Research Article
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Dilate kardiyomyopatili çocuk hastalarda klinik ve prognostik faktörler

Year 2021, Volume: 14 Issue: 4, 828 - 834, 01.10.2021
https://doi.org/10.31362/patd.843176

Abstract

Amaç: Dilate kardiyomiyopati, sol ventrikül kontraktilitesinde bozulma ve kardiyak dilatasyon ile seyreden bir hastalıktır. Tanısal ilerlemelere rağmen çocuklardaki çoğu vaka idiyopatiktir. Çalışmamızda dilate kardiyomiyopatili çocuk hastalarda prognozu etkileyen faktörleri değerlendirmeyi amaçladık.
Yöntemler: Dilate kardiyomiyopati tanısı konulan 59 hasta, Ocak 2005-Ocak 2013 tarihleri arasında retrospektif olarak incelendi. Prognoz açısından; hastalarda semptomların başlama yaşı, hastaların cinsiyeti, anne-baba akrabalığı, elektrokardiyografik bulgu ve telekardiyogramda kardiyomegali varlığı, ekokardiyografide ejeksiyon fraksiyonu ve kısalma fraksiyonu değerlendirildi.
Bulgular: Hastalarımızın ortalama yaşları 49.2±59.2 ay olup, erkek/kız oranı 1.03/1 idi. Hastalar en sık kalp yetersizliği semptom ve klinik bulguları ile başvurmuştu. En sık elektrokardiyografi bulgusu sol ventrikül hipertrofisi (%32.2) idi ve telekardiyografide hastaların %77.8’inde kardiyomegali saptandı. Ekokardiyografik incelemede; hastaların sol ventrikül ejeksiyon ve kısalma fraksiyonları tanı anında %38.4±14.5 ve %18.8±8.4 olup, takip süresi sonunda ise %51±18.6% ve %26.8±11.6 idi. Ortalama takip süresi 27.5±27.7 ay ve mortalite oranı %42.4 olarak bulundu. Prognozu etkileyen ana faktörler incelendiğinde; tanıda hastalarda elektrokardiyografik değişiklik ve kardiyomegali olması ve ejeksiyon fraksiyonlarının düşük olmasının sağkalımı etkilediği görüldü.
Sonuç: Dilate kardiyomiyopati tedavisi zor bir hastalıkdır ve prognozu kötüdür. Tanı anında elektrokardiyografik bulgu ve telekardiyografide kardiyomegali olması ve EF değerlerinin düşük olması hastaların sağkalımını etkileyen en önemli belirteçlerdir.

References

  • 1. Nugent, A.W., P.E. Daubeney, P. Chondros, et al., The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med, 2003. 348(17): p. 1639-46. DOI: 10.1056/NEJMoa021737.
  • 2. Richardson, P., W. McKenna, M. Bristow, et al., Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation, 1996. 93(5): p. 841-2. DOI: 10.1161/01.cir.93.5.841.
  • 3. Lipshultz, S.E., L.A. Sleeper, J.A. Towbin, et al., The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med, 2003. 348(17): p. 1647-55. DOI: 10.1056/NEJMoa021715.
  • 4. Cox, G.F., Diagnostic Approaches to Pediatric Cardiomyopathy of Metabolic Genetic Etiologies and Their Relation to Therapy. Prog Pediatr Cardiol, 2007. 24(1): p. 15-25. DOI: 10.1016/j.ppedcard.2007.08.013.
  • 5. Towbin, J.A., A.M. Lowe, S.D. Colan, et al., Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA, 2006. 296(15): p. 1867-76. DOI: 10.1001/jama.296.15.1867.
  • 6. Daubeney, P.E., A.W. Nugent, P. Chondros, et al., Clinical features and outcomes of childhood dilated cardiomyopathy: results from a national population-based study. Circulation, 2006. 114(24): p. 2671-8. DOI: 10.1161/CIRCULATIONAHA.106.635128.
  • 7. Maron, B.J., J.A. Towbin, G. Thiene, et al., Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation, 2006. 113(14): p. 1807-16. DOI: 10.1161/CIRCULATIONAHA.106.174287.
  • 8. Nandi, D. and J.W. Rossano, Epidemiology and cost of heart failure in children. Cardiol Young, 2015. 25(8): p. 1460-8. DOI: 10.1017/S1047951115002280.
  • 9. Puggia, I., M. Merlo, G. Barbati, et al., Natural History of Dilated Cardiomyopathy in Children. J Am Heart Assoc, 2016. 5(7). DOI: 10.1161/JAHA.116.003450.
  • 10. Dec, G.W., Jr., I.F. Palacios, J.T. Fallon, et al., Active myocarditis in the spectrum of acute dilated cardiomyopathies. Clinical features, histologic correlates, and clinical outcome. N Engl J Med, 1985. 312(14): p. 885-90. DOI: 10.1056/NEJM198504043121404.
  • 11. Lipshultz, S.E., Y.M. Law, A. Asante-Korang, et al., Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association. Circulation, 2019. 140(1): p. e9-e68. DOI: 10.1161/CIR.0000000000000682.
  • 12. Alvarez, J.A., E.J. Orav, J.D. Wilkinson, et al., Competing risks for death and cardiac transplantation in children with dilated cardiomyopathy: results from the pediatric cardiomyopathy registry. Circulation, 2011. 124(7): p. 814-23. DOI: 10.1161/CIRCULATIONAHA.110.973826.
  • 13. Burch, M., S.A. Siddiqi, D.S. Celermajer, C. Scott, C. Bull, and J.E. Deanfield, Dilated cardiomyopathy in children: determinants of outcome. Br Heart J, 1994. 72(3): p. 246-50. DOI: 10.1136/hrt.72.3.246.
  • 14. Muller, G., H.E. Ulmer, K.J. Hagel, and D. Wolf, Cardiac dysrhythmias in children with idiopathic dilated or hypertrophic cardiomyopathy. Pediatr Cardiol, 1995. 16(2): p. 56-60. DOI: 10.1007/BF00796818.
  • 15. Ture, M., H. Balik, A. Akin, M. Bilici, and A. Nergiz, The relationship between electrocardiographic data and mortality in children diagnosed with dilated cardiomyopathy. Eur J Pediatr, 2020. 179(5): p. 813-819. DOI: 10.1007/s00431-020-03569-9.
  • 16. Manolio, T.A., K.L. Baughman, R. Rodeheffer, et al., Prevalence and etiology of idiopathic dilated cardiomyopathy (summary of a National Heart, Lung, and Blood Institute workshop. Am J Cardiol, 1992. 69(17): p. 1458-66. DOI: 10.1016/0002-9149(92)90901-a.
  • 17. Zecchin, M., A.D. Lenarda, M. Bonin, et al., Incidence and predictors of sudden cardiac death during long-term follow-up in patients with dilated cardiomyopathy on optimal medical therapy. Ital Heart J, 2001. 2(3): p. 213-21.
  • 18. Jefferies, J.L. and J.A. Towbin, Dilated cardiomyopathy. Lancet, 2010. 375(9716): p. 752-62. DOI: 10.1016/S0140-6736(09)62023-7.
  • 19. Adwani, S.S., B.F. Whitehead, P.G. Rees, et al., Heart transplantation for dilated cardiomyopathy. Arch Dis Child, 1995. 73(5): p. 447-52. DOI: 10.1136/adc.73.5.447.

Clinical and prognostic factors in children with dilated cardiomyopathy

Year 2021, Volume: 14 Issue: 4, 828 - 834, 01.10.2021
https://doi.org/10.31362/patd.843176

Abstract

Purpose: Dilated cardiomyopathy is characterized by impaired myocardial contractility and cardiac dilatation. Most cases in children are idiopathic despite diagnostic developments. In this study, we aimed to review children with dilated cardiomyopathy and attempt to determine the prognostic factors.
Materials and Methods: A total of 59 children with a diagnosis of dilated cardiomyopathy were evaluated retrospectively between January 2005 to January 2013. The age of onset of symptoms, gender, parental consanguinity, electrocardiography findings, presence of cardiomegaly in the telecardiograpy, left ventricular ejection fraction and shortening fraction on prognosis were evaluated.
Results: The mean age of patients was 49.2 ± 59.2 months and the male/female ratio was 1.03/1. Heart failure symptoms and signs were the most common cause of admission. The most common electrocardiographic finding was left ventricular hypertrophy in 32.2% and cardiomegaly was observed on telecardiography in 77.8%. The left ventricular ejection and shortening fraction of patients’ were 38.4± 14.5% and 18.8± 8.4% at the time of the diagnosis and 51± 18.6% and 26.8± 11.6% at the end of the follow-up, respectively. The mean follow-up period was 27.5± 27.7 months and the mortality rate was 42.4%. The main factors affecting prognosis have been found as detection of electrocardiographic changes, cardiomegaly, and lower left ventricular ejection fraction at the time of the diagnosis.
Conclusion: Dilated cardiomyopathy remains a challenging disease and the prognosis is poor. In our study, the most important prognostic markers affecting survival were determined by the presence of electrocardiographic changes, cardiomegaly, and a reduced ejection fraction.

References

  • 1. Nugent, A.W., P.E. Daubeney, P. Chondros, et al., The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med, 2003. 348(17): p. 1639-46. DOI: 10.1056/NEJMoa021737.
  • 2. Richardson, P., W. McKenna, M. Bristow, et al., Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation, 1996. 93(5): p. 841-2. DOI: 10.1161/01.cir.93.5.841.
  • 3. Lipshultz, S.E., L.A. Sleeper, J.A. Towbin, et al., The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med, 2003. 348(17): p. 1647-55. DOI: 10.1056/NEJMoa021715.
  • 4. Cox, G.F., Diagnostic Approaches to Pediatric Cardiomyopathy of Metabolic Genetic Etiologies and Their Relation to Therapy. Prog Pediatr Cardiol, 2007. 24(1): p. 15-25. DOI: 10.1016/j.ppedcard.2007.08.013.
  • 5. Towbin, J.A., A.M. Lowe, S.D. Colan, et al., Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA, 2006. 296(15): p. 1867-76. DOI: 10.1001/jama.296.15.1867.
  • 6. Daubeney, P.E., A.W. Nugent, P. Chondros, et al., Clinical features and outcomes of childhood dilated cardiomyopathy: results from a national population-based study. Circulation, 2006. 114(24): p. 2671-8. DOI: 10.1161/CIRCULATIONAHA.106.635128.
  • 7. Maron, B.J., J.A. Towbin, G. Thiene, et al., Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation, 2006. 113(14): p. 1807-16. DOI: 10.1161/CIRCULATIONAHA.106.174287.
  • 8. Nandi, D. and J.W. Rossano, Epidemiology and cost of heart failure in children. Cardiol Young, 2015. 25(8): p. 1460-8. DOI: 10.1017/S1047951115002280.
  • 9. Puggia, I., M. Merlo, G. Barbati, et al., Natural History of Dilated Cardiomyopathy in Children. J Am Heart Assoc, 2016. 5(7). DOI: 10.1161/JAHA.116.003450.
  • 10. Dec, G.W., Jr., I.F. Palacios, J.T. Fallon, et al., Active myocarditis in the spectrum of acute dilated cardiomyopathies. Clinical features, histologic correlates, and clinical outcome. N Engl J Med, 1985. 312(14): p. 885-90. DOI: 10.1056/NEJM198504043121404.
  • 11. Lipshultz, S.E., Y.M. Law, A. Asante-Korang, et al., Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association. Circulation, 2019. 140(1): p. e9-e68. DOI: 10.1161/CIR.0000000000000682.
  • 12. Alvarez, J.A., E.J. Orav, J.D. Wilkinson, et al., Competing risks for death and cardiac transplantation in children with dilated cardiomyopathy: results from the pediatric cardiomyopathy registry. Circulation, 2011. 124(7): p. 814-23. DOI: 10.1161/CIRCULATIONAHA.110.973826.
  • 13. Burch, M., S.A. Siddiqi, D.S. Celermajer, C. Scott, C. Bull, and J.E. Deanfield, Dilated cardiomyopathy in children: determinants of outcome. Br Heart J, 1994. 72(3): p. 246-50. DOI: 10.1136/hrt.72.3.246.
  • 14. Muller, G., H.E. Ulmer, K.J. Hagel, and D. Wolf, Cardiac dysrhythmias in children with idiopathic dilated or hypertrophic cardiomyopathy. Pediatr Cardiol, 1995. 16(2): p. 56-60. DOI: 10.1007/BF00796818.
  • 15. Ture, M., H. Balik, A. Akin, M. Bilici, and A. Nergiz, The relationship between electrocardiographic data and mortality in children diagnosed with dilated cardiomyopathy. Eur J Pediatr, 2020. 179(5): p. 813-819. DOI: 10.1007/s00431-020-03569-9.
  • 16. Manolio, T.A., K.L. Baughman, R. Rodeheffer, et al., Prevalence and etiology of idiopathic dilated cardiomyopathy (summary of a National Heart, Lung, and Blood Institute workshop. Am J Cardiol, 1992. 69(17): p. 1458-66. DOI: 10.1016/0002-9149(92)90901-a.
  • 17. Zecchin, M., A.D. Lenarda, M. Bonin, et al., Incidence and predictors of sudden cardiac death during long-term follow-up in patients with dilated cardiomyopathy on optimal medical therapy. Ital Heart J, 2001. 2(3): p. 213-21.
  • 18. Jefferies, J.L. and J.A. Towbin, Dilated cardiomyopathy. Lancet, 2010. 375(9716): p. 752-62. DOI: 10.1016/S0140-6736(09)62023-7.
  • 19. Adwani, S.S., B.F. Whitehead, P.G. Rees, et al., Heart transplantation for dilated cardiomyopathy. Arch Dis Child, 1995. 73(5): p. 447-52. DOI: 10.1136/adc.73.5.447.
There are 19 citations in total.

Details

Primary Language English
Subjects Paediatrics
Journal Section Research Article
Authors

Ayça Koca Yozgat 0000-0001-6690-721X

Selmin Karademir This is me 0000-0002-9084-0083

Publication Date October 1, 2021
Submission Date December 19, 2020
Acceptance Date April 30, 2021
Published in Issue Year 2021 Volume: 14 Issue: 4

Cite

AMA Koca Yozgat A, Karademir S. Clinical and prognostic factors in children with dilated cardiomyopathy. Pam Med J. October 2021;14(4):828-834. doi:10.31362/patd.843176

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