Cardiomiopatii dilatative.pdf

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    CARDIOMIOPATIA

    DILATATIV|

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    Report of the 1995 World Health

    Organization/International Society and Federation of

    Cardiology Task Force on the Definition andClassification of Cardiomyopathies

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    Dilated Cardiomyopathy

    Dilated cardiomyopathy is characterized by dilatation and impaired contractionof the left ventricle or both ventricles. It may beidiopathic,familial/genetic,viral345and/or immune,67alcoholic/toxic, or associated with recognized

    cardiovascular disease in which the degree of myocardial dysfunction is not

    explained by the abnormal loading conditions or the extent of ischemic damage

    (see below). Histology is nonspecific.

    http://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htm
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    Dilated Cardiomyopathy

    Dilated cardiomyopathy is characterized by dilatation and impaired contractionof the left ventricle or both ventricles. It may beidiopathic,familial/genetic,viral345and/or immune,67alcoholic/toxic, or associated with recognized

    cardiovascular disease in which the degree of myocardial dysfunction is not

    explained by the abnormal loading conditions or the extent of ischemic damage

    (see below). Histology is nonspecific.

    http://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htm
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    CREB = cycl ic AMP response-element binding protein

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    C. Costel, 35 ani

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    Ecocardiografie

    DTDVS=70 mm

    DTSVS=55 mmFE=35%

    Hipokinezie difuza

    Valve, septuri = fara modificari patologice semnificative

    Absenta lichidului pericardic, a maselor intracardiace

    C. Costel, 35 ani

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    AFECTAREA CARDIACA

    MODIFICARI ECG

    CARDIOMIOPATIE DILATATIVA

    ARITMII

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    MODIFICARI ECG

    R inalt in V1 cu raport R/S > 1

    Unde Q in precordialele stangi

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    CARDIOMIOPATIA DILATATIVA

    Prezenta la toate cazurile de la varsta de 18 ani

    (afectarea infraclinica este prezenta de la varsta de 6 ani)

    Forma clinica de CMD mai severa in varianta Becker-Kiener

    Este cauza de deces la 10% din cazuri

    Morfologic: fibroza miocardica (peretele VS postero-bazal +

    parcelar sept, VD, AD)

    Modificari degenerative ale sistemului de conducereCresterea eliminarilor de factor natriuretic atrial

    Prolaps de valva mitrala

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    ARITMII

    Tahicardia sinusala sustinuta sau labila

    Scurtarea intervalului PR

    Aritmii supraventriculare si ventriculare variate

    Blocuri AV

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    Caz tipicde distrofie musculara Duchenne (varianta Becker-Kiener)

    Debut in copilarie

    Defic it muscular centura pelvina + evolutie spre centura scapulo-humerala

    Pseudohipertrofie a gambei

    Mers leganat

    Hiperlordoza

    Manevra Gowers* pozitiva

    +

    CARDIOMIOPATIE DILATATIVA

    Pronostic rezervat

    Indicatie de transplant cardiac

    C. COSTEL, 35 ani

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    Caz tipicde distrofie musculara Duchenne (varianta Becker-Kiener)

    Debut in copilarie

    Defic it muscular centura pelvina + evolutie spre centura scapulo-humerala

    Pseudohipertrofie a gambei

    Mers leganat

    Hiperlordoza

    Manevra Gowers* pozitiva

    +

    CARDIOMIOPATIE DILATATIVA

    Pronostic rezervat

    Indicatie de transplant cardiac

    C. COSTEL, 35 ani

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    TABLOU CLINIC

    simptome [i semne

    elemente anamnestice utile dgper exclusionem

    DIAGNOSTIC

    Radiografie toracic\Ecg

    Ecocardiografia

    Angiocardiografie nuclear\ sau RMN (IRM)

    Cateterismul cardiac

    Biopsia endomiocardic\

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    B 47 ani

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    9.10

    B, 47 ani

    Dg. clinic: Distrofie muscular\ progresiv\; CMD dilatativ\

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    B 24 ani

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    9.1

    B, 24 ani

    Dg. clinic: CARDIOMIOPATIE DILATATIV|

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    Strategie1. Deoarece cardiomiopatia dilatativeste oboalterminalpentru diverse etiologii, n

    consecini tratamentul nu are nimic specific.2. Cnd cauza este bine identificat,tratamentul etiologic urcpe primul loc.3. n final, tratamentul este identic cu cel al

    insuficienei cardiace, indiferent de cauz.

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    Tratament farmacologic

    1. diuretice: spironolactona 100 - 200 mg pe zi (n funcie de severitateasindromului de retenie hidrosalin);

    2. inhibitori ECA;3. betablocante: carvedilol 3,25 mg - 25 mg/zi, bisoprolol 2,5 - 10 mg/zi,

    metoprolol 50 - 150 mg/zi;4. anticoagulantele: pot fi considerate ca utile la toi pacienii cu cardio-

    miopatie dilatativ, n special la cei care asociaz i fibrilaie atrial (cure de 10-14zile cu heparin cu greutate molecular mic, urmate de preparate oraledicumarinice, pe termen nelimitat)

    5. digitalicele se utilizeaz cu precauie sporit datorit riscului de intoxicaiedigitalic relativ, pe o fibr miocardic alungit de stresul tensiunii parietaleolul trebuie restricionat sever, pn la sevrage absolut;

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    Tratament chirurgical

    Tran sp lan t ca rd iac