RESUMO
In 39 patients with myotonic dystrophy, we found a high percentage of infrahissian cardiac conduction abnormalities (51%) and late potentials (46%), whereas spontaneous and inducible ventricular arrhythmias were rare. These results suggest that the prolongation of QRSD and the duration of the low-amplitude signal on the signal-averaged electrocardiogram were related to delayed activation of the His and Purkinje tissue rather than true late potentials.
Assuntos
Eletrocardiografia , Ventrículos do Coração/fisiopatologia , Distrofia Miotônica/fisiopatologia , Processamento de Sinais Assistido por Computador , Adulto , Nó Atrioventricular/fisiopatologia , Fascículo Atrioventricular/fisiopatologia , Complexos Cardíacos Prematuros/fisiopatologia , Eletrocardiografia Ambulatorial , Feminino , Bloqueio Cardíaco/diagnóstico , Bloqueio Cardíaco/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Distrofia Miotônica/diagnóstico , Ramos Subendocárdicos/fisiopatologia , Fatores de RiscoRESUMO
The incidence and the nature of medium-term complications of automatic implantable cardiac defibrillators (AICD) were studied. Seventy-nine AICD were implanted in 50 consecutive patients (42 men, aged 54.5 +/- 13.7 years). Forty-six patients had spontaneous ventricular arrhythmia. These arrhythmias were resistant to treatment (N = 9), reproducible with treatment (N = 28). In 4 patients, the indication was prophylactic, in 2 a Brugada syndrome, in 2 syncope with reinducible ventricular tachycardia and in 1 patient, torsades with a short coupling interval. Forty-six patients had underlying cardiac disease (ischaemic, N = 28, primary dilated cardiomyopathy, N = 10, others, N = 8). The ejection fraction was > 40% in 32 patients. The average follow-up was 41.3 +/- 34.9 months. Eight patients died, 2 from cardiac failure. Twenty-one patients (42%) had 1 or more complications related to their AICD. These occurred: in the operative period (N = 3): 1 post-shock atrioventricular block, 1 ruptured electrode and 1 increased threshold with amiodarone; in the postoperative period (N = 6): infection in 3 cases, cerebrovascular accident in 1 case, deep venous thrombosis of the left arm in 1 case, pneumothorax in 1 case. In the medium-term, the complications were mainly inappropriate electrical shocks observed in 14 patients related to atrial arrhythmias in 7 cases, sinus tachycardia in 1 case, over-detection of myopotentials in 2 cases and electrode dysfunction in 4 cases. In addition, the authors observed complications related to the material: AICD failure in 1 case, electrode displacement in 1 case, and electrode rupture in 3 cases. The authors conclude that AICD are effective for the treatment of malignant ventricular arrhythmias which justify strict specialist follow-up given the incidence and diversity of their complications.
Assuntos
Desfibriladores Implantáveis/efeitos adversos , Fibrilação Ventricular/terapia , Adulto , Idoso , Causas de Morte , Feminino , Seguimentos , Parada Cardíaca/etiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Acidente Vascular Cerebral/etiologia , Trombose Venosa/etiologiaRESUMO
OBJECTIVE: To identify intracardiac conduction abnormalities in patients with myotonic dystrophy from their clinical, ECG, and genetic features. METHODS: 39 consecutive patients (mean (SD) age 42. 9 (12.1) years; 16 female, 23 male) underwent clinical examination, genetic studies, resting and 24 hour ambulatory ECG, signal averaged ECG, and electrophysiological studies. RESULTS: 23 patients suffered from cardiac symptoms, 23 had one or more cardiac conduction abnormality on resting ECG, one had sinus deficiency, and 21 (53.8%) had prolonged HV intervals. No correlation was found between the severity of the neurological symptoms, onset of disease, cardiac conduction abnormalities on ECG, and the intracardiac conduction abnormalities on electrophysiological study. The size of the DNA mutation was longer in the abnormal HV interval group than in the normal HV interval group (3.5 (1.8) v 2.2 (1.0) kb, p < 0.02). Signal averaged ECG parameters (total QRS duration (QRSD) and duration of low amplitude signals = 40 microV (LAS 40)) were greater in patients with an abnormal HV interval than in those with a normal HV interval (123.4 (24.6) v 102.8 (12.3) ms and 47.5 (12.8) v 35.3 (8.8) ms, respectively; p < 0.005). Only the association of QRSD >/= 100 ms with LAS 40 >/= 36 ms identified patients with an abnormal HV interval with good sensitivity (80%) and specificity (83. 3%). CONCLUSIONS: Infrahissian conduction abnormalities are common in myotonic dystrophy and can be identified using signal averaged electrocardiography.