The Experts below are selected from a list of 306 Experts worldwide ranked by ideXlab platform
Ian D Sullivan - One of the best experts on this subject based on the ideXlab platform.
-
congenital fetal Heart Block a potential therapeutic role for intravenous immunoglobulin
Obstetrics & Gynecology, 2010Co-Authors: Anna L David, Ian D Sullivan, Ifat Ataullah, R Yates, Peter Charles, David J WilliamsAbstract:BACKGROUND: Congenital Heart Block affects 2% of all mothers with anti-Ro/La antibodies, can cause Heart failure in utero, and has a 20% mortality rate in the first 3 years of life. Maternal fluorinated steroids to prevent or reverse congenital Heart Block can cause pregnancy complications. Intravenous immunoglobulin (IVIG) has been given with maternal steroids to prevent the recurrence of congenital Heart Block, although its efficacy is unproven.CASE: We report the use of IVIG to prevent progression of 2:1 congenital Heart Block with intermittent complete Heart Block. After two maternal infusions of IVIG (0.4 g/kg) at 31 weeks of gestation, the fetal Heart rate reverted to long periods of sinus rhythm, which was sustained until postnatal life.CONCLUSION: Our case supports investigating IVIG in the prevention or treatment of this life-threatening condition. (Obstet Gynecol 2010;116:543-7)
-
transcatheter closure of perimembranous ventricular septal defect is the risk of Heart Block too high a price
Heart, 2007Co-Authors: Ian D SullivanAbstract:The late development of Heart Block in paediatric patients following device closure of a perimembranous ventricular septal defect may be a cause for concern. See article on page 355.
Antony Chu - One of the best experts on this subject based on the ideXlab platform.
-
abstract 17748 predictors of persistence of complete Heart Block post transcatheter aortic valve replacement
Circulation, 2016Co-Authors: Abdul Rahman Mourad, Antony ChuAbstract:Introduction: Transcatheter aortic valve replacement (TAVR) is an increasingly prevalent therapy in patients with severe symptomatic aortic stenosis (AS) deemed inoperable or at high risk for complications with surgical aortic valve replacement (SAVR). Atrioventricular (AV) conduction disturbances requiring permanent pacemaker (PPM) implantation may complicate TAVR. We report single-center data on 207 consecutive patients undergoing TAVR with placement of the Edward SAPIEN valve who required post-TAVR permanent pacemaker implantation for complete AV Block. Methods: A retrospective analysis of prospectively collected data includes clinical, procedural, echocardiographic, ECG and device interrogation of 207 consecutive patients who underwent TAVR procedure with placement of Edward SAPIEN valve at our institution from March 2012 to February 2016. We excluded 23 patients with prior permanent pacemaker. No patients met guideline indications for pre-TAVR permanent pacing. Results: A total of 24 patients (13%) required post-TAVR permanent pacemaker. At 30 days post-TAVR, 7 of these patients (29.1%) required 100% ventricular pacing. The remaining 17 (70.9%) patients who received pacemakers had resolution of AV Block by 30 days post-TAVR and did not require significant ventricular pacing. All the 7 patients who needed long term significant ventricular pacing had right bundle branch Block and 6 of them developed intra procedural complete Heart Block, where as majority of patient who did not require significant long term ventricular pacing did not have right bundle branch Block and did not develop intraprocedural Heart Block. Different valve sizes were present in both groups. Conclusions: Despite 13% of post-TAVR patients receiving permanent pacemakers for complete AV Block, 70.9% of these patients had resolution of AV Block at 30 days post-TAVR and did not require ventricular pacing. Right bundle branch Block (RBBB) and Intra-procedural Heart Block were highly predictive of long term pacing requirements in post TAVR patients who develop complete Heart Block, where as valve size did not have predictive significance.
Kyongjin Lee - One of the best experts on this subject based on the ideXlab platform.
-
complete Heart Block associated with device closure of perimembranous ventricular septal defects
The Journal of Thoracic and Cardiovascular Surgery, 2008Co-Authors: Dragos Predescu, Rajiv Chaturvedi, Mark K Friedberg, Lee N Benson, Akira Ozawa, Kyongjin LeeAbstract:Objective The development of the Amplatzer Membranous VSD Occluder (AGA Medical Corp, Plymouth, Minn) for closure of the perimembranous ventricular septal defect has ameliorated many of the technical difficulties of previous devices. Application of this new technology requires comparative evaluation with the current standard of surgical repair. We report our experience of complete Heart Block associated with device closure of a large perimembranous ventricular septal defect with unequivocal indications for intervention. Methods We performed a retrospective review of 20 patients between January 2003 and August 2005 who underwent perimembranous ventricular septal defect device closure, 18 with hemodynamically large shunts meeting the surgical criteria for intervention. The median age was 1.6 years (range, 0.5–16.2 years), and the median weight was 9.7 kg (range, 6.2–43 kg). Results Acute complete shunt occlusion was achieved in all patients. There were no acute procedural complications. The median follow-up time was 23.1 months (range, 1–37.8 months). Four (22%) had complete Heart Block at 17 days, 4.2 months, 8.8 months, and 37.5 months after implantation, respectively. No risk factors were identified for development of complete Heart Block, including age, weight, trisomy 21, preceding conduction abnormalities, perimembranous ventricular septal defect size related to body surface area or device size, and progressive device flattening. Conclusions Device closure of large perimembranous ventricular septal defects in infants and children with the Amplatzer Membranous VSD Occluder resulted in excellent closure rates but an unacceptably high rate of complete Heart Block.
Andrew C. Cook - One of the best experts on this subject based on the ideXlab platform.
-
Is complete Heart Block after surgical closure of ventricular septum defects still an issue
The Annals of thoracic surgery, 2006Co-Authors: Henrik Ørbæk Andersen, Marc R. De Leval, Victor Tsang, Martin J. Elliott, Andrew C. CookAbstract:BACKGROUND: A serious complication after surgical closure of ventricular septal defect (VSD) is complete Heart Block. In this retrospective study, we reviewed the incidence of complete Heart Block after surgical closure of a VSD at Great Ormond Street Hospital from 1976 to 2001 to identify any particular anatomic features that still predisposed patients to surgically-induced complete Heart Block and to provide anatomic guidelines to avoid this in future. METHODS: Data were extracted from our local database for patients having (1) isolated VSD or VSD in the setting of (2) tetralogy of Fallot with pulmonary stenosis or (3) tetralogy of Fallot with pulmonary atresia; (4) absent pulmonary valve syndrome; (5 and 6) coarctation or interruption of the aortic arch; and (7) subaortic fibrous shelf. We carefully reviewed the operative notes from all patients with postoperative complete Heart Block to discover any predisposing anatomical reasons to explain the complication. RESULTS: Two thousand seventy-nine patients had a VSD closure. Permanent complete Heart Block developed in 7 of 996 patients (0.7%) with an isolated defect and in 1 of 847 patients (0.1%) with tetralogy of Fallot. Four more patients had postoperative complete Heart Block. CONCLUSIONS: Instances of iatrogenic complete Heart Block continue to occur after surgical VSD closure, either because of unexpected biological variations or because of unawareness of the disposition of the atrioventricular conduction axis in particular circumstances. This report emphasizes the latter aspect in details and suggests a risk of iatrogenic complete Heart Block of less than 1%.
Jill P Buyon - One of the best experts on this subject based on the ideXlab platform.
-
autoimmune congenital Heart Block complex and unusual situations
Lupus, 2016Co-Authors: Pilar Britozeron, Jill P Buyon, Peter M Izmirly, Manuel Ramoscasals, Munther A KhamashtaAbstract:Autoimmune congenital Heart Block (ACHB) is an immune-mediated cardiac disease included among the manifestations collectively referred to as neonatal lupus. The placental transference of maternal Ro/La autoantibodies may damage the conduction tissues during fetal development leading to Blocking of signal conduction at the atrioventricular (AV) node in an otherwise structurally normal Heart. Irreversible complete AV Block is the main cardiac manifestation of ACHB, but some babies may develop endocardial fibroelastosis, valvular insufficiency, and/or frank cardiomyopathies with significantly reduced cardiac function requiring transplant. The severity of ACHB is illustrated by a global mortality rate of 20% and pacemaker rates of at least 64%, often within the first year of life. This review analyses the main complex and/or unusual clinical situations associated with ACHB, including unusual maternal immunological profiles, infrequent maternal autoimmune diseases, cardiac damage unrelated to AV Block, fetal invasive management, late complications after birth, risk of congenital Heart Block (CHB) in ovodonation and in vitro fertilization techniques, the role of maternal features other than autoimmunity, the influence of the birth order or the risk of CHB in twins and triplets.
-
the clinical spectrum of autoimmune congenital Heart Block
Nature Reviews Rheumatology, 2015Co-Authors: Pilar Britozeron, Jill P Buyon, Peter M Izmirly, Manuel Ramoscasals, Munther A KhamashtaAbstract:Autoimmune congenital Heart Block is more likely to occur in the babies of women with rheumatic diseases, particularly women seropositive for anti-Ro or anti-La autoantibodies. Here, the authors provide advice for the management of these women and their babies in juxtaposition to a systematic assessment of the epidemiology and classification of the disease.
-
prevention and treatment in utero of autoimmune associated congenital Heart Block
Cardiology in Review, 2014Co-Authors: Amit Saxena, Jill P Buyon, Peter M Izmirly, Barbara Mendez, Deborah M FriedmanAbstract:Transplacental transfer of maternal anti-Ro and/or anti-La autoantibodies can result in fetal cardiac disease, including congenital Heart Block and cardiomyopathy, called cardiac neonatal lupus (NL). Thousands of women are faced with the risk of cardiac NL in their offspring, which is associated with significant morbidity and mortality. There are no known therapies to permanently reverse third-degree Heart Block in NL, although several treatments have shown some effectiveness in incomplete Heart Block and disease beyond the atrioventricular node. Fluorinated steroids taken during pregnancy have shown benefit in these situations, although adverse effects may be concerning. Published data are discordant on the efficacy of fluorinated steroids in the prevention of mortality in cardiac NL. β-agonists have been used to increase fetal Heart rates in utero. The endurance of β-agonist effect and its impact on mortality are in question, but when used in combination with other therapies, they may provide benefit. No controlled experiments regarding the use of plasmapheresis in cardiac NL have been performed, despite its theoretical benefits. Intravenous immunoglobulin was not shown to prevent cardiac NL at a dose of 400 mg/kg, although it has shown effectiveness in the treatment of associated cardiomyopathy both in utero and after birth. Retrospective studies have shown that hydroxychloroquine may prevent the recurrence of cardiac NL in families with a previously affected child, and a prospective open-label trial is currently recruiting patients in order to fully evaluate this relationship.
-
Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital Heart Block in neonatal lupus
Current Rheumatology Reports, 2007Co-Authors: Deborah M Friedman, Ann Rupel, Jill P BuyonAbstract:Neonatal lupus syndrome is a model of passively acquired autoimmunity in which the pregnant woman’s serum contains specific antibodies to 52 or 60 kd SSA/Ro and/or 48 kd SSB/La, which cross the placenta and are associated with the development of congenital Heart Block in the fetus and/or a transient rash or various liver and blood cell abnormalities in the newborn. To date, congenital Heart Block is a permanent condition that entails significant morbidity and mortality, with nearly all affected infants requiring pacemakers and with an 80% cumulative probability of survival at 3 years of age. An intensive search is on for the specific etiopathophysiology and for new clinical tools to approach and treat this disease.
-
successful in utero therapy of fetal Heart Block
American Journal of Obstetrics and Gynecology, 1995Co-Authors: Joshua A Copel, Jill P Buyon, Charles S KleinmanAbstract:Abstract OBJECTIVE: Congenital complete Heart Block is associated with maternal autoantibodies to Ro and La proteins, which injure the fetal cardiac conduction system. We administered dexamethasone to the mothers of five fetuses with Heart Block caused by maternal antibodies. STUDY DESIGN: We diagnosed five cases of fetal Heart Block at 20 to 23 weeks and treated all mothers with dexamethasone 4 mg orally each day for the remainder of the pregnancy. All patients were positive for anti-SS-A (anti-Ro) and/or anti-SS-B (anti-La) antibodies. RESULTS: Four patients had complete Heart Block, and one had second-degree Block. In two patients (one with complete Heart Block, one with second-degree Heart Block) the degree of Block lessened with treatment. Hydrops in three complete Heart Block patients resolved after treatment. Maternal antibody levels did not change. Matched maternal and cord samples at delivery showed similar antibody levels. CONCLUSIONS: Complete Heart Block can respond to transplacental glucocorticoid therapy with improved cardiac conduction. Because there may be a concurrent myocarditis, treatment in utero may also improve cardiac contractility, leading to the observed rapid resolution of hydrops. Treatment with steroids that cross the placenta should be considered for newly diagnosed cases of complete Heart Block with positive antibody screens.
