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Paediatric cardiomyopathies: echocardiographic diagnosis, clinical profile, and demographic characteristics: the experience of a tertiary referral centre for Latin American paediatric cardiology

Published online by Cambridge University Press:  17 February 2020

Víctor M. Huertas-Quiñones ,
Camilo F. Mestra ,
Valeria Peña-Trujillo ,
Sebastián Gallo-Bernal Open the ORCID record for Sebastián Gallo-Bernal [Opens in a new window] ,
Mariana Villaveces  and
Laura C. Alarcón-Forero
Víctor M. Huertas-Quiñones
Affiliation:
Division of Pediatric Cardiology, Fundación Cardioinfantil, Cardiology Institute, Bogotá, Colombia School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia
Camilo F. Mestra
Affiliation:
Division of Pediatric Cardiology, Fundación Cardioinfantil, Cardiology Institute, Bogotá, Colombia
Valeria Peña-Trujillo
Affiliation:
Division of Pediatric Cardiology, Fundación Cardioinfantil, Cardiology Institute, Bogotá, Colombia School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia
Sebastián Gallo-Bernal*
Affiliation:
Division of Pediatric Cardiology, Fundación Cardioinfantil, Cardiology Institute, Bogotá, Colombia School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia
Mariana Villaveces
Affiliation:
Division of Pediatric Cardiology, Fundación Cardioinfantil, Cardiology Institute, Bogotá, Colombia School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia
Laura C. Alarcón-Forero
Affiliation:
Division of Pediatric Cardiology, Fundación Cardioinfantil, Cardiology Institute, Bogotá, Colombia School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia
*
Author for correspondence: S. Gallo-Bernal, MD, Division of Pediatric Heart Failure, Cardiomyopathy and Cardiac Transplantation, Fundación Cardioinfantil, Calle 163 A No. 13B-60, Bogotá110131, Colombia. Tel: +57-3112625543; Fax: +(57) 1-6672708; E-mail: juans.bernal@urosario.edu.co
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Abstract

Background:

Although multiple studies have been conducted in the adult population, there is a vast knowledge gap regarding the epidemiologic characteristics of cardiomyopathies in the paediatric population. This issue is even more crucial when the precarious situation of medical research in Latin America is considered. Given the potential impact that these disorders could have on Latin American health systems, a comprehensive epidemiologic study regarding the clinical profile and sociodemographic characteristics of these patients will influence the way we approach paediatric cardiomyopathies.

Methods:

An observational retrospective study was conducted at a tertiary referral centre for Colombian and Latin American paediatric cardiology. We analysed all cases of primary cardiomyopathies in children younger than 18 years of age who presented at our institution between 2010 and 2016. Cases of cardiomyopathies were classified according to World Health Organization guidelines.

Results:

From a total of 29,533 children who attended our institution during the study period, 89 new cases of primary cardiomyopathies were identified. The median age at diagnosis was 11 years (interquartile range 4–9). Dilated cardiomyopathy accounted for 57.3% (n = 51) of cases; hypertrophic cardiomyopathy, 12.3% (n = 11); restrictive cardiomyopathy, 8.9% (n = 8); non-compacted cardiomyopathy, 7.8% (n = 7); arrhythmogenic ventricular cardiomyopathy, 6.7% (n = 6); and unspecified cardiomyopathy, 6.7% (n = 6). Heart failure was observed in 53.93% of the patients. The overall mortality was 12.36% (n = 11), which included two of eight patients who underwent cardiac transplantation.

Keywords

Cardiomyopathypaediatric cardiologyepidemiologyheart failure
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 4 , April 2020 , pp. 462 - 467
Copyright
© The Author(s), 2020. Published by Cambridge University Press

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References

Maisch, B, Noutsias, M, Ruppert, V, et al.Cardiomyopathies: classification, diagnosis, and treatment. Heart Fail Clin. 2012; 8: 53–78.CrossRefGoogle Scholar
Lipshultz, SE, Law, YM, Asante-Korang, A, et al.Cardiomyopathy in children: classification and diagnosis: a scientific statement from the American Heart Association. Circulation [Internet]. 2019; 140: e9–68. Available from: http://www.ncbi.nlm.nih.gov/pubmed/31132865Google ScholarPubMed
Lee, TM, Hsu, DT, Kantor, P, et al.Pediatric cardiomyopathies. Circ Res. 2017; 121: 855–873.CrossRefGoogle ScholarPubMed
Lipshultz, SE, Cochran, TR, Briston, DA, et al.Pediatric cardiomyopathies: causes, epidemiology, clinical course, preventive strategies and therapies. Future Cardiol. 2013; 9: 817–848.CrossRefGoogle ScholarPubMed
Lipshultz, SE.Ventricular dysfunction clinical research in infants, children and adolescents. Prog Pediatr Cardiol. 2000; 12: 1–28.CrossRefGoogle ScholarPubMed
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: 1647–1655.CrossRefGoogle ScholarPubMed
Nugent, AW, Daubeney, PEF, Chondros, P, et al.The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med. 2003; 348: 1639–1646.CrossRefGoogle ScholarPubMed
Arola, A, Jokinen, E, Ruuskanen, O, et al.Epidemiology of idiopathic cardiomyopathies in children and adolescents: a nationwide study in Finland. Am J Epidemiol. 1997; 146: 385–393.CrossRefGoogle ScholarPubMed
Alvarez, JA, Orav, EJ, Wilkinson, JD, et al.Competing risks for death and cardiac transplantation in children with dilated cardiomyopathy. Circulation. 2011; 124: 814–823.CrossRefGoogle ScholarPubMed
Towbin, JA, Lowe, AM, Colan, SD, et al.Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA. 2006; 296: 1867–1876.CrossRefGoogle ScholarPubMed
Miranda, JO, Costa, L, Rodrigues, E, et al.Paediatric dilated cardiomyopathy: clinical profile and outcome. The experience of a tertiary centre for paediatric cardiology. Cardiol Young. 2015; 25: 333–337.CrossRefGoogle ScholarPubMed
Oechslin, E, Jenni, R.Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J. 2011; 32: 1446–1456.CrossRefGoogle ScholarPubMed
Floria, M, Tinica, G, Grecu, M.Left ventricular non-compaction -challenges and controversies. Mædica. 2014; 9: 282–288.Google ScholarPubMed
Jefferies, JL, Wilkinson, JD, Sleeper, LA, et al.Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: results from the pediatric cardiomyopathy registry. J Card Fail. 2015; 21: 877–884.CrossRefGoogle ScholarPubMed
Evans, RW.The economics of heart transplantation. Circulation. 1987; 75: 63–76.CrossRefGoogle ScholarPubMed
Gillum, RF.Idiopathic cardiomyopathy in the United States, 1970–1982. Am Heart J. 1986; 111: 752–755.CrossRefGoogle ScholarPubMed
Capelli, H, Kreutzer, C, Kreutzer, G.Development of pediatric cardiology in Latin America: accomplishments and remaining challenges. World J Pediatr Congenit Hear Surg. 2010; 2: 104–110.CrossRefGoogle Scholar
Araujo, JJ.Adults with congenital heart disease: a growing public health problem? Arch Cardiol México. 2018; 88: 251–252.CrossRefGoogle ScholarPubMed
Caneo, LF, Jatene, MB.Pediatric mechanical circulatory support systems in Latin America. Artif Organs. 2016; 40: 925–928.CrossRefGoogle ScholarPubMed
Patel, MD, Mohan, J, Schneider, C, et al.Pediatric and adult dilated cardiomyopathy represent distinct pathological entities. JCI Insight. 2017; 2: e94382.CrossRefGoogle ScholarPubMed
Chun, EJ, Choi, S Il, Jin, KN, et al.Hypertrophic cardiomyopathy: assessment with MR imaging and multidetector CT. RadioGraphics. 2010; 30: 1309–1328.CrossRefGoogle ScholarPubMed
Maron, BJ, Maron, MS, Semsarian, C.Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives. J Am Coll Cardiol. 2012; 60: 705–715.CrossRefGoogle ScholarPubMed
Houston, BA, Stevens, GR.Hypertrophic cardiomyopathy: a review. Clin Med Insights Cardiol. 2015; 8: 53–65.Google ScholarPubMed
Finocchiaro, G, Papadakis, M, Tanzarella, G, et al.Sudden death can be the first manifestation of hypertrophic cardiomyopathy. JACC Clin Electrophysiol. 2019; 5: 252–254.CrossRefGoogle ScholarPubMed
Maron, BJ, Shirani, J, Poliac, LC, et al.Sudden death in young competitive athletes: clinical, demographic, and pathological profiles. JAMA. 1996; 276: 199–204.CrossRefGoogle ScholarPubMed
Priori, SG, Blomström-Lundqvist, C, Mazzanti, A, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2015; 36: 2793–2867.CrossRefGoogle ScholarPubMed
Udeoji, DU, Philip, KJ, Morrissey, RP, et al.Left ventricular noncompaction cardiomyopathy: updated review. Ther Adv Cardiovasc Dis. 2013; 7: 260–273.CrossRefGoogle ScholarPubMed
Arbustini, E, Favalli, V, Narula, N, et al.Left ventricular noncompaction: a distinct genetic cardiomyopathy? J Am Coll Cardiol. 2016; 68: 949–966.CrossRefGoogle ScholarPubMed
Arbustini, E, Weidemann, F, Hall, JL.Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases? J Am Coll Cardiol. 2014; 64: 1840–1850.CrossRefGoogle ScholarPubMed
Ichida, F, Tsubata, S, Bowles, KR, et al.Novel gene mutations in patients with left ventricular noncompaction or barth syndrome. Circulation. 2001; 103: 1256–1263.CrossRefGoogle ScholarPubMed
Luxán, G, Casanova, JC, Martínez-Poveda, B, et al.Mutations in the NOTCH pathway regulator MIB1 cause left ventricular noncompaction cardiomyopathy. Nat Med. 2013; 19: 193–201.CrossRefGoogle ScholarPubMed
Chin, TK, Perloff, JK, Williams, RG, et al.Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990; 82: 507–513.CrossRefGoogle ScholarPubMed
Pignatelli, RH, McMahon, CJ, Dreyer, WJ, et al.Clinical characterization of left ventricular noncompaction in children. Circulation. 2003; 108: 2672–2678.CrossRefGoogle ScholarPubMed
Dawson, DK, McLernon, DJ, Raj, VJ, et al.Cardiovascular magnetic resonance determinants of left ventricular noncompaction. Am J Cardiol. 2014; 114: 456–462.CrossRefGoogle ScholarPubMed
Fazio, G, Novo, G, D’Angelo, L, et al.Magnetic resonance in isolated noncompaction of the ventricular myocardium. Int J Cardiol. 2010; 140: 367–369.CrossRefGoogle ScholarPubMed
Stöllberger, C, Wegner, C, Finsterer, J.Fetal ventricular hypertrabeculation/noncompaction: clinical presentation, genetics, associated cardiac and extracardiac abnormalities and outcome. Pediatr Cardiol. 2015; 36: 1319–1326.CrossRefGoogle ScholarPubMed
Corrado, D, Link, MS, Calkins, H.Arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 2017; 376: 61–72.CrossRefGoogle ScholarPubMed
Thiene, G, Nava, A, Corrado, D, et al.Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med. 1988; 318: 129–133.CrossRefGoogle ScholarPubMed
Hugh, C, Domenico, C, Frank, M.Risk stratification in arrhythmogenic right ventricular cardiomyopathy. Circulation. 2017; 136: 2068–2082.Google Scholar
McKenna, WJ, Maron, BJ, and Thiene, G.Classification, epidemiology, and global burden of cardiomyopathies. Circ Res. 2017; 121: 722–730.CrossRefGoogle ScholarPubMed
Ross, RD.The ross classification for heart failure in children after 25 years: a review and an age-stratified revision. Pediatr Cardiol. 2012; 33: 1295–1300.CrossRefGoogle Scholar
Murni, IK, Musa, NL.The need for specialized pediatric cardiac critical care training program in limited resource settings. Front Pediatr. 2018; 6: 59.CrossRefGoogle ScholarPubMed
Ashkanase, J, Jeewa, A, Arathoon, K, et al.Pediatric hypertrophic cardiomyopathy: survival and prognostic indicators in the current era. J Hear Lung Transplant. 2019; 38: S465.CrossRefGoogle Scholar
Miller, TL, Neri, D, Extein, J, et al.Nutrition in pediatric cardiomyopathy. Prog Pediatr Cardiol. 2007; 24: 59–71.CrossRefGoogle ScholarPubMed
Bailer, J, Kaufman, BD.Nutrition implications of heart failure and heart transplantation in children with dilated cardiomyopathy: a case series. ICAN Infant, Child, Adolesc Nutr. 2010; 2: 377–384.CrossRefGoogle Scholar
Medoff-Cooper, B, Ravishankar, C.Nutrition and growth in congenital heart disease: a challenge in children. Curr Opin Cardiol. 2013; 28.CrossRefGoogle ScholarPubMed
Benzecry, SG, Leite, HP, Oliveira, FC, et al.Interdisciplinary approach improves nutritional status of children with heart diseases. Nutrition. 2008; 24: 669–674.CrossRefGoogle ScholarPubMed
Cameron, JW, Rosenthal, A, Olson, AD.Malnutrition in hospitalized children with congenital heart disease. JAMA Pediatr. 1995; 149: 1098–1102.Google ScholarPubMed
Lewis, KD, Conway, J, Cunningham, C, et al.Optimizing nutrition in pediatric heart failure: the crisis is over and now it’s time to feed. Nutr Clin Pract. 2017; 088453361771250.CrossRefGoogle ScholarPubMed
Rajagopal, SK, Yarlagadda, VV, Thiagarajan, RR, et al.Pediatric heart failure and worsening renal function: association with outcomes after heart transplantation. J Hear Lung Transplant. 2012; 31: 252–258.CrossRefGoogle ScholarPubMed