cardiomiopatii CLASIFICARI NONCOMPACT TAKOTSUBO.pdf

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CURSURI CARDIOLOGIE

Pentru

STUDENTI & MEDICI

2012-2013

Conf. Dr. Ioan BostacaUniversitatea de MedicinGr.T.Popa IASI

CURS 9/29 X 2012


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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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Hypertensive cardiomyopathyoften presents with left ventricular hypertrophy in

association with features of dilated or restrictive cardiomyopathy with cardiac failure.

Inflammatory cardiomyopathyis defined by myocarditis in association with cardiac

dysfunction. Myocarditis is an inflammatory disease of the myocardium and isdiagnosed by established histological, immunological, and immunohistochemical

criteria. Idiopathic, autoimmune, and infectious forms of inflammatory

cardiomyopathy are recognized. Inflammatory myocardial disease is involved in the

pathogenesis of dilated cardiomyopathy and other cardiomyopathies, eg, Chagas'

disease, HIV, enterovirus, adenovirus, and cytomegalovirus.13

Metabolic cardiomyopathyincludes the following categories: Endocrine, eg,thyrotoxicosis, hypothyroidism, adrenal cortical insufficiency, pheochromocytoma,

acromegaly, and diabetes mellitus; familial storage disease and infiltrations, eg,

hemochromatosis, glycogen storage disease, Hurler's syndrome, Refsum's

syndrome, Niemann-Pick disease, Hand-Schller-Christian disease, Fabry-Anderson

disease, and Morquio-Ullrich disease; deficiency, eg, disturbances of potassium

metabolism, magnesium deficiency, and nutritional disorders such as kwashiorkor,anemia, beri-beri, and selenium deficiency; amyloid, eg, primary, secondary, familial,

and hereditary cardiac amyloidoses, familial Mediterranean fever, and senile

amyloidosis.


Organization/International Society and Federation ofCardiology Task Force on the Definition and

Classification of Cardiomyopathies
http://www.besancon-cardio.net/pro/recommandations/classif_c.htmhttp://www.besancon-cardio.net/pro/recommandations/classif_c.htm


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Hypertensive cardiomyopathyoften presents with left ventricular hypertrophy in

association with features of dilated or restrictive cardiomyopathy with cardiac failure.

Inflammatory cardiomyopathyis defined by myocarditis in association with cardiac

dysfunction. Myocarditis is an inflammatory disease of the myocardium and isdiagnosed by established histological, immunological, and immunohistochemical

criteria. Idiopathic, autoimmune, and infectious forms of inflammatory

cardiomyopathy are recognized. Inflammatory myocardial disease is involved in the

pathogenesis of dilated cardiomyopathy and other cardiomyopathies, eg, Chagas'

disease, HIV, enterovirus, adenovirus, and cytomegalovirus.13

Metabolic cardiomyopathyincludes the following categories: Endocrine, eg,thyrotoxicosis, hypothyroidism, adrenal cortical insufficiency, pheochromocytoma,

acromegaly, and diabetes mellitus; familial storage disease and infiltrations, eg,

hemochromatosis, glycogen storage disease, Hurler's syndrome, Refsum's

syndrome, Niemann-Pick disease, Hand-Schller-Christian disease, Fabry-Anderson

disease, and Morquio-Ullrich disease; deficiency, eg, disturbances of potassium

metabolism, magnesium deficiency, and nutritional disorders such as kwashiorkor,anemia, beri-beri, and selenium deficiency; amyloid, eg, primary, secondary, familial,

and hereditary cardiac amyloidoses, familial Mediterranean fever, and senile

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


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General system disease includes connective tissue

disorders, eg, systemic lupus erythematosus, polyarteritis

nodosa, rheumatoid arthritis, scleroderma, and

dermatomyositis. Infiltrations and granulomas include

sarcoidosis and leukemia.Muscular dystrophies include Duchenne, Becker-type,

and myotonic dystrophies.

Neuromuscular disorders include Friedreich's ataxia,

Noonan's syndrome, and lentiginosis.Sensitivity and toxic reactions include reactions to

alcohol, catecholamines, anthracyclines, irradiation, and

miscellaneous.

Alcoholic cardiomyopathy may be associated with a heavy

alcohol intake. At present we cannot define a causal versus a

conditioning role of alcohol or apply precise diagnostic

criteria.

Peripartal cardiomyopathy may first manifest in the

peripartum period. This is probably a heterogeneous group.



Cardiology Task Force on the Definition andClassification of Cardiomyopathies


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General system disease includes connective tissue

disorders, eg, systemic lupus erythematosus, polyarteritis

nodosa, rheumatoid arthritis, scleroderma, and

dermatomyositis. Infiltrations and granulomas include

sarcoidosis and leukemia.Muscular dystrophies include Duchenne, Becker-type,

and myotonic dystrophies.

Neuromuscular disorders include Friedreich's ataxia,

Noonan's syndrome, and lentiginosis.Sensitivity and toxic reactions include reactions to

alcohol, catecholamines, anthracyclines, irradiation, and

miscellaneous.

Alcoholic cardiomyopathy may be associated with a heavy

alcohol intake. At present we cannot define a causal versus a

conditioning role of alcohol or apply precise diagnostic

criteria.

Peripartal cardiomyopathy may first manifest in the

peripartum period. This is probably a heterogeneous group.


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Clasificarea CARDIOMIOPATIILOR (dup OMS, 1996)


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CLASIFICARE CLASIC

1. cardiomiopatii dilatative;2. cardiomiopatii hipertrofice (cu obstruc]ie sau f\r\

obstruc]ie): cardiomiopatia hipertrofic\ obstructiv\; cardiomiopatia hipertrofic\ neobstructiv\;

3. cardiomiopatia restrictiv\.


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CLASIFICARE(minimal, elementar)

Dilatativ\

Hipertrofic\

Restrictiv\

CM (displazia) aritmogen\ a VD


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DIMENSIUNI NORMALE CMD

CMH CMR


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Clasificarea CARDIOMIOPATIILOR (etiopatogenic) dup AHA


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Clasificarea CARDIOMIOPATIILOR (etiopatogenic) dup AHA


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ESC, Working Group on myocardial and pericardial diseases, 2008

Clasificare etiologic\:


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Clasificare etiologic\:A. Cardiomiopatii dilatative primitive (idiopatice).B. Cardiomiopatii dilatative secundare: cardiomiopatii inflamatorii:

infec]ioase (virusuri, bacterii, fungi); neinfec]ioase (colagenoze, granulomatoze).

cardiomiopatii nutri]ionale: avitaminoza B1; pelagra; scorbutul; deficitul de seleniu.

cardiomiopatii din boli endocrine [i metabolice: diabet zaharat (cardiomiopatia diabetic\); uremie (cardiomiopatia uremic\); tireotoxicoza (cardiotireoza); mixedem; feocromocitom (cardiomiopatia adrenergic\); acromegalia;


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Tablou clinicSimptomatologia se dezvolt\ lent, cu manifest\ri progresive de insuficien]\ cardiac\

stng\ [i dreapt\:

simptome de insuficien]\ cardiac\ stng\: dispnee, oboseal\, sl\biciunemuscular\. semne de insuficien]\ cardiac\ dreapt\:edeme;hepatomegalie;jugulare turgescente.Alte semne [i simptome: tahicardie sinusal\; sufluride insuficien]\ mitral\ sau tricuspidian\ (organo-func]ionale); galop atrial, ventricular sau de suma]ie.


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Alte elemente clinice sugestive pentru cardiomiopatie dilatativ\ primitiv\

(idiopatic\): existen]a n antecedentele pacientului a unor frecvente st\ri gripale

(miocardit\ cronic\); formare de trombusuri intracavitare, ce favorizeaz\ apari]ia emboliilor (siste-

mice sau pulmonare); comportamentul refractarla tratamentul clasic al insuficien]ei cardiace; vulnerabilitatea miocardului la tratamentul cu digital\, realiznd rapid

manifest\ri de intoxica]ie digitalic\ relativ\ (uneori aritmii amenin]\toare pentru

via]\),nainteaob]inerii efectului terapeutic scontat prin digitalizare optimal\.


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Explor\ri paraclinice1. Electrocardiogramaarat\ modific\ri nespecifice: tahicardie sinusal\; tulbur\ri de ritm ectopice: fibrila]ie atrial\; aspect de Q sau QS nderiva]iile V1-V4 sau nV5-V6

(aspect pseudo-infarct); anomalii ale segmentului ST [i ale undei T: subdenivelare de ST [i und\

T negativ\; tulbur\ri de conducere:

bloc de ram stng;blocuri atrio-ventriculare.


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2. Radiografia toracic\relev\:

cardiomegalie (ICT peste 0,55); staz\ venoas\ pulmonar\; radiografiile seriate arat\ progresia (evolu]ia natural\ defavorabil\) sau

regresia ICT (amelior\ri pasagere).


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3.Ecocardiografia: exclude alt\ boal\ ce evolueaz\ cu cardiomegalie; determin\ gradul de afectare a func]iei sistolice ventriculare; poate urm\ri ntimp evolu]ia bolii.

Modific\rile ecocardiografice sugestive pentru cardiomiopatie

dilatativ\ sunt:

hipokinezia difuz\ mai mult sau mai pu]in sever\; pere]i sub]iri; m\rirea diametrelor camerelor ventriculare; trombusuri intracavitare.


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Caracteristice pentru sc\derea debitului cardiacsunt: deschiderea limitat\ a valvei mitrale n diastol\,datorit\ unui flux transmitral sc\zut nchiderea sistolic\ incomplet\ a valvei mitrale

datorit\ cre[terii necorespunz\toare a presiunii dinventriculul stng; nchiderea precoce a valvei aortice datorit\

sc\derii ejec]iei ventriculului insuficient.Determin\ri Eco-cantitative privind performan]acardiac\: m\surarea frac]iei de ejec]ie;

m\surarea frac]iei de scurtare; eviden]ierea regurgita]iei mitrale sau

tricuspidiene.


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4.Angiocardiografia nuclear\, RMN (IRM)

- debitul cardiac,- frac]ia de ejec]ie, frac]ia de scurtare (cu o preciziemai mare dectecocardiografia)

5. Scintigrafia miocardic\efectuat\ cu diferite substan]eradiofarmaceutice poate exclude cardiopatia ischemic\.6.Cateterismul cardiacpoate determina, nplus,- presiunile telediastolice ventriculare, utile n

aprecierea disfunc]iei ventriculilor.- angiografia coronarian\ este normal\ (exclude

cardiomiopatia ischemic\ cu insuficien]\ cardiac\)

7. Biopsia endomiocardic\ este util\ n confirmareaunor boli specifice miocardice, cum ar fi sarcoidoza sauhemocromatoza.


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Specific ardiomyopathies

Definition and lassificationSpecific ardiomyopathies

References

The term specific cardiomyopathies is now used to describe heartmuscle diseases that are

associated with specific cardiac orsystemic disorders. These were previously defined as specificheart

muscle diseases.

Ischemic cardiomyopathypresents as a dilated cardiomyopathywith impaired contractile performancenot explained by the extentof coronary artery disease or ischemic damage.

Valvular cardiomyopathypresents with ventricular dysfunctionthat is out of proportion to the abnormal

loading conditions.



Cardiology Task Force on the Definition and

Classification of Cardiomyopathies
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.htm


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