Read by QxMD icon Read

congenital heart block

Pedro Pallangyo, Isaac Mawenya, Paulina Nicholaus, Henry Mayala, Amida Kalombola, Godwin Sharau, Naiz Majani, Mohamed Janabi
BACKGROUND: Congenital complete heart block is a life-threatening condition which is highly associated with autoimmune and connective tissue disorders. Presence of maternal autoantibodies for associated conditions increases the risk of delivering a child with congenital complete heart block, however, less than a half of all women with such antibodies are symptomatic even after delivery. Mortality rate is highest during the neonatal period (45 %) and about two-thirds of all cases will require permanent pacing at some point in their lives...
October 19, 2016: Journal of Medical Case Reports
Ehud Chorin, Dan Hu, Charles Antzelevitch, Aviram Hochstadt, Luiz Belardinelli, David Zeltser, Hector Barajas-Martinez, Uri Rozovski, Raphael Rosso, Arnon Adler, Jesaia Benhorin, Sami Viskin
BACKGROUND: The basic defect in long-QT syndrome type III (LQT3) is an excessive inflow of sodium current during phase 3 of the action potential caused by mutations in the SCN5A gene. Most sodium channel blockers reduce the early (peak) and late components of the sodium current (INa and INaL), but ranolazine preferentially reduces INaL. We, therefore, evaluated the effects of ranolazine in LQT3 caused by the D1790G mutation in SCN5A. METHODS AND RESULTS: We performed an experimental study of ranolazine in TSA201 cells expressing the D1790G mutation...
October 2016: Circulation. Arrhythmia and Electrophysiology
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
Katarzyna Iwanicka-Pronicka, Magdalena Socha, Maria Jędrzejowska, Małgorzata Krajewska-Walasek, Aleksander Jamsheer
Holt-Oram syndrome (HOS) features radial ray hypoplasia, heart defect and cardiac conduction impairment. Ulnar-mammary syndrome (UMS) characterizes congenital defects of the ulnar side of the upper limbs, underdevelopment of apocrine glands including hypoplasia and the dysfunction of mammary glands, hypogonadism and obesity. Inheritance of both conditions is autosomal dominant, mutations or deletions are found in the TBX5 and TBX3 gene, respectively. The Polish patient presented short stature, obesity, congenital malformation of the radial and ulnar side of the upper limbs, heart block, hypogonadism and dysmorphic features...
2016: SpringerPlus
Zijo Begic, Edin Begic, Senka Mesihovic-Dinarevic, Izet Masic, Senad Pesto, Mirza Halimic, Almira Kadic, Amra Dobraca
OBJECTIVE: To show the place and role of continuous electrocardiographic twenty-four-hour ECG monitoring in daily clinical practice of pediatric cardiologists. METHODS: According to protocol, 2753 patients underwent dynamic continuous ECG Holter monitoring (data collected from the "Register of ECG Holter monitoring" of Pediatric Clinic, UCC Sarajevo in period April 2003- April 2015). RESULTS: There were 50,5% boys and 49,5% girls, aged from birth to 19 years (1,63% - neonates and infants, 2,6% - toddlers, 9,95% - preschool children, 35,5% - gradeschoolers and 50,3% children in puberty and adolescence)...
July 16, 2016: Acta Informatica Medica: AIM
Anilkumar Singhi, Ejaz Ahmed Sheriff, Kothandam Sivakumar
Complex cyanotic congenital heart diseases with left isomerism are sometimes associated with atrioventricular nodal conduction disturbances that may need permanent pacing. Surgical palliation in such anatomy connecting the superior vena cava to the pulmonary artery precludes a transvenous access for an endocardial pacing lead to the ventricles. Epicardial leads in these patients fail if the pacing thresholds are very high. We report transhepatic permanent ventricular lead implantation for a young boy with heterotaxy complicated by complete heart block...
March 2016: Indian Pacing and Electrophysiology Journal
Veysel Tosun, Necmettin Korucuk, Mustafa Mustafa Karakaş, Ünal Güntekin
Congenitally corrected transposition of the great arteries is a rare abnormality accounting for approximately 1% of clinically apparent congenital heart disease. Age at time of diagnosis and survival rate vary and depend on associated anomalies, including pulmonary stenosis, ventricular septal defect, atrial septal defect, atrioventricular block, and atrioventricular valve regurgitation. Reported cases of corrected transposition of the great arteries with single coronary ostium anomaly and atrial septal defect are very rare...
September 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Patricia E Thomas, Scott L Macicek
BACKGROUND: Catheter ablation has been used to manage supraventricular arrhythmia in children since 1990. This article reviews the history of catheter ablation used to treat arrhythmia in children and discusses new frontiers in the field. We also address ablation in adult patients with a history of congenital heart disease (CHD) that was diagnosed and initially treated in childhood. METHODS: We conducted an evidence-based literature review to gather available data on ablation for supraventricular tachycardia in children and adult patients with CHD...
2016: Ochsner Journal
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
Ida Dzifa Dey, Jerry Coleman, Harriet Kwarko, Michael Mate-Kole
OBJECTIVE: To study maternal and fetal outcomes in Ghanaian women with systemic lupus erythematosus (SLE). METHODS: Retrospective study of pregnancies in women with SLE in a single centre in Ghana. RESULTS: The mean age was 30.1 years and all were nulliparous. Two out of the seven pregnancies were in disease remission at the time of booking. Nephritis without renal impairment was present in 7 pregnancies (6 women). One woman developed intrapartum eclampsia...
June 2016: Ghana Medical Journal
Jan Němec
Repolarization alternans precedes certain types of ventricular arrhythmias and its presence may be prognostically useful, but some ventricular arrhythmias are not preceded by repolarization alternans. Nonalternans changes of ventricular repolarization occur in patients with coronary artery disease and in subjects with congenital long QT syndrome. In animal experiments, nonalternans repolarization lability occurs in a canine model of chronic AV block with propensity to polymorphic ventricular tachycardia, in the perfused rabbit heart exposed to IKr block, and in a murine model of catecholaminergic polymorphic ventricular tachycardia...
August 17, 2016: Journal of Electrocardiology
Konrad Paczkowski, Ireneusz Haponiuk, Maciej Chojnicki, Radosław Jaworski
We present a case of a 2.5-year-old-girl with complex congenital heart disease: tricuspid atresia (TA), bulboventricular septal defect (VSD), hypoplastic right ventricle, d-transposition of the great arteries (d-TGA) with aortic outflow from redundant RV. Due to II/III degree atrioventricular block induced after diagnostic cardiac catheterization, an epicardial pacemaker was implanted during the Glenn procedure. Because of severe left ventricle outflow tract obstruction, she was finally referred for extracardiac TCPC (extracardiac Fontan type) with recruitment of PV and Damus-Kaye-Stansel anastomosis...
2016: Heart Surgery Forum
Keisuke Nakanishi, Ken Takahashi, Shiori Kawasaki, Hideo Fukunaga, Atsushi Amano
Pacemaker implantation in infants during the early postnatal period is difficult because of their small body size and susceptibility to infection. We describe the successful pacemaker implantation for complete heart block in an infant weighing 803 g.
October 2016: Journal of Cardiac Surgery
J C Galán Gutiérrez, F E Fernández Suárez, P Miranda García, L A Sopena Zubiria
Eisenmenger syndrome (ES) is a complex combination of cardiovascular abnormalities defined as pulmonary hypertension with investment or bidirectional flow through an intracardiac or aortopulmonary communication, usually secondary to a congenital heart disease not resolved promptly. It carries a significant risk of perioperative mortality, with an incidence close to 30% for non-cardiac surgery. We report the anaesthetic management in a ES patient undergoing breast surgery, which was successfully performed under general anaesthesia combined with thoracic analgesic blocks...
August 20, 2016: Revista Española de Anestesiología y Reanimación
Ariela Hoxha, Amelia Ruffatti, Aurelie Ambrosi, Vijole Ottosson, Malin Hedlund, Lars Ottosson, Madhanagopal Anandapadmanaban, Maria Sunnerhagen, Sven-Erik Sonesson, Marie Wahren-Herlenius
Congenital heart block (CHB) is a potentially lethal condition characterized by a third-degree atrioventricular block (AVB). Despite anti-Ro52 antibodies being detected in nearly 90% of mothers of affected children, CHB occurs in only 1-2% of anti-Ro/SSA autoantibody-positive pregnancies. Maternal antibodies have been suggested to bind molecules crucial to fetal cardiac function, however it remains unknown whether a single antibody profile associates with CHB or whether several specificities and cross-reactive targets exist...
August 22, 2016: Clinical and Experimental Immunology
Hiroko Asakai, Sofia Weskamp, Lucas Eastaugh, Yves d'Udekem, Andreas Pflaumer
OBJECTIVE: Secundum atrial septal defect (ASD) is a common congenital heart defect. There is limited data on both early and late atrioventricular (AV) block post ASD closure. The aim of this study was to determine the incidence and risk factors of AV block associated with ASD closure. METHODS: A retrospective analysis of all patients who underwent ASD closure either with a device or surgical method at the Royal Children's Hospital Melbourne between 1996 and 2010 was performed...
2016: Heart Asia
Cici Chenliu, Xiaoye Sheng, Pauline Dan, Yang Qu, Victoria Claydon, Eric Lin, Leif Hove-Madsen, Shubhayan Sanatani, Glen F Tibbits
BACKGROUND: Post-operative arrhythmias such as Junctional Ectopic Tachycardia (JET) and atrioventricular block (AVB) are serious post-operative complications for children with congenital heart disease. We hypothesize that ischemia reperfusion (I/R) related changes exacerbate these post-operative arrhythmias in the neonate heart and the administration of post-operative inotropes is contributory. OBJECTIVE: To study the effects of I/R and post-ischemic dopamine (DA) application on the automaticity and rhythmicity in immature and mature pacemaker cells and whole heart preparations...
July 20, 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
Todd S Roth, Jamil A Aboulhosn
Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is a frequent complication in adults with congenital heart disease. Regardless of etiology, the optimal treatment strategy for this difficult population is challenging. The new frontier of targeted PAH therapies has demonstrated improved functional capacity in the various phenotypes of PAH-CHD, with work currently in progress scrutinizing outcomes. In those who fail conventional medical therapy, heart and heart-lung (block) transplantation become the final therapeutic options, with the role of ventricular assist devices and the total artificial heart still under investigation in this group...
August 2016: Cardiology Clinics
Gautam Rawal, Raj Kumar, Sankalp Yadav, Deepak Verma
Persistent Left Superior Vena Cava (PLSVC) is a rare congenital vascular anomaly (incidence of 0.3-0.5% of the general population) which being mostly asymptomatic in its presentation, is usually detected incidentally. There are many practical clinical implications associated with it including arrhythmias. We report a rare case of PLSVC with absent Right Superior Vena Cava (RSVC) (isolated PLSVC), in a 55-year-old lady who had complete heart block followed by sepsis and was diagnosed to have this condition during the permanent cardiac pacemaker implantation and central venous catheter insertion showing an abnormal path of the catheter/pacing leads...
May 2016: Journal of Clinical and Diagnostic Research: JCDR
Anum Fayyaz, Biji T Kurien, R Hal Scofield
We compiled information on antibodies in Sjögren syndrome, focusing more on clinical manifestations associated with anti-Ro/SSA and anti-La/SSB antibodies and studies regarding novel antibodies. We reviewed previous as well as most recent studies with the subject heading Sjogren in combination with antibodies and congenital heart block (CHB). Almost half of asymptomatic mothers giving birth to children with CHB ultimately develop Sjögren. We discussed studies concerning the presence of antibodies predating clinical manifestations of disease...
August 2016: Rheumatic Diseases Clinics of North America
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"