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Fetal pacemaker

N W E Van den Berg, M G Slieker, I M van Beynum, C M Bilardo, D de Bruijn, S A Clur, J M J Cornette, I M E Frohn-Mulder, M C Haak, K E H van Loo-Maurus, G T R Manten, A B M H Rackowitz, L A J Rammeloo, A Reimer, M E B Rijlaarsdam, M W Freund
INTRODUCTION: Congenital atrioventricular block (CAVB) is a rare disorder with a significant morbidity and mortality. Consensus regarding the prescription and efficacy of prenatal corticosteroids is lacking. This nationwide study was initiated to evaluate the effects of prenatal treatment with corticosteroids on the outcome of CAVB in The Netherlands. METHODS: All fetuses identified with isolated congenital AVB-II° or AVB-III° in any of the eight academic fetal heart centers of The Netherlands between 2003 and 2013 were included and reviewed...
September 30, 2016: International Journal of Cardiology
Dey Madhusudan, Agarwal Raju, Nambula Vijaya
BACKGROUND: Autoimmune fetal congenital heart block (CHB) is the most severe manifestation of neonatal lupus, and it is seen when maternal autoimmune antibodies cross the placenta and damage the AV node of the fetus. CHB is mainly associated with maternal SLE with anti-Ro/SSA- and anti-La/SSB-positive status, and incidence of CHB increases when both the antibodies are present. This study was conducted to know the incidence of fetal CHB in patients of SLE who had ANA, anti-Ro/SSA and anti-La/SSB positivity...
October 2016: Journal of Obstetrics and Gynaecology of India
Adriana Nicholson Vest, Li Zhou, Yaniv Bar-Cohen, Gerald Eli Loeb
We have developed a rechargeable fetal micropacemaker in order to treat severe fetal bradycardia with comorbid hydrops fetalis, a life-threatening condition in pre-term non-viable fetuses for which there are no effective treatment options. The small size and minimally invasive form factor of our design limit the volume available for circuitry and a power source. The device employs a fixed-rate and fixed-amplitude relaxation oscillator and a tiny, rechargeable lithium ion power cell. For both research and clinical applications, it is valuable to monitor the electrode-myocardium interface in order to determine that adequate pacemaker output is being provided...
July 2016: Physiological Measurement
Na Eun Kim, Jae Hyuk Lee, In Sun Chung, Jun Yong Lee
Sjogren's syndrome is one of the most common autoimmune disorders and has a female predominance. Maternal circulating autoantibodies such as anti-Ro/SSA and anti-La/SSB antibodies can cause congenital heart block of fetus, and in severe case, emergency pacemaker implantation may be needed for neonate. Therefore, it is very important to understand maternal and fetal condition and pay attention to the status of the neonate during delivery. In this paper, we present a case of patient with Sjogren's syndrome who underwent cesarean section under spinal anesthesia...
June 2016: Korean Journal of Anesthesiology
Franziska Greulich, Mark-Oliver Trowe, Andreas Leffler, Carsten Stoetzer, Henner F Farin, Andreas Kispert
Initiation of cardiac excitation depends on a specialized group of cardiomyocytes at the venous pole of the heart, the sinoatrial node (SAN). The T-box transcription factor gene Tbx18 is expressed in the SAN myocardium and is required for formation of a large portion of the pacemaker. Previous studies suggested that Tbx18 is also sufficient to reprogram ventricular cardiomyocytes into SAN cells in rat, guinea-pig and pig hearts. To evaluate the consequences of misexpression of Tbx18 for imposing a nodal phenotype onto chamber myocardial cells in fetal mice, we used two independent conditional approaches with chamber-specific cre driver lines and an Hprt(Tbx18) misexpression allele...
August 2016: Journal of Molecular and Cellular Cardiology
Tao Fujioka, Masaki Nii, Yasuhiko Tanaka
Congenital complete atrioventricular block is a known lethal condition. Although antenatal diagnosis and the technical advances of pacemaker treatment have reduced its mortality, treatment of premature babies with significant myocardial damage remains a challenge. In this paper, we report the case of a premature low-birth-weight infant with congenital complete atrioventricular block and extremely low ventricular rate, fetal hydrops, and myocarditis who was successfully treated with staged permanent pacemaker implantation...
June 2016: Cardiology in the Young
Claudia Stöllberger, Christian Wegner, Abraham Benatar, Thomas K Chin, Joanna Dangel, Danielle Majoor-Krakauer, Tapas K Mondal, Shanthi Sivanandam, Norman H Silverman, Jaap van Waning, Josef Finsterer
Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology. Prenatal diagnosis of LVHT can be established by fetal echocardiography. A review of 106 published cases showed that 46 cases with prenatally diagnosed LVHT were alive 0.5-120 months after birth. Since the course of cases with prenatally LVHT after publication is unknown, we aimed to collect follow-up-information. Information regarding vital status, cardiac and extracardiac morbidity was gathered by contacting the authors of the 46 cases...
June 2016: Pediatric Cardiology
J Matěcha, L Riedlbauchová
OBJECT: Describing the course of pregnancy and perinatal outcomes in a patient with supraventricular tachycardia treated by electrical cardioversion. DESIGN: Case report. SETTING: Department of Obstetrics and Gynaecology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital. CASE REPORT: We report a case of electrical cardioversion performed during pregnancy. Young patient with a history of surgical correction of congenital heart defect (double outlet right ventricle, aortic arch hypoplasia and coarctation) with implanted pacemaker for dysfunction of both SA and AV node was diagnosed with persistent atrial flutter leading to incipient heart failure during pregnancy...
January 2016: Ceská Gynekologie
Peter M Izmirly, Amit Saxena, Sara K Sahl, Ummara Shah, Deborah M Friedman, Mimi Y Kim, Jill P Buyon
OBJECTIVES: Extension of disease beyond the atrioventricular (AV) node is associated with increased mortality in cardiac neonatal lupus (NL). Treatment of isolated heart block with fluorinated steroids to prevent disease progression has been considered but published data are limited and discordant regarding efficacy. This study evaluated whether fluorinated steroids given to manage isolated advanced block prevented development of disease beyond the AV node and conferred a survival benefit...
June 2016: Annals of the Rheumatic Diseases
Pamela I Doti, Ona Escoda, Sergi Cesar-Díaz, Silvia Palasti, Irene Teixidó, Georgia Sarquella-Brugada, Olga Gómez, Josep M Martínez, Gerard Espinosa
The objective of this study was to describe the clinical and immunological characteristics of maternal autoimmune-mediated fetal congenital heart block (CHB) in a cohort of pregnant women from an autoimmune disease pregnancy clinic. This is a retrospective observational study of all women presenting with CHB in our autoimmune disease pregnancy clinic from January 1997 to December 2014. In addition, perinatal outcome is also described. Fourteen patients accounting for 18 fetuses with CHB were identified. The median age was 32...
February 2016: Clinical Rheumatology
P Brito-Zerón, P M Izmirly, M Ramos-Casals, J P Buyon, M A Khamashta
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...
February 2016: Lupus
Sema Tanriverdi, Zulal Ulger, Betul Siyah Bilgin, Nilgun Kultursay, Mehmet Yalaz, Yuksel Atay, Ozge Altun Koroglu
INTRODUCTION: Neonatal lupus syndrome (NLS) is a passively acquired autoimmune condition due to the transplacental passage of maternal anti-Ro/SSA and anti-La/SSB antibodies in mothers with systemic lupus erythematosus (SLE), and congenital complete heart block (CHB) is its most serious manifestation. Skin and hepatic involvement may occur in later infancy. CASE PRESENTATION: A term infant with fetal bradycardia, detected at the 23rd gestational age, was diagnosed with CHB due to NLS and was successfully treated with a permanent epicardial pacemaker...
September 2015: Iranian Red Crescent Medical Journal
Amit Saxena, Peter M Izmirly, Sung Won Han, Paraskevi Briassouli, Tania L Rivera, Hua Zhong, Deborah M Friedman, Robert M Clancy, Jill P Buyon
BACKGROUND: Cardiac manifestations of neonatal lupus (cardiac NL) include congenital heart block and cardiomyopathy. Several candidate biomarkers were evaluated in cases at risk for cardiac NL on the basis of potential roles in inflammation, fibrosis, and cardiac dysfunction: C-reactive protein (CRP); NT-pro-B-type natriuretic peptide (NT-proBNP); troponin I; matrix metalloproteinase (MMP)-2; urokinase plasminogen activator (uPA); urokinase plasminogen activator receptor (uPAR); plasminogen; and vitamin D...
August 25, 2015: Journal of the American College of Cardiology
Håkan Eliasson, Sven-Erik Sonesson, Stina Salomonsson, Amanda Skog, Marie Wahren-Herlenius, Fredrik Gadler
BACKGROUND: Subgroups of pacemaker (PM)-treated children with isolated complete atrioventricular block are at risk of developing left ventricular (LV) dysfunction. OBJECTIVES: We aimed to compare the long-term outcome in anti-SSA-Ro/SSB-La antibody-exposed (AB+) and unexposed (AB-) patients and identify preimplantation variables associated with poor outcome. METHODS: In total, 127 PM-treated patients aged 0-17 years with isolated complete atrioventricular block were studied retrospectively...
November 2015: Heart Rhythm: the Official Journal of the Heart Rhythm Society
Claudia Stöllberger, Christian Wegner, Josef Finsterer
Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology. Aim of the review was to summarize the current knowledge about fetal LVHT, including clinical presentation, associated cardiac and extracardiac abnormalities and outcome. In 88 cases, LVHT was diagnosed by fetal echocardiography. In 36 %, no additional cardiac abnormalities were reported; in the remaining 64 %, one or more cardiac abnormalities were reported. Eight cases died prenatally, 17 were electively terminated, and 24 patients died after birth...
October 2015: Pediatric Cardiology
Andrew Ho, Patrick Gordon, Eric Rosenthal, John Simpson, Owen Miller, Gurleen Sharland
Isolated congenital complete heart block (CCHB) is a rare disease with significant associated morbidity and mortality. A diagnosis is often made in fetal life, but data regarding long-term outcomes are limited, and fetal therapy to improve prognosis is controversial. In our institution, 85 fetuses were diagnosed with CCHB from 1981 to 2013 in 80 mothers. There were 37 anti-Ro-positive pregnancies, 36 both anti-Ro and anti-La positive, 10 antibody negative, and 2 of unknown antibody status. Antenatal treatments were given in 14 fetuses, with 8 given fluorinated steroids, 4 beta sympathomimetics, and both in 2...
July 1, 2015: American Journal of Cardiology
Takekazu Miyoshi, Yasuki Maeno, Haruhiko Sago, Noboru Inamura, Satoshi Yasukouchi, Motoyoshi Kawataki, Hitoshi Horigome, Hitoshi Yoda, Mio Taketazu, Makio Shozu, Masaki Nii, Hitoshi Kato, Akiko Hagiwara, Akiko Omoto, Wataru Shimizu, Isao Shiraishi, Heima Sakaguchi, Kunihiro Nishimura, Michikazu Nakai, Keiko Ueda, Shinji Katsuragi, Tomoaki Ikeda
BACKGROUND: Because there is limited information on fetal bradyarrhythmia associated with congenital heart defects (CHD), we investigated its prognosis and risk factors. METHODS AND RESULTS: In our previous nationwide survey of fetal bradyarrhythmia from 2002 to 2008, 38 fetuses had associated CHD. Detailed clinical data were collected from secondary questionnaires on 29 fetuses from 18 institutions, and were analyzed. The 29 fetuses included 22 with isomerism, 4 with corrected transposition of the great arteries (TGA) and 3 with critical pulmonary stenosis; 14 had complete atrioventricular block (AVB), 8 had second-degree AVB, and 16 had sick sinus syndrome; 5 died before birth, and 10 died after birth (5 in the neonatal period)...
2015: Circulation Journal: Official Journal of the Japanese Circulation Society
Nuria Martínez-Sánchez, Ángel Robles-Marhuenda, Rita Álvarez-Doforno, Aurora Viejo, Eugenia Antolín-Alvarado, Lucía Deirós-Bronte, Jose Luis Bartha
OBJECTIVE: To evaluate the efficacy and safety of the combination of steroids, plasmapheresis and intravenous immunoglobulins (IVIG) on maternal anti Ro/SS-A antibody levels in cases of fetal cardiac involvement. MATERIAL AND METHODS: A series of three cases of positive anti-Ro/SS-A mothers with fetuses showing mild cardiac involvement were treated with a triple therapy composed of steroids, plasmapheresis and IVIG. Maternal antibody levels were measured several times before and after the application of each cycle of therapy...
May 2015: Autoimmunity Reviews
Li Zhou, Adriana N Vest, Ramen H Chmait, Yaniv Bar-Cohen, Jay Pruetz, Michael Silka, Kaihui Zheng, Ray Peck, Gerald E Loeb
A miniaturized, self-contained pacemaker that could be implanted with a minimally invasive technique would dramatically improve the survival rate for fetuses that develop hydrops fetalis as a result of congenital heart block. We are currently validating a device that we developed to address this bradyarrhythmia. Preclinical studies in a fetal sheep model are underway to demonstrate that the device can be implanted via a minimally invasive approach, can mechanically withstand the harsh bodily environment, can induce effective contractions of the heart muscle with an adequate safety factor, and can successfully operate for the required device lifetime of three months using the previously-developed closed loop transcutaneous recharging system...
2014: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
C Bosco, E Díaz, R Gutiérrez, J González, M Parra-Cordero, R Rodrigo, P Barja
Preeclampsia (PE) is a major health problem occurring in pregnant women and the principal cause of maternal morbidity and perinatal mortality. It is characterized by alteration of the extravilli trophoblast cell migration toward the endometrial spiral arteries with a concomitant reduction in maternal blood flow in the placenta. This result in a state of ischemia-hypoxia which triggers an oxidative stress stage with production of reactive oxygen species. A cascade of cellular and molecular events leads then to endothelial dysfunction, transduction pathway signal disruption and induction of apoptosis and necrosis mechanisms and therefore a significant reduction in the amount of nutrients required for normal fetal development...
January 2015: Medical Hypotheses
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