovascular Science, University College London, London, UK (JPK); and Monaldi Hospital, Second University of Naples, Naples, Italy (GL) 1
2
3
4
5
Lipshultz SE, Orav EJ, Wilkinson JD, et al. Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet 2013; 382: 1889–97. Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med 2003; 348: 1647–55. Nugent AW, Daubeney PE, Chondros P, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 2003; 348: 1639–46. Colan SD, Lipshultz SE, Lowe AM, et al. Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation 2007; 115: 773–81. Weintraub RG, Semsarian C. Paediatric cardiomyopathy: getting to the heart of the matter. Lancet 2013; 382: 1866–67.
Authors’ reply We thank Juan Pablo Kaski and Giuseppe Limongelli for their thoughtful comments about our Article. 1 We appreciate their recognition of the important findings from the Pediatric Cardiomyopathy Registry. Kaski and Limongelli agree that cardiomyopathy in childhood is phenotypically heterogeneous, but they state that their major issue is that the outcome of patients is mainly determined by the underlying aetiology rather than a specific phenotype. We agree that the cause of cardiomyopathy might be an important prognostic factor, but the cause must be identified quickly and accurately for it to affect management and subsequent prognosis. Unfortunately, this is not always possible. Despite the availability of a detailed comprehensive algorithm to determine the cause of pediatric cardiomyopathies,2 a 2006 Pediatric Cardiomyopathy Registry study showed that children with hypertrophic cardiomyopathy underwent causal testing only about half as often as children with other cardiomyopathies.3
Also, genetic testing for paediatric hypertrophic cardiomyopathy, which might greatly reduce the prevalence of idiopathic hypertrophic cardiomyopathy as a diagnosis, is still limited. In a rigorous analysis, we noted that causation was established in some of these children only years after presentation, and even then, nearly 75% of cases remained classified as idiopathic.4 In another disparity between physician beliefs about the importance of causal testing, we showed no differences in outcomes between children with myocarditis diagnosed by cardiac biopsy (using the Dallas criteria as the reference standard) and those who were diagnosed clinically by an experienced paediatric cardiologist.5 In another Pediatric Cardiomyopathy Registry report,6 we reference nine gene mutations in children with both Noonan syndrome and hypertrophic cardiomyopathy. These advances in identification of specific genetic causes are tempered by the fact that their associations with clinical outcomes have yet to be determined. Our data from the Pediatric Cardiomyopathy Registry show the importance of non-causal characteristics, such as presentation in infancy, heart failure at diagnosis, and specific echocardiographic abnormalities. 1 Indeed, in our Article, 1 patients were stratified both by functional phenotype (pure hypertrophic cardiomyopathy vs mixed hypertrophic cardiomyopathy with either dilated or restrictive features) and known aetiology, whereas additional risk stratification was on the basis of family history and clinical and echocardiographic characteristics. While we await results from new causal studies, including ongoing Pediatric Cardiomyopathy Registry studies of hypertrophic cardiomyopathy, particularly the discovery of genetic causes and their associations with outcomes, we believe that our report1 provides the best information available to help paediatric cardiologists to www.thelancet.com Vol 383 March 1, 2014
Correspondence
evaluate children newly diagnosed with hypertrophic cardiomyopathy. We declare that we have no competing interests.
*Steven E Lipshultz, E John Orav, James D Wilkinson, on behalf of the Pediatric Cardiomyopathy Registry Study Group [email protected] Department of Pediatrics, Wayne State University School of Medicine, Detroit, MI, USA (SEL); Department of Pediatrics, Leonard M Miller School of Medicine, University of Miami, Miami, FL 33101, USA (SEL, JDW); and Harvard Medical School, Boston, MA, USA (EJO) 1
2
3
4
5
6
Lipshultz SE, Orav EJ, Wilkinson JD, et al. Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet 2013; 382: 1889–97. Schwartz ML, Cox GF, Lin AE, et al. Clinical approach to genetic cardiomyopathy in children. Circulation 1996; 94: 2021–38. Cox GF, Sleeper LA, Lowe AM, et al. Factors associated with establishing a causal diagnosis in children with cardiomyopathy. Pediatrics 2006; 118: 1519–31. Colan SD, Lipshultz SE, Lowe AM, et al. Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation 2007; 115: 773–81. Foerster SR, Canter CE, Cinar A, et al. Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: an outcomes study from the Pediatric Cardiomyopathy Registry. Circ Heart Fail 2010; 3: 689–97. Wilkinson JD, Lowe AM, Salbert BA, et al. Outcomes in children with Noonan syndrome and hypertrophic cardiomyopathy: a study from the Pediatric Cardiomyopathy Registry. Am Heart J 2012; 164: 442–48.
Whether this intense legislative activity during the past 10 years has modified medical education and clinical practices is not known. I evaluated the knowledge in laïcité of 50 medical students from Paris Descartes University, Paris, and of 50 hospital clinicians from 13 different university medical centres in Paris using the questionnaire available in the appendix. Response rate was 99% for both medical students and clinicians. Strikingly, only 2% of medical students and 4% of clinicians stated that they received some teaching on laïcité in public hospitals. Only 31% of clinicians were aware of the differences between public and private hospitals, where rules vary slightly. The percentages of correct answers (about 60%) were quite similar between medical students and clinicians, suggesting that practice in public hospitals does not help to reinforce the academic education. Should we be satisfied with this paradoxal situation—a very detailed legislation but a lack of education in laïcité in the medical field? 70% of medical students are in favour of specific teachings on laïcité in hospitals. I declare that I have no competing interests.
Georgia Malamut [email protected] Université Paris Descartes-Sorbonne and Hôpital Européen Georges Pompidou, Paris 75015, France
Laïcité in medical schools: a French paradox? 10 years ago, in the context of the 2004 law to ban religious symbols in public schools, Sebastien Tassy and colleagues’ Correspondence 1 in The Lancet recalled the French attachment to the principles of laïcité (French secularism) in public hospitals. Since then, several reforms successively contributed to reshape laïcité in France (specifically in health-care facilities in 2005,2 for people admitted to hospitals in 2006,3 in public services in 2007,4 and the prohibition on full-faced covering up in public spaces in 20105). www.thelancet.com Vol 383 March 1, 2014
1 2
3
4
5
Tassy S, Polski L, Banet J, Gorincour G. Laïcité in hospitals. Lancet 2004; 363: 1401–02. Circulaire DHOS/G/2005/57 du 2 Février 2005 relative à la laïcité dans les établissements de santé. http://www.sante.gouv.fr/fichiers/ bo/2005/05-02/a0020035.htm (last accessed Feb 10, 2014). Circulaire DHOS/E1/DGS/SD1B/SD1C/ SD4A/2006/90 du 2 Mars 2006 relative aux droits des personnes hospitalisées et comportant une charte de la personne hospitalisée. http://circulaire.legifrance.gouv. fr/pdf/2009/04/cir_10571.pdf (last accessed Feb 10, 2014). Circulaire 5209/SG du 13 Avril 2007 relative à la charte de laïcité dans les services publics. http:// www.dgdr.cnrs.fr/bo/2007/07-07/521-bo0707cir5209.htm (last accessed Feb 10, 2014). LOI n° 2010-1192 du 11 Octobre 2010 interdisant la dissimulation du visage dans l’espace public. http://www.legifrance.gouv.fr/ affichTexte.do?cidTexte=JORFTEXT000022911 670&categorieLien=id (last accessed Feb 10, 2014).
Criminalising homosexuality threatens the fight against HIV/AIDS Homosexuality is criminalised in more than 70 countries,1 with severe implications for the health and wellbeing of the lesbian, gay, bisexual, and transgender (LGBT) community. Recent developments in several countries such as India, Uganda, and Nigeria—where HIV/AIDS remains a pressing public health issue—to reintroduce or strengthen criminalisation of homosexuality holds deep ramifications for patients and health-care workers tackling HIV/AIDS. Although temporarily thwarted in Uganda, the alleged proposal to criminalise a failure to “report” gay persons adds to the alarm.2 The success of the global campaign to reduce HIV transmission, radically improve antiretroviral therapy access, and maintain patient adherence has been rooted in the basis that tackling the heterosexual and homosexual epidemics collectively, through a respect for the rights of all people, including those most vulnerable to HIV, is the optimum approach.3 Criminalising homosexuality might do untold damage to HIV treatment and prevention efforts that have succeeded in engaging with the homosexual community, a feeling recently expressed by HIV/AIDS physicians in Europe.4 LGBT persons might already encounter stigma and discrimination at home and the workplace, making engagement with health-care programmes challenging; the prospect of criminal prosecution could dissuade them from seeking medical help altogether. Will health-care workers be afforded protection to treat homosexual patients in confidence? Or will they be under actual or perceived duress to report members of the homosexual community to authorities? These questions hold implications not only for the HIV/AIDS community, but
See Online for appendix
783
Ria Novosti/Science Photo Library
rather than a specific phenotype. This discrepancy is highlighted by the fact that patients with mixed phenotypes seem to have a worse prognosis than do those with idiopathic hypertrophic cardiomyopathy or malformation syndromes, yet these phenotypes can occur in patients with various underlying disorders, including inherited errors of metabolism and neuromuscular disease, but also malformation syndromes and idiopathic disease. Furthermore, the new category in this study, mixed hypertrophic cardiomyopathy, includes some patients identified as having idiopathic hypertrophic cardiomyopathy or hypertrophic cardiomyopathy associated with inherited errors of metabolism, malformation syndromes, or neuromuscular disorders in the authors’ previous reports,4 in which attempts at risk stratification on the basis of phenotype were also made. Finally, to develop a specific riskstratification algorithm applicable to each phenotypic category is problematic, because of the small numbers in each group and, in particular, the heterogeneous and nonuniform nature of the hypertrophic cardiomyopathy groups compared.5 Ultimately, heart failure at presentation seems to be the main determinant of the outcome, particularly in infancy, irrespective of the phenotype. Paediatric cardiomyopathies are a very heterogeneous group of disorders, and early diagnosis and aggressive clinical management are needed in children with specific aetiologies and heart failure at presentation. Although this study is an important attempt to describe real clinical scenarios, we believe that the emphasis on the phenotype alone could be misleading and future studies should concentrate on determining the specific aetiology, which is likely to be a stronger determinant of the natural history and outcome of hypertrophic cardiomyopathy in the paediatric population. 782
We declare that we have no competing interests.
*Juan Pablo Kaski, Giuseppe Limongelli [email protected] Inherited Cardiovascular Diseases Unit, Department of Cardiology, Great Ormond Street Hospital, London WC1N 3JH, UK (JPK); Institute of Cardiovascular Science, University College London, London, UK (JPK); and Monaldi Hospital, Second University of Naples, Naples, Italy (GL) 1
2
3
4
5
Lipshultz SE, Orav EJ, Wilkinson JD, et al. Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet 2013; 382: 1889–97. Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med 2003; 348: 1647–55. Nugent AW, Daubeney PE, Chondros P, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 2003; 348: 1639–46. Colan SD, Lipshultz SE, Lowe AM, et al. Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation 2007; 115: 773–81. Weintraub RG, Semsarian C. Paediatric cardiomyopathy: getting to the heart of the matter. Lancet 2013; 382: 1866–67.
Authors’ reply We thank Juan Pablo Kaski and Giuseppe Limongelli for their thoughtful comments about our Article. 1 We appreciate their recognition of the important findings from the Pediatric Cardiomyopathy Registry. Kaski and Limongelli agree that cardiomyopathy in childhood is phenotypically heterogeneous, but they state that their major issue is that the outcome of patients is mainly determined by the underlying aetiology rather than a specific phenotype. We agree that the cause of cardiomyopathy might be an important prognostic factor, but the cause must be identified quickly and accurately for it to affect management and subsequent prognosis. Unfortunately, this is not always possible. Despite the availability of a detailed comprehensive algorithm to determine the cause of pediatric cardiomyopathies,2 a 2006 Pediatric Cardiomyopathy Registry study showed that children with hypertrophic cardiomyopathy underwent causal testing only about half as often as children with other cardiomyopathies.3
Also, genetic testing for paediatric hypertrophic cardiomyopathy, which might greatly reduce the prevalence of idiopathic hypertrophic cardiomyopathy as a diagnosis, is still limited. In a rigorous analysis, we noted that causation was established in some of these children only years after presentation, and even then, nearly 75% of cases remained classified as idiopathic.4 In another disparity between physician beliefs about the importance of causal testing, we showed no differences in outcomes between children with myocarditis diagnosed by cardiac biopsy (using the Dallas criteria as the reference standard) and those who were diagnosed clinically by an experienced paediatric cardiologist.5 In another Pediatric Cardiomyopathy Registry report,6 we reference nine gene mutations in children with both Noonan syndrome and hypertrophic cardiomyopathy. These advances in identification of specific genetic causes are tempered by the fact that their associations with clinical outcomes have yet to be determined. Our data from the Pediatric Cardiomyopathy Registry show the importance of non-causal characteristics, such as presentation in infancy, heart failure at diagnosis, and specific echocardiographic abnormalities. 1 Indeed, in our Article, 1 patients were stratified both by functional phenotype (pure hypertrophic cardiomyopathy vs mixed hypertrophic cardiomyopathy with either dilated or restrictive features) and known aetiology, whereas additional risk stratification was on the basis of family history and clinical and echocardiographic characteristics. While we await results from new causal studies, including ongoing Pediatric Cardiomyopathy Registry studies of hypertrophic cardiomyopathy, particularly the discovery of genetic causes and their associations with outcomes, we believe that our report1 provides the best information available to help paediatric cardiologists to www.thelancet.com Vol 383 March 1, 2014
Correspondence
evaluate children newly diagnosed with hypertrophic cardiomyopathy. We declare that we have no competing interests.
*Steven E Lipshultz, E John Orav, James D Wilkinson, on behalf of the Pediatric Cardiomyopathy Registry Study Group [email protected] Department of Pediatrics, Wayne State University School of Medicine, Detroit, MI, USA (SEL); Department of Pediatrics, Leonard M Miller School of Medicine, University of Miami, Miami, FL 33101, USA (SEL, JDW); and Harvard Medical School, Boston, MA, USA (EJO) 1
2
3
4
5
6
Lipshultz SE, Orav EJ, Wilkinson JD, et al. Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet 2013; 382: 1889–97. Schwartz ML, Cox GF, Lin AE, et al. Clinical approach to genetic cardiomyopathy in children. Circulation 1996; 94: 2021–38. Cox GF, Sleeper LA, Lowe AM, et al. Factors associated with establishing a causal diagnosis in children with cardiomyopathy. Pediatrics 2006; 118: 1519–31. Colan SD, Lipshultz SE, Lowe AM, et al. Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation 2007; 115: 773–81. Foerster SR, Canter CE, Cinar A, et al. Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: an outcomes study from the Pediatric Cardiomyopathy Registry. Circ Heart Fail 2010; 3: 689–97. Wilkinson JD, Lowe AM, Salbert BA, et al. Outcomes in children with Noonan syndrome and hypertrophic cardiomyopathy: a study from the Pediatric Cardiomyopathy Registry. Am Heart J 2012; 164: 442–48.
Whether this intense legislative activity during the past 10 years has modified medical education and clinical practices is not known. I evaluated the knowledge in laïcité of 50 medical students from Paris Descartes University, Paris, and of 50 hospital clinicians from 13 different university medical centres in Paris using the questionnaire available in the appendix. Response rate was 99% for both medical students and clinicians. Strikingly, only 2% of medical students and 4% of clinicians stated that they received some teaching on laïcité in public hospitals. Only 31% of clinicians were aware of the differences between public and private hospitals, where rules vary slightly. The percentages of correct answers (about 60%) were quite similar between medical students and clinicians, suggesting that practice in public hospitals does not help to reinforce the academic education. Should we be satisfied with this paradoxal situation—a very detailed legislation but a lack of education in laïcité in the medical field? 70% of medical students are in favour of specific teachings on laïcité in hospitals. I declare that I have no competing interests.
Georgia Malamut [email protected] Université Paris Descartes-Sorbonne and Hôpital Européen Georges Pompidou, Paris 75015, France
Laïcité in medical schools: a French paradox? 10 years ago, in the context of the 2004 law to ban religious symbols in public schools, Sebastien Tassy and colleagues’ Correspondence 1 in The Lancet recalled the French attachment to the principles of laïcité (French secularism) in public hospitals. Since then, several reforms successively contributed to reshape laïcité in France (specifically in health-care facilities in 2005,2 for people admitted to hospitals in 2006,3 in public services in 2007,4 and the prohibition on full-faced covering up in public spaces in 20105). www.thelancet.com Vol 383 March 1, 2014
1 2
3
4
5
Tassy S, Polski L, Banet J, Gorincour G. Laïcité in hospitals. Lancet 2004; 363: 1401–02. Circulaire DHOS/G/2005/57 du 2 Février 2005 relative à la laïcité dans les établissements de santé. http://www.sante.gouv.fr/fichiers/ bo/2005/05-02/a0020035.htm (last accessed Feb 10, 2014). Circulaire DHOS/E1/DGS/SD1B/SD1C/ SD4A/2006/90 du 2 Mars 2006 relative aux droits des personnes hospitalisées et comportant une charte de la personne hospitalisée. http://circulaire.legifrance.gouv. fr/pdf/2009/04/cir_10571.pdf (last accessed Feb 10, 2014). Circulaire 5209/SG du 13 Avril 2007 relative à la charte de laïcité dans les services publics. http:// www.dgdr.cnrs.fr/bo/2007/07-07/521-bo0707cir5209.htm (last accessed Feb 10, 2014). LOI n° 2010-1192 du 11 Octobre 2010 interdisant la dissimulation du visage dans l’espace public. http://www.legifrance.gouv.fr/ affichTexte.do?cidTexte=JORFTEXT000022911 670&categorieLien=id (last accessed Feb 10, 2014).
Criminalising homosexuality threatens the fight against HIV/AIDS Homosexuality is criminalised in more than 70 countries,1 with severe implications for the health and wellbeing of the lesbian, gay, bisexual, and transgender (LGBT) community. Recent developments in several countries such as India, Uganda, and Nigeria—where HIV/AIDS remains a pressing public health issue—to reintroduce or strengthen criminalisation of homosexuality holds deep ramifications for patients and health-care workers tackling HIV/AIDS. Although temporarily thwarted in Uganda, the alleged proposal to criminalise a failure to “report” gay persons adds to the alarm.2 The success of the global campaign to reduce HIV transmission, radically improve antiretroviral therapy access, and maintain patient adherence has been rooted in the basis that tackling the heterosexual and homosexual epidemics collectively, through a respect for the rights of all people, including those most vulnerable to HIV, is the optimum approach.3 Criminalising homosexuality might do untold damage to HIV treatment and prevention efforts that have succeeded in engaging with the homosexual community, a feeling recently expressed by HIV/AIDS physicians in Europe.4 LGBT persons might already encounter stigma and discrimination at home and the workplace, making engagement with health-care programmes challenging; the prospect of criminal prosecution could dissuade them from seeking medical help altogether. Will health-care workers be afforded protection to treat homosexual patients in confidence? Or will they be under actual or perceived duress to report members of the homosexual community to authorities? These questions hold implications not only for the HIV/AIDS community, but
See Online for appendix
783
