Clinical spectrum of fascicular tachycardia.

AIMS: Ventricular tachycardia spreading from the anterior or posterior division of the left bundle branch is generally called fascicular tachycardia (FT). We will present our experience with FT, a type of ventricular tachycardia not necessarily implying the absence of heart disease and/or sensitivity to selective antiarrhythmic drugs, but only particular routes of left ventricular depolarization. METHODS: Since 1981 we have had the opportunity to study 10 cases of FT (nine men and one woman; aged 28-77 years, mean ±â€ŠSD 55 ±â€Š18.6 years) by means of echocardiography, coronary angiography (seven cases), endomyocardial biopsy (five cases), signal-averaged electrocardiogram (SAECG, nine patients), electrophysiological and electropharmacological evaluation. RESULTS: Seven patients had paroxystic, extrastimulus inducible FT that was sensitive to verapamil given intravenously (group A); three patients, on the other hand, showed repetitive or incessant FT, not modifiable by stimulation techniques and sensitive to class 1 antiarrhythmic drugs (group B). Patients presented histologic substrates ranging from the absence of heart disease to previous myocardial infarction or myocarditis. FT spontaneously disappeared within 2 years in group B, while frequently persisted in the long term in group A. CONCLUSIONS: FT is not a homogeneous group of ventricular tachycardia, as patients may differ according to clinical presentation, mechanisms that are involved in the genesis of the arrhythmia and natural history; the histologic substrate is highly variable, ranging from the total absence of heart disease to severe forms of myocardial involvement.

Impact of abciximab on prognosis in diabetic patients undergoing primary percutaneous coronary intervention.

BACKGROUND: The impact of diabetes in patients with acute myocardial infarction (AMI) treated with primary percutaneous coronary intervention (PCI) is unclear. The benefit of abciximab in this subset of patients remains controversial. METHODS AND RESULTS: Three hundred and twenty-seven consecutive and unselected patients with acute AMI treated with primary PCI were included in our single-center retrospective registry, 103 diabetic (31%) and 224 nondiabetic (69%). Abciximab was given at the physician's discretion. Diabetic patients were older (mean age 68.5±11 vs. 65±12 years; P=0.009), had an increased prevalence of hypertension (73 vs. 54%; P=0.001), a decreased prevalence of smoking (31 vs. 45%; P=0.02), a longer duration of symptoms before hospital admission (190 vs. 143 min; P=0.031), and a higher number of stents implanted (1.4 vs. 1.2; P=0.04). Other clinical and angiographic characteristics were comparable in the two groups. Diabetic patients had a higher incidence of the combined end-point of death and reinfarction rate at 30 days (18 vs. 10%; P=0.04) compared to nondiabetic patients. Abciximab treatment was associated with a lower in-hospital (23.8 vs. 5%; P=0.005) and 30-day (23.8 vs. 6.6%; P=0.012) mortality, and a lower incidence of death and reinfarction at 30 days (33.3 vs. 9.8%; P=0.003) in diabetic patients. In nondiabetic patients, abciximab was not associated with improved outcome measures. Advanced Killip class (III and IV) and abciximab were found to be independently associated with 30-day death or myocardial infarction [respectively, odds ratio (OR) 6.075, 95% confidence interval (CI) 1.59-23.218, P=0.008 and OR 0.177, 95% CI 0.034-0.938, P=0.042] in the propensity score-matched populations of diabetic patients. Advanced Killip class and thrombolysis in myocardial infarction score index were found to be independently associated with 30-day death or myocardial infarction (respectively, OR 6.607, 95% CI 1.5-29.106, P=0.013 and OR 1.094 95% CI 1.042-1.148, P<0.001) in the propensity score-matched populations of nondiabetic patients. CONCLUSIONS: In our registry diabetic patients treated with primary PCI for AMI had a worse in-hospital and 30-day outcome than nondiabetic patients. Adjunct pharmacologic treatment with abciximab was associated to a better prognosis only in diabetic patients.

Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry.

AIMS: We sought to examine the clinical presentation and natural history and to identify long-term prognostic predictors in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) as information concerning the natural history and risk stratification of ARVC is still incomplete. METHODS AND RESULTS: A cohort of 96 ARVC patients (68% males, 35 ± 15 years) was enrolled and underwent structured diagnostic protocol and follow-up. Primary study endpoints were death and heart transplantation (HTx). Clinical and echo-Doppler data were assessed as prognostic indicators. Sixty-five per cent of patients had right ventricular (RV) systolic dysfunction (RV fractional area change < 33%) and 24% had left ventricular (LV) systolic dysfunction (LV ejection fraction <50%). During a mean follow-up of 128 ± 92 months, 20 patients (21%) experienced cardiac death or underwent HTx. At multivariate analysis (Model 1), RV dysfunction [hazard ratio (HR): 4.12; 95% confidence interval (CI): 1.01-18.0; P = 0.05], significant tricuspid regurgitation (HR: 7.6; 95% CI: 2.6-22.0; P < 0.001), and amiodarone treatment (HR: 3.4; 95% CI: 1.3-8.8; P = 0.01) resulted as predictors of death/HTx. When inserting in the model, the 'ordinal dysfunction' (Model 2), which considers the presence of both RV and LV dysfunctions, this variable emerged as an independent prognostic predictor (HR: 6.3; 95% CI: 2.17-17.45; P < 0.001). At the receiver operating characteristic analysis, Model 2 was significantly more accurate in predicting long-term outcome compared with Model 1 (area under the curve 0.84 vs. 0.78, respectively; P = 0.04). CONCLUSION: In our tertiary referral centre ARVC population, the presence of LV dysfunction at diagnosis has an incremental power in predicting adverse outcome compared with RV dysfunction alone.

Idiopathic dilated cardiomyopathy: prognostic significance of electrocardiographic and electrophysiologic findings in the nineties.

BACKGROUND: With the exception of a few cases such as aborted sudden cardiac death, sustained ventricular tachycardia, and syncope of unexplained origin, there is no consensus on the clinical findings identifying patients with idiopathic dilated cardiomyopathy with an increased risk of sudden cardiac death or malignant ventricular arrhythmias. METHODS: To verify whether electrocardiographic and arrhythmologic features could be useful for prognostic stratification, 78 consecutive patients with an invasive diagnosis of idiopathic dilated cardiomyopathy, but without symptomatic ventricular arrhythmias, were enrolled in a prospective study. Signal-averaged ECG, 24 to 48 hour ECG monitoring and electrophysiologic study were performed at the time of diagnosis to identify arrhythmogenic predictors of outcome. Transplant-free and arrhythmic event-free survival was evaluated on the basis of initial parameters. RESULTS: During a mean follow-up of 85 months, 9 patients died (6 of sudden cardiac death and 3 of congestive heart failure), 10 patients underwent cardiac transplantation for refractory heart failure, and 3 presented with sustained ventricular tachycardia. The independent predictors for death and cardiac transplantation were an HV interval > 55 ms and the combination of frequent repetitive ventricular ectopics with a poor left ventricular function. A strong index of arrhythmic events proved to be the association of a prolonged HV interval with a wide (> 110 ms) QRS complex (odds ratio 4.53, 95% confidence interval 1.57-13.04, p < 0.005). CONCLUSIONS: An accurate measurement of the HV interval and QRS duration at baseline evaluation may add prognostic information in patients with idiopathic dilated cardiomyopathy. In our experience, abnormal values of both parameters identified a group of patients with a very high risk of late occurring arrhythmic events.

[The natural history of dilated cardiomyopathy: a review of the Heart Muscle Disease Registry of Trieste]. / Come è cambiata la storia naturale della cardiomiopatia dilatativa. Una revisione del Registro delle Malattie del Miocardio di Trieste.

Dilated cardiomyopathy (DCM), a heart muscle disease characterized by ventricular dilation and dysfunction, is a leading cause of mortality and morbidity. In the present paper we will consider the main results of studies on the natural history of DCM in 581 consecutive patients prospectively enrolled and systematically followed in the Heart Muscle Disease Registry of Trieste in the last 25 years. In the last decades prognosis of DCM significantly improved over time, mainly as a consequence of optimized treatment with ACE-inhibitors and beta-blockers. However, a strong heterogeneity of prognosis was observed among patients both in familial and sporadic cases. Early diagnosis and treatment allowed to recognize two distinct subgroups, one with a rapidly progressive downhill course, high mortality and urgent indication to heart transplantation, another with a more favorable outcome. Long-term optimized treatment with ACE-inhibitors (in 90% of cases) and beta-blockers (in 87% of cases) was associated with a remarkable clinical improvement in 50% of patients and apparent "healing" in 16% of cases. A systematic and accurate echocardiographic follow-up showed in these cases a significant improvement of the left ventricular ejection fraction (LVEF) with "reverse remodeling", frequently associated with a decrease of severity of functional mitral regurgitation and regression of the restrictive filling pattern. The response to optimal treatment showed a strong relation to long-term outcome. The 8-year transplant-free survival, starting from the evaluation at 2 years, was 31% in patients with persistent NYHA class III-IV, 64% in NYHA class I-II and LVEF < or = 40%, 83% in NYHA class I-II and LVEF > 40% and 94% in patients with apparent "healing" (p < 0.0001). Long-term follow-up showed a significant clinical progression of the disease in 33% of cases, independently of the initial clinical response to treatment. Predictive factors of a favorable response to beta-blocker treatment associated with ACE-inhibitors were a history of mild hypertension, an early diagnosis and treatment and the presence of sinus tachycardia. The risk of sudden death was increased particularly in patients with long-term persistent or progressive left ventricular dilation and dysfunction. A rigorous pharmacological approach (optimization of beta-blockers, withdrawal or decrease of dosage of digitalis), and selective non-pharmacological strategy (automated implantable cardioverter-defibrillators for primary prevention in high-risk patients) are potentially effective to decrease the incidence of sudden death during long-term follow-up. In conclusion, the Heart Muscle Disease Registry of Trieste gave us in the last 25 years new insights into the natural history of DCM, underlying the importance of a rigorous and systematic approach both at clinical presentation and during long-term follow-up on optimized medical treatment.

[How the natural history of dilated cardiomyopathy has changed. Review of the Registry of Myocardial Diseases of Trieste]. / Come è cambiata la storia naturale della cardiomiopatia dilatativa. Una revisione del Registro delle Malattie del Miocardio di Trieste.

Dilated cardiomyopathy (DCM), a heart muscle disease characterized by ventricular dilation and dysfunction, is a leading cause of mortality and morbidity. In the present paper we will consider the main results of studies on the natural history of DCM in 581 consecutive patients prospectively enrolled and systematically followed in the Heart Muscle Disease Registry of Trieste in the last 25 years. In the last decades prognosis of DCM significantly improved over time, mainly as a consequence of optimized treatment with ACE-inhibitors and beta-blockers. However, a strong heterogeneity of prognosis was observed among patients both in familial and sporadic cases. Early diagnosis and treatment allowed to recognize two distinct subgroups, one with a rapidly progressive downhill course, high mortality and urgent indication to heart transplantation, another with a more favorable outcome. Long-term optimized treatment with ACE-inhibitors (in 90% of cases) and beta-blockers (in 87% of cases) was associated with a remarkable clinical improvement in 50% of patients and apparent "healing" in 16% of cases. A systematic and accurate echocardiographic follow-up showed in these cases a significant improvement of the left ventricular ejection fraction (LVEF) with "reverse remodeling", frequently associated with a decrease of severity of functional mitral regurgitation and regression of the restrictive filling pattern. The response to optimal treatment showed a strong relation to long-term outcome. The 8-year transplant-free survival, starting from the evaluation at 2 years, was 31% in patients with persistent NYHA class III-IV, 64% in NYHA class I-II and LVEF < or = 40%, 83% in NYHA class I-II and LVEF > 40% and 94% in patients with apparent "healing" (p < 0.0001). Long-term follow-up showed a significant clinical progression of the disease in 33% of cases, independently of the initial clinical response to treatment. Predictive factors of a favorable response to beta-blocker treatment associated with ACE-inhibitors were a history of mild hypertension, an early diagnosis and treatment and the presence of sinus tachycardia. The risk of sudden death was increased particularly in patients with long-term persistent or progressive left ventricular dilation and dysfunction. A rigorous pharmacological approach (optimization of beta-blockers, withdrawal or decrease of dosage of digitalis), and selective non-pharmacological strategy (automated implantable cardioverter-defibrillators for primary prevention in high-risk patients) are potentially effective to decrease the incidence of sudden death during long-term follow-up. In conclusion, the Heart Muscle Disease Registry of Trieste gave us in the last 25 years new insights into the natural history of DCM, underlying the importance of a rigorous and systematic approach both at clinical presentation and during long-term follow-up on optimized medical treatment.