Hunt C E, Matalon S V, Thompson T R, Demuth S, Loew J M, Liu H M, Mastri A, Burke B
Am Rev Respir Dis. 1978 Jul;118(1):23-8. doi: 10.1164/arrd.1978.118.1.23.
Successful long-term phrenic nerve pacing has been reported in adults with acquired central hypoventilation syndrome. This report summarizes our experience with phrenic nerve pacing in 3 infants with congenital central hypoventilation syndrome. The electrodes were implanted in the lower thoracic portion of each phrenic nerve. In all patients. bilateral simultaneous pacing was required to maintain an adequate arterial PO2, tidal volume, and minute ventilation during quiet sleep. Case 1 died of problems primarily related to the severe cor pulmonale that had been present before pacemaker insertion; at autopsy, the pacemaker system was intact and there were no significant phrenic nerve abnormalities. Case 2 later developed failure of awake ventilatory control and died because of extensive phrenic nerve damage incurred by 19 days of continuous pacing. Case 3 has received quiet sleep pacemaker support since September 1977 and has been able to maintain normal quiet sleep ventilation in this manner. Phrenic nerve pacing can be successful in infants as long as continuous pacing is not required. Bilateral simultaneous pacing appears to be an appropriate alternative to home-based intermittent positive-pressure breathing for long-term management of children with central hypoventilation syndrome.
已有关于成功对患有获得性中枢性低通气综合征的成人进行长期膈神经起搏的报道。本报告总结了我们对3例先天性中枢性低通气综合征婴儿进行膈神经起搏的经验。电极植入每条膈神经的胸段下部。在所有患者中，在安静睡眠期间均需要双侧同时起搏以维持足够的动脉血氧分压、潮气量和分钟通气量。病例1死于主要与起搏器植入前就已存在的严重肺心病相关的问题；尸检时，起搏器系统完好，膈神经无明显异常。病例2后来出现清醒时通气控制衰竭，并因连续起搏19天导致广泛的膈神经损伤而死亡。病例3自1977年9月以来一直接受安静睡眠时的起搏器支持，并能够以这种方式维持正常的安静睡眠通气。只要不需要持续起搏，膈神经起搏在婴儿中可以成功。对于中枢性低通气综合征儿童的长期管理，双侧同时起搏似乎是家庭间歇性正压通气的合适替代方法。
