Gabriele Rinelli - Academia.edu (original) (raw)

Papers by Gabriele Rinelli

Research paper thumbnail of Left Ventricular Mass Indexation In Infants, Children And Adolescents: A Simplified Approach For The Identification Of Left Ventricular Hypertrophy In Clinical Practice

Research paper thumbnail of Advanced Parameters of Cardiac Mechanics in Children with CKD: The 4C Study

Clinical journal of the American Society of Nephrology : CJASN, Jan 24, 2015

Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult... more Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult patients with CKD. The aim of this study was to identify prevalence of subclinical abnormalities in cardiac function through the analysis of novel indices of cardiac mechanics in a large population of children with CKD. Between 2009 and 2011, the prospective observational Cardiovascular Comorbidity in Children with CKD Study enrolled patients with CKD ages 6-17 years old with eGFR=10-45 ml/min per 1.73 m(2) in 14 European countries. Cardiac morphology and function were assessed through echocardiography. The analysis presented encompasses global radial, longitudinal, and circumferential strains as well as time to peak analysis. Data were compared with 61 healthy children with comparable age and sex. Data on 272 patients with CKD with complete echocardiographic assessment are reported (age =12.8±3.5 years old; 65% boys). Patients with CKD showed mildly higher office BP values and higher p...

Research paper thumbnail of Staged Fontan operation for complex cardiac anomalies with subaortic obstruction

The Journal of thoracic and cardiovascular surgery, 1993

Ventricular hypertrophy is a recognized risk factor for the Fontan operation in cases of complex ... more Ventricular hypertrophy is a recognized risk factor for the Fontan operation in cases of complex cardiac anomalies with unrestricted pulmonary blood flow and subaortic obstruction. Between 1986 and 1991 we have treated 23 such patients with a new type of palliation combining a main pulmonary artery-ascending aorta anastomosis with a bidirectional cavopulmonary anastomosis. Their ages averaged 57 +/- 36 months (7 to 155 months). Nine patients had (S,L,L) single ventricule with transposition and the other 14 had more complex cardiac anomalies unsuitable for biventricular repair. Subaortic obstruction was defined by a hemodynamic criterion (n = 6) or by a morphologic criterion (n = 17). There were five hospital deaths (21.7%): All three patients older than 7 years of age died (p = not significant). Follow-up averaged 28 +/- 21 months (range 1 to 58 months). Among the 18 hospital survivors, the proportions surviving 1 and 5 years after the operation were 78% and 63%, respectively. Contr...

Research paper thumbnail of Impact of Three-Dimensional Echocardiography in Complex Congenital Heart Defect Cases: The Surgical View

Pediatric Cardiology, 2009

Other authors have demonstrated the ability of three-dimensional (3D) echocardiography to produce... more Other authors have demonstrated the ability of three-dimensional (3D) echocardiography to produce ''en face'' views of anomalies such as atrioventricular valve disease and atrial and ventricular septal defects. Few data exist about the usefulness of 3D images for more complex congenital heart defects and the surgical impact of this relatively new technology. This study, covering a period of 8 months and including 43 young patients affected by complex congenital heart defects, demonstrated that the routine use of 3D echocardiography is feasible and valuable for some types of cardiac defects. In fact, 3D images have provided more detailed anatomic definition of interrelations between structures in about one-third (15/43) of our cases, yielding new insight into the anatomy analogous to what can be derived from examining a heart specimen. Our surgeons found the 3D images particularly helpful for providing a realistic and almost specimen-like preview of the surgical anatomy that facilitates planning of the surgical program.

Research paper thumbnail of Closed Loop Stimulation Improves Ejection Fraction in Pediatric Patients with Pacemaker and Ventricular Dysfunction

Pacing and Clinical Electrophysiology, 2007

The aim of this prospective study was to evaluate the effect of the closed loop stimulation (CLS)... more The aim of this prospective study was to evaluate the effect of the closed loop stimulation (CLS) on the ejection fraction in pediatric patients, affected by complete atrioventricular block (CAVB) or CAVB and sinus node dysfunction (SND), with a previously implanted pacemaker (PM) and ventricular dysfunction. The role of electrical therapy in the treatment of pediatric patients with congenital atrioventricular (AV) blocks has been shown. Conventional right ventricular pacing seems to affect ventricular function. Up to now, the feasibility and the long-term results of biventricular pacing in pediatric patients were not entirely clear. In eight pediatric patients with a previously implanted single or dual chamber PM, ventricular dysfunction, and CAVB or SND and CAVB, a dual chamber PM INOS(2+)-CLS (Biotronik GmbH, Berlin, Germany) was implanted. The effect of the physiological modulation of CLS pacing mode on the ejection fraction was evaluated by Echo-Doppler examination. Measurements were performed before the substitution of the old PM and for up to 2 years of follow-up. All patients showed correct electrical parameters at implantation and during follow-up. The mean value of the ejection fraction measured before the replacement of the old PM was 36 +/- 7%, while after 2 years it was 47 +/- 1% (P < 0.003). No patient showed any worsening of the ejection fraction, while only one showed no improvement. DDD-CLS pacing seems to improve ventricular function in pediatric patients with CAVB and/SND in spite of the use of the apical right conventional stimulation.

Research paper thumbnail of Left Ventricular Geometry in Children with Mild to Moderate Chronic Renal Insufficiency

Journal of the American Society of Nephrology, 2005

Left ventricular hypertrophy (LVH) is the most important independent marker of cardiovascular ris... more Left ventricular hypertrophy (LVH) is the most important independent marker of cardiovascular risk in adults with chronic kidney disease. Cardiovascular morbidity seems increased even in children with chronic renal insufficiency (CRI), but the age and stage of CRI when cardiac alterations become manifest are unknown. For assessing the prevalence and factors associated with abnormal LV geometry in children with CRI, echocardiograms, ambulatory BP monitoring, and biochemical profiles were obtained in 156 children aged 3 to 18 yr with stages 2 through 4 chronic kidney disease (GFR 49 ؎ 19 ml/min per 1.73 m 2 ) and compared with echocardiograms obtained in 133 healthy children of comparable age and gender. LV mass was indexed to height 2.7 . Concentric LV remodeling was observed in 10.2%, concentric LVH in 12.1%, and eccentric LVH in 21% of patients. LVH was more common in boys (43.3 versus 19.4%; P < 0.005). Probability of LVH independently increased with male gender (odds ratio [OR] 2.62; P < 0.05) and standardized body mass index (OR 1.56; P ‫؍‬ 0.01). Low hemoglobin, low GFR, young age, and high body mass index were independent correlates of LV mass index (0.005 < P < 0.05). LV concentricity (relative wall thickness) was positively associated with serum albumin (P < 0.05). Probability of abnormal LV geometry increased with C-reactive protein >10 mg/dl (OR 26; P < 0.001). In conclusion, substantial cardiac remodeling of both concentric and eccentric type is present at young age and early stages of CRI in children. Prevalence of LVH is related to male gender, anemia, and ponderosity but not to BP. Additional effects of volume status and inflammation on cardiac geometry are also evident.

Research paper thumbnail of Aberrant Left Innominate Artery From the Left Descending Aorta in Right Aortic Arch: Echocardiographic Diagnosis

Journal of the American Society of Echocardiography, 2010

A right aortic arch with a left descending aorta and an aberrant left innominate artery is a rare... more A right aortic arch with a left descending aorta and an aberrant left innominate artery is a rare but recognized vascular anomaly that can result in compression of the trachea and the esophagus. This vascular anomaly has been diagnosed using cardiac catheterization and angiography. Recently, computed tomography and magnetic resonance imaging have been used for noninvasive diagnosis. The aim of this report is to highlight the possibility of echocardiographic diagnosis.

Research paper thumbnail of Minimally invasive or interventional repair of atrial septal defects in children: experience in 171 cases and comparison with conventional strategies

Journal of the American College of Cardiology, 2001

The goal of this study was to evaluate percutaneous interventional and minimally invasive surgica... more The goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children. BACKGROUND Concern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization.

Research paper thumbnail of Integrated Backscatter in Becker Muscular Dystrophy Patients With Functionally Normal Heart: Myocardial Ultrasound Tissue Characterization Study

Journal of the American College of Cardiology, 2006

Research paper thumbnail of ADVANCED PARAMETERS OF CARDIAC MECHANICS IN CHILDREN WITH CHRONIC KIDNEY DISEASE: THE 4C STUDY

Journal of the American College of Cardiology, 2014

Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult... more Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult patients with CKD. The aim of this study was to identify prevalence of subclinical abnormalities in cardiac function through the analysis of novel indices of cardiac mechanics in a large population of children with CKD. Between 2009 and 2011, the prospective observational Cardiovascular Comorbidity in Children with CKD Study enrolled patients with CKD ages 6-17 years old with eGFR=10-45 ml/min per 1.73 m(2) in 14 European countries. Cardiac morphology and function were assessed through echocardiography. The analysis presented encompasses global radial, longitudinal, and circumferential strains as well as time to peak analysis. Data were compared with 61 healthy children with comparable age and sex. Data on 272 patients with CKD with complete echocardiographic assessment are reported (age =12.8±3.5 years old; 65% boys). Patients with CKD showed mildly higher office BP values and higher p...

Research paper thumbnail of Change in Cardiac Geometry and Function in CKD Children During Strict BP Control: A Randomized Study

Clinical Journal of the American Society of Nephrology, 2013

Left ventricular hypertrophy (LVH) and abnormal systolic function are present in a high proportio... more Left ventricular hypertrophy (LVH) and abnormal systolic function are present in a high proportion of children with CKD. This study evaluated changes in left ventricular (LV) geometry and systolic function in children with mild to moderate CKD as an ancillary project of the Effect of Strict Blood Pressure Control and ACE Inhibition on Progression of Chronic Renal Failure in Pediatric Patients trial. Echocardiograms and ambulatory BP monitoring were performed at baseline and at 1- or 2-year follow-up in 84 patients with CKD and 24-hour mean BP above the 50th percentile and/or receiving fixed high-dose angiotensin converting enzyme inhibition and randomized to conventional or intensified BP control. LVH prevalence decreased from 38% to 25% (P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.05). Changes in LV mass index (LVMI) were restricted to patients with LVH at baseline (-7.9 g/m(2.7); P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.02). Changes in LVMI were independent of randomization, reduction in BP, hemoglobin, and estimated GFR. A significant increase in midwall fractional shortening was observed in the total cohort (P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.05), and was greater in the intensified group compared with the conventional BP control group (12%±1.9% versus 8%±1.5%; P=0.05). In multivariate analysis, improvement in myocardial function was associated with reduction in BP (r=-0.4; P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.05), independently of LVMI reduction. In children with CKD, angiotensin converting enzyme inhibition with improved BP control, LVH regression, and improved systolic function was observed within 12 months. Lowering BP to the low-normal range led to a slightly more marked improvement in myocardial function but not in LVMI.

Research paper thumbnail of Association of DiGeorge anomaly and caudal dysplasia sequence in a neonate born to a diabetic mother

Cardiology in the Young, 2013

Several studies have demonstrated a significantly increased risk of specific patterns of congenit... more Several studies have demonstrated a significantly increased risk of specific patterns of congenital anomalies in infants born to diabetic mothers. In particular, caudal dysplasia sequence has been linked to pregnancy complicated by maternal diabetes. In addition, several cases of infants born to diabetic mothers presenting with features of DiGeorge anomaly have been reported. Infants with DiGeorge anomaly can display additional manifestations within the spectrum of caudal dysplasia sequence, including vertebral anomalies and renal agenesis. We report a neonate presenting with the co-occurrence of features of both DiGeorge anomaly and caudal dysplasia sequence, born to a mother with poorly controlled insulin-dependent diabetes. The patient was affected by truncus arteriosus type A1 and hypertrophic cardiomyopathy. Maternal diabetes can cause a spectrum of manifestations, expressing with isolated DiGeorge anomaly or caudal dysplasia sequence, with intermediate phenotypes or with the co-occurrence of both the congenital anomalies in the same patient. The present observations argue for a feasible link between truncus arteriosus with hypertrophic cardiomyopathy, DiGeorge anomaly, and maternal diabetes.

Research paper thumbnail of New Findings concerning Cardiovascular Manifestations emerging from Long-term Follow-up of 150 patients with the Williams-Beuren-Beuren syndrome

Cardiology in the Young, 2009

We investigated the prevalence, type, and course of congenital cardiac defects and systemic hyper... more We investigated the prevalence, type, and course of congenital cardiac defects and systemic hypertension in our patients with Williams-Beuren-Beuren syndrome. We reviewed the clinical records of all patients with Williams-Beuren syndrome examined between 1981 and 2006. We identified 150 patients, aged from 7 months to 45 years, with a follow-up from 6 months to 25 years, the mean being 6.4 years. A cardiac anomaly was present in 113 of the 150 patients (75%). Defects were typical in over four-fifths of the group. We found supravalvar aortic stenosis in 73 of 113 patients (64.6%), isolated in 43. Pulmonary stenosis, isolated in 18 cases, was found in 51 of 113 (45.1%), while aortic coarctation and mitral valvar prolapse were each found in 7 (6.2%), 3 of the lesions is isolation. Atypical defects were found in 19 patients, tetralogy of Fallot in 2, atrial septal defects in 4, aortic and mitral valvar insufficiencies in 1 each, bicuspid aortic valves in 2, and ventricular septal defects in 9, 4 of the last being isolated. Systemic hypertension, observed in 33 patients (22%), was poorly controlled in 10. Diagnostic and/or interventional cardiac catheterization was undertaken in 24 patients, with 30 surgical procedures performed in 26 patients. Of the group, 3 patients died. Cardiac defects were present in three-quarters of our patients. Pulmonary arterial lesions generally improved, while supravalvar aortic stenosis often progressed. Atypical cardiac malformations, particularly ventricular septal defects, occurred frequently. Systemic hypertension was found in one-fifth, even in the absence of structural cardiac defects. The short-term mortality was low.

Research paper thumbnail of Granulicatella bacteraemia in children: two cases and review of the literature

BMC Pediatrics, 2013

Background: Granulicatella spp. is a fastidious bacteria responsible for bacteremia and endocardi... more Background: Granulicatella spp. is a fastidious bacteria responsible for bacteremia and endocarditis which are fatal in about 20% of the cases. These severe infections are uncommon in children under 17 years of age and have proven extremely difficult to treat. Cases presentation: We report a brief review of the literature and two cases of NVS bacteremia by Granulicatella complicated by infective endocarditis (IE). The first one is that of a 7-year-old Caucasian female with Shone syndrome and IE involving the pulmonary valve homograft, confirmed by echocardiography. The second case is that of a 5-year-old Caucasian male. In this patient echocardiogram was negative for signs of IE; however, a "possible" IE was suspected on the basis of a cardiac catheterization 3 weeks before the onset of fever. Since in both our patients clinical failure of first line antibiotic treatment was observed, we used a combination of meropenem with another anti-streptococcal drug with excellent results. Conclusion: In Granulicatella bacteremia in the pediatric population, combination antimicrobial therapy including meropenem should be considered as a second line treatment in non-responding patients.

Research paper thumbnail of Double-Outlet Right Atrium: Anatomic and Clinical Considerations

The Annals of Thoracic Surgery, 2007

Double-outlet right atrium (DORA) is a condition in which the right atrium empties into both vent... more Double-outlet right atrium (DORA) is a condition in which the right atrium empties into both ventricles. Described are the anatomic and clinical findings of this rare anomaly and our experience with its surgical treatment. A retrospective review was conducted of all medical records and two-dimensional echocardiographic assessments, and the operative reports of patients with DORA were reexamined to identify the specific anatomic features and the relative adaptation of surgical technique. From January 1983 to November 2004, 12 patients with DORA underwent surgical treatment. The diagnosis was made in all cases by two-dimensional echocardiography. All patients had mild-to-moderate signs of arterial oxygen desaturation. All patients also had either a partial (n = 11) or a complete (n = 1) atrioventricular canal with or without other associated cardiac anomalies, including persistent left superior vena cava. Eleven patients survived the surgical repair, the only death occurred in a patient with Ellis Von-Creveld syndrome, with multiple skeletal malformations, severe chest hypoplasia, and respiratory distress. DORA is a rare and peculiar cardiac anomaly with well-defined characteristics. Its recognition, best achieved by two-dimensional echocardiography, is crucial for a good surgical correction.

Research paper thumbnail of Systolic Ejection Murmurs in the Elderly: Aortic Valve and Carotid Arteries Echo-Doppler Findings

Angiology, 1991

Two-dimensional echographic and color Doppler studies of the heart and carotid arteries (CA) were... more Two-dimensional echographic and color Doppler studies of the heart and carotid arteries (CA) were performed in 45 patients greater than sixty-five years old without aortic stenosis, 23 with (Group 1) and 22 without (group 2) precordial ejection systolic murmur (SM). Aortic cusps thickening was found in 11 Group 1 (48%) and 2 Group 2 (9%) patients (p less than 0.001). Aortic root and aortic arch size were similar in the two groups. Maximum aortic flow velocity was significantly greater in Group 1 (200 60 cm/sec) than in Group 2 (120 20 cm/sec) (p less than 0.001). Left ventricular outflow systolic maximum velocity was similar in the two groups. A bilateral neck murmur was heard in 10/23 Group 1 patients (43%); in this group, patients with cervical SM had a greater maximum aortic flow velocity than those without cervical SM (230 + 60 cm/sec vs 172 + 32 cm/sec, p less than 0.001). In Group 1, 3 patients had a cervical SM louder on one neck side; only in these 3 patients were ipsilateral obstructive CA plaques found. A unilateral neck SM was heard in 4/22 Group 2 patients (18%); in these 4, ipsilateral obstructive CA were found. (1) in the elderly, precordial ejection SM is related to mild increase in maximum aortic flow velocity and thickening of aortic cusps; (2) in patients with precordial SM radiated to both neck sides, maximum aortic flow velocity tends to be more markedly increased; (3) in patients with precordial SM, a cervical SM louder on one neck side should suggest coexistent ipsilateral CA stenosis.

Research paper thumbnail of Pitfalls in Echocardiographic-Based Repair of Aortic Coarctation

The American Journal of Cardiology, 1997

The knowledge of echocardiographic pitfalls, which may impair the correct indication for surgery ... more The knowledge of echocardiographic pitfalls, which may impair the correct indication for surgery of aortic coarctation, is of great importance to pediatric cardiologists. We believe that only the suspicion of coronary artery anomalies is a clear indication to perform a cardiac catherization.

Research paper thumbnail of Comparison of three different atrial septal defect occlusion devices

The American Journal of Cardiology, 1998

Three different devices were used to close secundum-type atrial septal defects in 28 patients. Th... more Three different devices were used to close secundum-type atrial septal defects in 28 patients. The &quot;Amplatzer&quot; is associated with an easier and shorter procedure than are the &quot;Sideris Buttoned Device&quot; and the Microvena &quot;Angel Wings&quot; devices.

Research paper thumbnail of Abnormal intraventricular flow patterns in left ventricular dysfunction determined by color Doppler study

American Heart Journal, 1992

We examined the relation between left ventricular (LV) flow dynamics measured by color Doppler, a... more We examined the relation between left ventricular (LV) flow dynamics measured by color Doppler, and either global or regional LV function in 19 normal subjects (group 1), in 55 patients with old myocardial infarction (MI) (29 without [group 2] and 26 with LV aneurysm [group 3]), and in 16 with idiopathic dilated cardiomyopathy (group 4). We calculated by M-mode color Doppler a flow persistence index (FPI) (duration of flow directed in systole toward the apex/LV ejection time). Contrast echocardiography was performed as a control method in 14 patients of the four groups. In normal subjects, rapid systolic inversion of flow toward the aorta was evident (FPI: 0.11 +/- 0.16). In all but one patient in group 2, a similar LV flow pattern was observed, but FPI was greater (0.32 +/- 0.26). In groups 3 and 4, a paradoxical antegrade LV flow pattern was evident during the entire period of systole (FPI: 1.13 +/- 0.42 and 1.28 +/- 0.36, respectively). LV flow patterns were reproduced in echo-contrast studies. FPI was related to LV end-diastolic volume (r = 0.77), end-systolic volume (r = 0.82), and ejection fraction (r = -0.84). However, when data were analyzed separately in the different groups, these correlations were significant only in groups 2 and 3. Paradoxical flow pattern is not peculiar to regional LV dysfunction; it also occurs in global LV dysfunction. This LV flow abnormality may develop after MI even in the absence of severe LV dyssynergy or dilation, and is quantitatively related to the degree of LV dysfunction.

Research paper thumbnail of IMPAIRED CARDIAC MECHANICS IN A LARGE POPULATION OF CHILDREN WITH HEART TRANSPLANTATION: A SPECKLE TRACKING AND THREE-DIMENSIONAL ECHOCARDIOGRAPHY STUDY

Journal of the American College of Cardiology, 2014

Research paper thumbnail of Left Ventricular Mass Indexation In Infants, Children And Adolescents: A Simplified Approach For The Identification Of Left Ventricular Hypertrophy In Clinical Practice

Research paper thumbnail of Advanced Parameters of Cardiac Mechanics in Children with CKD: The 4C Study

Clinical journal of the American Society of Nephrology : CJASN, Jan 24, 2015

Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult... more Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult patients with CKD. The aim of this study was to identify prevalence of subclinical abnormalities in cardiac function through the analysis of novel indices of cardiac mechanics in a large population of children with CKD. Between 2009 and 2011, the prospective observational Cardiovascular Comorbidity in Children with CKD Study enrolled patients with CKD ages 6-17 years old with eGFR=10-45 ml/min per 1.73 m(2) in 14 European countries. Cardiac morphology and function were assessed through echocardiography. The analysis presented encompasses global radial, longitudinal, and circumferential strains as well as time to peak analysis. Data were compared with 61 healthy children with comparable age and sex. Data on 272 patients with CKD with complete echocardiographic assessment are reported (age =12.8±3.5 years old; 65% boys). Patients with CKD showed mildly higher office BP values and higher p...

Research paper thumbnail of Staged Fontan operation for complex cardiac anomalies with subaortic obstruction

The Journal of thoracic and cardiovascular surgery, 1993

Ventricular hypertrophy is a recognized risk factor for the Fontan operation in cases of complex ... more Ventricular hypertrophy is a recognized risk factor for the Fontan operation in cases of complex cardiac anomalies with unrestricted pulmonary blood flow and subaortic obstruction. Between 1986 and 1991 we have treated 23 such patients with a new type of palliation combining a main pulmonary artery-ascending aorta anastomosis with a bidirectional cavopulmonary anastomosis. Their ages averaged 57 +/- 36 months (7 to 155 months). Nine patients had (S,L,L) single ventricule with transposition and the other 14 had more complex cardiac anomalies unsuitable for biventricular repair. Subaortic obstruction was defined by a hemodynamic criterion (n = 6) or by a morphologic criterion (n = 17). There were five hospital deaths (21.7%): All three patients older than 7 years of age died (p = not significant). Follow-up averaged 28 +/- 21 months (range 1 to 58 months). Among the 18 hospital survivors, the proportions surviving 1 and 5 years after the operation were 78% and 63%, respectively. Contr...

Research paper thumbnail of Impact of Three-Dimensional Echocardiography in Complex Congenital Heart Defect Cases: The Surgical View

Pediatric Cardiology, 2009

Other authors have demonstrated the ability of three-dimensional (3D) echocardiography to produce... more Other authors have demonstrated the ability of three-dimensional (3D) echocardiography to produce ''en face'' views of anomalies such as atrioventricular valve disease and atrial and ventricular septal defects. Few data exist about the usefulness of 3D images for more complex congenital heart defects and the surgical impact of this relatively new technology. This study, covering a period of 8 months and including 43 young patients affected by complex congenital heart defects, demonstrated that the routine use of 3D echocardiography is feasible and valuable for some types of cardiac defects. In fact, 3D images have provided more detailed anatomic definition of interrelations between structures in about one-third (15/43) of our cases, yielding new insight into the anatomy analogous to what can be derived from examining a heart specimen. Our surgeons found the 3D images particularly helpful for providing a realistic and almost specimen-like preview of the surgical anatomy that facilitates planning of the surgical program.

Research paper thumbnail of Closed Loop Stimulation Improves Ejection Fraction in Pediatric Patients with Pacemaker and Ventricular Dysfunction

Pacing and Clinical Electrophysiology, 2007

The aim of this prospective study was to evaluate the effect of the closed loop stimulation (CLS)... more The aim of this prospective study was to evaluate the effect of the closed loop stimulation (CLS) on the ejection fraction in pediatric patients, affected by complete atrioventricular block (CAVB) or CAVB and sinus node dysfunction (SND), with a previously implanted pacemaker (PM) and ventricular dysfunction. The role of electrical therapy in the treatment of pediatric patients with congenital atrioventricular (AV) blocks has been shown. Conventional right ventricular pacing seems to affect ventricular function. Up to now, the feasibility and the long-term results of biventricular pacing in pediatric patients were not entirely clear. In eight pediatric patients with a previously implanted single or dual chamber PM, ventricular dysfunction, and CAVB or SND and CAVB, a dual chamber PM INOS(2+)-CLS (Biotronik GmbH, Berlin, Germany) was implanted. The effect of the physiological modulation of CLS pacing mode on the ejection fraction was evaluated by Echo-Doppler examination. Measurements were performed before the substitution of the old PM and for up to 2 years of follow-up. All patients showed correct electrical parameters at implantation and during follow-up. The mean value of the ejection fraction measured before the replacement of the old PM was 36 +/- 7%, while after 2 years it was 47 +/- 1% (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.003). No patient showed any worsening of the ejection fraction, while only one showed no improvement. DDD-CLS pacing seems to improve ventricular function in pediatric patients with CAVB and/SND in spite of the use of the apical right conventional stimulation.

Research paper thumbnail of Left Ventricular Geometry in Children with Mild to Moderate Chronic Renal Insufficiency

Journal of the American Society of Nephrology, 2005

Left ventricular hypertrophy (LVH) is the most important independent marker of cardiovascular ris... more Left ventricular hypertrophy (LVH) is the most important independent marker of cardiovascular risk in adults with chronic kidney disease. Cardiovascular morbidity seems increased even in children with chronic renal insufficiency (CRI), but the age and stage of CRI when cardiac alterations become manifest are unknown. For assessing the prevalence and factors associated with abnormal LV geometry in children with CRI, echocardiograms, ambulatory BP monitoring, and biochemical profiles were obtained in 156 children aged 3 to 18 yr with stages 2 through 4 chronic kidney disease (GFR 49 ؎ 19 ml/min per 1.73 m 2 ) and compared with echocardiograms obtained in 133 healthy children of comparable age and gender. LV mass was indexed to height 2.7 . Concentric LV remodeling was observed in 10.2%, concentric LVH in 12.1%, and eccentric LVH in 21% of patients. LVH was more common in boys (43.3 versus 19.4%; P < 0.005). Probability of LVH independently increased with male gender (odds ratio [OR] 2.62; P < 0.05) and standardized body mass index (OR 1.56; P ‫؍‬ 0.01). Low hemoglobin, low GFR, young age, and high body mass index were independent correlates of LV mass index (0.005 < P < 0.05). LV concentricity (relative wall thickness) was positively associated with serum albumin (P < 0.05). Probability of abnormal LV geometry increased with C-reactive protein >10 mg/dl (OR 26; P < 0.001). In conclusion, substantial cardiac remodeling of both concentric and eccentric type is present at young age and early stages of CRI in children. Prevalence of LVH is related to male gender, anemia, and ponderosity but not to BP. Additional effects of volume status and inflammation on cardiac geometry are also evident.

Research paper thumbnail of Aberrant Left Innominate Artery From the Left Descending Aorta in Right Aortic Arch: Echocardiographic Diagnosis

Journal of the American Society of Echocardiography, 2010

A right aortic arch with a left descending aorta and an aberrant left innominate artery is a rare... more A right aortic arch with a left descending aorta and an aberrant left innominate artery is a rare but recognized vascular anomaly that can result in compression of the trachea and the esophagus. This vascular anomaly has been diagnosed using cardiac catheterization and angiography. Recently, computed tomography and magnetic resonance imaging have been used for noninvasive diagnosis. The aim of this report is to highlight the possibility of echocardiographic diagnosis.

Research paper thumbnail of Minimally invasive or interventional repair of atrial septal defects in children: experience in 171 cases and comparison with conventional strategies

Journal of the American College of Cardiology, 2001

The goal of this study was to evaluate percutaneous interventional and minimally invasive surgica... more The goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children. BACKGROUND Concern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization.

Research paper thumbnail of Integrated Backscatter in Becker Muscular Dystrophy Patients With Functionally Normal Heart: Myocardial Ultrasound Tissue Characterization Study

Journal of the American College of Cardiology, 2006

Research paper thumbnail of ADVANCED PARAMETERS OF CARDIAC MECHANICS IN CHILDREN WITH CHRONIC KIDNEY DISEASE: THE 4C STUDY

Journal of the American College of Cardiology, 2014

Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult... more Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult patients with CKD. The aim of this study was to identify prevalence of subclinical abnormalities in cardiac function through the analysis of novel indices of cardiac mechanics in a large population of children with CKD. Between 2009 and 2011, the prospective observational Cardiovascular Comorbidity in Children with CKD Study enrolled patients with CKD ages 6-17 years old with eGFR=10-45 ml/min per 1.73 m(2) in 14 European countries. Cardiac morphology and function were assessed through echocardiography. The analysis presented encompasses global radial, longitudinal, and circumferential strains as well as time to peak analysis. Data were compared with 61 healthy children with comparable age and sex. Data on 272 patients with CKD with complete echocardiographic assessment are reported (age =12.8±3.5 years old; 65% boys). Patients with CKD showed mildly higher office BP values and higher p...

Research paper thumbnail of Change in Cardiac Geometry and Function in CKD Children During Strict BP Control: A Randomized Study

Clinical Journal of the American Society of Nephrology, 2013

Left ventricular hypertrophy (LVH) and abnormal systolic function are present in a high proportio... more Left ventricular hypertrophy (LVH) and abnormal systolic function are present in a high proportion of children with CKD. This study evaluated changes in left ventricular (LV) geometry and systolic function in children with mild to moderate CKD as an ancillary project of the Effect of Strict Blood Pressure Control and ACE Inhibition on Progression of Chronic Renal Failure in Pediatric Patients trial. Echocardiograms and ambulatory BP monitoring were performed at baseline and at 1- or 2-year follow-up in 84 patients with CKD and 24-hour mean BP above the 50th percentile and/or receiving fixed high-dose angiotensin converting enzyme inhibition and randomized to conventional or intensified BP control. LVH prevalence decreased from 38% to 25% (P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.05). Changes in LV mass index (LVMI) were restricted to patients with LVH at baseline (-7.9 g/m(2.7); P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.02). Changes in LVMI were independent of randomization, reduction in BP, hemoglobin, and estimated GFR. A significant increase in midwall fractional shortening was observed in the total cohort (P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.05), and was greater in the intensified group compared with the conventional BP control group (12%±1.9% versus 8%±1.5%; P=0.05). In multivariate analysis, improvement in myocardial function was associated with reduction in BP (r=-0.4; P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.05), independently of LVMI reduction. In children with CKD, angiotensin converting enzyme inhibition with improved BP control, LVH regression, and improved systolic function was observed within 12 months. Lowering BP to the low-normal range led to a slightly more marked improvement in myocardial function but not in LVMI.

Research paper thumbnail of Association of DiGeorge anomaly and caudal dysplasia sequence in a neonate born to a diabetic mother

Cardiology in the Young, 2013

Several studies have demonstrated a significantly increased risk of specific patterns of congenit... more Several studies have demonstrated a significantly increased risk of specific patterns of congenital anomalies in infants born to diabetic mothers. In particular, caudal dysplasia sequence has been linked to pregnancy complicated by maternal diabetes. In addition, several cases of infants born to diabetic mothers presenting with features of DiGeorge anomaly have been reported. Infants with DiGeorge anomaly can display additional manifestations within the spectrum of caudal dysplasia sequence, including vertebral anomalies and renal agenesis. We report a neonate presenting with the co-occurrence of features of both DiGeorge anomaly and caudal dysplasia sequence, born to a mother with poorly controlled insulin-dependent diabetes. The patient was affected by truncus arteriosus type A1 and hypertrophic cardiomyopathy. Maternal diabetes can cause a spectrum of manifestations, expressing with isolated DiGeorge anomaly or caudal dysplasia sequence, with intermediate phenotypes or with the co-occurrence of both the congenital anomalies in the same patient. The present observations argue for a feasible link between truncus arteriosus with hypertrophic cardiomyopathy, DiGeorge anomaly, and maternal diabetes.

Research paper thumbnail of New Findings concerning Cardiovascular Manifestations emerging from Long-term Follow-up of 150 patients with the Williams-Beuren-Beuren syndrome

Cardiology in the Young, 2009

We investigated the prevalence, type, and course of congenital cardiac defects and systemic hyper... more We investigated the prevalence, type, and course of congenital cardiac defects and systemic hypertension in our patients with Williams-Beuren-Beuren syndrome. We reviewed the clinical records of all patients with Williams-Beuren syndrome examined between 1981 and 2006. We identified 150 patients, aged from 7 months to 45 years, with a follow-up from 6 months to 25 years, the mean being 6.4 years. A cardiac anomaly was present in 113 of the 150 patients (75%). Defects were typical in over four-fifths of the group. We found supravalvar aortic stenosis in 73 of 113 patients (64.6%), isolated in 43. Pulmonary stenosis, isolated in 18 cases, was found in 51 of 113 (45.1%), while aortic coarctation and mitral valvar prolapse were each found in 7 (6.2%), 3 of the lesions is isolation. Atypical defects were found in 19 patients, tetralogy of Fallot in 2, atrial septal defects in 4, aortic and mitral valvar insufficiencies in 1 each, bicuspid aortic valves in 2, and ventricular septal defects in 9, 4 of the last being isolated. Systemic hypertension, observed in 33 patients (22%), was poorly controlled in 10. Diagnostic and/or interventional cardiac catheterization was undertaken in 24 patients, with 30 surgical procedures performed in 26 patients. Of the group, 3 patients died. Cardiac defects were present in three-quarters of our patients. Pulmonary arterial lesions generally improved, while supravalvar aortic stenosis often progressed. Atypical cardiac malformations, particularly ventricular septal defects, occurred frequently. Systemic hypertension was found in one-fifth, even in the absence of structural cardiac defects. The short-term mortality was low.

Research paper thumbnail of Granulicatella bacteraemia in children: two cases and review of the literature

BMC Pediatrics, 2013

Background: Granulicatella spp. is a fastidious bacteria responsible for bacteremia and endocardi... more Background: Granulicatella spp. is a fastidious bacteria responsible for bacteremia and endocarditis which are fatal in about 20% of the cases. These severe infections are uncommon in children under 17 years of age and have proven extremely difficult to treat. Cases presentation: We report a brief review of the literature and two cases of NVS bacteremia by Granulicatella complicated by infective endocarditis (IE). The first one is that of a 7-year-old Caucasian female with Shone syndrome and IE involving the pulmonary valve homograft, confirmed by echocardiography. The second case is that of a 5-year-old Caucasian male. In this patient echocardiogram was negative for signs of IE; however, a "possible" IE was suspected on the basis of a cardiac catheterization 3 weeks before the onset of fever. Since in both our patients clinical failure of first line antibiotic treatment was observed, we used a combination of meropenem with another anti-streptococcal drug with excellent results. Conclusion: In Granulicatella bacteremia in the pediatric population, combination antimicrobial therapy including meropenem should be considered as a second line treatment in non-responding patients.

Research paper thumbnail of Double-Outlet Right Atrium: Anatomic and Clinical Considerations

The Annals of Thoracic Surgery, 2007

Double-outlet right atrium (DORA) is a condition in which the right atrium empties into both vent... more Double-outlet right atrium (DORA) is a condition in which the right atrium empties into both ventricles. Described are the anatomic and clinical findings of this rare anomaly and our experience with its surgical treatment. A retrospective review was conducted of all medical records and two-dimensional echocardiographic assessments, and the operative reports of patients with DORA were reexamined to identify the specific anatomic features and the relative adaptation of surgical technique. From January 1983 to November 2004, 12 patients with DORA underwent surgical treatment. The diagnosis was made in all cases by two-dimensional echocardiography. All patients had mild-to-moderate signs of arterial oxygen desaturation. All patients also had either a partial (n = 11) or a complete (n = 1) atrioventricular canal with or without other associated cardiac anomalies, including persistent left superior vena cava. Eleven patients survived the surgical repair, the only death occurred in a patient with Ellis Von-Creveld syndrome, with multiple skeletal malformations, severe chest hypoplasia, and respiratory distress. DORA is a rare and peculiar cardiac anomaly with well-defined characteristics. Its recognition, best achieved by two-dimensional echocardiography, is crucial for a good surgical correction.

Research paper thumbnail of Systolic Ejection Murmurs in the Elderly: Aortic Valve and Carotid Arteries Echo-Doppler Findings

Angiology, 1991

Two-dimensional echographic and color Doppler studies of the heart and carotid arteries (CA) were... more Two-dimensional echographic and color Doppler studies of the heart and carotid arteries (CA) were performed in 45 patients greater than sixty-five years old without aortic stenosis, 23 with (Group 1) and 22 without (group 2) precordial ejection systolic murmur (SM). Aortic cusps thickening was found in 11 Group 1 (48%) and 2 Group 2 (9%) patients (p less than 0.001). Aortic root and aortic arch size were similar in the two groups. Maximum aortic flow velocity was significantly greater in Group 1 (200 60 cm/sec) than in Group 2 (120 20 cm/sec) (p less than 0.001). Left ventricular outflow systolic maximum velocity was similar in the two groups. A bilateral neck murmur was heard in 10/23 Group 1 patients (43%); in this group, patients with cervical SM had a greater maximum aortic flow velocity than those without cervical SM (230 + 60 cm/sec vs 172 + 32 cm/sec, p less than 0.001). In Group 1, 3 patients had a cervical SM louder on one neck side; only in these 3 patients were ipsilateral obstructive CA plaques found. A unilateral neck SM was heard in 4/22 Group 2 patients (18%); in these 4, ipsilateral obstructive CA were found. (1) in the elderly, precordial ejection SM is related to mild increase in maximum aortic flow velocity and thickening of aortic cusps; (2) in patients with precordial SM radiated to both neck sides, maximum aortic flow velocity tends to be more markedly increased; (3) in patients with precordial SM, a cervical SM louder on one neck side should suggest coexistent ipsilateral CA stenosis.

Research paper thumbnail of Pitfalls in Echocardiographic-Based Repair of Aortic Coarctation

The American Journal of Cardiology, 1997

The knowledge of echocardiographic pitfalls, which may impair the correct indication for surgery ... more The knowledge of echocardiographic pitfalls, which may impair the correct indication for surgery of aortic coarctation, is of great importance to pediatric cardiologists. We believe that only the suspicion of coronary artery anomalies is a clear indication to perform a cardiac catherization.

Research paper thumbnail of Comparison of three different atrial septal defect occlusion devices

The American Journal of Cardiology, 1998

Three different devices were used to close secundum-type atrial septal defects in 28 patients. Th... more Three different devices were used to close secundum-type atrial septal defects in 28 patients. The &quot;Amplatzer&quot; is associated with an easier and shorter procedure than are the &quot;Sideris Buttoned Device&quot; and the Microvena &quot;Angel Wings&quot; devices.

Research paper thumbnail of Abnormal intraventricular flow patterns in left ventricular dysfunction determined by color Doppler study

American Heart Journal, 1992

We examined the relation between left ventricular (LV) flow dynamics measured by color Doppler, a... more We examined the relation between left ventricular (LV) flow dynamics measured by color Doppler, and either global or regional LV function in 19 normal subjects (group 1), in 55 patients with old myocardial infarction (MI) (29 without [group 2] and 26 with LV aneurysm [group 3]), and in 16 with idiopathic dilated cardiomyopathy (group 4). We calculated by M-mode color Doppler a flow persistence index (FPI) (duration of flow directed in systole toward the apex/LV ejection time). Contrast echocardiography was performed as a control method in 14 patients of the four groups. In normal subjects, rapid systolic inversion of flow toward the aorta was evident (FPI: 0.11 +/- 0.16). In all but one patient in group 2, a similar LV flow pattern was observed, but FPI was greater (0.32 +/- 0.26). In groups 3 and 4, a paradoxical antegrade LV flow pattern was evident during the entire period of systole (FPI: 1.13 +/- 0.42 and 1.28 +/- 0.36, respectively). LV flow patterns were reproduced in echo-contrast studies. FPI was related to LV end-diastolic volume (r = 0.77), end-systolic volume (r = 0.82), and ejection fraction (r = -0.84). However, when data were analyzed separately in the different groups, these correlations were significant only in groups 2 and 3. Paradoxical flow pattern is not peculiar to regional LV dysfunction; it also occurs in global LV dysfunction. This LV flow abnormality may develop after MI even in the absence of severe LV dyssynergy or dilation, and is quantitatively related to the degree of LV dysfunction.

Research paper thumbnail of IMPAIRED CARDIAC MECHANICS IN A LARGE POPULATION OF CHILDREN WITH HEART TRANSPLANTATION: A SPECKLE TRACKING AND THREE-DIMENSIONAL ECHOCARDIOGRAPHY STUDY

Journal of the American College of Cardiology, 2014