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Background: Isolated ventricular noncompaction (IVNC) is characterized by multiple prominent trabeculations and deep intertrabecular recesses. Some reports prove that the chronic heart failure may occur in approximately half of the patients. In this report we investigate the correlation between the number of non compacted segments and entity of systolic dysfunction from the registry and subregistries of the SIEC., Method: To identify the correlation between ventricular dysfunction and number of segments involved in non compaction we evaluated a consecutive series of 238 patients affected by non compaction, from the SIEC (Società Italiana di Ecografia Cardiovascolare) registry. The average age of patients was 41.5 years (range: 1-92 years), 137 were males and 101 females. In 122 cases we found ventricular systolic dysfunctions with an EF average of 34.6%. The number of affected segments by non-compactation and diastolic dysfunction were found to be non-independent predictors of LV systolic dysfunction., Conclusion: From the analyses we carried out, it seems that ventricular dysfunction seems to be completely independent from the segment numbers of non compacted segments., (Copyright (c) 2009. Published by Elsevier Ireland Ltd.)

Background: It is well-known that a reduction of the cardiac frequency variability, measurable with the Heart Rate Variability (HRV) system, is an indirect expression of the sympathetic-autonomic tone. Another index, Heart Rate Turbulence (HRT), has been recently suggested as a possible unit of measurement for the sympathetic-autonomic tone: this system allows to estimate the baro-reflex response of the carotid arteries to an early ventricular extra-systole by analysing heart rate variations induced by a premature beat., Methods and Results: In our research we have analyzed this phenomenon in patients affected by moderate or severe cardiac failure. In particular, we divided 110 patients into two arms: subjects with or without a history of resuscitated arrhythmic death, that is, patients with high or low arrhythmic potential. In a detailed analysis of the sympathetic-autonomic tone, using both the above-mentioned parameters, HRV showed an irrelevant statistical difference between the two arms; on the contrary, HRT showed a significant statistical difference., Conclusions: If our conclusions will be confirmed by next larger reports, HRT could become a reliable index for screening the arrhythmic potential of patients affected by cardiac failure, to select the ones who need a defibrillator implantation.

Aim: Heart failure with normal left ventricle (LV) ejection fraction is commonly understood as diastolic heart failure because this expression implies the presence of LV diastolic dysfunction diagnosed by specific echocardiographic findings, such as slow LV relaxation and increased LV stiffness. In this work the authors propose a new parameter named Motion Index, which is measurable by M-Mode technique and it is likely linked to diastolic dysfunction., Methods: A patient population composed by 134 subjects was enrolled. They all were in New York Heart Association (NYHA) functional class II. Echocardiogram carried out in all patients allowed the authors to distinguish 2 patient arms depending on the presence or absence of diastolic dysfunction, evaluated by flow Doppler and tissue Doppler., Results: After carrying out every echocardiographic examination, the authors also measured the new parameter that called Motion Index, and found that it had an average value of 46 in patients with normal diastolic function and 33.5 in patients with diastolic dysfunction. This parameter did not depend on systolic dysfunction., Conclusions: Data obtained showed a statistically significant correlation between Motion Index and means of diastolic function assessed by both flow and tissue Doppler.

Background: Noncompaction of left ventricular myocardium is a rare congenital cardiomyopathy resulting from an incomplete myocardial morphogenesis that leads to the persistence of the embryonic myocardium. This condition is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. It is not clear, in noncompaction of myocardium, whether intertrabecular recesses could be responsible for thrombi formation and thromboembolic complications., Methods: The prevalence of stroke and echocardiographic finding of thrombus was evaluated in a continuous series of 229 patients (men and women) affected by noncompaction of the left ventricular myocardium, who were included in the SIEC registry. We excluded patients affected by atrial fibrillation., Results: The mean age of the patients was 49.5 years. Fifty percent of the patients were affected by a ventricular systolic dysfunction. The mean period of follow-up was 7.3 years. Only four patients had a history of ischemic stroke. A large thrombus into the left ventricular chamber was observed in a 1-year-old child affected by Behcet's disease (high risk of thrombi formation)., Conclusion: Noncompaction of the left ventricular myocardium, by itself, does not seem to be a risk factor for stroke or embolic results, so there is no indication for oral anticoagulant therapy.

Background: The pathogenesis of idiopathic monomorphic ventricular tachycardia is not clear. We suppose that a lack of balance of the sympathetic system could be involved. Frequency domain analyses of the heart rate can be useful to understand autonomic system balance. Therefore we performed this evaluation on a sample of seven children affected by idiopathic monomorphic ventricular tachycardia., Methods and Results: We performed a Holter recording for palpitations on all the children, with an average age of 12 (range: 7-18 years). In all the patients many episodes of repeated sustained or nonsustained ventricular tachycardia were demonstrated, with an average heart rate of 170 bpm. We excluded any structural heart defect through echocardiography and magnetic resonance imaging studies in all the children. A negative tridimensional electroanatomic mapping was performed on five of them. Holter analysis of ventricular rate variability was performed in the frequency domain. Two main components were distinguished in a spectrum calculated on the basis of 24 h-long recordings. We studied low frequency and high frequency components. We compared the values obtained with those of a control group of 10 healthy children, admitted to our cardiology division, day-care system. Affected patients showed a reduction of average high frequency as a sign of a reduction of vagal activity and an average increase of the low frequency/high frequency ratio., Conclusions: The data may confirm our hypothesis of the involvement of the sympathetic nervous system in idiopathic monomorphic ventricular tachycardia in children.

Aim: Long QT syndrome is a rare arrhythmic disease with a low incidence in the general population. There are no sure clinical or electrocardiographic parameters that could lead to a correct prognostic stratification in patients affected by this syndrome. The correlation between the incidence of a sudden death or dangerous ventricular arrhythmias and the duration of QTc interval is still a controversial topic., Methods: Twenty nine children affected by QT long syndrome were admitted to the Division of Pediatric Cardiology of the Casa del Sole Hospital of Palermo (Italy). Their diagnosis was made by electrocardiogram (ECG). The average age of the patients was 7.6 years. The average follow-up was 4 years and three months. A therapy with beta-blocker was administered to all the children. During the follow-up of 4 year and three months, patients were genotyped. Twenty-three out of 29 children had at least one relative affected by the syndrome. Three of them had a familiar dead because of this syndrome and everyone had a duration of maximum QTc higher than the cut off (P=0.0002). All the people who died had not followed the therapy with beta-blocker. Patients with a maximum QTc recorded <500 had not familiar death by this syndrome., Results: Holter and echocardiogram recorded periodically during the observation did not show dangerous arrhythmic events. All children maintained a good health during the follow-up., Conclusion: Although conducted on a small study population, the data analysis recorded during this study suggests that in patients affected by QT long syndrome younger than 16 years old undergoing a beta-blocker therapy the prognosis is excellent. The duration of QTc interval appears as a negative prognostic factor, although the beta-blocker therapy has been reduced considerably the incidence of sudden death.

Background: Takotsubo cardiomyopathy consists of reversible systolic left ventricular apical ballooning associated with chest pain. Electrocardiographic abnormalities and the minimal rise of serum cardiac markers are similar to those in acute myocardial infarction, but without evidence of myocardial ischemia or injury. To date, many reports concerning this kind of acute reversible heart failure have been published, but the information available about the management of affected patients is scarce and the clinical data are incomplete., Aims: In the present study, we report a collection of 40 patients who were affected by Takotsubo cardiomyopathy obtained in a multicentric international study, aiming to investigate the origins, and the clinical and instrumental patterns, and to establish the best diagnostic criteria for this syndrome., Methods and Results: In the analysed group, the mean age was 68 years, of whom 85% were women. On admission to hospital, 68% of patients reported chest pain. An electrocardiogram (ECG) showed anterolateral (34%) or anterior (36%) ST segment elevation. The ECG demonstrated hyperkinesis of the basal segments with a severe hypokinesis of the other segments. Mean ejection fraction was 42.53%. Three patients died within the first 24 h from acute heart failure. The remaining 37 patients showed a complete resolution of symptoms and a complete normalization of the kinesis deficiency. Sixteen patients underwent myocardial scintigraphy, nine cases underwent myocardial biopsy and two patients received an ergonovine test., Conclusion: Our results demonstrate a good course of Takotsubo cardiomioathy, after the initial phase. An echocardiogram is an important tool for improving the diagnosis.

Objectives: The noncompaction of the left ventricular myocardium is a rare congenital heart disease, characterized by an excessive prominence of trabecular meshwork, spaced out by deep intertrabecular recesses, consequent to the arrest of the normal myocardium embryogenesis. Although there are numerous descriptions, the physiopathological effects of the structural alterations, just like the clinical spectrum and the evolution of the disease, are not totally clarified. In the present study, we have evaluated the natural history of the disease, the familial incidence and the alterations of the systolic and diastolic function., Methods: We collected a series of 21 young patients who were affected by noncompaction of left ventricular myocardium. In all cases, a diagnosis was echocardiographically made on the basis of a reported spongy/compacted ratio > 2 in one or more segments of the left ventricle. Thirteen patients were male and eight were female, with a mean age of 12.7 years (range 21 days to 27 years). The average follow-up time was 7.8 years (range 1-18 years); all patients were periodically tested by ECG Holter and two-dimensional and Doppler echocardiogram. In 14 patients, the last echocardiographic evaluation included the analysis of tissue Doppler imaging (TDI)., Results: The noncompaction of left ventricular myocardium was isolated in nine cases and associated with a structural cardiopathy in 12 cases: with atrial septal defect in four cases, ventricular septal defect in four cases, aortopulmonary window in one case, aortic coarctation in one case and bicuspid aortic valve in one case. One case presented a type Kent ventricular pre-excitation. Twelve cases were symptomatic at the moment of the diagnosis (for heart failure in 11 cases and for syncope in one case). Nine cases were asymptomatic and the diagnosis was made during a family screening or occasionally. In ten of the 11 subjects affected by congestive heart failure, medical therapy re-established a good haemodynamic balance (in two cases, it was possible to suspend the therapy). In one case with congestive heart failure and pulmonary hypertension in New York Heart Association class III, we recommended heart transplantation. We did not find any dysrhythmia in any of the cases. Diastolic function impairment, tested by transmitral blood pressure monitoring and TDI, was found in seven of 14 patients, all with reduced left ventricular contractility., Conclusions: We noticed a considerable variability of clinical presentation in our cases, according to the number of the ventricular segments affected by the anomaly. According to our data, middle-term prognosis appears to be better than that previously reported in the literature. We found a reduction of the systolic function only in 50% of cases, all with severe involvement of the apical and postero-lateral segments. Diastolic function was compromised only in those patients with severe impairment of systolic function.

The coronary artery fistulas are rare congenital anomalies with a very low incidence. These can be symptomatic or asymptomatic because the hemodynamic consequences of the fistula vary and depend on the shunt dimensions. Discordant opinions instead are present in the literature for the defect closing in asymptomatic patients. Here, we describe a patient affected by a coronary right fistula canalized in a small accessory right atrial chamber. During follow-up, we observed a progressive dilatation of the right coronary artery (maximum diameter 10.3 mm) with hemodynamic overload of the right sections.

Aim: Recent scientific evidence has emphasized the possible role of inhibitors of the renin-angiotensin system in preventing arrhythmic relapses in patients with paroxysmal or persistent atrial fibrillation and co-existing left ventricular hypertrophy or left ventricular dysfunction., Methods: In order to verify the effects of these drugs on patients with a normal heart, we collected a series of 187 patients admitted to our division of cardiology for paroxysmal or persistent atrial fibrillation. All patients underwent cardioversion (with antiarrhythmic drugs and/or by electrical cardioversion) and were discharged in sinus rhythm. Episodes of recurrent arrhythmia were recorded during a mean follow-up period was 2 years. Patients were subdivided into 2 groups according to therapy: group 1 comprised patients receiving renin-angiotensin system inhibitors, group 2 comprised those not receiving therapy with these agents. All 91 patients in group 1 and 76 of those in group 2 had hypertension. Among the 91 patients in the group 1, 55 were treated with angiotensin-converting enzyme (ACE) inhibitors and 36 with angiotensin receptor blockers. There were no statistically significant differences in cardiovascular risk factors or antiarrhythmic drug use between the 2 groups., Results: In group 1, 83% of patients experienced <2 recurrences of atrial fibrillation during the follow-up period, while 17% had >2 episodes. In group 2, 86% of patients experienced <2 relapses during the follow-up period, while the remaining 14% had >2 relapses. There was no statistically significant difference between the 2 groups (P=0.85). A subgroup analysis showed that treatment with angiotensin receptor blockers, beta-blockers, diuretics, and calcium-channel blockers brought no advantage in sinus rhythm maintenance., Conclusion: In our sample of hypertensive patients with a healthy heart, treatment with ACE inhibitors showed no statistically significant advantage in the prevention of atrial fibrillation relapses.

Even if a complete recovery is not an available outcome for Alzheimer disease, it is possibile to improve the clinical symptoms (selfsufficiency, cognitive impairment and behavioral disturbances) with pharmacological and non-pharmacological therapies. The treatment of the patient with dementia is a complex one, that cannot rely only on the use of drugs but needs of a global approach that take into account all the different aspect of the disease. The most used drugs are the cholinesterase inhibitors that have been shown to stabilize or slow down cognitive and functional decline and retard institutionalization, but new treatments are on the way. Extremely important is a strong alliance with the family. Non pharmacological cognitive rehabilitation techniques are also useful in potentiating residual cognitive functions in the patient and in supporting the family and the caregivers.

Background: Isolated left ventricular non-compaction is the result of incomplete myocardial morphogenesis, leading to persistence of the embryonic myocardium. The condition is recognized by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricle. Whether these intertrabecular recesses are a favorable substrate for ventricular arrhythmias is unclear. Some reports have found that the fatal ventricular arrhythmias may occur in approximately half of the patients. In this report we investigated about this association., Methods and Results: In total we evaluated a continuous series of 238 patients affected by non-compaction. Periodic Holter monitoring was performed every 6 months for 4 years. Only 11 patients had documented ventricular tachycardia, which was sustained in two cases and non-sustained in nine. In no cases we observed ventricular fibrillation., Conclusions: Non-compaction alone does not seem to be a risk factor for malignant ventricular arrhythmias.

Isolated noncompaction of left ventricular myocardium is a rare congenital heart disease, characterized by an excessive prominence of trabecular meshwork, spaced out by deep intertrabecular recesses, consequent to the arrest of the normal myocardial embryogenesis. Although there are numerous descriptions, the pathophysiological effects of the structural alterations, like the clinical spectrum and the evolution of the disease, are not fully clarified. In this paper we evaluated the natural history of the disease, the family incidence and the alterations of the systolic and diastolic function. An interesting case report is described concerning a patient affected by noncompaction and atrial fibrillation.

Background: The natural history of atherosclerosis has not clearly been elucidated yet. Some works reported that flogosis plays a role in plaque instability. Why does this inflammatory process start? We investigated the correlation between Chlamydia pneumoniae acute infection and plaque rupture., Methods: We compared blood concentrations of IgM anti-Chlamydia pneumoniae in patients affected by acute myocardial infarction (AMI) and in patients affected by stable angina., Results: Our results showed a minimal statistical difference, with a more positive value in patients with AMI. Subsequently, the group affected by AMI was divided into two subgroups with and without plaque rupture: the subgroup with plaque rupture showed a higher blood concentration of Chlamydia antibodies. This subgroup was also divided into two other subgroups according to blood white cell concentration: the subgroup with normal concentration of white blood cells showed the highest value of Chlamydia antibodies., Conclusions: Chlamydia pneumoniae could play an important role in atherosclerotic plaque instability

Tako-tsubo cardiomyopathy is a recently described disease characterized by chest pain, transient left ventricular dysfunction and specific electrocardiographic changes. The disease takes its name from the typical left apical ballooning observed at left ventriculogram. Tako-tsubo cardiomyopathy was first described by Sato in 1990. Since then sporadic cases were reported by Japanese authors, and only a few European publications are available. We describe 2 cases of patients affected by this syndrome.