scholarly journals Naxos disease patient with sustained ventricular tachycardia and multifocal thrombi in the right ventricle: Concerns on therapeutic management

Author(s):  
Eirini Dri ◽  
Ioannis Leontsinis ◽  
Eleftherios Tsiamis ◽  
Polychronis Dilaveris ◽  
Georgios Latsios ◽  
...  
2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Padmanabhan

Abstract OnBehalf Cornwall A term neonate with poor condition at birth was noted to be markedly bradycardic when crying. ECG showed first degree heart block. Echocardiogram demonstrated poor bi-ventricular function. He was treated for Hypoxic ischemic encephalopathy (HIE). In the next few hours he developed short episodes of ventricular tachycardia with left bundle branch block and non-conducted P waves suggesting an origin of tachycardia from the right ventricle. His repeat ECHO suggested that he had right ventricular infarction due to a very rare finding of intermittent occlusion of the origin of the right coronary artery by an echogenic mass. He was transferred for emergency cardiac surgery to remove a clot of 1.6cm, occluding the right coronary artery. Histology findings were in keeping with a thrombus. Thrombophilia screen for both parents and infant were negative. MRI Brain did not show evidence of HIE suggesting his poor condition at birth was secondary to intermittent coronary ischemia. Discussion: Myocardial infarction (MI) in neonates is a rarely encountered and potentially life-threatening condition, with mortality rate as high as 90%. We present one of the first reported cases of successful surgical management of an acute right coronary artery thrombosis after an early diagnosis. The cause of thrombosis remains unclear in our patient. They were born in poor condition with initial pH 6.9 and lactate of 10, but with a structurally normal heart and negative thrombophilia screen. Perinatal asphyxia is a potential cause; however there is doubt that this may be a symptom rather than cause of the right coronary artery occlusion. Early diagnosis was key in management after a high level of clinical suspicion. He made significant recovery with near normal RV function, and is currently on captopril and carvedilol post-surgery. This is one of the first cases to document near full return of cardiac function following ischaemia to the right ventricle Abstract 478 Figure. 5


2020 ◽  
Vol 8 (4) ◽  
pp. 294-299
Author(s):  
Megan Barber ◽  
Jason Chinitz ◽  
Roy John

The moderator band in the right ventricle is being increasingly recognised as a source for arrhythmias in the absence of identifiable structural heart disease. Because it carries part of the conduction system from the right ventricle septum to the free wall, it is a source of Purkinje-mediated ventricular arrhythmias that manifest as premature ventricular contractions (PVC) or repetitive ventricular tachycardia. More importantly, short coupled PVCs triggering polymorphic ventricular tachycardia and VF have been localised to the moderator band and ablation of these Purkinje mediated PVCs can effectively prevent recurrent VF. The exact mechanism of arrhythmogenesis is still debated but stretch, fibrosis and ion channel alterations might be responsible. Arrhythmias originating in this region of the right ventricle may thus be another cause for idiopathic VF that is potentially treatable with catheter-based ablation techniques. Recognition of the typical PVC morphology can point to the moderator band as the source of idiopathic VF and an opportunity for timely intervention. The available data on the anatomy, electrophysiology and management options are reviewed.


1996 ◽  
Vol 19 (5) ◽  
pp. 868-871 ◽  
Author(s):  
MUHARREM GULDAL ◽  
REMZI KARAOGUZ ◽  
ANTONIO PACIFICO ◽  
GERALD M. LAWRIE ◽  
TURHAN AKYOL

1989 ◽  
Vol 3 (2) ◽  
pp. 117-126 ◽  
Author(s):  
TERESA KUS ◽  
MARTIN FROMER ◽  
MARC DUBUC ◽  
MOHAMMAD SHENASA

2014 ◽  
Vol 26 (2) ◽  
pp. 145-150 ◽  
Author(s):  
FRANCESCO SANTORO ◽  
LUIGI DI BIASE ◽  
PATRICK HRANITZKY ◽  
JAVIER E. SANCHEZ ◽  
PASQUALE SANTANGELI ◽  
...  

2015 ◽  
Vol 26 (4) ◽  
pp. 764-771 ◽  
Author(s):  
Lin Wu ◽  
Hong Tian ◽  
Feng Wang ◽  
Xuecun Liang ◽  
Gang Chen

AbstractObjectiveMost idiopathic right ventricular tachycardias originate from the outflow tract. We present a case series of idiopathic incessant ventricular tachycardia arising from unusual sites of the right ventricle in children, which were well resolved by catheter ablation.MethodsA retrospective review was performed of all three patients who underwent ablation of idiopathic ventricular tachycardia below the level of the right ventricular outflow tract using three-dimensional mapping in our institute.ResultAll three patients presented with tachycardia-induced cardiomyopathy due to incessant ventricular tachycardia on first admission. The sites of successful ablation were at the proximal right bundle branch, distal right bundle branch, and apex of the right ventricle, respectively. No complications occurred, and there has been no recurrence of ventricular tachycardia after the final ablation at an average follow-up period of 9 months. All three patients have achieved normalisation of left ventricular size and systolic function.ConclusionIncessant idiopathic ventricular tachycardia originating from unusual sites of the right ventricle in children, resulting in significant symptoms and impaired ventricular function, can be successfully treated with catheter ablation.


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