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The electrocardiograms and the vectocardiograms of 26 childrens, up to 3 years of age, affected by A-V canal, were been analyzed. The pattern of QRS loop in the 3 planes, the voltage of right maximum spatial vector (RMSV) and left maximum spatial vector (LMSV), their projection onto the horizontal (azimuth) and frontal plane (elevation) were related to right ventricular systolic pressure. The qualitative and quantitative evaluation of VCG Showed a better correlation with the haemodynamic data than the electrocardiogram. In all but 3 cases, the high frequency of superior orientation and counterclockwise rotation of QRS loop in the frontal plane was found; moreover the good correlation of right ventricular systolic pressure to the rotation of QRS loop on the horizontal plane was also confirmed. In fact, the latter was counterclockwise in the cases with a low pressure, whereas it became clockwise in those with higher pressure. Moreover a good direct correlation of the right ventricular pressure with forward orientation of LMSV on the horizontal plane was found. In differential diagnoses with other congenital heart diseases with superior orientation and anticlockwise rotation on the frontal plane, useful results were obtained by using the calculation of elevation time (the interval between point 0 and intersection of the QRS loop with the axis) which is significantly lower in the A-V canal. Terminal forces directed posteriorly and to the right and with a delay no longer than 0,03 inches do not warrant the diagnosis of left anterior hemiblock with a right bundle branch block associated. On the contrary, on the basis of anatomical and electrophysiologic studies we believe that in this disease there is an asinchrony in the activation i.e. the postero-inferior region, then the left lateral wall and finally the tree high part of right ventricle wall are sequentially activated.

36 patients, less than two years old, affected by Fallot's tetralogy were studied by vectorcardiography. The configuration of the QRS loop in the three orthogonal planes, the voltage of the 0.10 sec spatial vector, RMSV and LMSV vectors, and their azimuth and elevation were evaluated. Such data have been correlated to arterial oxygen saturation. Qualitative analysis showed a clockwise or figure eight QRS loop on the H and F planes in the great majority of cases; and only in a counterclockwise loop on the H plane was the suspicion of an arterial oxygen saturation greater than 85% especially in cases older than two months. The terminal forces of the QRS loop on the H plane were always directed under the O point, Whereas in the valvular pulmonary stenosis, terminal forces are generally superior to the O point. Quantitative analysis demonstrated the constant increase of the RMSV and the direct relationship of the LMSV to arterial oxygen saturation, and so it was the principal parameter for evaluation of the left ventricular volume and the size of pulmonary flow and the degree of pulmonary stenosis. The relationship between the azimuth of 0.01 sec spatial vector to O2 saturation was highly significant, showing a progressive anterior development of the former, as the latter increased.

The feasibility of atrial septostomy through the ombelical vein is discussed. The advantages and the pitfalls of this technique are examined. On the basis of our experience with 8 cases thus treated in the last year, it is concluded that a careful use of this technique in patients under six days of age is indicated.

53 patients affected by congenital valvular or discrete aortic stenosis have been evaluated in order to compare the observed gradient with the one expected according to multifactorial analysis based on electrocardiographic and auscultatory data deviced by Ellison et al. There Authors employed this formula for the evaluation of the valvular form only. In our experience all cases of valvular stenosis showing an estimated gradient less than or equal to 40 mmHg were found to have a gradient less than or equal to 40 mmHg at the catheterization. This statement is true in the discrete form as well, only if the estimated gradient was less than or equal to 30 mmHg. The regression curve between the two gradients shows a r = 0.718 (p less than 0.01); an even better correlation was found in the valvular forms (r = 0.816; p less than 0.01). These results are very useful since they allow to restrict indication to catheterization only to the cases in which the obstruction is estimated to necessitate a surgical intervention.

Contrast echocardiography is described as an important technique for the diagnosis of the anomalies of the venous system. Six patients with intrahepatic interruption of the inferior vena cava (IVC), ten patients with persistent left superior vena cava (PLSVC) connecting to coronary sinus (CS), one patient with PLSVC connecting to left atrium (LA) and ten patients, control group, with normal venous connections were studied by two-dimensional echocardiography; the injections of dextrose were made into the left hand, right hand, leg; the locations of the transducer were: subcostal position, parasternal and suprasternal. All patients underwent cardiac catheterization and cineangiography. When the injection was made into a leg vein, in the patients with infrahepatic interruption of the IVC we observed the contrast medium descending from the superior vena cava into the right atrium (RA). In the cases with PLSVC connecting to CS, contrast medium injected into the left hand, the sequential clouding of CS, RA and right ventricle was seen. The pattern of opacification in the case of PLSVC connecting to LA is discussed. In conclusion, contrast echocardiographic study of the connections of the venous system may be useful in planning the way for cardiac catheterization and also for the diagnosis of some anomalies which might be mistaken even at the time of the hemodynamic study.

Balloon aortography is a new technique for the angiographic study of pulmonary atresia with ventricular septal defect. A Swan-Ganz angiographic catheter is employed, whose balloon is inflated to stop blood flow in the descending aorta, thus ensuring the opacification of the pulmonary circulation. By this method we have studied 18 patients. Anatomic details of the pulmonary arterial circulation were obtained in all. No complications occurred. Balloon aortography is a simple and safe technique for the routine study of patients with pulmonary atresia and ventricular septal defect, whereas selective injection in collateral arteries or pulmonary veins, which carries more risk and is more complex, should be adopted only in selected cases.

Over the last decades, there has been a significant increase in incidence and prevalence of heart failure, a major cause of cardiac morbidity and mortality. Measurements of neurohormones, in particular B-type natriuretic peptide (BNP), can significantly improve diagnostic accuracy, and also correlate with long-term morbidity and mortality in patients with chronic heart failure presenting to the emergency department. BNP is secreted by cardiac ventricles mainly in response to wall stress and neurohormonal factors like the sympathetic nervous system, endothelins, and the rennin-angiotensin-aldosterone system. BNP increases myocardial relaxation and oppose the vasoconstrictive, sodium retaining, and natriuretic effects caused by vasoconstrictive factors. BNP is the first biomarker to prove its clinical value for the diagnosis of left ventricular systolic and diastolic dysfunction but also for the right ventricular dysfunction, guiding prognosis and therapy management. Emerging clinical data will help further refine biomarker-guided therapeutic and monitoring strategies involving BNP.

Purpose The present study aimed to assess the impact of the ketogenic diet on arterial morphology and endothelial function of the big vessels of the neck and on cardiac diastolic function, in a cohort of epileptic children and young adults treated with the ketogenic diet. Methods Patients were recruited based on the following inclusion criteria: (1) patients who were or had been on the ketogenic diet for a time period of at least six months. Each patient underwent measurement of carotid intima media thickness, carotid artery stiffness, echocardiography, and diastolic function assessment. Patients with drug resistant epilepsy, matched for number, age and sex and never treated with ketogenic diet, were recruited as controls. Results The population study was composed by 43 epilepsy patients (23 males), aged between 19 months and 31 years (mean 11 years). Twenty-three patients were or had been treated with ketogenic diet, and 20 had never been on it (control group). Subjects treated with the ketogenic diet had higher arterial stiffness parameters, including AIx and β-index and higher serum levels of cholesterol or triglycerides compared to those who had never been on the diet (control group) ( p Conclusions Arterial stiffness is increased in children and young adults treated with the ketogenic diet, before the increase of the intima media thickness. This supports that arterial stiffness is an early marker of vascular damage.

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Background The closed-loop stimulation (CLS) pacemaker algorithm is a system that permanently monitors the contractile state of the myocardium and converts the intrinsic information into rate regulation. The role that the CLS algorithm plays in the prevention of syncope recurrences still remains unclear. The aim of our prospective, randomised, single-blind, crossover study was to evaluate the effect of dual-chamber CLS in the prevention of syncope recurrence in patients with refractory vasovagal syncope (VVS) and a cardioinhibitory response to head-up tilt test (HUT) during a 36 months follow-up. Method sand results We studied 50 patients (mean age 53±5.1; 33 male) with the indication for permanent dual-chamber cardiac pacing for HUT-induced vasovagal cardioinhibitory syncope. They were randomised after 1 month of stabilisation period to CLS algorithm features programmed OFF or ON for 18 months each, using a crossover design. The number of syncopal and presyncopal episodes during active treatment was lower than those registered during no treatment (n syncopal episodes: 2 vs 15; p=0.007; n presincopal episodes: 5 vs 30; p = 0.004). Lead parameters remained stable over time, and there were no lead-related complications. Conclusions Based on these 36 months follow-up data, it is concluded that dual-chamber CLS is an effective algorithm for preventing syncope recurrences in healthy patients with tilt-induced vasovagal cardioinhibitory syncope.

Aims Prevalence and clinical significance of right atrial enlargement (RAE) has been poorly characterized in hypertrophic cardiomyopathy.Methods One hundred and sixty consecutive patients with hypertrophic cardiomyopathy (35.5 +/- 20 years; 64% men) were studied. They underwent clinical examination, standard ECG, M-mode, 2D and Doppler echocardiography, stress test and ECG Holter monitoring. Major adverse cardiac events were considered: cardiac death (sudden death, heart failure death); cardiac transplant; resuscitated cardiac arrest or appropriate implantable cardioverter defibrillator discharge. Genetic analysis of eight sarcomeric genes was performed using Sanger sequencing.Results RAE was observed in 22 patients (14%), associated with left atrial enlargement in all cases. Patients with RAE were likely to have restrictive mitral pattern (P < 0.001) and had higher New York Heart Association (P < 0.001), N-terminal prohormone of brain natriuretic peptide (P< 0.001), left atrial volume index (P < 0.001), lateral (P=0.04) and septal (P=0.002) E/e', systolic pulmonary artery pressure (P< 0.001) and lower ejection fraction (all P < 0.001). On cardiopulmonary exercise testing, peak VO2 was lower and VE/VCO2 higher in patients with RAE (P < 0.001). During a mean follow-up of 4 +/- 2.1 years, 30 major adverse cardiac events in 24 patients (15%) were observed. Cox proportional hazards regression analysis identified RAE as an independent predictor of major adverse cardiac events (odds ratio=2.6; confidence interval 1.5-4.6; P=0.001). In patients with RAE who were genetically tested, there was a higher prevalence of sarcomeric genemutations (68%), doublemutations (16%) and troponin T mutations (21%).Conclusion RAE is present in a small subset of patients with hypertrophic cardiomyopathy, and largely reflects increased pulmonary pressures because of severe diastolic and/or systolic left ventricular dysfunction. Patients with RAE had a higher prevalence of sarcomeric gene mutations, troponin T mutations and complex genotypes. In conclusion, RAE may serve as a very useful marker of disease progression and adverse outcome in patients with sarcomeric hypertrophic cardiomyopathy.

Enlargement and dysfunction of the right atrium might be an early sign for pulmonary hypertension in systemic sclerosis (SSc). This is the first study to analyse right atrial morphology and function in SSc patients compared to healthy controls by speckle-tracking two-dimensional strain echocardiography (2DSE) at rest and during exercise. Furthermore, right atrial function was correlated with further clinical findings. Adult patients with SSc for > 3 years (n = 90) and 55 age- and gender-matched healthy controls underwent a panel of non-invasive assessments including transthoracic echocardiography, pulsed Doppler myocardial imaging and 2DSE at rest and during exercise. Furthermore, serological tests and high-resolution chest computed tomography were performed. SSc patients showed significant impairment of right atrial function and the right atrial enlargement, measured by 2DSE at rest and during exercise compared to controls (both p < 0.001). These findings were more evident in SSc patients with pulmonary fibrosis (p < 0.001) and in patients with high pulmonary artery systolic pressures (PAPs) during exercise. In the SSC patients, right atrial lateral strain was significantly associated with PAPs during effort, right atrial area, left ventricle stroke volume and inferior vena cava diameter using multivariable analysis. The findings of this study suggest that a high proportion of SSc patients reveal right atrial dysfunction even without manifest pulmonary hypertension. Impaired right atrial function occurred mostly in patients with pulmonary fibrosis and/or elevated PAPs during exercise, was independently associated with prognostic factors and may therefore be useful for risk stratification. Further studies are needed to analyse if right atrial dysfunction assessed by 2DSE may help to improve early diagnosis of pulmonary hypertension.

Takotsubo syndrome (TTS) is an enigmatic disease with a multifactorial and still unresolved pathogenesis. A genetic predisposition has been suggested based on the few familial TTS cases. Conflicting results have been published regarding the role of functional polymorphisms in relevant candidate genes, such as α1-, β1-, and β2-adrenergic receptors; G protein-coupled receptor kinase 5; and estrogen receptors. Further research is required to help clarify the role of genetic susceptibility in TTS.

To test whether two-dimensional longitudinal strain (2DSE) performed after revascularization by percutaneous coronary intervention (PCI) could predict left ventricular (LV) remodeling in patients with recent non-ST elevation myocardial infarction (NSTEMI).In 70 patients (62.7 ± 8.7 years) with recent NSTEMI (between 72 hours and 14 days), undergoing coronary angiography for recurrent angina, myocardial deformation parameters were measured by 2DSE before and 24 hours after reperfusion therapy. Strain in all LV segments was averaged to obtain a global value (Global longitudinal Strain--GLS). Infarct size was estimated by clinical parameters and cardiac markers. After 6 months from intervention, LV negative remodeling was defined as lack of improvement of LV function, with increase in LV end-diastolic volume of greater than or equal than 15%.At follow-up, patients were subdivided into remodeled (n=32) and non-remodeled (n = 38) groups. Patients with negative LV remodeling had significantly lower baseline LV ejection fraction (44.8±6.9 vs. 48.7 ± 5.5 %; p0.05), higher peak troponin I (p0.001) and reduced GLS (- 10.6±6.1 vs - 17.6 ± 6.7 % p0.001) than those without LV remodeling. GLS showed a close correlation with peak troponin I after PCI (r = 0.64, P0.0001) and LV WMSI (r = 0.42, p0.01). By multivariable analysis, diabetes mellitus (P0.005), peak of Troponin I after PCI (P0.0005), GLS at baseline (OR: 4.3; p0.0001), and lack of improvement of GLS soon after PCI (OR: 1.45, P0.01) were powerful independent predictors of negative LV remodelling at follow-up. In particular, a GLS ≤ 12 % showed a sensitivity and a specificity respectively of 84.8% and 87.8% to predict negative LV remodelling at follow-up.in patients with recent NSTEMI, longitudinal LV global and regional speckle-tracking strain measurements are powerful independent predictors of LV remodeling after reperfusion therapy.

Aims Left ventricular (LV) twist and untwist play a major role in LV mechanics. We sought to acquire new pathophysiological insights in cardiomyopathies (CM) studying LV twist dynamics by speckle tracking imaging (STI). Methods and results Standard echo-Doppler and STI study were performed in 67 CM patients divided in four age- and sex-matched subgroups: 18 with apical hypertrophic cardiomyopathy (Group A); 20 with asymmetrical hypertrophic cardiomyopathy (Group B); 15 with dilated cardiomyopathy (Group C); 14 with LV non-compaction (Group D). As controls, 34 age- and sex-matched normal subjects were studied. Compared with control group, all CM Groups showed significantly lower longitudinal, circumferential, and radial myocardial deformations ( P < 0.05). LV twist was correlated with ejection fraction (EF; r = 0.62; P < 0.0001). Furthermore, all CM patients had a significantly lower twist rate ( P < 0.05) and delayed onset of untwist ( P < 0.01). Of interest a significant correlation was found between isovolumic relaxation time and untwist onset ( r = 0.485, P < 0.0001). In addition, a significant correlation was found between longitudinal deformations and the onset of untwist (strain: r = 0.46, P = 0.0001; strain rate: r = 0.33, P = 0.0056) and between longitudinal strain rate and twisting rate ( r = −0.38; P = 0.0015). Conclusion (i) All CM patients show an impairment of longitudinal, circumferential, and radial myocardial deformations; (ii) LV peak twist is impaired only in CM with reduced EF but preserved in those with normal or increased EF; (iii) LV twist is prolonged and untwisting onset is delayed in all CM, suggesting that a mechanical adaptation to subclinical systolic abnormalities might induce, by a prolonged LV twist, the early onset of diastolic dysfunction.

We report the case of a 16-year-old boy with cardiomyopathy induced by inappropriate sinus tachycardia (IST). The patient was resistant to treatment with conventional rate-decreasing medications. Therapy with the selective sinus node I(f) current inhibitor ivabradine was started. After 3 months of ivabradine therapy, an improvement in ejection fraction and a successful decrease in heart rate were observed. No side effects occurred. We suggest that ivabradine, currently used to treat stable angina, could be considered as a second-line treatment in patients with symptomatic and refractory IST.

BACKGROUND: The aim of the study was to detect if right ventricular (RV) ejection fraction assessed by real-time 3-dimensional echocardiography (RT3DE) could predict patients with dilated cardiomyopathy (DCM) with greater functional impairment in response to cardiopulmonary exercise. METHODS AND RESULTS: Seventy chronic heart failure patients with DCM (55.5 ± 9.1 years; 48 males; 30 ischemic; New York Heart Association Class III: 48) underwent both left ventricular (LV) and RV analysis by RT3DE. Postprocessing software provided data of RT3DE systolic dyssynchrony index of 16 LV segments (systolic dyssynchrony index [SDI]) and of both LV and RV ejection fraction. Cardiac magnetic resonance was performed in a subgroup of 40 DCM patients to confirm RT3DE measurements. All the patients underwent also bicycle cardiopulmonary exercise test with evaluation of oxygen consumption (VO2) peak% (percentage of the predicted value), VE/VCO2 slope, and circulatory power (CP). Mean LV ejection fraction was 29.8 ± 4.6%. RT3DE LV SDI index was 8.4.4 ± 4.2, and RV ejection fraction was 51.3 ± 4.6%. By cardiopulmonary test, mean VO2 peak was 15.2 ± 4.4 mL·kg·min, and mean CP was 2.1 ± 0.8. By univariable analyses, significant correlations were detectable between SDI index and VO2 peak% (r = -0.56; P < .0001) and peak CP (r = -0.48; P < .0005). Also RV ejection fraction directly correlated with VO2 peak% (r = 0.58; P < .0001) and inversely with VE/VCO2 slope (r = -0.44; P < .001). By multivariable analysis, SDI index (β coefficient = -0.46; P < .001) and 3D RV ejection fraction (β coefficient = 0.42; P < .001) emerged as the only independent determinants of VO2 peak% during cardiopulmonary test. CONCLUSIONS: Increased LV electromechanical dyssynchrony and impaired RV function in DCM patients are independently associated with worse ability to perform aerobic exercise.

An unusual case of two different types of atrial arrhythmia after orthotopic heart transplantation is reported: a macro-reentrant atrial tachycardia in the recipient atrium, and a common-type, counterclockwise, isthmus-dependent atrial flutter in the donor atrium. The clinical symptoms were caused by atrial flutter arising from the donor atrium. Radiofrequency catheter ablation of the electrically active, donor-derived portion of the cavotricuspid isthmus was performed successfully.

Contrast-induced nephropathy (CIN) is most commonly defined as acute renal failure occurring within 48-72 h of exposure to an intravascular radiographic contrast medium that is not attributable to other causes. In the international literature, a 25% increase in serum creatinine levels or an increase in absolute values of 0.5 mg/dl from baseline has been suggested to define CIN. The reported incidence of CIN varies widely, ranging from 2 to 50%. This variability results from differences in the presence or absence of risk factors. With a retrospective analysis we evaluated the use of saline hydration plus N-acetyl cysteine (NAC) to prevent CIN in a low-risk population of patients undergoing coronary artery angiography compared with an historic low risk group not treated. From January 2009 to December 2009, 152 consecutive patients who underwent coronary artery angiography with a low osmolarity contrast agent were enrolled in our study, and compared with an historic control group consisting of 172 low-risk patients. Nephrotoxic drugs such as diuretics, ACE-I and ARBs were stopped at least 24 h before the procedure. Inclusion criteria to define low-risk population were the absence of: diabetes, age >65 years, or baseline creatinine >1.4 mg/dl. We have treated group A (152 patients, 47.3%) with a saline hydration (1 ml/kg/h) plus N-acetyl cysteine 600 mg 12 h before and 12 h after the procedure; group B (group control of 170 patients, 52.7%) were not treated. The overall incidence of CIN was 7.1% (23 patients). In particular, the incidence of CIN was 2.6% (4 patients) in the group A and 11.2% (19 patients) in the group B (p = 0.002). In the multivariate analysis, including risk factor such as age, hypertension, hypercholesterolemia, current smoking habit baseline creatinine level, contrast index and hydration, the last variable was the only one inversely correlated independently with the incidence of CIN (p = 0.001). In conclusion, intravenous hydration with saline and NAC is an effective and low cost tool in preventing CIN in patients undergoing coronary artery angiography, and, according to the current guidelines, should be used in all high-risk patients for CIN. Our results show that even in patients at low risk, hydration with saline 0.9% plus NAC is useful and significantly reduces the incidence of CIN.

BACKGROUND: The aim of this study was to define the range of left ventricular (LV) velocities and deformation indexes in highly trained athletes, analyzing potential differences induced by different long-term training protocols. METHODS: Standard echocardiography, pulsed-wave tissue Doppler echocardiography, and two-dimensional strain echocardiography of the interventricular septum and lateral wall were performed in 370 endurance athletes and 280 power athletes. Using pulsed-wave tissue Doppler, the following parameters of myocardial function were assessed: systolic peak velocities (S(m)), early (E(m)) and late (A(m)) diastolic velocities, and the E(m)/A(m) ratio. By two-dimensional strain echocardiography, peaks of regional systolic strain and LV global longitudinal strain were calculated. RESULTS: LV mass index and ejection fraction did not significantly differ between the two groups. However, power athletes showed an increased sum of wall thicknesses (P < .01) and relative wall thickness, while LV stroke volume and LV end-diastolic diameter (P < .001) were greater in endurance athletes. By pulsed-wave tissue Doppler analysis, E(m) and E(m)/A(m) at both the septal and lateral wall levels were higher in endurance athletes. By two-dimensional strain echocardiography, myocardial deformation indexes were comparable between the two groups. E(m)/A(m) ratios ≥ 1 were found in the overall population, while 90 % of athletes had an E(m) ≥ 16 cm/sec, S(m) ≥ 10 cm/sec, and global longitudinal strain ≤ -16%. Multivariate analyses evidenced independent positive association between Em peak velocity and LV end-diastolic volume (P < .001) and an independent correlation of global longitudinal strain with the sum of LV wall thicknesses (P < .005). CONCLUSIONS: This study describes the full spectrum of systolic and diastolic myocardial velocities and deformation indexes in a large population of competitive athletes.

Background Recent data show that there is an unexpectedly high prevalence of ‘inappropriate’ pulmonary responses to exercise among patients with systemic sclerosis (SS). However, no consensus exists as to which threshold of pulmonary artery systolic pressure (PASP) can be considered diagnostically relevant. Aim To evaluate pulmonary vascular reserve and right ventricular function changes induced by exercise in SS patients without overt pulmonary arterial hypertension. Methods and results The study enrolled 172 consecutive SS patients in NYHA class IeII, with a peak tricuspid regurgitant jet velocity at echocardiography not greater than 3 m/s, and 88 control subjects. Echocardiography was performed at rest and at the end of a maximal exercise test. SS patients showed a higher exercise-induced PASP than control subjects (36.968.7 vs 25.963.3 mm Hg, p¼0.00008). The response to effort was higher in the presence of moderate interstitial lung disease (39.769.3 vs 36.068.4 mm Hg, p¼0.016) or subclinical left ventricular diastolic dysfunction (42.365.8 vs 37.068.6 mm Hg, p¼0.015). In control subjects, PASP values were normally distributed at rest and after exercise. In SS patients, the distribution was normal at rest but bimodal after exercise, with a second peak at 52.2 mm Hg including 13% of the total SS population. Patients in this subgroup showed subtle abnormalities of right ventricular function at rest and, most importantly, a blunted increase in right ventricular systolic function with exercise. Conclusion Exercise echocardiography may identify a subset of SS patients with an inappropriate exercise-induced increase in PASP and early signs of right ventricular dysfunction.