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Pentru
STUDENTI & MEDICI
2012-2013
Conf. Dr. Ioan Bostaca
Universitatea de Medicin Gr.T.Popa IASI
CURS 9/29 X 2012
CARDIOMIOPATIA
DILATATIV|
Report of the 1995 World Health
Organization/International Society and Federation of
Cardiology Task Force on the Definition and
Classification of Cardiomyopathies
Dilated Cardiomyopathy
Dilated cardiomyopathy is characterized by dilatation and impaired contraction
of the left ventricle or both ventricles. It may be idiopathic, familial/genetic,
viral3 4 5 and/or immune,6 7 alcoholic/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.
?
Dilated Cardiomyopathy
Dilated cardiomyopathy is characterized by dilatation and impaired contraction
of the left ventricle or both ventricles. It may be idiopathic, familial/genetic,
viral3 4 5 and/or immune,6 7 alcoholic/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.
CREB = cyclic AMP response-element binding protein
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.
Report of the 1995 World Health
Organization/International Society and Federation of
Cardiology Task Force on the Definition and
Classification of Cardiomyopathies
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.
C. Costel, 35 ani
ICT=0.62
C. Costel, 35 ani
Ecocardiografie
DTDVS=70 mm
DTSVS=55 mm
FE=35%
Hipokinezie difuza
Valve, septuri = fara modificari patologice semnificative
Absenta lichidului pericardic, a maselor intracardiace
C. Costel, 35 ani
C. Costel, 35 ani
AFECTAREA CARDIACA
MODIFICARI ECG
CARDIOMIOPATIE DILATATIVA
ARITMII
MODIFICARI ECG
R inalt in V1 cu raport R/S > 1
Unde Q in precordialele stangi
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 conducere
Cresterea eliminarilor de factor natriuretic atrial
Prolaps de valva mitrala
ARITMII
Tahicardia sinusala sustinuta sau labila
Scurtarea intervalului PR
Aritmii supraventriculare si ventriculare variate
Blocuri AV
Caz tipic de distrofie musculara Duchenne (varianta Becker-Kiener)
Debut in copilarie
Deficit 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
Caz tipic de distrofie musculara Duchenne (varianta Becker-Kiener)
Debut in copilarie
Deficit 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
TABLOU CLINIC
simptome [i semne
elemente anamnestice utile dg per exclusionem
DIAGNOSTIC
Radiografie toracic\
Ecg
Ecocardiografia
Angiocardiografie nuclear\ sau RMN (IRM)
Cateterismul cardiac
Biopsia endomiocardic\
9.10
B, 47 ani
Dg. clinic: Distrofie muscular\ progresiv\; CMD dilatativ\
9.11
B, 48 ani (fratele bolnavului cu ECG din imaginea precedent\)
Dg. clinic: Distrofie muscular\ progresiv\; CMD dilatativ\
9.1
B, 24 ani
Dg. clinic: CARDIOMIOPATIE DILATATIV|
Strategie
1. Deoarece cardiomiopatia dilatativ este o
boal terminal pentru diverse etiologii, n
consecin i tratamentul nu are nimic specific.
2. Cnd cauza este bine identificat,
tratamentul etiologic urc pe primul loc.
3.