<HTML><BODY style="word-wrap: break-word; -khtml-nbsp-mode: space; -khtml-line-break: after-white-space; "><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Dear Sergio, dear Dr Cagnolatti,</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">I do not think that it is possible to conlcude that the index case has </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Brugada Syndrome. Based on the description the 54 year old man has no </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">ECG pattern suggestive for Brugada Syndrome, he has no typical </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">arrhythmic event (monomorphic VT is no typical of the syndrome) . </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Obviously the pharmacological challenge (at least with procainamide if </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">in the country of Dr Cagnolatti ajmaline or flecainide are not </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">available) should be performed. I presume that Dr Cagnolatti has </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">excluded short QT Syndrome, long QT, CPVT and the other inherited </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">arrhythmogenic syndromes. I would like to know the trigger for the first </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">cardiac arrest, if the patient had experienced syncopal events or </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">palpitations before the cardiac arrest: such information may help assess </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">the probability that the patient has Brugada Syndrome. I would also try </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">to get an ECG other family members (does the patient have brothers and </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">sisters?) to evaluate if in the family there are other individuals with </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">an ECG suggestive for Brugada Sydnrome.</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Obviously the management of the son is the fundamental clinical </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">challenge. If the boy is asymptomatic, even in the presence of a </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">spontaneous pattern, I would do nothing else than monitoring his </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">clinical course. I would consider the implant of a Reveal subcutaneous </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">events monitor just to be sure that there are no arrhythmic events. I </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">think that data from several labs concur nowadays to dicourage </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">programmed electrical stimulation for risk stratification, but of course </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Dr Cagnolatti should decide whether he wants to propose this option to </DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">his patient: I would not.</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Silvia G Priori, MD;PhD</DIV><DIV><BR class="khtml-block-placeholder"></DIV><BR><DIV><BLOCKQUOTE type="cite"><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; min-height: 14px; "><BR></DIV> <DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Dear Sergio,</FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">About a case of reanimation of sudden death in a 54-year-old patient, with no structural heart disease, with all the studies made: coronary angiography, MNR, Holter, EPS. </FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Everything appears to be normal. Monomorphic arrhythmic storm is detected at the moment the patient is admitted. The patient is provided life support and he recovers in 24 hs. ICD implantation is decided. After three months he displays three shocks by arrhythmic storm, incessant ventricular tachycardia. Mapping and focus ablation is decided, in outflow tract with mismatch. </FONT></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">To this moment, the patient has not displayed any event. It's been three months, and in a Holter register in one of his sons, typical Brugada pattern is observed. The patient is 17 years old, asymptomatic. I cannot perform pharmacological test. What should I do next? </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">Dr. Cagnolatti <<A href="mailto:drcagnolattia@hotmail.com">drcagnolattia@hotmail.com</A>></FONT></DIV><FONT class="Apple-style-span" face="Arial"><BR></FONT><DIV> <SPAN class="Apple-style-span" style="border-collapse: separate; border-spacing: 0px 0px; color: rgb(0, 0, 0); font-family: Helvetica; font-size: 12px; font-style: normal; font-variant: normal; font-weight: normal; letter-spacing: normal; line-height: normal; text-align: auto; -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; ">--</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Dr.<SPAN class="Apple-converted-space"> </SPAN>Sergio<SPAN class="Apple-converted-space"> </SPAN>Dubner</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">President<SPAN class="Apple-converted-space"> </SPAN>of<SPAN class="Apple-converted-space"> </SPAN>Scientific<SPAN class="Apple-converted-space"> </SPAN>Committee</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-family: Lucida Grande; font-size: 13px; "><BR style="font-family: Lucida Grande; font-size: 13px; "></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Dr.<SPAN class="Apple-converted-space"> </SPAN>Edgardo<SPAN class="Apple-converted-space"> </SPAN>Schapachnik</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">President<SPAN class="Apple-converted-space"> </SPAN>of<SPAN class="Apple-converted-space"> </SPAN>Steering<SPAN class="Apple-converted-space"> </SPAN>Committee</DIV><DIV><BR class="khtml-block-placeholder"></DIV><DIV><BR class="khtml-block-placeholder"></DIV><BR class="Apple-interchange-newline"></SPAN> </DIV><BR><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">_______________________________________________</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Scd-forum mailing list</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><A href="mailto:Scd-forum@scd-symposium.org">Scd-forum@scd-symposium.org</A></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; "><A href="http://www.grupoakros.com.ar/mailman/listinfo/scd-forum">http://www.grupoakros.com.ar/mailman/listinfo/scd-forum</A></DIV> </BLOCKQUOTE></DIV><BR><DIV> <SPAN class="Apple-style-span" style="border-collapse: separate; border-spacing: 0px 0px; color: rgb(0, 0, 0); font-family: Helvetica; font-size: 12px; font-style: normal; font-variant: normal; font-weight: normal; letter-spacing: normal; line-height: normal; text-align: auto; -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; ">--</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Dr.<SPAN class="Apple-converted-space"> </SPAN>Sergio<SPAN class="Apple-converted-space"> </SPAN>Dubner</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">President<SPAN class="Apple-converted-space"> </SPAN>of<SPAN class="Apple-converted-space"> </SPAN>Scientific<SPAN class="Apple-converted-space"> </SPAN>Committee</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-family: Lucida Grande; font-size: 13px; "><BR style="font-family: Lucida Grande; font-size: 13px; "></DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">Dr.<SPAN class="Apple-converted-space"> </SPAN>Edgardo<SPAN class="Apple-converted-space"> </SPAN>Schapachnik</DIV><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; ">President<SPAN class="Apple-converted-space"> </SPAN>of<SPAN class="Apple-converted-space"> </SPAN>Steering<SPAN class="Apple-converted-space"> </SPAN>Committee</DIV><DIV><BR class="khtml-block-placeholder"></DIV><DIV><BR class="khtml-block-placeholder"></DIV><BR class="Apple-interchange-newline"></SPAN> </DIV><BR></BODY></HTML>