-
European Heart Journal. Case Reports Jul 2022Implantable cardioverter defibrillators (ICDs) are most effective in treating sudden cardiac death. However, accurate diagnostic workup of broad complex tachycardia is...
BACKGROUND
Implantable cardioverter defibrillators (ICDs) are most effective in treating sudden cardiac death. However, accurate diagnostic workup of broad complex tachycardia is crucial to ensure correct indication for ICD treatment and to avoid unnecessary invasive treatment and device-associated morbidity.
CASE SUMMARY
We present a case of atypical atrial flutter with 2:1 atrioventricular (AV) conduction via a left-posterior accessory pathway (AP), leading to the diagnosis of Wolff-Parkinson-White (WPW) syndrome. Upon admission, the 72-year-old patient showed a regular broad complex tachycardia with superior axis and positive concordance in precordial leads, suggestive of either ventricular tachycardia (VT), antidromic AV re-entrant tachycardia (AVRT), or supraventricular tachycardia with antegrade conduction via a left-posterior AP. Interrogation of the two-chamber ICD, which was very likely implanted unjustified in a peripheral clinic before, revealed atrial flutter with 2:1 AV conduction. Remarkably, after the restoration of sinus rhythm, no classic echocardiogram (ECG) criteria for preexcitation syndrome were detected. An invasive electrophysiological study proved the diagnosis of a bidirectionally conducting, left-posterior AP, which was successfully ablated.
DISCUSSION
Differential diagnosis of broad complex tachycardia with superior axis and positive concordance of chest leads consists of i) VT with a left ventricular exit at the posterior mitral annulus, ii) antidromic AVRT involving a left-posterior AP, and iii) supraventricular tachycardia predominantly conducted via a left-posterior AP. The absence of classic ECG criteria for preexcitation syndrome does not rule out AP sufficiently, highlighting the importance of minimal surface-ECG preexcitation criteria. In the case of detection of minimal surface-ECG preexcitation criteria, administration of adenosine rules out or proves the existence of an AP noninvasively and cost-effectively.
PubMed: 35821973
DOI: 10.1093/ehjcr/ytac250 -
Herzschrittmachertherapie &... Sep 2022MELAS syndrome is defined as a combination of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes resulting from mutations in mitochondrial...
MELAS syndrome is defined as a combination of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes resulting from mutations in mitochondrial DNA. All medical interventions in these patients appear challenging due to a high risk of lactate acidosis or anesthesiological complications. Of note, previous reports suggest that these patients have a higher incidence of Wolff-Parkinson-White (WPW) syndrome. Here, a case of successful catheter ablation of a posteroseptal bypass tract using analgosedation in a patient with MELAS syndrome combined with WPW syndrome is presented.
Topics: Catheter Ablation; Humans; MELAS Syndrome; Wolff-Parkinson-White Syndrome
PubMed: 35804205
DOI: 10.1007/s00399-022-00881-9 -
Journal of Cardiovascular... Aug 2022The notion that medically-refractory arrhythmias might one day be amenable to interventional therapy slowly began to appear in the early 1960's. At that time, there were... (Review)
Review
INTRODUCTION
The notion that medically-refractory arrhythmias might one day be amenable to interventional therapy slowly began to appear in the early 1960's. At that time, there were no "interventional electrophysiologists" or "arrhythmia surgeons" and there was little appreciation of the relationship between anatomy and electrophysiology outside the heart's specialized conduction system.
METHODS
In this review, we describe the evolution of collaboration between electrophysiologists and surgeons.
RESULTS
Although accessory atrio-ventricular (AV) connections were first identified in 1893 and the Wolff-Parkinson-White (WPW) syndrome was described 37 years later (1930), it was another 37 years (1967) before those anatomic AV connections were proven to be responsible for the clinical syndrome. The success of the subsequent surgical procedures for the WPW syndrome, AV node reentry tachycardia, automatic atrial tachycardias, ischemic and non-ischemic ventricular tachycardias and atrial fibrillation over the next two decades depended on a close, sometimes daily, collaboration between electrophysiologists and surgeons. In the past two decades, that tight collaboration was largely abandoned until the recent introduction of "hybrid procedures" for the treatment of atrial fibrillation.
CONCLUSIONS
A retrospective assessment of the 50 years of interventional therapy for arrhythmias clearly demonstrates the clinical benefits of a close collaboration between electrophysiologists and arrhythmia surgeons, regardless of which one is actually performing the intervention.
Topics: Atrial Fibrillation; Humans; Retrospective Studies; Surgeons; Tachycardia, Atrioventricular Nodal Reentry; Wolff-Parkinson-White Syndrome
PubMed: 35695795
DOI: 10.1111/jce.15598 -
Cardiology Journal 2022
Topics: Accessory Atrioventricular Bundle; Catheter Ablation; Child; Electrocardiography; Humans; Physical Functional Performance; Pilot Projects; Pre-Excitation Syndromes; Wolff-Parkinson-White Syndrome
PubMed: 35621093
DOI: 10.5603/CJ.a2022.0027 -
Journal of Interventional Cardiac... Oct 2022Patients with WPW syndrome have an increased mortality rate compared to the general population. Although asymptomatic preexcitation has previously been considered...
PURPOSE
Patients with WPW syndrome have an increased mortality rate compared to the general population. Although asymptomatic preexcitation has previously been considered benign, recent studies have found that also asymptomatic patients have clinical and electrophysiological factors associated with increased risk of sudden cardiac death. This study compares the baseline electrophysiological characteristics of accessory pathways in symptomatic and asymptomatic patients with preexcitation. We hypothesized that a significant proportion of asymptomatic patients has inducible orthodromic tachycardia during programmed electrical stimulation.
METHODS
This retrospective study includes 1853 patients with preexcitation who underwent invasive electrophysiological testing in two Swedish University Hospitals between 1991 and 2018. The mean age was 36 ± 17 years with a range of 3-89 years. Thirty-nine percent was women. A total of 269 patients (15%) were children younger than 18 years. Electrophysiological data included effective refractory period of the accessory pathway (APERP, in 1069 patients), tachycardia cycle length, inducibility and type of tachycardia, and AP localization.
RESULTS
A total of 1703 (93%) patients reported symptoms suggesting tachyarrhythmias before the study and 128 (7%) were asymptomatic. The proportion of potentially dangerous pathways with short APERP (≤ 250 ms) were similar in symptomatic and asymptomatic patients (187/949, 20% vs. 25/108, 23%) (P = 0.40) as was the mean APERP (303 ± 68 ms vs. 307 ± 75) (P = 0.61). The proportion of patients who had inducible arrhythmia was larger in the symptomatic group (64% vs. 31%) (P < 0.001).
CONCLUSION
The results of this study strengthen the present guideline recommendation (IIA) to consider invasive risk assessment in patients with asymptomatic preexcitation.
Topics: Accessory Atrioventricular Bundle; Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Electrocardiography; Female; Humans; Middle Aged; Pre-Excitation Syndromes; Retrospective Studies; Tachycardia; Wolff-Parkinson-White Syndrome; Young Adult
PubMed: 35618980
DOI: 10.1007/s10840-022-01252-7 -
Molecular Genetics & Genomic Medicine Jul 2022PRKAG2 cardiac syndrome is a rare autosomal dominant genetic disorder caused by a PRKAG2 gene variant. There are several major adverse cardiac presentations, including... (Review)
Review
BACKGROUND
PRKAG2 cardiac syndrome is a rare autosomal dominant genetic disorder caused by a PRKAG2 gene variant. There are several major adverse cardiac presentations, including hypertrophic cardiomyopathy (HCM) and life-threatening arrhythmia. Two cases with pathogenic variants in the PRKAG2 gene are reported here who presents different cardiac phenotypes.
METHODS
Exome sequencing and variant analysis of PRKAG2 were performed to obtain genetic data, and clinical characteristics were determined.
RESULTS
The first proband was a 9-month-old female infant (Case 1), and was identified with severe DCM and resistant heart failure. The second proband was a 10-year-old female infant (Case 2), and presented with HCM and ventricular preexcitation. Exome sequencing identified a de novo c.425C > T (p.T142I) heterozygous variant in the PRKAG2 gene for Case 1, and a c.869A > T (p.K290I) for Case 2. The mutated sites in the protein were labeled and identified as p.K290 in the CBS domain and p.T142 in the non-CBS domain. Differences in the molecular functions of CBS and non-CBS domains have not been resolved, and variants might lead to the different cardiomyopathy phenotypes. Single-cell RNA analysis demonstrated similar expression levels of PRKAG2 in cardiomyocytes and conductive tissues. These results suggest that the arrhythmia induced by the PRKAG2 variant was the primary change, and not secondary to cardiomyopathy.
CONCLUSION
In summary, this is the first case report to describe a DCM phenotype with early onset in patients possessing a PRKAG2 c.425C > T (p.T142I) pathogenic variant. Our results aid in understanding the molecular function of non-CBS variants in terms of the disordered sequence of transcripts. Moreover, we used scRNA-seq to show that electrically conductive cells express a higher level of PRKAG2 than do cardiomyocytes. Therefore, variants in PRKAG2 are expected to also alter the biological function of the conduction system.
Topics: AMP-Activated Protein Kinases; Arrhythmias, Cardiac; Cardiomyopathies; Cardiomyopathy, Hypertrophic; Female; Humans; Myocytes, Cardiac
PubMed: 35588295
DOI: 10.1002/mgg3.1962 -
Cardiovascular Ultrasound May 2022PRKAG2 syndrome is a rare disease characterized as left ventricular hypertrophy (LVH), ventricular preexcitation syndrome, and sudden cardiac death. Its natural course,...
Echocardiographic characteristics of PRKAG2 syndrome: a research using three-dimensional speckle tracking echocardiography compared with sarcomeric hypertrophic cardiomyopathy.
BACKGROUND
PRKAG2 syndrome is a rare disease characterized as left ventricular hypertrophy (LVH), ventricular preexcitation syndrome, and sudden cardiac death. Its natural course, treatment, and prognosis were significantly different from sarcomeric hypertrophic cardiomyopathy (HCM). However, it is often clinically misdiagnosed as sarcomeric HCM. PRKAG2 patients tend to experience delayed treatment. The delay may lead to adverse outcomes. This study aimed to identify the echocardiographic parameters which can differentiate PRKAG2 syndrome from sarcomeric HCM.
METHODS
Nine PRKAG2 patients with LVH, 41 HCM patients with sarcomere gene mutations, and 202 healthy volunteers were enrolled. Clinical characteristics, conventional echocardiography, and three-dimensional images were recorded, and reviewed by an attending cardiologist. We evaluated the parameters of left ventricular strains from three-dimensional speckle tracking echocardiography (3D STE) by TomTec software. Receiver operating characteristic (ROC) curves analysis was used to assess clinical and echocardiographic parameters' differential diagnosis potential.
RESULTS
The heart rate (HR) of the PRKAG2 group was significantly lower than both the healthy group (53.11 ± 10.14 vs. 69.22 ± 10.48 bpm, P < 0.001) and the sarcomeric HCM group (53.11 ± 10.14 vs. 67.23 ± 10.32 bpm, P = 0.001). The PRKAG2 group had similar interventricular septal thickness (IVS), posterior wall thickness (PWT), and maximum wall thickness (MWT) to the HCM group (P > 0.05). The absolute value of GLS in the PRKAG2 group was significantly higher than HCM patients (-18.92 ± 4.98 vs. -13.43 ± 4.30%, P = 0.004). SV calculated from EDV and ESV in PRKAG2 syndrome showed a higher value than sarcomeric HCM (61.83 ± 13.52 vs. 44.96 ± 17.53%, P = 0.020). The area under the ROC curve (AUC) for HR + GLS was 0.911 (0.803 -1). For HR + GLS, the sensitivity and specificity of the best cut-off value (0.114) were 69.0% and 100%, respectively.
CONCLUSIONS
PRKAG2 patients present deteriorated LV diastolic function and preserved LV systolic function. Bradycardia and preserved GLS are useful to identify PRKAG2 syndrome from sarcomeric HCM, which may be beneficial for clinical decision-making.
Topics: AMP-Activated Protein Kinases; Cardiomyopathy, Hypertrophic; Echocardiography; Echocardiography, Three-Dimensional; Heart Ventricles; Humans; Hypertrophy, Left Ventricular; Sarcomeres; Ventricular Function, Left
PubMed: 35509080
DOI: 10.1186/s12947-022-00284-3 -
Kardiologia Polska 2022
Topics: Accessory Atrioventricular Bundle; Bundle of His; Catheter Ablation; Electrocardiography; Heart Transplantation; Humans; Wolff-Parkinson-White Syndrome
PubMed: 35475463
DOI: 10.33963/KP.a2022.0112 -
The Journal of Innovations in Cardiac... Apr 2022In the background of an accessory pathway (AP), the H-V interval can vary during atrial/coronary sinus pacing, but only with a concomitant change in the QRS morphology...
In the background of an accessory pathway (AP), the H-V interval can vary during atrial/coronary sinus pacing, but only with a concomitant change in the QRS morphology and the degree of pre-excitation. In an interesting case of a 62-year-old woman, the H-V interval varied during coronary sinus pacing despite a fixed pre-excitation. This appears to have happened due to infra-Hisian complete atrioventricular dissociation, which resulted from iatrogenic mechanical bumping of the left anterior fascicle in the background of right bundle branch block and left posterior hemiblock.
PubMed: 35474859
DOI: 10.19102/icrm.2022.130401 -
Annals of Noninvasive Electrocardiology... Sep 2022One-to-one atrioventricular conduction during atrial flutter is one of the most severe life-threatening arrhythmias and is hemodynamically perilous. Rapid wide QRS...
One-to-one atrioventricular conduction during atrial flutter is one of the most severe life-threatening arrhythmias and is hemodynamically perilous. Rapid wide QRS tachycardia often not only occurs in patients with ventricular tachycardia but is also found in supraventricular tachycardia/atrial flutter with preexistent QRS prolongation, supraventricular tachycardia/atrial flutter with QRS prolongation caused by an IC antiarrhythmic drug, and supraventricular tachycardia/atrial flutter with preexcitation. Furthermore, atrial flutter with 1:1 AVC via an accessory pathway is an uncommon presentation of Wolff-Parkinson-White syndrome. We present a case of atrial flutter with 1:1 rapid AVC in the presence of Wolff-Parkinson-White syndrome. Physicians should be familiar with the rapid wide QRS complex ECG pattern associated with AFL with 1:1 AVC via an accessory pathway. Establishing the definitive diagnosis is essential for selecting an appropriate treatment strategy for improving outcomes.
Topics: Accessory Atrioventricular Bundle; Anti-Arrhythmia Agents; Atrial Flutter; Electrocardiography; Humans; Tachycardia, Supraventricular; Wolff-Parkinson-White Syndrome
PubMed: 35429345
DOI: 10.1111/anec.12959