Interventional VSD-Closure with the Nit-Occlud® Lê VSD-Coil in 110 Patients: Early and Midterm Results of the EUREVECO-Registry.

In August 2010, the Nit-Occlud® Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud® Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.2 years, mean weight 28.82 kg), 81 perimembranous VSDs (48 with aneurysm), 30 muscular VSDs, mean procedure time was 121.1 min, and mean fluoroscopy time was 26.3 min. Short- and midterm term follow-up was possible in 100/102 patients, there was 1 embolization and 1 explantation after 24 months. Immediate complete closure occurred in 49 of 101 patients (48.5%), trivial residual shunt was present in 51 (50.0%), closure rate was 95% after 6 months and 97% after 1 year. Out of the 102 patients, there were 2 severe complications (1.8%) (1 severe hemolysis, 1 embolization) and 8 moderate/transient (=7.2%) including 1 transient AV block. During a mean follow-up period of 31.3 months (range 24-48) and a total follow-up time of 224.75 patient years, no further problems occurred. VSD closure with the Nit-Occlud® Lê VSD coil is feasible and safe with a minimal risk of severe side effects. The long-term effects and safety require further clinical follow-up studies.

Left Ventricular Mass Indexing in Infants, Children, and Adolescents: A Simplified Approach for the Identification of Left Ventricular Hypertrophy in Clinical Practice.

OBJECTIVE: To determine a simplified method to identify presence of left ventricular hypertrophy (LVH) in pediatric populations because the relationship between heart growth and body growth in children has made indexing difficult for younger ages. STUDY DESIGN: Healthy children (n = 400; 52% boys, 0-18 years of age) from 2 different European hospitals were studied to derive a simplified formula. Left ventricular mass (LVM) was calculated according to the Devereux formula. The derived approach to index LVM was tested on a validation cohort of 130 healthy children from a different hospital center. RESULTS: There was a strong nonlinear correlation between height and LVM. LVM was best related to height to a power of 2.16 with a correction factor of 0.09. Analysis of residuals for LVM/[(height(2.16)) + 0.09] showed an homoscedastic distribution in both sexes throughout the entire height range. A partition value of 45 g/m(2.16) was defined as the upper normal limit for LVM index. As opposed to formula suggested by current guidelines (ie, LVM/height(2.7)) when applying the proposed approach in the validation cohort of 130 healthy participants, no false positives for LVH were found (0% vs 8%; P < .01). CONCLUSIONS: Our data support the possibility to have a single partition (ie, 45 g/m(2.16)) value across the whole pediatric age range to identify LVH, without the time-consuming need of computing specific percentiles for height and sex.

Coronary artery ectasia in Noonan syndrome: Report of an individual with SOS1 mutation and literature review.

Noonan syndrome (NS) is the second most frequent hereditary syndrome with cardiac involvement. Pulmonary valve stenosis and hypertrophic cardiomyopathy are the most prevalent cardiovascular abnormalities. We report on a 14-year-old girl with NS due to SOS1 mutation with pulmonary stenosis and idiopathic coronary ectasia. To the best of our knowledge, this is the first report describing coronary ectasia in a patient with NS secondary to a SOS1 mutation. We include a literature review of this rare association.

Endomyocardial biopsy safety and clinical yield in pediatric myocarditis: An Italian perspective.

OBJECTIVES: The objective of this investigation is to evaluate the safety, the impact of endomyocardial biopsy (EMB) results in myocarditis management and the incidence of different etiologies of myocarditis in a pediatric population. BACKGROUND: Although EMB is an established diagnostic tool to evaluate suspected myocarditis, there is lack of clear diagnostic and management guidelines for myocarditis in pediatric patients, particularly in infants. METHODS: We performed a retrospective database review and subsequent outcomes analysis from five Italian pediatric cardiology centers to identify patients aged 0-18 years who underwent EMB for suspected myocarditis or inflammatory cardiomyopathy (ICMP) between 2009 and 2011. RESULTS: EMB was performed in 41 children, of which 16 were male. The population ranged between 16 days of age to 17 years (mean age at EMB = 5.2 ± 4.9 years). The overall incidence of EMB-related complications was 15.5% (31.2% in infants, and 6.8% in children > 1 year of age; P = 0.079) while the incidence of EMB-driven treatment changes was 29.2%. Histological examination together with PCR on heart biopsy specimens allowed an etiological diagnosis in 26/41 patients (63%). Among the 15 patients (36.5%) with diagnosis of dilated cardiomyopathy (DCM) 11 had idiopathic DCM. Finally, we found an overall incidence of death/cardiac transplantation of 24%. CONCLUSIONS: In a pediatric population with suspected myocarditis/ICMP, EMB was useful in confirming the diagnosis only in 41% of cases but showed an overall diagnostic power of 63%. As complications of EBM are not negligible, particularly in infants, the risk/benefit ratio should be taken into account in each patient.

Incidence of Respiratory Disease During the First Two Years of Life in Children with Hemodynamically Significant Congenital Heart Disease in Italy: A Retrospective Study.

Children affected by hemodynamically significant congenital heart disease (HSCHD) experience severe respiratory complications that can increase the frequency of hospitalizations. The aim of the SINERGY study was to describe the incidence of respiratory diseases and to collect information on active and passive immunoprophylaxis in the first 2 years of life. In this retrospective, multicenter, and epidemiologic study, children with HSCHD were enrolled across 11 Italian sites. Children born between December 31, 2007, and December 31, 2012, were observed during their first 2 years of life. Data were collected through hospital database searches and parent interviews. Four hundred twenty children were enrolled: 51.7 % were female, 79.5 % were born full-term (≥37 weeks), and 77.6 % weighed >2500 g at birth. The most frequent heart defects were ventricular septal defect (23.1 %) and coarctation of the aorta (14.3 %). The incidence of respiratory diseases was 63.1 %. Frequent respiratory diseases not requiring hospitalization were upper respiratory tract infections (76.4 %), acute bronchitis (43.3 %), and influenza (22.1 %), while those requiring hospitalization were bronchitis and bronchiolitis (8.3 % each one). While active immunoprophylaxis was applied with wide compliance (diphtheria/pertussis/tetanus, 99.5 %; Haemophilus influenzae type b, 72.5 %; pneumococcus, 79.9 %; meningococcus, 77.4 %), only 54 % of children received respiratory syncytial virus (RSV) passive prophylaxis (palivizumab). Of the 35 hospitalizations due to bronchiolitis, 27 (77.1 %) did not receive prophylaxis against RSV, compared with 8 (22.9 %) who received prophylaxis (P < 0.0001). Children with HSCHD are at major risk of respiratory diseases. Passive immunoprophylaxis can help to prevent hospitalizations for bronchiolitis.

Holt-Oram syndrome with intermediate atrioventricular canal defect, and aortic coarctation: functional characterization of a de novo TBX5 mutation.

Holt-Oram syndrome (HOS) is a rare autosomal dominant disorder characterized by upper limb defects and congenital heart defects (CHD), which are often simple septal and conduction defects, less frequently complex CHDs. We report on a 9 year-old boy with clinical and radiologic features of HOS consisting of bilateral asymmetric hypoplastic thumbs, generalized brachydactyly, limited supination due to radioulnar synostosis, and sloping shoulders, and intermediate atrioventricular canal defect (AVCD) with aortic coarctation. A de novo, previously described mutation, (Arg279ter) was identified in the TBX5 gene. Molecular characterization of this mutation was carried out due to the atypical CHD. In order to investigate whether the mutated transcript of TBX5 was able to escape the post-transcriptional surveillance mechanism and to produce a truncated TBX5 protein, we analyzed the TBX5 transcript, and protein pattern in HOS, and WT cardiac tissues. Our results demonstrate that the mutant TBX5 transcript is cleared by the cellular mechanism of surveillance. This data provides some support for the hypothesis that a dominant negative mutation, which strongly impairs the WT allele, might be too hazardous to be maintained. The literature suggests that HOS is relatively common among syndromes associated with AVCD.

Identification of TBX5 mutations in a series of 94 patients with Tetralogy of Fallot.

Tetralogy of Fallot (TOF) (OMIM #187500) is the most frequent conotruncal congenital heart defect (CHD) with a range of intra- and extracardiac phenotypes. TBX5 is a transcription factor with well-defined roles in heart and forelimb development, and mutations in TBX5 are associated with Holt-Oram syndrome (HOS) (OMIM#142900). Here we report on the screening of 94 TOF patients for mutations in TBX5, NKX2.5 and GATA4 genes. We identified two heterozygous mutations in TBX5. One mutation was detected in a Moroccan patient with TOF, a large ostium secundum atrial septal defect and complete atrioventricular block, and features of HOS including bilateral triphalangeal thumbs and fifth finger clinodactyly. This patient carried a previously described de novo, stop codon mutation (p.R279X) located in exon 8 causing a premature truncated protein. In a second patient from Italy with TOF, ostium secundum atrial septal defect and progressive arrhythmic changes on ECG, we identified a maternally inherited novel mutation in exon 9, which caused a substitution of a serine with a leucine at amino acid position 372 (p.S372L, c.1115C>T). The mother's clinical evaluation demonstrated frequent ventricular extrasystoles and an atrial septal aneurysm. Physical examination and radiographs of the hands showed no apparent skeletal defects in either child or mother. Molecular evaluation of the p.S372L mutation demonstrated a gain-of-function phenotype. We also review the literature on the co-occurrence of TOF and HOS, highlighting its relevance. This is the first systematic screening for TBX5 mutations in TOF patients which detected mutations in two of 94 (2.1%) patients.

Coronary plaque composition assessed by intravascular ultrasound virtual histology: association with long-term clinical outcomes after heart transplantation in young adult recipients.

OBJECTIVES: To assess coronary plaque composition by virtual histology intravascular ultrasound (VH-IVUS) analysis in young adult recipients and to correlate these findings with time from heart transplant (HTx) and long-term outcomes. BACKGROUND: Rapid progression of coronary allograft vasculopathy after heart transplantation is a powerful predictor of mortality and clinical events at long-term. METHODS: Forty consecutive young adult recipients transplanted during childhood undergoing VH-IVUS during coronary surveillance have been prospectively included in this study. According to the time interval from HTx to VH-IVUS assessment, our cohort was divided into two groups (group A: ≤5 years, n = 13; group B: >5 years, n = 27). RESULTS: Group B showed an higher percentage of necrotic core and dense calcium (12 ± 2 vs. 5 ± 1%, P = 0.04; 8.2 vs. 2.1%, P = 0.03; respectively). An "inflammatory plaque" (necrotic core and dense calcium ≥30%) was detected in 34.8% of patients in group B and in none among group A patients (P = 0.03). Patients in group B had a number of adverse clinical events significantly higher than group A patients (53.8 vs. 14.3%; HR 4.45; 95% CI 1.62-12.16; P = 0.029) at long-term follow-up (4.2 years). The multivariate regression analysis showed that age (HR 1.5; 95% CI 1.1-2.0; P = 0.007), time from HTx (HR 1.8; 95% CI 1.6-4.8; P = 0.02), and inflammatory plaque (HR 2.4; 95% CI 1.1-5.3; P = 0.03) were independent predictors of adverse clinical events. CONCLUSIONS: This study supports the hypothesis that time-dependent differences in plaque composition, as assessed by VH-IVUS, occur after HTx in young adult recipients, probably determining an increased risk of long-term clinical events.

"Baby Heart Project": the Italian project for accreditation and quality management in pediatric cardiology and cardiac surgery.

Optimization of the relationship between the supply and the demand for medical services should ideally be taken into consideration for the planning within each national Health System. Although government national health organizations embrace this policy specifically, the contribution of expert committees (under the scientific societies' guarantee in any specific medical field) should be advocated for their capability to collect and analyze the data reported by the various national institutions. In addition, these committees have the competence to analyze the need for the resources necessary to the operation of these centers. The field of pediatric cardiology and cardiac surgery may represent a model of clinical governance of particular interest with regard to programming and to a definition of the quality standards that may be extended to highly specialized institutions and ideally to the entire Health System. The "Baby Heart Project," which represents a model of governance and clinical quality in the field of pediatric cardiology and cardiac surgery, was born from the spontaneous aggregation of a committee of experts, supported by duly appointed Italian Scientific Societies and guided by a national agency for accreditation. The ultimate aim is to standardize both procedures and results for future planning within the national Health System.

Percutaneous treatment of abdominal coarctation in children using a covered stent.

Coarctation of the abdominal aorta is extremely rare. It generally involves a long segment of the descending aorta and causes uncontrolled and unexplainable hypertension in children. The therapeutic choice is very challenging because acute and chronic complications are reported for both the surgical and the percutaneous approaches. The two reported cases of abdominal coarctation were treated primarily and successfully through the use of covered stents. Three covered stents were implanted in two children. No complication occurred with either procedure. At this writing, an 18-month follow-up assessment has found the patients in good health with no restenosis at the coarctation site. Covered stent implantation in children with abdominal coarctation is a feasible, safe, and effective procedure. It provides adequate relief of symptoms and reduces the risk of aneurysm formation. To avoid covering important side branches with polytetrafluoroethylene, this type of procedure must be preceded by precise study of the aorta and its branches.

Rescue treatment by percutaneous closure of interatrial septal defect or PFO in infants with Berlin heart.

During the past two decades, important progress has been achieved in the treatment of end-stage congestive heart failure in newborns and infants. The use of ventricular assist devices (VAD) in these patients is now available as a bridge to heart transplantation. The use of a VAD may reveal the presence of a silent interatrial septal defect or a patent foramen ovale (PFO), inducing a right to left shunt resulting in systemic desaturation and hemodynamic instability. We present two cases of low weight infants on circulatory support with VADs and right to left shunt through interatrial septum that were successfully treated by percutaneous intervention with an occlusion device.

Subclinical cardiac dysfunction and exercise performance in childhood cancer survivors.

BACKGROUND: Although anthracycline cardiotoxicity is clearly related to the cumulative dose administered, subclinical cardiac dysfunction has been reported across a wide range of treatment regimens, and its clinical significance is still unclear. Purpose of this study is to investigate by exercise echocardiography for subclinical cardiac dysfunction in survivors of pediatric cancer treated with low-moderate anthracycline doses, and to evaluate whether it may alter the response of the cardiovascular system to dynamic exercise. PROCEDURE: Post-exercise left ventricular end-systolic wall stress (ESS), left ventricular posterior wall dimension and percent thickening at end systole, and cardiopulmonary exercise test-derived indexes of cardiac function were examined in 55 apparently healthy patients (mean age 13.5 ± 2.9 years, median anthracycline cumulative dose 240 mg/m(2)) and in 63 controls. RESULTS: Subclinical cardiac dysfunction was identified in 17 patients (30%) presenting reduced left ventricular posterior wall dimension or percent thickening, or increased values of left ventricular ESS as compared to controls (group A), while the remaining patients formed group B. Reduced oxygen consumption at peak exercise in both groups of patients was the only cardiopulmonary exercise test variable resulting significantly different between patients and controls: no differences were found among the groups of patients. CONCLUSIONS: Our results confirm that even patients treated with a median anthracycline dose of 240 mg/m(2) (range 100-490) are at considerable risk of exhibiting subclinical cardiac dysfunction that, however, does not seem to alter the physiologic response of the cardiovascular system to dynamic exercise.

Natural history and outcome of hepatic vascular malformations in a large cohort of patients with hereditary hemorrhagic teleangiectasia.

BACKGROUND: Hereditary hemorrhagic telangiectasia is a genetic disease characterized by teleangiectasias involving virtually every organ. There are limited data in the literature regarding the natural history of liver vascular malformations in hemorrhagic telangiectasia and their associated morbidity and mortality. AIM: This prospective cohort study sought to assess the outcome of liver involvement in hereditary hemorrhagic telangiectasia patients. METHODS: We analyzed 16 years of surveillance data from a tertiary hereditary hemorrhagic telangiectasia referral center in Italy. We considered for inclusion in this study 502 consecutive Italian patients at risk of hereditary hemorrhagic telangiectasia who presented at the hereditary hemorrhagic telangiectasia referral center and underwent a multidisciplinary screening protocol for the diagnosis of hereditary hemorrhagic telangiectasia. Of the 502 individuals assessed in the center, 154 had hepatic vascular malformations and were the subject of the study; 198 patients with hereditary hemorrhagic telangiectasia and without hepatic vascular malformations were the controls. Additionally, we report the response to treatment of patients with complicated hepatic vascular malformations. RESULTS: The 154 patients were included and followed for a median period of 44 months (range 12-181); of these, eight (5.2%) died from VM-related complications and 39 (25.3%) experienced complications. The average incidence rates of death and complications were 1.1 and 3.6 per 100 person-years, respectively. The median overall survival and event-free survival after diagnosis were 175 and 90 months, respectively. The rate of complete response to therapy was 63%. CONCLUSIONS: This study shows that substantial morbidity and mortality are associated with liver vascular malformations in hereditary hemorrhagic telangiectasia patients.

Stenting of aortic coarctation and exercise-induced hypertension in the young.

OBJECTIVES: To evaluate whether the stenting of aortic coarctation enhance the risk of exercise-induced hypertension (EIH). BACKGROUND: There is the theoretical concern that aortic stents may cause increased aortic wall impedance and therefore systolic hypertension during exercise. METHODS: Blood pressure and the Doppler derived peak and mean systolic pressure gradient (PSG and MSG) across the distal aorta at the peak of exercise were evaluated in young patients (mean age 14 +/- 3 years) with aortic coarctation successfully treated with surgery or with stent implantation at least 1 year before the test. Only patients who reached the 85% maximal predicted heart rate or whose exercise test was interrupted because of severe hypertension, and in whom significant aortic narrowings were excluded by a MRI or a CT scan performed in the six months preceding the exercise test were included in the study. RESULTS: Seventeen patients formed the surgery-group, while 15 patients the stent-group. Patients in surgery-group were younger at coarctation repair and with a longer follow-up than those in stent-group. No difference was present regarding age, body surface area, gender, and presence, and degree of mildly hypoplastic aortic segments between the two groups as well as between patients with or without EIH. EIH was found in 35% of surgery-group patients and in 33% of stent-group patients. PSG and MSG were similar in the patients with or without EIH. CONCLUSIONS: EIH can be found in a high number of young patients successfully treated for aortic coarctation but at intermediate follow-up stent implantation does not seem to enhance the risk of EIH.

Long-term cardiovascular effects of pre-transplant native kidney nephrectomy in children.

Left ventricular (LV) hypertrophy (H) and hypertension are prevalent in children with end-stage renal disease (ESRD) and after renal transplantation. Severe hypertension prior to renal transplantation has traditionally been an indication for native kidney nephrectomy. The impact of nephrectomy on cardiovascular disease has not been well documented. We retrospectively evaluated echocardiographic and ambulatory blood pressure monitoring (ABPM) data in 67 young adults who had undergone transplantation in the pediatric age with a mean follow-up of 10.4 years. Unilateral or bilateral nephrectomies had been performed in 32 patients. The number of antihypertensive drugs used prior to transplantation was significantly higher in the nephrectomized groups. At follow-up the amount of antihypertensive medications was similar between groups and no significant differences were observed in mean arterial blood pressure (MAP) or LV mass index (LVMi). LVH was observed in 50% of non-nephrectomized patients, 45.4% of patients with unilateral nephrectomy, and 44.4% of patients without native kidneys (p = n.s.). In conclusion, unilateral or bilateral nephrectomies prior to transplantation do not appear to influence blood pressure control or the prevalence of LVH after renal transplantation. Longitudinal studies with repeated assessment of LVMi, before and after renal transplantation, are needed to assess the impact of residual activity of native kidneys on arterial blood pressure and cardiac structural changes, even in normotensive patients, to evaluate cardiovascular morbidity.

Pneumatic pulsatile ventricular assist device as a bridge to heart transplantation in pediatric patients.

Despite the remarkable advances with the use of ventricular assist devices (VAD) in adults, pneumatic pulsatile support in children is still limited. We report on our experience in the pediatric population. A retrospective review of 17 consecutive children offered mechanical support with Berlin Heart as a bridge to heart transplant from February 2002 to April 2010 was conducted. The median patient age was 3.9 years (75 days to 13.3 years). The median patient weight was 14.1 kg (2.9-43kg). Before VAD implantation, all children were managed by multiple intravenous inotropes and mechanical ventilation (14) or extracorporeal membrane oxygenation (3). All patients had right ventricular dysfunction. Nine patients required biventricular mechanical support (BVAD), but in all other cases a single left ventricular assist device proved sufficient (47%). The median duration of VAD support was 47 days (1-168 days). The median pre-VAD pulmonary vascular resistance index (Rpi) was 5.7 WU/m(2) (3.5 to 14.4WU/m(2) ). Eleven patients (65%) were successfully bridged to heart transplantation after a median duration of mechanical support of 68 days (6-168 days). Six deaths occurred (35%), three for neurological complications, one for sepsis, and two others for device malfunctioning. Since 2007, the survival rate of our patients has increased from 43% to 80%, and the need for BVAD has decreased from 86% to 30%. In two patients with Rpi >10WU/m(2) , unresponsive to pulmonary vasodilatator therapy, Rpi dropped to 2.2 and 2WU/m(2) after 40 and 23 days of BVAD support, respectively. Seven patients (41%) required at least one pump change. Of 11 patients undergoing heart transplant, four developed an extremely elevated (>60%) panel reactive antibody by enzyme-linked immunosorbent assay, confirmed by Luminex. All of them experienced at least one acute episode of rejection in the first month after heart transplant, needing plasmapheresis. The survival rate after heart transplantation was 100% with a median follow-up of 25.4 months (6 days to 7.7 years). Mechanical support in children with end-stage heart failure is an effective strategy as a bridge to heart transplantation with a reasonable morbidity and mortality. BVAD support may offer an additional means to reverse extremely elevated pulmonary vascular resistance.

Patient-specific three-dimensional aortic arch modeling for automatic measurements: clinical validation in aortic coarctation.

AIM: A validated algorithm for automatic aortic arch measurements in aortic coarctation (CoA) patients could standardize procedures for clinical planning. METHODS: The model-based assessment of the aortic arch anatomy consisted of three steps: first, machine-learning-based algorithms were trained on 212 three-dimensional magnetic resonance (MR) data to automatically allocate the aortic arch position in patients and segment the aortic arch; second, for each CoA patient (N = 33), the min/max aortic arch diameters were measured using the proposed software, manually and automatically, from noncontrast-enhanced three-dimensional steady-state free precession MRI sequence at five selected sites and compared ('internal comparison' referring to the same environment); third, moreover, the same min/max aortic arch diameters were compared, obtaining them independently, manually from common MR management software (MR Viewforum) and automatically from the model (external comparison). The measured sites were: aortic sinus, sino-tubular junction, mid-ascending aorta, transverse arch and thoracoabdominal aorta at the level of the diaphragm. RESULTS: Manual and software-assisted measurements showed a good agreement: the difference between diameter measurements was not statistically significant (at α = 0.05), with only one exception, for both internal and external comparison. A high coefficient of correlation was attained for both maximum and minimum diameters in each site (for internal comparison, R > 0.73 for every site, with P < 2 × 10). Notably, in tricuspid aortic valve patients external comparison showed no statistically significant difference at any measurement sites. CONCLUSION: The automatically derived aortic arch model, starting from three-dimensional MR images, could be a support to take the measurements in CoA patients and to quickly provide a patient-specific model of aortic arch anomalies.

Percutaneous closure of patent foramen ovale in patients with presumed paradoxical embolism: periprocedural results and midterm risk of recurrent neurologic events.

OBJECTIVE: To report our data on selected patients with previous paradoxical embolism who underwent transcatheter patent foramen ovale (PFO) closure. METHODS: Between July 2001 and July 2007, percutaneous PFO closure was performed on 128 patients (65 women, mean age: 46 +/- 12.8 years). Patent foramen ovale closure was recommended for secondary prevention in patients with previous transient ischemic attacks (52.5%), stroke (46%), or peripheral embolism (1.5%). RESULTS: Implantation was successful in all patients, and at the end of intervention, complete PFO closure was achieved in 70.3% of them. There were no "major" complications (ie, deaths, device embolization or thrombosis, need for cardiac surgery). The overall incidence of complications (mostly hemorrhagic) was 7%. The mean follow-up period was 32 months. Complete closure had been achieved in 78.4% and in 82.5% of patients at the third month of transesophageal echocardiography examination and at the sixth month of transcranial Doppler examination, respectively. There were no recurrent thromboembolic events during the follow-up period. CONCLUSIONS: Percutaneous closure of PFO is a feasible procedure, but it is not a risk-free technique. However, in correctly selected patients (ie, large PFO and those at risk for neurologic relapse), nearly complete PFO closure seems to provide protection from future neurologic ischaemic events at midterm follow-up.

Percutaneous rescue of left ventricular embolized amplatzer septal occluder device.

Transcatheter atrial septal defect (ASD) closure has proven to be safe and effective, and it is rapidly becoming the standard treatment for secundum-type ASDs. However, device embolization occurs in about 0.55% of cases, regardless of ASD and device size and/or physician's expertise. Percutaneous retrieval of the embolized device is possible in about 50% of cases, and several techniques have been described, including the use of large sheaths, gooseneck snares, or bioptomes. This is the first successful and reported case of retrieval of an embolized amplatzer septal occluder (ASO) from the left ventricle that was achieved by using a modified snare technique. This was done after an unsuccessful attempt at retrieval with a bioptome that led to a never previously reported complication. Of the 32 reported cases of ASO embolization, percutaneous retrieval was successful in 16 cases (50%) and none were obtained following left ventricular embolization. Even in experienced hands, the observed rate of embolization when using an ASO device is not negligible. Percutaneous retrieval is also possible from the left ventricle but in some cases nonstandardized devices and strategies may be required.

Aortic arch geometry and exercise-induced hypertension in aortic coarctation.

Hypertension at rest or during effort is not uncommon in patients with aortic coarctation (CoA), even those with a successful repair or mild degree of obstruction. Anatomic factors and functional abnormalities have been proposed as causes of this finding. Recently, aortic arch geometry was reported in association with hypertension at rest in patients with successful CoA repair. Forty-one patients (age 15.7 +/- 4.6 years) without significant obstruction at rest (mean systolic Doppler gradient at rest < or =25 mm Hg) were selected for the study. All patients underwent a maximal cardiopulmonary exercise test and magnetic resonance imaging of the aorta. Aortic arch shape was defined on global geometry as normal, gothic, and crenel. Percentage of anatomic narrowing (AN) was also calculated. Twenty-four patients (58%) showed exercise-induced hypertension (EIH). Regarding the shape of the aortic arch, normal geometry was present in 17 patients (41%), 9 (21%) had gothic geometry, and 15 (36%) had crenel geometry. There were no differences among the 3 geometries in regard to the incidence of EIH (70.6% in normal, 55.6% in gothic, and 46.7% in crenel) or AN (36.9% in normal, 33.5% in gothic, and 36.6% in crenel). In conclusion, our results fail to show a correlation between a specific aortic arch shape and the incidence of EIH and significant AN in patients with native or residual CoA or repeat CoA. Therefore, at present, the role of aortic arch geometry in identifying patients at risk of EIH is still uncertain.