<HTML><BODY style="word-wrap: break-word; -khtml-nbsp-mode: space; -khtml-line-break: after-white-space; "><FONT class="Apple-style-span" face="Arial">Español - Portugués</FONT><DIV><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV><FONT class="Apple-style-span" face="Arial">Español</FONT></DIV><DIV><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Por lo que he visto, esta niña de Irán casi con seguridad sufre de síndrome de Jervell y Lange-Nielsen. En base a la descripción clínica, el uso de CDI es racional, mientras se continúa con terapia máxima de beta bloqueantes para reducir el riesgo de descarga. Yo sugeriría que los médicos a cargo de esta niña lean nuestro extenso estudio sobre síndrome de J-LN publicado en Circulation, 14 de febrero de 2006, que se basó en 186 pacientes. Estoy disponible para tipificar genéticamente a esta niña sin costo. </FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 13px/normal GB18030 Bitmap; min-height: 17px; "><FONT class="Apple-style-span" face="Arial" size="3"><SPAN class="Apple-style-span" style="font-size: 12px;"><BR></SPAN></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 13px/normal GB18030 Bitmap; min-height: 17px; "><FONT class="Apple-style-span" face="Arial" size="3"><SPAN class="Apple-style-span" style="font-size: 12px;"><BR></SPAN></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Peter J. Schwartz</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Professor Chairman</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Dept. of Cardiology</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">University of Pavia</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Pavia, Italy</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">---------</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Portugués</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Do que eu tenho visto, este pequeno garoto do Irã quase certamente sofre de síndrome de Lange-Nielsen. Baseado na descrição clínica o uso do CDI é racional, enquanto continua a terapia máxima com betabloqueadores para reduzir o risco de choques. Eu sugeriria que o´s médicos responsáveis por este pequeno menino lessem nosso extenso estudo sobre sindrome de J-LN publicado em 14 de feverieo de 2006 no Circulation, que foi baseado em 186 destes pacientes. Eu estou a disposição para genotipar esta criança de graça. </FONT></DIV><P style="margin: 0.0px 0.0px 0.0px 0.0px"><FONT class="Apple-style-span" face="Arial"> </FONT></P><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Peter J. Schwartz</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Professor Chairman</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Dept. de Cardiologia</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Universidade de Pavia</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Pavia, Itália</FONT></DIV><DIV><FONT class="Apple-style-span" face="Arial"><BR></FONT><DIV> <SPAN class="Apple-style-span" style="border-collapse: separate; -khtml-border-horizontal-spacing: 0px; -khtml-border-vertical-spacing: 0px; color: rgb(0, 0, 0); font-variant: normal; letter-spacing: normal; line-height: normal; -khtml-text-decorations-in-effect: none; text-indent: 0px; -apple-text-size-adjust: auto; text-transform: none; orphans: 2; white-space: normal; widows: 2; word-spacing: 0px; "><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">--</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Dr.</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Sergio</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Dubner</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">President</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">of</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Scientific</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Committee</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 13px/normal Lucida Grande; min-height: 16px; "><FONT class="Apple-style-span" face="Arial" size="3"><SPAN class="Apple-style-span" style="font-size: 12px;"><BR style=""></SPAN></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Dr.</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Edgardo</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Schapachnik</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">President</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">of</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Steering</FONT><SPAN class="Apple-converted-space"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Committee</FONT></DIV><DIV><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV></SPAN></DIV><DIV><BLOCKQUOTE type="cite"><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; min-height: 14px; "><FONT class="Apple-style-span" face="Arial"><BR></FONT></DIV> <FONT class="Apple-style-span" face="Arial">Español - Portugués</FONT><DIV><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV><FONT class="Apple-style-span" face="Arial">Español</FONT></DIV><DIV><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Estimado Dr. Dubner:</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 13px/normal Lucida Grande; min-height: 16px; "><FONT class="Apple-style-span" face="Arial" size="3"><SPAN class="Apple-style-span" style="font-size: 12px;"><BR></SPAN></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Gracias a Ud. y a sus colegas por este interesante simposio en linea. </FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Recientemente un niño de 5 años, sin evidencias de cardiopatía estructural fue derivado a nuestro centro para evaluar su síncope. También tenía historia de sordera congénita. Uno de los episodios de síncope ocurrió 48 hs luego de retirar los beta bloqueantes y el segundo episodio con 10 mg de propranolol tres veces por día. El ECG de superficie reveló un QTc=600 ms y frecuencia cardíaca de 55 lpm. Pensamos que el inicio en la niñez, sordera congénita y QTc=600 colocaría a este paciente en un riesgo mayor de eventos cardíacos y por lo tanto, sería un candidato para implante de CDI. Sin embargo, su magnitud corporal pequeña y riesgo futuro de terapia inadecuada con sus desvastadoras consecuencias, son aspectos desfavorables para el implante de CDI. Nuestros factores restrictivos para el aumento de la dosis de beta bloqueantes es una frecuencia cardíaca basal baja. Cuál es la recomendación del comité científico del simposio sobre MSC con respecto a nuestro paciente?</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 13px/normal Lucida Grande; min-height: 16px; "><FONT class="Apple-style-span" face="Arial" size="3"><SPAN class="Apple-style-span" style="font-size: 12px;"><BR></SPAN></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Desde ya les agradezco sus recomendaciones. </FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 13px/normal GB18030 Bitmap; min-height: 17px; "><FONT class="Apple-style-span" face="Arial" size="3"><SPAN class="Apple-style-span" style="font-size: 12px;"><BR></SPAN></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Cordialmente, </FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 13px/normal GB18030 Bitmap; min-height: 17px; "><FONT class="Apple-style-span" face="Arial" size="3"><SPAN class="Apple-style-span" style="font-size: 12px;"><BR></SPAN></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Majid Haghjoo,MD</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Tel: +98 21 2392 2931</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Fax:+98 21 2204 8174</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Tehran, Iran</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">-----------</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Portugués</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial"><BR class="khtml-block-placeholder"></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Caro Dr. Dubner,</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Obrigado a você e seus colegas por este belo simpósio. Recentemente, uma criança de 5 anos de idade sem evidência de doença cardíaca foi referida para o nosso centro para avaliação de síncope. Ele também tinha história de de surdez congênita. Um dos episódios de síncope ocorreu 48 horas após a retirada do betabloqueador e o segundo episódio em uso de de 10mg de propranolol 3 vezes ao dia. O ECG de superfície revelou QTc = 600ms e frequencia cardíaca de 55bpm. Nós pensamos queo início na infância, a surdez congênita e o QTc = 600ms colocariam este paciente num grupo de alto risco para eventos cardíacos e portanto seria um candidato para implante de CDI. Entretanto, o pequeno tamanho do corpo e o risco futuro de terapia inapropriada com suas consequências devastadoras são aspectos desfavoráveis para o implante do CDI. Nosso fatore limitante para aumentar a dose do betabloqueador foi a frequência cardíaca basal baixa. Qual a recomendação do comitê científico do simpósio de MCS a respeito dete paciente?</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 12px/normal Helvetica; min-height: 14px; "><FONT class="Apple-style-span" face="Arial"><BR></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Agradeço antecipadamente pela sua recomendação.</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 12px/normal Helvetica; min-height: 14px; "><FONT class="Apple-style-span" face="Arial"><BR></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Respeitosamente,</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; font: normal normal normal 12px/normal Helvetica; min-height: 14px; "><FONT class="Apple-style-span" face="Arial"><BR></FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Majid Haghjoo,MD</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Departmento de Marcapasso e Eletrofisiologia</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Centro Médico e de pesquisa Cardiovascular Rajaie</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Tel: +98 21 2392 2931</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Fax:+98 21 2204 8174</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Tehran, Iran</FONT></DIV></BLOCKQUOTE></DIV><FONT class="Apple-style-span" face="Arial"><BR></FONT></DIV></BODY></HTML>