Sudden death due to arrhythmogenic right ventricular cardiomyopathy: Two case reports

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of primary myocardial disease characterized by the regional or global replacement of right ventricular myocardium by fatty and fibrolipomatous tissues. The ARVC, usually presenting with different clinical manifestations and pathological changes, were mainly seen in young men and is one of the main causes of sudden death in the young. Here two autopsied cases of Chinese men aged 30 and 23 years old who appeared healthy but died suddenly while at work are reported respectively. One of the victims had extensive and severe pathological changes in his heart involving the left ventricular wall as well as the ventricular septum and the right atrium. Not only was there a global fatty and fibrolipomatous tissue replacement of the right ventricular myocardia, but also mild sarcoplasmic coagulation in the myocardium and focal lymphocytic infiltration in the myocardial interstitium of the right ventricular wall. In addition, slight atherosclerosis of the coronary artery and intimal thickening of the sino-atrial node were observed. It is believed that there are no marked differences in the pathological changes of ARVC between Chinese patients and patients from western countries. The etiology and pathogenesis of ARVC could not be explained by a single cause or factor and they are probably related to various congenital and acquired causes or factors.     

云南省宁蒗县不明原因猝死病例调查研究

目的探讨云南省宁蒗县不明原因猝死病例的死因。方法对云南省宁蒗县不明原因猝死病例进行现场流行病学调查,采集样本进行病理学、病毒学等相关检验及分析研究。结果猝死病例的临床表现类似阿-斯综合征发作,心电图示窦性心动过速、Q-T间期延长、ST段改变、室颤,病理检验主要以心肌间质水肿、弥漫性炎细胞浸润、心肌纤维断裂、心肌灶性坏死以及其他脏器炎细胞浸润等改变为主;同发病例的临床表现亦类似阿-斯综合征发作,心电图示Q-T间期延长、室扑,粪便标本病毒分离阴性,但是从中检测到了埃可病毒11型(ECHO11)的基因片段。结论猝死病例及同发病例与既往报道的云南地方性爆发性心肌炎类似,心肌炎性改变可能诱发快速心律失常,引起阿-斯综合征发作,最后导致发病甚至猝死。     

Primary Prevention of Sudden Cardiac Death in Heart Failure: Will the Solution Be Shocking?

Sudden cardiac death (SCD) may occur in as many as 40% of all patients who suffer from heart failure. This review describes the scope of the problem, risk factors for SCD, the effect of medications used in heart failure on SCD and the potential effect of the implantable cardioverter-defibrillator in primary prevention.     

Intratympanic steroid therapy for inner ear diseases, a review of the literature

To evaluate the value of clinical trials on intratympanic steroid therapy in Ménière’s disease (MD), idiopathic sudden sensorineural hearing loss (ISSNHL) and rapidly progressive sensorineural hearing loss (RPSNHL). Medline and Pubmed databases from 1966 to present were searched for clinical studies on intra- or transtympanic (cortico)steroid therapy of MD, ISSNHL and RPSNHL. Results were cross-checked with additional databases to obtain a complete data set. Clinical trials were evaluated on the basis of comparability, internal and external validity. Articles were judged using the following questions: was a randomised double-blind controlled trial performed? Which criteria were used to confirm the diagnosis of MD, ISSNHL, RPSNHL? Which therapy was evaluated? How long was the follow-up? Which criteria were used to evaluate the results? Reliable evidence on the efficiency, optimum dosage and administration schedule of intratympanic steroid therapy in MD, ISSNHL and RPSNHL is lacking, therefore further investigation is required.     

QT及JT离散度对心性猝死预测价值的探讨

测定３２例心性猝死和３０例非猝死性心性死亡病人入院后的首次心电图ＱＴ离散度（ＱＴｄ）和ＪＴ离散度（ＪＴｄ），产以３０例存活病人作对照，结果显示：（１）心性猝死组ＱＴｄ，ＪＴｄ较存活组和非猝死性心性死亡组显著增大（前者Ｐ均〈０．０１，后者Ｐ均〈０．０５，而非猝死性心性死亡组与存活组ＱＴｄ，ＪＴｄ比较差异均无统计学意义。（２）在心性猝死病人中，死亡直接原因为快速室性心律失常组（２３例）的ＱＴｄ，ＪＴｄ     

Prediction of Arrhythmic Events After Acute Myocardial Infarction Using Two Methods for Late Potentials Recording

One hundred consecutive patients recovering from an acute myocardiai infarction underwent, prior to home discharge, signal-averaged electrocardiography (ECG), left ventriculography. and 24-hour Holter ECG recording. The signal-averaged ECG was recorded and analyzed using two procedures: the orthogonal bipolar XYZ lead configuration with a bidirectional filter: and a precordial unipolar lead configuration with a uonrecursive digital filter. An abnormal signal-averaged ECG was seen in 40% of patients with the XYZ system and in 30% of patients in the precordial method, abnormal ejection fraction (< 40%) in 24% of patients and high grade ectopy activity in 22%. During the 24-month follow-up period, 12 patients (12%) had an arrhythmic event defined as either sudden death (11 patients) or sustained ventricular tachycardia (1 patient). Neither the signal-averaged ECG with the XYZ configuration, the abnormal ejection fraction, nor the high grade ectopy were able to statistically predict a higher arrhythmic event rate. The signal-averaged ECG with the precordial configuration was able to statistically predict a higher arrhythmic event rate, P < 0.03; odds ratio = 3.96. The combination of the orthogonal XYZ configuration signal-averaged ECG with the ejection fraction (P < 0.01, odds ralio = 7.33), or with ejection fraction and Holter monitoring (P < 0.06. odds ratio = 6.17) was able to predict a higher arrhythmic event rate. The combination of the precordial configuration signal-averaged ECG with the ejection fraction (P < 0.002, odds ratio = 14.4), or with ejection fraction and Holter monitoring (P < 0.06. odds ratio =10) was able to better predict a higher arrhythmic event rate. The combination of a normal or abnormal signal-averaged ECG and ejection fraction gave a sensitivity, specificity, positive, or negative value prediction of arrhythmic events of 60%, 90.6%, 37.5%, and 96%, respectively. It must be emphasized that the number of arrhythmic events during the 2-year follow-up was small and further study is required to determine the true predictive value of each method for arrhythmic events.     

Heart rate and late mortality in cardiac transplant recipients

There are currently 104 patients at this centre who have survivedat least 3 months after orthotopic cardiac transplantation.Seven of these long-term survivors have subsequently died andin three cases death was sudden and unexpected. All three ofthese patients had been noted to have inappropriately high restingheart rates (>130 b.min^–1) The rhythm was sinus tachycardia,supraventricular tachycardia or both intermittently. The heart rates of all 104 long-term survivors were recordedfrom ECGs taken at routine follow-up visits every 3 months forone year and annually thereafter. The overall mean heart ratewas 100±13.2 b.min^–1. Four patients, including the three identified above, had meanheart rates greater than the 95th centile. The mortality ratein this group is 75%. Four deaths have occurred in the remaining100 patients (P <0.001). In our series, an inappropriately high resting heart rate dueto sinus tachycardia or supraventricular tachycardia in long-termsurvivors of cardiac transplantation, is an adverse prognosticsign.