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Signal-averaged electrocardiographic parameter progression as a marker of increased electrical instability in two cases with an overt from of arrhythmogenic right ventricular cardiomyopathy
In arrhythmogenic right ventricular cardiomyopathy (ARVC) the fibrofatty substitution of the RV myocardium constitutes the substrate for reentrant circuits, leading to the onset of ventricular arrhythmias. This pathological process also accounts for "delayed ventricular potentials" that could be recorded as late potentials using the signal-averaged ECG technique (SAECG). This study examined two patients affected by overt forms of ARVC who showed a worsening of the electrical instability associated with a fast progression of SAECG parameters, while all the other clinical findings remained unchanged. This suggests a possible role of SAECG parameter progression as a marker of increased electrical instability
Letter regarding article by Nasir et al, "Electrocardiographic features of arrhythmogenic right ventricular dysplasia/cardiomyopathy according to disease severity: a need to broaden diagnostic criteria"
abstract not availabl
Arrhythmogenic right ventricular cardiomyopathy is a life-threatening disease at high risk for cardiac arrest during effort. Minor forms are as dangerous as major forms?
The aim of the present study was the evaluation of physical activity likely to lead to the onset of life-threatening ventricular arrhythmias in subjects affected by arrhythmogenic right ventricular cardiomyopathy. In particular, strenuous physical activity can be dangerous in patients with minor or 'concealed' forms of the disease, in whom no previous diagnosis has been made. In these patients preventive strategies cannot be adopted due to the lack of a clinical diagnosis, and so physical effort can be responsible for the first noticeable symptom of the disease
Echocardiographic findings in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia.
LETTER (abstract not available
Pregnancy in women with arrhythmogenic right ventricular cardiomyopathy/dysplasia.
OBJECTIVES:
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a cardiac disease characterised by myocardial necrosis followed by fibro-fatty substitution leading to the onset of ventricular arrhythmias. The aim of the present study was to analyse pregnancy in women affected by this condition.
STUDY DESIGN:
Six women affected by ARVC/D who underwent a pregnancy were studied with a follow-up programme, consisting of 12-lead ECG, signal-averaged ECG, 24-h ECG and two-dimensional and Doppler echocardiogram performed before the beginning of the pregnancy, at 3rd and 7th month of gestation and after the delivery.
RESULTS:
All women were on antiarrhythmic therapy during pregnancy; two complained of palpitations in the last 3 months. Delivery was performed at full terms in all, with caesarean section and epidural anaesthesia in four. Mean weight at birth was 3490g. No adverse reactions on the newborns were detected. All patients were advised against breast-feeding. No significant morphological changes were detected. During the period following the delivery (1-6years, mean 2,6years) one subject experienced a sustained ventricular tachycardia.
CONCLUSIONS:
Pregnancy seems to be well tolerated in patients affected by ARVC/D, but a programmed clinical protocol is mandatory particularly in the last trimester and puerperium, due to increased risk of ventricular arrhythmias
Long-term follow-up of the signal-averaged ECG in arrhythmogenic right ventricular cardiomyopathy: correlation with arrhythmic events and echocardiographic findings
AIMS:
The aims of our study were to evaluate late potential changes during long-term follow-up in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and to correlate these results with echocardiographic findings and sustained ventricular tachycardia (VT) occurrence.
METHODS AND RESULTS:
We studied 31 patients (22 males and 9 females; mean age 29+/-16) during 8 years of follow-up by signal-averaged ECG (SAECG) and echocardiography. Ten subjects experienced episodes of sustained VT. During follow-up, all the SAECG parameters showed a progressive significant increase in late potentials. In contrast, echocardiographic indices did not show evidence of relevant modifications. Patients with sustained VT were characterized by significantly lower left and right ventricular ejection fractions, longer values of filtered QRS at 25/40/80-250 Hz filters, and longer high-frequency low-amplitude (HFLA) signals at 25-250 Hz at baseline. The analysis of SAECG modification during follow-up indicated that only HFLA signals at 25-250 Hz increased significantly in the sustained VT group.
CONCLUSION:
We detected a progressive increase in delayed ventricular conduction by SAECG not associated with significant echocardiographic changes. Therefore, the conduction disturbance seems to increase independently from anatomical alterations. The baseline SAECG and echocardiographic parameters, more than their modifications during follow-up, appear to be useful in identifying patients with sustained VT
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