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SUMMARY
Arrhythmogenic Right Ventricular Dysplasia is due to a type of cardiomyopthy genetically determined. This cardiomyopathy is marked
by myocardial cell loss with partial or total replacement of right ventricular muscle by adipose and brous tissue.
Those patients can be asymptomatic or ARVD can be an important cause of ventricular arrhythmia and sudden death. Biventricular heart
failure can be present too. In April of 2010 one task force had published a change in the diagnosis ndings of ARVD including the new
echocardiographic ndings and measurements in order to improve the echocardiographic diagnosis.
Descriptors: Arrythmogenic Right Ventricular Dysplasia; Echocardiography; Diagnosis
Introduo
A Displasia Arritmognica do Ventrculo Direito (DAVD) uma doena do msculo cardaco, geneticamente determinada, caracterizada por
substituio brogordurosa do miocrdio ventricular direito (VD), podendo acometer tambm o
miocrdio ventricular esquerdo (VE). Inicialmente,
a deposio brogordurosa ocorre no epicrdio ou
no intramiocrdio, evoluindo com extenso transmural, levando ao alamento e dilatao da parede
at a formao de aneurismas, tipicamente localizados nas pores inferior, apical e infundibular do
Instituio
Hospital Nossa Senhora de Ftima. Patos de Minas-MG
Correspondncia
Glauco Franco Santana
Rua Padre Caldeira n 386 Centro
38700-044 Patos de Minas MG
gsantana@cardiol.br
51
A presente reviso abordar os critrios ecocardiogrcos de DAVD, denindo as questes tcnicas do exame e os meios mais adequados para
melhorar a acurcia diagnstica e apontando para
potenciais metodologias de auxlio na investigao
da doena. Os critrios diagnsticos no baseados
no estudo ecocardiogrco fogem ao objetivo desta reviso, no sendo, portanto, alvo da discusso.
Histrico
Critrios diagnsticos
O ECO, por sua grande disponibilidade, baixo custo, ausncia de contraindicaes e ampla
experincia pelo uso h vrias dcadas, permanece importante ferramenta diagnstica na DAVD,
apesar de limitaes j plenamente conhecidas
da comunidade mdica, como janela inadequada
(principalmente em obesos e portadores de doena pulmonar obstrutiva crnica) e caractersticas
anatmicas especiais do ventrculo direito (VD).
O diagnstico de DAVD considerado denitivo quando ocorre a presena de 2 critrios maiores
ou 01 critrio maior e 2 critrios menores ou 4 critrios menores de diferentes categorias, e considerase diagnstico borderline a presena de 01 critrio
maior e 01 critrio menor ou 3 critrios menores
de diferentes categorias. A presena de 01 critrio
maior isolado ou 2 critrios menores de diferentes
categorias torna o diagnstico de DAVD possvel5.
Os critrios ecocardiogrcos de alteraes estruturais compatveis com DAVD, reconhecidas
ao ECO, so divididos em maiores e menores.
Os critrios maiores implicam, obrigatoriamente,
no achado de alteraes da motilidade regional
do VD acinesia, discinesia ou aneurisma (hipocinesia no considerado critrio diagnstico)
associados a um dos Tabela 2: Sensibilidade e especicidade dos critrios ecocardiogrcos para DAVD
seguintes achados: dimetro da via de sada do
VD (VSVD) ao corte
paraesternal eixo longo
maior do que 32mm
(ou dimetro corrigido
pela superfcie corprea
da VSVD maior do que
19mm/m2), ou dimetro da VSVD ao corte
paraesternal eixo curto
maior do que 36mm
(ou dimetro corrigido
pela superfcie corprea
maior que 21 mm/m2),
ou variao da rea do
VD menor ou igual a
CPL corte paraesternal longitudinal, CPT corte paraesternal transversal, VSVD via de sada
33%.
do ventrculo direito, VD ventrculo direito
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Aspectos tcnicos
O dimetro da VSVD deve ser medido na teledistole, na deexo do complexo QRS. No corte
paraesternal longitudinal do VE, deve-se medir na
poro proximal da VSVD (Figura 2). No corte
paraesternal transversal da base do corao, a dimenso linear da VSVD deve ser medida da parede artica anterior at a parede livre do VD, acima
da valva artica (Figura 3). Tais localizaes referem-se poro proximal da VSVD mais adequa54
Novas tcnicas
Referncias
1.
Basso C, Corrado D, Marcus FI, Nava A, Thiene G. Arrythmogenic right ventricular cardiomyopathy. Lancet.
2009;373(9671):1289300.
2. Anderson EL. Arrhythmogenic right ventricular dysplasia. Am Fam Physician. 2006;73(8):1391-8.
3. Dalal D, Nasir K, Bomma C, Prakasa K, Tandri H,
Piccini J, et al. Arrhythmogenic Right Ventricular
Dysplasia: A United States Experience. Circulation.
2005;112(25);3823-32.
4. Diagnosis of arrhythmogenic right ventricular dysplasia
cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the Europeam Society
of Cardiology and of the Scientc Corenal on Cardiomyopathies of the International Society and Federation
of Cardiology; McKennal WJ, Thine G, Nera A et al. Br
Heart J. 1994;71(3):2158.
5. Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce
B, Bluemke DA, et al. Diagnosis of arrhythmogenic
right ventricular cardiomyopathy/dysplasia - proposed
modication of the Task Force Criteria. Circulation.
2010;121(13):1533-41.
6. Baran A, Nanda NC, Falko M, Barold SS, Gallagher JJ.
Two dimensional echocardiographic detection of arrythmogenic right ventricular dysplasia Am Heart J. 1982;
103(6):1066-7.
7. Blomstrm-Lundqvist C, Beckman-Suurkla M, Wallentin I, Jonsson R, Olsson SB. Ventricular dimensions
and wall motion assessed by echocardiography in patients
with arrhythmogenic right ventricular dysplasia. Eur Heart J. 1988;9(12):1291-302.
8. Yoerger DM, Marcus FI, Sherrill D, Calkins H, Towbin JA, Zareba W, et al. Echocardiographic ndings in
patients Meeting Task Force Criteria for Arrhythmogenic Right Ventricular Dysplasia. J am Coll Cardiol.
2005;45(6):860 5.
9. Horton KD, Meece RW, Hill JD. Assessment of the right
ventricle by echocardiography: a primer for cardiac sonographers. J Am Soc Echocardiogr. 2009;22(7):776-92.
10. Rudski LG, Lai WW, Alalo J, Hua L, Handschumacher
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
MD, Chandrasekaran K, et al. Guidelines for the echocardiographic assessment of the right heart in adults: a
report from the American Society of Echocardiography. J
Am Soc Echocardiogr. 2010;23(7):685-713.
Suaide Filho CE. Ecocardiograa : princpios e aplicaes clnicas.Rio de Janeiro: Livraria e Editora Revinter
Ltda; 2007.
Prakasa KR, Wang J, Tandri H, Dalal D, Bomma C,
Chojnowski R, et al. Utility of tissue Doppler and
strain echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Am J Cardiol.
2007;100(3):507-12.
Teske AJ, Cox MG, De Boeck BW, Doevendans PA,
Hauer RN, Cramer MJ. Echocardiographic tissue deformation imaging quanties abnormal regional right
ventricular function in arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Soc Echocardiogr.
2009;22(8):920-7.
Aneg MA, Lindstrom L, Fluur C, Nylander E. Longterm follow-up in arrhythmogenic right ventricular cardiomyopathy using Tissue Doppler Imaging. Scand Cardiovasc J. 2008;42(6):368-74.
Teske AJ, Cox MGPJ, Peterse MC, Cramer MJ, Hauer
RNW. Case report: Echocardiographic deformation imaging detects left ventricular involvement in a young boy
with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Int J Cardiol. 2009;135(1):e24-6.
Teske AJ, De Boeck BWL, Melman PG, Sieswerda GT,
Doevendans PA, Cramer MJM. Echocardiographic
quantication of myocardial function using tissue deformation imaging, a guide to image acquisition and analysis using tissue Doppler and speckle tracking. Cardiovasc
Ultrasound. 2007,5:27.
Herbots L, Kowalski M, Vanhaecke J, Hatle L, Sutherland
GR. Characterizing abnormal regional longitudinal function in arrhythmogenic right ventricular dysplasia. The
Potential Clinical Role of Ultrasonic Myocardial Deformation Imaging. Eur J Echocardiogr. 2003;4(2):1017.
Shiota T. 3D echocardiography: evaluation of the right
ventricle. Curr Opin Cardiol. 2009,24(5):4104.
Kjaergaard J, Svendsen JH, Sogaard P, Chen X, Nielsen
HB, Kber L, et al. Advanced quantitative echocardiography in arrhythmogenic right ventricular cardiomyopathy. J Am Soc Echocardiogr. 2007;20(1):27-35.
Prakasa KR, Dalal D, Wang J, Bomma C, Tandri H,
21.
22.
23.
24.
25.
26.
27.
28.
29.
30.
Dong J, et al. Feasibility and variability of three dimensional echocardiography in arrhythmogenic right
ventricular dysplasia/cardiomyopathy. Am J Cardiol.
2006;97(5):7039.
Olszewski R, Timperley J, Cezary S, Monaghan M,
Nihoyannopoulis P, Senior R, et al. The clinical applications of contrast echocardiography. Eur J Echocardiogr.
2007;8(3):S13-S23.
Lpez-Fernndez T, Garca-Fernndez MA, David EP,
Yangela MM. Usefulness of contrast echocardiography
in arrhythmogenic right ventricular dysplasia. J Am Soc
Echocardiogr. 2004;17(4):391-3.
Nemes A, Vletter WB, Scholten MF, ten Cate FJ. Contrast echocardiography for perfusion in right ventricular
cardiomyopathy. Eur J Echocardiogr. 2005;6(6): 470-2.
Camarozano A, Rabischosky A, Maciel BC, Brindeiro
Filho D, Horowitz ES, Pena JLB, et al/ Sociedade Brasileira de Cardiologia. Diretrizes das indicaes da ecocardiograa. Arq Bras Cardiol. 2009;93(6 supl.3):e265-73.
Patel MR, Spertus JA, Brindis RG, Hendel RC, Douglas
PS, Peterson E,et al. ACCF proposed method for evaluating the appropriateness of cardiovascular imaging. J Am
Coll Cardiol. 2005;46(8):1606-13.
Marcus FI, Zareba W, Calkins H, Towbin JA, Basso
C, Bluemke DA, et al. Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation
and diagnostic evaluation: Results from the North
American Multidisciplinary Study. Heart Rhythm.
2009;6(7):984 92.
Wood MJ, Picard MH. Utility of echocardiography in
the evaluation of individuals with cardiomyopathy. Heart. 2004;90(6):70712.
Bilge M, Eryonucu B, Gler N. A case of arrithmogenic right ventricular cardiomyopathy in sinus rhythm
associated with thrombus in the rigth atrium. J Am Soc
Echocardiogr. 2000;13(2):1546.
Hulot JS, Jouven X, Empana JP, Frank R, Fontaine G.
Natural history and risk stratication of arrhythmogenic
right ventricular dysplasia/cardiomyopathy. Circulation.
2004;110(14);1879-84.
Lemola K, Brunckhorst C, Helfenstein U, Oechslin E,
Jenni R, Duru F. Predictors of adverse outcome in patients with arrhythmogenic right ventricular dysplasia/
cardiomyopathy: long term experience of a tertiary care
centre. Heart. 2005;91(9):116772.
57