De Wolf Rik, van der Palen Roel L F, Ten Harkel Arend D J, Hazekamp Mark G, Blom Nico A
Department of Pediatrics, Division of Pediatric Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands.
Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, The Netherlands.
Eur Heart J Case Rep. 2024 Jul 10;8(7):ytae339. doi: 10.1093/ehjcr/ytae339. eCollection 2024 Jul.
Left ventricular apical pacing (LVAP) is considered to preserve left ventricular (LV) systolic function in both patients with and without congenital heart disease. However, sporadic LVAP-associated cardiac dysfunction in children with complex structural heart disease was recently reported. We present the case of a 2.5-year-old child with complex congenital heart disease and LVAP-induced cardiomyopathy.
Corrective surgery for double outlet right ventricle, subpulmonary ventricular septal defect, and transposition of the great arteries was done at the age of 1.5 months. Late complete atrioventricular block occurred, necessitating VVI pacemaker insertion with LV apical epicardial leads. He presented with heart failure and dilated cardiomyopathy 1.5 years after pacemaker insertion and required persistent circulatory support with intravenous inotropes. Speckle tracking echocardiography identified an important LV apical to basal dyssynchrony. After excluding any coronary artery involvement, cardiac resynchronization therapy was performed. Speckle tracking echocardiography guided lead placement resulted in improved LV contraction synchrony. Cardiac function recovered progressively in combination with oral heart failure medication and is almost normal at 10-month follow-up.
Right ventricular pacing is a well-known cause of pacing-induced cardiomyopathy. The LV apex and LV free wall are thought to be most optimal locations for ventricular pacing in children. However, LVAP can also be the cause of a pacing-induced cardiomyopathy and decreased systolic LV function in children with complex congenital heart disease due to lack of LV contraction synchrony. Cardiac resynchronization therapy can reverse this LV dysfunction and remodelling.
左心室心尖部起搏（LVAP）被认为可在先天性心脏病患者和非先天性心脏病患者中保留左心室（LV）收缩功能。然而，最近有报道称，患有复杂结构性心脏病的儿童出现散发性LVAP相关的心脏功能障碍。我们报告一例2.5岁患有复杂先天性心脏病并因LVAP诱发心肌病的患儿。
患儿1.5个月大时接受了右心室双出口、肺动脉瓣下室间隔缺损和大动脉转位的矫正手术。后期出现完全性房室传导阻滞，需要在左心室心外膜置入VVI起搏器电极。起搏器置入1.5年后，患儿出现心力衰竭和扩张型心肌病，需要静脉注射正性肌力药物进行持续循环支持。斑点追踪超声心动图显示左心室心尖部至心底存在明显不同步。排除冠状动脉受累后，进行了心脏再同步治疗。在斑点追踪超声心动图引导下放置电极，改善了左心室收缩同步性。结合口服心力衰竭药物，心脏功能逐渐恢复，随访10个月时几乎正常。
右心室起搏是起搏诱发心肌病的一个众所周知的原因。左心室心尖部和左心室游离壁被认为是儿童心室起搏的最佳位置。然而，由于缺乏左心室收缩同步性，LVAP也可能是患有复杂先天性心脏病儿童起搏诱发心肌病和左心室收缩功能下降的原因。心脏再同步治疗可以逆转这种左心室功能障碍和重塑。
