Gianfranco Butera

Detection of Transposition of the Great Arteries in Fetuses Reduces Neonatal Morbidity and Mortality

BACKGROUND Transposition of the great arteries (TGA) is a life-threatening malformation in neonates, but it is amenable to complete repair. Prenatal detection, diagnosis, and early management may modify neonatal mortality and mortality. METHODS AND RESULTS Preoperative and postoperative morbidity and mortality were compared in 68 neonates with prenatal diagnosis and in 250 neonates with a postnatal diagnosis of TGA over a period of 10 years. The delay between birth and admission was 2+/-2.8 hours in the prenatal group and 73+/-210 hours in the neonatal group (P<0.01). Clinical condition at arrival, including metabolic acidosis and multiorgan failure, was worse in the neonatal group (P<0.01). Once in the pediatric cardiology unit, the management was identical in the 2 groups (atrioseptostomy, PGE1 infusion, operation date). Preoperative mortality was 15 of 250 (6%; 95% CI, 3% to 9%) in the neonatal group and 0 of 68 in the prenatal group (P<0.05). Postoperative morbidity was not different (25 of 235 versus 6 of 68), but hospital stay was longer in the neonatal group (30+/-17 versus 24+/-11 days, P<0.01). In addition, postoperative mortality was significantly higher in the neonatal group (20 of 235 versus 0 of 68, P<0.01); however, the known risk factors for operative mortality were identical in the 2 groups. CONCLUSIONS Prenatal diagnosis reduces mortality and morbidity in TGA. Prenatal detection of this cardiac defect must be increased to improve early neonatal management. In utero transfer of fetuses with prenatal diagnosis of TGA in an appropriate unit is mandatory.

Early and late complications associated with transcatheter occlusion of secundum atrial septal defect

OBJECTIVES The goal of this study was to report the early and late complications experienced in atrial septal defect (ASD) transcatheter closure. BACKGROUND Atrial septal defect transcatheter occlusion techniques have become an alternative to surgical procedures. A number of different devices are available for transcatheter ASD closure. The type and rate of complications are different for different devices. METHODS Between December 1996 and January 2001, 417 patients (mean age: 26.6 +/- 19 years) underwent transcatheter occlusion of secundum type ASD. Complications were categorized into major and minor. Two different devices were used: the CardioSEAL/STARFlex in 159 patients and the Amplatzer septal occluder in 258 patients. RESULTS Thirty-four patients experienced 36 complications during the hospitalization (8.6%, 95% confidence interval: 6.1% to 11.1%). Ten patients underwent elective surgical repair because of device malposition (three patients) or device embolization (seven patients). Twenty-four patients experienced 25 minor complications: unsatisfactory device position or embolization. Devices were retrieved using a gooseneck snare and/or a basket; 11 patients experienced arrhythmic problems. Other complications were: pericardial effusion, thrombus formation on the left atrial disc, right iliac vein dissection, groin hematoma, hemorrhage in the retropharynx and sizing balloon rupture. Two patients had late complications: peripheral embolization in the left leg one year after implantation of an Amplatzer device and sudden death 1.5 year later. CONCLUSIONS Our series of patients with ASD by transcatheter occlusion shows that the procedure is safe and effective in the vast majority of cases. To further reduce the complications rate, the criteria of device selection according to ASD morphology and some technical tips during implantation are discussed.

Transcatheter closure of perimembranous ventricular septal defects. Early and long-term Results

OBJECTIVES We sought to analyze safety, efficacy, and follow-up results of percutaneous closure of perimembranous ventricular septal defects (pmVSD). BACKGROUND Results of pmVSD transcatheter closure have been reported in the literature; however, follow-up data are still limited. METHODS Between January 1999 and June 2006, 104 patients underwent percutaneous closure of a pmVSD at our institution. An Amplatzer VSD device (muscular or eccentric) (AGA Medical Corp., Golden Valley, Minnesota) was used in all subjects. RESULTS The mean age at closure was 14 years (range 0.6 to 63 years). The attempt to place a device was successful in 100 patients (96.2%). The median device size used was 8 mm (range 4 to 16 mm). No deaths occurred. Total occlusion rate was 47% at completion of the procedure, rising to 84% at discharge and 99% during the follow-up. A total of 13 early complications occurred (11.5%), but in all but 2 subjects (1.9%) these were transient. The median follow-up was 38.5 months. The most significant complication was complete atrioventricular block (cAVB), which required pacemaker implantation in 6 subjects (5.7%; 2 in the early phase and 4 during the follow-up). Cox proportional hazards regression analysis showed that the only variable significantly associated with the occurrence of this complication was age at the time of the procedure (p = 0.028; relative risk 0.25). All subjects experiencing this problem were <6 years old. CONCLUSIONS In the current era and in experienced hands, pmVSD closure can be performed safely and successfully. The major concern is the occurrence of cAVB; therefore, very careful monitoring of rhythm is mandatory during follow-up.

Clinical ResearchCongenital Heart DiseaseTranscatheter Closure of Perimembranous Ventricular Septal Defects: Early and Long-Term Results

OBJECTIVES We sought to analyze safety, efficacy, and follow-up results of percutaneous closure of perimembranous ventricular septal defects (pmVSD). BACKGROUND Results of pmVSD transcatheter closure have been reported in the literature; however, follow-up data are still limited. METHODS Between January 1999 and June 2006, 104 patients underwent percutaneous closure of a pmVSD at our institution. An Amplatzer VSD device (muscular or eccentric) (AGA Medical Corp., Golden Valley, Minnesota) was used in all subjects. RESULTS The mean age at closure was 14 years (range 0.6 to 63 years). The attempt to place a device was successful in 100 patients (96.2%). The median device size used was 8 mm (range 4 to 16 mm). No deaths occurred. Total occlusion rate was 47% at completion of the procedure, rising to 84% at discharge and 99% during the follow-up. A total of 13 early complications occurred (11.5%), but in all but 2 subjects (1.9%) these were transient. The median follow-up was 38.5 months. The most significant complication was complete atrioventricular block (cAVB), which required pacemaker implantation in 6 subjects (5.7%; 2 in the early phase and 4 during the follow-up). Cox proportional hazards regression analysis showed that the only variable significantly associated with the occurrence of this complication was age at the time of the procedure (p = 0.028; relative risk 0.25). All subjects experiencing this problem were <6 years old. CONCLUSIONS In the current era and in experienced hands, pmVSD closure can be performed safely and successfully. The major concern is the occurrence of cAVB; therefore, very careful monitoring of rhythm is mandatory during follow-up.

Melody transcatheter pulmonary valve implantation. Results from the registry of the Italian society of pediatric cardiology

Percutaneous implantation of pulmonary valve has been recently introduced in the clinical practice. Our aim was to analyze data of patients treated in Italy by using the Melody Medtronic valve.

Transcatheter closure of atrial septal defect in young children: Results and follow-up

OBJECTIVES This study sought to analyze the safety, efficacy, and follow-up results of percutaneous closure of secundum atrial septal defect (ASD) in young children. BACKGROUND Results of ASD transcatheter closure in adults are widely reported but there are no large published series concerning young children. METHODS Between December 1996 and February 2002, 48 of 553 patients percutaneously treated at our institution were children age <or=5 years. Indications for closure were: elective closure in 32 patients; frequent respiratory infections in 8; failure to thrive in 2; liver transplantation in 5; and a fenestrated Fontan in 1. The procedure was carried out under general anesthesia with fluoroscopy and transesophageal control. Two different devices were used: 1) the CardioSEAL/StarFLEX (CS/SF) and 2) the Amplatzer septal occluder (ASO). Basal physical examinations and echocardiograms were performed prior to the procedure and at follow-ups (1, 6, and 12 months, and yearly thereafter). RESULTS The mean age at closure was 3.6 +/- 1.3 years. A CS/SF was used in 10 subjects; an ASO was used in 38 patients. No deaths or immediate major complications occurred. The total occlusion rate was 87% at procedure, rising to 94% at discharge. The mean follow-up was 18 +/- 14 months. No midterm major or minor complications occurred. The occlusion rate rose to 100% at 12 months of follow-up. Symptomatic patients improved significantly. CONCLUSIONS In the current era and in experienced hands, ASD closure can be performed safely and successfully, even in very young children.

Quality of life and perceived health status in surviving adults with univentricular heart

OBJECTIVE To evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics. DESIGN AND SETTING Retrospective, cross sectional study conducted in a regional paediatric cardiology centre. PATIENTS The health records of 89 survivors with univentricular heart (median age 21 years; range 17–49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Dukes measures was assessed. RESULTS The Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (p = 0.05) and perceived health measures (p = 0.02). A higher educational level predicted a better score for physical (p = 0.004), mental (p = 0.01), and general health measures (p = 0.02). Orthopaedic problems worsened the social score (p = 0.05). Psychosocial problems worsened the pain score (p = 0.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (p = 0.02). Patients younger than 23 years scored better for almost all health and dysfunction measures. CONCLUSIONS Despite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life.

Treatment of isolated secundum atrial septal defects: Impact of age and defect morphology in 1,013 consecutive patients

BACKGROUND Percutaneous closure of atrial septal defect (ASD) is a valid alternative to surgical approach. Current device has significantly improved the success rate also in complex cases. The aim of this study is to assess the impact of age, defect size, and morphologic features on successfully percutaneous ASD closure. METHODS Between January 2000 and September 2004, 1,013 consecutive patients underwent closure of an isolated type II ASD at our institution. The following outcomes have been evaluated: (1) role of percutaneous ASD closure as alternative to surgical repair, in current daily practice; (2) impact of age on the selected closure approach; (3) analysis of morphologic variety of ASD and its effect on the closure technique; (4) possible role of specific device selection according to ASD morphology to improve procedural success. RESULTS During the study period, up to 80% of secundum ASDs were suitable for percutaneous closure with the currently available devices. Need for surgical ASD closure was more common in pediatric patients, likely reflecting the more frequent diagnosis of larger and complex defects at a young age. Accurate ADS morphology assessment and appropriate device selection are key elements to obtain procedural success. In particular, among all the ASD characteristics, the rim absence is the main limiting factor to a successful percutaneous ASD closure. A trend of reduction in peri-procedural adverse events was observed during the study period, with complications needing immediate cardiac surgery occurred only in 1% of cases. CONCLUSIONS Percutaneous ASD closure is feasible and associated with low complication rate. A thorough analysis of morphologic aspects is mandatory in order to select the appropriate device and the optimal approach. Surgical closure remains the treatment of choice in selected patients.

Relation of genotype 22q11 deletion to phenotype of pulmonary vessels in tetralogy of Fallot and pulmonary atresia–ventricular septal defect

Objective To compare the morphology of the pulmonary vessels in tetralogy of Fallot or pulmonary atresia–ventricular septal defect (PA–VSD) with (del22q) and without 22q11 deletion (non-del22q). Patients 94 consecutive infants (54 with tetralogy of Fallot, 40 with PA–VSD) were studied using ultrasound and catheterisation. Molecular investigations Identification of the 22q deletion was performed either by fluorescent in situ hybridisation or polymerisation chain reaction genotyping. Results 25 patients were del22q (16/40 (40%) PA–VSD v 9/54 (17%) tetralogy of Fallot; p < 0.02). Major aortopulmonary collateral arteries was more common in patients with PA–VSD-del22q (p < 0.03). Such collaterals were identified in 13 patients: 10 del22q and three non-del22q (p < 0.001). The size of the right and left pulmonary arteries expressed as a standard deviation (SD) difference of the normal range was −4.2 (quartiles −5.3 and −2.9) for PA–VSD del22q, and −2.6 (−3.1 and −1.8) for PA–VSD non-del22q (p = 0.02). The mean (SD) difference between the measured and theoretical Nakata index was −373 (94) for PA–VSD del22q v−245 (93) in PA–VSD non-del22q (p = 0.0002). In tetralogy of Fallot patients with and without del22q, the size of the pulmonary arteries was similar (p = 0.6). Conclusions A “specific” phenotype could be defined in patients with deletion: PA–VSD, major aortopulmonary collateral arteries with complex loop morphology, and small central pulmonary arteries. Differences in the morphology of the pulmonary vessels may indicate a different timing of the faulty developmental pathway in patients with and without 22q11 deletion.

Percutaneous versus surgical closure of secundum atrial septal defects: a systematic review and meta-analysis of currently available clinical evidence

AIMS To summarise data from studies comparing surgical (SC) versus percutaneous closure (PC) of atrial septal defects (ASDs). METHODS AND RESULTS Electronic databases, journals and major international conference proceedings were systematically searched for pertinent clinical studies comparing the two methods of closure (percutaneous and surgical) published up to December 2008, including only those reporting on more than 20 patients. Primary endpoints: occurrence of death and of total and major early complications. Thirteen original studies (3,082 patients) were included. All studies were non-randomised. One death was reported in the surgical group (0.08%; 95% C.I. 0-0.23%). Analysis of postprocedural complications showed a 31% rate (95% CI 21-41%) in SC patients and a 6.6% rate (95% CI 3.9-9.2%) in PC subjects. The adjusted OR for SC vs. PC total complications was 5.4 (95% CI 2.96-9.84; p<0.0001), significantly in favour of PC. The postprocedural major complication rate was 6.8% (95% CI 4-9.5%) in SC patients and 1.9% (95% CI 0.9-2.9%) in PC patients. The adjusted OR for SC vs. PC major complications was 3.81 (95% CI 2.7-5.36; p=0.006), again favouring PC. CONCLUSIONS The largest cohort to date of patients with secundum ASD shows that treatment by a percutaneous approach has a significantly lower rate of either total or major early postprocedural complications compared to surgery.