PROPOSAL ON NATIONAL REGISTRY ON CARDIOMYOPATHIES IN CHILDREN. Dr. BA Animasahun

Transcription

1 PROPOSAL ON NATIONAL REGISTRY ON CARDIOMYOPATHIES IN CHILDREN Dr. BA Animasahun Associate Professor/ Consultant Paediatric Interventional Cardiologist Lagos State University College of Medicine/ Lagos State University Teaching Hospital, Ikeja, Lagos

2 Department of Paediatrics, LASUTH

3 FCS, LASUCOM, Ikeja, Lagos

4 INTRODUCTION Cardiomyopathies are a heterogeneous group of disorders associated with structural remodeling and or functional abnormality of the cardiac muscle They are an important cause of morbidity and mortality in children

5 CLASSIFICATION Several classification schemes have evolved, -World Health Organization classification(1995) -American Heart Association classification(2006) -European Society of Cardiology classification (2007)

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7 Types of Cardiomyopathies Dilated cardiomyopathy (DCM) Hypertrophic cardiomyopathy (HCM) Restrictive cardiomyopathy (RCM) Unclassified (arrhythmogenic right ventricular cardiomyopathy, Left ventricular noncompaction)

8 EPIDEMIOLOGY Overall incidence of cardiomyopathy in children is unknown as it varies from country to country

9 United States cases per 100,000 in children less than or equal to 18 years Australia per 100,000 children younger than 10 years Finland per 100,000 children aged 20 years or younger Nigeria- 9 cases per 100,000 children in a hospital based study

10 Dilated cardiomyopathy is the most common worldwide Multicentre study in North America reported 0.57 cases per 100,000 per year. 6.9% per thousand in children less than 13 years

11 In Nigeria Most common acquired heart disease in childrenmuticentre study by Sadoh et al 9 in the north and south Second most common acquired heart disease in children- Bode-Thomas et al and Sani et al in the North -Animasahun et al in south-west

12 Hypertrophic cardiomyopathy is the second most common type of cardiomyopathy -Lower incidence for expression of the disease in childhood with a rate of 3 to 5 cases per 1 million children

13 Sadoh et al reported 18 cases Animasahun et al reported 5 cases

14 Restrictive cardiomyopathy is the least common form of cardiomyopathy

15 Sani et al in the Northern part of Nigeria reported 7 cases of endomyocardial fibrosis Sadoh et al reported 3 cases of endomyocardial fibrosis in the northern and southern part of Nigeria Animasahun et al reported 4 cases of endomyocardial fibrosis in the South-west

16 RISK FACTORS Infections Drugs, Metabolic disorders Genetic/familial Neuromuscular disorders Infiltrative myocardial diseases Coronary artery diseases Collagen vascular diseases

17 Cardiomyopat hy type Dilated cardiomyopat hy Hypertrophic cardiomyopat hy Restrictive cardiomyopat hy Symptoms Cough, poor feeding, irritability, shortness of breath, failure to gain weight Asymptomatic, syncope, presyncope, palpitations, angina Weight loss, abdominal discomfort Signs Tachycardia with narrow pulse pressure, quiet precordium, mitral and tricuspid regurgitation murmur Overactive precordial impulse with a heave, systolic ejection murmur in aortic area, fourth heart sound, apical Pitting edema of the lower extremities, hepatomegaly, ascites

18 Cardiomyopat hy type Dilated cardiomyopat hy Hypertrophic cardiomyopat hy Restrictive cardiomyopat hy ECG findings Left atrial and ventricular hypertrophy, nonspecific T wave abnormalities, deep Q-waves with ST segment depression Left ventricular hypertrophy, ST segment and T wave abnormalities,w olff-parkinson White syndrome and conduction defects Prominent P wave, nonspecific ST and T wave changes ECHO findings Marked dilatation of the left ventricle with global hypokinesia, ejection fraction less than 50%, Decreased left ventricular diameter, interventricular septum thicker than posterior wall, systolic Typically, preserved ejection fraction until late stages, marked dilatation of both atria,

19 AIM To determine the incidence, risk factors and outcome of cardiomyopathies in Nigerian children

20 OBJECTIVES To determine the incidence of cardiomyopathies among Nigerian children To determine the risk factors for cardiomyopathies among Nigerian children To detect the common clinical manifestations of cardiomyopathies among Nigerian children To determine the outcome of Nigerian children with cardiomyopathies

21 SUBJECTS AND METHODS Study population All patients presenting to the paediatric cardiologist in paediatric cardiology centres in Nigeria are prospectively recruited for the study Inclusion criteria Age less than 18 years Echocardiography evidence of cardiomyopathy Exclusion criteria Children with diagnosis of congenital heart disease Children with diagnosis of rheumatic heart disease

22 Dilated cardiomyopathy Echocardiographic evidence, including at least two left ventricular measurements (decreased fractional shortening, decreased posterior wall thickness, or increased end-diastolic dimension) exceeding 2 standard deviations for age or for body surface area, The presence of dilated left atrium with poorly contractile left ventricle (left ventricular systolic or diastolic dysfunction) with LVEF less than 50%

23 Hypertrophic cardiomyopathy (HCM) Asymmetric septal hypertrophy, septal disorganization systolic anterior motion of the anterior leaflet of MV, the ratio of septal to posterior wall thickness is greater than 1.3

24 Restrictive cardiomyopathy echocardiographic pattern with one or both atria enlarged relative to ventricles of normal or small size with evidence of impaired diastolic filling and in the absence of significant valvar heart disease and shortened mitral and tricuspid deceleration time (<150 ms) which is shortened further during inspiration.

25 Others Questionnaire Socio-economic profile When to start Agreement

26 REFERENCES Robert l. Spicer, Stephanie M. Warie. Diseases of the Myocardium. In: Kliegman, Behrman, Stanton, St Geme, Schor editors, Nelson Textbook of Pediatrics. 20th edition vol.2 Canada: Elsevier; p Hong YM. Cardiomyopathies in children. Korean Journal of Pediatrics Feb 1; 56(2):52-9 Yuan SM. Cardiomyopathy in the pediatric patients. Pediatrics & Neonatology Jan 31 John F. Van Hare. Disturbances Of Rate and Rhythm of the Heart. In: Kliegman, Behrman, Stanton, St Geme, Schor editors, Nelson Textbook of Pediatrics. 20 th edition vol.2 Canada: Elsevier; p. 2250

27 Paediatric cardiomyopathy: new insights and development myopathy_talk.pdf Animasahun BA, Madise-Wobo AD, Ogunkunle OO, Gbelee OH, Oke DA. Cardiomyopathies among children attending a tertiary hospital in south-western Nigeria. J Clin Exp Res Cardiol. 2015;2(3):302 Bode-Thomas F, Ige OO, Yilgwan C. Childhood acquired heart diseases in Jos, north central Nigeria. Nigerian Medical Journal: Journal of the Nigeria Medical Association Jan;54(1):51 Sani UM, Ahmed H, Jiya NM. Pattern of acquired heart diseases among children seen in Sokoto, North-Western Nigeria. Nigerian Journal of Clinical Practice. 2015;18(6):718-25

28 Sadoh EW, Uzodimma CC, Daniels Q. Childhood acquired heart disease in Nigeria: an echocardiographic study from three centres. African Health Sciences. 2014;14(3): Animasahun BA, Madise-Wobo AD, Kusimo OY. Nigerian Children with Acquired Heart Disease: The Experience in Lagos. The Journal of Tehran University Heart Center Oct;12(4):160 Lee TM, Hsu DT, Kantor P, Towbin JA, Ware SM, Colan SD, Chung WK, Jefferies JL, Rossano JW, Castleberry CD, Addonizio LJ. Pediatric cardiomyopathies. Circulation Research Sep 15;121(7): Elliott P, Andersson B, Arbustini E, Bilinska Z, Cecchi F, Charron P, Dubourg O, Kühl U, Maisch B, McKenna WJ, Monserrat L. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. European Heart Journal Oct 4;29(2):270-6

29 Classification and definitions of cardiomyopathies. Bhulan KS, Krishna KP, Kanchan K, Syed EH. cdn.intechopen.com/.../intech Thiene G, Corrado D, Basso C. Revisiting definition and classification of cardiomyopathies in the era of molecular medicine.2007 Towbin JA, Lowe AM, Colan SD, Sleeper LA, Orav EJ, Clunie S, Messere J, Cox GF, Lurie PR, Hsu D, Canter C. Incidence, causes, and outcomes of dilated cardiomyopathy in children. Jama Oct 18;296(15): Animasahun BA, Madise-Wobo AD, Ogunkunle OO, Gbelee OH, Oke DA. A Descriptive Study about Dilated Cardiomyopathy in Children in a Tertiary Hospital in Nigeria. J Clin Exp Res Cardiol. 2015;2(1):102

30 Poothirikovil Venugopaland. Paediatric dilated cardiomyopathy emedicine from WebMD.2014 Poothirikovil Venugopalan. Paediatric dilated cardiomyopathy emedicine from WebMD.2014 Poothirikovil Venugopalan. Paediatric dilated cardiomyopathy emedicine from WebMD.2014 Hypertrophic cardiomyopathy. American Heart Association.

31 Christina Y Miyake, Charles I Berul.Paediatric hypertrophic cardiomyopathy emedicine from WebMD.2017 Lipshultz SE, Sleeper LA, Towbin JA, Lowe AM, Orav EJ, Cox GF, Lurie PR, McCoy KL, McDonald MA, Messere JE, Colan SD. The incidence of pediatric cardiomyopathy in two regions of the United States. New England Journal of Medicine Apr 24;348(17): Christina Y Miyake, Charles I Berul.Paediatric hypertrophic cardiomyopathy clinical presentation emedicine from WebMD.2017

32 Christina Y Miyake, Charles I Berul.Paediatric hypertrophic cardiomyopathy workup. emedicine from WebMD.2017 Kimberly Y Lin, Robert E Shaddy.Paediatric restrictive cardiomyopathy overview. emedicine from WebMD.2017

33 References Snider R (1997) Cardiomyopathies. In: Snider R, Serwer G, Ritter S, editors. Echocardiography in Pediatric Heart Disease. United States of America: Walsworth. 2nd ed Harmon WG, Sleeper LA, Cuniberti L, Messere J, Colan SD (2009) Treating Children with Idiopathic Dilated Cardiomyopathy (From the Pediatric Cardio- myopathy Registry). Am J Cardiol 104: Webber SA, Lipshultz S, Sleeper LA, Lu M, Wilkinson JD (2012) Outcomes of Restrictive Cardiomyopathy in Childhood and the Influence of Phenotype: A Report From the Pediatric Cardiomyopathy Registry. Circulation 126: