Fetal autoimmune atrioventricular block (AVB) is a rare but potentially life-threatening condition. It results from the passage of maternal anti-SSA/Ro or Anti SSB/La auto-antibodies into the fetal circulation, leading to inflammation and fibrosis of the AV node and often to irreversible damage. Besides AVB, these antibodies can also cause cardiomyopathies, but there is no evidence linking them to tachyarrhythmias. We present the case of a patient with significant risk factors for fetal AVB: a prior history of hydrops fetalis, high anti-SSA/Ro antibody levels and hypothyroidism. In this case, the use of dexamethasone and intravenous immunoglobulin may have contributed to reversing the first-degree atrioventricular block detected at 19 weeks of gestation. Additionally, at 21 weeks, the fetus developed a tachyarrhythmia that needed treatment with flecainide. Soon after the birth, the newborn underwent ECG Holter and Wolff-Parkinson-White Syndrome (WPWS) was diagnosed. To our knowledge, the coexistence of fetal AVB and WPWS has never been described.

Complete congenital heart block (CHB), a rare and fatal bradyarrhythmia observed in children, carries significant mortality and morbidity. When congenital heart block occurs in isolation with a structurally normal heart, it prompts suspicion of an autoimmune etiology, wherein maternal antibodies are transmitted transplacentally, impacting the fetal conducting system. The manifestation of congenital complete atrioventricular block (CCAVB) can lead to complications such as dilated cardiomyopathies, arrhythmias, and fibroelastosis in certain cases. Notably, dilated cardiomyopathy is a significant prognostic factor in children diagnosed with congenital heart block. Pathological investigations have revealed the presence of antibodies, complements, and indicators of inflammation or fibrosis across the myocardium, emphasizing the shared molecular mechanisms between CCAVB and the development of dilated cardiomyopathy (DCM). This article presents the case of a one-year-old female child who presented with signs of dilated cardiomyopathy, later identified through retrospective evaluation as having autoimmune congenital heart block. The mother of the child was diagnosed with Sjogren\'s syndrome, characterized by positive anti-RO titers. Remarkably, the child remained asymptomatic for a year without the need for pacing intervention. The child\'s condition was successfully stabilized with appropriate treatment, and plans for pacemaker insertion will be considered once specific criteria are met. The onset of cardiomyopathy in a known case of CCAVB should serve as a crucial alert for prognostic considerations and the potential necessity for early-pacing intervention.

BACKGROUND: It has been reported that the complete heart block (CHB) in neonatal lupus (NL) cannot be reversed. This study reported a case of NL-CHB that was reversed by transcutaneous pacing and repeated plasmapheresis.
METHODS: A 35+ 6-week male preterm baby was transferred to the neonatal intensive care unit of the Army Medical Center in May 2020 for slight cyanosis around the lips and nose. Two days after birth, a sudden decrease in heart rate was observed during electrocardiogram (EGG) monitoring. Physical examination revealed a bluish-purple discoloration around the lips and an irregular heartbeat. EGG showed the presence of isolated P (142 bpm) and QRS (78 bpm) waves, ventricular escape beats, and a diagnosis of NL-CHB. To reverse the condition, transcutaneous pacing and five sessions of plasmapheresis were performed. At a 1.5-year follow-up, the baby exhibited well-developed cardiac structure and normal neurodevelopment.
CONCLUSIONS: Transcutaneous pacing and repeated plasmapheresis might be possible to reverse CHB in NL.

Systemic lupus erythematosus (SLE) is a heterogeneous chronic, multisystem, inflammatory autoimmune disorder with variable clinical features, with its manifestations being attributed to the presence of multiple autoantibodies and their subsequent autoimmune reactions. Multiple organs may be involved, with the kidneys, the joints, and the skin being the most common, increasing maternal and fetal morbidity and mortality. Our current article describes the case of a 32-year-old primigravida who was referred to our department after the detection of fetal bradycardia and the strong suspicion of an underlying cardiac abnormality. After a detailed fetal and maternal assessment, the diagnosis of SLE-associated fetal congenital heart block was established, and the appropriate management and treatment were provided, factors that led to the uncomplicated delivery and prompt successful management of an otherwise severely affected fetus. Our work, also, includes a detailed review of the accumulated evidence regarding the association between autoantibodies and congenital heart block, the available screening modalities of the condition, and its potential therapeutic interventions.

Cardiac electrical stimulation in children usually is needed in the setting of complete congenital atrioventricular block, atrioventricular block after heart surgery, and bradycardia associated with some specific channelopathies. In cases of atrioventricular block, the high percentage of ventricular stimulation raises concern on the deleterious effects of chronic stimulation of the right ventricle. In recent years, physiologic stimulation has developed as a valid approach for adult patients and a great interest has risen in offering conduction system pacing also to the pediatric population. We present three pediatric cases of stimulation of the conduction system (His bundle or left bundle branch), in order to show the intrinsic particularities and challenges implied in these new techniques.

UNASSIGNED: The SelectSecure lumenless 3830 pacing lead is often considered to be the pacing lead of choice for transvenous pacing in children because of its small diameter, lead strength, and reliable long-term sensing and pacing characteristics. One of the potential long-term pitfalls of a sturdy pacing lead is relative retraction with growth in children resulting in late lead dislodgement.
UNASSIGNED: We report two cases of late SelectSecure 3830 lead dislodgement at 11.8 years (Case 1) and 8.8 years (Case 2), respectively, post the initial implantation. Case 1 was diagnosed with congenital complete heart block (CHB) at 9 months old when he presented with unconfirmed diphtheria infection. Case 2 was diagnosed with CHB at 14 weeks of age with positive maternal anti-Ro antibodies. Both patients underwent implantation of a transvenous permanent pacemaker implantation with Medtronic SelectSecure 3830 lead due to symptomatic bradycardia. Apart from a pulse generator change at 8.5 years (Case 1) and 7 years (Case 2), respectively, post-implant due to normal battery depletion, both patients are well in the interim.
UNASSIGNED: As part of the pacemaker follow-up for rapidly growing children, we recommend more frequent surveillance of lead \'tautness\' by chest radiography especially in children with CHB with no underlying heart rhythm.

Anti-Ro/SSA antibodies are associated with a risk of 1-2 % to develop complete atrioventricular block (AVB) in fetuses of positive mothers. Complete AVB is irreversible, but studies suggest that anti-inflammatory treatment during the transition period from a normal fetal heart rate (FHR) to an AVB might stop this progression and restore sinus rhythm. The most efficient method for diagnostic evaluation of this arrhythmia is the pulsed-Doppler fetal echocardiography. However, weekly or bi-weekly recommended fetal echocardiographic surveillance can rarely identify an AVB in time for treatment success, also because the transition from a normal rhythm to a third degree AVB is very fast. Daily FHR monitoring in a medical facility could increase the chances of identifying the AVB onset but is difficult to realize. For this reason, an alternative method of FHR monitoring, performed directly by mothers in their home context, has been recently proposed. We present a case report utilizing this approach and review the current evidence about this condition.

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Maternal autoantibodies to Ro/SSA are often linked to congenital heart block and rarely associated with structural defects. We describe the case of a fetus with anti-Ro-mediated second-degree block at 19 weeks, which progressed to a complete block, fibroelastosis, atrioventricular valve insufficiency, and semilunar valve stenosis/insufficiency at 20, 22, 24, and 26 weeks, respectively, although the fetus received transplacental anti-arrhythmic drugs. The 2150-g fetus was vaginally delivered at 35 weeks. An external pacemaker was inserted immediately after birth and replaced with permanent pacemaker at the age of 3 months. The newborn has had a good outcome with well-controlled heart rate.

Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins.
The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35. While the first twin\'s physical examination proved her to be normal with a pulse rate consistent with her age, the second twin had a pulse rate of approximately 40 beats/minute.The patient was confirmed to have congenital complete atrioventricular block.
Despite this case appears to be an isolated one, a discordant complete atrioventricular block regression without any autoimmune evidence should be included in the differential diagnosis of bradycardia in infants.