Non-surgical treatment of ruptured sinus of Valsalva aneurysm.
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Biomedical subjects
Publications and source records attributed to Maria Giovanna Russo.
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AIMS: The prevalence of obesity is increasing among children in the developed world. The association of obesity and abnormal cardiac function is still debated. The reported changes may reflect the role of comorbidities that contribute to ventricular dysfunction. Obese children, without arterial hypertension, may be a unique clinical opportunity to evaluate the effect of obesity, per se, on myocardial function, excluding the influence of possible comorbidities. We sought to define the preclinical effects of obesity on the cardiovascular system, of healthy children with excess weight who have no other clinically appreciable cause of heart disease, using the more sensitive ultrasonic-derived strain and strain rate (SR) imaging. METHODS AND RESULTS: We studied 300 subjects divided into two groups: (i) obese children (Group O: n=150; age, 12+/-3 years); (ii) healthy lean children comparable for age, sex, and pubertal stage (Referents: n=150; mean age, 12+/-3 years). Systolic (SBP) and diastolic blood pressure (DBP), as well as 24 h-SBP and 24 h-DBP were comparable between groups. Left ventricular (LV) mass/height(2.7) was increased (P<0.0001) in Group O (46+/-12 g/m(2.7)) when compared with Referents (31+/-14 gm(2.7)). Standard echocardiographic indices of global systolic function were similar in the two groups. Intima-media thickness measured at the common carotid artery was not significantly different (P=0.4) in obese children (0.46+/-0.09 mm) when compared with Referents (0.45+/-0.07 mm). Obese children showed regional longitudinal peak systolic myocardial deformation properties (SR=-1.4+/-0.7 s(-1)) lower (P<0.0001) than those of Referents (SR=-2.2+/-0.5) in both left and right ventricle. In multivariable analysis, average peak systolic SR was significantly correlated with homeostasis model assessment of insulin resistance (P<0.01; coefficient, 0.02; SE, 0.011), and insulin serum concentration (P<0.01; coefficient, 0.05; SE, 0.023). Average LV peak systolic strain was significantly correlated with body mass index (P=0.0001; coefficient, 0.06; SE, 0.016), LVM/H(2.7) (P=0.006; coefficient, 0.016; SE, 0.018). CONCLUSIONS: Our study demonstrated that obesity, in absence of hypertension, is associated with significant reduction in systolic myocardial deformation properties already in childhood involving both right and left ventricle. Obesity not only is a risk factor for later cardiovascular disease, but also is associated with contemporaneous and significant impairment of longitudinal myocardial deformation properties.
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BACKGROUND: Recent studies indicate that T-cell activation may play an important role in the pathophysiology of acute coronary syndromes (ACS). However, although those studies detected T-cell expansion in peripheral blood cells, demonstration of specific T-cell expansion within the plaque of patients with ACS is lacking. The present study aims to address whether a specific, immune-driven T-lymphocyte recruitment occurs within the unstable plaque of patients with ACS. METHODS AND RESULTS: We simultaneously examined the T-cell repertoire using CDR3 size analysis both in coronary plaques (obtained by directional atherectomy) and in peripheral blood of patients with either ACS (n=11) or chronic stable angina (n=10). Unstable plaques showed a 10-fold increase in T-cell content by quantitative PCR. Using spectratyping analysis, we found several specific T-cell clonotype expansions only in unstable plaque from each patient with ACS, indicating a specific, antigen-driven recruitment of T cells within unstable lesions. CONCLUSIONS: For the first time, T-cell repertoire was investigated directly into coronary plaques; using this approach, we demonstrate that coronary plaque instability in the setting of ACS is associated with immune-driven T-cell recruitment, specifically within the plaque.
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OBJECTIVE: Transcatheter closure of atrial septal defects (ASDs) is currently a reliable alternative to surgery, even though challenging in the case of complex septal anatomy. The aim of this study was to evaluate the feasibility and mid-term results of percutaneous closure of complex ASDs in a tertiary referral centre compared with simple ASD closure. METHODS: Between April 2000 and November 2004, 209 patients were submitted to transcatheter ASD closure; 83 patients (39.7%) presented with a complex defect (large ASDs with a deficient rim or a multifenestrated/aneurysmal septum) and were treated using different devices tailored to the atrial septal anatomy. RESULTS: The transcatheter procedure was successful in 72 patients (86.8%), using a single device in 69 patients and two devices in the remaining three patients. Overall, 71 Amplatzer septal occluders, two multifenestrated Amplatzer septal occluders and two Cardioseal/Starflex devices were used. Procedural and fluoroscopy times were 141 +/- 45 min and 28 +/- 22 min, respectively (P < 0.0001 vs. simple ASD closure for both comparisons). Procedure-related complications were recorded in nine patients (12.5%) (P < 0.01 vs. simple ASD closure). One patient required surgical repair of a femoral arteriovenous fistula and another developed mitral valve dysfunction. Immediate ASD occlusion was recorded in 59.7% of patients, reaching 95.9% at the last follow-up control (P = NS vs. simple ASD closure for both comparisons). CONCLUSIONS: Percutaneous closure of complex ASDs may be considered technically feasible, relatively safe and highly effective, although the procedure is still significantly more demanding than transcatheter closure of simple ASDs.
A 12-year-old female with the Wolff-Parkinson-White syndrome underwent an electrophysiologic study followed by radiofrequency catheter ablation of the left-lateral accessory pathway. After several unsuccessful attempts, the procedure was stopped because of early recurrence of accessory pathway conduction. Twenty-four hours after the procedure, the patient was found without ventricular pre-excitation pattern on the electrocardiogram. During a 12-month follow-up, the Wolff-Parkinson-White pattern was no longer present. A transesophageal electrophysiologic study showed no further tachycardia induction and the patient is still asymptomatic. This report raises the issue that ablation-induced lesions may evolve considerably during the first few days after ablation, leading to either recurrent accessory pathway conduction or long-lasting complete cure.
Sudden death is a tragic complication of hypertrophic cardiomyopathy. We report the case of a young patient with hypertrophic cardiomyopathy in whom an episode of atrial fibrillation triggered ventricular fibrillation and cardiac arrest. A 21-year-old man with nonobstructive hypertrophic cardiomyopathy underwent cardioverter-defibrillator implantation for primary prevention of sudden death, after risk stratification with noninvasive strategies. After 6 weeks, during a moderate effort, the patient had a syncopal episode, preceded by palpitations and dizziness, and terminated by the cardioverter-defibrillator. Device interrogation revealed an episode of atrial fibrillation with high ventricular response, spontaneously followed by ventricular tachycardia/fibrillation. Atrial fibrillation is a potential trigger of life-threatening arrhythmias and sudden death in patients with hypertrophic cardiomyopathy. Clinical investigation of risk markers for sudden death should be encouraged to identify high-risk patients who may benefit from a prophylactic therapy with an implantable cardioverter-defibrillator.
OBJECTIVE: Left ventricular (LV) remodelling in prenatally diagnosed LV outflow tract obstructive lesions such as aortic stenosis and aortic coarctation is important for prenatal counselling and postnatal management. The purpose of this study was to document the spectrum and the progression of different LV remodelling patterns and to identify prenatal markers of hypoplastic left heart syndrome (HLHS). METHODS: We studied 29 fetuses with LV outflow tract obstruction: 13 with isolated aortic stenosis, 14 with isolated aortic coarctation and two with combined aortic stenosis and aortic coarctation. Echocardiographic evaluation was performed 4 and 8 weeks after the first observation and at birth. RESULTS: None of the fetuses had HLHS (LV end-diastolic diameter z score higher than -2) at first prenatal echocardiography (24.5 + or - 3.6 weeks). Fetuses were divided into two groups: group A (n = 25) with a LV end-diastolic volume at birth > 20 ml/m(2); group B (n = 4) with a LV end-diastolic volume at birth < 20 ml/m(2) (LV hypoplasia). At first echocardiographic evaluation, the two groups showed a significantly different aorta to pulmonary ratio (0.44 + or - 0.08 vs. 0.86 + or - 0.14; P < 0.001); other LV echocardiographic features were not significantly different. The growth of the mitral (0.10 + or - 0.02 vs. 0.43 + or - 0.28 mm/week; P < 0.02) and aortic annulus (0.08 + or - 0.01 vs. 0.26 + or - 0.14 mm/week; P < 0.05) was significantly slower in group B. CONCLUSIONS: Our data suggest that LV outflow tract obstruction can progressively evolve in HLHS during pregnancy. A smaller aorta to pulmonary ratio was the only significant difference at initial echocardiographic evaluation in the two groups. Moreover, serial echocardiographic examinations are necessary to recognize fetuses at risk for HLHS caused by a subnormal growth rate of the mitral and aortic annulus.
The increasing number of adult patients with congenital heart disease and the better survival of patients with complex disease into adulthood, as a result of the success of pediatric cardiology and cardiac surgery over the last years, have increased the need for specific structures, the so-called grown-up congenital heart disease units, able to provide comprehensive care to these patients. Many of the adult patients with congenital heart disease will require, over time, further operations, urgent in-hospital admission for a wide range of complications such as arrhythmias, hemorrhage, heart failure and bacterial endocarditis. Furthermore, these patients may often experience despair due to their awareness of residual morbidities and the knowledge of possible early mortality, or limitations in their social lives and educational or occupational attainment. Provision of care for children with congenital heart disease is well established in most parts of the world. In contrast, clinical services for the adults with congenital heart disease are scarce. In this scenario, adult cardiologists are not always equipped to deal with the range ad complexity of grown-up patients with congenital heart disease, whereas pediatric cardiologists cannot be expected to manage the many acquired adult diseases in a pediatric medical environment.
The association between large, left-sided patent ductus arteriosus and severe, peripheral, right pulmonary artery stenosis with no other cardiac malformation is an unreported condition that might be misdiagnosed as pulmonary hypertension due to long-standing ductal shunt. A 57-year-old man with supposed hypertensive patent ductus arteriosus underwent confirmatory cardiac catheterization. At angiography, a severe pre-hilar right pulmonary artery stenosis (peak pressure gradient, 65 mmHg) was found to complicate the hemodynamic picture of a moderate-to-large patent ductus arteriosus (QP/QS, 1.7:1), by causing pulmonary hypertension (mean pressure, 65 mmHg) and left-to-right pulmonary flow imbalance. Both lesions were treated in a single procedure of right pulmonary artery stenting and patent ductus arteriosus closure, after which the pulmonary artery pressure significantly decreased (mean, 35 mmHg). In our opinion, a thorough hemodynamic evaluation followed by pulmonary angiography should be mandatory before proceeding to patent ductus arteriosus closure in the adult patient who has "hypertensive" ductus, in whom possible associated malformations can be missed due to a poor echocardiographic window.
BACKGROUND: Right chamber dilatation and right-to-left volumetric unbalance are well-known cardiac consequences of atrial septal defect (ASD) shunt, accounting for most of its long-term complications. Thus, cardiac volumetric unloading is a major aim of ASD closure. Different from surgery, transcatheter option might be considered as an "unbiased" tool to evaluate the cardiac geometric remodeling following ASD closure. METHODS: Extent and time-course of cardiac geometric changes were assessed by echocardiography 24 h, 1 and 6 months after percutaneous closure of large ASD (mean diameter 17+/-6 mm, QP/QS 2.2+/-0.9) in 42 asymptomatic patients (age 22+/-18 years). RESULTS: Transcatheter closure was accomplished using the Amplatzer Septal Occluder device (mean 23+/-7 mm, median 24 mm), achieving a complete occlusion in all patients at the 6-month follow-up control. After ASD closure, right atrial (RA) volume reduced from 45+/-24 to 28+/-12 ml (-37.8%, p<0.001), while left atrial (LA) volume did not significantly change. Inlet and infundibulum right ventricular (RV) end-diastolic diameters reduced by 23+/-2% and 23+/-3%, respectively (p<0.001 for both measurements), although with a different time-course of changes. Finally, transverse left ventricular (LV) end-diastolic diameter increased from 39+/-7 to 44+/-5 mm (+11.4%, p<0.01). These geometric changes resulted in an RV/LV diameter ratio decrease by 34+/-3% (p<0.001). Nearly 90% of cardiac remodeling ensued within 1 month from shunt disappearance (50% within 24 h). CONCLUSIONS: Percutaneous ASD closure results in early and striking cardiac geometric changes that almost completely revert the right-to-left volumetric unbalance. Most of this geometric remodeling ensues within a few weeks from ASD closure.
An 11-year-old boy was successfully treated by stent implantation for native aortic coarctation. At the 1-year control a severe re-coarctation was found at Doppler analysis and subsequent angiography revealed a transverse stent fracture. A stent-in-stent implantation was performed. Several hypotheses could explain this complication. Interventional cardiologists may pay more attention in following up these patients!
This study sought to evaluate global and regional left ventricular (LV) function before and early after device closure of atrial septal defects (ASDs) in patients with normal pulmonary pressure. Global LV diastolic function was unaffected by ASD closure. An improvement in global LV systolic function at rest resulted in an increase in stroke volume at rest. Nevertheless, total cardiac output did not change after the procedure, because of a decrease in heart rate at rest counterbalancing the increase in stroke volume. Thus, lateral and inferior LV regional systolic function were preserved after device implantation. Moreover, no changes in regional LV diastolic function were highlighted during the study.
For the first time, peak systolic strain and strain rate (SR) were used to assess right ventricular (RV) and left ventricular (LV) regional function in children after transcatheter and surgical atrial septal defect (ASD) closure. Fifteen patients with successful ASD device closure (the ASD-D group, mean age 9 +/- 3 years), 15 age- and gender-matched patients with successful ASD surgical closure (the ASD-S group, mean age 9 +/- 3 years), and 15 age- and gender-matched controls were enrolled. Regional RV and LV longitudinal function was significantly reduced in the ASD-S group compared with controls in all the studied segments. The ASD-D group presented significantly (p <0.001) reduced strain and SR values only on the basal and mid segments of the septal wall compared with controls. In the ASD-D group, only the RV basal segment showed significantly (p <0.001) reduced strain and SR values compared with controls but significantly greater values than those measured in the ASD-S group. SR imaging indexes could provide new, noninvasive, clinically relevant insight into regional changes in RV function and support the transcatheter approach to ASD for its less negative impact on RV and LV function.
BACKGROUND: Congenital heart disease (CHD) is the most common malformation in the fetal and neonatal period but little is known about its cause. The distribution analysis of CHD in dizygotic twins could provide a useful tool to evaluate the role of genetic and environmental factors in the development of CHD. Dizygotic twins are siblings with different genes, growing together in the same womb. AIM OF STUDY: To investigate the occurrence of CHD in a large sample of dizygotic twins of nonconsanguineous healthy parents, comparing the data from non-twin patients. METHODS: From January 1999 to December 2002, we enrolled 1743 CHD patients with, at least 1 sibling, and 66 pairs of dizygotic twins, referred to our tertiary center. The diagnosis of CHD was based on clinical and echocardiographic evaluation. RESULTS: Considering only the sibling nearest in age for each non-twin patient the recurrence was 67/1743 (3.8%). Among these 67 patients, 35 (52.2%) had a sibling with the same or similar CHD. Conversely, considering all 1886 siblings, recurrence of CHD in the non-twin group was 70/1743 (4%). Of the 70 patients, 36 (36/70, 51.4%) had a sibling with the same suspected pathogenic mechanism of CHD. In 9/66 pairs of twins (13.6%), both siblings had a CHD. In the nine pairs of twins in whom both siblings had a CHD, the percentage of concordance (based on the suspected pathogenic mechanism) for CHD was 100% (p<0.05). CONCLUSIONS: Our findings suggest that the higher recurrence and concordance of CHD found in dizygotic twins could depend on some poorly identified environmental risk during the pregnancy.
AIMS: Ostium secundum atrial septal defect (osASD) is one of the most common cardiac malformations. Few data are available on the familial recurrence of congenital heart disease (CHD), in particular, in a large group of patients with isolated osASD. The aim is to investigate the familial recurrence of CHD in up to third-degree relatives from a large sample of consecutively enrolled patients with osASD, taking into account the influence of degree of relatedness (as number of relatives). METHODS AND RESULTS: From January 1998 to December 2002, we enrolled 583 patients with osASD and 408 healthy subjects, referred to our tertiary centre. We hypothesized that a positive family history required at least one relative with CHD to constitute a risk factor. In this model of analysis, the null hypothesis is a similar familial history between cases and controls. Among 583 patients with osASD, 109 (19%) had at least one relative with CHD. Among the 408 healthy subjects studied, only 23 (6%) had a family history of CHD. A familial recurrence of CHD was demonstrated in 72 of 312 (23%) patients with isolated osASD and in 37 of 271 (13.6%) patients with non-isolated osASD. Familial recurrence of isolated osASD was demonstrated in 22 of 312 patients (7%) with an isolated osASD and only in six of 271 patients (2.2%) with non-isolated osASD. The familial recurrence risk of isolated osASD in patients with isolated osASD was higher in sibs, especially in sisters (33.3%). CONCLUSION: This study underscores the role of genetic factors in the determination of CHD, particularly osASD. Our results could represent the basis for further studies to calculate a 'value of family history' to adapt the familial recurrence to the real size of each family group. In this way, we could select families with a 'tendency' to develop CHD, particularly osASD. In these families, we could analyse the genetic pattern to establish abnormalities and the bases of CHD.
BACKGROUND: Diagnostic assessment and treatment have been described in detail in symptomatic WPW syndrome, but little information exists about significance and prognosis of an incidentally found ventricular pre-excitation (VPE) in asymptomatic children. The aim of the study was to evaluate, retrospectively, the role of electrophysiological study (EPS) in the assessment of the arrhythmic risk in asymptomatic patients with VPE. MATERIAL AND METHODS: Sixty-two asymptomatic children and adolescents (38 M/24 F, aged 9.8+/-5.1 years) referred to our Division between 1996 and 2002 for an incidentally found VPE underwent an EPS for arrhythmic risk stratification. The following parameters were examined: anterograde effective refractory period of the accessory pathway (AP), the 1-to-1 conduction over the AP, the inducibility of atrio-ventricular re-entrant tachycardia (AVRT) and the inducibility of atrial fibrillation (AF) with measurement of minimal RR between two consecutive preexcitated QRS complexes, the average RR interval of all cycles, and the percentage of preexcitated QRS complexes. RESULT: During the EPS, 36 patients (58.1%) experienced sustained SVT. The tachycardia was initiated in the basal state in 22 patients and after isoproterenol in the other 14. Orthodromic AVRT (cycle length 305.9+/-48.5 ms) was recorded in 29 patients. In three patients, both orthodromic and antidromic AVRT were recorded, with different cycle length (CL). Antidromic AVRT alone (CL 239.5+/-13.7 ms) was recorded in four patients. AF was recorded in nine patients: in six patients, it was recorded after the induction of orthodromic or antidromic AVRT, in the other three cases AF was the first and only arrhythmic event. The minimal RR between two consecutive pre-excitated QRS ranged between 250-230 ms (mean 237.5+/-9.6 ms). In the 26 patients who presented no induced sustained tachycardia in the EPS, the 1:1 conduction over the AP ranged between 210 and 600 ms (mean 279.6+/-75.2 ms). CONCLUSIONS: Electrophysiological evaluation remains the gold standard for assessing risk of life-threatening arrhythmias in patients with VPE. However, a high proportion of healthy children and adolescents with VPE can experience sustained AVRT and/or AF during EPS. These results raise questions about the necessity of an aggressive treatment approach to prevent those "rare" cases of sudden death.