368 results on '"congenital heart block"'
Search Results
2. Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period
- Author
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Virginie Meau-Petit, William Regan, Eric Rosenthal, Hitarth Bhatt, and Hayley Hernstadt
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Pediatrics ,medicine.medical_specialty ,Neonatal intensive care unit ,Gestational Age ,Infant, Premature, Diseases ,Congenital heart block ,Antenatal steroid ,Cohort Studies ,Neonate ,Pregnancy ,Complete heart block ,Risk of mortality ,Humans ,Medicine ,business.industry ,Infant, Newborn ,Infant ,medicine.disease ,Heart Block ,Intraventricular hemorrhage ,Pediatrics, Perinatology and Child Health ,Necrotizing enterocolitis ,Cohort ,Gestation ,Original Article ,Female ,Prematurity ,business ,Infant, Premature ,Cohort study - Abstract
Congenital complete heart block (CCHB) is a very rare condition, with high risk of mortality. Prematurity is associated with immaturity of the cardiovascular system. Morbidity related to CCHB and prematurity has never been described. We describe a tertiary perinatal center experience over a 15-year period on CCHB management and complications in preterm infants. This is a single-center observational cohort study. All neonates admitted to neonatal intensive care unit with a diagnosis of isolated CCHB between January 2006 and January 2021 were identified. All preterm neonates (
- Published
- 2021
3. Pregnancy control in patients with systemic lupus erythematosus/antiphospholipid syndrome. Part 2: Pregnancy follow-up
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Paloma Vela-Casasempere, Gerard Espinosa, Nuria Martínez Sánchez, Jose L. Bartha, Beatriz Marco, Luis Sáez-Comet, Paloma Delgado, Juan Antonio López, Amaia Ugarte, Manel Casellas, Guillermo Ruiz-Irastorza, María Galindo-Izquierdo, Víctor M. Martínez-Taboada, Esther Rodríguez Almaraz, Angel Robles, and Universidad de Cantabria
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Gynecology ,medicine.medical_specialty ,Embarazo ,Lactancia ,Síndrome Antifosfolípido ,business.industry ,Lupus Eritematoso Sistémico ,Puerperium ,Antiphospholipid Syndrome ,medicine.disease ,Systemic Lupus Erythematosus ,Congenital heart block ,Anticoncepción ,Contraception ,Rheumatology ,Pregnancy ,Antiphospholipid syndrome ,Lactation ,Medicine ,In patient ,business ,Puerperio - Abstract
Objective: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology set up a working group for the preparation of three consensus documents. Methods: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. Results: This second document contains the recommendations regarding the management of pregnancy in women with SLE and APS, including complications such as lupus activity, congenital heart block, thrombotic and obstetric manifestations of APS and placental vascular disease. Conclusions: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy. Objetivo: Las sociedades españolas de ginecología y obstetricia, de medicina interna y de reumatología han constituido un grupo de trabajo paritario para la elaboración de 3 documentos de consenso sobre el control del embarazo en mujeres con lupus eritematoso sistémico (LES) y síndrome antifosfolípido (SAF).Métodos: Cada una de las sociedades científicas implicadas propuso 5 representantes en base a su experiencia en el área del control del embarazo en pacientes con enfermedades autoinmunes. Las recomendaciones se elaboraron siguiendo la metodología Delphi. Resultados: En este segundo documento se incluyen las recomendaciones que abordan el manejo del embarazo normal y sus complicaciones en mujeres con LES/SAF. Se presentan las recomendaciones relacionadas con el seguimiento del embarazo, la actividad lúpica, el bloqueo cardíaco congénito, las manifestaciones trombóticas y obstétricas del SAF y los defectos de placentación. Conclusiones: Estas recomendaciones multidisciplinares se consideran herramientas en la toma de decisiones para los clínicos involucrados en la asistencia a pacientes con LES/SAF durante el embarazo.
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- 2021
4. False Asystole Alarms Post-Temporary Pacemaker Placement Due to Pseudo-fusion
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Frank Zimmerman, Hoang H. Nguyen, and Alyssa Vermeulen
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Bradycardia ,medicine.medical_specialty ,business.industry ,ComputerApplications_COMPUTERSINOTHERSYSTEMS ,030204 cardiovascular system & hematology ,medicine.disease ,Congenital heart block ,Cardiac surgery ,03 medical and health sciences ,0302 clinical medicine ,030228 respiratory system ,Telemetry ,Intensive care ,Pediatrics, Perinatology and Child Health ,medicine ,ComputerSystemsOrganization_SPECIAL-PURPOSEANDAPPLICATION-BASEDSYSTEMS ,Pacemaker Placement ,False alarm ,Medical emergency ,medicine.symptom ,Asystole ,Cardiology and Cardiovascular Medicine ,business - Abstract
An infant with congenital heart block and hemodynamically significant bradycardia underwent therapeutic temporary pacing wires placement. Post-operatively, frequent "asystole" alarms were observed on telemetry causing distress to both the family and the nursing staff. Investigation of these alarms showed that pacemaker malfunction led to monitor pseudo-malfunction. The alarms were alleviated with mindful setting of the pacemaker and telemetry monitor parameters. This case highlights the challenges of pacemaker placement and monitoring of very small infants in the intensive care setting. Awareness of these challenges would help in troubleshooting pacemaker and telemetry monitor issues.
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- 2020
5. Long-term physical and neurodevelopmental outcomes after antenatal betamethasone administration for congenital heart block prevention
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Atsuko Murashima, Yuka Sano Wada, Mikako Goto, Yushi Ito, and Ikuko Hama
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Pediatrics ,medicine.medical_specialty ,Betamethasone ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Pregnancy ,parasitic diseases ,Humans ,Medicine ,Anti-SSA/Ro Antibody ,Child ,Glucocorticoids ,030203 arthritis & rheumatology ,business.industry ,Mortality rate ,Body Weight ,food and beverages ,Obstetrics and Gynecology ,Heart Block ,Pediatrics, Perinatology and Child Health ,Female ,business ,030217 neurology & neurosurgery ,medicine.drug - Abstract
Congenital heart block (CHB) is associated with a mortality rate of 20% and requires a pacemaker in 70% of cases. Steroids can reduce morbidity and prevent the onset of CHB but may have adverse effects on growth and neurodevelopment. This study aimed to clarify the long-term effects of antenatal betamethasone administration on growth and neurodevelopment.The subjects were children with a high risk of CHB due to a high level of maternal anti-SSA/Ro antibody or a maternal history of a previous delivery of a offspring with CHB to whom antenatal betamethasone was administered. Data on body weight, height, and blood pressure were collected as physical outcomes. The Wechsler Intelligence Scale for Children (fourth edition) or the Kyoto Scale of Psychological Development and the Pervasive Developmental Disorders Autism Society Japan Rating Scale was administered to assess the neurodevelopmental outcome.Fourteen children were enrolled. The body weight and height were within normal range in all children. All children had normal intelligence, and none had autism.Our study suggested that antenatal betamethasone administration has no negative effects on long-term physical and neurodevelopmental outcomes.
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- 2020
6. Pulmonary hypertension associated with congenital heart block and neonatal lupus syndrome: A series of four cases
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Nathalie Morel, Sophie Malekzadeh-Milani, Patrick Barbet, Damien Bonnet, Martina Evangelista, Marilyne Levy, Nathalie Costedoat-Chalumeau, and Alice Maltret
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Male ,Cardiac Catheterization ,Pediatrics ,medicine.medical_specialty ,Hypertension, Pulmonary ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,medicine ,Humans ,Lupus Erythematosus, Systemic ,030203 arthritis & rheumatology ,Pregnancy ,Systemic lupus erythematosus ,business.industry ,Hemodynamics ,Infant, Newborn ,medicine.disease ,Pulmonary hypertension ,Neonatal lupus syndrome ,Heart Block ,Echocardiography ,Female ,Presentation (obstetrics) ,Tomography, X-Ray Computed ,business ,Immunosuppressive Agents - Abstract
Objective Neonatal lupus syndrome has multisystemic manifestations among which pulmonary involvement has been rarely reported. We describe the clinical presentation, management, and outcome of a series of four neonates who developed reversible pulmonary hypertension associated with auto-immune congenital complete heart block. Method Data from the French registry of neonatal lupus syndrome were retrospectively reviewed. Results Between 2000 and March 2020, 231 children were included in the French registry, four/73 followed in our institution developed pulmonary hypertension. Diagnosis was suspected on transthoracic echocardiography at a median age of 42 days [range 10-58], and confirmed by right heart catheterization in all; 2 of them where paced at time of diagnosis and 2 were not. All had some degree of hypoxemia and respiratory distress. Hypoxemia was always reversible under O2 et NO. Lung CT demonstrated ground glass anomalies in all. One patient had a lung biopsy consistent with pulmonary hypertension secondary to lung disease. Management included immunosuppressive therapy in 3 associated with sildenafil in 2. Pulmonary hypertension resolved in all at a median age of 4 weeks [range 3-6] after treatment initiation and after one year for the one child who did not receive specific treatment. Conclusion Clinical, hemodynamical, imaging and histological findings advocate for pulmonary hypertension associated with respiratory disease as a rare manifestation of neonatal lupus syndrome.
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- 2020
7. A novel technique for extraction of a leadless pacemaker that embolized to the pulmonary artery in a young patient: A case report
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Sanjaya Gupta, John J. Borsa, John Papagiannis, Ken Cho, and Svjetlana Tisma-Dupanovic
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Novel technique ,medicine.medical_specialty ,business.industry ,Extraction (chemistry) ,Large sheath/double snare technique ,Case Report ,Congenital heart block ,Surgery ,Atrioventricular synchrony ,medicine.artery ,Pulmonary artery ,Device Embolization ,Medicine ,Leadless pacemaker ,Device embolization ,Cardiology and Cardiovascular Medicine ,business ,Surgical site infection - Published
- 2020
8. Hydroxychloroquine to Prevent Recurrent Congenital Heart Block in Fetuses of Anti-SSA/Ro-Positive Mothers
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Robert R. Clancy, Jill P. Buyon, Noël Zahr, Mimi Y. Kim, Rebecca E. Cohen, Joshua A. Copel, Mala Masson, Colin K.L. Phoon, Peter M. Izmirly, Kimberly Robins, Amit Saxena, Bettina F. Cuneo, Benjamin J. Wainwright, Nathalie Costedoat-Chalumeau, and Deborah M. Friedman
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Adult ,Male ,medicine.medical_specialty ,hydroxychloroquine ,anti-SSA/Ro antibodies ,Administration, Oral ,030204 cardiovascular system & hematology ,Article ,03 medical and health sciences ,0302 clinical medicine ,prevention ,Pregnancy ,Internal medicine ,Secondary Prevention ,medicine ,Humans ,Prospective Studies ,030212 general & internal medicine ,Enzyme Inhibitors ,Autoantibodies ,Systemic lupus erythematosus ,Dose-Response Relationship, Drug ,congenital heart block ,business.industry ,Infant, Newborn ,Hydroxychloroquine ,Endocardial fibroelastosis ,neonatal lupus ,medicine.disease ,Rash ,Clinical trial ,Fetal Diseases ,Heart Block ,magnetocardiography ,Gestation ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies ,medicine.drug ,Anti-SSA/Ro autoantibodies - Abstract
Background Experimental and clinical evidence support the role of macrophage Toll-like receptor signaling in maternal anti-SSA/Ro–mediated congenital heart block (CHB). Objectives Hydroxychloroquine (HCQ), an orally administered Toll-like receptor antagonist widely used in lupus including during pregnancy, was evaluated for efficacy in reducing the historical 18% recurrence rate of CHB. Methods This multicenter, open-label, single-arm, 2-stage clinical trial was designed using Simon’s optimal approach. Anti-SSA/Ro–positive mothers with a previous pregnancy complicated by CHB were recruited (n = 19 Stage 1; n = 35 Stage 2). Patients received 400 mg daily of HCQ prior to completion of gestational week 10, which was maintained through pregnancy. The primary outcome was 2° or 3° CHB any time during pregnancy, and secondary outcomes included isolated endocardial fibroelastosis, 1° CHB at birth and skin rash. Results By intention-to-treat (ITT) analysis, 4 of 54 evaluable pregnancies resulted in a primary outcome (7.4%; 90% confidence interval: 3.4% to 15.9%). Because 9 mothers took potentially confounding medications (fluorinated glucocorticoids and/or intravenous immunoglobulin) after enrollment but prior to a primary outcome, to evaluate HCQ alone, 9 additional mothers were recruited and followed the identical protocol. In the per-protocol analysis restricted to pregnancies exposed to HCQ alone, 4 of 54 (7.4%) fetuses developed a primary outcome as in the ITT. Secondary outcomes included mild endocardial fibroelastosis (n = 1) and cutaneous neonatal lupus (n = 4). Conclusions These prospective data support that HCQ significantly reduces the recurrence of CHB below the historical rate by >50%, suggesting that this drug should be prescribed for secondary prevention of fetal cardiac disease in anti-SSA/Ro-exposed pregnancies. (Preventive Approach to Congenital Heart Block With Hydroxychloroquine [PATCH]; NCT01379573 )
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- 2020
9. Autoimmune-mediated congenital heart block
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Catherine Trad, Rebecca E. Cohen, Peter M. Izmirly, Rohit Bhan, Amit Saxena, Jill P. Buyon, and Benjamin J. Wainwright
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Adult ,medicine.medical_specialty ,Inflammation ,Disease ,030204 cardiovascular system & hematology ,Autoantigens ,Congenital heart block ,Autoimmune Diseases ,Pathogenesis ,03 medical and health sciences ,Fetal Heart ,0302 clinical medicine ,Pregnancy ,Fibrosis ,Internal medicine ,medicine ,Humans ,Autoantibodies ,030203 arthritis & rheumatology ,Lupus Vulgaris ,business.industry ,Autoantibody ,Obstetrics and Gynecology ,Dilated cardiomyopathy ,Endocardial fibroelastosis ,General Medicine ,medicine.disease ,Pregnancy Complications ,Heart Block ,Cardiology ,Female ,medicine.symptom ,business - Abstract
Autoimmune-mediated congenital heart block (CHB) is a severe manifestation of neonatal lupus in which conduction tissues of the fetal heart are damaged. This occurs due to passive transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies and subsequent inflammation and fibrosis of the atrioventricular (AV) node. Notably, the disease manifests after the fetal heart has structurally developed, ruling out other anatomical abnormalities that could otherwise contribute to the block of conduction. Complete AV block is irreversible and the most common manifestation of CHB, although other cardiac complications such as endocardial fibroelastosis (EFE), dilated cardiomyopathy, and valvular insufficiency have been observed. In this review, we detail the classification, prevalence, pathogenesis, and clinical management recommendations for autoimmune CHB.
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- 2020
10. Management in maternal autoantibody-mediated clinical foetal myocardial disease
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Julia Murlewska, Maciej Słodki, Maria Respondek-Liberska, Iwona Strzelecka, Krzysztof Preis, and Elżbieta Smolewska
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medicine.medical_specialty ,lcsh:Diseases of the circulatory (Cardiovascular) system ,congenital heart block ,business.industry ,Autoantibody ,foetal lupus syndrome ,Congenital heart block ,Foetal echocardiography ,foetal echocardiography ,lcsh:RC666-701 ,Internal medicine ,medicine ,Cardiology ,foetal myocarditis ,Myocardial disease ,business ,foetal cardiomyopathy - Abstract
Our group presents our reflections, based on the current literature, concerning the obstetric and rheumatologic management of the foetus identified with clinical myocardial disease mediated by maternal autoantibodies (MAb). The European League Against Rheumatism (EULAR) have recommended the use of hydroxychloroquine (HCQ) during asymptomatic lupus pregnancies since 2007. Foetal echocardiography is indicated if there is suspected foetal dysrhythmia or myocarditis, especially in the involvement of positive maternal anti-Ro/SSA or anti-La/SSB antibodies weekly from 16 weeks of gestation upwards. The obstetric management should be guided by the degree of cardiac failure on foetal echocardiography. Foetal therapy with steroids, intravenous immunoglobulin (IVIG), and plasmapheresis should be reasonably introduced in the lowest effective doses for the shortest duration of time. The aim of the management should be to reverse incomplete heart block and other MAb-mediated foetal myocardial disease, presumably induced due to ongoing inflammation. In irreversible cases the treatment should be stopped, due to its possible maternal side effects. Delivery of the affected newborn should be performed in a tertiary centre, and pacemaker implantation might be an option for neonates.
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- 2019
11. Facial rash in a newborn
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Sílvia Mota, Carla Sá, Nicole Silva, Filipa Almeida, Ana Paula Vieira, and Almerinda Pereira
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congenital heart block ,facial rash ,sjögren syndrome type a antigen antibodies ,skin lesions ,Medicine ,neonatal lupus ,Pediatrics ,RJ1-570 ,neonatal autoimmune disease - Abstract
A female newborn, born at 38 weeks from an uncomplicated pregnancy in a healthy 36-years-old mother, presented at birth with desquamative erythematous plaques with irregular borders, distributed bilaterally in the orbital, nasal and malar regions. Although there was no history of maternal autoimmune disease, neonatal lupus (NL) was suspected. Maternal and newborn screenings were positive for Sjögren syndrome type A antigen (anti-Ro/SSA) antibodies. No other alterations of NL were found in the newborn. Rheumatologic consultation on the mother showed no other alterations besides the antibodies. The newborn was discharged on day 3 of life without treatment and recommendations to avoid sun exposure. Outpatient follow-up was ensured in neonatology, dermatology and pediatric cardiology. The rash resolved during the first year of life, leaving slight local skin atrophy. NL is a rare transferred autoimmune disease with an incidence estimated as 1:20,000 live births. It occurs due to placental transfer of maternal autoantibodies. The major manifestations are cardiac and cutaneous, but hepatic, hematologic or neurologic findings may also be present. The rash usually affects the face and scalp and may be present at delivery but more often develops later, after exposure to ultraviolet light. It usually resolves within the first year of life without sequelae. NL is the leading cause of congenital heart block, but if it is not present at birth it rarely develops. Some mothers do not have a known autoimmune disease at the time of birth but may develop it later in life. Despite NL being a passively acquired autoimmune disease, the child is at increased risk of rheumatologic disease in childhood or adolescence.
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- 2021
12. Fetal Congenital Heart Block Associated With Maternal Primary Systemic Lupus Erythematosus and Sjogren’s Syndrome
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Syed R Ali, Priyanka Anvekar, Petras Lohana, Sameera Khan, and Mohammed Sheeraz Alam
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medicine.medical_specialty ,Pregnancy ,Fetus ,sjogren's disease ,congenital heart block ,Obstetrics ,Heart block ,business.industry ,medicine.medical_treatment ,General Engineering ,Fetal Bradycardia ,medicine.disease ,Pediatrics ,Cardiac pacemaker ,Congenital heart block ,Polycystic ovarian disease ,internal medicine and rheumatology ,Rheumatology ,medicine ,Internal Medicine ,Gestation ,systemic lupus erythromatosus ,business ,skin and connective tissue diseases ,cardiac pacemaker - Abstract
Congenital heart block is a grave condition reported in 0.5% of 100 live births. Systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS) are chronic autoimmune and inflammatory condition, which affects multiple systems. The association of SLE and SS with pregnancy has been seen in the past. Usually, it shows anti-Ro/SSA and anti-Ro/SSB auto-antibodies in maternal serum, which is proportional to fetal Outcome. In this report, we present a case of a 29-year-old female gravida 4, para one and aborta 3, with a history of polycystic ovarian disease and multiple abortions. At 20 weeks of gestation, her antenatal examination revealed fetal bradycardia and heart block, which further led to SLE and SS diagnosis in her. She was treated with steroids to prevent further fetal complications. The patient delivered a healthy neonate at 38 weeks of gestation. The neonate eventually received a cardiac pacemaker and is now on regular follow-up.
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- 2021
13. Congenital Heart Block in Subsequent Pregnancies of SSA/Ro-Positive Mothers
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Annette Wacker-Gussmann and Janette F. Strasburger
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Pediatrics ,medicine.medical_specialty ,2019-20 coronavirus outbreak ,Coronavirus disease 2019 (COVID-19) ,business.industry ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,MEDLINE ,Hydroxychloroquine ,Congenital heart block ,medicine ,Cardiology and Cardiovascular Medicine ,business ,Magnetocardiography ,medicine.drug ,Anti-SSA/Ro autoantibodies - Published
- 2020
14. Wide heterogeneity in treatment protocols and inappropiate use of prednisolone for anti-Ro/La associated-congenital heart block: a systematic review of 492 cases
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Benazir Hymabaccus, Yusuf Ziya Şener, Serdar Ceylan, Emre Bilgin, Omer Karadag, Fatih Gürler, Umut Kalyoncu, B. Armagan, Levent Kılıç, Abdulsamet Erden, and Alper Sari
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medicine.medical_specialty ,business.industry ,Internal medicine ,Prednisolone ,medicine ,business ,Congenital heart block ,Rheumatology ,medicine.drug ,Anti-SSA/Ro autoantibodies - Published
- 2019
15. First clinical experience with the Kora pacemaker system in congenital complete heart block in newborn infants
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Gernot Grangl, Ante Burmas, Stefan Kurath-Koller, Sabrina Schweintzger, Martin Koestenberger, and Andreas Gamillscheg
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Male ,Cardiac output ,Pacemaker, Artificial ,medicine.medical_specialty ,Time Factors ,Congenital heart block ,Pacemaker system ,Sampling Studies ,Electrocardiography ,03 medical and health sciences ,Child Development ,Rare Diseases ,0302 clinical medicine ,Heart Rate ,030225 pediatrics ,Internal medicine ,Heart rate ,medicine ,Humans ,030212 general & internal medicine ,Adverse effect ,Children ,business.industry ,Infant, Newborn ,lcsh:RJ1-570 ,lcsh:Pediatrics ,Equipment Design ,Pacemaker ,Heart Block ,Treatment Outcome ,Congenital complete heart block ,Tolerability ,Echocardiography ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Cardiology ,Female ,business ,Respiratory minute volume ,Follow-Up Studies ,Research Article ,Skin traction - Abstract
Background To report first clinical experience on three cases of congenital complete heart block and the use of a pacemaker system with a maximum lower rate interval of 95 beats per minute. Methods We retrospectively analyzed three patients treated with a pacemaker system with a maximum lower rate interval of 95 beats per minute suffering from congenital complete heart block. We report a follow up period of 2.9 years, focusing on the patients’ growth, development, and adverse events, as well as pacemaker function. Results In all three patients pacemaker function was impeccable, including minute ventilation sensor rate adaption. All patients showed limited growths as expected, adequate development, good feeding tolerability and circadiane heart rate adaption. One patient experienced skin traction and revision. All patients showed high aortic velocity time integral values after birth. Conclusion The use of a pacemaker system with a maximum lower rate interval of 95 beats per minute in infants suffering from congenital complete heart block and showing high aortic VTI values seems to be feasible and to result in limited growths but adequate development. Electronic supplementary material The online version of this article (10.1186/s12887-019-1494-7) contains supplementary material, which is available to authorized users.
- Published
- 2019
16. Posterior Spinal Fusion in a Scoliotic Patient With Congenital Heart Block Treated With Pacemaker
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Chris Yin Wei Chan, Weng Hong Chung, Mun Keong Kwan, Jaseemuddeen Abu Bakar, Kok Han Chee, Mohd Shahnaz Hasan, Chee Kidd Chiu, and Tat Seng Wong
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Male ,Pacemaker, Artificial ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Scoliosis ,Thoracic Vertebrae ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Lead Dislodgement ,Orthopedics and Sports Medicine ,Thoracotomy ,030222 orthopedics ,business.industry ,medicine.disease ,Surgery ,Heart Block ,Spinal Fusion ,medicine.anatomical_structure ,Congenital complete heart block ,Spinal fusion ,Thoracic vertebrae ,Neurology (clinical) ,Permanent pacemaker ,business ,030217 neurology & neurosurgery - Abstract
Study design Case report. Objective To describe the technical difficulties on performing posterior spinal fusion (PSF) on a pacemaker-dependent patient with complete congenital heart block and right thoracic scoliosis. Summary of background data Congenital complete heart block requires pacemaker implantation at birth through thoracotomy, which can result in scoliosis. Corrective surgery in this patient was challenging. Height gain after corrective surgery may potentially cause lead dislodgement. The usage of monopolar electrocautery may interfere with the function of the implanted cardiac device. Methods A 17-year-old boy was referred to our institution for the treatment of right thoracic scoliosis of 70°. He had underlying complete congenital heart block secondary to maternal systemic lupus erythematosus. Pacemaker was implanted through thoracotomy since birth and later changed for four times. PSF was performed by two attending surgeons with a temporary pacing inserted before the surgery. The monopolar electrocautery device was used throughout the surgery. Results The PSF was successfully performed without any technical issues and complications. Postoperatively, his permanent pacemaker was functioning normally. Three days later, he was recovering well and was discharged home from hospital. Conclusion This case indicates that PSF can be performed successfully with thoughtful anticipation of technical difficulties on a pacemaker-dependent patient with underlying congenital heart block. Level of evidence 5.
- Published
- 2019
17. Neonatal lupus
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T. P. Makarova, Kh. M. Vakhitov, N. N. Firsova, Yu. S. Melnikova, and T. A. Novitskaya
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integumentary system ,children ,congenital heart block ,Pediatrics, Perinatology and Child Health ,skin lesion ,neonatal lupus ,Pediatrics ,RJ1-570 - Abstract
Neonatal lupus is a complex of symptoms diagnosed in newborns whose mothers suffer from autoimmune rheumatic diseases; it is manifested by two main signs: diseases of the skin and heart. A newborn may have classic ring-shaped erythema, telangiectasia, skin atrophy, follicular plugs and scales. This skin lesion is usually combined with a partial or complete heart block due to fibrosis of its pathways. There is a Clinical case of a child with neonatal lupus from own practice.Conflict of interest: The authors of this article confirmed the lack of conflict of interest and financial support, which should be reported.
- Published
- 2018
18. Case series of late lead dislodgement of Medtronic SelectSecure 3830 pacing leads in growing paediatric patients
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Sarah Gallagher, Kevin Walsh, and Li Yen Ng
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Bradycardia ,medicine.medical_specialty ,Atrium (architecture) ,business.industry ,Late lead dislodgement ,Congenital heart block ,Surgery ,law.invention ,Transvenous pacing ,Congenital complete heart block ,law ,Case report ,Paediatric pacing ,Pacemaker lead failure ,medicine ,Lead Dislodgement ,Artificial cardiac pacemaker ,Case Series ,AcademicSubjects/MED00200 ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Lead (electronics) ,Paediatric patients - Abstract
Background 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.Case summary 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.Discussion 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.
- Published
- 2021
19. Ventricular pacing and myocardial function in patients with congenital heart block: Is it time to consider de novo biventricular pacing?
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Seshadri Balaji and Anjan S. Batra
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medicine.medical_specialty ,business.industry ,Heart Ventricles ,medicine.medical_treatment ,Cardiac resynchronization therapy ,Ventricular pacing ,Myocardial function ,Congenital heart block ,Cardiac Resynchronization Therapy ,Heart Block ,Physiology (medical) ,Internal medicine ,Cardiology ,Humans ,Medicine ,In patient ,Cardiology and Cardiovascular Medicine ,business - Published
- 2021
20. B-PO02-159 IMPACT OF PACING ON LEFT VENTRICULAR FUNCTION IN PATIENTS WITH CONGENITAL HEART BLOCK
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Aditya Bhonsale, Sandeep Jain, Floyd Thoma, Samir Saba, Suresh Mulukutla, Gautam Rangavajla, Krishna Kancharla, and Mark Estes
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medicine.medical_specialty ,Ventricular function ,business.industry ,Physiology (medical) ,Internal medicine ,Cardiology ,Medicine ,In patient ,Cardiology and Cardiovascular Medicine ,business ,Congenital heart block - Published
- 2021
21. Successful 2-Stage Pacemaker Implantation for Atrioventricular Block in a Low-Birth-Weight Infant with Congenital Heart Disease: Case Report
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Matthew A. Hazle, Leigh Kline, and Yoshio Ootaki
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Heart Defects, Congenital ,medicine.medical_specialty ,Pacemaker, Artificial ,Heart disease ,Pacemaker system ,Congenital heart block ,Pacemaker implantation ,Electrocardiography ,Heart Rate ,Internal medicine ,medicine ,Humans ,Stage (cooking) ,Atrioventricular Block ,business.industry ,Infant, Newborn ,General Medicine ,Infant, Low Birth Weight ,medicine.disease ,Low birth weight ,medicine.anatomical_structure ,Ventricle ,cardiovascular system ,Cardiology ,Surgery ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Atrioventricular block - Abstract
Congenital heart block is a potentially life-threatening condition with high morbidity and mortality, especially in the presence of congenital heart disease. We present the case of a low-body-weight premature infant with complex single ventricle congenital heart disease and high-grade atrioventricular block. A 2-staged pacing approach provided atrio-ventricular synchrony and allowed her to grow until a permanent dual-chamber pacemaker system could be implanted.
- Published
- 2020
22. Mortality in congenital heart block – Authors' reply
- Author
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Nathalie Morel, N. Costedoat-Chalumeau, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Conservatoire National des Arts et Métiers [CNAM] (CNAM), HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Serva, Camille, and Conservatoire National des Arts et Métiers [CNAM] (CNAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)
- Subjects
030203 arthritis & rheumatology ,medicine.medical_specialty ,[SDV.MHEP.RSOA] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system ,business.industry ,Immunology ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,[SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system ,Internal medicine ,medicine ,Cardiology ,Immunology and Allergy ,business ,ComputingMilieux_MISCELLANEOUS - Abstract
International audience
- Published
- 2020
23. Successful perinatal management and pacemaker stimulation during the first hour of life in a 1.6 kg newborn with autoimmune congenital complete heart block diagnosed prenatally
- Author
-
Anna Wójtowicz, Tomasz Mroczek, Hubert Huras, Janusz Skalski, and Agata Włoch
- Subjects
Adult ,medicine.medical_specialty ,Pacemaker, Artificial ,business.industry ,Autoantibody ,Infant, Newborn ,Parturition ,Obstetrics and Gynecology ,Stimulation ,Congenital heart block ,Heart Block ,Congenital complete heart block ,Echocardiography ,Pregnancy ,Internal medicine ,Prenatal Diagnosis ,medicine ,Cardiology ,Humans ,Female ,business - Published
- 2020
24. Comorbidity and long-term outcome in patients with congenital heart block and their siblings exposed to Ro/SSA autoantibodies in utero
- Author
-
Reyhan Bilici Salman, Şeyma Yıldız, Neslihan Kayahan, Hazan Karadeniz, Aslıhan Avanoğlu Güler, and Hasan Satış
- Subjects
030203 arthritis & rheumatology ,0301 basic medicine ,Pediatrics ,medicine.medical_specialty ,education.field_of_study ,business.industry ,Immunology ,Population ,Autoantibody ,medicine.disease ,Comorbidity ,General Biochemistry, Genetics and Molecular Biology ,Congenital heart block ,03 medical and health sciences ,030104 developmental biology ,0302 clinical medicine ,Rheumatology ,In utero ,Immunology and Allergy ,Medicine ,In patient ,business ,education - Abstract
We read with great interest the article ‘Comorbidity and Long-Term Outcome in Patients with Congenital Heart Block and Their Siblings Exposed to Ro/SSA Autoantibodies In Utero’ by Mofors et al .1 A large and long-term follow-up population consisting of patients with congenital heart block (CHB) as a result of in utero Ro/SSA autoantibody exposure were studied. It contributes valuable information to our knowledge; however, …
- Published
- 2020
25. A Broader Perspective on Anti-Ro Antibodies and Their Fetal Consequences-A Case Report and Literature Review
- Author
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Ana-Maria Zagrean, Anca Marina Ciobanu, Mihaela Roxana Popescu, Andreea Dudu, Ciprian Jurcut, and Anca Maria Panaitescu
- Subjects
030203 arthritis & rheumatology ,Risk awareness ,autoimmune channelopathy ,Fetus ,medicine.medical_specialty ,lcsh:R5-920 ,business.industry ,Mortality rate ,Clinical Biochemistry ,Perspective (graphical) ,fetal complete heart block ,apoptosis ,Review ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,anti Ro/SSA antibodies ,Medicine ,Clinical case ,business ,Intensive care medicine ,lcsh:Medicine (General) ,long-term consequences ,Anti-SSA/Ro autoantibodies - Abstract
The presence of maternal Anti-Ro/Anti-La antibodies causes a passively acquired autoimmunity that may be associated with serious fetal complications. The classic example is the autoimmune-mediated congenital heart block (CHB) which is due in most cases to the transplacental passage of Anti-Ro/Anti-La antibodies. The exact mechanisms through which these pathologic events arise are linked to disturbances in calcium channels function, impairment of calcium homeostasis and ultimately apoptosis, inflammation and fibrosis. CHB still represents a challenging diagnosis and a source of debate regarding the best management. As the third-degree block is usually irreversible, the best strategy is risk awareness and prevention. Although CHB is a rare occurrence, it affects one in 20,000 live births, with a high overall mortality rate (up to 20%, with 70% of in utero deaths). There is also concern over the lifelong consequences, as most babies need a pacemaker. This review aims to offer, apart from the data needed for a better understanding of the issue at hand, a broader perspective of the specialists directly involved in managing this pathology: the rheumatologist, the maternal–fetal specialist and the cardiologist. To better illustrate the theoretical facts presented, we also include a representative clinical case.
- Published
- 2020
26. Surveillance of congenital heart block in highly specialised care – Authors' reply
- Author
-
Nathalie Morel, N. Costedoat-Chalumeau, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), and Conservatoire National des Arts et Métiers [CNAM] (CNAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)
- Subjects
030203 arthritis & rheumatology ,medicine.medical_specialty ,business.industry ,Immunology ,030204 cardiovascular system & hematology ,Congenital heart block ,3. Good health ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,[SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system ,medicine ,Immunology and Allergy ,Intensive care medicine ,business ,ComputingMilieux_MISCELLANEOUS - Abstract
International audience
- Published
- 2020
27. Report of Congenital heart block and Atrial Septal Aneurysm in a case of Neonatal Lupus
- Author
-
Mohinish S
- Subjects
medicine.medical_specialty ,Atrial septal aneurysm ,business.industry ,Neonatal lupus ,Internal medicine ,Cardiology ,Medicine ,General Medicine ,business ,Congenital heart block - Published
- 2020
28. A multicentre study of 244 pregnancies in undifferentiated connective tissue disease: maternal/fetal outcomes and disease evolution
- Author
-
Cristiano M. de Freitas, Silvia Gallo Cassarino, Marcello Govoni, Elena Rubini, Silvia Grazietta Foddai, Massimo Radin, Irene Cecchi, Chiara Benedetto, Luca Marozio, Laura Andreoli, Francesca Crisafulli, Karen Schreiber, Dario Roccatello, Beatrice Bacco, Franco Franceschini, Melissa Padovan, Savino Sciascia, Danieli Andrade, Tiziana Bertero, and Alessandra Bortoluzzi
- Subjects
Adult ,Gestational hypertension ,medicine.medical_specialty ,undifferentiated connective tissue disease ,autoantibodies ,Intrauterine growth restriction ,autoimmune disease ,anti-nuclear antibodies ,congenital heart block ,neonatal lupus ,pregnancy ,pregnancy complications ,NO ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,medicine ,Humans ,Pharmacology (medical) ,030212 general & internal medicine ,Undifferentiated Connective Tissue Diseases ,Retrospective Studies ,030203 arthritis & rheumatology ,Pregnancy ,Fetal Growth Retardation ,Obstetrics ,business.industry ,Pregnancy Outcome ,LS6_12 ,Retrospective cohort study ,Stillbirth ,medicine.disease ,Abortion, Spontaneous ,Gestational diabetes ,Antibodies, Antinuclear ,Cohort ,Antibodies, Antiphospholipid ,Gestation ,Female ,Live birth ,business ,Live Birth - Abstract
Objectives To investigate fetal/perinatal and maternal outcomes from a large multicentre cohort of women diagnosed with UCTD. Methods This multicentre retrospective cohort study describes the outcomes of 224 pregnancies in 133 consecutive women with a diagnosis of UCTD, positive for ANA and aged Results Of the 224 pregnancies analysed, 177 (79%) resulted in live births, 45 (20.1%) in miscarriages (defined as pregnancy loss before 12 weeks’ gestation), 2 (0.9%) in stillbirths (pregnancy loss after 20 weeks’ gestation) and 6 (2.7%) cases showed intrauterine growth restriction. Miscarriages and stillbirths were strongly associated with the presence of aPL and ENA antibodies (P Conclusion In our multicentre cohort, women with UCTD had a live birth rate of 79%. Women with UCTD should be referred to specialist follow-up when planning a pregnancy. ENA profiling and aPL testing should be mandatory in this setting, and further therapeutic approaches and management should be planned accordingly.
- Published
- 2020
29. Congenital heart block in neonatal lupus erythematosus
- Author
-
Marietta Branson and Richard L. Pullen
- Subjects
030203 arthritis & rheumatology ,medicine.medical_specialty ,business.industry ,030204 cardiovascular system & hematology ,Assessment and Diagnosis ,LPN and LVN ,medicine.disease ,Congenital heart block ,Nurse Assisting ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Cardiology ,Medicine ,Fundamentals and skills ,Neonatal lupus erythematosus ,business - Published
- 2018
30. Bradycardia in a Neonate
- Author
-
Farhad Salehzadeh and Ross E. Petty
- Subjects
Bradycardia ,Pediatrics ,medicine.medical_specialty ,business.industry ,medicine.disease ,Asymptomatic ,Congenital heart block ,medicine ,medicine.symptom ,Neonatal lupus erythematosus ,Permanent pacemaker ,skin and connective tissue diseases ,Complication ,business - Abstract
The most important complication of neonatal lupus erythematosus (NLE) is congenital heart block (CHB) Infants with NLE are born to mothers who have antibodies against Ro (SSA) and/or La (SSB) antigens, although the mother may be completely asymptomatic Most children with CHB will eventually require a permanent pacemaker during childhood The risk of having a second baby with congenital heart block is within the range of 15–20%
- Published
- 2019
31. Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center
- Author
-
Zhen Bing, Zipu Li, Degong Shi, Sujuan Hu, and Benzhen Wang
- Subjects
medicine.medical_specialty ,Cardiomyopathy ,Torsades de pointes ,030204 cardiovascular system & hematology ,QT interval ,Pediatrics ,03 medical and health sciences ,0302 clinical medicine ,Prednisone ,030225 pediatrics ,Internal medicine ,Junctional ectopic tachycardia ,medicine ,cardiovascular diseases ,Original Research ,congenital heart block ,Left bundle branch block ,business.industry ,steroid ,lcsh:RJ1-570 ,Endocardial fibroelastosis ,Dilated cardiomyopathy ,lcsh:Pediatrics ,neonatal lupus ,medicine.disease ,Pediatrics, Perinatology and Child Health ,Cardiology ,cardiovascular system ,business ,cardiomyopathy ,autoantibody ,medicine.drug - Abstract
Objective: To describe the clinical characteristics of maternal autoantibody-mediated arrhythmia and/or cardiomyopathy, and to explore the therapeutic role of glucocorticoids in these diseases. Methods: This was a retrospective observational study of 2 fetuses and 14 children who presented with autoantibody-mediated arrhythmia and/or cardiomyopathy in our hospital from September 2010 to December 2018. Results: In total, 16 patients were identified, including 2 fetuses, and 14 children. One mother suffered from Sjogren's syndrome, two suffered from systemic lupus erythematosus (SLE), and the remaining 13 were asymptomatic carriers of autoantibodies. Two fetuses were diagnosed with complete congenital heart block (CHB) and had mean heart rates of 45 and 50 bpm. In the 14 surviving children, third-degree CHB was detected in 4 children, second- to third-degree CHB in 4, corrected QT interval (QTc) prolongation in 1, atrioventricular dissociation, and junctional ectopic tachycardia in 1, complete left bundle branch block (CLBBB) with dilated cardiomyopathy (DCM) in 3, and endocardial fibroelastosis (EFE) in 1. All of the 14 surviving babies received intravenous immunoglobulin and glucocorticoids. None of the children received pacemaker implantation. During the follow-up, one 3-month-old girl who had complete CHB, DCM, and Torsades de pointes almost recovered after the administration of prednisone for ~8 years. Three cases with complete CHB had no improvement after 3-5 years of follow-up. One case with EFE and three cases with CLBBB and DCM were in stable condition now. Children with QTc prolongation and junctional ectopic tachycardia returned to a regular rhythm. Conclusions: Autoantibody-mediated arrhythmias and/or cardiomyopathy are severe complications related to maternal autoantibodies, and the administration of steroid may be beneficial in reversing complete CHB.
- Published
- 2019
32. VP23.17: Perinatal management of congenital heart block
- Author
-
A. Mohd Jamil, Z. Yahaya, N. Abd Malik, V.R.L. Velayudham, M. Itam, and P. Yubbu
- Subjects
medicine.medical_specialty ,Reproductive Medicine ,Radiological and Ultrasound Technology ,business.industry ,Internal medicine ,medicine ,Cardiology ,Obstetrics and Gynecology ,Radiology, Nuclear Medicine and imaging ,General Medicine ,business ,Congenital heart block - Published
- 2021
33. Should we treat congenital heart block with fluorinated corticosteroids?
- Author
-
Véronique Ramoni, Angela Tincani, N. Costedoat-Chalumeau, Micaela Fredi, Antonio Brucato, Nathalie Morel, and Silvia Breda
- Subjects
medicine.medical_specialty ,Immunology ,Cardiomyopathy ,Context (language use) ,030204 cardiovascular system & hematology ,Congenital heart block ,Neonatal lupus ,03 medical and health sciences ,0302 clinical medicine ,Adrenal Cortex Hormones ,medicine ,Humans ,Immunology and Allergy ,Intensive care medicine ,Fluorinated steroids ,Therapy ,030203 arthritis & rheumatology ,Window of opportunity ,business.industry ,Infant, Newborn ,Retrospective cohort study ,medicine.disease ,Heart Block ,Gestation ,Observational study ,business ,Steroids, Fluorinated - Abstract
Cardiac involvement is the most severe manifestation of neonatal lupus. It is usually diagnosed as an advanced (second or third degree) atrio-ventricular (AV) congenital heart block (CHB) starting around 20 weeks of gestation. At this gestational time, a window of opportunity might exist, and fluorinated corticosteroids (FS) have been attempted as a therapeutic option. Unfortunately the quality of the studies is methodologically low, due to the retrospective design and to the rarity of autoimmune CHB. Some case reports and small case series basically suggested a possible utility of FS, mainly in the context of incomplete CHB, while larger observational retrospective studies did not find any efficacy of FS either on mortality, on the rate of pacemaker implantation, to reduce the degree of incomplete CHB or to reduce the development of cardiomyopathy. Of note the distinction between complete and incomplete AV block may be very difficult and time consuming in utero. Overall FS should not be recommended in CHB, but might be considered for a short time for a recent incomplete CHB.
- Published
- 2017
34. Prenatal sonographic monitoring of progressive cardiac damages caused by anti-Ro antibodies: A case report
- Author
-
Fuanglada Tongprasert, Thitikarn Lerthiranwong, Rekwan Sittiwangkul, and Theera Tongsong
- Subjects
030203 arthritis & rheumatology ,Atrioventricular valve ,medicine.medical_specialty ,Fetus ,030219 obstetrics & reproductive medicine ,business.industry ,Transplacental ,medicine.disease ,Congenital heart block ,Surgery ,03 medical and health sciences ,Stenosis ,0302 clinical medicine ,Internal medicine ,Heart rate ,medicine ,Cardiology ,Radiology, Nuclear Medicine and imaging ,business ,Dexamethasone ,medicine.drug ,Anti-SSA/Ro autoantibodies - Abstract
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.
- Published
- 2017
35. Could Timing Be Everything for Antibody-Mediated Congenital Heart Block?
- Author
-
Shreya S Sheth and Carolyn A. Altman
- Subjects
medicine.medical_specialty ,biology ,business.industry ,030204 cardiovascular system & hematology ,Antibodies ,Congenital heart block ,Fetal Diseases ,03 medical and health sciences ,Fetal Heart ,Heart Block ,0302 clinical medicine ,Pregnancy ,Internal medicine ,medicine ,Cardiology ,biology.protein ,Humans ,Female ,030212 general & internal medicine ,Antibody ,Cardiology and Cardiovascular Medicine ,business - Published
- 2018
36. Left Epicardial vs. Best-site Right Ventricular Transvenous Pacing in Congenital Heart Block Patients: Ventricular Function Comparisons using 2 D Speckle Imaging
- Author
-
Sanjeev Aggarwal, Gautam Singh, Peter P. Karpawich, and Diana M. Torpoco Rivera
- Subjects
medicine.medical_specialty ,Ventricular function ,business.industry ,Retrospective cohort study ,Single Center ,Congenital heart block ,Transvenous pacing ,medicine.anatomical_structure ,Congenital complete heart block ,Ventricle ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Cardiology ,Speckle imaging ,business - Abstract
Background: Pacemaker implantation is recommended in symptomatic patients with congenital complete heart block (CHB) with either an epicardial (epi) or transvenous (TV) approach depending on patient age / size. However, there is a paucity of data on updated left ventricle (LV) functional indices between the two approaches. Objective: Our aim was to compare LV function using speckle strain imaging in patients with LV Epi vs. best site right (R) TV pacing Methods: This was a single center, retrospective study of patients with isolated CHB …
- Published
- 2021
37. Foetal bradycardia due to congenital heart blocks: a study of three cases with review literature
- Author
-
H. Atapattu, D. M. Casather, D. P. Kaluarachchi, and Tiran Dias
- Subjects
Fetal Arrhythmia ,Foetal bradycardia ,Pediatrics ,medicine.medical_specialty ,Fetus ,business.industry ,Medicine ,Prenatal diagnosis ,Fetal Bradycardia ,Disease ,Electrical conduction system of the heart ,business ,Congenital heart block - Abstract
Congenital heart block (CHB) is a cardiac disease with an overall prevalence of approximately1:20000 live births. Structural heart defects, congenital malformations of the cardiac conduction system, maternal connective tissue disorders and fetal myocarditis are the main entities that can cause CHBs. Here we report three cases of congenital heart blocks due to three different pathologies and their outcomes. In the first case, we could not detect a structural pathology for congenital heart block; however, in second and third cases there were significant structural cardiac defects that could contribute to the fetal bradycardia. Prenatal management of fetal arrhythmia may improve the outcome of an affected fetus or neonate. Precise and timely prenatal diagnosis is critical for the selection of the appropriate prenatal treatments. However, there are many limitations in managing CHB even today and more useful strategies need to be investigated.
- Published
- 2021
38. Mortality in congenital heart block
- Author
-
Marie Wahren-Herlenius and Sven-Erik Sonesson
- Subjects
medicine.medical_specialty ,Rheumatology ,business.industry ,Internal medicine ,Immunology ,Cardiology ,Immunology and Allergy ,Medicine ,business ,Congenital heart block - Published
- 2020
39. ASSOCIATIONS AND OUTCOMES OF CONGENITAL HEART BLOCK IN THE NEONATAL PERIOD: A NATIONWIDE DATA ANALYSIS
- Author
-
Estefania Rivera Flores, Saleem Almasarweh, Prithvi Sendi, Ronald J. Kanter, Jun Sasaki, anas abdul kayoum, and Balagangadhar R. Totapally
- Subjects
Pediatrics ,medicine.medical_specialty ,business.industry ,Period (gene) ,medicine ,Cardiology and Cardiovascular Medicine ,business ,Congenital heart block - Published
- 2021
40. Complete congenital heart block: A case of multilevel block
- Author
-
Jeffrey P. Moak, Ian W Hovis, and David N. Schidlow
- Subjects
medicine.medical_specialty ,Bachmann bundle ,Case Report ,030204 cardiovascular system & hematology ,Neonatal lupus ,Interatrial dissociation ,Congenital heart block ,Complete congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Block (telecommunications) ,Complete heart block ,Diseases of the circulatory (Cardiovascular) system ,Medicine ,Atrial dissociation ,Biatrial dissociation ,030203 arthritis & rheumatology ,business.industry ,Interatrial Block ,RC666-701 ,Dual-chamber epicardial pacemaker ,Cardiology ,Interatrial block ,Cardiology and Cardiovascular Medicine ,business - Published
- 2017
41. Near-zero fluoroscopy implantation of dual-chamber pacemaker in pregnancy using electroanatomic mapping
- Author
-
Hakan Paydak, Monica Lo, Jason Payne, and Waddah Maskoun
- Subjects
Electroanatomic mapping ,medicine.medical_specialty ,Heart block ,Case Report ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Complete heart block ,Fluoroscopy ,Diseases of the circulatory (Cardiovascular) system ,030212 general & internal medicine ,Dual Chamber Pacemaker ,Fetus ,Pregnancy ,medicine.diagnostic_test ,business.industry ,Gestational age ,medicine.disease ,RC666-701 ,Cardiology ,Pacemaker, Pregnancy ,Radiology ,3D electroanatomic mapping ,Cardiology and Cardiovascular Medicine ,business ,Near-zero fluoroscopy - Abstract
Background: Complete Heart Block (CHB) is uncommon in pregnancy and challenging to manage due to risks of radiation to the fetus. Methods: A 27-year-old with known 1st degree AV block at 11 weeks gestational age presents with a 5-week history of fatigue, dizziness, and severe dyspnea. BP was 110/60
- Published
- 2017
42. Low titer, isolated anti Ro/SSA 60 kd antibodies is correlated with positive pregnancy outcomes in women at risk of congenital heart block
- Author
-
Anna Ghirardello, Marta Tonello, Ornella Milanesi, Silvia Visentin, Alessandra Zambon, Amelia Ruffatti, Elena Mattia, Alessia Cerutti, and Ariela Hoxha
- Subjects
0301 basic medicine ,Time Factors ,Congenital heart block ,Gastroenterology ,0302 clinical medicine ,Pregnancy ,Prospective Studies ,biology ,Incidence ,Pregnancy Outcome ,Antibody titer ,General Medicine ,Anti-Ro/SSA 52kd ,Titer ,Italy ,Antibodies, Antinuclear ,Anti-La/SSB ,Anti-p200 ,Anti-Ro/SSA 60 kd ,Isolated autoantibodies ,Rheumatology ,Female ,Antibody ,Adult ,medicine.medical_specialty ,Pregnancy Complications, Cardiovascular ,Enzyme-Linked Immunosorbent Assay ,03 medical and health sciences ,stomatognathic system ,Internal medicine ,medicine ,Humans ,Clinical significance ,Autoantibodies ,030203 arthritis & rheumatology ,business.industry ,Infant, Newborn ,Autoantibody ,medicine.disease ,eye diseases ,stomatognathic diseases ,Heart Block ,030104 developmental biology ,Immunology ,biology.protein ,business ,Follow-Up Studies ,Anti-SSA/Ro autoantibodies - Abstract
Congenital heart block (CHB) is an autoantibody mediated disorder presumably caused by placental transmission of maternal autoantibodies to Ro/SSA 52 kd, p200, Ro/SSA 60 kd, La/SSB ribonucleoproteins. This study investigated the clinical significance of isolated anti-Ro/SSA 52 kd, anti-p200, anti-Ro/SSA 60 kd, and anti-La/SSB antibodies in positive pregnant patients. One hundred sixty-three pregnant women positive to anti-Ro/SSA 52 kd and/or anti-Ro/SSA 60 kd and/or anti-La/SSB antibodies were prospectively enrolled in the study. Anti-Ro52, anti-Ro60, anti-p200, and anti-La antibodies were assayed using home-made ELISA assays. Isolated antibody positivity was found in 25 women (15.3%), while multiple antibody positivity in 138 (84.7%). Twenty-four developed CHB, and the 139 had a favorable pregnancy outcome. The prevalence of isolated anti-Ro/SSA 60 kd antibodies was significantly higher (p
- Published
- 2017
43. Surgical Placement of Permanent Pacemaker Following Temporary Trasvenous Pacemaker in a Child with Congenital Heart Block and Moderate Size PDA: A Case Report
- Author
-
Kazi Zahidul Hoque, Manzoor Hussain, Masumul Gani Chowdhury, Abu Sayeed Munsi, and Rezoana Rima
- Subjects
medicine.medical_specialty ,business.industry ,Anesthesia ,Medicine ,General Medicine ,Permanent pacemaker ,business ,Congenital heart block ,Surgery - Abstract
not availableBangladesh J Child Health 2016; VOL 40 (2) :124-128
- Published
- 2017
44. Minimally Invasive Epicardial Pacemaker Implantation in Neonates with Congenital Heart Block
- Author
-
Martino Martinelli Filho, Roberto Costa, Roger G. Carrillo, and Kátia Regina da Silva
- Subjects
Heart Defects, Congenital ,Male ,medicine.medical_specialty ,lcsh:Diseases of the circulatory (Cardiovascular) system ,Pacemaker, Artificial ,Cardiac pacing ,Treatment outcome ,Operative Time ,030204 cardiovascular system & hematology ,Congenital heart block ,Pacemaker implantation ,Medical illustration ,Infants, Newborns ,03 medical and health sciences ,0302 clinical medicine ,Postoperative Complications ,Medical Illustration ,medicine ,Humans ,Minimally Invasive Surgical Procedures ,Atrioventricular Block ,Intraoperative Complications ,business.industry ,Follow up studies ,Cardiac Pacing, Artificial ,Infant, Newborn ,Reproducibility of Results ,Original Articles ,Equipment Design ,medicine.disease ,Surgery ,Electrodes, Implanted ,Pacemaker ,Heart Block ,Treatment Outcome ,030228 respiratory system ,lcsh:RC666-701 ,Operative time ,Female ,Radiography, Thoracic ,Cardiology and Cardiovascular Medicine ,business ,Atrioventricular block ,Follow-Up Studies - Abstract
Background: Few studies have characterized the surgical outcomes following epicardial pacemaker implantation in neonates with congenital complete atrioventricular block (CCAVB). Objective: This study sought to assess the long-term outcomes of a minimally invasive epicardial approach using a subxiphoid access for pacemaker implantation in neonates. Methods: Between July 2002 and February 2015, 16 consecutive neonates underwent epicardial pacemaker implantation due to CCAVB. Among these, 12 (75.0%) had congenital heart defects associated with CCAVB. The patients had a mean age of 4.7 ± 5.3 days and nine (56.3%) were female. Bipolar steroid-eluting epicardial leads were implanted in all patients through a minimally invasive subxiphoid approach and fixed on the diaphragmatic ventricular surface. The pulse generator was placed in an epigastric submuscular position. Results: All procedures were successful, with no perioperative complications or early deaths. Mean operating time was 90.2 ± 16.8 minutes. None of the patients displayed pacing or sensing dysfunction, and all parameters remained stable throughout the follow-up period of 4.1 ± 3.9 years. Three children underwent pulse generator replacement due to normal battery depletion at 4.0, 7.2, and 9.0 years of age without the need of ventricular lead replacement. There were two deaths at 12 and 325 days after pacemaker implantation due to bleeding from thrombolytic use and progressive refractory heart failure, respectively. Conclusion: Epicardial pacemaker implantation through a subxiphoid approach in neonates with CCAVB is technically feasible and associated with excellent surgical outcomes and pacing lead longevity.
- Published
- 2017
45. Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life
- Author
-
Yimin Hua and Kaiyu Zhou
- Subjects
Pediatrics ,medicine.medical_specialty ,Autoimmune Diseases ,Congenital ,Disease Management ,Heart Block ,High-risk ,Pregnancy ,Prenatal Care ,MEDLINE ,lcsh:Medicine ,Prenatal care ,Review Article ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,030203 arthritis & rheumatology ,Fetus ,business.industry ,lcsh:R ,General Medicine ,medicine.disease ,Research findings ,Female ,business - Abstract
Objective: Congenital heart block (CHB) is a rare but life-threatening disorder. More than half of CHB cases are associated with maternal autoimmune, which are termed as autoimmune-associated CHB. This review summarized the recent research findings in understanding autoimmune-associated CHB, discussed the current diagnostic approaches and management strategies, and summarized the problems and future directions for this disorder. Data Sources: We retrieved the articles published in English from the PubMed database up to January 2017, using the keywords including “Autoimmune-associated”, “Autoimmune-mediated”, and “Congenital heart block”. Study Selection: Articles about autoimmune-associated CHB were obtained and reviewed. Results: Observational studies consistently reported that transplacental maternal antibodies might recognize fetal or neonatal antigens in various tissues and result in immunological damages, but the molecular mechanisms underlying CHB pathogenesis still need illuminated. Multiple factors were involved in the process of atrioventricular block development and progression. While several susceptibility genes had been successfully defined, how these genes and their protein interact and impact each other remains to be explored. With currently available diagnostic tools, fetal ultrasound cardiography, and fetal magnetocardiography, most of CHB could be successfully diagnosed and comprehensively evaluated prenatally. The efficacy of current approaches for preventing the progression and recurrence of CHB and other autoimmune-mediated damages was still controversial. Conclusions: This review highlighted the relationships between autoimmune injuries and CHB and strengthened the importance of perinatal management and therapy for autoimmune-associated CHB.
- Published
- 2017
46. Invited Commentary
- Author
-
Andreas Pflaumer
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,business.industry ,Long term follow up ,Epicardial pacing ,Thoracic Surgical Procedures ,Congenital heart block ,Ventricular Dysfunction, Left ,Heart Block ,Lv dysfunction ,Internal medicine ,Cardiology ,Humans ,Medicine ,Surgery ,Child ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Published
- 2020
47. Surveillance of congenital heart block in highly specialised care
- Author
-
Marie Wahren-Herlenius and Sven-Erik Sonesson
- Subjects
Pediatrics ,medicine.medical_specialty ,Rheumatology ,business.industry ,Immunology ,Immunology and Allergy ,Medicine ,business ,Congenital heart block - Published
- 2020
48. CONGENITAL COMPLETE HEART BLOCK IN PREGNANT WOMEN: A MULTIDISCIPLINARY APPROACH TO DIAGNOSTICS AND TREATMENT
- Author
-
R E Kalinin, I A Suchkov, N.D. Mzhavanadze, V.O. Povarov, and I I Shitov
- Subjects
medicine.medical_specialty ,Pregnancy ,business.industry ,Heart block ,Gestational age ,medicine.disease ,Congenital heart block ,Congenital complete heart block ,Internal medicine ,medicine ,Etiology ,Cardiology ,In patient ,business ,Atrioventricular block - Abstract
Treatment options in patients with complete congenital heart block (atrioventricular block of III degree) may be controversial. Major obstacles are faced in pregnant women with newly detected heart block. Type of arrhythmia, presence of hemodynamic instability or syncope, anatomy of heart chambers and vessels, gestational age significantly influence the treatment. This article presents substantial information on etiology, pathogenesis, diagnostics, and possible treatment options in pregnant women with complete congenital heart block.
- Published
- 2016
49. Congenital second-degree heart block and total anomalous pulmonary venous return associated with microduplication of 1q32.2
- Author
-
Adaora Madubuko, Michele Takyi, Rajeev Mehta, Seyni Gueye-Ndiaye, Lauren Walzer, Surasak Puvabanditsin, and Vidya Puthenpura
- Subjects
medicine.medical_specialty ,lcsh:Diseases of the circulatory (Cardiovascular) system ,Heart block ,1q32 microduplication ,lcsh:Medicine ,Single-nucleotide polymorphism ,Total anomalous pulmonary venous return ,Congenital heart block ,Internal medicine ,Gene duplication ,Cardiac conduction ,medicine ,total anomalous pulmonary venous return ,congenital heart block ,business.industry ,lcsh:R ,congenital ,lcsh:RJ1-570 ,Chromosome ,lcsh:Pediatrics ,medicine.disease ,lcsh:RC666-701 ,Pediatrics, Perinatology and Child Health ,Cardiology ,Cardiology and Cardiovascular Medicine ,business ,Venous return curve - Abstract
We report a term female infant with congenital heart block and total anomalous of pulmonary venous return. The results of single nucleotide polymorphism oligonucleotide microarray analysis showed an interstitial duplication of approximately 818 Kb, which involved 11 genes, including the entire LAMB3 gene which is known to associate with cardiac conduction defect. Our report adds to the collective knowledge that the cardiac conduction defect is a clinical feature of chromosome 1q32.2 duplication.
- Published
- 2018
50. Cesarean delivery in congenital heart block and need of temporary pacing: A case report
- Author
-
Amarjyoti Hazarika and Sumit Soni
- Subjects
lcsh:RD78.3-87.3 ,medicine.medical_specialty ,Anesthesiology and Pain Medicine ,business.industry ,lcsh:Anesthesiology ,Medicine ,Cesarean delivery ,business ,Letters to Editor ,Congenital heart block ,Surgery - Published
- 2019
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