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2.
Int Heart J ; 60(5): 1201-1205, 2019 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-31484869

RESUMO

Right-sided accessary pathways in patients with Wolff-Parkinson-White (WPW) syndrome may cause cardiac dyssynchrony and dilated cardiomyopathy, with a characteristic septal shape, irrespective of any supraventricular tachycardia episodes. We report on two infants (13 and 5 months), whose right-sided accessary pathway-induced dilated cardiomyopathy was successfully treated by flecainide for the first time. After the flecainide administration, an abnormal aneurysmal dilation of the basal interventricular septum was almost restored to normal, and the decreased ejection fraction recovered. Flecainide use may be an important therapeutic option for this entity to avoid catheter ablation during infancy.


Assuntos
Feixe Acessório Atrioventricular/diagnóstico por imagem , Feixe Acessório Atrioventricular/tratamento farmacológico , Cardiomiopatia Dilatada/diagnóstico por imagem , Flecainida/uso terapêutico , Síndrome de Wolff-Parkinson-White/diagnóstico por imagem , Feixe Acessório Atrioventricular/complicações , Cardiomiopatia Dilatada/tratamento farmacológico , Cardiomiopatia Dilatada/etiologia , Cardiomiopatia Dilatada/terapia , Ecocardiografia Doppler/métodos , Eletrocardiografia/métodos , Feminino , Seguimentos , Humanos , Lactente , Injeções Intravenosas , Masculino , Medição de Risco , Amostragem , Resultado do Tratamento , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/fisiopatologia
3.
Am J Emerg Med ; 37(7): 1340-1345, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31027937

RESUMO

The evaluation and treatment of wide QRS-complex tachycardia remains a challenge, and mismanagement is quite common. Diagnostic aids such as wide-complex tachycardia algorithms perform poorly in the real-life setting. The purpose of this review is to offer a simple clinical-electrocardiographic approach for the initial evaluation and management of the adult patient with stable wide-complex tachycardia that does not require recollection of complex guidelines or algorithms.


Assuntos
Fibrilação Atrial/diagnóstico , Eletrocardiografia , Taquicardia/diagnóstico , Taquicardia/tratamento farmacológico , Síndrome de Wolff-Parkinson-White/diagnóstico , Administração Intravenosa , Antiarrítmicos/administração & dosagem , Fibrilação Atrial/complicações , Diagnóstico Diferencial , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Taquicardia/complicações , Síndrome de Wolff-Parkinson-White/complicações
4.
Medicine (Baltimore) ; 98(5): e14267, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30702587

RESUMO

RTIONALE: Left ventricular noncompaction (LVNC) is a genetic cardiomyopathy characterized by the presence of a thin compacted layer of myocardium and a spongy subendocardial layer with trabeculations and recesses. LVNC associated Wolf-Parkinson-White syndrome is very rare. PATIENT CONCERNS: A 32-year-old male presented with short episodes of palpitations and a syncope 6 months before his hospitalization. DIAGNOSIS: His ECG revealed the presence of a right posterior accessory pathway. Echocardiography identified trabeculations of the septal, apical, and lateral wall of the left ventricle, consistent with left ventricular noncompaction. Cardiac MRI confirmed the diagnosis, as the ratio between the noncompacted and compacted myocardial layer was 2.3. INTERVENTIONS: The electrophysiological study revealed a malignant right posterior accessory pathway. Catheter ablation was successfully performed at the level of posterior tricuspid annulus. Programmed ventricular stimulation could not induce any arrhythmia at the end of the procedure. OUTCOMES: During 15 months of follow-up, the patient presented no more episodes of palpitations or syncope. LESSONS: Left ventricular noncompaction with right accessory pathway is a rare association with genetic basis and gives a higher risk of sudden cardiac death. Catheter ablation of the accessory pathway is a valuable way of treatment in this category of patients, lowering the risk of sudden cardiac death.


Assuntos
Miocárdio Ventricular não Compactado Isolado/complicações , Miocárdio Ventricular não Compactado Isolado/cirurgia , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/cirurgia , Adulto , Ablação por Cateter , Ecocardiografia , Eletrocardiografia , Humanos , Masculino
5.
J Cardiovasc Electrophysiol ; 30(1): 102-108, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30255617

RESUMO

INTRODUCTION: The objective of the current study was to elucidate the effects of the accessory pathways (APs) on the left ventricular (LV) wall motion and radiofrequency catheter ablation (RFCA) on AP-related regional wall motion abnormality (RWMA) in patients with Wolff-Parkinson-White (WPW) syndrome. METHODS AND RESULTS: We included 348 consecutive patients (age, 37.6 ± 17.3 years; men, 58.3%) with WPW syndrome who underwent RFCA for AP. We analyzed electrocardiographic data, the AP location, LV ejection fraction (LVEF), and RWMA patterns and their changes after RFCA. The locations of APs were right, septal, and left in 78, 94, and 176 patients, respectively. RWMA at the AP location (44.9%, 51.1%, and 17.6%, respectively; P < 0.001), decreased LVEF (10.3%, 6.4%, and 1.1%, respectively; P = 0.004), and dyskinesia (16.7%, 16.0%, and 1.1%, respectively; P < 0.001) were significantly more frequent in patients with a right or septal AP than in those with a left AP. In 31 of 50 patients with RWMA who underwent post-RFCA echocardiography (62.0%), RWMA was completely improved after RFCA. Right AP (odds ratio [OR], 22.084; 95% confidence interval [CI], 3.628-134.420; P = 0.001) and dyskinesia (OR, 6.275; 95% CI, 1.186-33.213; P = 0.031) were significantly associated with the absence of RWMA improvement after RFCA. CONCLUSIONS: AP-related RWMA is frequent in patients with right or septal APs. A substantial number of patients with right AP or dyskinesia may show no improvement in RWMA after RFCA.


Assuntos
Feixe Acessório Atrioventricular , Ablação por Cateter , Volume Sistólico , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda , Síndrome de Wolff-Parkinson-White/cirurgia , Potenciais de Ação , Adulto , Feminino , Frequência Cardíaca , Humanos , Masculino , Pessoa de Meia-Idade , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologia , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/diagnóstico , Síndrome de Wolff-Parkinson-White/fisiopatologia , Adulto Jovem
7.
J Electrocardiol ; 51(6): 1067-1070, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30497732

RESUMO

A 13 kg, 20 month-old, Caucasian girl, presented with cardiomyopathy, biventricular dysfunction and pre-excitation on electrocardiogram. She had normal intracardiac anatomy with severely dilated left ventricle and severely diminished biventricular function (Fig. 1). She was treated with milrinone and epinephrine infusions, mechanical ventilation and listed for heart transplant. She underwent Berlin Heart EXCOR biventricular assist device (BiVAD) placement (30 ml LVAD and 25 ml RVAD pumps). No supraventricular tachycardia (SVT) was inducible or noted during her hospitalization. First ablation attempt without BiVAD support was unsuccessful; however, 18 days post BiVAD implantation, another electrophysiology study and successful radiofrequency ablation of a right anterolateral accessory pathway was performed on BiVAD support. After successful ablation and loss of pre-excitation, the cardiac dysfunction rapidly improved with initial improvement noted as early as 48 h after the successful ablation. Due to recovery of cardiac function, a BiVAD wean protocol was initiated and BiVAD explantation was performed 48 days after the implant (30 days after the successful ablation). To the best of our knowledge, this is the first report of successful BiVAD explantation.


Assuntos
Feixe Acessório Atrioventricular/cirurgia , Ablação por Cateter , Remoção de Dispositivo , Coração Auxiliar , Síndrome de Wolff-Parkinson-White/terapia , Ecocardiografia , Eletrocardiografia , Feminino , Coração/diagnóstico por imagem , Humanos , Lactente , Disfunção Ventricular/complicações , Disfunção Ventricular/terapia , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/fisiopatologia
10.
Turk Kardiyol Dern Ars ; 46(6): 488-493, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30204140

RESUMO

A 31-year-old male patient presented with complaints of palpitations, dizziness, and recurrent episodes of syncope. A 12-lead electrocardiogram (ECG) revealed manifest ventricular preexcitation, which suggested Wolff Parkinson White syndrome. In addition, an incomplete right bundle branch block and a 3-mm ST segment elevation ending with inverted T-waves in V2 were consistent with coved-type (type 1) Brugada pattern. An electrophysiological study was performed, and during the mapping, the earliest ventricular activation with the shortest A-V interval was found on the mitral annulus posterolateral site. After successful radiofrequency catheter ablation of the accessory pathway, the Brugada pattern on the ECG changed, which prompted an ajmaline provocation test. A type 1 Brugada ECG pattern occurred following the administration of ajmaline. Considering the probable symptom combinations of these 2 coexisting syndromes and the presence of recurrent episodes of syncope, programmed ventricular stimulation was performed and subsequently, ventricular fibrillation was induced. An implantable cardioverter-defibrillator was implanted soon after.


Assuntos
Síndrome de Brugada/complicações , Síndrome de Wolff-Parkinson-White/complicações , Adulto , Ajmalina/administração & dosagem , Animais , Antiarrítmicos/administração & dosagem , Síndrome de Brugada/fisiopatologia , Síndrome de Brugada/terapia , Bloqueio de Ramo , Ablação por Cateter , Desfibriladores Implantáveis , Tontura , Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Humanos , Masculino , Recidiva , Síncope , Fibrilação Ventricular/etiologia , Fibrilação Ventricular/terapia , Síndrome de Wolff-Parkinson-White/fisiopatologia , Síndrome de Wolff-Parkinson-White/terapia
11.
JACC Clin Electrophysiol ; 4(4): 433-444, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-30067481

RESUMO

OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population. BACKGROUND: Children with WPW syndrome are at risk of sudden death. METHODS: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects. RESULTS: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia. CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.


Assuntos
Fibrilação Atrial , Morte Súbita , Síndrome de Wolff-Parkinson-White , Adolescente , Fibrilação Atrial/epidemiologia , Fibrilação Atrial/etiologia , Criança , Morte Súbita/epidemiologia , Morte Súbita/etiologia , Feminino , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/epidemiologia , Síndrome de Wolff-Parkinson-White/mortalidade
12.
Emergencias (Sant Vicenç dels Horts) ; 30(3): 194-200, jun. 2018. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-172962

RESUMO

La muerte súbita e inesperada en jóvenes es un evento raro pero devastador. Aunque su incidencia es baja, se registran de 1,3 a 8,5 casos de muerte súbita cardiaca (MSC) por cada 100.000 jóvenes y se asocia frecuentemente con una autopsia negativa. Muchas de las causas de la MSC con autopsia negativa se heredan de forma autosómica dominante. Algunas causas de MSC con autopsia positiva en pacientes jóvenes incluyen la miocardiopatía hipertrófica (MCH) y la displasia arritmogénica de ventriculo derecho. Las causas de autopsia negativa incluyen causas arritmogénicas heredadas, como el síndrome de QT largo, el síndrome de Brugada, la taquicardia ventricular polimórfica catecolaminérgica, el syndrome de Wolff-Parkinson-White (WPW) y la fibrilación ventricular idiopática. La pregunta importante para los profesionales de urgencias es: ¿cómo podemos predecir y prevenir la MSC en los jóvenes antes de la autopsia?


Sudden unexpected death in the young, though rare, is devastating for both the family and the community. Although only 1.3 to 8.5 cases of sudden cardiac death (SCD) occur per 100 000 young people, autopsy is often inconclusive. Many causes of SCD are related to autosomal dominant inherited risk, however; therefore, answers are important for survivors. Causes of autopsy-positive SCD in young patients include hypertrophic cardiomyopathy and arrhythmogenic right ventricular dysplasia. Autopsy-negative SCD has been related to inherited arrhythmogenic causes such as long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, WolffParkinson-White syndrome, and idiopathic ventricular fibrillation. The important question for the emergency physician is how SCD can be predicted and prevented in the young so that there is no need for an autopsy


Assuntos
Humanos , Morte Súbita Cardíaca/prevenção & controle , Síndrome de Brugada/complicações , Síndrome do QT Longo/complicações , Taquicardia Ventricular/complicações , Biomarcadores/análise , Autopsia/estatística & dados numéricos , Síndrome de Wolff-Parkinson-White/complicações , Cardiomiopatia Hipertrófica/complicações
14.
Am J Cardiol ; 121(11): 1442-1444, 2018 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-29706203
15.
J Int Med Res ; 46(5): 2054-2060, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29562793

RESUMO

This report describes a 66-year-old Caucasian male who acutely developed severe, bilateral impairment of visual acuity at 24 years of age. Leber's hereditary optic neuropathy (LHON) was suspected but the diagnosis was not genetically confirmed until the age of 49 years when the primary LHON mutation m.3460G>A was detected. Since onset, visual acuity had slightly improved. The family history was positive for LHON (brother, two sisters of mother, female cousin) and genetically confirmed in his brother and one aunt. Since the age of 65 years, he had experienced recurrent vertigo. His cardiological history was positive for arterial hypertension, noncompaction, myocardial thickening, intermittent right bundle-branch-block (RBBB) and Wolff-Parkinson-White (WPW) syndrome. In addition to LHON, he presented with polyneuropathy, hyperCKaemia, carotid artery occlusion, and a history of stroke. Cardiological investigations at 66 years of age revealed mildly reduced systolic function, enlarged atria, and nonsustained ventricular tachycardias. He underwent an electrophysiological investigation, but radiofrequency ablation was ruled out due to a 'bizarre' cardiac conduction system. Instead, an implantable cardioverter defibrillator was proposed but refused by the patient. Since the vertigo did not resolve it was attributed to polyneuropathy. This case demonstrates that LHON may be associated with noncompaction, myocardial thickening, reduced systolic function, enlarged atria, RBBB, WPW syndrome and nonsustained ventricular tachycardias. WPW syndrome in LHON may require invasive antiarrhythmic treatment.


Assuntos
Mutação/genética , Atrofia Óptica Hereditária de Leber/complicações , Atrofia Óptica Hereditária de Leber/genética , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/genética , Idoso , Ecocardiografia , Humanos , Masculino
16.
J Cardiol ; 71(5): 484-487, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29305187

RESUMO

BACKGROUND: Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC). AIM: We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA. METHODS: We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features). We reviewed echocardiograms and CMR scans for concomitant morphological abnormalities (LVNC, PDA - patent ductus arteriosus, VSD - ventricular septal defect, ASD - atrial septal defect, mitral valve prolapse, BAV - bicuspid aortic valve, CoA - coarctation of aorta). RESULTS: The studied group consisted of 84 consecutive patients (mean age 38±15 years, 50 women) with EA. We found four patients (4.8%) with LVNC, two of them had cardiac arrest, one had VT, and one was symptomless, but had QTc prolongation in Holter recordings. Concomitant abnormalities were VSD (4.8%), PDA (1.2%), CoA (1.2%), mitral valve prolapse (1.2%), and BAV (2.4%). The most common anomaly was ASD type II - 23 patients (27.3%) and WPW - Wolff-Parkinson-White's syndrome - 9 patients (10.7%). CONCLUSIONS: Non-compaction is a notable abnormality in adult patients with EA and it may affect their prognosis. Although other concomitant lesions were more common, only patients with LVNC suffered from cardiac arrest or ventricular arrhythmia.


Assuntos
Arritmias Cardíacas/fisiopatologia , Anomalia de Ebstein/fisiopatologia , Parada Cardíaca/fisiopatologia , Ventrículos do Coração/fisiopatologia , Adolescente , Adulto , Idoso , Valva Aórtica/anormalidades , Valva Aórtica/patologia , Arritmias Cardíacas/complicações , Anomalia de Ebstein/complicações , Ecocardiografia , Eletrocardiografia , Feminino , Parada Cardíaca/complicações , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Comunicação Interatrial/complicações , Comunicação Interatrial/fisiopatologia , Comunicação Interventricular/complicações , Comunicação Interventricular/fisiopatologia , Doenças das Valvas Cardíacas/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Prolapso da Valva Mitral/complicações , Prolapso da Valva Mitral/fisiopatologia , Mutação , Prevalência , Prognóstico , Estudos Retrospectivos , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/fisiopatologia , Adulto Jovem
17.
Europace ; 20(7): 1175-1181, 2018 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-29016834

RESUMO

Aims: The relationship between ventricular pre-excitation and left ventricular dysfunction has been described in the absence of sustained supraventricular tachycardia in a series of case reports. There have been no systematic studies about the effect of ventricular pre-excitation with different accessory pathway locations on ventricular wall motion and left ventricular (LV) systolic function. Methods and results: Thirty patients were selected for each of 4 groups, including those with right septal pathways (Group 1), right free-wall pathways (Group 2), left free-wall pathways (Group 3), and non-pre-excited patients undergoing electrophysiological evaluation for supraventricular tachycardia. We analysed the influence of the location of the accessory pathway on ventricular wall motion, systolic function, ventricular synchronism, and LV size. Right-sided accessory pathways were associated with abnormal motion of the interventricular septum, LV dyssynchrony, decreased LV systolic function, and increased LV diameter. Eighteen of 60 cases (30.0%) with right-sided accessory pathways had LV dyssynchrony, and these patients had lower LV ejection fraction and higher LV end-diastolic diameter. Conclusion: Right-sided accessory pathways may impair ventricular wall motion and LV systolic function, resulting in decreased LV ejection fraction and increased LV end-diastolic diameter. These effects occurred in patients with LV dyssynchrony. These effects, including LV dyssynchrony, resolved after radiofrequency ablation. A right-sided free-wall accessory pathway may have more detrimental effects than a septal accessory pathway. Left ventricular dyssynchrony and abnormal interventricular septal motion appeared to be responsible for the pathogenesis of LV dysfunction and remodelling.


Assuntos
Feixe Acessório Atrioventricular/fisiopatologia , Taquicardia Supraventricular/complicações , Disfunção Ventricular Esquerda/etiologia , Função Ventricular Esquerda , Síndrome de Wolff-Parkinson-White/complicações , Feixe Acessório Atrioventricular/cirurgia , Potenciais de Ação , Criança , Pré-Escolar , Ecocardiografia , Técnicas Eletrofisiológicas Cardíacas , Feminino , Frequência Cardíaca , Humanos , Masculino , Ablação por Radiofrequência , Recuperação de Função Fisiológica , Volume Sistólico , Sístole , Taquicardia Supraventricular/diagnóstico , Taquicardia Supraventricular/fisiopatologia , Taquicardia Supraventricular/cirurgia , Resultado do Tratamento , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Remodelação Ventricular , Síndrome de Wolff-Parkinson-White/diagnóstico , Síndrome de Wolff-Parkinson-White/fisiopatologia , Síndrome de Wolff-Parkinson-White/cirurgia
19.
Pediatr Emerg Care ; 34(12): e239-e242, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27753716

RESUMO

OBJECTIVES: We report on a rare but severe complication of adenosine use in a child with reentry tachycardia. METHODS AND RESULTS: Treatment with adenosine, which is the standard medical therapy of atrioventricular reentry tachycardia, led to the development of an irregular wide complex tachycardia, caused by rapid ventricular response to atrial fibrillation. The girl was finally stabilized with electrical cardioversion. We analyze the pathomechanism and discuss possible treatment options. CONCLUSIONS: Atrial fibrillation, as well as its conduction to the ventricles, can be caused by adenosine. Rapid ventricular response in children with Wolff-Parkinson-White syndrome is more frequent than previously believed. A patient history of atrial fibrillation is a contraindication for cardioversion with adenosine and needs to be assessed in children with reentry tachycardia. High-risk patients may potentially profit from prophylactic comedication with antiarrhythmic agents, such as flecainide, ibutilide, or vernakalant, before adenosine administration.


Assuntos
Adenosina/efeitos adversos , Antiarrítmicos/efeitos adversos , Taquicardia/tratamento farmacológico , Síndrome de Wolff-Parkinson-White/diagnóstico , Criança , Cardioversão Elétrica/métodos , Eletrocardiografia , Feminino , Humanos , Síndrome de Wolff-Parkinson-White/complicações , Síndrome de Wolff-Parkinson-White/terapia
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