Epicardial pacing lead implantation for congenital complete atrioventricular block immediately after birth: a case report.

BACKGROUND: The incidence of congenital complete atrioventricular block is estimated to be 1 per 20,000 deliveries. In the fetal period, the fetal mortality rate is high, but the treatment strategy has not yet been established. In severe cases, early postnatal pacing therapy is necessary. CASE PRESENTATION: A 0-day-old Japanese baby girl was diagnosed with fetal congenital complete atrioventricular block during a prenatal physical examination. A joint conference was held preoperatively among multidisciplinary departments, and a cesarean section was performed at 37 weeks pregnancy, immediately followed by scheduled internal ventricular pacing lead implantation in an adjacent room. Percutaneous pacing was ineffective. The epicardial pacing lead was sutured at 17.5 minutes after birth, and perioperative management was successful with a heart rate and pulse rate of 150 beats per minute. CONCLUSION: The infant with a congenital complete atrioventricular block was rescued by an uneventful epicardial lead implantation.

Reversible complete atrioventricular block in a neonate with maternal anti-Sjögren's syndrome-associated antibody: atypical phenotype of autoimmune congenital heart block.

The fetus of anti-Sjögren's syndrome-associated antibody-positive mother developed complete atrioventricular block at 39 weeks of gestation and required urgent ventricular pacing after birth. Unexpectedly, the patient recovered from the atrioventricular block within a few days. Fraction analysis of maternal anti-Sjögren's syndrome-associated antibody revealed positivity for isolated anti-Ro/SSA 60 kDa antibody, which is abnormal as most patients with complete atrioventricular block present with anti-Ro/SSA 52 kDa positivity, which may indicate a potentially atypical late and reversible manifestation of an autoimmune congenital atrioventricular block in this patient.

His bundle pacing in a young child guided by electroanatomical mapping.

Pediatric patients with complete congenital atrio-ventricular (AV) block are generally exposed to life-long dyssynchronous right ventricular (RV) pacing. His bundle pacing (HBP) is an alternative method of pacing that better restores physiological ventricular activation which could prevent pacing-induced cardiomyopathy. We present a case of a 5-year-old child with complete AV block who underwent successful permanent HBP implantation. Three-dimensional electro-anatomical mapping system was used to facilitate the procedure and reduce the fluoroscopy time. There were no acute procedure-related complications, and electrical parameters were stable at short-term follow-up.

Stent interventional treatment of superior vena cava syndrome with a pacemaker placement complication in an infant.

Background: Epicardial pacemaker is the preferred method for newborns and children under 10 kg of weight. However, its placement presents a high rate of premature failure. Case report: The case of an infant requiring placement of pacemaker by congenital atrioventricular block with prenatal diagnosis is described. The patient showed critical stenosis of the superior vena cava as a complication. Interventional resolution using a stent at the site of stenosis corrected the superior vena cava syndrome effectively. Conclusions: This case represents a clinically viable alternative for the treatment of superior vena cava syndrome, which can be used after other conventional therapies have failed.

Introducción: El marcapasos epicárdico es el método preferido en recién nacidos y niños que pesan menos de 10 kg. Sin embargo, su colocación cuenta con una alta incidencia de fracaso prematuro. Caso clínico: Se presenta el caso de un lactante que requirió la colocación de un marcapasos por bloqueo atrioventricular congénito diagnosticado prenatalmente. El paciente presentaba estenosis crítica de la vena cava superior como complicación secundaria a los cables del marcapasos. La resolución intervencionista mediante un stent en el sitio de estenosis corrigió el síndrome de vena cava superior de forma efectiva. Conclusiones: Este caso representa una alternativa clínicamente viable en el tratamiento del síndrome de vena cava superior, que puede emplearse después de otras terapias convencionales que hayan fallado.

Follow-up of isolated congenital complete atrioventricular block with longitudinal measurements of serum NT-proBNP and cardiothoracic ratio.

There are a few children with isolated congenital complete atrioventricular block (ICCAVB) who do not require a pacemaker. We report a female infant born at 36 weeks by emergency cesarean section because of fetal heart rate abnormalities who was diagnosed as having ICCAVB. In accordance with the echocardiographic findings, we simultaneously measured the cardiothoracic ratio (CTR) by chest radiography and serum N-terminal pro-BNP (NT-proBNP) and have continued to follow her as an outpatient for about 8 years. CTR and NT-proBNP showed strong positive correlation (r=0.894, p<0.05). In such few children with ICCAVB as this patient, CTR measurement during their follow-up would be very useful to easily understand their cardiac load status.

Tratamiento intervencionista con stent para el síndrome de vena cava superior con complicación por el uso de marcapasos en un lactante / Stent interventional treatment of superior vena cava syndrome with a pacemaker placement complication in an infant

Resumen Introducción: El marcapasos epicárdico es el método preferido en recién nacidos y niños que pesan menos de 10 kg. Sin embargo, su colocación cuenta con una alta incidencia de fracaso prematuro. Caso clínico: Se presenta el caso de un lactante que requirió la colocación de un marcapasos por bloqueo atrioventricular congénito diagnosticado prenatalmente. El paciente presentaba estenosis crítica de la vena cava superior como complicación secundaria a los cables del marcapasos. La resolución intervencionista mediante un stent en el sitio de estenosis corrigió el síndrome de vena cava superior de forma efectiva. Conclusiones: Este caso representa una alternativa clínicamente viable en el tratamiento del síndrome de vena cava superior, que puede emplearse después de otras terapias convencionales que hayan fallado.

Abstract Background: Epicardial pacemaker is the preferred method for newborns and children under 10 kg of weight. However, its placement presents a high rate of premature failure. Case report: The case of an infant requiring placement of pacemaker by congenital atrioventricular block with prenatal diagnosis is described. The patient showed critical stenosis of the superior vena cava as a complication. Interventional resolution using a stent at the site of stenosis corrected the superior vena cava syndrome effectively. Conclusions: This case represents a clinically viable alternative for the treatment of superior vena cava syndrome, which can be used after other conventional therapies have failed.

Discordant immune complete heart block and growth restriction in dichorionic twin pregnancy with permanent pacemaker implantation of an 1140 g neonate.

Fetal atrioventricular block is a rare pathology, mostly due to placental transmission of maternal SSA/Ro and SSB/La antibodies, and can lead to severe fetal or neonatal outcomes. We report a case of dichorionic, diamniotic twin pregnancy, with maternal SSA/Ro antibodies. Isolated complete atrioventricular block was diagnosed at 23 weeks in one fetus (Twin A), while the second fetus (Twin B) remained in normal sinus rhythm. Severe asymmetric intrauterine growth restriction occurred in Twin A. Delivery was by caesarean section at 32 + 2 weeks. Neonatal permanent pacemaker was inserted on the first day after birth in 1140 g neonate. Discordant heart block in twin pregnancy has already been reported in a few dichorionic pregnancies, but the pathway of discordant disease expression remains unclear. Extraction decision is a dilemma between cardiac failure prevention and prematurity associated twin morbidity. This case shows a successful pacing in a very low birth weight neonate.

[Pacemaker Dislocation into the Peritoneal Cavity].

A 16-day-old neonate with congenital complete atrioventricular block underwent epicardial pacemaker implantation under the rectus. Four months later, abodominal X-ray imaging revealed dislocation of the generator from the abdomen to the pelvis. The infant was diagnosed with intraperitoneal pacemaker dislocation. However, there were no abdominal manifestations or complications associated with the bowel, urinary tract, and vascular system. Surgical refixation was performed in a hybrid room. Fluoroscopy helped avoid bowel injury when removing the generator from the peritoneal cavity. The pacing lead, which was adherent and entangled with the omentum, was released under direct vision. The generator was placed in a new pocket created in the subcutaneous layer of the anterior fascia of the rectus.

A "long-standing" malpositioned pacing lead. Long-term follow-up after extraction.

Transvenous pacemaker (PM) catheters can be unintentionally placed in the left ventricle (LV) during the implantation procedure. An 8-year-old girl was discovered with a malpositioned pm wire, seven years after the implant. Trans-thoracic echocardiogram revealed the lead traversing the inter-atrial septum, crossing the mitral valve and embedded in the basal lateral wall of the LV. This is a report of a 14-year long follow-up after the surgical extraction of the malpositioned PM lead.

A Case of Congenital Complete Atrioventricular Block Treated with Transdermal Tulobuterol.

Congenital complete atrioventricular block (CCAVB) is a condition in which the atria and ventricles beat independently of each other. CCAVB cases require permanent pacemaker implantation until adulthood. Nevertheless, consensus regarding postnatal medical therapy for bradycardia has not been reached. Here we report the case of a newborn with CCAVB, whose intractable bradycardia was successfully treated with transdermal tulobuterol. Tulobuterol is a selective ß2-adrenoceptor agonist, widely used safely as bronchodilator in children. It also has positive inotropic and chronotropic effect via ß1-adrenoceptors. We believe the tulobuterol patch can be used as an optional therapy for CCAVB where pacemaker implantation is not available.

Maternal steroid therapy for fetuses with second-degree immune-mediated congenital atrioventricular block: a systematic review and meta-analysis.

INTRODUCTION: The aim of this study was to explore the effect of maternal fluorinated steroid therapy on fetuses affected by second-degree immune-mediated congenital atrioventricular block. MATERIAL AND METHODS: Studies reporting the outcome of fetuses with second-degree immune-mediated congenital atrioventricular block diagnosed on prenatal ultrasound and treated with fluorinated steroids compared with those not treated were included. The primary outcome was the overall progression of congenital atrioventricular block to either continuous or intermittent third-degree congenital atrioventricular block at birth. Meta-analyses of proportions using random effect model and meta-analyses using individual data random-effect logistic regression were used. RESULTS: Five studies (71 fetuses) were included. The progression rate to congenital atrioventricular block at birth in fetuses treated with steroids was 52% (95% confidence interval 23-79) and in fetuses not receiving steroid therapy 73% (95% confidence interval 39-94). The overall rate of regression to either first-degree, intermittent first-/second-degree or sinus rhythm in fetuses treated with steroids was 25% (95% confidence interval 12-41) compared with 23% (95% confidence interval 8-44) in those not treated. Stable (constant) second-degree congenital atrioventricular block at birth was present in 11% (95% confidence interval 2-27) of cases in the treated group and in none of the newborns in the untreated group, whereas complete regression to sinus rhythm occurred in 21% (95% confidence interval 6-42) of fetuses receiving steroids vs. 9% (95% confidence interval 0-41) of those untreated. CONCLUSIONS: There is still limited evidence as to the benefit of administered fluorinated steroids in terms of affecting outcome of fetuses with second-degree immune-mediated congenital atrioventricular block.

An extremely rare clinical entity: congenitally corrected transposition with situs inversus and single coronary artery presented with complete atrioventricular block in a young man.

Congenitally corrected transposition of the great arteries is a rare form of CHD. Situs inversus is a much less common variant of a congenitally corrected transposition of the great arteries. In rare cases, transposition events may be accompanied by various cardiac anomalies. However, situs inversus patients with congenitally corrected transposition, single coronary artery anomaly, and atrioventricular block together have not been reported previously. This combination of abnormalities is presented as a first in the literature.

Factors influencing fetal cardiac conduction in anti-Ro/SSA-positive pregnancies.

Objectives: Congenital heart block (CHB) develops in 1-2% of anti-Ro/SSA-positive pregnancies and has a recurrence rate of 12-20%, which indicates that factors other than maternal autoantibodies are crucial for CHB to occur. Here, we aimed to evaluate the influence of factors previously associated with CHB on the occurrence of milder forms of fetal cardiac conduction disturbances, shown to occur in up to 30% of anti-Ro/SSA-positive pregnancies, and on neonatal outcome in a large cohort of prospectively followed pregnancies. Methods: The association of maternal age, season of the year and history of atrioventricular block (AVB) with the development of fetal Doppler and neonatal ECG conduction disturbances was evaluated in 212 anti-Ro52/SSA-positive singleton pregnancies. Results: Maternal age was significantly higher in AVB II-III pregnancies but was not correlated with fetal AV time intervals in fetuses without signs of AVB II-III. AV time intervals of fetuses surveilled during the winter were significantly longer than those of fetuses surveilled during the summer. Fetal AV time intervals in consecutive pregnancies from the same women were significantly correlated. A history of AVB II-III was associated with significantly longer AV time intervals, and AVB I-III was observed at birth in 38% of babies born after a sibling with abnormal fetal AV conduction. Conclusion: Our study shows that AV time intervals in anti-Ro/SSA antibody-exposed fetuses during the CHB risk period are influenced by the season of the year, and reveals that the recurrence of conduction disturbances in antibody-exposed fetuses is higher than previously reported when milder forms are taken into account.

Implante de marcapasso em paciente com veia cava superior esquerda persistente / Pacemaker implant in patients with persistent left superior vena cava

A persistência da veia cava superior esquerda, embora rara, é a anomalia venosa mais encontrada no tórax, com prevalência de 0,3-0,5% na população geral. Em associação com a cardiopatia congênita, sua prevalência aumenta para 2,8-4,3%. Pacientes com cardiopatia congê- nita que necessitem de tratamento cirúrgico podem evoluir, durante a cirurgia, com bloqueio atrioventricular total e necessitar de implante de marcapasso. Apresentamos o caso de uma paciente com cardiopatia congênita, incluindo veia cava superior esquerda persistente, submetida a várias intervenções cirúrgicas, evoluindo com bloqueio atrioventricular total e necessitando de implante de marcapasso bicameral

The persistence of the left superior vena cava, though rare, is the most common venous anomaly in the chest, with a prevalence of 0.3%-0.5% in the general population. When combined with congenital heart disease, its prevalence increases to 2.8%-4.3%. Patients with congenital heart disease who require surgical treatment might evolve to a condition of complete heart block, and consequently require a pacemaker implant. We will present the case of a patient with congenital heart disease, including persistent left superior vena cava, which has been subjected to several surgical interventions, evolving with total ventricular atrial block and requiring a bicameral pacemaker implant

Spontaneous Remission of Congenital Complete Atrioventricular Block in Anti-Ro/La Antibody-Negative Monozygotic Twins: Case Report.

BACKGROUND: 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. CASE REPORT: 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. CONCLUSION: 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.

Key Role of Pacing Site as Determinant Factor of Exercise Testing Performance in Pediatric Patients with Chronic Ventricular Pacing.

Chronic right ventricular (RV) apical pacing has been associated with deterioration of functional capacity and chronotropic incompetence during exercise testing in children. The effects of alternative pacing site on exercise performance in pediatric population remain unknown. We evaluated the influence of ventricular pacing site on exercise capacity in pediatric patients with complete congenital atrioventricular block requiring permanent pacemaker therapy. Sixty-four children paced from RV apex (n = 26), RV midseptum (n = 15) and left ventricular (LV) apex (n = 23) were studied cross-sectionally. Treadmill exercise stress testing was performed according to modified Bruce protocol. LV apical pacing was associated with greater exercise capacity. In comparison with the other study groups, children with RV apical pacing showed significantly lower VO2peak (37 ± 4.11; p = 0.003), O2 pulse (8.78 ± 1.15; p = 0.006), metabolic equivalents (7 ± 0.15; p = 0.001) and exercise time (6 ± 3.28; p = 0.03). Worse values in terms of maximum heart rate (139 ± 8.83 bpm; p = 0.008) and chronotropic index (0.6 ± 0.08; p = 0.002) were detected in the RV apical pacing group although maximal effort (respiratory exchange ratio) did not differ among groups (p = 0.216). Pacing from RV apex (odds ratio 9.4; confidence interval 2.5-18.32; Wald 4.91; p = 0.0036) and low peak heart rate achieved (odds ratio 3.66; confidence interval 0.19-7.4; Wald 4.083; p = 0.015) predicted significantly decrease in exercise capacity. Duration of pacing, gender, VVIR mode, baseline heart rate and QRS duration had not significant impact on exercise capacity. The site of ventricular pacing has the major impact on exercise capacity in children requiring permanent pacing. Among the sites assessed, LV apex is related to the better exercise performance.

Isolated congenital complete heart block in a five-year-old seronegative girl born to a woman seropositive for human immunodeficiency virus: a case report.

BACKGROUND: Congenital complete heart block is a life-threatening condition which is highly associated with autoimmune and connective tissue disorders. Presence of maternal autoantibodies for associated conditions increases the risk of delivering a child with congenital complete heart block, however, less than a half of all women with such antibodies are symptomatic even after delivery. Mortality rate is highest during the neonatal period (45 %) and about two-thirds of all cases will require permanent pacing at some point in their lives. CASE PRESENTATION: We report a case of isolated complete heart block in a 5-year-old HIV-free girl of African descent born to an HIV-infected woman with no prior history of autoimmune disorders. She was referred to us with chief complaints of recurrent syncopal attacks and effort intolerance since birth. A physical examination was unremarkable except for her being small for her age (body mass index 16.3 kg/m2) and bradycardia. Her vital signs were within acceptable range with the exception of her pulse rate, which ranged between 22 and 34 beats/minute. An echocardiogram revealed a sinus bradycardia, otherwise a structurally normal heart. An electrocardiogram showed atrioventricular dissociation in keeping with third-degree atrioventricular block. The child underwent a permanent epicardial pacemaker insertion and has been symptom-free following pacing. CONCLUSIONS: Despite its infrequency and life-threatening potential, patients with congenital complete heart block have an excellent survival rate with timely diagnosis and intervention. An incidental detection of bradycardia in a fetus during routine obstetrical ultrasound examination should increase the index of suspicion for congenital complete heart block and warrant a screening for associated maternal autoantibodies.