The VALEO vascular stent for cardiovascular lesions in children.

AIMS: The VALEO vascular stent is a stainless steel, pre-mounted, open-cell stent. Redilation to large diameters and low profile are advantages in growing children. Clinical experience is scarce. This study aimed to analyse our experience with the use of the VALEO vascular stent in children. METHODS AND RESULTS: Between June 2010 and December 2012, 41 VALEO stents were inserted during 30 transcatheter (group 1) and three perioperative procedures (group 2). Data were retrospectively analysed. Median age at implantation was 3.8 years (four days - 23 years), and median weight was 13.3 kg (2.2-53 kg). Indications were: pulmonary artery (n=23), pulmonary vein (n=1) or subhepatic vein (n=1) stenosis, and ductus arteriosus stenting (n=8). Stent placement was achieved in all but one. Acute complications (n=11) included stent dislodgement in two patients and fracture in three patients, with vessel predilatation as a risk factor. Median "stent" follow-up reached 7.83 months (0.4-34.3 months) and included 26 recatheterisations (23 patients, median interval 6.2 months, range 0.2-33 months). Ten stents were redilated. Surgery in six patients (interval 1.9 to 10 months) showed patent endothelialised stents. No "late" type II or III stent fractures were seen. CONCLUSIONS: The VALEO stent is useful in children. Low radial force is counterbalanced by high flexibility, allowing implantation in distal and tortuous lesions. Early fractures occur. Longer-term follow-up is needed.

Melody ® transcatheter pulmonary valve implantation: results from a French registry.

BACKGROUND: Percutaneous implantation of pulmonary valves has recently been introduced into clinical practice. AIM: To analyse data of patients treated in France between April 2008 and December 2010. METHODS: Prospective, observational, multi-centric survey by means of a database registry of the Filiale de cardiologie pédiatrique et congénitale. RESULTS: Sixty-four patients were included, with a median (range) age of 21.4 (10.5-77.3) years. The majority (60.9%) of the patients were New York Heart Association (NYHA) class II. The most common congenital heart disease was tetralogy of Fallot with or without pulmonary atresia (50%). Indication for valve implantation was stenosis in 21.9%, regurgitation in 10.9% and association of stenosis and regurgitation in 67.2%. Implantation was successful in all patients. Pre-stenting was performed in 96.9% of cases. Median (range) procedure time was 92.5 (25-250) minutes. No significant regurgitation was recorded after the procedure, and the trans-pulmonary gradient was significantly reduced. Early minor complications occurred in five cases (7.8%). Three patients died during a median follow-up of 4.6 (0.2-5.2) years, two from infectious endocarditis and one from end-stage cardiac failure. Surgical reintervention was required in three patients. Follow-up with magnetic resonance imaging demonstrated significant improvements in right ventricular volumes and pulmonary regurgitation in mixed and regurgitant lesions. CONCLUSIONS: Transcatheter pulmonary valve implantation is highly feasible and mid-term follow-up demonstrates sustained improvement of right ventricular function. Late endocarditis is of concern, therefore longer follow-up in more patients is urgently needed to better assess long-term outcome. CLINICAL TRIAL REGISTRATION: NCT01250327.

Stenting right ventricular outflow in an infant with tetralogy of Fallot and well-developed pulmonary arteries.

A right ventricular outflow tract stent was implanted in a 1-month-old (3.5 kg) baby boy with tetralogy of Fallot and worsening cyanosis to relieve infundibular and pulmonary valve stenosis in the setting of well-developed pulmonary artery branches. This management allowed a symmetric and optimal growth of the pulmonary artery branches, with right and left pulmonary arteries measuring 7-mm (z-score, +1.3 SD) and 7.3-mm (z-score, +1.7 SD), respectively, 3 months after stent implantation. No signs of pulmonary overcirculation developed, and complete surgical repair was performed at the age of 6 months.

Management of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals: Focus on the strategy of rehabilitation of the native pulmonary arteries.

Pulmonary atresia with ventricular septal defect (VSD), hypoplastic native pulmonary arteries (PAs) and major aorto-pulmonary collateral arteries (MAPCAs) is a rare and complex congenital cardiac disease. In broad outline, two surgical approaches are available for patients with this condition. The first is characterized by one or several stages of complete unifocalization of the supplying MAPCAs, with or without incorporation of the native pulmonary arteries (PAs), connection of the right ventricle to the 'neo-Pas' and, if possible, concomitant or delayed closure of the VSD. The second strategy is based on rehabilitation of the native pulmonary arteries. The first step is a direct right ventricle to native PA connection, to promote the growth of native PAs. The establishment of antegrade flow also allows an easier approach for interventional catheterization, enabling dilatation or stenting of the stenosis and then closure of the communicant collaterals. When the development of the native PAs is satisfactory, the complete repair is performed. If it is necessary to suture a MAPCA to the PA ('unifocalization'), this is accomplished by connecting the collateral artery to an already developed native branch. Our team developed this multidisciplinary strategy with good results. Based on this experience as well as on the published literature, we describe this strategy of management of patients with pulmonary atresia, VSD, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals (MAPCAs).

Anaemia is a predictor of early death or cardiac transplantation in children with idiopathic dilated cardiomyopathy.

INTRODUCTION: The aim of our study was to establish the prevalence and the prognostic value of haematological abnormalities in children with cardiac failure. PATIENTS AND METHODS: A series of 218 consecutive children with a first diagnosis of idiopathic dilated cardiomyopathy were retrospectively examined. Haematological evaluation was performed at first diagnosis. Death or cardiac transplantation was the main outcome measure. RESULTS: The median age was 0.6 years, ranging from 1 day to 15.8 years and median follow-up was 2.65 years, ranging from 0 to 17.2 years. After a median interval of 0.2 years, ranging from 0 to 8.7 years, 56 patients died and 25 were transplanted. Event-free survival at 1 and 5 years was 68% (95% confidence interval, 63-75%) and 62% (95% confidence interval, 56-69%). Blood levels of haemoglobin less than 10 grams per decilitre, urea over 8 millimoles per litre, and C-reactive protein over 10 milligrams per litre were found in 24%, 20%, and 24% of patients, respectively. The log-rank test showed that haemoglobin (p = 0.000) and C-reactive protein (p = 0.021) were predictors of death or transplantation. In the multivariate Cox model, haemoglobin (hazard ratio = 0.735; confidence interval = 0.636-0.849; p = 0.000) and urea (hazard ratio = 1.083; confidence interval = 1:002-1:171; p = 0.045) were predictive of poor outcome. Cubic spline functions showed that the positive role of haemoglobin on survival was linear for values less than 12 grams per decilitre and null for values more than 12 grams per decilitre. Adaptive index models for risk stratification and Classification and Regression Tree analysis allowed to identify the cut-off values for haemoglobin (less than 10.2 grams per decilitre) and urea (more than 8.8 millimoles per litre), as well as to derive a predictor model. CONCLUSIONS: In children with idiopathic dilated cardiomyopathy, anaemia is the strongest independent prognostic factor of early death or transplantation.

Feasibility of transcatheter closure in unselected patients with secundum atrial septal defect, using Amplatzer devices and a modified sizing balloon technique.

OBJECTIVES: We aimed to assess (1) the role of surgical versus transcatheter closure techniques and (2) the impact of a modified implantation technique to optimize closure of secundum septal defects with the Amplatzer device. BACKGROUND: Despite several comparative studies, the respective roles of surgical and transcatheter closure are not clarified. Additionally, the impact of modified method of implantation on device closure remains unknown. METHODS: Fifty-seven unselected patients were referred for secundum atrial septal defect closure in 2009, at a median age and weight of 27.5 (0.8-88) years and 40.6 (5.6-97) kg, respectively. Transcatheter closure was attempted in 53 cases under transesophagal echocardiography guidance in children (n = 28) and intracardiac echocardiography guidance in adults. If standard closure failed, a sizing balloon catheter inflated in the left atrium was used as a support to secure the position of the device upon deployment. RESULTS: Fifty of the 57 cases (88%) were successfully closed with a median Amplatzer Septal Occluder size of 20 (10-40) mm, using the sizing balloon technique in eight (16%) cases. No major complication occurred. A trivial residual shunt remained in two patients (4%) whereas a mild mitral regurgitation appeared in one. By univariate analysis, a deficient superior-posterior rim and a large defect (>15 mm(2) /m(2) ) were associated with the use of the sizing balloon technique (P = 0.04 and 0.03, respectively). A deficient superior-posterior rim and pulmonary hypertension were associated with failure to close the defect (P = 0.02 and 0.03, respectively). CONCLUSION: The majority of secundum atrial septal defect is amenable to transcatheter closure, using a modified implantation technique in 16% of cases.

Long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, pulmonary artery hypoplasia, and major aortopulmonary collaterals.

OBJECTIVE: The study objective was to report the long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals. METHODS: Since 1991, 20 patients with profound pulmonary artery hypoplasia (mean Nakata index 26 ± 14 mm(2)/m(2)) have undergone a medico-surgical strategy of native pulmonary artery rehabilitation to achieve complete repair with satisfactory hemodynamics (right ventricle to aortic pressure ratio < 0.8). RESULTS: The first step, right ventricle to pulmonary artery connection, was performed at a median age of 4.1 (0.1-18.7) months with 1 operative death. After a median duration of 4.3 (1.1-26) months, the second step of interventional catheterizations followed (median, 2 (1-7)/patient), consisting of 36 pulmonary angioplasties, 11 stent implantations, and 20 collateral occlusions. Significant pulmonary artery growth was obtained in all cases with a Nakata index of 208 ± 85 mm(2)/m(2) before surgical correction (P < .001). The third step of surgical repair was performed at a median age of 1.9 (0.6-10.7) years, with right ventricular outflow reconstruction and ventricular septal defect closure fenestrated in 3 cases. During a mean follow-up of 8.2 ± 4.5 years, pulmonary artery rehabilitation was pursued in most patients, with 47 pulmonary angioplasties, 15 stent implantations, and 11 collateral occlusions. Three patients with a poor hemodynamic result died. At last visit, the 16 survivors are in New York Heart Association class I (n = 12) or II (n = 4) with satisfactory hemodynamics in 13 cases. CONCLUSIONS: Pulmonary artery rehabilitation allows complete repair in the majority of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals. However, long-term management often requires pursuit of the rehabilitation process.

Ventricular septal defect closure in a small children with the Amplatzer Duct Occluder II.

Transcatheter technique for muscular ventricular septal defect closure remains challenging, particularly in small patients. We report the successful use of the low profile Amplatzer Duct Occluder II for multiple ventricular septal defect closure in an infant with D-transposition of the great arteries, status postpulmonary artery banding. The multiple apical ventricular septal defects were successfully closed with two Amplatzer Duct Occluder II and one Amplatzer Muscular Ventricular Septal Defect Occluder during two interventional catheterizations. The patient subsequently underwent successful surgical repair.

Preoperative evaluation of candidates for total cavopulmonary connection: the role of echocardiography and cardiac catheterization.

AIM: To evaluate the information provided by echocardiography and cardiac catheterization in patients with partial cavopulmonary connection (PCPC) with or without additional pulmonary blood flow (PBF) in whom total cavopulmonary connection (TCPC) was planned. METHODS: We retrospectively evaluated the results provided by echocardiography and cardiac catheterization in 110 consecutive patients with PCPC (35 with isolated PCPC, 38 with associated antegrade PBF, 37 with associated retrograde PBF) in whom TCPC was planned. RESULTS: Eight patients had superior vena cava syndrome; all others suffered from cyanosis and fatigue. Pulmonary artery size could be determined in 54% of patients without additional PBF, in 47% of those with associated retrograde PBF and in 68% of those with associated PBF (p=0.20). Concomitant clinical signs of increased central venous pressure and echocardiographic signs of ventricular dysfunction and/or hypoplastic pulmonary arteries identified four patients in whom TCPC was not low-risk. PAP exceeding 16 mmHg was found in 9% of patients without and 16% of those with associated PBF (p=0.38). The Nakata index was higher in patients with associated antegrade PBF than in those without associated PBF (p=0.004) or in those with associated retrograde PBF (p=0.002). Of the 16 patients who needed concomitant interventional catheterization, 10 had associated retrograde PBF (p<0.05). In 11 patients (nine with and two without additional PBF), TCPC was contraindicated. CONCLUSION: Cardiac catheterization is still necessary before TCPC, mostly in patients with additional PBF, to identify those at risk and those needing interventional catheterization.