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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.

Background In the last few years, several new drugs such targeted therapy and immunotherapy have shown activity and have been approved for the treatment of metastatic renal cell carcinoma (mRCC). In mRCC, nivolumab, anti-PD-1 antibody, has recently been approved for the treatment after failure of anti-angiogenic treatment. Literature data are very limited in some subgroups of patients such as those with end-stage renal disease requiring hemodialysis that were excluded from the pivotal clinical trial. Patients and Methods We retrospectively reviewed the medical records of 8 patients with metastatic renal cell carcinoma undergoing dialysis treated with nivolumab to evaluate the treatment-related toxicities and the clinical outcomes. Results Among 8 patients treated with nivolumab, partial responses were observed in one patient and stable disease in five patients. Therefore, the objective response rate was 12,5%, and the disease control rate was 75%. The estimated median PFS was 16 months (95% CI 1,2 – 30,8 mo). Median overall survival was 26 months (95% CI 9,7 – 42,2 mo). There were no grade 4 adverse events and only 2 patients (25%) had grade 3 adverse events. Conclusions Even though the retrospective nature of this survey and the relatively small sample size represent major limitations, these data indicate that treatment with nivolumab is feasible with no unexpected toxicity and good efficacy in this cohort of patients. These results merit further confirmation by a larger prospective trial.

Pulsus alternans was observed in a child with left ventricular dysfunction secondary to systemic hypertension. The Echo-Doppler of supravalvular aorta and a simultaneous electrocardiographic trace (ECG) clearly demonstrated the mechanism of an apparent abrupt halving of pulse frequency compared with the ECG, similar to an electromechanical dissociation 2: 1. Besides the extreme pulsus alternans, a P wave and a PP cycle length change were also observed. Both mechanical and electrical alternans disappeared with afterload reduction by nitroprusside treatment.

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 (Societa 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.

ASYMPTOMATIC VENTRICULAR PREEXCITATION IN CHILDREN OBJECTIVE: This retrospective study was planned for a good risk assessment of asymptomatic patients affected by ventricular pre-excitation. METHODS: From 1985 to 2007, 124 patients with an atrioventricular pathway (electrocardiographic signs of ventricular pre-excitation) were admitted to our cardiology division. The average age was 7 years (range 1 month to 18 years). The mean follow-up period in the whole population of patients was 4.2 years (range 1-13 years). Four patients were lost during the follow-up. During this period, all patients remained in good health. In all of them, we performed a Holter evaluation every year. An intermittent pathway was detected in 18 patients (15%), and four of them (3.4%) showed a supraventricular tachycardia even though they were asymptomatic patients. An ergometric test was performed in 76 asymptomatic patients; 16 children (21%) showed a total abrupt vanishing of delta wave. A transoesophageal electrophysiological evaluation was performed in 14 patients. CONCLUSION: According to our data, asymptomatic Wolff-Parkinson-White syndrome in children has a good outcome during a short-term (4 years) follow-up. The usefulness of electrophysiological evaluation (in particular its predictive value) is uncertain.

Noncompaction of the ventricular myocardium (LVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. In 2002 Jenni et al. [Jenni R, Wyss CA, Oechslin EN, Kaufmann PA. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol 2002; 39:450-454.] reported a microvascular dysfunction in 12 patients affected by non compaction: areas of restricted myocardial perfusion have been documented by scintigraphy, suggesting a reduction of Coronary flow reserve. McMahon et al reported in a recent article a reduction of TD velocities in children with noncompaction of the left ventricle, compared with normal controls. The authors concluded their work saying that the reduction of lateral mitral Ea velocity helps to predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation. In a precedent report our group reported a strong correlation between pathological tissue Doppler and reduction of ejection fraction. Recently we scanned with a Signa HD 1.5 T (GE, Milwaukee, USA) 8 patients affected by non compaction. Transmural Gd-enhancement was detected in 5/8 patients (62%). In all patients with late enhancement a reduction of EF has demonstrated. In our opinion the late enhancement can depend on a CFR, and is the determinant of the tissue Doppler alterations. So the TD alteration is associated with EF, and is an indirect index of poor clinical outcome, like EF.

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.

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. © 2008 Italian Federation of Cardiology.

Background: Isolated left ventricular noncompaction is the result of incomplete myocardial morphogenesis, leading to persistence of the embryonic myocardium. The condition is recognised by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricle. Whether these intertrabecular recesses are a favorable substrate for supraventricular arrhythmias is unclear, even if the incidence of chronic heart failure seems to be high. Results: We evaluated a continuous series of 238 patients affected by noncompaction. In 4 cases the patients reported palpitations and in 4 an episode of syncope. Periodic holter monitoring was performed every 6 months for 4 years. Only 9 patients had documented atrial fibrillation. In no cases we observed supraventricular tachycardia. Conclusions: Noncompaction alone does not seem to be a risk factor for supraventricular arrhythmias. © 2007 Elsevier Ireland Ltd. All rights reserved.

The Tissue doppler analysis is a new echocardiographic approach to evaluate the diastolic function. In this report we performed a Tissue doppler analysis in a series of 15 children affected by non-compaction. The bidimensional echo-cardiogram showed a systolic function which was reduced only in 8 patients. Alterations of the diastolic function were founded in 7 patients: in 2 cases, a reduction of the E tissue wave was present in all segments. In 3 patients the diastolic dysfunction was limited to apical and lateral segments. In the last 2 children a reduction of the E wave interested only the apical segments. There was a strong correlation between systolic and diastolic dysfunction: in fact all patients with diastolic dysfunction also presented a severe reduction of the 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.

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. © 2007 Italian Federation of Cardiology.