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Objectives This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. Background The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. Methods From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. Results The primary efficacy outcome was met for 87.1% patients after a mean follow-up of 4.6 ± 1.8 years, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8-95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular regurgitation was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01-2.00%). At 5 years, freedom from infective endocarditis was 98.4% (95% CI 89.1-99.8%). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. Conclusions This study documents the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance.

Summary Background Closure of patent foramen ovale is well-managed in adults, but is performed less frequently in children. Aim To analyse all patent foramen ovale closures performed in the past 20 years in French paediatric centres. Methods Retrospective study of patent foramen ovale closures in children without cardiopathy in nine centres between 2000 and 2019. Results Forty-one procedures were carried out in children (median age: 14.9 years). Thirty-one patent foramen ovales were closed after a transient ischaemic attack or stroke, six for a left-to-right shunt and four for other reasons. Transthoracic echocardiography was used for 72.2% of the diagnoses and transoesophageal echocardiography for 27.8%. A substantial degree of shunting was found in 42.9% of patients and an atrial septal aneurysm in 56.2%. General anaesthesia with transoesophageal echocardiography guidance was performed in 68.3% of the procedures; local anaesthesia and transthoracic echocardiography or intracardiac echocardiography was performed in 31.7%. The success rate was 100%. The median fluoroscopy time was 4.14 minutes: 3.55 minutes with transoesophageal echocardiography; and 4.38 minutes with transthoracic echocardiography (P = 0.67). There was only one periprocedural complication (2.4%). Postoperatively, 80,5% of patients were treated with aspirin and 12,2% with an anticoagulant. The rate of complete occlusion was 56.8% immediately after the procedure, 68.6% at 1 year and 92.3% at the last follow-up. There were no delayed complications or cases of recurrent stroke during follow-up (median follow-up: 568 days). Conclusion Closure of patent foramen ovale in children appears to be safe and effective, as we noted a low rate of immediate complications, no delayed complications and no stroke recurrence in this indication.

We report a case of antegrade transcatheter occlusion of a paravalvular leak (PVL) after bioprosthetic pulmonary valve replacement in a 31-year-old patient with tetralogy of Fallot. The leak resulted in severe pulmonary regurgitation with right ventricle volume overload. This case is the first-ever report of a successful percutaneous closure of PVL using the Occlutech paravalvular leak device.

Minimally invasive cardiac surgery is mainly dedicated to acquired left-sided valve diseases. Ministernotomy is widely used for aortic valve repair or replacement, whereas pulmonary valve repair via this approach has been reported only recently. This article aims to describe the use of ministernotomy for pulmonary valve replacement in adult congenital patients.

Background Percutaneous treatment of native coarctation (coA), gold standard in adult patients, is still debated in children. We aim to report the French multicentre experience. Methods All patients aged 1 to 18 yrs with native coA, treated percutaneously between 01/2000 and 12/2018, were included in this multcentric (9 centres) retrospective survey. Results One hundred and thirty three children (34 female) were included. Indication for treatment was hypertension in 109, exercise hypertension in 8 and other reasons in 16 patients. Mean age at intervention was 11.5 ± 3.9 years, mean weight 41 ± 18 kg. Procedure consisted of isolated balloon dilatation in 5 (mean age 2,63 yrs, mean weight 12 kg), balloon dilatation followed by stent implantation in 8 (mean age 11 yrs, mean weight 37 kg), and immediate stent implantation in 120 (mean age 12 yrs, mean weight 43 kg). Stents used (n = 124/128) were covered in 43, non-covered in 77, including 38 open cell stents. Invasive gradient across coA dropped from 28 mmHg (range 5 to 64) at baseline (n = 124) to 3 mmHg (range -5 to 20) after intervention (n = 105). Three associated lesions were treated: 1 PDA closure, 1 aortic valvuloplasty and 1 left SVC occlusion. Serious vascular complications occurred in 8 patients (6%) (3 pseudo-aneurysms, 2 dissections, 2 stenosis and 1 occlusion) requiring surgery in 2. Reintervention was performed in 22%: 2 patients had surgery, 28 were treated percutaneously (14 balloon dilatations, 14 stent implantations). Mean follow-up reached 4.9 ± 4.7 years. At latest follow-up, 25% remained hypertensive with 15 patients (11%) on anti-hypertensive medication. MRI, CTscan or fluoroscopy imaging, are available in 52 patients during follow-up (39%). Rate of aneurysm formation, stent fracture and risks factors for reintervention or residual hypertension will be presented. Conclusions Percutaneous treatment of native coarctation was safe and efficient in our french population. Close follow-up is required for longer-term results.

Objectives This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. Background The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. Methods From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. Results The primary efficacy outcome was met for 87,1% patients after a mean follow-up of 55 ± 21 months, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8–95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular insufficiency was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01–2.00%). At 5 years freedom from infective endocarditis was 98.4% (95% CI 89.1–99.8%) ( Fig. 1 ). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. Conclusions This study confirms the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance.

Objectives This study sought to assess procedural characteristics, early clinical outcome, and long-term complications after transcatheter closure of atrial septal defect (ASD) in children. Background Transcatheter closure has become the preferred strategy in most cases of isolated secundum ASD. However, reported experience in the pediatric population is limited. Methods A 1998 to 2016 retrospective multicenter study was performed in 9 French tertiary institutions. All children who had an attempt of percutaneous ASD closure with an Amplatzer Septal Occluder were included. Results In 1,326 children (39% males; median age, 9 years [0.7 to 18]; weight, 29 kg [3.6 to 92]), transcatheter ASD closure was performed. Median ASD size was 15 mm (3 to 41); 254 (19.1%) patients had a large ASD (≥20 mm/m2). Procedural success rate was 95.3% (95% confidence interval: 93.9% to 96.3%). No death was observed but periprocedural complications occurred in 24 patients (1.8%). After a median follow-up of 3.5 years (range 6 months to 18 years; 173 patients [13%] followed >10 years), delayed major complications were minimal (n = 12; 1.04%) including no death and/or cardiac erosion. Periprocedural and delayed complications rates were significantly higher in children ≤15 kg (5.2% vs. 1.5%; p = 0.007 and 3.1% vs. 0.7%; p Conclusions Transcatheter ASD closure using Amplatzer Septal Occluder is safe in children with a minimal rate of periprocedural complications and a favorable long-term outcome, especially with no death or cardiac erosion despite a substantial proportion of large defects. Children ≤15 kg and those with large ASDs had a greater risk of complications.

Summary Background Transcatheter perimembranous ventricular septal defect (pmVSD) closure remains challenging and is seldom used in France given the risk of atrioventricular block (AVB). pmVSD closure with the Nit-Occlud Le VSD coil was recently introduced in France as an alternative to occluder devices. Aims To study the safety and feasibility of pmVSD closure with the Nit-Occlud Le VSD coil. Methods All consecutives cases of pmVSD closure with the Nit-Occlud Le VSD coil in 20 tertiary French centres were included between January 2015 and December 2018. Results Among 46 procedures in five centres, indications for pmVSD closure were left ventricle overload (76.1%), exertional dyspnoea (17.4%), history of infective endocarditis (4.3%) and mild pulmonary hypertension (2.2%). The median (interquartile [IQR]) age of the patients was 13.9 (5.7–31.8) years. Aneurismal tissue was identified in 91.3% of patients. VSD median (IQR) size was 8 (7–10) mm on the left ventricle side and 5 (4–6) mm on the right ventricle side. Implantation was successful in 40 patients (87.0%; 95% confidence interval [CI] 73.7–95.1%). Severe complications occurred in six patients (13.0%, 95% CI 4.9–26.3%), mainly severe haemolysis (8.7%, 95% CI 2.4–20.8%). One aortic valve lesion required surgical aortic valvuloplasty. Occurrence of severe complications was significantly related to the presence of haemolysis (P = 0.001), residual shunt (P = 0.007) and multi-exit VSD (P = 0.005). Residual shunt was observed in 40% of cases with the implanted device shortly after closure and 15% after a median follow-up of 27 months. No immediate or delayed device embolization or complete AVB was recorded. Conclusion pmVSD closure with the Nit-Occlud Le VSD Coil is feasible in older children and adults. However, residual shunting (leading to haemolysis) is a dreaded complication that should not be tolerated. pmVSD closure with the Nit-Occlud Le VSD as a therapeutic strategy remains controversial and is limited to selected patients.

Decision making for univentricular or anatomical repair remains challenging in children with borderline left ventricle. Unpredictable outcomes have led many caregivers to pursue a single-ventricle strategy. We describe 2 cases of patients with borderline left ventricle initially palliated with univentricular strategy followed by very late conversion to anatomical repair 4 and 16 years later. Anatomical conversion should be considered for these patients even many years later. During the first palliation stages, hemodynamic conditions preserving the potential for growth of the left ventricle should be maintained.

BackgroundThe Occlutech Figulla ASD device series (OFSO) shows an improved device design for interventional ASD closure, larger follow-up series are missing. MethodsWe retrospectively reviewed the feasibility, safety, implantation properties, results, and follow-up of ASD closure using Occlutech devices over a 5 year period by establishing a multi-institutional collaborative result registry with 16 contributing centers from 11 countries (IRFACODE). ResultsIn 1315 patients of all age groups (female 66.9%), successful (98%) ASD closure was performed (mean age 28.9 years, weight 52 kg, height 148.6 cm). Of the defects, 47.9% showed no or only a deficient aortic rim;in 11.9%, there was more than one defect;a septum aneurysm was present in 21.5%;and the mean implanted device size was 20.5 mm. Immediate closure was achieved in 78.6%, at discharge in 83.1%, and 96.4% and 97.3% at 6 and 12 months follow-up, respectively. During a mean follow-up of 2.7 years (in total 3597 patient years), significant complications were minimal (total=8

We report the case of a 14-year-old boy with severe protein-losing enteropathy after Fontan surgery that led to lymphangiectasia, which caused gastrointestinal haemorrhage and required invasive treatment to stop the bleeding. Through this case and a review of the literature on protein-losing enteropathy after Fontan surgery, we highlight a rare and serious presentation of the disease and the difficulties of diagnosis and management.

Background The Coaguchek INRange® is a self-testing meter to measure the INR, that is reimbursed in France since August 2017 for patients with mechanical valve. Methods We evaluated the use of this anticoagulation method in a French population of GUCH (Grown-Up patients with Congenital Heart Disease) with mechanical valve. Results Since September 2018, 52 patients of 37 ± 11 years old were asked to attend a training course of 2 hours on anticoagulation and Coaguchek INRange® use, provided by specialized nurses, before getting home with the device. Patients had to attend a 3 months’ medical re-evaluation appointment. 29 (56%) patients had an aortic mechanical valve, 12 (23%) a mitral one, 8 (15%) a double aortic and mitral one, 2 (4%) a double aortic and pulmonary one and 1 (2%) a tricuspid one. 28 patients (54%) had a mechanical valve for ≥ 10 years (group 1). In group 1, patients were older (41 ± 10 years old vs. 34 ± 10, P = 0.01). Fluindione was the preferred oral anticoagulant (30 patients, 57%), and was more frequent in group 1 (18 patients, 64%). In group 1, 61% of patients usually managed themselves dosage adjustment whereas in the other group, 52% of patients referred to their doctor. Thirty-one patients (62%) had a higher target of INR than recommended in last European guidelines. Concerning follow-up, 26 patients (50%) did not attend the 3 months appointment and had to be rescheduled or contacted by phone. 8 patients (15%) did not use the device at 3 months: 5 for variation > 15% compared to laboratory plasma technique and 3 for not trusting the new device. INR at 3 months was obtained in only 40 (77%) patients, and was in the attended target in 62% of cases. Conclusion GUCH patients with mechanical valves, especially when present for ≥ 10 years, seem to validate Coaguchek, even though evaluation remains hard since a great proportion of them did not attend follow-up appointment, stick to their old INR target and did not use the device as often as recommended.

Objectives The short-term efficacy and safety of transcatheter closure (TCC) for the management of coronary artery fistulas (CAFs) was investigated in pediatric patients. Background CAFS are rare with potentially severe complications and their management is still a matter of debate. Percutaneous closure appears to be the treatment of choice in anatomically suitable vessels but limited data are available in the pediatric population. Methods This retrospective, observational, multicenter, national study included patients under 16 years of age who underwent TCC of a congenital CAF. Patients with additional congenital heart defect were excluded. Results 61 patients (36 girls, 25 boys) with a median age of 0.6 year [0–15.4] at diagnosis and 3.9 years [0–16] at procedure were included. The CAF was large in 48 patients (79%); it was distal in 23 (38%) and proximal in 22 (36%). Most patients (77%) were asymptomatic at diagnosis. Clinical signs of congestive heart failure were present in seven patients (11%). Perioperative complications included three cases of ST elevation myocardial infarction (exclusively during attempted closure of a distal CAF), three devices migrations, and one case of leg ischemia. One patient died after surgical closure of a large distal CAF that could not be closed by TCC. Follow-up data were collected for 43 patients (70%) for a median of 91 days [0–4,824]. The Kaplan-Meyer estimate for complete occlusion at 2 years was 73 ± 7.6%. Conclusion TCC in the pediatric population appears to be effective and associated with few complications. © 2015 Wiley Periodicals, Inc.

Objective Off-pump valve replacement using self-expandable stents is an emerging technique for pulmonary valve disease. However, significant limitations are the lack of easily available valve substitute to be inserted within the stent and, in the setting of repaired tetralogy of Fallot, the existence of huge pulmonary trunk. We report the first experimental results of a transventricular approach using a decellularized porcine xenograft mounted in a self-expandable stent. Methods Pulmonary valve replacement was realized in 15 lambs by direct access of the infundibulum through a left thoracotomy, combined with pulmonary artery banding. Animals were followed by transthoracic echocardiography and, after control hemodynamic study, were electively killed either at day 7, month 1, or month 4 after implantation. Results Implantation succeeded in all lambs. Two animals died after implantation (1 pneumothorax and 1 endocarditis). Doppler echocardiographic follow-up did not show any significant transvalvular gradient and showed only mild pulmonary regurgitation. The hemodynamic control before termination revealed a systolic pulmonary valve gradient of 18.5 ± 12.4 mm Hg at 1 week (n = 4), 13.5 ± 10.6 mm Hg at 1 month (n = 4), and 4.3 ± 4.9 mm Hg at 4 months (n = 5). Gross examination demonstrated the presence of connective tissue between the valved stent and pulmonary wall, which increased with time. Conclusion Fifteen lambs underwent successful deployment of a self-expandable valved stent in the pulmonary position using a transventricular approach. This technique combined with pulmonary artery banding could be a therapeutic option for pulmonary insufficiency after repair of tetralogy of Fallot with a transannular patch.

Purpose To review the use of thoracic endovascular aortic repair (TEVAR) for late pseudoaneurysm formation after surgical repair of aortic coarctation. Methods From May 2001 to May 2005, 8 patients (5 men; mean age 47.6 years, range 18-73) with a history of aortic coarctation repairs 17 to 40 years prior were referred to our institution for an anastomotic thoracic pseudoaneurysm. TEVAR was performed successfully in 7 patients; 1 died of suspected aneurysm rupture before the scheduled procedure. A carotid-subclavian bypass was performed in 3 patients. Results All the procedures were immediately successful. No type I endoleaks were seen on the final control angiogram, but 2 of the patients with carotid-subclavian bypasses required additional left subclavian artery embolization due to type II endoleak. One of these patients died before embolotherapy on the 5th postoperative day from presumed aneurysm rupture (14% 30-day mortality rate). Over a follow-up period ranging from 15 to 72 months (mean 37), all the false aneurysms have remained thrombosed and the mean diameter has decreased from 44 to 23 mm. No endograft-related complications have occurred, and no further interventions have so far been necessary. Conclusion TEVAR is a feasible alternative treatment for patients who have already undergone surgical repair of aortic coarctation. Technical issues regarding the endovascular strategy should be discussed with a multidisciplinary team to define the correct interventional plan.

Introduction Infantile haemangioma (IH) is a very common vascular tumour that affects up to 10% of newborns. Since 2008, oral propranolol is used to treat complicated IH, like haemangioma that obstruct vital structures or ulcerated haemangioma. Objective The aim of this study was to investigate, by a retrospective review, the therapeutic results and effects of propranolol on cardiovascular and biological parameters in infants treated for complicated infantile haemangioma and to assess its safety. Results All paediatric patients with complicated IH who started systemic propranolol from February 2009 to December 2014 were included. 218 patients (155 girls and 63 boys) were treated by propranolol. The mean weight was 6780g (2115g to 20000g). Median age at beginning of treatment was 4.7 months (10 days to 6 years). The most frequent localisation of IH was facial (63 patients), palpebral (52 patients), perineal (20 patients), labial (14 patients), airway obstruction (8 patients) and 1 PHACE syndrome. Median length of therapy was 7.5 months for facial IH, 6 months for palpebral, 5.6 months for perineal IH and 7 months for subglottic localisation. Adverse events were observed: hypoglycemia (n=11 patients aged less 6 months), arterial hypotension (n=103 patients, especially at the second and third dose with dose titration), bradycardia (n=120). Transthoracic echocardiography was realised in 158 patients: 19 pathologies were found (8 PDA, 4 ASD, 4 mitral regurgitations, 2 VSD and 1 coarctation). Other adverse events occurred in 49 patients (wheezing, acrocyanosis, diarrhoea, sleep disturbance) leading to modification in management. Complete response was observed for all but 11 (partial or no response). Conclusion In appropriated patients, propranolol therapy is effective for severe or complicated IH. We must be aware of frequent adverse events under beta-blocker in these patients.

Purpose Transcatheter closure of the arterial duct using the Occlutech PDA occluder. Methods and results From March 2013 to April 2015, 16 patients underwent percutaneous arterial duct closure with the new Occlutech PDA occluder. There were 10 females and 6 males. All patients had significant L-to-R shunt with enlarged left ventricle. At implantation, the mean age was 87 months (median 35 months) and mean weight was 16±11kg (7.2 to 54kg). The procedure was realized under local anaesthesia. Size of the duct was 2.96±0.94mm (range 1.7 to 5.5mm) on angiography. According to Krichenko classification of PDA, ducts were: type A (n=14), Type B (n=1) and type E (n=1). The systolic pulmonary artery pressure was 43±17mmHg (range 24 to 91mmHg). Implantation succeeded in all. Closure was performed by the standard 4/6mm occluder (n=8), the standard 6/8mm occluder (n=3), the standard 5/7mm occluder (n=3), the standard 3.5/ 5mm (n=1) and the standard 8/10mm occluder (n=1) using a 6 or 7 F delivery sheath. After implantation, trivial shunt was noticed on angiography in 12 patients, 4 had no shunt. The fluoroscopic time was 6.5±8.7 minutes and radiation dose 6.0±4 Gycm2. After closure, femoral thrombosis was noticed in 2 patients but resolved completely under heparin therapy. On control Doppler echocardiography (1 to 18 months), duct was closed in all patients but one with only one month follow-up. No obstruction of the left pulmonary artery or isthmic stenosis was noticed. Conclusions Percutaneous closure of PDA with the new Occlutech PDA occluder is safe and effective. The device is easy to handle and there is no learning curve for operators using the classic Amplatzer duct occluder. Further studies with longer follow-up are necessary to confirm these good results.

Aim: To report results of transcatheter occlusion of moderate to large patent arterial ducts, having a minimum diameter above 2.5 mm, with the Amplatzer duct occluder, and to compare these with results achieved using Rashkind or Sideris devices and Cook detachable coils. Design and setting: Retrospective study conducted on intention-to-treat data from a tertiary referral centre. Patients: Since 1989, 116 consecutive patients, 77 females and 39 males, underwent percutaneous closure with several devices. We used the Rashkind double umbrella in 23 patients, the Sideris buttoned device in 39 patients, coils in 17 patients, and the Amplatzer duct occluder in 37 patients. The median age of the patients was 37 months, and the median weight 13 kg. The mean minimum diameter of the duct was 3.8 ± 1.22 mm, with a median of 3.5 mm, and range from 2.5 to 10 mm. Results: Implantation succeeded in all but 9 of the children (92%). The time of fluoroscopy was shorter, and full occlusion was better as judged on angiography, in patients closed using the Amplatzer device, despite closure of larger ducts, than in patients closed using other devices (p < 0.0001, p = 0.0003, and p = 0.0015 for the Rashkind, Sideris, and coils, respectively). Complications included embolisation in 2 patients, and haemolysis in 3 patients. In 12 patients, a second device was inserted because of residual shunting noted during follow-up. Complete occlusion was achieved earlier after implantation (p = 0.0002), and the rate of complete occlusion was better in patients receiving an Amplatzer device (97%, p = 0.024) than in patients undergoing closure with other devices. Conclusion: Transcatheter closure of moderate to large patent arterial ducts using the Amplatzer duct occluder is an effective and safe procedure, providing better results than those achieved using other occluders.

The interventional management of D-Transposition of great arteries DTGA is more required with the advances in NICU and pediatric cardiology. Objective To describe our experience in balloon atrial septestomy (BAS) in D-TGA patients weighing Methods we reported retrospectively all BAS in VLBW infants from January 2002 to June 2013. We collected information about the vital parameters before and after BAS. We described the atrial septestomy. Results We have 3 patients with a mean weight of 1333 g. D-TGA was diagnosed at a mean age of 3 days. Four atrial septestomy attempts were done at a mean age of 10.3 days. One infant had two attempts with balloon dilatation in the first, then BAS 24 h later. Procedures were done in catheterization laboratory in three cases and in NICU in one case. Patients were ventilated, sedated and transported by the NICU team. Venous access was obtained by the femoral vein puncture in two cases, femoral vein cutdown in one case and by the umbilical vein in one case. BAS were performed successfully in two cases, one using 4Fr. Swan Ganz catheter, and the other with 5 Fr. Z-Med septestomy catheter. BAS failed in two procedures, one because of atrial wall perforation, and the second because of restrictive foramen ovale which was dilated by a coronary balloon. Complications occurred in 3 cases, one minor (balloon rupture), and two major; atrial perforation in one patient, and severe bradycardia in one patient. BAS was effective with sustained clinical improvement. BAS failure in two patients was followed by heart failure in one patient and the death in the other. Conclusion BAS is mandatory in VLBW infants with D-TGA and restrictive atrial communication. It should be performed quickly if possible to use the valuable umbilical access, and to avoid the clinical compromise. A holeend BAS catheter is required for successful procedure. FO balloon dilatation constitutes another life-saving option.

Summary Background Single-centre experience in transcatheter closure of atrial septal defect (ASD) using the Figulla ® ASD Occluder (FSO; Occlutech GmbH, Jena, Germany) and the Amplatzer ® Septal Occluder (ASO; Saint-Jude Medical, Zaventem, Belgium) has been reported. Aim To perform a retrospective comparison of the two occluders. Methods From September 2009 to December 2012, 131 consecutive patients underwent percutaneous ASD occlusion: One hundred with the ASO device; 31 with the FSO device. Results There were no significant differences between the two groups regarding patient characteristics, stretched diameter, age and device size. In the ASO group, implantation succeeded in all but two patients because of deficient rim. Another patient had device embolization in the aorta retrieved percutaneously. During follow-up, 86 patients had no residual shunt and nine patients had a residual shunt (small in seven; moderate in two). Two other patients had persistent interatrial small shunt caused by an adjacent ASD close to the device. In the FSO group, implantation succeeded in all but two patients: one because of deficient posterior rim; and one because of complete atrioventricular block that resolved after device extraction. During follow-up, no shunt was observed in all but one patient. At late follow-up (up to 36 months), full occlusion was observed in 88 (88.0%) patients in the ASO group and 28 (90.3%) patients in the FSO group (with no significant difference between groups). Conclusion Transcatheter closure of ASD with the FSO is feasible and safe. FSO results compare favorably with ASO results. However, additional long-term studies that include more patients are mandatory.

Background Hypoxaemia resulting from a right-to-left shunt occurs in patients with atrial septal defects and high pulmonary vascular resistance, but it is uncommon without pulmonary hypertension. Methods We report on 11 consecutive patients (age: 59–78 years) in whom a patent foramen ovale or a small atrial septal defect with normal right-sided pressures led to significant cyanosis with clinical symptoms. Six of them had associated platypnoea and orthodeoxia. The diagnosis was confirmed by contrast transoesophageal echocardiography showing an atrial right-to-left shunt. Results All but one were successfully treated by percutaneous closure of the inter-atrial defect. In one patient, delivery of the occluder failed due to kinking of the introducing sheath. Four complications were observed following the procedure: two supraventricular arrhythmias and a cerebrovascular accident, all resolved without sequelae; one patient died from a septic shock unrelated to the procedure. During follow-up (up to 30 months), no patient experienced any episode of desaturation due to inter-atrial shunting. Conclusion Cyanosis without pulmonary arterial hypertension in the adult should prompt the performance of contrast transoesophageal echocardiography to identify a possible atrial right-to-left shunt. Percutaneous closure of the defect allows efficient and rapid correction of the hypoxaemia and avoids the need for surgical closure.

Traumatic induced VSD is a rare but serious disease because of the acute hemodynamic changes. We reported one center experience in the interventional catheterization closure of traumatic induced VSD during the last ten years. We had 3 patients with four VSD. Mean age was 60 (40-71) years. VSD was muscular secondary to external trauma in one patient, and to transapical transcatheter replacement of both of the aortic and mitral valves in the second. Last patient had membranous and muscular VSD post Ross-Konno intervention. All patients had acute congestive heart failure. All procedures were performed under general anesthesia and transesophageal echocardiography control. Arteriovenous loop was always used to introduce the delivery sheath to the left ventricle. VSDs diameter was evaluated by echography and ranged from 9 to 13mm. Device diameter was chosen 1 to 2mm over the echo-graphic measures. Multiple devices were used (Amplatzer® septal occluder, Amplatzer® muscular VSD occluder, Occlutech® Figulla septal occluder). Mean procedures time was 113 (100-145) min, and mean irradiation dose was 160 (103-203) Gycm2. Non significant residual shunt was observed in all patients, but the heart failure was resolved in all. Complications were registered in three procedures: transient hemolytic anemia, severe bradycardia, tricuspid cordage rupture and groin hematoma. Conclusion Traumatic VSD closure is required because of the acute hemo-dynamic changes. Trancatheter closure is effective. Complications are frequents because of the critical clinical status.

Background Balloon dilatation of the pulmonary valve remains controversial as a palliative procedure in tetralogy of Fallot. Aims To determine the clinical outcome, growth of the pulmonary vascular tree and findings at surgery of balloon dilatation of the pulmonary valve performed as a palliation in infants with tetralogy of Fallot. Methods and Results Thirty-three severely cyanosed infants (mean age of 3 months for the whole population, including seven neonates) underwent this procedure from June 1990 to January 1997. After balloon dilatation, systemic oxygen saturation increased from a mean value of 76±9% to 88±7% (P

Construction of a modified Blalock-Taussig shunt using polytetrafluroethylene is frequently used for palliation in infants with complex cyanotic heart disease. This study was performed to assess angiographically the late patency rate of such prosthetic shunts. A modified Blalock-Taussig shunt had been constructed in 82 children and, of these, 63 (77%) patients had post-operative angiography and were included in this study. There were 29 males and 34 females, ranging in age from 1 day to 164 months. A second shunt was needed in 7 patients. Thus, 70 shunts were studied. Of the shunts, 16 were 4 mm in diameter, 28 shunts 5 mm in diameter, and 26 shunts 6 mm in diameter. Postoperative angiography was performed on 1 day to 132 months (median 39 months) after construction of the shunts. Acute post operative occlusion occurred in 2(3%) patients, who had 5 and 6 mm shunts, respectively. At the latest follow-up (median 39 ± 26 months), 6(9%) late occlusions were noticed. Overall patency was 89% for all the shunts, with 94% of the 4 mm shunts, 85% of the 5 mm and 88% of the 6 mm shunts being patent. Regression analysis showed a progressive reduction in diameter of the graft with time. This narrowing of the graft may have been related to the formation of a ‘neointima’ due to fibrous hyperplasia.

Many devices are available for atrial septal defect (ASD) usually in adults and children. We report here one center experience in transcatheter closure of ASD in infancy, using the Amplatzer septal occluder. From January 2003 to March 2010, 10 males and 5 female, mean age 10.9±6.4 months, with weight 6.7±2.5 kg underwent transcatheter closure of atrial septal defect. The patients had significant left-to-right shunting (n=12), right-to-left shunting with hypoxemia (n=3). For the left-to-right shunting, the ASD was closed for pulmonary hypertension (n=10), for recurrent chest infection and bronchiolitis (n=5). The ASD was associated with other congenital heart defect (n=5), prematurity and bronchopulmonary dysplasia (n=6), foetopathy (n=1) and encephalopathy (n=1). The ASD was evaluated by transthoracic echocardiography (TEE) before the procedure. Percutaneous closure was realized with TEE for all patients. They received intravenous heparin. For 4 patients implantation was performed after a balloon test occlusion (mean 11±3.4 mm diameter). The ASO device size was 10.4±3.9 mm. The fluoroscopy time was 7.9±3.9 minutes and the time of the procedure 55.6±21.8 minutes. No per procedural complications were observed for all but for two (one anomalous device deployment and one insufficient rim). These two patients were subsequently surgically repaired. Patients left the hospital with a low dose of aspirin for 6 months. During follow-up, four patients had a minimal residual shunt on TEE performed up to one year after the implantation. No later cardiac complication was observed. To conclude, transcatheter closure of atrial septal defect in infant with the ASO is feasible and a possible alternative to surgical closure. In addition, ASD occlusion improves the respiratory status and reduces the level of pulmonary hypertension. Additional long-term results and a large study are both necessary to establish the future of this population

International audience; AIMS: The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined. METHODS AND RESULTS: We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 119 (84.4%) and complete in 100 (70.9%) patients. There was progression to complete AV block in 29/41 (70.7%) patients with incomplete AV block over 2.8 ± 3.4 years (1-155 months), but all patients with incomplete AV block may not have been included in the study. Narrow QRS complex was present in 18 of 26 patients (69.2%) with congenital, and 106 of 115 (92.2%) with childhood AV block. Pacemakers were implanted in 112 children (79.4%), during the first year of life in 18 (16.1%) and before 10 years of age in 90 (80.4%). The mean interval between diagnosis of AV block and pacemaker implants was 2.6 ± 3.9 years (0-300 months). The pacing indication was prophylactic in 70 children (62.5%). During a mean follow-up of 11.6 ± 6.7 years (1-32 years), no patient died or developed dilated cardiomyopathy (DCM). The long-term follow-up was uncomplicated in 127 children (90.1%). CONCLUSION: In this large multicentre study, the long-term outcome of congenital or childhood non-immune, isolated AV block was favourable, regardless of the patient's age at the time of diagnosis. No patient died or developed DCM, and pacemaker-related complications were few.

SummaryIn the past 10 years, stent implantation has become a real alternative to surgery in the management of both native and recurrent coarctation of the aorta in adolescents and adults. The purpose of this report is to provide a detailed review of stent implantation techniques, including pre-procedure imaging, technical aspects and results. The success rate is usually high (around 90%), and the procedure results in an increase in the diameter of the coarcted segment, a decrease in the transisthmic systolic gradient and a better control of systemic hypertension. The most serious complication, rupture of the aorta, can be fatal, but is rare (

Hypoxemia related to interatrial right-to-left shunt may have a significant impact on exercise tolerance. We report here one centre experience in transcatheter closure of interatrial RL shunt. From August 1995 to 2009, 63 consecutive patients (37 females and 26 males, mean age 54 ± 24 years), underwent transcatheter closure of interatrial RL shunt because of cyanosis and shortness of breath at exercise. Associated malformations were modified Fontan (n = 4), Ebstein anomaly (n = 1), RV hypoplasia (n = 1), pectus excavatum (n = 2), aortic aneurysm (n = 1). Ten pts had a past history of stroke. A real platypnea-orthodeoxia syndrome was noticed in only 17 pts. The vast majority of patients had cardiac catheterization under local anaesthesia with a sole fluoroscopic control. None of them had pulmonary artery hypertension. Transcatheter closure was performed with a Sideris device (n = 10), a PFO Amplatzer occluder (n = 40), an ASD Amplatzer occluder (n = 8), a Cardioseal device (n = 3), a VSD Amplatzer occluder (n = 2). Device implantation succeeded in all but two (1 Sideris and 1 Cardioseal device). The fluoroscopic time was 12 ± 8 minutes. All patients had better clinical tolerance after closure with an oxygen saturation > 92%. All underwent serial echocardiographic follow-up with bubble study. One month after implantation, no shunt was noticed in all but 6 pts (tiny RL shunt). In 5 of these remaining pts, the 6-month control did not see any residual shunting. Transcatheter closure of interatrial RL shunt responsible of cyanosis is an effective and a safe method. Many devices using a double disk system can be employed and provide excellent results. The classic platypnea-orthodeoxia syndrome is observed in

PurposeRight ventricular outflow tract (RVOT) reconstruction in infancy or childhood leads to lifetime iterative surgery for pulmonary stenosis or regurgitation. Transcatheter pulmonary valve implantation (TPVI) was developed with the aim to reduce the number of reoperations and operative-related morbidity.Methods and ResultsFrom 12/2008 to 03/2010, 21 patients were referred to Marie-Lannelongue Hospital for TPVI. Mean age was 24 yrs (11.5–55 yrs), mean weight was 60kg (20–85kg). Most of them had a Tetralogy of Fallot variant (n=12) and 70% had 3 or more previous surgery. RVOT stenosis was predominant in 90% of patients. RVOT reconstruction techniques were: valved conduit (n=12), non-valved conduit (n=1), homograft (n=6), RVOT patch (n=2). RVOT balloon inflation test was performed only if cardiac CT scan showed coronary arteries located next to the RVOT. Two patients were excluded, 1 because an aorto-pulmonary fistula after Ross operation, and 1 with previous arterial switch because of compression of the reimplanted left coronary artery at RVOT balloon inflation test. Initial stenting and TPVI were performed during the same procedure in 18/19. There was immediate significant decrease in right ventricular systolic pressure (mean 88.6 to 52.2mmHg), mean RVOT gradient (mean 43.7 to 22mmHg) and pulmonary regurgitation (grade 2 or more before, none after). One patient with a 16mm homograft had significant residual stenosis (mean RVOT gradient 45mmHg) after Melody implantation. All patients were discharged within 2 days. At a mean follow-up of 9.3 months (1 to 24 months) all patients are alive and well and neither Melody valve dysfunction nor stent fracture or migration was noticed.ConclusionPercutaneous pulmonary valve implantation is a safe alternative to surgery. Our experience suggests that patients with RVOT conduit < 18mm and those with previous arterial switch operation should be referred to surgery without considering transcatheter procedure.

Purpose Percutaneous closure of patent ductus arteriosus (PDA) using the Amplatzer duct occluder (ADO) is an effective and a true alternative to surgical closure. However, closure of PDA in infants with a low weight remains challenging for the interventionist. Methods From April 1999 to February 2009, 203 patients underwent percutaneous PDA closure. We focus here on the 17 infants weighing ≤ 6 kg and in whom duct was closed using the ADO. All patients were symptomatic: failure to thrive, dyspnoea, and frequent respiratory infections. There were 15 females and 2 males. At implantation, the mean age was 5.1 ± 4 months, and the mean weight was 4.6 ± 0.9 (range 2.8 to 6 kg). The procedure was realized under local anaesthesia (n = 14) and general anaesthesia (n = 3). In 5 of the 17 pts, implantation was performed from a sole venous femoral access without arterial puncture and device release was controlled by transthoracic echocardiography. Size of the duct was 3.6 ± 1.3 mm (range 1.7 to 6 mm) and systolic pulmonary artery pressure was 54 ± 19 mm Hg (range 26 to 96 mm Hg). Implantation succeeded in all but one without any adverse event. Closure was performed by six 5/4 mm ADO, five 6/4 mm ADO, five 8/6 mm ADO, and one 10/8 mm ADO. One patient with failed procedure underwent subsequently surgical closure of the duct. During follow-up (0 to 58 months), Doppler echocardiography showed decrease in ductal shunting: complete occlusion was noticed in 5 pts at day one, 10 pts at one month, 14 pts at 3 months, and 16 pts at one year after implantation. No patient but one had persistent pulmonary hypertension and only one had a moderate stenosis on the left pulmonary artery due to device protrusion. One patient died 15 months after implantation from a non related device cause. Conclusions In skilled hands, percutaneous closure of large PDA in symptomatic infants weighing ≤ 6 kg is safe and effective. Such procedure can solve clinical problems and offer a real alternative to the classic surgical correction.

ObjectivesWe report the multicentric French experience with transcatheter closure in children weighing 15 kilograms or less, with the aim of assessing the efficacy of the procedure in this age group.PatientsWe included all children weighing 15 kilograms or less, and seen between January, 1997, and June, 2004, who had successful transcatheter closure of an interatrial communication within the oval fossa.ResultsTranscatheter closure was performed in 35 patients weighing 15 kilograms or less, of whom 14 were male and 21 female. The procedures were undertaken in 8 different centres, the patients having a median age of 3 years, with a range from zero to 6.2 years, and a mean weight of 13 kilograms, with a range from 3.6 to 15 kilograms. All the patients were symptomatic, with associated cardiac malformations present in 4 cases, and extracardiac anomalies in 4 patients, including Down’s syndrome in 3, and Adams Oliver syndrome in the other case. In 1 patient, emergency cardiac surgery was needed 24 h after the procedure to correct a previously undiagnosed divided right atrium. No other complication occurred. After a median follow-up of 2 years, with a range from 0.5 to 5.2 years, all the patients are asymptomatic, except for one long-standing patient with bronchodysplasia. In 1 other patient, a small residual bidirectional shunt was detected by echocardiography. No patient presented significant arrhythmia. In the patients followed-up for more than 12 months, we found a significant gain in weight gain.ConclusionTranscatheter closure of an interatrial communication within the oval fossa is efficient in children weighing 15 kilograms or less, and can be proposed as a first line of treatment in symptomatic patients. Children with retarded growth tend to have complete recovery within one year of closure.

We report the case of a patient with a congenital aortic valve stenosis associated with a long-term undiagnosed intralobar pulmonary sequestration. The important blood flow through the aberrant artery led to progressive congestive heart failure and severe hemoptysis at the age of 25 years. We demonstrate the regression of cardiac symptoms and left ventricular diameter after surgical resection of the sequestration.

Objective To report a specific pathophysiology of hemidiaphragmatic paralysis that may result in severe hypoxemia. Design Case series. Setting Intensive care unit in a cardiology hospital. Patients The series included three patients with refractory hypoxemia in whom a diagnosis of right-to-left-shunt through a patent foramen ovale was made by contrast echocardiography. The three patients had a complete right hemidiaphragmatic paralysis. Intervention Permanent percutaneous closure of the patent foramen ovale was successfully proceeded in all cases. Main result These procedures resulted in complete normalization of arterial oxygen saturation. Conclusion To our knowledge, only three previous reports have described the association of right-to-left shunt through a patent foramen ovale and hemidiaphragmatic paralysis. Such association may be underestimated.

This report describes the case of a 67-year-old man in whom atrial right-to-left shunt developed after a right pneumonectomy, leading to dyspnea with severe arterial desaturation. Transcatheter occlusion of the patent foramen ovale was successfully performed using a buttoned device. Review of literature and mechanisms of these atrial right-to-left shunts are discussed.

Dyspnea and the decrease in arterial saturation in the upright position in elderly subjects is described as platypnea-orthodeoxia syndrome (POS). POS is secondary to the occurrence of an atrial right-to-left shunt through a patent foramen ovale (PFO).This French multicentric study reports on 78 patients (mean age 67 +/- 11.3 years) with POS who had transcatheter closure of the PFO; frequently associated diseases were pneumonectomy (n = 36) and an ascending aortic aneurysm (n = 11). In all patients, the diagnosis was confirmed by transthoracic or/and transesophageal echocardiography. Five different closure devices were used: Amplatz (n = 45), Cardioseal (n = 13), Sideris (n = 11), Das Angel Wings (n = 8) and Starflex (n = 1). Closure was successful in 76 patients (97%).Oxygen saturation increased immediately after occlusion from 84.6 +/- 10.7% to 95.1 +/- 6.4% (p0.001) and dyspnea improved from grade 2.7 +/- 0.7 to grade 1 +/- 1 (p0.001). A small residual shunt was immediately observed in 5 patients (3 with the Cardioseal device, 1 with the Sideris and 1 with the Amplatz) leading to the implantation of a second device in one case (Cardioseal). Two early deaths occurred unrelated to the procedure (one due to sepsis probably related to pneumonectomy, another due to respiratory insufficiency). Other complications were: a small shunt between the aorta and the left atrium, two atrial fibrillations and a left-sided thrombus which disappeared with anticoagulant therapy. At a mean follow-up of 15 +/- 12 months, there were 7 late deaths related to the underlying disease.Percutaneous occlusion of the foramen ovale is safe and gives excellent results thanks to continuing improvement in available devices. This technique enables some patients in an unstable condition to avoid a surgical closure.

PURPOSE To report percutaneous closure of aortocaval fistulas with the Amplatzer Duct Occluder. CASE REPORTS An aortocaval fistula was diagnosed after surgical repair of an abdominal aortic aneurysm in a 73-year-old man. A 3-year-old girl was treated for a congenital aortocaval fistula in another case. An 8 x 6-mm Amplatzer Duct Occluder was introduced via a 6-F introducer in each case, successfully occluding the fistulous track. Both patients are well and without any echocardiographic evidence of a shunt at 6 months. CONCLUSIONS In selected patients, transcatheter closure of aortocaval fistula with the Amplatzer Duct Occluder could be an alternative to open surgery. Further evaluation is necessary.

We report the case of an infant born prematurely at 27 weeks gestational age with life-threatening pulmonary hypertension crisis as a result of left upper pulmonary vein stenosis. Surgical treatment consisted of a lobectomy, which is a safe and effective procedure. Evidence strongly suggests that the venous stenosis may have resulted from hypertonic drugs infused through an umbilical catheter facing the upper left venous-atrial junction.

The aim of this study is to report complications of transcatheter ASD closure using the Amplatzer Septal Occluder (ASO) (St Jude Medical).From December 1999 to October 2013 (April 2014), 760 patients underwent ASD closure with the ASO. Closure was mostly performed under general anaesthesia and transoesophageal echocardiography control. Choice of the device diameter was established after balloon sizing and measurement of the stretched diameter.Mean age of the patients was 31.9±22 years (0.5 month - 84 years). The stretched diameter was 22.5±6.6 mm (5-40mm) and device dimension 22±6.7mm (4-40mm). Duration of the procedure was 41±15 minutes (10-120 minutes) and fluoroscopic time 7.63±6.65 minutes (1-92 minutes). Dose of radiation was 18.7±22 Gy.cm2 (median 12 Gy.cm2).Implantation succeeded in 96.3% of pts and failure was mainly related to deficient rim. No device related death was noticed. Embolization occurred in 4 pts (0.5%): 1 in the aorta, 1 in the left ventricle, and 2 in the pulmonary artery. All but one underwent surgical extraction and ASD closure. The patient with aortic embolization had percutaneous device extraction and underwent subsequently successful implantation with a larger device. No patient required blood transfusion for any groin hematoma. One patient without aortic rim had hemopericardium one month after implantation; this was corrected by drainage with no recurrence and ASD full occlusion was noticed on colour Doppler control. No late complication was observed. The rate of full occlusion on Doppler control is more than 90%, and the remainings have trivial shunt.Transcatheter ASD occlusion with the Amplatzer Septal Occluder is a safe and effective procedure. The rate of immediate complication is very low and need for immediate surgery following the implantation is rare (

Agenesis of the ductus venosus is a rare anomaly that may be responsible of cardiomegaly and hydrops in fetuses. We report 2 cases in combination with pulmonary artery hypertension (PAH). Agenesis of ductus venosus was diagnosed in 2 boys at the second term of pregnancy. None had associated hydrops. Chromosomal karyotyping was normal in both. Both of them had extracardiac malformation: one had oesophageal atresia (requiring surgery at day 1), the second had malformation of the ear and micropenis. Both had neonatal PAH. The first patient received bosentan at the second month of life. He had persistent PAH (PA pressure of 119/48, mean 77 mm Hg) and underwent closure of a small VSD at the age of 7 months. Two years and half after surgery, he remains in NYHA class II with similar PA pressure despite bosentan therapy. He is awaiting for combination therapy with sildenafil. The second presented pulmonary distress at birth requiring mechanical ventilation. He was placed immediately under a combination therapy of epoprostenol and NO for 4 days, received then treprostinil for 15 days and sildenafil. He could be weaned off the ventilator after 3 days. One month and half after birth, he is doing well under sildenafil but has persistent moderated PAH (tricuspid regurgitation velocity of 3 m/s). The antenatal existence of high grade arteriovenous shunt could explain high pulmonary artery pressure. However, the persistence of PAH in these patients remains unclear. In combination with research of chromosomal anomaly and congenital malformation, infants with agenesis of the ductus venosus should benefit after birth of serial ultrasound examination to rule out PAH.

We report our experience using the buttoned device to close defects within the oval fossa and probe-patent oval foramens, comparing the findings with those obtained with the Amplatzer septal occluder. from 1992 to 1997, we used the buttoned device to close defects in 73 consecutive patients, 64 with defects in the oval fossa and nine with patent foramens. We compared this experience with a further series of 62 patients seen from 1997 to 1999 in whom the Amplatzer septal occluder was used. Successful implantation was achieved in three-quarters of those with septal defects in whom the buttoned device was used, in all of those in whom the buttoned device was used for patent foramens, and in nine-tenths of those in whom closure was attempted using the Amplatzer occluder. Immediate surgery was needed in 3 patients in whom a buttoned device was used, one because of embolization and two with residual shunts and a straddling device. Similar immediate surgery was needed to retrieve one embolized Amplatzer occluder. During follow-up, surgery was needed in a further 7 patients, all having had insertion of a buttoned device, because of atrial perforation in one and a significant residual shunt in the remainder. At late follow-up, the rate of complete occlusion was 69% in the patients in whom the buttoned device was used to close a septal defect, 100% when the buttoned device was used for patent foramens, and 95% in those treated with the Amplatzer occluder. Our experience shows that the Amplatzer occluder produced a significantly higher rate of occlusion for larger defects, and with a shorter fluoroscopy time than the buttoned device. The Amplatzer septal occluder, therefore, is our preferred device for closure of defects within the oval fossa.

Severe mechanical haemolysis occurred in an 11-month-old boy after implantation of the new Amplatzer duct occluder. Temporary balloon occlusion of the aortic ampulla was performed 4 days after the initial procedure leading to prompt abolition of the haemolysis.

The Ross procedure could provide an ideal aortic valve replacement method in children and young adults. We evaluated midterm echocardiographic results to assess pulmonary homograft function as well as pulmonary autograft dimensions and function. In all, 105 patients (26 women and 79 men) underwent the Ross procedure; median age at implant was 29 years. All patients underwent free root replacement. Transvalvular gradients and autograft dimensions were measured at 3 levels (annulus, sinuses of Valsalva, and proximal aorta) at discharge, at 6 months, and annually thereafter. Perioperative mortality was 4.7%. The mean period for echocardiographic follow-up in 100 patients was 32.7 months (range 0.5 to 7 years), during which 4 noncardiac-related deaths occurred. Two patients underwent late reintervention. No moderate or severe regurgitation was recorded. There was 1 case of mild homograft regurgitation and 4 of mild autograft regurgitation at late follow-up. Autograft peak gradients were low and reproducible (5 ± 2.8 mm Hg at discharge vs 5.5 ± 3.5 mm Hg at last follow-up, p=NS). Homograft peak gradients increased significantly without severe obstruction (7.8 ± 5.7 mm Hg at discharge vs 15.8 ± 9.2 mm Hg at last follow-up). The diameter of the autograft annulus was stable during follow-up, whereas autograft dimensions at sinuses and proximal aorta increased significantly. One group of patients was identified with sinus diameter increases >20% (group A). The 90 remaining patients were classified into group B. The only parameter significantly different between the 2 groups was the sinus diameters measured at discharge (1.74 cm/m 2 (group A) vs 1.92 cm/m 2 (group B); p=0.036). In 100 patients and with echocardiographic follow-up for up to 7 years, the Ross procedure showed excellent results. For 10% of patients, we observed a 20% dilation of sinus diameters, but in only 3 patients (3%) was this beyond the upper normal limit.

Background . Pulmonary autograft aortic valve replacement has been introduced in our institution in selected adult patients in light of the known disadvantages and limitations of conventional prosthetic valves. Methods . We prospectively evaluated the use of the pulmonary autograft in a series of 70 young adults (31.2 ± 8.7 years, range 16 to 49 years) operated on from March 1992 to April 1997 with aortic root replacement only. Results . There were no in-hospital deaths and two noncardiac-related late deaths during follow-up of up to 62 months (mean 33 months). Thromboembolic complications were not observed. One patient required reoperation for infective endocarditis 4.3 years after surgery. Discharge echo-Doppler studies showed normal autograft and allograft valve function. Serial echo-Doppler studies showed no significant progression of aortic insufficiency and no dilatation of the autograft. A severe stenosis of the pulmonary allograft developed in 1 patient. Conclusion . Aortic root replacement with a pulmonary autograft, although more complex than conventional prosthetic valve replacement, is a safe, effective, and reproducible procedure in properly selected adult patients. Long-term results remain to be evaluated.

A continuous murmur in a 10 month old boy with no medical history led to the diagnosis of a congenital coronary artery fistula between the left anterior descending coronary artery and the apex of the right ventricle. At 5 years old the child underwent unsuccessful …

Background . The aim of this study was to assess by angiography the late effects of both classic and modified Blalock-Taussig shunts in terms of growth and development of stenosis and distortion. Methods . At a mean of 51 months postoperatively, we retrospectively reviewed the results in 78 patients who underwent creation of Blalock-Taussig shunts (25 classic and 71 modified). Results . At the level of the anastomosis, the shunt caused a reduction in diameter of the pulmonary artery in 49% of all shunts, major stenosis (>50% narrowing of the lumen) in 14%, and distortion of the pulmonary artery in 19%, findings that did not correlate with the type of shunt. Distortion did correlate with younger age at the time of shunt operation ( p = 0.01). Conclusions . After a Blalock-Taussig shunt, growth of the pulmonary arteries occurred but did not exceed the normal growth of the pulmonary arterial tree. Moreover, a shunt procedure can cause distortion and stenosis of the pulmonary artery, which may have important implications for future corrective surgical intervention. All these findings support earlier complete surgical repair of correctable congenital cardiac defects.

The syndrome of tetralogy of Fallot with absent pulmonary valve is characterized by aneurysmal dilatation of the pulmonary arteries causing tracheobronchial obstruction of varying degree. Relief of this obstruction is the main goal of the surgical repair and can best be achieved by appropriate pulmonary arterioplasty. We describe our current technique to repair this syndrome in infants and older children including pulmonary arterioplasty, veno tricular septal defect closure, and right ventricular outflow tract reconstruction without valve insertion.

Background. The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution. Methods. Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 ± 4 months in group A and 72 ± 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure. Results. Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time ( p = 0.005) and longer aortic cross-clamping time ( p = 0.019). In fact, these were clearly related to more complex anatomy ( p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 ± 47 months in group A and 38 ± 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence on Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%. Conclusions. Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.

The Amplatzer Vascular Plugs (AVP) are self-expandable devices made from a nitinol wire mesh that have been used to occlude various collateral vessels and shunts in congenital heart disease. We report here our experience. Methods From 2004 to 2012, 49 procedures with AVP were realized in 41 patients (17 females and 24 males). AVPs were employed to occlude systemic venous shunts (n=14) mainly in Fontan circulation, arterial systemic shunts (n=7), arterio-venous fistulas in Rendu-Osler (n=8), patent vertical vein following total repair of TAPVD (n=5), arterial systemic sequestration in Scimitar syndrome (n=4), fenestration (n=4) and miscellaneous lesions (Blalock shunt, n=2; coronary fistulas, n=2; VSD, n=1; aneurysm, n=1; patent ductus arteriosus, n=1). Results Mean age of patients was 13.8±10.5 years, mean weight 28±11 kg. Thirty one received plug I (mean size 8.3±4.0 mm), 24 pts the plug IV (mean size 5.5±1.5 mm), and 4 pts the Plug II (mean size 17±4 mm). Implantation succeeded in all but 3 in whom the plug was retrieved. One patient with PDA underwent surgical ligation subsequently, a second had successful occlusion of a Blalock shunt with another plug, and the remaining had complete VSD occlusion with another larger device. One device embolized in pulmonary artery that could be retrieved during the procedure. The fluoroscopic time was 19±12 minutes. Full occlusion was confirmed by control angiography, CT scan or echocardiography. During follow-up, no evidence of hemodynamic or vascular compromise was noticed. Conclusion AVPs are effective and safe in the treatment of collateral vessels and shunts. The AVP IV is especially useful in embolizing difficult-to-reach, tortuous vessels with small delivery catheter; it is a good alternative to coils. Plugs I and II are appropriate for occlusion of larger vascular shunts. Choice of AVP is mainly determined by the anatomy and morphology of the shunt.