Search

Muscular ventricular septal defects (MVSDs) account for approximately 20% of all congenital ventricular septal defects. Large defects in infants result in early heart failure, failure to thrive and pulmonary hypertension. Although percutaneous closure of MVSDs has been employed safely and effectively in children, adolescents and adults, its application in the small infant (weight <6 kg) carries a higher risk for complications including arrhythmias, hemodynamic compromise, cardiac perforation, tamponade and death. Perventricular closure of such defects, introduced by Amin and coworkers in the late 1990s, has become an attractive treatment modality for these small and high-risk patients. Experience worldwide has shown that the procedure is feasible, reproducible, safe and effective. In this article, the authors review the indications, the step-by-step technique and the results of perventricular closure of MVSDs using the AMPLATZER mVSD device (AGA Medical, MN, USA). [ABSTRACT FROM AUTHOR]

We report a case wherein a dysmorphic four-month-old infant (weighing 4.5 kgs) with an 8 mm atrial septal defect (ASD), a 1.5 mm patent ductus arteriosus (PDA), a 2 mm mid-muscular ventricular septal defect (VSD) associated with chronic lung disease, and severe pulmonary hypertension, was successfully managed using a hybrid approach, without the use of cardiopulmonary bypass (CPB). Through a median sternotomy, the PDA was ligated and the ASD was closed with a 9 mm Amplatzer septal occluder implanted through peratrial access. The VSD was left untouched. Serial echocardiograms showed complete closure of the ASD and PDA, with progressive normalization of the pulmonary artery (PA) pressures within three months. The child rapidly gained weight and was weaned from sildenafil and oxygen administration. After 12 months, the VSD closed spontaneously and the child remained well, with normal PA pressures. A hybrid approach without the use of CPB should be considered in the management of infants with congenital heart disease, associated with chronic lung disease and pulmonary hypertension.

OBJECTIVE: To evaluate the initial clinical experience with the Helex septal occluder for percutaneous closure of atrial septal defects. METHODS: Ten patients underwent the procedure, 7 patients with ostium secundum atrial septal defects (ASD) with hemodynamic repercussions and 3 patients with pervious foramen ovale (PFO) and a history of stroke. Mean age was 33.8 years and mean weight was 55.4 kg. Mean diameter by transesophageal echocardiography and mean stretched ASD diameter were 11.33 ± 3.3mm, and 15.2 ± 3.8mm, respectively. The Qp/Qs ratio was 1.9 ± 0.3 in patients with ASD. RESULTS: Eleven occluders were placed because a patient with 2 holes needed 2 devices. It was necessary to retrieve and replace 4 devices in 3 patients. We observed immediate residual shunt (< 2mm) in 4 patients with ASD, and in those with patent foramen ovale total occlusion of the defect occurred. No complications were noted, and all patients were discharged on the following day. After 1 month, 2 patients with ASD experienced trivial residual shunts (1mm). In 1 patient, we observed mild prolapse in the proximal disk in the right atrium, without consequences. CONCLUSION: The Helex septal occluder was safe and effective for occluding small to moderate atrial septal defects. Because the implantation technique is demanding, it requires specific training of the operator. Even so, small technical failures may occur in the beginning of the learning curve, but they do not involve patient safety.

Abstract We report a technique to palliate hypoplastic left heart syndrome, with no PDA stenting, but with double polytetrafluoroethylene shunt from pulmonary artery to ascending and descending aorta by combined thoracotomies. A 30-day-old female was operated with this technique. Five months after first operation, the child was submitted to Norwood/Glenn operation. Good hemodinamic recovery and initial clinical evolution was observed. The child was extubated in 8th post operatory day and reentubated in the next day due to pulmonary infection. Despite antibiotic treatment, the child died after systemic infectious complications.

Stenting for CoA has become an acceptable treatment modality in the last 20 years. However little is known about arterial changes after this procedure. To assess arterial structure and function including peripheral reactivity and stiffness and intima-media thickness (IMT) pre and post stenting for coarctation of the aorta (CoA). Twenty-one patients [median age: 15 years (8-39)] were studied at baseline, 1 day, 6 months and 1 year after stenting. Twenty-one healthy subjects (1:1 matched) were used as controls. Left ventricular (LV) mass, ejection fraction, flow-mediated dilation (FMD) and nitrate-mediated dilation (NMD) of left brachial artery, common carotid (CC) and right subclavian artery (RSCA) IMT and pulse wave velocity (PWV) were assessed by echocardiography and vascular ultrasound. CoA patients had higher LV indexed mass (p < 0.0001), impaired FMD (p < 0.0001) and NMD (p < 0.0001), increased PWV (p < 0.0001), carotid and RSCA IMT (both p < 0.0001). All procedures were successful and resulted in significant gradient reduction (p < 0.001). One year after stenting there was improvement in LV function (p = 0.034) and although there was significant reduction of LV mass (103.29 ± 24.77 vs. 74.39 ± 22.07 g/m(2), p < 0.0001) values did not normalize. There was no significant change in FMD, NMD, PWV and CC or RSCA IMT. In patients with CoA, arterial reactivity is impaired and LV mass, arterial stiffness and thickness are increased. Although stenting is successful to relieve the obstruction resulting in better LV function and mass reduction, arterial structure and function remains abnormal after 1 year of follow-up.

Objectives: To evaluate the mid-term outcomes after percutaneous closure of the secundum atrial septal defects (ASD) using the Figulla-Occlutech device (FOD)., Background: Transcatheter closure has become the method of choice for most patients with ASD. Although the FOD may have some advantageous characteristics there is a paucity of data on later outcomes after the use of this relatively new device., Methods: Observational, single arm study including 200 non-consecutive patients who underwent ASD closure between 04/09 and 07/15 in 2 centers. Device performance, deployment technique, and immediate and mid-term outcomes were assessed., Results: Median age and weight were 24 years (4-72) and 58 kg (15-92), respectively. Single defects were observed in 171 patients (median size of 19 mm). The remainder had multiple or multifenestrated defects. Implantation of FOD (median size of 24 mm) was successful in all (99%), but 2 patients (1 with deficient postero-inferior rim; 1 with a large ASD for the size of the child). Embolization with device retrieval occurred in 2 (1%). Median follow-up of 36 months was obtained in 172 patients. Serial echocardiographic assessment showed complete closure in all but 2 patients, in whom an additional small non-significant posterior defect was purposely left untouched. There have been no episodes of late arrhythmias, device embolization, cardiac erosion, endocarditis, thromboembolism, wire fracture, or death., Conclusions: Transcatheter closure of ASDs in older children, adolescents, and adults using the FOD was highly successful in a wide range of anatomical scenarios with high closure rates and no complications in mid-term follow-up., (© 2016, Wiley Periodicals, Inc.)

Objectives: We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures., Background: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology., Methods: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's., Results: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741., Conclusion: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures., (© 2015 Wiley Periodicals, Inc.)

Introduction: Cost-effectiveness (CE) studies of percutaneous (PC) versus surgical (SC) atrial septal defect closure are lacking., Methods: A systematic literature review in children and a CE analysis based on a model of long-term outcomes were performed. Direct costs of PC and SC were US$8700 (defined arbitrarily) and US$5700 (actually paid), respectively. Three-times the Brazilian GDI (US$28,700) per year of life saved (with a discount rate of 5%) was used as a limit for willingness-to-pay., Discussion: PC had a high (US$104,500) incremental CE ratio despite lower complication rates, shorter hospital stay and better (nonsignificant) adjusted life expectancy. PC would be cost-effective if it cost US$6400 or SC had an 8% loss of utility or its indirect costs were US$2250. Costs of PC should be reduced to be cost-effective in the Brazilian public health system. Indirect costs and impact on quality of life should be further assessed.

Objectives: We examine normalized air Kerma area product (PKA ) by body weight (PKA /BW) as a reference value of radiation dose and benchmark PKA /BW in pediatric laboratories using a multicenter registry database., Background: Reduction of radiation dose is an important quality improvement task in pediatric cardiac catheterization laboratories. Physicians need to agree on a standard method of reporting radiation dose that would allow comparisons to be made between operators and institutions., Methods: This was a multicenter observational study of radiation dose in pediatric laboratories. Patient demographic, procedural and radiation data including fluoroscopic time and PKA (µGy m(2) ) were analyzed. PKA /BW was obtained by indexing PKA to body weight., Results: A total of 8,267 pediatric catheterization procedures (age <18 years) were included from 16 institutions. The procedures consisted of diagnostic (n = 2,827), transplant right ventricular (RV) biopsy (n = 1,172), and interventional catheterizations (n = 4268). PKA correlated with body weight better than with age and best correlated with weight-fluoroscopic time product. PKA /BW showed consistent values across pediatric ages. Interventional catheterizations had the highest PKA /BW (50th, 75th, and 90th percentiles: 72, 151, and 281 μGy m(2) /kg), followed by diagnostic (59, 105, and 175 μGy m(2) /kg) and transplant RV biopsy (27, 79, and 114 μGy m(2) /kg)., Conclusion: PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc., (© 2014 Wiley Periodicals, Inc.)

Objectives: To evaluate the feasibility, safety, and efficacy of implantation of the new Nit Occlud ASD-R® (NOASD-R) device for percutaneous closure of ostium secundum atrial septal defects (ASD-OS)., Background: Device catheter implantation has become the method of choice for most patients with ASD-OS. No single device has proven to be ideal for this type of procedure. The NOASD-R has a distinct design that may help to overcome limitations of other devices., Methods: A prospective, single arm, observational study including all consecutive patients receiving the NOASD-R device for ASD-OS closure between October 2011 and September 2013 was performed. Patient selection, device design, deployment technique, complications, and procedural outcomes were evaluated., Results: Seventy-four patients underwent attempted transcatheter ASD-OS closure using the NOASD-R device. Implantation of the occluder was successful in 73 patients (98.6%). The majority of patients were female (79.5%) with a median age of 17.2 years (range: 2-74). A 2-D transthoracic color-Doppler echocardiogram (TTE) obtained at the 3 or 6 month follow-up visit showed complete occlusion of the ASD-OS in 72/73 patients (98.6%). At a mean follow-up interval of 11.4 ± 6.8 months there have been no episodes of late device embolization, cardiac perforation or erosion, endocarditis, thromboembolism, wire fracture, embolic neurologic events, or death., Conclusions: We report the first worldwide clinical experience using the NOASD-R device for ASD-OS closure. The procedure was feasible, with a high rate of successful implantations, and safe. High ASD-OS closure rates and no complications were encountered during short-term follow-up. © 2014 Wiley Periodicals, Inc., (© 2014 Wiley Periodicals, Inc.)

Fetal interventions have been performed for some congenital heart diseases. However, these procedures have not gained wide acceptance due to concerns about their efficacy and safety. The aim of this study was to report on a preliminary experience with fetal cardiac interventions in Brazil. Twenty-two cardiac interventions were performed in 21 fetuses. Thirteen fetuses had critical aortic stenosis (CAS), 4 had hypoplastic left heart syndrome (HLHS) and intact interatrial septum or small patent foramen ovale, 1 had pulmonary atresia with intact ventricular septum (IVS), and 3 had critical pulmonary stenosis (CPS). The main outcome variables evaluated were technical success and procedural complications as well as pregnancy and postnatal outcomes. Success was achieved in 20 of 22 procedures (91%) with 1 failed aortic and 1 failed pulmonary valvuloplasties. There was 1 fetal death. No maternal complications occurred. One patient with CAS, severe mitral regurgitation, and hydrops died postnatally within 5 months of age. All patients with HLHS and restrictive atrial septum died after interventional or surgical procedures and prolonged hospitalizations. All patients with CPS/IVS survived and achieved a biventricular (BV) circulation after neonatal valvuloplasty and ductal stenting. A BV circulation was achieved in 4 of 8 patients with CAS and evolving HLHS (one still in utero), including 2 with initial borderline left ventricles (LV) in whom surgical LV overhaul was performed at 9 months of age. In this preliminary experience, the feasibility of fetal cardiac interventions and their outcomes were similar to those previously reported.

Background: There is a scarcity of data comparing percutaneous and surgical closure of the secundum atrial septal defect (ASD)., Objectives: Assessment of safety and efficacy of both methods of treatment in a referral center affiliated with the Ministry of Health., Methods: Observational, prospective, non-randomized study of two cohorts of children and adolescents younger than 14 years, treated by catheterization or surgery. Data was collected prospectively in the percutaneous group (A) and retrospectively in the surgical group (B)., Results: A total of 75 patients (pts) were enrolled in group A from April 2009 to October 2011 and 105 pts were treated in group B from January 2006 to January 2011. Age was older and weight was higher in group B and the ASD diameter was similar in both groups. Technical success was achieved in all procedures and there were no deaths. Complications (most minor) occurred in 68% of group B and 4% of A (p < 0.001). Rates of total occlusion or non-significant residual shunts were similar in both groups. Median hospitalization time was 1.2 days in group A and 8.4 days in group B (p < 0.001)., Conclusion: Both treatment modalities are safe and effective, showing excellent outcomes. However, the percutaneous treatment has lower morbidity and shorter in-hospital stay length. These observations support the concept that percutaneous treatment of atrial septal defects should be regarded as the method of choice to manage selected patients with this condition.

Purpose of Review: To provide an overview of the various modalities that have emerged in the last years as adjunctive tools to provide detailed imaging of the cardiovascular system in the catheterization laboratory., Recent Findings: Completion angiography after cardiac surgery for congenital heart disease using a dedicated hybrid operating cardiac suite may aid in early diagnosis and therapy of postsurgical abnormalities. The application of three-dimensional rotational digital angiography results in a more precise delineation of the three-dimensional anatomy of vascular structures outside the heart with rapid image processing and reduction of the amount of contrast used and ionizing radiation dose. Combined catheterization/magnetic resonance (MR) hybrid laboratories have the potential to accurately assess changes in the anatomy and physiology pretranscatheter and posttranscatheter interventions. Fully MR-guided transcatheter procedures have become possible in animal models. Real-time three-dimensional transesophageal echocardiography (TEE) has helped to guide transcatheter procedures in septal and valvar structures. Miniaturization of probes has expanded the applications of TEE for smaller neonates. The use of intravascular ultrasound has helped to better understand the mechanisms of vascular diseases and transcatheter treatments., Summary: Knowledge and proper application of new imaging modalities during interventions in the catheterization laboratory may improve the outcomes of patients with congenital and acquired structural heart disease.

Objectives: To report on the early results of treatment of coarctation of the aorta by dilation with a new polytetrafluoroethylene covered stent., Background: Transcatheter dilation of aortic coarctation carries the risk of aneurysm or rupture. Covered stent implantation reduces this risk but requires a large delivery system. The Advanta V12 LD covered stent is premounted and requires a 9-11 Fr delivery system., Methods: Covered stents on balloons of a diameter sufficient to anchor the stent in the coarctation were implanted using the smallest available delivery system. Secondary dilation with larger diameter balloons was performed until the pressure gradient was <20 mm Hg and the stent was opposed to the aortic wall., Results: Twenty-five patients with aortic coarctation underwent stent implantation. Coarctation diameter increased from (6.3 + or - 3.5) mm to (14.4 + or - 2.3) mm (P < 0.0001). Peak pressure gradient decreased from (25.3 + or - 11.6) mm Hg to (2.5 + or - 3.0) mm Hg (P < 0.0001). The stent achieved the desired diameter in all cases. There were no complications. At short-term median follow-up of 4.9 months, all patients are alive and well with no evidence of recoarctation or aneurysm., Conclusions: These initial results show that the covered Advanta V12LD stent is safe and effective in the immediate treatment of coarctation of the aorta through a low profile delivery system of 8-11 Fr. Long term follow up is required.

Fistulas in the tracheobronchial tree (bronchopleural and tracheoesophageal fistulas) have a multifactorial etiology and present a variable incidence in the literature. In general, the related morbidity and mortality are high. Once such a fistula has been diagnosed, surgical closure is formally indicated. However, the clinical status of affected patients is usually unfavorable, which precludes the use of additional, extensive surgical interventions. In addition, attempts at endoscopic closure of these fistulas have seldom been successful, especially when the fistula is large in diameter. We report the cases of three patients submitted to endoscopic closure of fistulas, two of which were larger than 10 mm in diameter, by means of the insertion of atrial septal defect occluders. The procedure was minimally invasive, and the initial results were positive. The results indicate that this is a promising technique for the resolution of tracheobronchial tree fistulas.

Congenital heart diseases are one of the most common structural defects present at birth, with an approximate incidence of 8 per 1000 live births. As most countries in South America have a high birth rate, they are a significant public health concern. This paper provides a brief overview of the burden of congenital heart disease in South America, focusing on its local prevalence, facilities for treatment and outcomes after medical, surgical or catheter intervention for the most common diseases.

Permeability of a Blalock-Taussig shunt can increase the risk of endocarditis and ventricular overload. Percutaneous embolization of these shunts gives variable results. We report our experience in 10 patients with percutaneous closure of modified Blalock-Taussig shunts using retrograde arterial embolization with Gianturco coils. The patients' median age was 2.8 years, and their median weight was 12 kg. Most patients had minor stenosis of the distal portion of the anastomosis. In all cases, complete closure of the shunt was achieved without complications using a median of one coil per patient. The technique was feasible, safe, effective, and inexpensive.

Initial surgical reconstruction for hypoplastic left heart syndrome (HLHS) is associated with satisfactory outcomes only in a few referral centers. Moreover, there is a persistent high-risk period for sudden death while the patient waits for the next surgical procedure. The development of a less invasive approach, so-called "hybrid," postponing a major surgery outside the neonatal period, might reduce the immediate and late surgical burden on these patients. This is a retrospective study of a contemporary series of patients with HLHS seen in two separate institutions. Patients with HLHS or its variants who underwent a "hybrid" management were included in the study. Data are described as the mean and standard deviation or absolute numbers and percentage, as appropriate. From January 2004 to June 2006, 15 patients (10 male; 5 +/- 3.8 days old and 2.9 +/- 0.5 kg) were included in the study. Ten had both mitral and aortic atresia; the ascending aorta and atrial septal defect measured 2.5 +/- 1.4 and 4.9 +/- 1.2 mm, respectively. There were six hospital survivors after stage I (mortality rate 60%). During the interstage period, all but one patient needed additional procedures. One patient died of bacterial meningitis 4 months after stage I. Four patients were submitted to stage II operation at 6.6 +/- 0.5 months of age and one is waiting for the operation. All four required early reinterventions for pulmonary artery stenosis. Only one was discharged home and was not yet submitted to the third stage. The hybrid approach for HLHS was associated with poor results in this early experience from two independent institutions in a developing country. This might have been related to infrastructure and technical problems, as well as our own learning curve. Institutions working under the same conditions might face similar problems during their initial experience.

Purpose: We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini adjustable banding system., Description: Through a mid-sternotomy, a 5-day-old neonate underwent bilateral pulmonary artery banding using this new system, combined with placement of a main pulmonary artery to the innominate artery shunt., Evaluation: The patient had an uneventful postoperative course. Three percutaneous adjustments of the banding system were necessary to keep the arterial oxygen saturation in the 75% to 85% range. On day 48 of life, she was submitted to stent placement (6 mm) within the atrial septum to treat a restrictive atrial septal defect. Afterward, seven additional percutaneous adjustments of the banding system were necessary. The Norwood operation and the bidirectional Glenn shunt were carried out on the day 106 of life. The bands were removed with no pulmonary artery distortion., Conclusions: The clinical use of this innovative pulmonary artery banding system was feasible, safe, and effective. This allowed for customization of the pulmonary blood flow according to the underlying clinical needs, resulting in a more precise balance between the pulmonary and systemic circulations.

Objectives: To describe a series of 8 consecutive infants (5 with transposition of the great arteries [TGA] and 3 with hypoplastic left heart syndrome [HLHS]) who underwent nonconventional septostomy techniques., Background: For some complex congenital heart defects, an unrestrictive atrial septal defect (ASD) is essential to achieve an adequate cardiac output and/or systemic saturation. In some scenarios, the use of conventional septostomy techniques may be technically difficult, hazardous, and/or ineffective., Methods: Use of transhepatic approach, cutting balloons, and radiofrequency perforation with stenting of the atrial septum., Results: The size of the ASD and the oxygen saturation increased in all patients with no major complications. In those with TGA, the ASDs were considered to be of good size at the arterial switch operation. Two of the 3 patients with hybrid palliation for HLHS have developed some degree of obstruction within the interatrial stent over 2-3 months. At surgery, the stents were found to be secured within the septum with one showing significant fibrous ingrowth after uneventful removal. The other had some nonobstructive ingrowth., Conclusions: Creation or enlargement of ASDs in infants using new nonconventional transcatheter techniques is feasible, safe, and effective, at least in the short-to-mid-term follow-up. Infants with TGA seem to benefit the most because the procedure results in satisfactory clinical stability for subsequent early surgical intervention. In infants with HLHS palliated by a hybrid approach, stent implantation to the atrial septum seems to buy enough time to bring them to the phase II safely despite progressive in-stent obstruction., ((c) 2007 Wiley-Liss, Inc.)

Although uncommon, significant postoperative residual leaks may occur after repair of any type of ventricular septal defect (VSD). Post-traumatic VSDs are even rarer, but can be occasionally seen after penetrating or blunt chest trauma. When these defects are associated with significant left-to-right shunting (Qp/Qs > 1.5) with persistent left ventricular volume overload, intervention is generally recommended. Surgical treatment requires cardiopulmonary bypass with its attendant morbidity, increased hospital stay and possible long-term neurological impairment. With the evolving experience of transcatheter closure of postinfarction and native perimembranous and muscular VSDs, this less invasive method became an attractive alternative to manage these postoperative and post-traumatic defects. In this paper, we report on 3 patients with such residual leaks after repair of a perimembranous defect, which was closed using a perimembranous Amplatzer VSD occluded after a failed attempt using a NitOcclud coil. One patient had a mid-muscular post-traumatic defect after a penetrating chest wound, and another patient had a residual leak after a patch repair of a large post-traumatic muscular defect with outlet extension after a blunt chest trauma. Both defects were closed using muscular Amplatzer VSD occluders. All procedures were uncomplicated, and there were no technical difficulties with device implantation. All 3 patients' defects were completely closed at follow up. Percutaneous closure of traumatic and residual postoperative VSDs appears to be safe and effective. A larger number of patients and longer follow-up period are needed before the widespread use of this technique can be recommended.

Background: We report a multiinstitutional study on intermediate-term outcome of intravascular stenting for treatment of coarctation of the aorta using integrated arch imaging (IAI) techniques., Methods and Results: Medical records of 578 patients from 17 institutions were reviewed. A total of 588 procedures were performed between May 1989 and Aug 2005. About 27% (160/588) procedures were followed up by further IAI of their aorta (MRI/CT/repeat cardiac catheterization) after initial stent procedures. Abnormal imaging studies included: the presence of dissection or aneurysm formation, stent fracture, or the presence of reobstruction within the stent (instent restenosis or significant intimal build-up within the stent). Forty-one abnormal imaging studies were reported in the intermediate follow-up at median 12 months (0.5-92 months). Smaller postintervention of the aorta (CoA) diameter and an increased persistent systolic pressure gradient were associated with encountering abnormal follow-up imaging studies. Aortic wall abnormalities included dissections (n = 5) and aneurysm (n = 13). The risk of encountering aortic wall abnormalities increased with larger percent increase in CoA diameter poststent implant, increasing balloon/coarc ratio, and performing prestent angioplasty. Stent restenosis was observed in 5/6 parts encountering stent fracture and neointimal buildup (n = 16). Small CoA diameter poststent implant and increased poststent residual pressure gradient increased the likelihood of encountering instent restenosis at intermediate follow-up., Conclusions: Abnormalities were observed at intermediate follow-up following IS placement for treatment of native and recurrent coarctation of the aorta. Not exceeding a balloon:coarctation ratio of 3.5 and avoidance of prestent angioplasty decreased the likelihood of encountering an abnormal follow-up imaging study in patients undergoing intravascular stent placement for the treatment of coarctation of the aorta. We recommend IAI for all patients undergoing IS placement for treatment of CoA., ((c) 2007 Wiley-Liss, Inc.)

Background: We report a multi-institutional experience with intravascular stenting (IS) for treatment of coarctation of the aorta., Methods and Results: Data was collected retrospectively by review of medical records from 17 institutions. The data was broken down to prior to 2002 and after 2002 for further analysis. A total of 565 procedures were performed with a median age of 15 years (mean=18.1 years). Successful reduction in the post stent gradient (<20 mm Hg) or increase in post stent coarctation to descending aorta (DAo) ratio of >0.8 was achieved in 97.9% of procedures. There was significant improvement (P<0.01) in pre versus post stent coarctation dimensions (7.4 mm+/-3.0 mm vs. 14.3+/-3.2 mm), systolic gradient (31.6 mm Hg+/-16.0 mm Hg vs. 2.7 mm Hg+/-4.2 mm Hg) and ratio of the coarctation segment to the DAo (0.43+/-0.17 vs. 0.85+/-0.15). Acute complications were encountered in 81/565 (14.3%) procedures. There were two procedure related deaths. Aortic wall complications included: aneurysm formation (n=6), intimal tears (n=8), and dissections (n=9). The risk of aortic dissection increased significantly in patients over the age of 40 years. Technical complications included stent migration (n=28), and balloon rupture (n=13). Peripheral vascular complications included cerebral vascular accidents (CVA) (n=4), peripheral emboli (n=1), and significant access arterial injury (n=13). Older age was significantly associated with occurrence of CVAs. A significant decrease in the technical complication rate from 16.3% to 6.1% (P<0.001) was observed in procedures performed after January 2002., Conclusions: Stent placement for coarctation of aorta is an effective treatment option, though it remains a technically challenging procedure. Technical and aortic complications have decreased over the past 3 years due to, in part, improvement in balloon and stent design. Improvement in our ability to assess aortic wall compliance is essential prior to placement of ISs in older patients with coarctation of the aorta., (Copyright (c) 2007 Wiley-Liss, Inc.)

We report a case in which a neonate with complete transposition of the great arteries was submitted to an atrial septostomy through transhepatic access due to congenital interruption of the inferior vena cava. The technical aspects of the procedure are discussed.

The association of a right aortic arch with an ipsilateral patent ductus arteriosus is rare, especially when there are no other intracardiac anomalies. We report three female patients aged 26, 35 and 9 years with this combination in whom previous attempts at surgical closure by thoracotomy and sternotomy were unsuccessful and who subsequently underwent successful percutaneous closure of the defects using Amplatzer devices. In two patients, although angiography demonstrated the presence of type-A patent ductus arteriosus, it was not possible to determine the minimum diameter accurately and it was necessary to measure it using a sizing balloon. An Amplatzer duct occluder was used in two patients and an Amplatzer muscular ventricular septal defect occluder, in the other. In all patients, full closure was confirmed in the catheterization laboratory and the patients were discharged on the same day with no complications. Percutaneous closure of a right patent ductus arteriosus associated with a right aortic arch is feasible, safe and effective.

Background: Congenital heart defects are the result of incomplete heart development and, like many diseases, have been associated with high homocysteine concentration., Methods: We evaluated homocysteine, folic acid and vitamin B(12) concentrations, and the mutations 677C>T and 1298A>C in MTHFR, 844ins68 in CBS and 2756A>G in MTR genes in 58 patients with congenital heart defects, 38 control subjects, and mothers of 49 patients and 26 controls., Results: Control and patients presented normal range concentrations for homocysteine (7.66 +/- 3.16 microM and 6.95 +/- 3.12 microM, respectively), folic acid (8.31 +/- 3.00 ng/mL and 11.84 +/- 10.74 ng/mL) and vitamin B(12,) (613.56 +/- 307.57 pg/mL and 623.37 +/- 303.12 pg/mL), which did not differ among groups. For the mothers studied, homocysteine and vitamin B(12) concentrations also did not differ between groups. However, folic acid concentrations of mothers showed significant difference, the highest values being in the group of patients. No difference was found in allele frequencies among all groups studied., Conclusions: In the studied groups, high homocysteine seems not to be correlated with congenital heart defects, as well as folic acid and vitamin B(12). The mutations studied, in isolation, were not related to congenital heart defects, but high concentration of maternal homocysteine is associated with the presence of three or four mutated alleles.

Objective: Hypoplastic left heart syndrome remains a challenge for worldwide surgeons. Initial palliation employing bilateral pulmonary artery banding along with ductal stent implantation and atrial septostomy has been proposed as an alternative approach. However, the surgically placed bands are fixed and may become inadequate after sternum closure or with somatic growth of the patient. We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini banding system that allows for fine percutaneous adjustments of pulmonary blood flow., Method: Through a mid sternotomy, a 5 day-old neonate underwent bilateral pulmonary artery banding using this new system combined with placement of a main pulmonary artery to innominate artery shunt., Results: The patient had an uneventful postoperative course. Three percutaneous adjustments of the banding system were necessary to keep the arterial oxygen saturation in the 75%-85% range. On the 48th day of life, she was submitted to stent placement (6 mm) within the atrial septum to treat a restrictive atrial septal defect. The Norwood operation and the bidirectional Glenn shunt were carried out on the 106th day of life. The bands were removed with no distortion of the pulmonary arteries., Conclusions: The clinical use of this innovative pulmonary artery banding system was feasible, safe and effective. It allowed for customization of the pulmonary blood flow according to the underlying clinical needs, resulting in a more precise balance between the pulmonary and systemic circulations.

Background: The new Amplatzer device has been used with success for occlusion of the perimembranous ventricular septal defects and the index of complications is very low., Results: The reported case corresponds to a very rare severe acute tricuspid stenosis, caused by adherence of the prosthesis to the anterior tricuspid leaflet., Conclusion: Device release was achieved by manipulation on the tricuspid apparatus.

Objectives: To evaluate whether double balloon pulmonary valvuloplasty (DBPV) with the Multi-Track system (MTS) may help to simplify the procedure., Background: DBPV is usually required for patients with pulmonary valve stenosis with large annulus. However, it needs two venous accesses and can be technically demanding., Methods: From 07/03, 20 consecutive patients (19 +/- 10 yrs) with typical pulmonary valve stenosis underwent DBPV using the MTS (G1). The results were compared with those achieved by conventional DBPV performed in a matched historical group of 28 patients (21 +/- 11 yrs; P = NS) (G2)., Results: MTS balloons were easily advanced through the skin and inflated across the valve. Similar results were observed in regards to residual gradients (12 +/- 11 vs 14 +/- 10 mm Hg; P = NS) and right ventricular to systemic pressures (0.35 +/- 0.22 vs 0.37 +/- 0.26; P = NS). Procedure and fluoroscopic times were significant lower in G1 (78 +/- 24 vs 126 +/- 28; 15 +/- 12 vs 25 +/- 8 min, respectively; both P < 0.001). There was no major complication. Median follow-up was 1.8 yr for G1 and 5 yr for G2 (P = 0.037). At the last visit, peak instantaneous gradient across the right ventricular outflow tract by echocardiography was a mean 22 +/- 10 mm Hg for G1 and 25 +/- 9 mm Hg for G2 (P = NS). No patient had severe pulmonary insufficiency or required reintervention., Conclusions: The use of the MTS helped to expedite the procedure providing satisfactory midterm clinical outcomes, similar to those observed with the conventional DBPV technique.

Surgery had been the traditional treatment for native coarctation of the aorta, one of the most common cardiovascular congenital malformations. As a less invasive mode of treatment, balloon angioplasty has emerged as an alternative to surgery but has not gained universal acceptance due to its rates of restenosis secondary to vessel recoil and concerns over aortic wall injury resulting in aneurysm formation. To overcome these problems, endovascular stents were introduced in the management of this condition. The early- and intermediate-term results are encouraging, with low rates of restenosis and complications. In this article, the authors review the current evidence on coarctation stenting and discuss future trends in this area.

We report our experience with the use of covered stents for the management of coarctation of the aorta. From December 2001 to March 2004, nine patients (seven males; median age, 31 years; mean weight, 65 +/- 15 kg) underwent implantation. Indications included critical or atretic native coarctation (n = 4), patients >50 years of age (n = 2), associated patent ductus arteriosus (n = 1) or adjacent aneurysm (n = 1), and the presence of a circumferential fracture within a previously implanted stent (n = 1). The covered balloon-expandable Cheatham-Platinum stent and the self-expandable stent graft Braile were employed. Adequate implantation was observed in all patients. Gradients were reduced from 54 +/- 14 to 3 +/- 8 mmHg and the coarctation site increased from 2.4 +/- 2.9 to 15.9 +/- 4.3 mm. The patent ductus arteriosus was immediately closed and the aneurysm excluded. Two patients >35 years with aneurysmal ascending aorta and metallic aortic prosthesis had aneurysm formation at follow-up, with one undergoing aneurysm exclusion using a Braile stent. Although covered stents are useful in the management of selected patients with coarctation, aneurysm formation may still occur in patients with markers of aortic wall weakness. Refinements in the deployment technique and/or the stent design are needed to eliminate this risk.

Objectives: This research was undertaken to assess the status of the coronary wall morphology late after the arterial switch operation (ASO) for transposition of the great arteries employing intravascular ultrasound (IVUS)., Background: Long-term patency of the reimplanted coronary arteries is a key issue after ASO. Follow-up studies have demonstrated coronary obstruction in up to 8% of patients that may be related to progressive fibrocellular intimal thickening., Methods: Twenty-two asymptomatic children were enrolled at a median age of 9.5 years (range 5 to 22 years); IVUS images were obtained in 20 children at cardiac catheterization 5.0 to 21.6 years after the operation (in two cases IVUS study was not feasible due to technical constraints). Quantitative analysis was performed in 37 coronary arteries involving segments with a mean length of 28.4 +/- 1.8 mm., Results: Thirty-three arteries (89%) displayed variable degrees of proximal eccentric intimal proliferation, with the maximal intimal thickening being 0.26 +/- 0.14 mm (range 0.06 to 0.71 mm) at the most thickened site. According to the Stanford classification, all children had coronary artery involvement with 50% having moderate-to-severe lesions (>0.3 mm). No risk factors for such abnormalities were encountered, including age, origin of the coronary arteries, hemodynamics, and follow-up duration after surgery., Conclusions: Intravascular ultrasound assessment late after the ASO revealed proximal eccentric intimal thickening in most of the studied vessels. This observation suggests the development of early atherosclerosis in the reimplanted coronary arteries, which may have a role in the genesis of late coronary events.

More information is needed to clarify whether stenting is superior to balloon angioplasty (BA) for unoperated coarctation of the aorta (CoA). From September 1997, 21 consecutive adolescents and adults (24 +/- 11 years) with discrete CoA underwent stenting (G1). The results were compared to those achieved by BA performed in historical group of 15 patients (18 +/- 10 years; P = 0.103; G2). After the procedure, systolic gradient reduction was higher (99% +/- 2% vs. 87% +/- 17%; P = 0.015), residual gradients lower (0.4 +/- 1.4 vs. 5.9 +/- 7.9 mm Hg; P = 0.019), gain at the CoA site higher (333% +/- 172% vs. 190% +/- 104%; P = 0.007), and CoA diameter larger (16.9 +/- 2.9 vs. 12.9 +/- 3.2 mm; P < 0.001) in G1. Aortic wall abnormalities were found in eight patients in G2 (53%) and in one in G1 (7%; P < 0.001). There was no major complication. Repeat catheterization (n = 33) and/or MRI (n = 2) was performed at a median follow-up of 1.0 year for G1 and 1.5 for G2 (P = 0.005). Gradient reduction persisted in both groups, although higher late gradients were seen in G2 (median of 0 mm Hg for G1 vs. 3 for G2; P = 0.014). CoA diameter showed no late loss in G1 and a late gain in G2 with a trend to being larger in G1 (16.7 +/- 2.9 vs. 14.6 +/- 3.9 mm; P = 0.075). Two patients required late stenting due to aneurysm formation or stent fracture in G1. Aortic wall abnormalities did not progress and one patient required redilation in G2. Blood pressure was similar in both groups at follow-up (126 +/- 12/81 +/- 11 for G1 vs. 120 +/- 15/80 +/- 10 mm Hg for G2; P = 0.149 and 0.975, respectively). Although satisfactory and similar clinical outcomes were observed with both techniques, stenting was a better means to relieve the stenosis and minimize the risk of developing immediate aortic wall abnormalities., (Copyright 2005 Wiley-Liss, Inc.)

Objectives: To determine the long-term outcomes and risk factors for, reintervention after balloon dilation of congenital aortic stenosis in children aged 6 months or older., Background: Although balloon dilation of congenital aortic stenosis has become a primary therapeutic strategy, few data are available regarding long-term outcomes., Methods: We carried out a retrospective review of 87 children who had undergone balloon dilation of the aortic valve at median age of 6.9 years., Results: The procedure was completed in 98% of the children, with an average reduction in the gradient across the valve of 64 +/- 28%, and without mortality. Of the children, 76 had been followed for a mean of 6.3 +/- 4.2 years. Reintervention on the aortic valve was required in 32 children, with 12 undergoing reintervention within 6 months, with 1 death. Another patient had died over the period of follow-up due to a non-cardiac event. Estimated freedom from reintervention was 86% at 1 year, 67% at 5 years, and 46% at 12 years. Parametric modeling of the hazard function showed a brief early phase of increased risk, superimposed on an ongoing constant risk. The only incremental risk factor for the early phase was a residual gradient immediately subsequent to the procedure greater than 30 mmHg. Incremental risk factors for the constant phase included the presence of symmetric valvar opening, and greater than moderate regurgitation immediately after dilation., Conclusion: Long-term survival was excellent, albeit that the need for further reintervention was high due to the palliative nature of the procedure.

Percutaneous closure of perimembranous ventricular septal defects (VSDs) has been feasible, safe, and effective with the new Amplatzer membranous septal occluder. We report further experience with this device with emphasis on morphological aspects of the VSDs and technical issues. Ten patients (median age and weight, 14 years and 34.5 kg, respectively) with volume-overloaded left ventricles underwent closure under general anesthesia and transesophageal guidance (TEE). The VSD diameter was 7.1 +/- 4.0 mm by angiography and 7.8 +/- 3.7 mm by TEE. Three patients had defects associated with aneurysm-like formations (two with multiple exit holes), four had defects shrouded by extensive tricuspid valve tissue, two had defects with little or no tricuspid valve involvement, and one had a right aortic cusp prolapse with trivial aortic regurgitation. Implantation was successful in all patients, although in two the initial device had to be changed for a larger one. Kinkings in the delivery sheath, inability to position the sheath near the left ventricular apex, and device prolapse through the VSD prompted modifications in the standard technique of implantation. Device orientation was excellent except in one case. Nine patients had complete occlusion within 1-3 months. Device-related aortic or tricuspid insufficiency, arrhythmias, and embolization were not observed. Two patients had slight gradients across the left ventricular outflow tract, normalizing after 3 months. The Amplatzer membranous septal occluder was suitable to close a wide range of perimembranous VSD sizes and morphologies with good short-term outcomes. Longer follow-up is required., (Copyright 2004 Wiley-Liss, Inc.)

The aim of this study was to evaluate the feasibility, safety and efficacy of transcatheter closure of secundum atrial septal defects (ASD) in patients with complex anatomy. From September 1997 to July 2003, a total of 40 patients (median age, 34 years; 65% female) with complex ASDs, defined as the presence of a large defect (stretched diameter >26 mm) associated with a deficient rim (n=23); multiple defects (n=8); a multi-fenestrated septum (n=5); and defects associated with an aneurysmal septum irrespective of their size (n=4) underwent closure. The Helex device was used in 4 patients and the Amplatzer in the remaining. Two devices were implanted in 2 patients each. Implantation was unsuccessful in 5 patients, with 4 having large defects associated with a deficient anterior rim and a floppy posterior septum. Occlusion was observed in 22 of 35 patients (63%) immediately after implantation and in 31 (89%) at a mean follow-up of 18+/-9 months. No major complications occurred. Right ventricular end-diastolic dimensions (indexed for body surface area) decreased from 135+/-25% before closure to 124+/-15% 24 hours after closure, and to 92+/-12% after 12 months. Two patients with 2 distant defects and 2 patients with large defects remained with shunts (<4 mm) at the latest visit. Transcatheter closure of complex secundum ASDs was feasible, safe and effective; however, large defects associated with a deficient anterior rim and a floppy posterior septum may not be suitable for this approach.

The isolated perimembranous ventricular septal defect is one of the most common congenital cardiac malformations. Although surgery has been performed and has a low associated risk, it still involves morbidity due to factors such as residual leaks, atrioventricular block, postpericardiotomy syndrome and arrhythmias. It has been speculated that percutaneous closure of these defects could minimize such complications. Recently, a device designed specifically for perimembranous ventricular septal defect closure, the Amplatzer membranous ventricular septal defect occluder, has been developed. Initial clinical experience with this device has been very encouraging with results showing a high rate of complete closure and a low incidence of complications at mid-term follow-up. In this article, the authors review their own and others' experience with this device.

We describe a case in which a discrete atretic segment of the right pulmonary artery (due to a Blalock-Taussig shunt) was reconstructed using a new radiofrequency system, balloon dilation, and stent implantation in an 18-month-old patient. The shunt was coil-occluded. The technique and applications of this novel approach are discussed., (Copyright 2003 Wiley-Liss, Inc.)

Objective: To report short and midtem follow-up results of balloon aortic valvuloplasty to treat congenital aortic stenosis., Methods: Seventy-five patients (median age: 8 years) underwent the procedure through the retrograde femoral or carotid route., Results: The procedure was completed in 74 patients (98.6%). The peak-to-peak systolic gradient dropped from 79.6 27.7 to 22.3 17.8 mmHg (P<0.001), the left ventricular systolic pressure dropped from 164 39.1 to 110 24.8 mmHg (P<0.001), and the left ventricular end diastolic pressure dropped from 13.3 5.5 to 8.5 8.3 mmHg (P< 0.01). Four patients (5.3%) died due to the procedure. Aortic regurgitation (AoR) appeared or worsened in 27/71 (38%) patients, and no immediate surgical intervention was required. A mean follow-up of 50 38 months was obtained in 37 patients. Restenosis and significant AoR were observed in 16.6% of the patients. The estimates for being restenosis-free and for having significant AoR in 90 months were 60% and 50%, respectively., Conclusion: Aortic valvuloplasty was considered the initial palliative method of choice in managing congenital aortic stenosis, with satisfactory short- and midterm results.

We report two cases in which adult patients with large and short patent ductus arteriosus with shallow/no aortic ampulla and associated pulmonary hypertension had the defects successfully closed percutaneously using Amplatzer devices designed for atrial septal defect occlusion. The defects were stretched with a sizing balloon for optimal ductal size determination and device selection. Surgical treatment for associated discrete membranous subaortic stenosis was deferred in one patient because of significant gradient reduction in the left ventricular outflow tract after ductal occlusion. The technique of this novel approach and its possible advantages are discussed.

A 7-month-old boy who was status post-bidirectional Glenn shunt implantation with residual ventricular flow to the pulmonary arteries (PA) presented with massive edema of the head and systemic desaturation soon after surgery. After test occlusion, an Amplatzer duct occluder was implanted at the PA banding site with subsequent decrease in the PA pressure, resolution of the edema and extubation.