cardiomiopatia dilatativa in patologia copilului
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cardiomiopatia dilatativaTRANSCRIPT
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CARDIOMIOPATIA DILATATIVA IN PATOLOGIA
COPILULUI
Prim autor: Ionescu Alexandra AN.V MG. Autor: Teches Simona AN. V MG
Autor: Comsulea Maria AN. V MG
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DEFINITIE
Cardiomiopatiile reprezinta un grup heterogen de afectiuni ale miocardului, asociate cu disfunctie mecanica si/sau electrica, ce duc de obicei la hipertrofie sau dilatatie ventriculara si sunt determinate de o varietate de cauze,frecvent genetice.Cardiomiopatiile pot fi limitate la inima sau fac parte din boli sistemice,conducand adesea la insuficienta cardiaca progresiva si deces.
Maron BJ, Towbin JA, Thiene G et al:Contemporary definitions and Clasification of the Cardiomiopathy-An AHA scientific Statement.Circulation 2006; 113:1807-1816
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IDIOPATICE
Dilatative Hipertrofica Restrictiva Displazia
aritmogena a VD Neclasificate
SPECIFICE
• Ischemica
• Valvulara
• Hipertensiva
• Metabolica (endocrina,
glicogenoze)
• Boli sistemice (sarcoidoza, leucoze,
boli infiltrative)
• Boli neuromusculare (ataxia
Fridereich, distrofia musculara
Duchenne)
• Peripartum
• Toxice (alcool,iradiere, citostatice)
CLASIFICARE CARDIOMIOPATII
Richardson P, McKenna W, Bristow M, et al: 1995 WHO/Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies. Circulation 93:841, 1996.
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DEFINITIE
Cardiomiopatia dilatativa (CMD) este o afectiune a miocardului de cauza necunoscuta insotita de dilatarea ventriculului stang/drept sau a ambilor ventriculi si reducerea functiei sistolice ce duce la insuficienta cardiaca congestiva.
Maron BJ, Towbin JA, Thiene G et al:Contemporary definitions and Clasification of the Cardiomiopathy-An AHA scientific Statement.Circulation 2006; 113:1807-1816
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INCIDENTA
Reprezinta forma clinica de cardiomiopatie cea mai frecvent intalnita la varsta copilariei.
Incidenta anuala: 0,57 cazuri la 100000 La copiii < 1 an: 4,4 cazuri la 100000 La copiii > 1 an: 0,34 cazuri la 100000
Towbin JA, Lowe AM, Colan SD, et al: Incidence causes and outcomes of dilated cardiomiopathy in children. JAMA, 2006; 296: 1867-1876
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ETIOPATOGENIA
Cele mai multe cazuri sunt idiopatice.Exista 4 ipoteze:
Factori familiali/genetici Miocardite virale Factori imunologici Anomalii metabolice
CMD cu etiologie cunoscuta:Boli de colagen Anomalii coronarieneSindroame malformative Deficite nutritionaleFactori toxici (chimioterapice, droguri, alcool)
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MANIFESTARI CLINICE Congestie venoasa
pulmonara Tahipnee Wheezing Raluri de staza Dificultati de alimentatie Iritabilitate
Congestie venoasa sistemica Hepatomegalie Edeme periferice-faciale
Modificari adaptative: Tahicardie Paloare
Afectarea debitului cardiac Puls periferic slab
perceput Hipotensiune-colaps
Supraincarcare de volum si presiune Modificari ale zgomotelor
cardiace Suflu sistolic de
regurgitare mitrala functionala
Alte modificari: Transpiratii Curba ponderala deficitara
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CLASIFICAREA INSUFICIENTEI CARDIACE
Adult si copil mare Sugar si copil mic
NYHA Clasa I:
fara limitarea activitatii fizice Clasa II:
limitare usoara a activitatii fizice
fara simptome in repaus Clasa III:
limitare importanta a activitatii fizice
aparitia simptomelor la eforturi usoare
Clasa IV: limitare severa a activitatii
fizice simptomele persista si in
repaus.
ROSS Clasa I:
fara limitarea activitatii fizice Clasa II:
tahipnee usoara sau diaforeza in timpul alimentatiei la sugar
dispnee de efort la copilul mare Clasa III:
tahipnee marcata sau diaforeza in timpul alimentatiei
retard ponderal important la sugar
dispnee marcata la efort Clasa IV:
tahipnee, tiraj intercostal, diaforeza
prezente in repaus la sugar si copilul mare
retard ponderal.
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EXPLORARI PARACLINICE
Radiografia toracica:CardiomegaliaStaza venoasa
pulmonara Indicele cardio-
toracic > 0,6Revarsate
pleurale
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ELECTROCARDIOGRAFIA
Evidentiaza: tahicardia sinusala, hipertrofii ventriculare si atriale, modificari ST-T, aritmii.
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ECOCARDIOGRAFIA
Demonstreaza: Dilatarea cavitatilorScaderea contractilitatiiRegurgitarea valvulara
Calculeaza fractia de ejectie
Exclude alte afectiuni cardiace
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CATETERISMUL CARDIAC SI ANGIOGRAFIA
Ofera date hemodinamice Permite efectuarea biopsiei
endocardiaceDiagnostic etiologic (miocardite)Prognostic in vederea transplantului
cardiac
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TRATAMENTObiective:
Ameliorarea fenomenelor de insuficienta cardiacaPrevenirea complicatiilor(trombembolii,aritmii)
1. TRATAMENT IGIENO-DIETETIC
Repaus in functie de severitatea insuficientei cardiace.
Regim alimentar: Hiposodat Aport caloric corespunzator Restrictie lichidiana-in forme severe
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2. TRATAMENT MEDICAMENTOS
1. Inotropice: Simpatomimetice: Dopamina, Dobutamina Tonicardiace: Digoxin, Lanoxin Levosimendan (modulator al canalelor ionice)
2. Diuretice: Furosemid Spironolactona Hidroclorotiazida
3. Vasodilatatoare: Inhibitorii ECA: Captopril, Perindopril
4. Beta-blocante: Carvedilol,Metoprolol
5. Altele: Terapia imunosupresiva, hormon de crestere, calciu blocante, peptid natriuretic.
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3.TRANSPLANT CARDIAC
Reprezinta metoda terapeutica eficienta la pacientii cu CMD la care celelalte modalitati terapeutice au esuat.
Indicatiile de transplant cardiac in CMD la copil:Simptomatologie grava cu insuficienta cardiaca
NIHA IVSperanta de viata sub 6 luniVarsta peste 12 ani
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REZULTATELE SI DISCUTIILE STUDIULUI
Studiul clinic si paraclinic:Un lot de 16 copii; Cu varsta medie de 4 ani; Internati in perioada 2009-2010.
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INCIDENTA
97,04%
CMD2,96%
Internari in perioada 2009-2010
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Baieti 43,75
%
Fete56,25%
Distributie pe sexe
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50%
31,25%
18,75%
Varsta de debut
< 1 an 1-3 ani > 3 ani
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ETIOLOGIE Infectie virala in
antecedente 18,75%:V.varicelo-zosterianV.rujeolicCMV
Nu s-a demonstrat implicarea virala in producerea bolii.
Id-iopatic 81,25
%
Infec-tie vi-rala
18,75%
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Medie50%
Severa31,25
%
Usora18,75%
Clasificare CMD
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NYHA III/IV37%
NYHA III19%
NYHA II12%
NYHA I32%
Clasificarea insuficientei cardiace
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EXAMINARI PARACLINICE
Electrocardiografie, Holter EKG Radiografie toracica Ecocardiografia:
Investigatia electiva pentru stabilirea diagnosticului
Urmarirea parametrilor sistolici la pacientii aflati sub tratament.
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TRATAMENT
0
2
4
6
8
10
12
14
16
DiureticeIECA: CaptoprilTonicardiace: DigoxinBeta-blocante: CarvedilolInotropic IV: Lev-osimendanTransplant cardiac
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COMPLICATII SI COMORBIDITATI
0
2
4
6
8
10
12
14
IM functionalaInfectii respiratoriiDefect septal: DSAHTPRetard ponderalTrombi intracardiaciTulburari de ritm
Complicatii si comorbiditati
Nr.
pacie
nti
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DURATA DE EVOLUTIE A CMD
•Durata medie de evolutie a bolii este de 2 ani.•La 11 pacienti boala evolueaza de 1 an.•La 3 pacienti evolueaza de 5 ani.
0.5 1 1.5 2 2.5 3 3.5 4 4.5 5 5.5 6 6.50
2
4
6
8
10
12
14
Series 1
Ani
Nr.
pacie
nti
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CONCLUZII
CMD este o problema grava de morbiditate si mortalitate infantila, a reprezentat 2,96 % din totalul internarilor in perioada 2009-2010.
Infectiile respiratorii superioare intalnite in proportie de 56,25% au constituit factori de decompensare a insuficientei cardiace.
Prognosticul este mediocru in ciuda eforturilor terapeutice si se coreleaza strans cu gradul de disfunctie al ventriculului stang.
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VA MULTUMESC !