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Patients with Eisenmenger syndrome (ES) carry a better prognosis from diagnosis than patients with other causes of pulmonary hypertension (PH), but their life span has not yet been clarified.To clarify both survival from diagnosis and life span in ES, and in closed shunt with pulmonary arterial hypertension (PAH), as compared with other causes of PH.Data on all adult patients with PH attending our centre over the past decade was collected. Outcome was defined as death or transplantation.We studied 149 patients, including 30 (20%) patients with ES and 12 (8%) patients with closed shunt with PAH. Median age at diagnosis was lower for patients with ES and closed-shunt with PAH compared to patients with other causes of PH (P0.001 and P=0.008 respectively). Median follow-up was 4.25 years. Survival from diagnosis was longer in ES compared to other causes of PH (logrank; P=0.02) and similar between closed-shunt with PAH and other causes of PH (logrank; P=0.3). Survival rates at 3, 6 and 9 years from diagnosis were: 73%, 50% and 47% for ES, 75%, 25% and 0% for closed-shunt with PAH, 65%, 23% and 9% for other causes of PH. Life span was similar in those three groups (logrank; P=0.2 and P=0.7, respectively).Life span is similar in patients with ES, with a closed-shunt associated with PAH, and in patients with other causes of PH.

Transcatheter closure has become an accepted alternative to surgical repair for ostium secundum atrial septal defects (ASD). However, large ASDs (>38 mm) and defects with deficient rims are usually not offered transcatheter closure but are referred for surgical closure. Several studies have reported the feasibility of transcatheter closure in complex cases with a variety of modified implantation methods such as balloon assisted technique (BAT). AA Pillai and co-authors report the transcatheter closure of ASD ≥35 mm with the BAT. However, the true significance of their study is rather in demonstrating the superiority of BAT to conventional technique and other modified implantation techniques in patients with ASD rather than feasiblity of transcatheter closure of large defect. Finally, a single dimension does not reflect the true ASD size because many defects are not round in shape but rather oval or even crescentric. Hence, future studies will need not only to demonstrate the ideal implantation method but also the appropriate 3-dimensional (3D) imaging definition of the defect in this patient population.

Purpose: Acute myopathy of intensive care has been described infrequently in children and never after organ transplantation. We report a case of acute myopathy of intensive care in a child after heart transplantation. Clinical features: An II-yr-old girl, with no previous medical history, developed acute cardiomyopathy leading to cardiac shock. Family history revealed four cases of unidentified myopathy and/or cardiomyopathy. Preoperatively, while muscle biopsy was near normal, myocardial biopsy revealed non specific mitochondrial disorders. A few days after heart transplantation, she developed acute hypotonia and flaccid quadriplegia, consistent with the diagnosis of acute myopathy of intensive care. Nerve conduction studies were normal, electromyography showed myopathic changes and a new muscle biopsy from quadriceps femoris showed severe loss of myosin filaments and ATPase activity in type 2 fibres. A large laboratory screening failed to demonstrate a metabolic disease or a known myopathy. Muscle strength recovered progressively in three weeks allowing home discharge. A few months later, she was free of symptoms and muscle biopsy showed full histopathological recovery. Conclusion: Acute myopathy of intensive care can occur in children after heart transplantation. It should be suspected in the presence of muscle weakness and difficulty in weaning from ventilatory support. Electromyography confirmed a myogenic process and muscle biopsy allowed diagnosis. Full clinical and histopathological recovery usually occur within three weeks.

An 11-year-old boy with a past history of asthma was admitted to the pediatric intensive care unit (ICU) for a non–Q-wave myocardial infarction that occurred during sustained exercise (a handball match). He presented with chest pain, anterior ST-segment depression on the ECG (Figure 1⇓), and elevation of creatinine kinase (peak, 2580 mU; MB, 240). Initial physical examination was normal, and his ICU course was uncomplicated. He was discharged to the ward after 2 days, and cardiac catheterization was performed 8 days after admission. Left ventriculography (Figure 2⇓) revealed mild apical hypokinesia with an ejection fraction of 55%. Selective coronary arteriography showed no atherosclerotic lesion but hypoplasia of the distal left anterior descending (LAD) and right coronary (RCA) arteries (Figures 3⇓ and 4⇓). There was …

Progressive dyspnea and cyanosis occurred in a 41-year-old patient status after Mustard atrial switch repair for transposition of great arteries. Cardiac catheterization and magnetic resonance imaging revealed the association of superior limb systemic venous baffle obstruction and leaks with right-to-left shunting. He underwent successful dilation of the venous channel and obstruction of baffle leaks by using a covered stent.

Background Echocardiography has been widely used in postoperative assessment after stage I Norwood procedure, but its accuracy in detecting aortic arch obstruction (AAO) has not been determined. This study was designed to determine the accuracy of echocardiography in the diagnosis of AAO after stage I Norwood procedure, identify echocardiographic predictors of arch obstruction, and examine the time course of its development. Methods The records and echocardiography reports of 139 patients who survived stage I Norwood procedure were reviewed. Reference standard for the diagnosis of AAO was catheterization, surgery, or autopsy. Results AAO was diagnosed by reference standard criteria in 31 (22%) patients. Echocardiography correctly diagnosed AAO in 19 patients, missed the diagnosis in five, and wrongly predicted AAO in eight, yielding a 73% sensitivity, 92% specificity, 70% positive predictive value, and 88% accuracy. Moderate or severe right ventricular dysfunction, moderate or severe tricuspid regurgitation, and an abnormal abdominal aortic Doppler flow pattern were more common in patients with AAO. The probability of AAO developing within 6 months after stage I Norwood procedure was 21.1%, with a very small likelihood after that point. Beyond the first 30 days after surgery, the risk of death was higher in patients in whom AAO developed compared with those in whom it did not (relative risk 5.9, 95% confidence interval 2.7 to 13.2). Conclusions Echocardiography is a highly specific modality in detecting AAO after stage I Norwood procedure but its sensitivity is limited. Because of the increased risk of death associated with AAO and because most obstructions develop between 1 and 6 months postoperatively, early cardiac catheterization with possible intervention should be considered in patients with moderate or severe right ventricular dysfunction, moderate or sever tricuspid regurgitation, or an abnormal abdominal Doppler flow pattern during that period. (Am Heart J 1998;135:230-6.)

Background: It is well recognized that a right ventricle (RV) may eventually fail if working as systemic. This has led to arterial switch operation for transposition of the great arteries (TGA), but more recently to anatomic correction of ‘corrected transposition’ or atrio-ventricular discordance (AVD)combining an atrial switch and a ventricular outflow repair (arterial switch or Rastelli type). Methods: We report here 8 cases of such anatomic correction of AVD in patients from 3 months to 10 years. Of these patients, 6 had situs solitus, and 2 situs inversus; 7 patients had a ventricular septal defect (VSD); 5 had an increased pulmonary flow, and 3 had a decreased pulmonary flow with pulmonary stenosis (PS) or pulmonary atresia (PA). In all, 6 had discordant and 1 had concordant ventriculo-arterial (VA) relations, and 1 had double-outlet RV, 2 had an associated coarctation, and 1 had a cor triatriatum. Of the 8 patients, 5 had pulmonary artery hypertension, 3 a moderate to severe tricuspid insufficiency and 6 had undergone one or more previous operations. Correction was done between 3 months and 10 years of age, with 7 having a Senning procedure (twice left-sided) and 1 a Mustard type operation. VA outflows were repaired with: 4 arterial switches, 1 1eft ventricle to aorta rerouting, 1 right ventricular outflow tract (RVOT) repair and 2 Rastelli. A tricuspid annuloplasty was done once. Results: All patients survived the operation, but 2 patients needed a reoperation (both successful): late obstruction of pulmonary venous channel after a left-sided Senning, and residual VSD closure. Mean follow-up is 45 months (3‐122). Of the 8 patients, 6 patients are in sinus rhythm, 2 have junctional rhythm. A patient with junctional rhythm and occasional tachycardia died suddenly 18 months after surgery 1 week after satisfactory evaluation. All have a normal left ventricle function as evidenced by echocardiography. Conclusions: Despite a more demanding procedure, the ‘anatomic repair’ of hearts with AVD is possible, even in infancy, with good early and mid-term result. Despite the potential late rhythmic problems of the Senning operation (somehow more frequent with AVD in our experience), it may become, with increasing experience, the procedure of choice in corrected transposition. © 1998 Elsevier Science B.V. All rights reserved.

Isolation of the left subclavian artery is a rare aortic arch anomaly. Association with a right interrupted aortic arch and a double patent ductus arteriosus is exceptional. We report a case of such an association with successful surgical correction.

Evaluate the incidence, diagnosis, and outcome of coronary compression (CC) during right-ventricular outflow tract interventions.The incidence, risk factors, diagnosis, and outcomes of CC during percutaneous pulmonary valve implantation are poorly defined.One-hundred consecutive patients (May 2008 to January 2012) undergoing transcatheter right-ventricular outflow tract treatment in two institutions were studied.CC occurred in six patients (6%) with a right ventricular outflow conduit stenosis, at a median age of 24.5 (13-49) years. It involved the left main coronary artery in four and the right coronary artery originating from the left anterior descending coronary artery in two patients. Conduit types were homograft (n = 3), bioprosthesis (n = 2), and a pericardial patch (n = 1). Median diameter was 23 (17-24) mm at surgical implantation. CC was diagnosed through a selective coronary angiogram during balloon dilation of the conduit in the first three patients and through an aortic root angiogram for the three next cases because we recognized that proximal compression could be masked during coronary artery cannulation. It was suspected on pre-procedure imaging (magnetic resonance imaging and/or computed tomography) in three cases. Patients with abnormal coronary anatomy tend to be at increased risk of CC (P = 0.0504). One institution had a higher incidence of CC (P = 0.04). CC resolved after balloon deflation. No patient underwent conduit stenting. Four patients underwent surgical reconstruction of right ventricular outflow tract.CC is accurately diagnosed during right-ventricular outflow tract interventions. We recommend an aortic root angiogram during dilation with a non-compliant balloon matching the diameter and length of the intended conduit implant.

We report a case of successful recanalisation of the left pulmonary artery after occlusion due to embolic thrombi in a 9-month-old infant after surgical repair of a common atrioventricular canal with tetralogy of Fallot. A transhepatic approach was used because of caval vein thrombosis. After the failure of high-pressure balloon angioplasty, the left pulmonary artery was successfully recanalised with cutting balloons, followed by stent implantation with an excellent result.

Objective: To report our experience with perventricular closure of muscular (apical) ventricular septal defects (VSDs) in small infants, with echocardiographic guidance only, in a nonhybrid suite. Methods: Eight infants with nine large muscular (apical) VSDs underwent perventricular device closure in a nonhybrid operating room, with transesophageal and epicardial echocardiography guidance, at a mean age and weight of 3.07 (0.3-7.28) months and 3.7 (2.5-6.2) kg, respectively. Five patients had multiple VSDs. Four had associated cardiac defects. Results: Nine Amplatzer muscular VSD devices with a mean size of 10 (4-14) mm were deployed. Seven patients were discharged from the intensive care unit with a mean length of stay of 8.6 days. Four patients had minimal postprocedural residual shunt; no one had a residual shunt at six-month follow-up. Mid-term results are excellent. Conclusion: Perventricular closure is feasible under echocardiographic guidance only in small patients, even without hybrid suite. This may be a good approach for very symptomatic low-weight infants with apical VSD and may also be useful, in any center, at any time, and in any operating room, to treat an associated apical VSD, even unexpected.

Aortic coarctation is an unusual cause of hypertension during pregnancy and its management is not clarified. We report transcatheter balloon dilatation and stenting for native aortic coarctation in a 22-year-old pregnant woman with severe and uncontrolled systemic hypertension. Arterial blood pressure could be successfully controlled with medical treatment during the rest of the pregnancy and the patient underwent uneventful delivery. No adverse events or recoarctation was observed during 24 months clinical follow-up. In conclusion, native aortic coarctation can be successfully treated during pregnancy with transcatheter therapy. More experience is needed to confirm the safety and efficacy of such management.

Percutaneous transcatheter closure of a patent foramen ovale (PFO) remains challenging when femoral venous approach is not available. We describe the successful closure of a PFO using the right internal jugular venous approach and a deflectable catheter delivery system in a patient with a PFO, recurrent stroke, and an inferior vena cava filter.

Percutaneous transcatheter closure of a patent foramen ovale (PFO) remains challenging when femoral venous approach is not available. We describe the successful closure of a PFO using the right internal jugular venous approach and a deflectable catheter delivery system in a patient with a PFO, recurrent stroke, and an inferior vena cava filter.

Background— The origin of congenital or childhood nonimmune isolated atrioventricular (AV) block remains unknown. We hypothesized that this conduction abnormality in the young may be a heritable disease. Methods and Results— A multicenter retrospective study (13 French referral centers, from 1980–2009) included 141 children with AV block diagnosed in utero, at birth, or before 15 years of age without structural heart abnormalities and without maternal antibodies. Parents and matched control subjects were investigated for family history and for ECG screening. In parents, a family history of sudden death or progressive cardiac conduction defect was found in 1.4% and 11.1%, respectively. Screening ECGs from 130 parents (mean age 42.0±6.8 years, 57 couples) were compared with those of 130 matched healthy control subjects. All parents were asymptomatic and in sinus rhythm, except for 1 with undetected complete AV block. Conduction abnormalities were more frequent in parents than in control subjects, found in 50.8% versus 4.6%, respectively ( P P P P SCN5A mutation screening identified 2 mutations in 2 patients among 97 children. Conclusions— ECG screening in parents of children affected by idiopathic AV block revealed a high prevalence of conduction abnormalities. These results support the hypothesis of an inheritable trait in congenital and childhood nonimmune isolated AV block.

Summary Background Owing to the wide variety of surgical substrates used for right ventricular outflow tract (RVOT) reconstruction, the predictors of successful outcomes in such patients are unclear. Aims To compare haemodynamic outcomes of percutaneous pulmonary valve implantation (PPVI) in patients with dysfunctional RVOT. Methods This was a multicentre prospective study on all consecutive patients who underwent PPVI from May 2008 to December 2009. All patients underwent prestenting using a bare-metal stent. The patients were divided into two groups based on the surgical substrate used for RVOT reconstruction. Results Baseline demographics, including right ventricle to pulmonary artery (RV-PA) pressure gradient and RV/aortic (Ao) pressure ratio, were similar in both groups. The mean RV-PA gradient and RV/Ao pressure ratio showed immediate and significant improvement after PPVI. At last follow-up, the RV-PA gradient and RV/Ao pressure ratio were significantly higher in patients with non-expandable conduits ( P = 0.002 and P = 0.008, respectively). Patients with conduits greater than 20 mm showed better immediate and midterm outcomes compared with other patients. Patients with non-expandable conduits less or equal to 20 mm diameter showed good immediate outcomes but poor midterm haemodynamic outcomes compared with those with expandable conduits less or equal to 20 mm diameter ( P = 0.03). Conclusions PPVI is successful with a wide variety of surgical substrates used for RVOT reconstruction; there was immediate haemodynamic improvement in all patients. However, patients with non-expandable conduits less or equal to 20 mm had the worst outcomes. This information should be integrated into the decision-making process before selecting patients for PPVI.

SummaryPulmonary 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).

The outcomes of homografts (HGs) in the reconstruction of the right ventricular outflow tract (RVOT) in non-Ross patients are often considered disappointing, compared with Ross patients; and the risk factors for HG degeneration are still controversial. The objective of this study was to determine the durability and prognostic factors related to the HGs implanted in non-Ross patients and to propose potential ways to improve the results.A retrospective study (1993-2010) included 115 consecutive non-Ross patients who received a HG for RVOT reconstruction. The median age at implantation was 2.8 years (4 days-58 years). The main heart defects were pulmonary atresia with ventricular septal defect (n = 40; 34%), truncus arteriosus (n = 28; 24%) and tetralogy of Fallot (n = 23; 20%). Thirty-eight percent had preoperative pulmonary hypertension. A low-dose corticosteroid therapy was used during the postoperative period in patients displaying a HG-related inflammatory response (no septic context) (n = 11). The median diameter of the implanted HG was 22 mm (range 9-30 mm). The median age of the HG donor was 14 years (range 0.5-65 years). ABO compatibility rules were not systematically respected for the HG implantation: 43% of the implanted HGs were ABO compatible with the recipient. The endpoints were HG stenosis (peak gradient ≥20 mmHg), regurgitation (moderate or severe), dysfunction (peak gradient ≥ 50 mmHg or regurgitation moderate or severe) and failure (explantation or balloon dilation).Freedom from HG explantation and failure were 89 and 80% at 5 years, and 76 and 69% at 10 years, respectively. HG donor age30 years [hazard ratio (HR): 2; P = 0.012], preoperative pulmonary hypertension (HR: 3; P = 0.02) and HG mismatch (HR: 5; P = 0.04) were multivariate risk factors for HG stenosis, regurgitation and failure, respectively. HG diameter22 mm was a multivariate risk factor for HG regurgitation (HR: 8; P0.001), dysfunction (HR: 9; P = 0.02) and failure (HR: 5; P = 0.03). ABO incompatibility increased the risk of HG stenosis (HR: 4; P = 0.009) and dysfunction (HR: 2; P = 0.04). The use of corticosteroids significantly protected against the risk of HG regurgitation (HR: 0.08; P = 0.04) in the multivariate analysis.The cryopreserved HG implanted to reconstruct the RVOT in non-Ross patients remains one of the most acceptable options in this specific non-Ross population. The outcomes of HGs in non-Ross patients might be improved by implanting an ABO-compatible HG with an adapted diameter, coming from a donor30 years and by optimizing the perioperative afterload of the HG.

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.

Aortic coarctation is an unusual cause of hypertension during pregnancy and its management is not clarified. We report transcatheter balloon dilatation and stenting for native aortic coarctation in a 22-year-old pregnant woman with severe and uncontrolled systemic hypertension. Arterial blood pressure could be successfully controlled with medical treatment during the rest of the pregnancy and the patient underwent uneventful delivery. No adverse events or recoarctation was observed during 24 months clinical follow-up. In conclusion, native aortic coarctation can be successfully treated during pregnancy with transcatheter therapy. More experience is needed to confirm the safety and efficacy of such management.

Stents have contributed to the development of the interventional catheterization of congenital heart diseases (CHD). The aim of this study was to describe current indications and results. Methods Participation to this study was proposed to all catheterization laboratories specialized in CHD in France (M3C network). All cases with stents implantation in 2013 in pediatric and adult CHD were retrospectively included. Adverse events were assessed using the Bergersen classification. Risk markers were searched using univariate and multivariate analysis. Results 174 stents were implanted in 127 patients. Patients were aged 15.7±15.1 year old (min 5 day-old - max 70 year-old). Main indications were transcatheter pulmonary valve replacement (prestenting and revalvulation; n=29 patients, 23.0%), pulmonary artery branches angioplasty (n=34, 27.0%), aortic coarctation or recoarctation stenting (n=37, 29.4%) and ductus arteriosus stenting (n=11, 8.7%). 18 Patients (14.3%) were under one-year old and 35 (27.8%) were over 18 year-old. Main pathologies were tetralogy of Fallot and variants (ACC-CHD 8.3, n=45, 35.7%), and aortic coarctation (ACCCHD 9.2, n=36, 28.6%). More than one stent was implanted in 32 procedures (24.4%, max. 5 stents). Main stents implanted were the CP stent (33.0%), EV3 LD max (22.0%), Valeo (16.2%) and valved stents (15.0%). 98.5% Procedures were considered as successful. Adverse events were observed in 16.7% procedures (stent related in 12.1% procedures). Serious adverse events were observed in 12.9%. After uni- and multivariate adjustment, only procedure type was related to the risk of total adverse events but not to stent related adverse events. Age, weight, center, type of stents, genetic disorder and type of CHD were not significantly related to the risk of adverse events. Conclusion Stents are used in various CHD catheterization procedures, from infancy to adult age. Adverse events rate is significant and seems related to the type of procedure.

A 19-year-old man with multiple-system injuries including a serious head injury and two poorly tolerated traumatic ventricular septal defects, was admitted to our hospital. Transcatheter closure of the cardiac defects was attempted instead of surgical repair because the required anticoagulation for cardiopulmonary bypass could precipitate intracranial bleeding. The two ventricular septal defects were successfully closed with Amplatzer devices, but the patient remained in hemodynamically unstable condition and subsequently died. Transcatheter closure of traumatic ventricular septal defect is an alternative to surgical repair, although it remains a hazardous procedure and requires experienced anesthesia management.

The study aim was to determine the dynamics and risk factors related to cryopreserved homografts implanted to reconstruct the right ventricular outflow tract (RVOT) during the Ross procedure.A retrospective study, conducted between 1993 and 2009, included 107 consecutive patients who underwent a Ross procedure with implantation of a homograft (102 pulmonary, five aortic). The median patient age at implant was 18.8 years (range: 2 months - 67 years). The main cardiac defects were congenital aortic stenosis (n = 44), congenital aortic regurgitation (n = 20), and combined (n = 23) and acquired (n = 20) aortic regurgitation. The study endpoints were homograft stenosis (peak gradientor = 20 mmHg), regurgitation (gradeor = moderate), homograft dysfunction (stenosisor = 50 mmHg or regurgitationor = moderate), homograft explant, and failure (explant or balloon dilation). The mean follow up was 5.7 +/- 0.4 years. The statistical analysis was conducted using univariate and multivariate Cox regression tests.The overall patient survival was 97% at 18 years. Homograft stenosis occurred in 54% of patients, regurgitation in 18%, dysfunction in 27%, explant in 5%, and failure in 6%. Freedom from homograft dysfunction was 75% and 55% at five and 10 years, respectively, while freedom from homograft failure was 99% and 84% at five and 10 years, respectively. The main multivariate risk factors for dysfunction were: homograft diameter22 mm (OR: 11, p = 0.019), aortic homograft (OR: 18, p = 0.019) and preoperative right ventricle/left ventricle pressure ratio0.4 (OR: 5, p = 0.018). Univariate risk factors for failure were: homograft diameter22 mm (p = 0.006), donor age30 years (p = 0.03), cold ischemia time2 days (p = 0.04), and decontamination time12 h (p = 0.01).In the Ross procedure, the cryopreserved homograft represents an excellent means to reconstruct the RVOT, with a good long-term longevity. Almost 85% of patients did not require any surgery or percutaneous intervention at 10 years after implantation. The homograft long-term durability depends on both homograft-related and patient-related factors. Pulmonary homografts, with a diameter22 mm, a donor age30 years, and a cold ischemia time2 days are to be preferred.

Objective: After treatment of transposition of the great arteries (TGA), ventricular septal defect (VSD), pulmonary stenosis, or atresia by various surgical procedures, two main problems led to reoperation: RV—PA conduit or connection stenosis and subaortic stenosis. We report here our mid- and long-term experience of a technique described in 1997 using a segment of aortic autograft as a RV—PA conduit. Methods: Between 1993 and 2005, 25 patients with TGA, VSD, PS, or atresia were corrected with a technique using an intra-ventricular rerouting with conal septum resection (as in reconstruction-endo-ventriculaire (REV)) and interposition of tubular segment of autograft aorta between RV and PA without Lecompte maneuver (as in Rastelli), using in some cases an additional monocusp patch. The patient’s age at correction ranged from 2.5 months to 11 years (mean 2.2 years); seven patients were under 1 year and 13 had a previous shunt procedure. Patients were regularly followed by twodimensional(2D)annualechocardiogram, occasionally bycatheterizationandmorerecentlybyMNR.Results:There wasonepostoperativedeath essentially due to RV failure. There was one late death after 15 years. There were 23 long-term survivors with a mean follow-up of 12.8 years. All patients were in class I category and all of them were in sinus rhythm. Three of them needed a reoperation at 4, 5, and 6 years for subaortic stenosis and one of them with abnormal chordae in the left ventricle (LV) outflow, Only one patient needed a reoperation for RV—PA stenosis, 13 years after correction done at age 2.5 months, the autograft tissue not being implied and found normal at histology. Mean RV pressure by echo at last follow-up was 41 mmHg. Ten patients exhibit a mild-to-moderate pulmonary regurgitation. Freedom from reoperation for RV—PA obstruction is 90%at10and15years.Conclusion:Comparedwithallothertechniques(Rastelli,REV, andNikaidoh)ourautografttechniqueprovidesthebestreported outcome for RV—PA outflow freedom from reoperation, the aortic segment employed being a living tissue susceptible to growth. However, it remains most often a long-term valveless procedure and does not avoid occasional unexpected LV—AO stenosis. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

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 mm2/m2) 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. © 2011 Wiley-Liss, Inc.

IntroductionIn our centre, device closure is the first-intention therapy for secundum atrial septal defect (ASD). In difficult cases we use a Meditech sizing balloon to optimize position of the device. We sought to determine (1) the efficacy of this sizing balloon-assisted technique (SBT) and (2) the percentage of ASDs that can be percutaneously closed.PatientsIn 2009, 65 patients (37 female) were referred for secundum ASD closure, at a median age and weight of 27.5(0.8 to 88) years and 40.6(5.6 to 97) kg, respectively. Six had pulmonary hypertension.ResultsEight patients had surgical closure because of referral cardiologist and/or patient's choice. Among the 57 remaining patients, 4 were unsuitable by echocardiography and surgically closed. Transcatheter closure was attempted in 53 cases, including 28 children (53%), and was successful in 50 cases, including 10 with SBT (20%). Amplatzer devices were used in all the patients with a median Amplatzer septal occluder (ASO) size of 20 (10 to 40) mm. A 12 mm ASO could not be positioned in a 5.6 kg infant. Surgical closure was contraindicated for poor general condition (former 26 weeks premature baby with severe bronchodysplasia). Transcatheter closure failed in 2 cases, despite SBT. No major complication occurred. Five patients (10%) had a trivial residual shunt. By univariate analysis, the deficiency of superior rim ( 25mmHg) were associated with failure to percutaneously close the ASD (p = 0.02 and 0.03, respectively). Out of 57 patients candidates for transcatheter closure, 50 were successfully closed percutaneously (87%).ConclusionTranscatheter closure of ASD is successfully accomplished in the majority of the cases. The SBT is safe and useful for device positioning and delivery in patients with large ASDs and deficient superior rim.

Introduction Percutaneous pulmonary valve insertion has been recently introduced in clinical setting. Patient selection is widely accepted. These candidates are however heterogeneous, in regard of heart defects, and type of surgical right ventricular outflow tract (RVOT) reconstruction. It is presently unclear in the literature if type of surgical reconstruction matters for the success of the pulmonary valve insertion. Our goal was to compare the hemodynamic results of percutaneous pulmonary valve in patients with homografts, prosthetic conduit or RVOT reconstructed with patch. Patients and methods We reviewed patients included over the last 6 months in the prospective study (REVALV) for patients undergoing RVOT intervention for severe stenosis and/or insufficiency. Only valved stent group is analyzed here. All patients undergoing valved stent implantation are previously pre-stented with a bare metal stent according to present recommendations. Thirty-seven patients were included, distributed in three groups according to type of RVOT reconstruction (homograft REVALV is a multicentric prospective study for patients undergoing RVOT intervention for severe stenosis and/or insufficiency. Patients are distributed in three groups according to type of RVOT reconstruction (homograft, n = 10; prosthetic conduit, n = 20; RVOT enlargement by patch, n = 7). Results Overall, all groups were similar in RV to AP gradient improvement (after pre-stenting mean 30,79 vs 28 p = NS; final result mean 23,71 vs 28,17, p = NS), RV to aorta pressures ratio (after pre-stenting 0,187 vs 0,3117 p = NS; final result man 0,315 vs 0,317, p = NS). If considering non-extensible synthetic tubes we observe that RV-to-AP improvement is significantly worst to the rest of the group (mean 7,07 vs 0,17, p = 0,005). When focusing on outflow tract diameter, results did not differ in homograft group and patch group. In contrast, diameter did play a role in those patients having a synthetic tube, with a cut-off at 20 mm diameter. Below 20 mm, relieve of outflow tract gradient was significantly worse than for bigger conduits. Discussion Pulmonary valve insertion is efficient in all type of RVOT reconstruction at least in the short term. The diameter of the conduits did not play a role in RVOT obstruction relief as long as surgical substrates are homografts or patch enlargement. In patients with prosthetic conduits, size matters. In non-extensible synthetic tubes results are worst. Reduced distensibility and progressive diameter reduction may lead to not consider these patients as good candidates for this procedure.

Stents have contributed to the development of the interventional catheterization for congenital heart diseases (CHD). The aim of this study was to describe indications, results and adverse events in current practice. Methods The participation to this study was proposed to all catheterization laboratories specialized in CHD in France (M3C network). All cases with stents implantation in 2013 in pediatric and adult CHD were retrospectively included. Adverse events were assessed using the Bergersen classification. Results 174 stents were implanted during 132 procedures in 127 patients by 6 main operators in 9 centers. Patients were aged 15.7 ± 15.1 years old (min 5 day-old - max 70 years old). Sixteen types of procedures were identified. Main indications were transcatheter pulmonary valve replacement (29 patients, 23.0%), pulmonary artery branches angioplasty (34 patients, 27.0%), aortic (re)coarctation stenting (37 patients, 29.4%) and ductus arteriosus stenting (11 patients, 8.7%). 18 patients (14.3%) were under one y.o and 35 (27.8%) were over 18 y.o. Main pathologies were tetralogy of Fallot and variants (ACC-CHD 8.3, n = 45, 35.7%), and aortic coarctation (ACC-CHD 9.2, n = 36, 28.6%). More than 1 stent was implanted in 32 procedures (24.4%, max. 5 stents). Main stents implanted were the CP stent (33.0%), EV3 LD max (22.0%), Valeo (16.2%) and valved stents (15.0%). 98.5% procedures were considered as successful. Serious adverse events were observed in 12.9%. After multivariate adjustment, only procedure type was related to the risk of total adverse events (OR: 3.0, 95%CI 1.1–7.9) but not to stent related adverse events. Age, weight, center, operator, type of stents, stent diameter, genetic disorder and type of CHD were not significantly related to the risk of adverse events. Conclusion Stents are used in various CHD catheterization procedures, from infancy to adult age. Adverse events rate is significant and seems related to the type of procedure.

Williams–Beuren syndrome (WBS), a genetic disease involving the elastin gene, is characterized by elfin face and mental and growth retardation. Cardiovascular involvement primarily comprises supravalvular aortic stenosis and peripheral pulmonary stenosis. Neonates with WBS are commonly asymptomatic, although a few articles report with the presence of aortic coarctation (COA). We sought to determine the frequency and the clinical profile of infants with WBS and COA.

Eisenmenger syndrome is the last-stage of congenital heart diseases with untreated left to right shunt. Tolerance and prognosis are better than with idiopathic pulmonary hypertension. However, patients follow-up is often performed in inappropriate non-specialized care centres which makes the collection of epidemiological data difficult. Consequently, population registries are necessary and would increase knowledge of natural history. Finally, complications are often multiple and unexpected with lack of specialized management. Such patients require expert supervision with a multidisciplinary approach in experienced and specialized centres.

International audience; BACKGROUND: Limited data are available describing paediatric pulmonary arterial hypertension. AIMS: To characterize the epidemiology, management and impact on quality of life and outcome of paediatric pulmonary arterial hypertension, excluding persistent pulmonary hypertension of the newborn and pulmonary arterial hypertension caused by congenital heart disease. METHODS: In this multicentre study, children with pulmonary arterial hypertension were included and followed prospectively for two years at 21 referral centres in France. WHO functional class, 6-minute walk distance and quality of life (CHQ-PF50 questionnaire) were evaluated. RESULTS: Fifty children were included with a mean age of 8.9 +/- 5.4 years from May 2005 until June 2006. The estimated prevalence for pulmonary arterial hypertension was 3.7 cases/million. Patients had idiopathic pulmonary arterial hypertension (60%), familial pulmonary arterial hypertension (10%), pulmonary arterial hypertension associated with, but not caused by, congenital heart disease (24%), pulmonary arterial hypertension associated with connective tissue disease (4%) or portal hypertension (2%). During follow-up, the combination of pulmonary arterial hypertension-specific therapies was increasingly prescribed (44% patients versus 22% at inclusion). Patients remained stable regarding clinical status, 6-minute walk distance and quality of life. Survival estimates after one and two years were 86% (95% CI 76, 96) and 82% (95% CI 71, 93), respectively. CONCLUSIONS: In children, idiopathic/familial pulmonary arterial hypertension accounts for the majority of cases. A specific pulmonary arterial hypertension group in conjunction with congenital heart disease can be identified that resembles patients with idiopathic pulmonary arterial hypertension. Combined pulmonary arterial hypertension-specific therapies may have contributed to disease stability and favourable survival.

Introduction Pulmonary arterial hypertension (PAH) is a devastating disease with poor survival but there are limited data describing its impact in the paediatric population. Objective The objectives of this national prospective registry were to collect clinical and epidemiological data and to investigate the outcome of children with PAH. Methods All consecutive patients Results Fifty patients were included between May 2005 and June 2006. Mean age at diagnosis was 8.7 ± 5.4 years and male/female ratio was 1/1. The prevalence of pediatric PAH was 4.2 cases/million. A history of prematurity, cancer and major surgery was noticed in respectively 19%, 6% and 4% of patients. At inclusion, 28% of patients were in WHO FC III or IV. Aetiology of PAH was idiopathic in 60%; familial in 10%, associated with but not due to CHD in 24%, related to connective tissue disease in 4% and to portal hypertension in 2%. Nine patients (018%) died during the 2-year follow up. Survival estimates at 1 and 2 years were 86% and 82%. Seventy three percents of patients improved or did not change WHO FC (n = 44). Patients remained stable regarding 6MWD (n = 25), hemodynamics (n = 11), and QoL (n = 19). During the 2-year follow-up, combination of PAH-specific therapies was increasingly prescribed (44% patients vs. 22% at inclusion). Conclusions This first national paediatric registry showed (1) the presence of multiple PAH aetiologies in children, that could be different from those seen in adults, (2) the association with other co-morbidities, and (3) the stabilization or even improvement in patient's condition with the therapeutic management recommended in the current era.

Ischaemic intestinal disease (IID) in neonates with congenital heart defects (CHDs) other than patent ductus arteriosus (PDA) can represent a complex issue.1 2 The aim of this study was to determine the respective roles played by the CHD and the cardiac surgery in the occurrence of IID, and thus to discuss the timing for cardiac surgery in these children. A retrospective study from 1996 to 2006 screened 4915 newborns for IID and CHD. Isolated PDA being excluded, 12 of these newborns had …

We describe our experience with 2 patients having Costello syndrome, aged 11 and 36 months, who suffered systolic anterior motion of the aortic leaflet of the mitral valve and obstructive cardiac hypertrophy requiring surgery, comparing their cardiac characteristics to those described previously. We conclude that the heterogeneous nature of the cardiac hypertrophy in this syndrome can be considered and managed as the sum of a diffuse hypertrophy accessible to beta-blockade, and an asymmetric hypertrophy accessible to surgical myomectomy.

Purpose To evaluate the efficacy and safety of intravenous sildenafil for immediate postoperative pulmonary hypertension (PH) in pediatric patients undergoing congenital heart surgery. Methods A double-blind, multicenter, placebo-controlled, dose-ranging, parallel-group trial was conducted. Patients were randomized to one of three doses of intravenous sildenafil, or placebo, for a minimum of 24 h. Results The study was heavily underpowered. Whereas enrollment of 228 patients (57 per treatment arm) was required to achieve the sample size estimate to detect difference between arms, the sponsor terminated the study after 15 months owing to slow patient accrual. Seventeen patients (median age 5 months) experiencing postoperative PH were randomized and treated, five with placebo and four each with low-, medium-, and high-dose sildenafil. In the first 24 h, 40% of placebo and 17% of sildenafil patients required additional therapy (p = 0.330). Median time to extubation (3 versus 8 days, p = 0.023) and intensive care unit stay (6 versus 15 days, p = 0.008) were shorter for sildenafil patients. Mean ± standard deviation systolic pulmonary artery pressure was reduced with sildenafil (46 ± 11 to 35 ± 6 mmHg, p = 0.027 versus placebo). No adverse events or systemic hypotension were attributed to sildenafil. Conclusion Intravenous sildenafil reduced pulmonary artery pressure and shortened time to extubation and intensive care unit stay in children with postoperative PH. Electronic supplementary material The online version of this article (doi:10.1007/s00134-010-2065-4) contains supplementary material, which is available to authorized users.

Congenital pulmonary vein stenosis (PVS) presents as an isolated lesion or in association with other congenital heart anomalies. The most extensive forms of the disease are uniformly fatal because neither surgical repair nor transcatheter therapy results in long-term relief of the stenosis. A case is presented involving single-ventricle physiology associated with extensive and recurrent congenital PVS despite multiple attempts with surgical therapy. Heart-lung transplantation was ultimately performed after drug-eluting stents were placed in pulmonary veins as a bridge to the transplantation.

Background Controversy remains as to whether isolated cleft of the mitral valve and cleft of the atrioventricular septal defect are different entities. Our objectives were to provide a precise description of isolated cleft of the mitral valve and to clarify its surgical management and outcome. Methods Patients with surgical repair of isolated cleft of the mitral valve were included. Results Ten patients (9 female) underwent repair at a mean age of 12.1 ± 10.5 years and mean weight of 32.1 ± 17.8 kg. Preoperative echocardiography showed mild or less than mild mitral regurgitation in 6 cases and moderate to severe regurgitation in 4. Intraoperative examination confirmed in all cases a cleft dividing the anterior leaflet of an otherwise normal mitral valve. Attachment of the cleft to the ventricular septum by accessory chordae was found in 3 cases whereas preoperative echocardiography found such attachments in 5. Direct suture of the cleft was performed in 9 cases, associated with repair of tricuspid valve straddling (n = 1), subaortic stenosis (n = 1), and ventricular septal defect (n = 1). One patient with thickened cleft's edges required an Alfieri-type repair. After a mean follow-up of 4.9 years (range, 1.3 to 11.9), all patients are asymptomatic without significant mitral regurgitation. Conclusions Echocardiographic description of isolated cleft of the mitral valve is not always as accurate as intraoperative analysis. This is a distinct morphologic entity from the cleft of the left-sided valve of atrioventricular septal defect, and seems associated with a strong female predominance, with various cardiac and extracardiac features. Surgical repair is successful with excellent midterm results.

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.