新生儿期起搏治疗免疫性先天性完全性房室阻滞3例

3例免疫性先天性完全性房室阻滞(CCAVB)的新生儿,20~24周胎龄起病,胎心率35~55次/min,母亲均诊断为干燥综合征,经激素等治疗胎儿CCAVB无逆转。分别于生后24 h、生后即刻及44日龄(校正胎龄36+5周)经剑突下小切口植入心外膜永久起搏器,电极固定于近右室流出道中后间隔部位。随访3个月至2.5年起搏阈值稳定,心功能及生长发育正常。     

Recovery from congenital complete atrioventricular block

Summary Congenital complete atrioventricular block without any other intracardiac anomaly reverted to sinus rhythm at the age of 7 years. The girl had had heart failure and Adams-Stokes attacks when aged 1 year, requiring pacemaker implantation. Electrocardiography confirmed sinus rhythm for the first time at the age of 7 years. At the same time, a treadmill test yielded a normal tracing without any arrhythmias.     

房室传导阻滞的起搏治疗

心脏起搏治疗各种原因导致的儿童完全性房室传导阻滞,使该类儿童患者寿命明显提高,已逐渐被儿科医生和患儿家长所接受.对于儿童起搏器植入的适应证、起搏电极的植入路径和位置的选择成为关注热点,相关指南和专家共识在不断更新.最新专家共识推荐心内膜起搏电极植入位置为右室间隔,心外膜起搏电极为左室,可避免由于长期右室心尖起搏左右心室不同步导致的心功能不全.     

Myocardial scintigraphy after pacemaker implantation for congenital complete atrioventricular block

Patients with isolated congenital complete atrioventricular block (CCAVB) occasionally develop dilated cardiomyopathy (DCM), despite early pacemaker implantation. However, the etiology of the DCM and its relationship to permanent ventricular pacing are not fully understood. Twenty-five patients with CCAVB underwent ^99m technetium (Tc) myocardial perfusion scintigraphy. Five patients were studied before and after pacing, providing a total of 30 image sets, which were divided into three groups; group 1: CCAVB before pacemaker implantation (PMI) (n = 11); group 2: CCAVB after PMI who did not subsequently develop DCM (n = 13); group 3: CCAVB after PMI who subsequently developed DCM (n = 6). Perfusion defects on single-photon-emission computed tomography (SPECT) were identified in group 1, 0 of 11 patients; group 2, 85% of patients; and group 3, 100% of patients. In groups 2 and 3, in patients with right ventricular pacing, the perfusion defects were mainly in the septum or between the apex and septum. On 20 segments’ polar maps, the distribution of %uptake showed a similar pattern in groups 2 and 3, the degree of decreased %uptake and the number of segments with decreased %uptake being more severe in group 3. “Artificial” left bundle branch block (LBBB) pattern myocardial contraction induced by right ventricular pacing decreased myocardial perfusion around the apex and septum. Some patients with CCAVB will develop left ventricular dysfunction caused by artificial LBBB-induced interventricular asynchrony.     

Congenital complete atrioventricular block

The various etiologies, pathologic findings, clinical concerns, and features of congenital complete atrioventricular block are presented and discussed. In addition, prenatal and antenatal diagnostic techniques are explained and analyzed. Lastly, treatment and the issues involved in deciding proper treatment are discussed in such a way that the general pediatrician can help the family to understand and handle the problem.     

Perinatal management of congenital complete atrioventricular block: Report of nine cases

Abstract Background: The addition of fetal ultrasonography has allowed the prenatal diagnosis and observation of congenital complete atrioventricular block (CCAVB). Thus, the management of the affected fetuses should be modified accordingly.
Methods: The medical records were reviewed to identify patients with CCAVB, and clinical and laboratory data were collected.
Results: Nine patients with CCAVB, diagnosed prenatally, were identified. The gestational age at the time of diagnosis ranged from 18 to 38 weeks (median, 25 weeks). The gestational age and birthweight ranged from 29 to 41 weeks and from 1420 to 4075 g, respectively. An accompanying congenital cardiac anomaly was noted in three cases, including one with polysplenia syndrome. The heart rate at birth ranged from 30 to 80 bpm. In three neonates, isoproterenol was given for bradycardia. One fetus with hydrops fetalis associated with complex heart disease was treated with maternally administered digoxin, with resolution of the fluid accumulation. A permanent pacemaker was implanted in six cases: two within 1 day of birth; two during the neonatal period; one at 51 days; and the other at 2 years and 11 months of age. There were no deaths or major complications and all have remained well beyond their infancy.
Conclusions: With the improvement of diagnostic methodology, more cases with CCAVB, including those with complex heart disease, could be diagnosed during fetal life. Their outcome has been steadily improving with the advances in perinatal management, pacemakers and implant techniques, irrespective of associated structural cardiac defects.     

Transient complete atrioventricular block during liver transplantation

Abstract:  Intraoperative dysrhythmias commonly occur in the surgical management of congenital heart diseases. It may also be seen in other surgical procedures. The initiating factors for an arrhythmia during surgery is usually a transient insult such as hypoxemia, cardiac ischemia, catecholamine excess, electrolyte abnormality and acidosis. CAVB is a life-threatening dysrhythmia in all ages. We herein report a case of transient CAVB in a 30-month-old boy during living-related liver transplantation for bile duct paucity-associated liver cirrhosis. Moreover, we discuss the probable etiology and treatment of CAVB in liver transplantation.