DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20210115

Childhood cardiomyopathies: a study in tertiary care hospital of Kashmir

Abid Ahmad Shairgojri, Khalid Mohiuddin, Amber Bashir, Muzafar Jan

Abstract


Background: Cardiomyopathy constitutes a group of diseases that directly affect the structural or functional ability of myocardium. They are the most common form of heart diseases that are inherited in children and responsible for sudden deaths in healthy young adults. The Aim of our study was to provide a detailed description of clinical profile, epidemiology and etiology of cardiomyopathies in children.

Methods: This was a hospital based prospective observational study, conducted over a period of two and half years from September 2017 to March 2020 in the Post Graduate Department of Pediatrics Government Medical College Srinagar. Study group included all patients aged between 1 month and 18 years diagnosed with cardiomyopathy. They were subjected to a detailed clinical history and physical examination. All the patients underwent echocardiography.

Results: During the study period 37 patients were diagnosed with different types of cardiomyopathies. Dilated cardiomyopathy was commonest seen in 19 (51.35%), followed by hypertrophic cardiomyopathy 13 (35%) cases. Fast breathing was most common presenting complaint in dilated cardiomyopathy while most of cases with hypertrophic cardiomyopathy were asymptomatic. There was one case of distinct form of cardiomyopathy isolated left ventricular hypoplasia. Males were more involved 21 (56.76%) cases. Majority of patients 18 (48.64%) were less than one year at time of diagnosis. Parental consanguity was seen in 6 (16.22%) cases. Underlying cause was identified in 10 (27.02%) cases with myocarditis being the commonest cause.

Conclusions: Pediatric cardiomyopathy represents a considerable percentage of children with cardiac disorders. Dilated cardiomyopathy is the most common type usually presented with congestive heart failure, majority of cases of hypertrophic CMP were asymptomatic. Male preponderance was seen in dilated and hypertrophic cardiomyopathy. Parental consanguinity was seen in significant number of patients. Myocarditis and inborn error of metabolism was seen in significant number of patients.


Keywords


Cardiomyopathies, Children, Heart, Myocarditis

Full Text:

PDF

References


Richardson P. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies. Circulation. 1996;93:841-2.

Wilkinson JD, Landy DC, Colan SD, Towbin JA, Sleeper LA, Orav EJ, et al. The pediatric cardiomyopathy registry and heart failure: key results from the first 15 years. Heart Fail Clin. 2010;6(4):401-13.

Lipshultz SE, Sleeper LA, Towbin JA, Lowe AM, Orav EJ, Cox GF, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med. 2003;348(17):1647-55.

Nugent AW, Daubeney PE, Chondros P, Carlin JB, Cheung M, Wilkinson LC, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med. 2003;348(17):1639-46.

Arola A, Jokinen E, Ruuskanen O, Saraste M, Pesonen E, Kuusela AL, et al. Epidemiology of idiopathic cardiomyopathies in children and adolescents: a nationwide study in Finland. Ame J Epidemiol. 1997;146(5):385-93.

Alvarez JA, Orav EJ, Wilkinson JD, Fleming LE, Lee DJ, Sleeper LA, et al. Competing risks for death and cardiac transplantation in children with dilated cardiomyopathy: results from the pediatric cardiomyopathy registry. Circulation. 2011;124(7):814-23.

Towbin JA, Lowe AM, Colan SD, Sleeper LA, Orav EJ, Clunie S, et al. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA. 2006;296(15):1867-76.

Jefferies JL, Wilkinson JD, Sleeper LA, Colan SD, Lu M, Pahl E, et al. Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: results from the pediatric cardiomyopathy registry. J Cardiac Fail. 2015;21(11):877-84.

Kasper EK, Agema WR, Hutchins GM, Deckers JW, Hare JM, Baughman KL. The causes of dilated cardiomyopathy: a clinicopathologic review of 673 consecutive patients. J Am Coll Cardiol. 1994;23(3):586-90.

Lipshultz SE, Law YM, Asante-Korang A, Austin ED, Dipchand AI, Everitt MD, et al. Cardiomyopathy in children: classification and diagnosis: a scientific statement from the American Heart Association. Circulation. 2019;140(1):e9-68.

Hong YM. Cardiomyopathies in children. Korean J Pediatr. 2013;56(2):52.

Bakeet MA, Mohamed MM, Allam AA, Gamal R. Childhood cardiomyopathies: a study in tertiary care hospital in Upper Egypt. Electron Phys. 2016;8(11):3164.

Miranda JO, Costa L, Rodrigues E, Teles EL, Baptista MJ, Areias JC. Paediatric dilated cardiomyopathy: clinical profile and outcome. The experience of a tertiary centre for paediatric cardiology. Cardiol Young. 2015;25(2):333.

Fernandez-Valls M, Srichai MB, Stillman AE, White RD. Isolated left ventricular apical hypoplasia: a new congenital anomaly described with cardiac tomography. Heart. 2004;90(5):552-5.

Choh NA, Amreen S, Mir AB, Malik AH, Hameed M, Shaheen F, et al. Isolated left ventricular hypoplasia-A singularity. Ann Pediatr Cardiol. 2020;13(4):337.