- Nonreentrant atrial tachycardia occurs independently of hypertrophic cardiomyopathy in RASopathy patients. [Journal Article]
- AJAm J Med Genet A 2018 Jul 28
- Multifocal atrial tachycardia (MAT) has a well-known association with Costello syndrome, but is rarely described with related RAS/MAPK pathway disorders (RASopathies). We report 11 patients with RASo...
Multifocal atrial tachycardia (MAT) has a well-known association with Costello syndrome, but is rarely described with related RAS/MAPK pathway disorders (RASopathies). We report 11 patients with RASopathies (Costello, Noonan, and Noonan syndrome with multiple lentigines [formerly LEOPARD syndrome]) and nonreentrant atrial tachycardias (MAT and ectopic atrial tachycardia) demonstrating overlap in cardiac arrhythmia phenotype. Similar overlap is seen in RASopathies with respect to skeletal, musculoskeletal and cutaneous abnormalities, dysmorphic facial features, and neurodevelopmental deficits. Nonreentrant atrial tachycardias may cause cardiac compromise if sinus rhythm is not restored expeditiously. Typical first-line supraventricular tachycardia anti-arrhythmics (propranolol and digoxin) were generally not effective in restoring or maintaining sinus rhythm in this cohort, while flecainide or amiodarone alone or in concert with propranolol were effective anti-arrhythmic agents for acute and chronic use. Atrial tachycardia resolved in all patients. However, a 4-month-old boy from the cohort was found asystolic (with concurrent cellulitis) and a second patient underwent cardiac transplant for heart failure complicated by recalcitrant atrial arrhythmia. While propranolol alone frequently failed to convert or maintain sinus rhythm, fleccainide or amiodarone, occasionally in combination with propranolol, was effective for RASopathy patient treatment for nonreentrant atrial arrhythmia. Our analysis shows that RASopathy patients may have nonreentrant atrial tachycardia with and without associated cardiac hypertrophy. While nonreentrant arrhythmia has been traditionally associated with Costello syndrome, this work provides an expanded view of RASopathy cardiac arrhythmia phenotype as we demonstrate mutant proteins throughout this signaling pathway can also give rise to ectopic and/or MAT.
- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Atrioventricular node (AV) measures approximately 1 by 3 by 5 millimeters and sits within an anatomic region bordered anteriorly by septal tricuspid valve annulus, superiorly by the tendon of Todaro...
Atrioventricular node (AV) measures approximately 1 by 3 by 5 millimeters and sits within an anatomic region bordered anteriorly by septal tricuspid valve annulus, superiorly by the tendon of Todaro and posteriorly by the coronary sinus ostium. This anatomic region is also commonly referred to as triangle of Koch. AV node is a subendocardial structure located in the inferior-posterior right atrium. The blood supply to the AV node is from the AV nodal branch of the right coronary artery (90%) or the left circumflex artery (10%) depending on right or left dominant blood supply to the heart. The first septal perforator of the left anterior descending artery also supplies blood to the AV node. Paroxysmal supraventricular tachycardia (PSVT) accounts for intermittent episodes of supraventricular tachycardia with sudden onset and termination. PSVT is part of the narrow QRS complex tachycardias with a regular ventricular response in contrast to multifocal atrial tachycardia, atrial fibrillation, and atrial flutter. SVTs are classified based on the origin of the rhythm and whether the rhythm is regular or irregular. Atrial in origin and regular rhythm: Sinus tachycardia. Inappropriate sinus tachycardia. Sinoatrial nodal reentrant tachycardia. Atrial flutter. Atrial fibrillation. Atrial in origin and irregular rhythm: Multifocal atrial tachycardia. Atrial flutter with variable block. Atrial fibrillation. AV node in origin and regular rhythm: Junctional tachycardia Atrioventricular nodal reentrant tachycardia. Atrioventricular reentrant tachycardia. AV node in origin and irregular rhythm: None.
- Clinical Implication of Multifocal Atrial Tachycardia in Children for Pediatric Cardiologist. [Editorial]
- KCKorean Circ J 2018; 48(2):173-175
- The Complexity of Pediatric Multifocal Atrial Tachycardia and Its Prognostic Factors. [Journal Article]
- KCKorean Circ J 2018; 48(2):148-158
- CONCLUSIONS: MAT usually affects infants and has a favorable prognosis, particularly in the idiopathic infant group. However, in the presence of other comorbidities, MAT may have a variable clinical course.
- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Multifocal atrial tachycardia is a supraventricular tachycardia with a rapid, irregular atrial rhythm arising from multiple ectopic foci within the atria. This arrhythmia is characterized by a heart ...
Multifocal atrial tachycardia is a supraventricular tachycardia with a rapid, irregular atrial rhythm arising from multiple ectopic foci within the atria. This arrhythmia is characterized by a heart rate of greater than 100 beats per minute with organized atrial activity yielding three or more different non-sinus P-wave morphologies in the same lead. There are irregular PP intervals and an isoelectric baseline between P waves (Figure 1). This condition is typically seen in elderly patients with a variety of underlying conditions, the most common of which is a chronic obstructive pulmonary disease (COPD). While the pathogenesis is not well understood, it is generally asymptomatic, and most patients are hemodynamically stable. For the majority of patients, no treatment is required beyond treatment of underlying conditions. However, evaluation is important as this arrhythmia is a poor prognostic sign in the setting of acute illness.
- A hol(e)y predicament. [Journal Article]
- RCRespirol Case Rep 2017; 5(4):e00237
- Endocardial cushion defects are congenital abnormalities that result in valvular dysfunction as well as defects (or "holes") in the septa of the heart. They are typically diagnosed in early infancy; ...
Endocardial cushion defects are congenital abnormalities that result in valvular dysfunction as well as defects (or "holes") in the septa of the heart. They are typically diagnosed in early infancy; presentation late in life is rare. We present the case of a 72-year-old female admitted to the hospital with dyspnoea and palpitations. She was found to have multifocal atrial tachycardia. She suffered cardiac arrest associated with refractory hypoxaemia that required mechanical ventilation and vasodilator therapy with inhaled nitric oxide. Echocardiography revealed a large ostium primum atrial septal defect (ASD) complicated by Eisenmenger syndrome. It is likely that her arrhythmia, a sequela from her long-standing congenital abnormality, led to sudden decompensation. In this case presentation, we review the aetiology, presentation, and complications of ASDs.
- Nationwide experience of catecholaminergic polymorphic ventricular tachycardia caused by RyR2 mutations. [Multicenter Study]
- HHeart 2017; 103(12):901-909
- CONCLUSIONS: In a national cohort of RyR2 mutation-positive CPVT patients, SCD, ASCD and syncope were presenting events in the majority of probands and also occurred in 36% of relatives identified through family screening. Probands were younger at disease onset and more prone to fatal or near-fatal events than relatives.
- [Perinatal Presentation and Complicated Course of a Multifocal Atrial Tachycardia]. [Case Reports]
- ZGZ Geburtshilfe Neonatol 2016; 220(6):265-268
- We report a male newborn who became symptomatic with supraventricular tachycardia on the first day of life. Neither adenosine nor electric cardioversion could terminate the tachycardia, therefore int...
We report a male newborn who became symptomatic with supraventricular tachycardia on the first day of life. Neither adenosine nor electric cardioversion could terminate the tachycardia, therefore intravenous esmolol (β-receptor blocker) was initiated. Inspite of subsequent administration of various antiarrhythmic medications in increasingly higher doses, repeated supraventricular tachycardic episodes occurred. The electrocardiogram showed typical findings of a multifocal atrial tachycardia as the underlying cause. When tachycardic episodes occurred, they also presented as atrial flutter at 460 bpm and a 2:1 block. Finally, high dosage of amiodarone (10 mg/kgbw/d) led to continuous control of the heart rate without tachycardic episodes. To date our patient is mostly in sinus rhythm but without tachycardic episodes and doing well.
- Dofetilide in Overdose: A Case Series from Poison Center Data. [Journal Article]
- CTCardiovasc Toxicol 2017; 17(3):368-371
- Dofetilide is a class III antiarrhythmic used for treating atrial dysrhythmias. Though its adverse effects are well described in routine use, very little is known about dofetilide toxicity in overdos...
Dofetilide is a class III antiarrhythmic used for treating atrial dysrhythmias. Though its adverse effects are well described in routine use, very little is known about dofetilide toxicity in overdose. This is a retrospective case series of consecutive patients reported to our poison center after dofetilide overdose. Twenty-seven cases were included. Seventeen patients were treated at a healthcare facility, and of these, eight were admitted. Twenty-one patients took one extra capsule, four took someone else's medication, one took three extra capsules, and one had a large intentional overdose. Ten patients had co-ingestants reported, including three QT-prolonging agents. No one required cardioversion, defibrillation, CPR, or overdrive pacing. The patient who reported taking 90 times his usual dose in suicide attempt was the only patient to have significant clinical effects. He experienced an 8-beat run of non-sustained ventricular tachycardia, frequent multifocal PVCs, and ventricular bigeminy. He received magnesium sulfate and potassium chloride supplementation. In this series, unintentional small overdoses did not result in significant clinical effects and were often managed successfully at home, despite the fact that information showing a single capsule can cause torsades. This study is limited by its small sample size, retrospective design, and reliance on incomplete information.
New Search Next
- Lipomatous hypertrophy of the interatrial septum in a child with atrial tachycardia. [Case Reports]
- PIPediatr Int 2016; 58(6):523-525
- A 13-year-old girl who had been on home parenteral nutrition for 6 months has been presented with multifocal atrial tachycardia and atrial fibrillation. Echocardiography and multislice computed tomog...
A 13-year-old girl who had been on home parenteral nutrition for 6 months has been presented with multifocal atrial tachycardia and atrial fibrillation. Echocardiography and multislice computed tomography showed fat accumulation on the interatrial septum. Lipomatous hypertrophy of the interatrial septum has never been reported in children.