<HTML><BODY style="word-wrap: break-word; -khtml-nbsp-mode: space; -khtml-line-break: after-white-space; "><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">Dear Dr. Makarov: </FONT><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">About the </FONT></SPAN><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">essentiality</FONT></SPAN><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial"> to perform diagnostic studies in family members of a victim of SD by noncoronary origin and had no background of cardiological disease, from my point of view, they really need it.</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">We saw a 28-y-o young man last year who asked for help to make sure if he has the risk to die from heart disease.</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">The reason is that</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">his mother had SD when she was riding bicycle on the going to work road and his younger sister had SD when she was doing physical exercise in Junior High School.</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">The young man and his father were scared by these events.</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Fortunately, after doing examinations such as 12 lead ECG, Holter, Exercise test, Echocardiogram, no abnormality was found for him and his father, suggesting that the carriers of disease-causing gene (whatever they suffer from) might all gone and the left people may not be a carrier.</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">The first three useful diagnostic studies should be ECG (sometimes including Holter recording), Exercise test, Echocardiogram.</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial">Based on the indication from these data, further study such as EPS may be considered.</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial"> </FONT><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">Cuilan Li</FONT><SPAN style="mso-spacerun: yes"><FONT class="Apple-style-span" face="Arial"> </FONT></SPAN><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">Cuilan Li, Ph. D</FONT><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">Department of Cardiology,</FONT><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">Peking University People’s Hosp., </FONT><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">Beijing 100044, China</FONT><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial">Tel: 86-10-68314422 Ext. 5940</FONT><FONT class="Apple-style-span" face="Arial"><O:P></O:P></FONT></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial"><A href="mailto:licuilan@gmail.com">licuilan@gmail.com</A></FONT><O:P></O:P></SPAN></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></SPAN></DIV><DIV><BLOCKQUOTE type="cite"><DIV style="margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; min-height: 14px; "><BR></DIV><P align="justify" style="margin-top: 0px; margin-right: 0px; margin-bottom: 10px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Dear collegues! I want to add my comments to clinical case thad presented Dr. Cuilan Li et al. “Clinical presentation and management of a patient with catecholamine sensitive polymorphic ventricular tachycardia (CPVT)”. By my mention it is typical clinical and ECG pattern for children and young patients with some of kind of catecholaminergic VT (CVT) that we described before (Makarov L. et al. Short PR interval, high circadian index and bradycardia – pattern with high risk of syncope and sudden death in children with catecholaminergic ventricular tachycardia. Europ Heart J 2004; 25: 222, Suppl. Abstract) and during this symposium: bidirectional VT, bradycardia, short PR interval, and double tachycardia (VT and SVT). Some of this patients also demonstrated of the shortening of the QT interval (possibly as result of calcium overload of cardiomyocites). I am sure in arrythmogenic origin of syncope in present patient and useful of the active anthyarrhythmic therapy for it. From drugs more effective Beta-blockers (1-2 mg/kg) or/and calcium antagonists (verapamil), but authors had detected proarrhythmia from verapamil, that not typical for our experience. In the some patients the propafenone can be effective. Most of our patients with CVT showed improvement in their clinical symptoms: syncope events and ventricular tachycardia did not recur. But in a numerous patients that tolerant to therapy with recurrent syncope implantation of the ICD could be useful. I am not sure in useful of the EPI study for this patient, repetitive of Holter, stress test and/or IV infusion of isoproterenol more frequently detected typical bidirectional VT. Concern authors question # 3 (The catecholamine concentration in blood was normal for this patient. What do we expect of the catecholamine concentrations in CPVT patients in general?)- Fisher J.et al. (JACC1999; 34 (7): 2015-22) showed normal or lower level of plasma catecholapimes in rest in this patients, but parid increasing of it during exercise, to compare with normal range.</FONT></P><P align="justify" style="margin-top: 0px; margin-right: 0px; margin-bottom: 10px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Dr. Leonid Makarov M.D., Ph.D. professor (Pediatry)</FONT></P><P align="justify" style="margin-top: 0px; margin-right: 0px; margin-bottom: 10px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">Moscow Institute pediatry and children surgery.</FONT></P><P align="justify" style="margin-top: 0px; margin-right: 0px; margin-bottom: 10px; margin-left: 0px; "><FONT class="Apple-style-span" face="Arial">125412 Taldomskaya str.2 , Moscow, Russia.</FONT></P><P align="justify" style="margin-top: 0px; margin-right: 0px; margin-bottom: 10px; margin-left: 0px; "><A href="mailto:leo@oss.ru"><FONT class="Apple-style-span" face="Arial">leo@oss.ru</FONT></A></P><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><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></BODY></HTML>