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The anomalous origin of the left coronary artery from the pulmonary artery in the position of a non-facing coronary sinus is extremely rare. The anatomical position of the ectopic ostia will determine which is the appropriate operative approach to create a dual-coronary supply. This report describes a technique of modified extra-anatomical rerouting using autologous pericardium patch and pulmonary artery flap to create a neo-composite coronary trunk passing anterior to the right ventricular outflow tract.

Background Pulmonary homografts (PH) are used as a first-line treatment for surgical right ventricular outflow tract (RVOT) reconstruction in patients with congenital heart disease (CHD). Despite a better freedom from reintervention than prosthetic conduits, PH are not spared from failure and cases of early dysfunction are regularly described. Aims The aim of this study was to assess the rate of early PH dysfunction in patients of the Bordeaux University Hospital and to identify associated risk factors. Methods A monocentric retrospective study was conducted in children and adults with CHD and PH implantation for RVOT reconstruction. Clinical and echocardiographic data were collected during follow-up. PH dysfunction was defined as a peak of gradient greater than 50 mmHg and/or as pulmonary regurgitation greater than moderate. Early dysfunction was defined as occurring within two years postoperatively. Primary endpoint was the early PH dysfunction rate at 2 years. The dysfunction-free survival curve was calculated according to the Kaplan-Meier method. A logistic regression with univariate then multivariate analysis was performed to identify risk factors for early dysfunction. Results Between January 2002 and November 2020, 112 PH were implanted in 110 patients and 11 cases of homograft dysfunction were reported during the follow-up, including 9 cases of early dysfunction. The rate of early dysfunction was 9.4 [3.3–15.1] % and freedom from reintervention was 94.6 [90.0–99.0] % at two years. The only independent risk factor identified by the multivariate analysis was duration of extracorporeal circulation (P = 0.007) but the length of stay in intensive care unit (P = 0.088) and the initial maximum pulmonary transvalvular gradient (P = 0.06) were also close to significance in the multivariate analysis. Conclusion Although PH provide a durable substitute for RVOT reconstruction, a significant proportion of patients presents early PH dysfunction and requires premature reintervention. An inflammatory mechanism is suspected but dedicated studies should be conducted to validate this hypothesis.

Atresia of the main stem of the left coronary artery is the least observed congenital coronary anomaly; most patients tend to receive a coronary artery bypass graft, although some anatomical corrections have been described. A 17-year-old female patient with left coronary artery main stem atresia underwent a coronary trunk construction with an autologous pericardial patch in our department. At a 3-year follow-up, the patient was asymptomatic, with a normal cardiac stress test. The coronary computed tomography showed no stenosis between the aorta and coronary bifurcation. Long-term patency has yet to be determined.

Plasma troponin is a risk factor for cardiac events in various populations. We evaluated the determinants and predictive value of plasma cardiac troponin I (cTnI) during the long-term follow-up of heart transplantation.We prospectively measured cTnI in 100 heart transplant recipients, 13.0 ± 6.0 years after transplantation, during a routine visit including echocardiography and laboratory measurements. The patients were followed for 21.3 ± 4.9 months after this troponin measurement. cTnI ≥0.006 μg/L (detection threshold) was found in 37 of these 100 patients. Plasma troponin significantly increased with the presence and severity of coronary lesions detected by means of coronary angiography and was significantly associated with age, left ventricular mass, history of post-transplantation heart failure, body mass index, and plasma creatinine. Of 37 patients with cTnI ≥0.006 μg/L, 13 had a cardiac event during follow-up compared with 2 of 63 patients with cTnI0.006 μg/L (P.0001). The relation between cTnI and cardiac events remained significant in multivariate analysis.cTnI is frequently detectable during long-term follow-up after heart transplantation and is associated with chronic graft rejection and renal failure. A detectable cTnI may help identify heart transplant recipients at high risk of cardiac events.

A 21-year-old man with univentricular and uniatrial heart came for a third attempt at ablation of recurrent supraventricular tachycardia. He had undergone a previous cardiac surgery of a Blalock–Taussig shunt, and bidirectional Glenn, and the electrophysiological study revealed an atrioventricular

We present the case of a 4-month-old child with atrioventricular canal associated with severe left atrioventricular valve dysfunction who previously underwent 3 surgical valve reconstructions without significant improvement. A Hybrid Melody valve (Medtronic, Minneapolis, MN) insertion was planned. Because of the low weight, the risk of left ventricular outflow tract obstruction was significant and therefore evaluated preprocedurally using a cardiac computed tomography-derived 3-dimensional printed model. In vitro tests showed good anchorage of the valve without subaortic obstruction and the procedure was then achieved with an excellent clinical result.

Background How reliable is home blood pressure monitoring (HBPM) in heart transplant recipients is not known. Possibly, it may underestimate hypertensive burden, because blood pressure (BP) nondipper profile is frequent among these patients. This prospective study has been designed to determine whether HBPM adequately identifies hypertension in heart transplant recipients. Method We compared HBPM with ambulatory blood pressure monitoring (ABPM) for the diagnosis of uncontrolled hypertension in 74 patients 13.5±6.7 years after heart transplantation. HBPM was measured with a validated semiautomatic device twice every morning and twice every evening on 7 consecutive days, within 15 days of ABPM. We also measured the relationship between HBPM, ABPM, and organ damage as measured by albuminuria and left ventricular mass. Results A nondipper profile was found in 53 (72%) patients. HBPM and ABPM were close according to Pearson bivariate correlations. There was no significant correlation between left ventricular mass and BP either at HBPM or ABPM. Proteinuria significantly correlated with systolic BP either at HBPM (R=0.42; P=0.0002) or ABPM (R=0.25; P=0.03). HBPM adequately classified 61 of 74 (82%) patients as hypertensives or as nonhypertensives or controlled hypertensives. Conclusion Despite a high prevalence of nondipper profile, HBPM gives a reliable estimate of BP burden in most heart transplant recipients. Thus, our results strongly suggest that HBPM is useful for the long-term follow-up of heart transplant recipients.

Background A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium. Methods We retrospectively studied such patients in 5 institutions. Results Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n = 2) and secundum atrial septal defects (n = 3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n = 2), secundum atrial septal closure (n = 2), left upper pulmonary vein stenosis stenting (n = 1), and coarctation stenting (n = 1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40 years of age tend to be symptomatic at presentation (p = 0.056). Conclusion In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients. © 2013 Elsevier Ireland Ltd.

A neonate with absent pulmonary valve syndrome (APVS) and features of tetralogy of Fallot presented respiratory distress. The prolonged need for mechanical ventilation was an indication for surgical repair. Preoperative thoracic CT imaging showed nearly complete closure of the distal trachea, compressed by the aneurysmal dilatation of the pulmonary arteries, and its reopening after ventilator pressure was increased. Management of APVS may require high-pressure ventilation to prevent bronchial collapse due to compression by the pulmonary arteries. In this context, thoracic CT imaging is very useful for adjusting ventilation support so that surgical repair can be performed in optimal conditions.

International audience; Background. - The ageing graft frequently shows coronary lesions and a restrictive physiology. Aims. - To determine the presenting features and outcome of chronic heart failure in heart transplant recipients. Methods. - In this cohort study, we compared 44 consecutive heart transplant recipients who developed chronic heart failure more than 1 year after heart transplantation with 44 control heart transplant recipients who did not develop heart failure. Results. - We found that patients who developed heart failure had more frequently a history of hypertension or diabetes before transplantation. During the 12 months after transplantation, significantly more patients had moderate-to-severe acute rejections (>= grade 2R) in the heart failure group than in the control group. At the time of heart failure diagnosis, systolic left ventricular function was preserved in 50% of patients and coronary angiography was normal or near normal in 36% of patients. Half of the 44 patients in the heart failure group died within 2 years of heart failure diagnosis. Ascites and end-stage renal failure requiring dialysis were significantly more frequent during follow-up in the heart failure group than in the control group (respectively, 10/44 vs 0/44 [P=0.001] and 18/44 vs 5/44 [P=0.003]). Conclusion. - In heart transplant recipients presenting with heart failure, systolic left ventricular function is frequently preserved and coronary angiography is frequently abnormal, but may be normal or near normal. During follow-up, the main features of these patients are a high mortality rate after heart failure diagnosis, a frequent need for renal dialysis and frequent ascites. (C) 2016 Elsevier Masson SAS. All rights reserved.

Background Scimitar syndrome may be corrected using different techniques. Repair using an extracardiac conduit has rarely been performed. This study assessed the intermediate-term outcomes of this technique in adults. Methods From January 2000 to June 2011, 7 adult patients underwent correction with a ringed polytetrafluoroethylene conduit used to connect the scimitar vein (SV) to the left atrium, posterior to the inferior vena cava (IVC). Preoperative and perioperative data were reviewed retrospectively. Results All patients (32 ± 10.6 years old) were symptomatic (3 patients were New York Heart Association [NYHA] functional class II, 4 had recurrent pneumonia), with a ratio of pulmonary to systemic blood flow greater than 2, without significant pulmonary hypertension. In all patients, the SV drained the entire right lung venous return to the IVC below the diaphragm. Surgical repair was performed by sternotomy, normothermic cardiopulmonary bypass, and aortic cross-clamping. Four patients required additional closure of an atrial septal defect. Mean conduit diameter was 14 mm (range, 12 to 16 mm). Patients received long-term platelet suppression therapy with aspirin. There were no deaths and no reoperations. Mean follow-up time was 9.1 ± 3.6 years. Postoperative morbidity was nil. No evidence of subclinical stroke or embolization was found in postoperative magnetic resonance imaging of the brain. No thrombi on the prosthesis or in the left atria were detected at the latest echocardiogram, with laminar flow from the SV to the left atrium. At last follow-up, all patients were in NYHA class I. Conclusions Correction of scimitar syndrome with an extracardiac conduit can be easily and safely performed in adults, with excellent intermediate-term durability, without graft thrombi or stenosis. This technique avoids deep hypothermic circulatory arrest when the SV is short or enters the IVC in an unusually caudad location.

Pulmonary valve replacement (PVR) often is performed in adults with repaired tetralogy of Fallot (TOF). For patients who have tricuspid regurgitation (TR), tricuspid valve (TV) repair associated to PVR is still debated.We sought to evaluate perioperative factors related to TV repair when performed at the time of PVR in patients with repaired TOF.We retrospectively reviewed 104 patients with repaired TOF (or its equivalent) who underwent PVR (2002-2014).Mean age at initial complete correction and at PVR was 20.1 ± 17.2 months and 26.3 ± 9.5 years, respectively. Forty-one patients had significant preoperative TR: 24 were moderate (group M) and 17 were severe (group S). A total of 16 TV repair were performed (8 for each group). Moderate and severe tricuspid regurgitation observed in the first year following the initial complete repair were significantly associated with severe TR at PVR (P .001). In group M patients, TR was improved regardless of TV repair, whereas, in group S, residual significant TR was reported in 7 patients who did not have TV repair. No cases were observed for patients who underwent concomitant TV repair (P = .002). Among these patients with residual significant TR, 2 needed a tricuspid valve replacement. The functional status (New York Heart Association classification) of group S patients was significantly improved by concomitant TV repair.In adults with repaired TOF, TV repair is a safe procedure when performed at the time of PVR. If, at mid-term follow-up, there is probably no benefit of TV repair when preoperative TR is moderate, TV repair may improve both tricuspid valve function and functional status of the patients in case of severe preoperative TR.

Percutaneous closure has evolved to become the first-line treatment strategy for most cases of secundum atrial septal defect (ASD) in both adults and children. Its safety and efficacy have been proved; percutaneous ASD occlusion offers many advantages over surgical closure, including avoidance of cardiopulmonary bypass, avoidance of sternotomy scar, shorter hospitalization, and a potentially lower incidence of postprocedural complications. Periprocedural course and short-term outcome have been widely described, with low mortality and morbidity rates. However, the wide use of ASD closure devices and the growing experience worldwide brought some delayed and rare complications to light. Device thrombosis and cardiac erosion are the most severe late complications of device closure, whereas atrial arrhythmias are the most common. Other delayed complications include nickel allergy, cardiac conduction abnormalities, valvular damage, and device endocarditis. The long-term complication rate is not null and, although rare, some of these complications may be sudden and potentially life-threatening. Moreover, the occurrence and rate of these complications vary with the different devices used currently or in the past. Therefore, both operators and patients need to be aware of these issues to assist them in the choice of intervention or device, or both, and to adapt follow-up modalities. In this review, we sought to describe the type, incidence, and outcome of these rare but potentially serious device closure delayed complications.

ObjectiveThe study objective was to report the long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals.MethodsSince 1991, 20 patients with profound pulmonary artery hypoplasia (mean Nakata index 26 ± 14 mm2/m2) have undergone a medico-surgical strategy of native pulmonary artery rehabilitation to achieve complete repair with satisfactory hemodynamics (right ventricle to aortic pressure ratio

Background Considering more recently proposed techniques, we have evaluated our midterm and long-term results of Rastelli one-patch repair in complete atrioventricular canal. Methods Between 1984 and 2005, 107 patients with a complete atrioventricular canal underwent a Rastelli one-patch procedure. Two groups were identified: 1984 to 1995 and 1995 to 2005 (respectively, 56 and 51 patients). Mean age at surgery was 5.3 ± 3.4 months; mean weight was 5.5 ± 3 kg; trisomy 21 was present in 81 patients; complete atrioventricular canal type A was found in 67 patients, type C in 40 patients. There were 12 cases of potentially parachute mitral valve and 14 associated anomalies treated simultaneously (pulmonary obstruction 11, coarctation 3). The coronary sinus was always left on the right side. After functional and anatomic evaluation, the cleft was closed completely in 8 and partially in 29, and was left intact in 70 cases. Results Early survival was 86% ± 3%. Five patients underwent early reoperation for residual ventricular septal defect (n = 2) and mitral valve repair (n = 3). Nine patients underwent late reoperations with successful repair: subaortic stenosis (n = 4) and mitral valve repair (n = 5). Late survival at 10 and 15 years was 84% ± 3%. Freedom from reoperation for mitral regurgitation was 94% ± 3% at 10 years, and 91% ± 3% at 15 and 20 years. At last follow-up 30 patients had mild and 3 had moderate mitral regurgitation. Conclusions Rastelli single-patch repair in complete atrioventricular canal is a safe and reproducible technique. Among survivors, freedom from late reoperation for mitral regurgitation is very satisfactory. A properly taught, learned, and transmitted Rastelli one-patch technique compares very well with any other proposed technique.

a Department of Cardiology, La Timone Hospital, Aix-Marseille Universite, Marseille, France b Department of Cardiac Surgery, La Timone Hospital, Aix-Marseille Universite, Marseille, France c Department of Pathology, La Timone Hospital, Aix-Marseille Universite, Marseille, France d Department of Medical Genetics, La Timone Hospital, Aix Marseille Universite, INSERM, GMGF UMR S 910, 13385 Marseille, France

OBJECTIVES We sought to highlight the clinical, morphologic, and pathogenetic features in patients with a cleft mitral valve (MV). BACKGROUND Few studies have addressed the morphologic features of cleft MV and the outcome of these patients. The pathogenetic features, including the developmental relation to an atrioventricular (AV) septal defect, remain unclear. METHODS We reviewed the patients with cleft MV that were diagnosed by echocardiography since 1980. Patients with an AV canal, ventriculo-arterial discordance, and hypoplastic ventricles were excluded. RESULTS Twenty-two patients were identified at a median age of 0.5 years (range 0 to 10.6). In three patients, no chordal attachments of the cleft to the ventricular septum were seen. Ten patients had significant mitral regurgitation (MR), and three had subaortic obstruction by the cleft. Associated cardiac lesions and extracardiac features were present in 13 and 10 patients, respectively. During the median follow-up period of 1.5 years (range 0 to 11.8), two patients died of extracardiac causes, and one neonate died of severe subaortic obstruction. Surgical repair was performed in 10 patients at a median age of 5.2 years (range 1.3 to 10.6). Multivariate analysis showed no predictors for MV surgery. One patient was re-operated for mitral stenosis associated with aortic valve stenosis. Follow-up echocardiography demonstrated moderate MR in two unoperated patients and moderate MV stenosis in two operated patients. CONCLUSIONS A cleft of the MV comprises a wide spectrum. Important morphologic differences exist with an AV septal defect, although the two lesions may be pathogenetically related. Surgical repair always seems possible. Long-term echocardiographic follow-up is warranted.

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

In non-transplant patients mild hyperhomocysteinemia is an independent risk factor for vascular disease. The aim of this study was to determine whether hyperhomocysteinemia is associated with graft vascular disease. Fasting total plasma homocysteine was assessed in 18 patients with graft vasculopathy and 18 transplanted patients without graft vasculopathy matched for age, sex and the time since transplant. All were on cyclosporin. Graft vasculopathy was defined at coronary angiography as stenoses ]25%, or aneurysms. We found that hyperhomocysteinemia (] 15 mmol:l) is common among transplanted heart recipients and significantly more frequent in the patients with graft vasculopathy (17:18 versus 11:18). Accordingly, the mean homocysteinemia was significantly higher in the group with graft vasculopathy (23.69 7.8 versus 16.99 7.1 mmol:l, P 0.01). The elevation of homocysteine plasma levels in the heart transplant recipients has probably multiple causes. The main cause seems to be renal failure. Additional causes could be azathioprine treatment or genetic polymorphisms. These results suggest that besides the immunological factors, homocysteine can play an additional role in the pathogenesis of graft vascular disease. © 1998 Elsevier Science Ireland Ltd. All rights reserved.

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.

We describe a patient in whom we found dual orifices in a straddling and overriding tricuspid valve, with two normally sized ventricles and a cleft in the mitral valve. The patient underwent successful surgical repair. We discuss the concept of “double-orifice right atrium”, as well as the need to differentiate the isolated cleft of the morphologically mitral valve from the zone of apposition between the left ventricular components of the bridging leaflets seen in the setting of atrioventricular septal defect with common atrioventricular junction. We emphasise the unusual association of these abnormalities of the right and left atrioventricular valves in patients with separate atrioventricular junctions.

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.

A three-year-old boy was referred for persistent arterial duct. Transthoracic echocardiography showed a right aortic arch and an unusual Doppler flow in the arch vessels and the pulmonary artery. The tomodensitometry showed a right-sided aortic arch, with successive origin of the right common carotid, the right subclavian artery, and an aberrant (lusoria) left subclavian artery. The left common carotid took origin from the pulmonary trunk. During surgery, a fibrous cord independent from the anomaly was identified. An end-to-side anastomosis between the left carotid and the ascending aorta was done and the fibrous cord was divided. Was this fibrous cord a ductal ligament?

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.

advised. 1 There are no current guidelines for the screening of other cancers. We report 5 patients with asymptomatic renal cancer discovered among our HTR cohort. First, incidental renal cancers were found between 2006 and 2009 in 2 patients who underwent abdominal computed tomography for other purposes (Patients 1 and 2, Table 1). This led us to perform routine abdominal ultrasound imaging in all our HTRs at more than 3 years after transplant, from October 2009 to October 2010. Of 96 patients, 93 provided informed consent and underwent screening. Median time after transplantation was 12 years (range, 4 –25 years). Fifty-five patients had a history of smoking, 78 had hypertension, 8 required renal dialysis, and 43 had a creatinine clearance 60 ml/min, without dialysis. Among these 93 patients, we found renal cancer in 3 additional patients and 2 patients with an atypical renal cyst that required follow-up. The renal tumor was ablated in 5 patients and was found to be clear cell carcinoma in 2, papillary in 2, and chromophobe cell in 1 (Table 1). All were T1 stage disease. Time to transplant was 15 years or more in 4 of these patients. One of the patients with renal cancer died 10 months later from a primary lung cancer. None had renal cancer relapse, with a follow-up ranging from 8 months to 4 years. This prevalence of asymptomatic renal cancer in HTR is far higher than expected in the general population. For instance, across Europe, the incidence of renal carcinoma among men ranges from 2.09/100,000 to 15.2/100,000. 2 The relative incidence of malignancy as a cause of death in patients surviving more than 10 years was 27% in our cohort (9 of 33 deaths), an incidence not statistically different from that previously reported in International Society for Heart and Lung Transplantation registry (23% of 3952 deaths, p 0.7). 3 We think that the high prevalence of renal cancer in these patients is best explained by a long mean time from transplant resulting in prolonged exposure to usual risk factors of renal cancer such as immunosuppressive therapy, hypertension, and renal failure. 2 Finally, our study suggests that routine abdominal echography for renal cancer screening could be useful in the very long-term care of these patients. However, we need to demonstrate that the benefits of this screening outweigh harm. First, echography may result in cancer over-diagnosis if the tumor growth is too slow to cause symptoms before the patient dies of another cause. 4,5 Second, this screening may lead to unnecessary and dangerous surgical interventions or biopsies. 4 Indeed, renal biopsy resulted in a hematoma in 1 patient of our series without carcinoma, prolonging hospitalization. Thus, further studies are needed to determine more accurately define the benefit/risk ratio of such screening.

Background The ideal technique for addressing partial anomalous pulmonary venous drainage into the superior vena cava (SVC), with or without sinus venosus atrial septal defect (ASD), is debated. The risk of sinus node dysfunction, systemic, or pulmonary venous channels obstruction has led to different techniques being developed. We present our experience with 45 patients operated on using a vertical transcaval approach, without atrial or cavoatrial junction incision. Methods Between 2001 and 2010, 45 patients (28 females, 17 males, with a mean age of 5 years (range, 8 months to 70 years), underwent operations using 1 patch of autologous pericardium, after vertical SVC incision anterior to the anomalous pulmonary veins: 43 had associated sinus venosus ASD, and 6 had associated left SVC. Access was through sternotomy in 19 and right posterior thoracotomy in 26. An additional right atrial incision, without crossing the cavoatrial junction, was used in 2 patients without ASD. Mean cardiopulmonary bypass time was 76 minutes. Mean cross-clamp time was 44 minutes. Results No deaths or important morbidities occurred. Mean follow-up was 4.4 years (range, 2 months to 9.3 years). All patients had regular echocardiographic examination, electrocardiogram, and midterm 24-hour Holter electrocardiogram. No new arrhythmias occurred. All patients showed unobstructed caval and pulmonary venous flow. Conclusions The vertical transcaval approach is a simple, highly reproducible technique for correction of partial anomalous pulmonary venous drainage into the SVC. It yields excellent results, with unobstructed pulmonary and systemic venous flow and without arrhythmia development. It can also be performed through a cosmetic right posterior thoracotomy.

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.

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.

Previous studies have shown that heart rate is a predictor of coronary events and cardiovascular mortality. Moreover, pathophysiological studies indicate that a relatively high heart rate has detrimental effects on the progression of coronary atherosclerosis. Heart transplant recipients frequently have sinus tachycardia related to cardiac denervation. Therefore we evaluated the prognostic importance of heart rate in heart transplant recipients.143 consecutive heart transplant recipients (age at transplantation: 48.5±13 years) with at least a 2 year survival after transplantation were studied with a 9.5 year mean follow-up (range 2-23 years). Basal heart rate was measured at rest 3 months after heart transplantation in the absence of beta-blocker therapy or acute rejection. Survival without cardiac event was compared between patients with basal heart rate < or >95/min (median) according to Kaplan-Meier method using log-rank test.Fifty-six patients had coronary irregularities or stenoses at routine biennal coronary angiography and 87 had angiographically normal coronary arteries during the follow-up. Mean basal heart rate did not significantly differ between these two groups (96.4/min vs 98.3/min p=0.34). Twenty-seven patients had a coronary event (myocardial infarction, percutaneous coronary intervention or coronary death). Survival without coronary event did not significantly differ between patients with basal heart rate > or < 95/min (logrank p= 0,97).ConclusionThis series does not support a prognostic influence of heart rate for cardiac allograft vasculopathy or coronary events in heart transplant recipients.

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.

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.

Background/Objective: Secondary subaortic stenosis (SSS) can occur after surgery for various congenital heart defects with or without initial left ventricular outflow tract obstruction (LVOTO). The objective of this study was to highlight the anatomical lesions and surgical procedures associated with the development of SSS after surgery on defects without initial LVOTO. Methods: A retrospective study of 4710 patients was performed (1984—2005). The criterion for inclusion was a fixed subaortic obstruction requiring surgery, after an open- or closed-heart operation. The criterion for exclusion was an LVOTO at the time of the first operation. Results: Twenty-eight patients were studied. The mean age at initial surgery was 32 months (4 days—47 years; median: 2 months). SSS occurred after three main types of surgery: repair of coarctation of the aorta, repair of AVSD and LV—aorta rerouting for double outlet right ventricle or transposition of great arteries. The mean delay of occurrence was 4.4 years (2 months—19 years). Frequently associated initial anatomical conditions were coarctation of the aorta (40%), lesions of the mitral valve (32%),bicuspidaortic valve (21%) and left superiorvena cava (LSVC) (14%). Preoperative anatomical lesions of the LVOTwere present in 93% of the cases. After the initial operation, only one patient had a mean echo-Doppler pressure gradient across the LVOT > 20 mmHg. SSS was most frequently a subaortic membrane (n = 23). The mean pressure gradient across SSS at the time of reoperation was 47 29 mmHg. Five patients developed a second SSS after 7.4 years (mean). One patient developed a third SSS. No patient died. When compared with patients without SSS, significant risk factors for SSS were low age at surgery (32 vs 74.9 months, p < 10 4 ), pre-existing coarctation of the aorta (40 vs 10%, p < 10 4 ), bicuspid aortic valve (21 vs 6%, p = 0.002) and LSVC (14 vs 4%, p = 0.02). Conclusions: SSS development is multifactorial, depending on initial anatomical lesions and initial surgery. Low age at initial surgery, coarctation of the aorta, bicuspid aortic valve and LSVC significantly increase the risk of SSS. These elements warrant long-term follow-up for early detection of SSS. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

In December 1992, cardiac transplantation (for right heart failure with partially occluded pulmonary arteries but with a normal left ventricle) was accomplished with partial resection of right atrium and ventricle but with preservation of the left heart, obtaining a three-ventricle heart. The short-term result (27 months) is good, with normal native and grafted heart rhythms and functions, allowing us to conceive of the possible application of this procedure in some other selected cases.

Until 1984, cystic fibrosis (CF) was considered as a contra-indication to lung transplantation for the following reasons: 1) The importance of the bronchopulmonary sepsis with polyresistant organisms inducing an increased post-operative risk. 2) The persistence of the native trachea and infected sinuses with a potential for mid-term additional septic risk in immunosuppressed patients. 3) The systemic aspect of the disease, with continuous pancreatic and hepatic evolving disease. 4) The unknown risk of constitution of the disease on the transplanted lung.

Background . In patients with abnormal ventriculo-arterial connections, a mitral valve cleft different from an atrioventricular canal is occasionally associated. It may cause outflow obstruction, mitral regurgitation, and complicate biventricular repair. Methods . A retrospective review identified 21 patients operated upon with mitral valve cleft, abnormal ventriculo-arterial connections, and two well-developed ventricles. Eight patients had a ventricular outflow obstruction due to the mitral valve, whereas 2 had more than mild mitral regurgitation. One patient required initial mitral valve surgery. Eleven patients underwent biventricular repair, associated with mitral valve repair in 2 cases: arterial switch operation (n = 4), Senning operation (n = 3) associated with an arterial switch operation in one case, intraventricular repair (n = 3), and Rastelli-type extracardiac conduit repair (n = 1). Single-ventricle palliation was preferred in 10 patients with major mitral valve straddling (n = 5), outflow tract obstruction (n = 2), and noncommitted or multiple VSDs (n = 3). Results . There were three hospital deaths, two of which occurred after biventricular repair and one after an early reoperation after a bidirectional cavopulmonary anastomosis. Postoperatively after biventricular repair, 1 patient required permanent pacemaker implantation and 3 patients were reoperated on for subaortic stenosis (n = 1) and mitral regurgitation (n = 2), with one late death. By multivariate analysis, patients with a double-outlet right ventricle were at greater risk of death ( p = 0.04). After a mean follow-up period of 60.7 months (± 68.6 months), 16 patients are in New York Heart Association (NYHA) class I. One patient with a moderate mitral regurgitation on Doppler study is in NYHA class II. Conclusions . The surgical management remains controversial in patients with abnormal ventriculo-arterial connections and mitral valve cleft. Biventricular repair may not always be feasible, especially in cases of complex intracardiac anatomy associated with mitral valve straddling. Single-ventricle palliation can be achieved in these patients, although it is unknown whether the long-term results are as good as those obtained with biventricular repair.

The simultaneous existence of double orifice right and left atrioventricular valves in the absence of ostium primum defects is rare and scarcely reported. We present the case of a 20-month old boy diagnosed with tetralogy of Fallot with pulmonary atresia who was found to have associated double-orifice mitral and tricuspid valves.

Objective: Among 63 patients with pulmonary atresia and ventricular septal defect (VSD), 10 patients with extreme hypoplasia of the pulmonary arteries (PA) (mean Nakata index 20.6 mm 2 /m 2 ), but with confluent arteries and a diminutive main PA, and major aorto‐ pulmonary collaterals (MAPCAS), have been submitted to a ‘rehabilitation’ of the PA with several stages: (i) connection between RV and PAs, (ii) interventional catheterizations, (iii) complete correction with or without unifocalisation. We report here the results of this approach. Methods: The RV‐PA connection was direct (nine cases) or with an homograft conduit (one case), done under normothermic cardiopulmonary by-pass in patients aged 4.9 months (range 0.1‐18 months). Subsequently, six underwent interventional catheterizations (dilations and stents in the PA, MAPCAS occlusion by coils). Complete correction was done in seven patients (mean age 30 months, range 8‐ 49). One patient is awaiting correction. Results: One patient died after the first stage. All patients having had the third stage had a satisfactory development of the PA, had a complete closure of the VSD and a satisfactory reconstruction of the PA bifurcation. There was one death of severe pulmonary infection 6 months after repair. All other patients have been followed by catheterization and/or echocardiograms. With a follow-up of 83 ^ 65 months, all patients are improved, 50% have no cardiac medications, none has residual shunt, RV/LV pressure ratio is 0.6 (range 0.3‐1). Conclusions: The strategy of ‘rehabilitation’ of PA allowing: (i) antegrade flow in the PA, (ii) interventional catheterizations, (iii) growth of the PA with possible angiogenesis, (iv) complete correction, is a logical approach to be undertaken in the young patient and is a valid alternative to strategies relying more on MAPCAS for pulmonary vascular supply. The therapeutic sequences depend upon the individual anatomy. q 2001 Elsevier Science B.V. All rights reserved.

We describe in this article a new approach in transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO). It consists of an operation combining the intracardiac infundibular resection of the Lecompte procedure to allow a direct VSD-aorta connection and the right ventricle-pulmonary artery (RV-PA) connection with a segment of tubular aortic autograft, (with or without an additional monocusp patch). Thus, a viable conduit, capable of growth, is interposed between RV and PA. We have performed this procedure in 19 patients (age, 2 months to 11 years), 6 being under 1 year. All patients are doing well after a maximum follow-up of 72 months, exhibiting growth of the conduit and satisfactory RV pressures. This new approach allows primary correction even in the young and can be extended, we think, for other defects with pulmonary stenosis or atresia. It shares, however, with many other procedures, the valveless nature of the repair with the potential late need for valve replacement surgery. Copyright 2000 by W.B. Saunders Company

International audience; Objective: Systemic embolism is a serious complication after classical orthotopic transplantation, presumably originating from enlarged left atrium. We speci®cally studied this problem after classical and modi®ed bicaval transplantation. Methods: Between December 1985 and March 1999 we consecutively performed 72 classical and 106 modi®ed heart transplantation. Modi®cation included bicaval anastomosis and recipient left atrium maximal reduction. Mean age was 47 years. All the patients received an antiplatelet therapy and were routinely followed. When clinical signs of systemic embolism were present, a neurological evaluation and transesophageal echocardiography were done. Sixty matched patients (30 of each group) had comparative transesophageal echocardiography study, at least 6 months after transplantation. Results: Perioperative mortality was 17.4%. Mean follow-up was 6.8 2 1 /47 years. All patients were in sinus rhythm. Among 147 survivors, 11 patients who underwent classical transplantation had a systemic embolism, 1 month to 12 years after transplantation, 15.3%, (11/72). Two limb ischemia and one mesenteric ischemia (needing surgery), seven strokes (one death, two permanent neurological de®cit). There was no systemic embolism in the modi®ed technique group (P ˆ 0:013). Left atrial comparative transesophageal echocardiography study showed a larger left atrial surface in classical transplantation. 33 ^ 4 cm 2 versus 20 ^ 3 cm 2 in a modi®ed technique, P ˆ 0:01. Spontaneous echo contrast was present in 56% of classical technique group associated with atrial thrombosis in nine patients, there were no atrial thrombosis in modi®ed technique group and spontaneous echocontrast was present in 0.5% (P ˆ ,0:001). Conclusion: The occurrence of systemic embolism, left atrial spontaneous echocontrast and thrombosis when using classical technique, and the absence of these complications with the bicaval technique justi®ed the use of this method. Our experience with atrial thrombosis and spontaneous echocontrast rises the question of anticoagulation in classical transplantation. q

Background: In most cases of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, a Lecompte procedure (reparation a l'etage ventriculaire) is possible without interposition of a conduit between the right ventricle and pulmonary artery. However, the anterior location of the pulmonary arteries after the Lecompte maneuver may be a potential cause for right ventricular outflow obstruction, which continues to be reported in 5% to 25% of cases. We have used a tubular segment of aortic autograft to connect the pulmonary artery, left in the orthotopic posterior position (without the Lecompte maneuver), to the right ventricle in 10 consecutive patients with transposition, ventricular septal defect, and left ventricular outflow tract obstruction. Methods: Ten consecutive patients aged 2 months to 11 years (mean 32 months) have undergone a modified Lecompte operation. Eight had severe pulmonary stenosis, two had pulmonary atresia, and four had a restrictive ventricular septal defect at the time of the operation. Two had multiple ventricular septal defects. Seven had undergone one ( n = 5) or two ( n = 2) previous modified Blalock-Taussig shunts. All patients underwent a total correction with left ventricular–aortic intraventricular connection (four needed a ventricular septal defect enlargement), connection between the right ventricle and pulmonary arteries with a tubular segment of autograft aorta, without the Lecompte maneuver (anterior location of the bifurcation of the pulmonary arteries) on the right ( n = 6) or the left ( n = 4) of the aorta. No valvular device was used for the right ventricular outflow repair. Results: No early or late deaths occurred. One patient with multiple ventricular septal defects needed an early (2 weeks) reoperation for a residual muscular ventricular septal defect. All patients are currently in New York Heart Association class I, without medications, in sinus rhythm, at a mean follow-up of 30 months. Late results up to 3.6 years show no calcification on the chest roentgenogram, and at the most recent echocardiogram, right ventricular pressures were low (25 to 40 mm Hg, mean 33 mm Hg) and no significant gradient (over 10 mm Hg) was found between the right ventricle and pulmonary arteries. Left and right ventricular function was satisfactory. Conclusion: This modification of the Lecompte operation using a segment of autograft allows an excellent early and late result, with no danger of compression of anteriorly placed pulmonary arteries, no significant right ventricular outflow obstruction, and normal appearance of the tubular autograft. In view of laboratory and clinical evidence, normal growth of the autograft can be anticipated. It allows an elective correction of transposition, ventricular septal defect, and left ventricular outflow tract obstruction without a previous Blalock-Taussig shunt (three patients) and correction at a young age (three patients younger than 1 year). (J Thorac Cardiovasc Surg 1997;114:746-54)

Several data confirm that a high heart rate is associated with various complications of heart transplantation that may impair the prognosis. However, we still need evidence that heart rate, per se, influences graft vasculopathy.