Congenital Heart Disease Center, Nara Medical University, Nara, Japan; Department of Transitional Medicine, Division of Congenital Heart Disease, Shizuoka General Hospital, Shizuoka, Japan; Department of Cardiology, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan.
Congenital Heart Disease Center, Nara Medical University, Nara, Japan.
J Cardiol. 2022 Jan;79(1):30-35. doi: 10.1016/j.jjcc.2021.08.017. Epub 2021 Sep 6.
Preexcitation-induced cardiomyopathy (PIC) is defined as a disease presenting ventricular dyssynchrony because of preexcitation through an accessory pathway (AP), being a cousin of pacing-induced cardiomyopathy. The present review aims at providing perspective of this uncharted subgroup.
In order to determine mechanisms and clinical characteristics of PIC, 63 patients in 29 literature reports were reviewed.
A median age at onset was 4 (0.1-59) years; 55 patients (87%) under 18 years old including 16 infants. Twenty patients (32%) experienced supraventricular tachycardia prior and subsequent to the PIC onset. Heart failure and left ventricular (LV) dysfunction did not correlate with other clinical features. All the 65 APs identified (duplicated in 2 patients) were located on the right side of the atrioventricular junction; at the septal area (in 55%) or the anterolateral aspect (in 22%). AP conduction was successfully eliminated by medical or interventional treatments where attempted. LV function returned to normal within 6 months in 67% of patients, while recovery took longer than 3 years in 8%. Frequently seen at the basal segments of the interventricular septum were early contraction within the QRS complex, dyskinesis at mid-systole, and aneurysm/bulging or local thinning.
Several characteristic factors became clear as described above. Rebound stretch following early shortening of the interventricular septum is seemingly the major mechanism of PIC, and thus a right septal or right anterior/anterolateral AP needs attention as a higher risk for PIC.
预激性心肌病(PIC)定义为由于旁道(AP)的预激而导致心室不同步的疾病,是起搏诱导性心肌病的表亲。本综述旨在提供该未知亚组的观点。
为了确定 PIC 的机制和临床特征,对 29 篇文献报告中的 63 例患者进行了回顾。
发病时的中位年龄为 4(0.1-59)岁;55 例(87%)年龄小于 18 岁,其中 16 例为婴儿。20 例(32%)在 PIC 发病前和发病后经历了室上性心动过速。心力衰竭和左心室(LV)功能障碍与其他临床特征无关。确定的 65 个 AP(在 2 名患者中重复)均位于房室结的右侧;在间隔区(55%)或前外侧(22%)。尝试通过药物或介入治疗消除 AP 传导。67%的患者在 6 个月内 LV 功能恢复正常,而 8%的患者恢复时间超过 3 年。在间隔的基底部经常看到 QRS 复合波内的早期收缩、收缩中期的运动障碍以及动脉瘤/膨出或局部变薄。
如上所述,出现了一些特征性因素。间隔的早期缩短后的反弹拉伸似乎是 PIC 的主要机制,因此右间隔或右前/前外侧 AP 需要引起注意,因为其 PIC 风险更高。