- Isolated congenital complete heart block in a five-year-old seronegative girl born to a woman seropositive for human immunodeficiency virus: a case report. [Journal Article]
- JMJ Med Case Rep 2016 Oct 19; 10(1):288
- CONCLUSIONS: Despite its infrequency and life-threatening potential, patients with congenital complete heart block have an excellent survival rate with timely diagnosis and intervention. An incidental detection of bradycardia in a fetus during routine obstetrical ultrasound examination should increase the index of suspicion for congenital complete heart block and warrant a screening for associated maternal autoantibodies.
- Paroxysmal atrioventricular block after heart transplantation in children: an early sign of rejection? [Case Reports]
- PTPediatr Transplant 2016 Oct 14
- In OHT recipients, pathologic evaluation of the heart during acute rejection can show involvement of both the conduction system and the myocardium. We here describe the cases of a 9-year-old male wit...
In OHT recipients, pathologic evaluation of the heart during acute rejection can show involvement of both the conduction system and the myocardium. We here describe the cases of a 9-year-old male with DCM and a 13-year-old female with RCM, who developed third-degree PAVB associated with acute rejection 36 months and 24 months after OHT, respectively. We conclude that PAVB could be considered an early sign of acute rejection after OHT in children who exhibit post-transplantation presyncope or syncope.
- Cryoablation of atrioventricular nodal re-entrant tachycardia: 7-year follow-up in 515 patients-confirmed safety but very late recurrences occur. [Journal Article]
- EEuropace 2016 Oct 13
- CONCLUSIONS: Cryoablation in AVNRT is safe with a long-term efficacy of 88%; however, very late recurrences occur.
- Figure incorrect. [Journal Article]
- NSNurs Stand 2001 Jun 6; 15(38):31
- Figure 10 published in 'Myocardial infarction: part 2' (art&science May 30) should have shown Mobitz Type II block, not third-degree block.
Figure 10 published in 'Myocardial infarction: part 2' (art&science May 30) should have shown Mobitz Type II block, not third-degree block.
- Life-threatening cardiac episode in a Polish patient carrying contiguous gene microdeletion of the TBX5 and the TBX3 genes. [Journal Article]
- SSpringerplus 2016; 5(1):1638
- 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 l...
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. At the age of 13 years he lost consciousness developing respiratory insufficiency caused by bradycardia in the course of sudden atrioventricular third degree heart block requiring immediate implantation of pace maker-defibrillator device. Microdeletion of the 12q24.21 was identified using array CGH method. This region includes contiguous genes the TBX5, TBX3, and part of RBM19. The patient initially diagnosed as having HOS, was found to present the UMS features as well. Array CGH method should be applied in patients suspected of HOS or UMS, especially when sequencing of TBX5 or TBX3 genes fails to identify causative mutation.
- Concomitant presentation of Anderson-Tawil syndrome and myasthenia gravis in an adult patient: A case report. [Journal Article]
- ETExp Ther Med 2016; 12(4):2435-2438
- Andersen-Tawil syndrome (ATS) is an autosomal dominant, multisystem channelopathy characterized by periodic paralysis, ventricular arrhythmias and distinctive dysmorphic facial or skeletal features. ...
Andersen-Tawil syndrome (ATS) is an autosomal dominant, multisystem channelopathy characterized by periodic paralysis, ventricular arrhythmias and distinctive dysmorphic facial or skeletal features. The disorder displays marked intrafamilial variability and incomplete penetrance. Myasthenia gravis (MG) is an autoimmune disorder that demonstrates progressive fatigability, in which the nicotinic acetylcholine receptor (AChR) at neuromuscular junctions is the primary autoantigen. The present study reports a rare case of a 31-year-old woman with a history of morbid obesity and periodic weakness, who presented with hemodynamic instability, cardiogenic shock and facial anomalies. Laboratory results revealed hypokalemia and an elevated anti-AChR antibody expression levels. Electrocardiography demonstrated prolonged QT-interval, ST-elevation, and subsequent third-degree atrioventricular block. Neurological examination revealed bilateral ptosis, horizontal diplopia, dysarthria and generalized weakness. No mutations in the potassium channel inwardly rectifying subfamily J member 2 gene were detected in the present case. The patient was treated with oral potassium supplementation and an acetylcholinesterase inhibitor (pyridostigmine), after which the symptoms were improved. To the best of our knowledge, the present case report was the first to describe concomitant presentation of both ATS and MG, which represents a diagnostic and therapeutic challenge.
- Traumatic Gerbode ventricular septal defect and third-degree heart block. [Journal Article]
- CYCardiol Young 2016 Sep 19; :1-3
- We present an interesting and rare case of traumatic Gerbode ventricular septal defect and complete heart block. The multimodality images illustrate the diagnosis well. This case is an excellent demo...
We present an interesting and rare case of traumatic Gerbode ventricular septal defect and complete heart block. The multimodality images illustrate the diagnosis well. This case is an excellent demonstration of the diagnostic utility of multimodality imaging.
- Complete heart block in pregnancy: case report, analysis, and review of anesthetic management. [Journal Article]
- JCJ Clin Anesth 2016; 33:58-61
- Maternal complete heart block can pose significant challenges for the anesthesiologist in the antepartum, peripartum, and postpartum periods. Some patients may present for the first time in the puerp...
Maternal complete heart block can pose significant challenges for the anesthesiologist in the antepartum, peripartum, and postpartum periods. Some patients may present for the first time in the puerperium with dizziness, weakness, syncope, or congestive heart failure as a result of the additional hemodynamic burden that accompanies pregnancy. Although there is an increase in permanent pacemaker placement in young symptomatic patients before pregnancy, prophylactic placement of pacemakers in asymptomatic parturients is not always indicated. The need for temporary or permanent pacemakers in asymptomatic women should be assessed on a case-by-case basis; many of these patients may be safely managed during labor and delivery without pacing. The parturient with complete heart block must be followed vigilantly during pregnancy and post delivery, as the need for pacemaker insertion can also arise in the postpartum period. We present a case of third-degree heart block in a 26-year-old parturient.
- Identification of discrete epitopes of Ro52p200 and association with fetal cardiac conduction system manifestations in a rodent model. [Journal Article]
- CEClin Exp Immunol 2016 Aug 22
- 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 af...
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/Sjögren's-syndrome-related antigen A (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. Here, we aimed to define further the reactivity profile of CHB-associated antibodies towards Ro52p200 (amino acid 200-239). We first analysed reactivity of a monoclonal anti-Ro52 antibody shown to induce AVB in rats (7.8C7) and of sera from anti-Ro52p200 antibody-positive mothers of children with CHB towards a panel of modified Ro52p200 peptides, and subsequently evaluated their potential to induce AVB in rats upon transfer during gestation. We observed that CHB maternal sera displayed a homogeneous reactivity profile targeting preferentially the C-terminal part of Ro52p200, in contrast to 7.8C7 that specifically bound the p200 N-terminal end. In particular, amino acid D233 appeared crucial to maternal antibody reactivity towards p200. Despite low to absent reactivity towards rat p200 and different binding profiles towards mutated rat peptides indicating recognition of different epitopes within Ro52p200, immunoglobulin (Ig)G purified from two mothers of children with CHB could induce AVB in rats. Our findings support the hypothesis that several fine antibody specificities and cross-targets may exist and contribute to CHB development in anti-Ro52 antibody-positive pregnancies.
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- Transfemoral aortic valve implantation with the repositionable Lotus valve for treatment of patients with symptomatic severe aortic stenosis: results from a single-centre experience. [Journal Article]
- EEuroIntervention 2016 Aug 20; 12(6):760-7
- CONCLUSIONS: In patients with severe aortic stenosis, transfemoral TAVI with the repositionable Lotus valve was associated with a high rate of device success, no moderate or severe residual aortic regurgitation, low rates of major vascular complications and mortality within 30 days.