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Summary Background The main difference between extreme tetralogy of Fallot (TOF) and pulmonary atresia with ventricle septal defect (PA/VSD) is anterograde pulmonary blood flow (APBF). It is speculated that the association of modified Blalock–Taussig shunt (mBTS) with APBF favours shunt thrombosis, but promotes better pulmonary artery growth. Aim To compare pulmonary artery growth after mBTS between TOF and PA/VSD. Methods From 1995 to 2018, 77 mBTS procedures were performed in infants (aged Results After matching, the preoperative Nakata index was similar in the two groups (TOF 101 ± 34 vs. PA/VSD 106 ± 35 mm2/m2; P = 0.75). Age and weight were similar (TOF 24 ± 20 days, 3.3 ± 0.6 kg vs. PA/VSD 24 ± 33 days, 3.3 ± 0.9 kg; P = 0.84 and P = 0.77, respectively). There was no difference in rates of in-hospital mortality (TOF 0% vs. PA/VSD 10%; P = 0.13) or mBTS thrombosis (TOF 15% vs. PA/VSD 10%; P = 0.63). The left and right pulmonary artery diameters at time of biventricular repair were similar (TOF 7.5 ± 2.2 and 6.7 ± 2.1 vs. PA/VSD 8 ± 2.7 and 7.1 ± 2.5 mm; P = 0.43 and P = 0.78, respectively), as were delta Nakata (TOF 112 ± 102 vs. PA/VSD 107 ± 66 mm2/m2; P = 0.89), median age for biventricular repair (P = 0.83) and reintervention rates (TOF 10% vs. PA/VSD 15%; P = 0.67). Conclusions We found no difference in pulmonary artery growth between APBF with mBTS versus mBTS alone. Thus, we could not show an increase in mBTS thrombosis with APBF.

Objective The difference between extreme Tetralogy of Fallot (T4F) and pulmonary atresia with ventricle septal defect (PA/VSD) is the anterograde pulmonary blood flow. It is speculated that the association of modified Blalock-Taussig shunt (mBTs) and additional pulmonary blood flow favours shunt thrombosis but promotes better pulmonary arterial (PA) growth. This study sought to compare (PA) growth after mBTs shunt between T4F and AP/VSD. Methods From 1995 to 2018, 79 mBTs were performed in infants ( Results After matching, the preoperative Nakata was similar (101 ± 8 mm2/m2in T4F; 106 ± 8 in AP/VSD P = 0.75). The age and weight were similar (24,3 ± 5 days, 3.3 ± 0.5 kg in T4F; 24,15 ± 4, 3.3 ± 0.9 in AP/VSD P = 0.84 and P = 0.77 respectively). The mBTs size was similar (4.15 ± 0.5 mm in T4F; 4.3 ± 0.5 in PA/VSD P = 0.35). There was no difference in in-hospital mortality (n = 0, in T4F; n = 2, 11% in AP/VSD, P = 0.14) and mBTs thrombosis (3,16% in T4F; 2,11% in AP/VSD, P = 0.18). The time to extubation tended to be longer in T4F (5 ± 1days vs. 2 ± 1 P = 0.06). The left and right PA diameter at time of biventricular repair were similar (7.5 ± 0.5 mm, 7 ± 0.2 in T4F; 8.1 ± 0.7 mm, 7 ± 1 in AP/VSD P = 0.43 and P = 0.78, Fig. 1 ) and the Nakata delta (112 ± 23 mm2/m2in T4F; 110 ± 17 in AP/VSD P = 0.78). Median time to complete repair was the same in the PA/VSD (12.26 [3.9-25] months) compared with T4F (9.7 [6.2–41.1] months) P = 0.87). The interstage reintervention were similar (3.16% in T4F; 4.22% in AP/VSD, P = 0.9). Conclusions Anterograde blood flow with mBTs did not increase the risk of mBTs thrombosis. We could not show benefit of anterograde blood flow with mBTS versus mBTS for pulmonary arteries growth. Anterograde blood flow did increase the time to extubation, probably by increasing total pulmonary blood flow.

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

Repair of the complete atrioventricular septal defect, as with many other congenital heart defects, has changed with time from the one-patch technique (Rastelli) to the two-patch technique and then to the “modified” onepatch technique. In this review, we have reported our experience with one-patch Rastelli type correction in babies under one-years of age. Our experience confirmed that the classical one-patch Rastelli technique is a safe, reproducible, teachable technique giving satisfactory late results. Diger bircok dogustan kalp defektlerinde oldugu gibi, komplet atriyoventrikuler septal defeklerin tamiri de zaman icinde tek yama tekniginden (Rastelli) cift yama teknigine ve daha sonra “modifiye” tek yama teknigine degisim sergilemistir. Bu incelemede Rastelli tipi tamir

Objective Abnormal origin of coronary artery from the pulmonary artery (ACAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. Immediate surgical correction can provide excellent results. This study aimed to determine the surgical outcome of ACAPA. Methods From 1993 to 2016, 31 consecutive patients underwent coronary re-implantation. Concomitant mitral valvuloplasty was performed in two. The study cohort was divided into two groups according to age (group 1: ≤ 6 months and group 2: > 6 months). Data from demographic characteristics, electrocardiography, echocardiography, surgery, intensive care unit stay and follow-up were evaluated. Results The study included 29 patients with anomalous left coronary artery from the pulmonary artery and 2 with anomalous right coronary artery from the pulmonary artery. The median age at repair was 4.75 [2.3–16.3] months and the median weight was 6.2 [4.3–9.4] kg. Preoperative echocardiography showed severe LV dysfunction in 56.7% of patients. The median preoperative LVEF was 33.5% [20–55]. Mitral regurgitation (MR) was moderate to severe in 13 patients (41.9%). Requirement to extracorporeal membrane oxygenation (ECMO) support systems was necessary in 2 cases (6.4%) before surgery and 5 cases (16.1%) after correction. Postoperative mortality rate was 9.7%. Younger age ( Conclusions Young age is significantly associated with a more severe presentation, more ECMO requirement and mortality. Lesser development of coronary collateral circulation can explain these findings. After establishment of a two-coronary circulation both ventricular function and MR tend to be normalized over time. This ‘recovery’ usually occurs within 6 months but may require more time (maximum of thirty months in our population).

Objective: This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). Methods: The records of all (n = 56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. Results: A wide range of ASD sizes (5-34 mm) and devices of various types and sizes (range 12-60 mm) were involved, including 13 devices less than 20 mm. Complications leading to surgery included embolisation (n = 29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n = 12), significant residual shunt (n = 12), aortic or atrial perforation or erosion (n = 9), haemopericardium with tamponade (n = 5), aortic or mitral valve injury (n = 2) and endocarditis (n = 1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% ( p = 0.001). Conclusions: Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory. (C) 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

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.

Background Surgical treatment of active aortic infective endocarditis is challenging, and the type of prosthesis to be implanted during the active phase remains controversial. Methods All consecutive patients with definite diagnosis of aortic infective endocarditis operated on during the active phase were included. Endpoints were in-hospital mortality and a combined endpoint including infective endocarditis recurrence, prostheses dysfunction, or long-term cardiovascular mortality. Results Among 127 consecutive patients, mean age 57 ± 15 years, 87% male, 30% with preexisting aortic prosthesis, and 63 (50%) with annulus abscess, 54 (43%) were treated with aortic homograft and 73 (57%) with conventional prosthesis. Median time between diagnosis and surgery was 10 days. In-hospital mortality was 9%, not different between homograft and conventional prostheses (11% versus 8%, p[ = 0.6). By multivariable analysis, prosthetic valve endocarditis (8.5 95% confidence interval: 2.2 to 33.6, ]p = 0.001) was the only variable independently associated with in-hospital mortality, which was not influenced by type valvular substitute ( p = 0.6), even in the subset with annulus abscess ( p = 0.2). Ten-year survival free from the combined endpoint was 44% ± 10%, not different between homograft and conventional prostheses (log rank p = 0.2). By multivariable analysis, comorbidity index (2.6 [1.05 to 6.3], p = 0.04) and prosthetic valve endocarditis (2.3 [1.2 to 4.6], p = 0.02) were independently predictive of the combined endpoint, which was not determined by type of valvular substitute ( p = 0.6) even in the subset with annulus abscess ( p = 0.5). Conclusions Implantation of conventional prostheses during the active phase of aortic endocarditis yields similar low operative mortality and long-term prognosis as compared with aortic homografts, even in patients with annulus abscess.

We evaluated the results of using extracorporeal membrane oxygenation (ECMO) as resuscitation for cardiac patients undergoing cardiopulmonary resuscitation (CPR) in our setting where neither perfusionists nor surgeons are always on site, and no circuit may be ready. Between 2003 and 2006, we used ECMO for all cardiac patients who underwent cardiac arrest in the pediatric intensive care unit (PICU) or Cath Laboratory. We reviewed retrospectively 14 consecutive files (15 episodes). Mean CPR time before ECMO institution was 44 minutes (10-110 minutes). The surgeons, perfusionist, and scrub nurse, not on site for three of these patients, had to be called in simultaneously with institution of CPR. Two died on ECMO, the third one was successfully transplanted after 5 days. Globally, 10 patients could be weaned (66%). Eight patients (57%) survived to hospital discharge, seven without obvious neurological damage. One patient was bridged to a left ventricular assist device (LVAD) and was eventually successfully transplanted. He had an ischemic brain lesion with good recuperation and no sequel. We obtained good results with resuscitation ECMO in our setting where a permanently on-site rapid deployment ECMO team is not present at all times.

Aims Aetiological investigations of pericardial effusion are often negative. We evaluate the interest of systematic analysis of fluid in order to diminish the number of pericarditis classified as idiopathic. Methods and results We performed a systematic analysis of pericardial fluid and biopsy specimens, using cultures and molecular analyses for the identification of bacteriological, fungal, and viral agents, as well as histopathological examination of 106 pericardial fluid samples. The aetiological diagnosis was determined clinically and by non-invasive procedures in 40 and nine patients, respectively. In the remaining 57 patients, 14 neoplasias and 17 infections were diagnosed. Molecular procedures identified seven viral (Enterovirus, Herpes simplex virus, and Epstein–Barr virus in four, two, and one of the cases, respectively), one fungal (Cryptococcus neoformans), and nine bacterial infections. Four of these bacteria were not diagnosed by culture because of prior antibiotics treatment (Mycobacterium tuberculosis in two cases, Streptococcus pneumoniae in one case, and Actinomyces neuii in one case). The aetiology remained undetermined in 26 patients. Conclusion The systematic use of molecular techniques permitted a significant increase in aetiological diagnoses of punctured pericardial effusions when compared with cultures (39.5 vs. 13.9%, respectively; P , 0.01). It is particularly beneficial for patients with a previous antibiotic regimen or suspicion of tuberculosis.

We retrospectively reviewed the files of 19 extracorporeal life support (ECLS) applications performed after cardiac surgery in 15 patients from January 2002 to December 2004. We placed 16 arteriovenous ECLS applications with oxygenator, 2 venovenous ECLS applications with oxygenator, and 1 biventricular ECLS application without oxygenator (graft dysfunction after heart transplant). Mean age was 4.9 +/- 7 years (median 5.9 months, range 11 days to 21 years). All patients underwent surgery for congenital heart disease, except for one patient who had a heart transplant. Indications were hemodynamic failure in 12 cases, respiratory failure in 5 cases, and mixed failure in 2 cases. Four patients were undergoing cardiopulmonary resuscitation during ECLS placement (no deaths). Mean delay between surgery and ECLS placement was 3.2 +/- 3.4 days (median 2 days). Mean ECLS duration was 3.4 +/- 5.8 days (mean 6 days, range 3-16 days). Three patients had further surgery for residual lesions. Thirteen patients (86.7%) survived to ECLS weaning; 12 patients survived to hospital discharge (80%). No survivor presented obvious neurologic damage. Specific morbidity included reentry for bleeding, multiple transfusions, and mediastinitis. These results support early placement of ECLS in children whenever a severe postoperative hemodynamic or respiratory failure, refractory to medical treatment, is present.

Background— The incidence of embolic events (EE) and death is still high in patients with infective endocarditis (IE), and data about predictors of these 2 major complications are conflicting. Moreover, the exact role of echocardiography in risk stratification is not well defined. Methods and Results— In a multicenter prospective European study, including 384 consecutive patients (aged 57±17 years) with definite IE according to Duke University criteria, we tested clinical, microbiological, and echocardiographic data as potential predictors of EE and 1-year mortality. Transesophageal echocardiography was performed in all patients. Embolism occurred before or after IE diagnosis (total-EE) in 131 patients (34.1%) and after initiation of antibiotic therapy (new-EE) in 28 patients (7.3%). Staphylococcus aureus and Streptococcus bovis were independently associated with total-EE, whereas vegetation length >10 mm and severe vegetation mobility were predictors of new-EE, even after adjustment for S aureus and S bovis . One-year mortality was 20.6%. In multivariable analysis, independently of the other predictors of death (age, female sex, creatinine serum >2 mg/L, moderate or severe congestive heart failure, and S aureus ) and comorbidity, vegetation length >15 mm was a predictor of 1-year mortality (adjusted relative risk=1.8; 95% CI, 1.10 to 2.82; P =0.02). Conclusions— In IE, vegetation length is a strong predictor of new-EE and mortality. In combination with clinical and microbiological findings, echocardiography may identify high-risk patients who will need a more aggressive therapeutic strategy.

Objectives: To identify the prognostic markers of a bad outcome in a large population of 104 patients with prosthetic valve endocarditis (PVE), and to study the influence of medical versus surgical strategy on outcome in PVE and thus to identify patients for whom surgery may be beneficial. Design: Multicentre study. Methods and results: Among 104 patients, 22 (21%) died in hospital. Factors associated with in-hospital death were severe co-morbidity (6% of survivors v 41% of those who died, p = 0.05), renal failure (28% v 45%, p = 0.05), moderate to severe regurgitation (22% v 54%, p = 0.006), staphylococcal infection (16% v 54%, p = 0.001), severe heart failure (22% v 64%, p = 0.001), and occurrence of any complication (60% v 90%, p = 0.05). By multivariate analysis, severe heart failure (odds ratio 5.5) and Staphylococcus aureus infection (odds ratio 6.1) were the only independent predictors of in-hospital death. Among 82 in-hospital survivors, 21 (26%) died during a 32 month follow up. A Cox proportional hazards model identified early PVE, co-morbidity, severe heart failure, staphylococcus infection, and new prosthetic dehiscence as independent predictors of long term mortality. Mortality was not significantly different between surgical and non-surgical patients (17% v 25%, respectively, not significant). However, both in-hospital and long term mortality were reduced by a surgical approach in high risk subgroups of patients with staphylococcal PVE and complicated PVE. Conclusions: Firstly, PVE not only carries a high in-hospital mortality risk but also is associated with high long term mortality and needs close follow up after the initial episode. Secondly, congestive heart failure, early PVE, staphylococcal infection, and complicated PVE are associated with a bad outcome. Thirdly, subgroups of patients could be identified for whom surgery is associated with a better outcome: patients with staphylococcal and complicated PVE. Early surgery is strongly recommended for these patients.

This non-randomized retrospective study included all patients operated on for CABG through median sternotomy between January 2000 and December 2002 by the same surgeon trained to both techniques. Using risk-adjusted comparison where expected mortality was given by the EuroSCORE value assessed pre-operatively, and studying mid-term survival and functional results we aimed to evaluate our indications for OPCAB versus conventional CABG through a consecutive series of 308 patients. Selected indications for OPCAB (n=154) were isolated LAD coronary system lesions and multivessel diseases with suitable anatomy in high surgical risk patients (EuroSCORE > or = 5). The first 154 patients operated on conventionally during the time-study interval were included in the control group. Expected mortality was significantly higher in the OPCAB group: 4.29 [95% CI: 3.83-4.77] vs. 3.54 [95% CI: 3.17-3.91] (P=0.024). Observed mortality was 1.3% and 2.6% for patients treated OPCAB and with conventional technique, respectively. Survival at three years was 91.5 and 93.8% in the conventional and OPCAB groups, respectively. Angina-free survival at three years was 95.8% and 89.6% in the conventional and OPCAB groups, respectively (P=0.04). To promote OPCAB in selected patients results in decreasing operative risk to the price of worsening late functional results.

Objectives: In the last decade, cardiac surgery in octogenarians is becoming a routinely performed procedure in our Western countries. The functional benefit of this surgery had already been proved. The aim of this study was to evaluate operative mortality, to identify pre- and postoperative risk factors of early and late mortality, to assess the Euroscore count in this high-risk group of patient and to evaluate late results of this surgery. Methods: We reviewed 215 consecutive patients with a mean age of 83G2 years having undergone valvular surgery. There were 127 female patients (57.1%) and 88 males (42.9%). One hundred and fifty-nine patients (74%) underwent aortic valve replacement 42 (19.5%) mitral surgery and 14 (6.5%) double valve surgery. There were 32 (14.9%) re-operative cases. Twenty-seven patients (12.6%) were operated on in emergency. There were 32 re-operations (14%). The EuroSCORE was used to assess predicted operative risk. Mean Euroscore additive count was 9.5G2.3 and mean logistic Euroscore was 15.1%. Results: Operative mortality was 8.8% (19 patients). Left ventricular dysfunction was the only pre-operative significant risk factors of mortality (PZ0.05). Low cardiac output (P!0.001), gastrointestinal complications (PZ0.03) and surgical reexploration (PZ0.001) were significant risk factors of mortality. Mean survival was 84% after one year and 56% after 5 years. Conclusions: Valvular surgery in octogenarians is a safe and low risk procedure compared to functional benefit and long-term survival. Our data how that logistic Euroscore overestimates the mortality in this high-risk group of patients. q 2004 Elsevier B.V. All rights reserved.

The aim of this study is to evaluate the benefit of the simultaneous use of a BioMedicus left ventricular assistance device (Medtronic, Minneapolis, MN, U.S.A.) and an intra-aortic balloon pump on regional blood flows, pressure, and pulsatility. Twelve pigs are studied. A BioMedicus pump was placed between the left atrium and the ascending aorta and an intra-aortic balloon pump was inserted through the left femoral artery. Blood flow and pressure were measured in the carotid, femoral, and coronary arteries and in the thoracic aorta below the intra-aortic balloon in the basal experimental condition with a full-flow BioMedicus pump and with a full-flow BioMedicus pump + intra-aortic balloon. The BioMedicus pump eliminates pulsatility in all sites and significantly decreases coronary and carotid blood flow. The adjunction of an intra-aortic balloon restores pulsatility to values comparable to those recorded in basal conditions. Coronary and carotid flows even increase to values higher than in the basal conditions. The simultaneous use of an intra-aortic balloon combined with the BioMedicus pump provides a pulsatile flow and increases coronary and carotid blood flows in pigs. An intra-aortic balloon can easily be combined with a BioMedicus pump whenever possible and may improve myocardial recovery in patients with postcardiotomy ventricular failure.

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.

Objective. The authors performed a multi-institutional, prospective, blind study on hearts from local donors to validate the feasibility and accuracy of the metabolic evaluation of human hearts by phosphorus-31 ( 31 P) magnetic resonance spectroscopy (MRS) before transplantation. Methods. Twenty-one hearts were separated into two groups according to a transplantation score (TS) on the basis of the current clinical and echocardiographic evaluation as follows: TS1 (n=7), grafts for United Network for Organ Sharing (UNOS) 1 patients only; and TS2 (n=14), grafts suitable for UNOS 2 patients. All hearts were cold preserved with Celsior and underwent ex situ 31 P MRS to measure ratios of various high-energy phosphate metabolites and the intracellular pH (pH i ). Results. The total duration of the MRS procedure was 32 min, thereby not unacceptably increasing the total ischemic time for the transplanted grafts. Phosphocreatine (PCr) and inorganic phosphate (P i ) was significantly different between the two groups (0.95±0.29 for TS1 and 2.05±0.74 for TS2). The difference in pH i was also significant (7.44±0.13 for TS1 and 7.64±0.19 for TS2). Conclusions. Determination of PCr-Pi and pHi as markers of ischemic injury before transplantation can be considered as an objective and accurate criterion for the decision to accept or refuse heart grafts for transplantation.

Objectives: The tight syndrome after open-heart procedures in neonates renders delayed sternal closure (DSC) a life-saving measure. The goal of this study is to analyze the risk factors that may predict the need for DSC. Methods: Between January 1991 and December 2000, 312 consecutive open-heart procedures in neonates (180 males, 132 females) were studied retrospectively. Median age was 11.9 days (range 1–30 days) and weight 3.63 kg (range 1.8–4.2 kg). The major pathologies were transposition of the great arteries (153), interruption of the aortic arch (IAA) (33), total anomalous pulmonary venous drainage (TAPVD) (24) and single ventricle (19). Two hundred and twenty-eight patients had profound hypothermia with circulatory arrest and 74 normothermic cardiopulmonary bypass (CPB), 195 had crystalloid cardioplegia and 111 blood cardioplegia. Median CBP time was 146 min (range 37–284 min) and aortic clamping 67.6 min (range 0– 164 min). Two hundred and fifty-five patients had a continuous ultrafiltration and 57 had a modified ultrafiltration. The criteria for DSC were hemodynamic instability, deterioration of the central venous saturation, metabolic status and/or high ventilatory pressures. Results: One hundred and nineteen patients had DSC (38.12%). Median CBP time was 145 min (range 37–284 min) and aortic clamping time 67.6 min (range 0–164 min). Twenty-one patients (6.7%) needed reopening in the intensive care unit (ICU) during the first 24 h. Among the studied factors, the age below 7 days (P ¼ 0:014), the diagnosis of IAA and TAPVD (P , 0:05), CBP duration over 185 min (P ¼ 0:048), clamping time over 98 min (P ¼ 0:039) and central venous saturation below 51% P ¼ 0:024) were statistically significant risk factors. All the patients who had more than 106 min of clamping, more than 196 min of cardiopulmonary bypass or less than 47% of central venous saturation were either left opened or reopened in the ICU. Conclusions: Many of the factors thought to be associated with the need for delaying the sternal closure had no statistical significance as risk factors. On the other hand, the diagnosis of IAA or TAPVD, an age less than 7 days, aortic clamping more than 98 min, CPB time more than 185 min and a post-bypass central venous saturation less than 51% were statistically significant risk factors that could be used in predicting the need for delaying the sternal closure. q 2002 Elsevier Science B.V. All rights reserved.

OBJECTIVES Total cavopulmonary connection (TCPC) performed in the second decade of life has rarely been studied. Thus, we investigated (bicentric study) early and late morbidity and mortality following completion of TCPC in these patients. METHODS From January 1999 to June 2014, 63 patients (14.5 ± 2.9 years) underwent TCPC (extracardiac conduit). Palliation before completion was an isolated bidirectional cavopulmonary shunt (BCPS) in 3 patients or BCPS associated with additional pulmonary blood flow (APBF) that was either antegrade (Group 1) in 38 (63%) or retrograde (Group 2) in 22 (37%). Preoperative and perioperative data were reviewed retrospectively. RESULTS Mean pulmonary arterial and ventricular end-diastolic pressures were 12.2 and 9.2 mmHg, respectively. Mean Nakata index was 279 ± 123 and 228 ± 87 mm 2 /m 2 in Groups 1 and 2, respectively ( P = 0.01). Aortic cross-clamping was performed in 22 from Group 1 and 8 from Group 2 ( P = 0.04). Mean follow-up was 4.57 years [0.8-15]. Nine patients had prolonged stays in the intensive care unit (>6 days). There were 1 early and 2 late deaths (non-cardiac related). Actuarial survival was 96% at 4 years. At last follow-up, single-ventricle function remained normal or improved in all patients (Group 1) compared to 82% in Group 2 ( P = 0.02). New York Heart Association (NYHA) class had improved in both groups: 47 patients were NYHA class II and 16 class III preoperatively vs 50 class I and 10 class II postoperatively ( P < 0.001). CONCLUSIONS Single-ventricle palliation with BCPS and APBF allowed completion of TCPC in the second decade of life, with encouraging mid-term results. However, BCPS with retrograde APBF was associated with single-ventricle dysfunction: thus, this technique needs to be used cautiously as long-lasting palliation.

Under a new name (the ’one-and-half patch’ technique) [1], the authors describe an intermediate technique (between those described at 1 and 2, below) for the surgical treatment of complete atrio-ventricular canal (CAVC) or atrio-ventricular septal defect (AVSD)—with a patch under the superior bridging leaflet (SBL) and direct attachment to the ventricular crest of the defect under the inferior bridging leaflet (IBL)— that is already advocated by some [2], who indicate that it can be used in cases with a "large" ventricular septal defect component. The technique was used in their centre in 16 cases, whereas others in their centre used the two-patch technique in 45 cases and the "modified" single-patch in 13 cases. They claimed a shorter cross-clamp time (66 m vs 81 m in the two-patch procedure) and fewer re-operations (0 vs 1 for two-patch and 3 for the modified technique). The mean follow-up period was three years. Despite the good results obtained, two questions arise: what are the advantages of this technique and is it of interest? Let us recall the two basic concepts in the repair of CAVC

BACKGROUND Previous reports have found an association between lymphomatoid papulosis and hypereosinophilic syndrome, as well as lymphomatoid papulosis and lymphoma. In the current study the authors report what to their knowledge is the first reported case of these three diseases occurring simultaneously in the same patient. METHODS The authors followed the clinical course of a 64-year-old man with lymphomatoid papulosis associated with severe hypereosinophilic syndrome complicated by involvement of the lungs and heart. RESULTS After 6 years of follow-up, the patient developped a large T-cell, CD30 positive lymphoma. The bone marrow biopsy was typical of hypereosinophilic syndrome associated with fibrosis, with focal lymphomatous infiltrates comprised of large cells resembling the type A cells of lymphomatoid papulosis. Complete remission of the lymphoma was obtained with chemotherapy. CONCLUSIONS This exceptional case report suggests a link between the three diseases. Lymphomatoid papulosis belongs to the spectrum of CD30 positive lymphoproliferative disorders and CD30 positive lymphocytes of lymphomatoid papulosis are known to have a Th2 profile with possible secretion of eosinopoietic cytokines. Cancer 2000;89:2138–43. © 2000 American Cancer Society.

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.

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.

We present the case of an infant with congenital heart disease which includes a partial atrioventricular canal defect with the absence of the atrial septum (common atrium) and an extremely uncommon course of the right-sided superior vena cava (SVC) including an intra-atrial segment coursing intramurally along the right posterolateral atrial wall, with an intracardiac orifice situated low within the right side of the atrium, close to the atrial orifice of the right hepatic veins. This feature of the anatomy was discovered intraoperatively at the time of surgical repair. Systemic venous anatomy also included interrupted inferior vena cava (IVC) with azygos continuation to a left-sided SVC draining directly into the left side of the atrium. The successful surgical procedure included tunneling of the left-sided SVC to the right side of the common atrium and atrial septation with a patch.

Acute inflammation in the lung is characterized by a phase of tissue injury followed by a phase of tissue repair. When the latter is excessive, fibrosis occurs. Alveolar macrophages (AM) can produce cytokines involved in both phases of acute lung inflammation, notably interleukin-6 (IL-6), involved in injury and transforming growth factor-beta (TGF-beta), mediating repair. We hypothesized that AM were activated in both phases, and studied IL-6 and TGF-beta production by AM during complications of lung transplantation, acute rejection (AR), and cytomegalovirus pneumonitis (CMVP). In addition, we analyzed these cytokines in bronchiolitis obliterans (BO), a fibrotic complication of lung transplantation linked to previous AR and CMVP. At the onset of AR and CMVP, IL-6 secretion increased, whereas AM TGF-beta content was increased, but not its secretion. In contrast, with time, IL-6 reached control value whereas TGF-beta secretion rose significantly. In BO, IL-6 was not oversecreted, but TGF-beta increased, notably before functional abnormalities occurred. These results show that during acute complications of lung transplantation, AM display an early activation with oversecretion of IL-6, which is involved in tissue injury, counterbalanced by a late activation in which TGF-beta predominates, mediating tissue repair. The results provide new insights into the pathogenesis of BO, which is linked to acute complications of lung transplantation through this biphasic AM activation.

We evaluated the prognostic value of plasma N-terminal-pro-brain natriuretic peptide (NT-pro-BNP) in 71 transplanted heart recipients during a 38 +/- 2 month follow-up. The negative predictive value of NT-pro-BNP levels/=800 pg/ml to predict death was 97% (95% confidence interval 92 to 100). Thus, NT-pro-BNP levels/=800 pg/ml predict a middle-term good survival in transplanted heart recipients.

The implant of a ventricular assist device is a standardized procedure. However, it carries a high-risk in reoperations, especially if there are patent by-pass grafts. An isolated thoracotomy has been previously described as an alternative access route in these patients, but it offers a limited exposure should intraoperative right-heart failure occur. In the following, we describe an alternative, off-pump technique that combines a median sternotomy with limited dissection of the right-heart with a left anterolateral thoracotomy. In our experience, it proves to be a safe way for the LVAD insertion in these high-risk patients.

Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate ( P= 0.006). Overall mortality was associated to malignancy ( P =0.0008), and adverse events during follow-up ( P =0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.

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.

The aim of this study was to determine the prevalence of hyperhomocysteinaemia in cardiac transplant recipients. Three groups of subjects were studied: 27 heart transplant recipients, 14 to 63 months (mean = 36.5) after transplantation; 10 patients with moderate chronic renal insufficiency without clinical evidence of vascular disease; 17 apparently healthy individuals. Twenty-five out of 27 transplanted patients had a coronaroangiography within 6 months of homocysteine measurement. Plasma homocysteine was measured both while the subject was fasting (t0) and 6 h after administration of 0.1 g.kg-1 of methionine (t6). Hyperhomocysteinaemia was present in 14/27 fasting transplanted patients and after methionine loading. Mean plasma levels of homocysteine at t0 were higher (P = 0.03) in transplanted heart recipients (15.4 +/- 7 mumol.l-1) than in the renal patients (9.9 +/- 5 mumol.l-1) despite similar mean plasma creatinin. In eight transplanted patients with angiographic coronary abnormalities of the cardiac graft, homocysteinaemia was at t0 17.1 +/- 9 mumol.l-1 and at t6 47.8 +/- 25 mumol.l-1. In 17 transplanted patients with angiographically normal coronary arteries, plasma homocysteine levels were at t0, 13.2 +/- 4 mumol.l-1 and at t6, 46.8 +/- 25 mumol.l-1. We conclude that hyperhomocysteinaemia is common in transplanted heart recipients, and partly related to renal insufficiency. No correlation was found between hyperhomocysteinaemia and angiographic evidence of coronary atherosclerosis of the graft, but the population of the study was possibly too small to establish this correlation.

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.

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.

In the last 3 1/2 years, we have performed 20 double-lung transplantations in children between 7 and 16 years old (mean age, 13 years). One patient had primitive bronchiolitis obliterans and the other 19, cystic fibrosis. Eight patients were operated on in an emergency situation, 7 of them requiring ventilator support before transplantation. The procedures were en bloc double-lung transplantation in the first 11 patients with separate bronchial anastomoses in 10, and sequential bilateral lung transplantation in the later 9 patients. There were no operative deaths. Two patients died in the hospital on postoperative days 37 and 73, and there were four late deaths, which were due to infection, rejection, and bronchiolitis obliterans. The acceptable incidence of airway complications, the improvement in lung function of survivors, and the acceptable midterm survival make double-lung transplantation an acceptable alternative to heart-lung transplantation in children. However, in very small children, heart-lung transplantation may be preferable because of the size of the airway anastomoses at risk.

Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

One hundred twenty cystic fibrosis patients were accepted for transplantation. Twenty-five patients underwent double-lung transplantation. Twenty-five patients died awaiting transplantation (20.6%). There were 13 female and 12 male patients. Their mean age was 28 years (range, 7 to 34 years), and mean percentage ideal body weight was 76% (range, 58.5% to 91.9%). Most patients were hypoxic and hypercarbic. Two patients underwent tracheal anastomosis, 15 had en bloc bronchial anastomoses, and 8 had sequential single-lung transplants. Operative mortality was 16%; all deaths were related to bleeding from extensive adhesions. Actuarial survival at 1 year was 64%. Rejection and infection were frequent during the first month and decreased thereafter. Airway complications occurred in 5 patients but were amenable to laser therapy and stenting. We conclude that double-lung transplantation is an acceptable modality for the treatment of cystic fibrosis patients with end-stage lung disease. It may be a better alternative to heart-lung transplantation considering the paucity of thoracic organ donors.

Background: The association between plasma adiponectin and metabolic syndrome may be impaired in heart transplant recipients, since renal failure is frequent among these patients. Thus, we studied the relationship between metabolic syndrome and plasma adiponectin in transplanted heart recipients. Methods: Ninety-five heart transplant recipients were prospectively included 8.3 ± 5.6 yr after transplantation in this cross-sectional study. All patients had physical examination, echocardiography or routine biennial coronary angiography, and laboratory measurements. Results: Metabolic syndrome was found in 31% of these patients. Plasma adiponectin was significantly lower in patients with metabolic syndrome (12.5 ± 8.3 μg/mL) than in patients without (16.7 ± 9.4 μg/mL, p = 0.03). Adiponectin levels were usually in the normal or high range (< 4 μg/mL in only two patients). Low creatinine clearance was associated with higher plasma adiponectin (R=−0.26, p = 0.01). Plasma adiponectin was not significantly different between the 28 patients with angiographic evidence of graft vasculopathy (13.9 ± 9.5 μg/mL) and the 67 patients without (16.1 ± 9.1 μg/mL, p = 0.3). Conclusions: Contrasting with a high frequency of metabolic syndrome in these patients, adiponectin levels were usually in the normal or high range, probably as a consequence of renal failure. This suggests that adiponectin is not a major determinant for insulin resistance among these patients.

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.

Dilated cardiomyopathy is often complicated by severe mitral regurgitation (MR) caused by dilation of the annulus. Heart transplantation remains the ultimate treatment when heart failure fails to respond to medical treatment. Mitral annuloplasty for severe MR in dilated or ischemic cardiomyopathy may delay or replace the need for heart transplantation. The case is reported of a six-year-old child with dilated cardiomyopathy, severe MR and high pulmonary arterial pressure. Following beneficial mitral annuloplasty in 1989, the patient is in excellent functional condition eighteen years later.

The anatomic findings in 11 cases of tricuspid atresia and in two cases of severe tricuspid stenosis, both combined with partial common atrioventricular (AV) canal, are presented in detail. Twelve cases were documented by postmortem examination and the diagnosis was confirmed by echocardiography and surgical observation in the one living patient. Clinical data available in nine cases and cardiac catheterization data obtained in eight are included in this report. In three cases (23%)--two with tricuspid atresia and one with extreme tricuspid stenosis--the tricuspid valve and right ventricle exhibited characteristics seen in Ebstein's anomaly. In all 13 cases, the great arteries were normally related. The ventricular septal defect(s) in 10 (83%) of the 12 postmortem cases rapidly became smaller and this resulted in marked diminution of the pulmonary blood flow and severe hypoxia. Only three of the eight patients with available cardiac catheterization and angiocardiographic data showed the scooped-out appearance of the left ventricular septal surface characteristic of AV canal defects. By contrast, two-dimensional echocardiography, available in the three most recent cases, accurately demonstrated all the defects present and represents the diagnostic method of choice. Early surgical intervention to establish a systemic to pulmonary artery anastomosis is essential for survival. More definitive surgical treatment can be achieved later by an atriopulmonary or cavopulmonary anastomosis with or without replacement of the cleft and often regurgitant mitral valve. The one living patient exemplifies this approach. This is the largest series of this unusual type of tricuspid atresia reported to date.

Between January 1987 and May 1990, six children underwent ventricular assisted circulation for recovery of myocardial function after cardiac surgery. Their ages ranged from 9 months to 12 years. Three patients had tetralogy of Fallot and one had atrioventricular discordance with ventriculoarterial concordance, ventricular septal defect, and under-over ventricles. Double outlet left ventricle and a large ventricular septal defect with pulmonary hypertension was present in the remaining two. The duration of circulatory support averaged 126 +/- 57 h. An extracorporeal membrane oxygenator (ECMO) was used in four patients, biventricular assistance with centrifugal pumps in one, and left ventricular assistance followed by ECMO in the last patient. Circulatory support was begun in the operating room in two patients who could not be weaned from cardiopulmonary bypass. Both these patients are long-term survivors. Assisted circulation was implanted in four patients in the intensive care unit because of low cardiac output refractory to any pharmacological treatment. Only one of these patients could be weaned from circulatory support but he died 20 days later because of multiorgan failure due to persistent poor myocardial function. The causes of death in the remaining three patients were intracranial hemorrhage, untreatable bleeding, and failure of myocardial recovery, respectively. We believe that early postoperative use of circulatory support can be a major determinant for recovery of myocardial function in patients who have severe low cardiac output following repair of congenital cardiac lesions. Our initial experience with the use of biventricular assistance in small children is encouraging. Nevertheless, further observations are clearly necessary to establish the role of postoperative circulatory support in children with congenital cardiac lesions.

A man, living in France, complained about dyspnea and weakness for the last year. At admission, the electrocardiogram showed atrial fibrillation. Transthoracic echocardiographyandcardiacmagnetic resonance imaging revealed a rare case of massively calcified left ventricular endomyocardial fibrosis (EMF), resulting in an obliteration of the apex (Fig. 1A and B). Despite the severity of the calcification intruding the fibrosis, h

This is a report of a case of the rare ectopia cordis malformation of the thoracoabdominal type without intracardiac anomalies. The patient had a successful single stage repair with reduction of the herniating heart and reconstruction of a cartilaginous cover to protect the heart. The result was very good and the follow up for 13 months was very satisfactory.

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.

A 43-year-old man with medical history of gastroenteritis 2 weeks previously was referred to our intensive care unit for acute chest pain. At admission, the ECG showed negative T waves in V1, V2, V3, and V4 leads, and his troponin serum level was 0.6 ng/mL. His C-reactive protein level was elevated at 70 mg/L, and the serum blood count showed hyperleukocytosis with hyperlymphocytosis and thrombocytosis. Two-dimensional transthoracic echocardiogram revealed a dilated and …