<HTML><BODY style="word-wrap: break-word; -khtml-nbsp-mode: space; -khtml-line-break: after-white-space; "><DIV class="MsoNormal"><SPAN lang="EN-US">Dear Dr. Perez Riera and other colleagues</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Last week I have met a special case, and here I want to get help from you.</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Female, 30 years old. She came into my hospital because of black spell and syncope for 2 weeks. The ECG shows atrial standstill and junctional/ventricular escape rhythm of 36, with atrium suspected to be retrograde excited (Fig 1***). After injection of 1 mg atropine for 10-20 min, the escape rate was only slightly increased to 50-55 bpm with standstill atrium (Fig 2***). After that isoprotorenol was intravenous infused to keep escape rate 50-55 bpm. Occasionally, paroxysmal atrial tachycardia/fibrillation and ventricular premature complex could be observed during monitoring, and AV was found not to be 1:1 conducted (Fig. 3***, P wave is clear especially on Lead V1). The routine blood test, heart enzyme, thyroid test and autoimmune system test are all inside normal range. Echocardiography reports the significant tricuspid annulus dilation, with tricuspid insufficiency, medium pulmonary artery hypertension, intra vena cava dilation (IVC 20), right system dilation (RA 52 X 61, RV 21), left atrial dilation (LA 43 X 57) and normal ventricular wall motion.</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">After observation for 2 days, the bradycardia continued and a DDDR pacemaker was implanted, with atrium lead in right appendage. During implantation the atrium was demonstrated to be retrograde activated, but no further EP test was performed. After implantation, the P wave could be only seen positively on lead V1, whereas in other leads it can’t be clearly observed (Fig 4***). Later echocardiography showed relatively weak right atrium motion, and left atrium standstill. Warfarin was administered to her with INR 1.8-2.5. </SPAN></DIV><DIV class="MsoNormal"><BR class="khtml-block-placeholder"></DIV><DIV class="MsoNormal">SEE ECGs</DIV><DIV class="MsoNormal"><A href="http://www.scd-symposium.org/files/yunlong1.jpg">http://www.scd-symposium.org/files/yunlong1.jpg</A></DIV><DIV class="MsoNormal"><A href="http://www.scd-symposium.org/files/yunlong2.jpg">http://www.scd-symposium.org/files/yunlong2.jpg</A></DIV><DIV class="MsoNormal"><A href="http://www.scd-symposium.org/files/yunlong3.jpg">http://www.scd-symposium.org/files/yunlong3.jpg</A></DIV><DIV class="MsoNormal"><A href="http://www.scd-symposium.org/files/yunlong4.jpg">http://www.scd-symposium.org/files/yunlong4.jpg</A></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"><B>Family histories: </B><O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Two years ago the lady got pregnant (during that period her ECG is normal) and then had a baby boy with normal ECG. She has a younger brother and a younger sister with normal ECG now. </SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">The lady’s father has 7 brothers and sisters, in which 4 of them died before 40 years old due to heart attack without clear diagnosis, because of the limited medical conditions in their hometown. Her father and one of her aunt have received pacemaker therapy around 20 years ago, and the patient did not know their clear diagnosis. Her father died from cerebral embolism later and her aunt also died later with uncertain reasons. </SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">She don’t know more about her family history now because most of her other relatives are scattering in China and living far away from her, and cannot be reached so easily. We are still encouraging her to find more of her family members.</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"><B>My question is:</B><O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><P class="MsoNormal" style="text-indent: -24px;margin-left: 18pt; "><SPAN lang="EN-US">1.<SPAN style="font:7.0pt " times="" new="" roman""=""> </SPAN></SPAN><SPAN lang="EN-US">Have you met such kind of familial bradycardia with atrial standstill and tricuspid annulus dilation before? </SPAN><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial" size="4"><SPAN class="Apple-style-span" style="font-size: 13.3333px;">Progressive Cardiac Conduction Defects (PCCD) or t</SPAN></FONT></SPAN><SPAN lang="EN-US">he </SPAN><SPAN lang="EN-US" style=""><FONT class="Apple-style-span" face="Arial" size="4"><SPAN class="Apple-style-span" style="font-size: 13.3333px;">Lev-Lenègre</SPAN></FONT></SPAN><SPAN lang="EN-US"> disease usually causes RBBB/LBBB or even complete AVB with sclerosis of the left "cardiac skeleton" (Mitral annulus abnormal). However, besides the sinus and/or atrial conduction defect, in this case right “cardiac skeleton” seems to be sclerosis. </SPAN></P><P class="MsoNormal" style="text-indent: -24px;margin-left: 18pt; "><SPAN lang="EN-US">2.<SPAN style="font:7.0pt " times="" new="" roman""=""> </SPAN></SPAN><SPAN lang="EN-US">The patient did not accept the cardiac Computed Tomography due to the cost. She doesn’t want EP test and myocardial biopsy now. In my hospital we cannot have a genetic test, either. What should we do now? If necessary we can encourage the patient to do more test.</SPAN></P><P class="MsoNormal" style="text-indent: -24px;margin-left: 18pt; "><SPAN lang="EN-US">3.<SPAN style="font:7.0pt " times="" new="" roman""=""> </SPAN></SPAN><SPAN lang="EN-US">After pacemaker implantation, her left atrium is standstill. Is it possible that her left atrium function could recover later?</SPAN></P><DIV class="MsoNormal" style="text-indent: 0px;"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Thanks for your time.</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">All the best</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US"> <O:P></O:P></SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Yunlong Xia</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Department of Cardiology</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">First Affiliate Hospital of </SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Dalian Medical University</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Dalian, China. 116011</SPAN></DIV><DIV class="MsoNormal"><SPAN lang="EN-US">Email: <A href="mailto:yunlong.xia@gmail.com">yunlong.xia@gmail.com</A></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><BR class="Apple-interchange-newline"></SPAN> </DIV><BR></BODY></HTML>