scholarly journals Twenty-five-year-old woman with palpitations and hypertrophic cardiomyopathy

Heart Asia ◽  
2019 ◽  
Vol 11 (1) ◽  
pp. e011174 ◽  
Author(s):  
Krittapoom Akrawinthawong ◽  
Vineet Kumar

Clinical introductionA 25-year-old woman with a diagnosis of hypertrophic cardiomyopathy (HCM) and pre-excitation on ECG presented with unexplained syncope and daily palpitation. Genetic testing was positive for lysosome-associated membrane protein 2 (LAMP2) mutation which confirmed the diagnosis of Danon disease. Her younger sister was diagnosed with a similar condition and received a defibrillator implantation. Her 12-lead ECG (figure 1) and a long strip tracing (figure 2) are shown below.Figure 112-lead ECG.QuestionWhere is the location of the accessory pathway and what is the next appropriate management?Anteroseptal pathway and catheter ablationMid-septal pathway and pacemaker/defibrillator implantationRight lateral pathway and catheter ablationFasciculoventricular pathway and electrophysiological studyLeft lateral pathway and electrophysiological study

2019 ◽  
Vol 3 (3) ◽  
Author(s):  
Bart A Mulder ◽  
Yvonne M Hoedemaekers ◽  
Maarten P van den Berg ◽  
Rosa L E van Loon ◽  
Anna M Wind ◽  
...  

Abstract Background Danon disease is a rare X-linked multisystemic disorder that has primarily been described in male patients. Case summary We present three female patients with Danon disease with a predominantly cardiac phenotype in whom disease onset and expression was very different from that of male patients. Case 1 was first admitted for acute heart failure and then readmitted a few months later for cardiac shock, necessitating mechanical support, and heart transplantation. Case 2 had complex arrhythmias for which many antiarrhythmic drugs were tried with only limited success. Her disease accelerated after her first pregnancy, and she showed reduced left ventricular function and dilated cardiomyopathy. Case 3 was referred for near syncope and ablated for an accessory pathway; she had extensive left ventricular hypertrophy. In all three cases, a final diagnosis of Danon disease was only made after genetic testing that identified a causal variant in the lysosome-associated membrane protein 2 gene. Discussion Danon disease in female patients is a challenging diagnosis that may not be identified until genetic testing has been performed.


2013 ◽  
Vol 8 (2) ◽  
pp. 103-109
Author(s):  
Sabina Hashem ◽  
Mohsin Hossain ◽  
M Atahar Ali ◽  
Amal Kumar Choudhury ◽  
SM Ahsan Habib ◽  
...  

Background: This study assessed the efficacy and safety of techniques used to ablate left-sided accessory atrioventricular pathways at atrial insertion sites by retrograde aortic approach. Radiofrequency catheter ablation of left-sided accessory pathways by way of retrograde aortic approach can be highly successful. Methods: This study were done in the department of Cardiology, National Institute of Cardiovascular Diseases (NICVD), Dhaka from June 2009 and March 2010, a total 30 patients with recurrent SVT who has left sided accessory atrioventricular pathways found after electrophysiological study, underwent attempted radiofrequency catheter ablation of one or more accessory atrioventricular pathways by retrograde aortic approach. The ablation catheter was inserted into the femoral artery and passed retrogradely across the aortic valve .Once an optimal target site has been identified, radiofrequency energy at a pre-selected temperature of 55 - 60 0 C and power output set at 50 watts was delivered through the ablation catheter. Loss of preexcitation or interruption of the tachycardia within 5 seconds of RF application was considered effective and RF current was continued in this location 30 to 60 seconds. Ablation success was defined at completion of procedure as acutely successful or unsuccessful on the basis of successful elimination of all ablation targets. Results: During EP study it was found that 9 patients had concealed accessory pathways and 21 had manifest pre-excitation. WPW left-lateral pathway was present in 10 (33.3%), concealed left lateral in 08 (26.7%) ,WPW left posterior in 11 (36.7%), concealed left posterior in 1 (3.3%). Out of 30 accessory pathways, 24 were successfully interrupted with radiofrequency catheter ablation with a primary success rate of 80 %. The ratio of atrial/ ventricular electrogram in successful sites was 0.83 ±0.27 (0.53-1.46). There were no major complications with retrograde aortic approach. Six failed patients were ablated via transseptal method. Conclusion: The retrograde atrial insertion approach to left-sided accessory pathway ablation is very safe and high effective, especially suitable for the failed patients by using retrograde ventricular insertion ablation procedure and by using single-catheter ablation of accessory pathway. DOI: http://dx.doi.org/10.3329/uhj.v8i2.16081 University Heart Journal Vol. 8, No. 2, July 2012


EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
J Ramos Jimenez ◽  
A Marco Del Castillo ◽  
VC Lozano Granero ◽  
C Lazaro Rivera ◽  
R Salgado ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Catheter ablation is recommended as first-line treatment in patients with atrioventricular nodal reentry tachycardias (AVNRT). However, the best therapeutic modality in patients with dual AV nodal physiology but non-inducible tachycardias in electrophysiological study (EPS) remains controversial, especially when no tachycardias have been documented. Our objective was to evaluate the results of empirical slow pathway ablation in patients showing dual AV nodal physiology but non-inducible AVNRT. Methods Multicenter, retrospective, observational registry of consecutive patients undergoing EPS due to clinical suspicion of paroxysmal supraventricular tachycardias (PSVT), but with no prior ECG documentation. Clinical, EPS and ablation (when performed) data were collected and andalyzed.  Results 427 patients of 12 centers were included. Mean age was 46.3 ±16.1 and 297 (69.6%) were females. AVNRT was induced in 188 patients (typical in 181 cases, atypical in 7). Dual AV nodal physiology with or without single nodal echo beats, but with no sustained tachycardia and without evidence of accessory pathway was present in 68 patients. Ablation of the slow pathway was performed in 187/188 patients with AVNRT and in 30/68 patients with dual physiology. Among subjects with non-inducible tachycardia, ablation reduced significantly recurrences (39.5% in non-ablated vs. 16.7%; p = 0.04), with a level equivalent to those with ablated AVNRT(14.4% vs. 16.7%; p = 0.75). Procedure-related complications were similar in both groups: empirical ablation n = 1; 3.3% vs. induced tachycardia n = 6; 3.2% (p = 0.98). Conclusions In patients with high clinical suspicion of PSVT but non-documented and non-inducible arrhythmias, the presence of dual AV nodal physiology makes AVNRT a likely mechanism of the clinical tachycardia. Catheter ablation of slow pathway reduces the risk of recurrence to a level equivalent to those with inducible and ablated AVNRT. AVNRT (n = 188) Dual nodal physiology (n = 68) p value Age (years) 48.6 ± 16.3 41.9 ± 14.0 <0.01 Female 71.8% 67.7% 0.52 Years symptomatic 9.3 ± 11.3 3.6 ± 8.1 <0.01 Sudden onset 83.9% 88.0% 0.54 Abrupt end 73.4% 74.6% 0.96 Previous rate-slowing drugs 30.9% 25.0% 0.36 Previous antiarrhythmic drugs 5.9% 2.9% 0.35 Isoproterenol in EPS 70.0% 89.7% <0.01


2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Ana Rita Pereira ◽  
Alexandra Briosa ◽  
Rita Miranda ◽  
Sofia Sequeira Almeida ◽  
Luís Brandão ◽  
...  

Background. Wolff-Parkinson-White syndrome is an uncommon cardiac disorder characterized by the presence of one or more accessory pathways that predispose patients to frequent episodes of arrhythmias. The prognosis is usually good, but there is a lifetime risk of malignant arrhythmias and sudden cardiac death. Case Summary. A 25-year-old male presented a witnessed out-of-hospital cardiac arrest with ventricular fibrillation rhythm. Due to rapid initiation of prehospital advanced life support, return of spontaneous circulation was observed. During the transport to the hospital, an irregular wide complex tachycardia suggestive of preexcited atrial fibrillation with haemodynamic instability was also observed and a synchronized shock was applied. Baseline 12-lead electrocardiogram was compatible with sinus rhythm and ventricular preexcitation pattern. After clinical stabilization, an electrophysiological study was performed confirming the presence of a left anterolateral accessory pathway with a short antegrade effective refractory period. Successful radiofrequency catheter ablation was achieved. Discussion. The reported clinical case recalls fundamental features of the Wolff-Parkinson-White syndrome and outlines the increasing evidence and importance of the invasive risk stratification and even catheter ablation in asymptomatic patients who suffer from this uncommon disease that may have a dramatic and fatal initial clinical manifestation.


2014 ◽  
Vol 9 (1) ◽  
pp. 18-24
Author(s):  
S Hashem ◽  
M Hossain ◽  
MA Ali ◽  
AK Chowdhury ◽  
Habib SM Ahsan ◽  
...  

Background: This study assessed the efficacy and safety of techniques used to ablate left-sided accessory atrioventricular pathways at atrial insertion sites by retrograde aortic approach. Radiofrequency catheter ablation of left-sided accessory pathways by way of retrograde aortic approach can be highly successful. Methods: This study were done in the department of Cardiology, National Institute of Cardiovascular Diseases (NICVD), Dhaka from June 2009 and March 2010, a total 30 patients with recurrent SVT who has left sided accessory atrioventricular pathways found after electrophysiological study, underwent attempted radiofrequency catheter ablation of one or more accessory atrioventricular pathways by retrograde aortic approach. The ablation catheter was inserted into the femoral artery and passed retrogradely across the aortic valve .Once an optimal target site has been identified, radiofrequency energy at a pre-selected temperature of 55 - 60 0 C and power output set at 50 watts was delivered through the ablation catheter. Loss of preexcitation or interruption of the tachycardia within 5 seconds of RF application was considered effective and RF current was continued in this location 30 to 60 seconds. Ablation success was defined at completion of procedure as acutely successful or unsuccessful on the basis of successful elimination of all ablation targets. Results: During EP study it was found that 9 patients had concealed accessory pathways and 21 had manifest pre-excitation. WPW left-lateral pathway was present in 10 (33.3%), concealed left lateral in 08 (26.7%), WPW left posterior in 11 (36.7%), concealed left posterior in 1 (3.3%). Out of 30 accessory pathways, 24 were successfully interrupted with radiofrequency catheter ablation with a primary success rate of 80 %. The ratio of atrial/ ventricular electrogram in successful sites was 0.83 ±0.27 (0.53-1.46). There were no major complications with retrograde aortic approach. Six failed patients were ablated via transseptal method. Conclusion: The retrograde atrial insertion approach to left-sided accessory pathway ablation is very safe and high effective, especially suitable for the failed patients by using retrograde ventricular insertion ablation procedure and by using single-catheter ablation of accessory pathway. DOI: http://dx.doi.org/10.3329/uhj.v9i1.19507 University Heart Journal Vol. 9, No. 1, January 2013; 18-24


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Valeria Novelli ◽  
Antonio Bisignani ◽  
Gemma Pelargonio ◽  
Guido Primiano ◽  
Maria Lucia Narducci ◽  
...  

2017 ◽  
Vol 73 (8) ◽  
pp. 505-509
Author(s):  
Agnieszka Noszczyk-Nowak ◽  
Gavin McAulay ◽  
Artur Fuglewicz ◽  
Krzysztof Nowak ◽  
Adrian Janiszewski ◽  
...  

The presence of an accessory atrioventricular conduction pathway may provide the anatomical basis for some tachyarrhythmias. Persistent tachyarrythmias may result in the development of systolic dysfunction or tachycardiomyopathy and eventual progression to heart failure. In this paper we report the successful ablation of an accessory pathway involved in the pathogenesis of persistent orthodromic atrioventricular reciprocating tachycardia (AVRT) and heart failure in a 3-year-old male Labrador retriever. Electrophysiological study and radiofrequency (RF) catheter ablation were performed under general anesthesia. Electrophysiological study revealed AVRT resulting from a retrograde conduction via a right posteroseptal accessory pathway. The successful RF ablation of the accessory pathway resulted in the resolution of the tachycardia and return of normal sinus rhythm. Medical management of heart failure was withdrawn and no episodes of recurrent tachyarrhythmia were documented during a 3-month follow-up.


2021 ◽  
Vol 162 (41) ◽  
pp. 1643-1651
Author(s):  
Ádám Riba ◽  
Barnabás Németh ◽  
Ferenc Árvai ◽  
Géza Lupkovics ◽  
Tamás Tahin

Összefoglaló. A ritmuszavarok előfordulása gyakoribb a terhes nők esetén, mint a nem várandósok körében. A legtöbb esetben terápiás beavatkozás nélkül is kihordható a magzat. Hemodinamikai instabilitás és magzatkárosodáshoz vezető fetalis hypoperfusio jöhet létre, amennyiben tartós, magas kamrai frekvenciával járó epizódok jelentkeznek. Ezekben az esetekben a ritmuszavar megszüntetése indokolttá válhat. Az antiarrhythmiás gyógyszerek korlátozottan és nagy körültekintéssel alkalmazhatók a gyermeket várók körében, így a katéterablatio jelenthet biztonságos és használható alternatívát. Ezen beavatkozásokat hagyományosan röntgensugár segítségével végzik, ez azonban az ionizáló sugárzásnak a magzati fejlődésre gyakorolt hatása miatt magas rizikót jelentene. Több éve elérhető a szív-elektrofiziológiában az ún. zéró fluoroszkópiás ablatio, mely a pitvarfibrilláció kezelésében és más ritmuszavarok esetében egyaránt alkalmazható. A terheseknél alkalmazott eljárást két eseten keresztül mutatjuk be. A röntgensugár használatát, a jelen cikkben bemutatott beavatkozások esetén is, sikerült teljesen kiküszöbölnünk. Az első, 23 hetes gravid páciensnél recidív paroxysmalis supraventricularis tachycardia miatt végeztünk elektrofiziológiai vizsgálatot. E vizsgálat során atrioventricularis nodalis reentry tachycardiát igazoltunk és abláltunk sikerrel. Második esetbemutatásunkban egy anteroseptalis járulékos köteg katéterablatiós megoldását mutatjuk be. A terhesség során jelentkező, az anyára és/vagy magzatára veszélyt jelentő ritmuszavar esetén a háromdimenziós térképező rendszer (szükség esetén intracardialis ultrahangvizsgálattal kiegészítve) biztonságos és hatásos alternatívát jelent, olyan esetekben, ha röntgensugár nem használható. Orv Hetil. 2021; 162(41): 1643–1651. Summary. Arrhythmias are more common in pregnant women than in others. In most cases, the fetus can be delivered without therapeutic intervention. Hemodynamic instability and fetal hypoperfusion leading to fetal harm may occur if persistent episodes of high ventricular rate occur. In these cases, resolution of the arrhythmia may be advised. Antiarrhythmic drugs can be used with limitations and great caution in those expecting a child, so catheter ablation may be a safe and usable alternative. These interventions are traditionally performed using X-ray, however, due to the effect of ionizing radiation on fetal development, this would pose a high risk. Zero-fluoroscopic ablation has been available for several years in cardiac electrophysiology, which can be used both in the treatment of atrial fibrillation and in other arrhythmias. The procedure which we used in pregnant women is presented in two cases. We also managed to completely eliminate the use of X-ray during the interventions presented in this article. In the first case, a 23-week-old gravid patient underwent electrophysiological examination for recurrent paroxysmal supraventricular tachycardia. In the electrophysiological study, atrioventricular nodal reentry tachycardia was confirmed and successfully ablated. In our second case study, we present a catheter ablation for anteroseptal accessory pathway. Three-dimensional mapping system (supplemented with intracardiac ultrasound, if necessary), in the case of significant arrhythmia, is a safe and effective alternative where X-rays, which poses a risk to the mother and/or the fetus, cannot be used during pregnancy. Orv Hetil. 2021; 162(41): 1643–1651.


2021 ◽  
Vol 22 (9) ◽  
pp. 4700
Author(s):  
Michelle M. Monasky ◽  
Emanuele Micaglio ◽  
Giuseppe Ciconte ◽  
Ilaria Rivolta ◽  
Valeria Borrelli ◽  
...  

Genetic testing in Brugada syndrome (BrS) is still not considered to be useful for clinical management of patients in the majority of cases, due to the current lack of understanding about the effect of specific variants. Additionally, family history of sudden death is generally not considered useful for arrhythmic risk stratification. We sought to demonstrate the usefulness of genetic testing and family history in diagnosis and risk stratification. The family history was collected for a proband who presented with a personal history of aborted cardiac arrest and in whom a novel variant in the SCN5A gene was found. Living family members underwent ajmaline testing, electrophysiological study, and genetic testing to determine genotype-phenotype segregation, if any. Patch-clamp experiments on transfected human embryonic kidney 293 cells enabled the functional characterization of the SCN5A novel variant in vitro. In this study, we provide crucial human data on the novel heterozygous variant NM_198056.2:c.5000T>A (p.Val1667Asp) in the SCN5A gene, and demonstrate its segregation with a severe form of BrS and multiple sudden deaths. Functional data revealed a loss of function of the protein affected by the variant. These results provide the first disease association with this variant and demonstrate the usefulness of genetic testing for diagnosis and risk stratification in certain patients. This study also demonstrates the usefulness of collecting the family history, which can assist in understanding the severity of the disease in certain situations and confirm the importance of the functional studies to distinguish between pathogenic mutations and harmless genetic variants.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Makoto Nishimori ◽  
Kunihiko Kiuchi ◽  
Kunihiro Nishimura ◽  
Kengo Kusano ◽  
Akihiro Yoshida ◽  
...  

AbstractCardiac accessory pathways (APs) in Wolff–Parkinson–White (WPW) syndrome are conventionally diagnosed with decision tree algorithms; however, there are problems with clinical usage. We assessed the efficacy of the artificial intelligence model using electrocardiography (ECG) and chest X-rays to identify the location of APs. We retrospectively used ECG and chest X-rays to analyse 206 patients with WPW syndrome. Each AP location was defined by an electrophysiological study and divided into four classifications. We developed a deep learning model to classify AP locations and compared the accuracy with that of conventional algorithms. Moreover, 1519 chest X-ray samples from other datasets were used for prior learning, and the combined chest X-ray image and ECG data were put into the previous model to evaluate whether the accuracy improved. The convolutional neural network (CNN) model using ECG data was significantly more accurate than the conventional tree algorithm. In the multimodal model, which implemented input from the combined ECG and chest X-ray data, the accuracy was significantly improved. Deep learning with a combination of ECG and chest X-ray data could effectively identify the AP location, which may be a novel deep learning model for a multimodal model.


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