Basso C, Corrado D, Rossi L, Thiene G
Department of Pathology, University of Padua Medical School, Padua, Italy.
Circulation. 2001 Jan 16;103(2):269-75. doi: 10.1161/01.cir.103.2.269.
Sudden death (SD) in ventricular preexcitation (VP) syndrome is believed to be the result of atrial fibrillation with rapid ventricular response over the accessory pathway. Previous reports are anecdotal and often lack autopsy validation.
Prevalence and clinicopathological features of VP were investigated in a series of 273 SDs in children and young adults (aged <or=35 years). Site of accessory atrioventricular (AV) connection was predicted by 12-lead ECG. Right and left AV ring together with the sinoatrial and AV septal junction were studied in serial histological sections. Ten patients (3.6%; male, mean age 24+/-7 years) had VP: 8 had Wolff-Parkinson-White (WPW) and 2 had Lown-Ganong-Levine (LGL) syndrome. Six patients had previous symptoms, and SD occurred at rest in all but 1. Pathological substrates of LGL consisted of AV-node hypoplasia and right-sided atrio-Hisian tract, respectively. In the 8 WPW patients, 10 total accessory AV pathways consisting of ordinary myocardium were found (7 left lateral, 2 right posterolateral, and 1 septal). These pathways were close to the endocardium (mean distance, 750+/-530 microm) and 310+/-190 microm thick. In 4 WPW patients (50%), isolated acute atrial myocarditis was found, which was polymorphous in 1 and lymphocytic in 3.
VP accounted for 3.6% of SD in young people and was not preceded by warning symptoms in 40%. A left accessory pathway was the most frequent substrate, and its subendocardial location supports the feasibility of catheter ablation. Isolated atrial myocarditis may act as a trigger of paroxysmal atrial fibrillation that leads to SD.
心室预激（VP）综合征中的猝死（SD）被认为是房颤经旁路快速心室反应的结果。既往报道多为个案，且常缺乏尸检验证。
在一系列273例儿童及年轻成人（年龄≤35岁）猝死病例中研究VP的患病率及临床病理特征。通过12导联心电图预测房室（AV）旁路连接部位。对右、左房室环以及窦房和房室间隔连接处进行连续组织学切片研究。10例患者（3.6%；男性，平均年龄24±7岁）有VP：8例为预激综合征（WPW），2例为短P-R综合征（LGL）。6例患者既往有症状，除1例外在所有患者中SD均发生于静息状态。LGL的病理基础分别为房室结发育不全和右侧房希氏束。在8例WPW患者中，共发现10条由普通心肌构成的房室旁路（7条位于左侧，2条位于右侧后外侧，1条位于间隔部）。这些旁路靠近心内膜（平均距离750±530微米），厚度为310±190微米。在4例WPW患者（50%）中发现孤立性急性心房心肌炎，其中1例为多形性，3例为淋巴细胞性。
VP占年轻人猝死的3.6%，40%的患者无预警症状。左侧旁路是最常见的基础病变，其心内膜下位置支持导管消融的可行性。孤立性心房心肌炎可能是导致SD的阵发性房颤的触发因素。
