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Background: The purpose of this study was to find homograft-related factors that might be associated with the development of stenosis after the Ross procedure, as well as to identify the natural dynamics of stenosis and find echographic cutoff values after one year of follow-up that might predict such an outcome., Methods: We followed up 71 patients (mean age, 24.27 +/- 16.57 years) who had such a procedure prospectively by transthoracic echocardiography, between 1993 and 2002. Follow-up was 55.26 +/- 29.63 months and was 90.14% complete. Homografts were harvested from heart-beating donors or cardiac transplant recipients. Allograft stenosis was analyzed and risk factors were identified by univariate, multivariate, and survival analysis methods. Stenosis was defined as a mean gradient greater than or equal to 20 mm Hg., Results: There were two reoperations and 21 homografts were stenotic at the last follow-up, ten of which were already so at one year after the procedure. Cox regression analysis revealed a transhomograft gradient greater than 9 mm Hg at 1 year after the procedure (hazard ratio [HR] = 10.04) and homograft size (HR = 0.75) as independent predictors for stenosis. Stenosis-free survival was 85.94 +/- 4.35%, 75.51 +/- 5.55%, and 68.56 +/- 6.34 after 1, 3, and 5 years, respectively. A cutoff value of 9 mm Hg at 1 year of follow-up could predict different stenosis-free survival rates., Conclusions: Homograft size is the most important homograft-related factor for stenosis. Most of the increase in transhomograft gradient occurs in the first 24 months. A gradient of 9 mm Hg or more after 1 year predicts the late occurrence of stenosis.

The aim of this study was to determine the surgical results of repair of coarcta tion of the aorta in children under 1 year of age and to assess the risk of recoarctation. Between 1984 and 2004, 206 children under 1 were operated for coarctation of the aorta. Three groups of patients were identified: Group I (isolated coarctation, N=99), Group II (associated ventricular septal defect, N=63), and Group III (complex congenital heart disease, N=44). The transverse aorta was hypoplastic in 51% of cases. Uni- and multivariate analysis of the risk factors for mortality and recoarctation was performed. An extended resection anastomosis was performed in 206 patients. Reconstruction of the transverse aorta was performed in 32 cases. The hospital mortality was 3.9% (N=8). The presence of a complex intracardiac anomaly was a risk factor for mortality on multivariate analysis (p=0.023). In univariate analysis, a two-step management of patients in Group III was a significant risk factor for mortality (p=0.036). Thirty patients (14.6%) had recoarctation (gradient > 20 mmHg, follow-up 40 +/- 44 months). On multivariate analysis the severity of the immediate postoperative gradient was the only risk factor for recoarctation. The authors conclude that surgery for coarctation of the aorta is associated with excellent results in children under 1 year of age. The prognosis is related to the presence of associated severe cardiac malformations. Survival seems to be better if treatment is carried out in a single stage in this group. The risk of recoarctation is related to the immediate postoperative transisthmic gradient which justifies extensive repair of hypoplastic aortic arches.

Background and Aim of the Study: Cardiac surgery in octogenarians is now performed routinely, and generally results in a good improvement in functional capacity. The study aim was to evaluate operative mortality and to identify preoperative and postoperative risk factors of mortality., Methods: A total of 200 consecutive patients (79 males, 121 females; mean age 83 years; range: 80-90 years) who underwent valvular surgery at the authors' institution between 1991 and 2002 was reviewed. Among patients, 154 underwent aortic valve replacement (77.0%), 35 mitral surgery (17.5%), and 11 aortic and mitral valve surgery (5.5%). Forty-five patients (22.5%) had concomitant myocardial revascularization, and 23 (11.5%) were operated on in an emergency setting. Preoperative risk factors studied included endocarditis (2.0%), ventricular dysfunction (22.5%), pulmonary hypertension (33.5%), renal dysfunction (31.5%), chronic obstructive pulmonary disease (7.0%) and arteriopathy (9.0%). The mean EuroSCORE, which was used to assess predicted operative risk, was 9.1; the score was < 9 in 104 patients (52%) and > 9 in 96 (48%)., Results: Hospital mortality was 7% (n = 14). There were no significant preoperative risk factors of mortality. Postoperative complications occurred in 115 patients (57.5%), including low cardiac output (16.0%), supraventricular arrhythmia (29.5%), pulmonary complications (9.5%), gastrointestinal ischemia (2.0%), wound infection (2.0%) and surgical re-exploration (5.5%). Low cardiac output (p < 0.001), gastrointestinal ischemia (p = 0.03) and surgical reexploration (p = 0.004) were significant risk factors of mortality., Conclusion: Valvular surgery in octogenarians is a safe and low-risk procedure. The present data tended to show that the EuroSCORE overestimates mortality in this group of patients.

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 post-operative 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 83+/-2 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 9.5+/-2.3 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 (P=0.05). Low cardiac output (P<0.001), gastrointestinal complications (P=0.03) and surgical reexploration (P=0.001) 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.

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.

We report our results of truncus arteriosus surgical reparation in a period of 10 years. From January 1993 to November 2003, 17 patients were operated with a median age and weight of 66 days and 3.5 kg respectively. According to the Van Praagh classification there were 13 cases of type A1, 2 cases of type A2 and 2 cases of A4. The connection between the right ventricle and the pulmonary artery was performed by a homograft (n = 12), a Contegra tube (n = 1) or a Barbero-Marcial intervention (n = 4). In one case, a plasty of the truncus was performed. Patients were classified in two groups: group A for those operated between 1993 and 1997 (n = 8) and group B for those operated after 1997 (n = 9). Five patients died (29%) in the post-operative period, 4 in the group A (50%) and one in group B (11%). The mean duration of intensive care stay was 12.2 (+ 7.4) days. Statistical analysis did not reveal any difference between both groups, especially concerning post-operative treatment or prognosis. In the multivariate analysis, an age below 30 days was a risk factor of post-operative death (OR = 16.5, 95% CI = 1.09 - 250; p = 0.043). After a mean follow-up of 3.9 (+ 3.5) years, 2 patients required a redo intervention for replacement of the pulmonary artery homograft. All 12 suvivors are asymptomatic without any pulmonary hypertension. In conclusion, the results of surgical reparation of the truncus arteriosus seem to improve with experience. According to recent progresses in surgery and intensive care, the intervention can be scheduled beyond the neonatal period without additive risk and with potentially less consecutive redo interventions.

The angioplasty of native coarcatations of the aorta remains a controversial treatment due to recurrences and the potential risk of aneurysm or of descending aorta dissection during catheterization. The interest of a systematic implantation of an endoprothesis is poorly documented. We report our experience in a small series of 3 patients aged from 7, 28 to 52 years at the moment of the angioplasty of their native aortic coarctation. In all the three cases it corresponded to a "membranous" type, localized a the level of the isthmus without hypoplasia of the aortic arch. All presented a refractory hypertension. One patient presented an intermittent claudication related to a low perfusion of lower limbs. The angioplasty was performed with BIB balloon, associated at the same time with the implantation of a Palmaz P308 stent in two cases and Genesis PG2910P in the last patient. The efficacy was immediate in all the 3 cases with stopping antihypertensive drugs at the very day of the procedure. The immediate results were complicated by a bilateral hematoma of the scarpa in a context of excessive anticoagulation in one patient requiring blood tranfusion. After a follow-up of one, 12 and 21 months, all the 3 patients are asymptomatic without any significant residual hypertension. The control scan of the infant confirmed the absence of re-coarctation. In conclusion, the angioplasty followed by systematic implantation of an endoprosthesis is a safe and effective technique for treating simple forms of native coarctations of the aortic isthmus. It can be proposed as a first line treatment for big infants and adults affected by localized types.

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.

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.

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 ( 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 P MRS to measure ratios of various high-energy phosphate metabolites and the intracellular pH (pHi)., 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 (Pi ) was significantly different between the two groups (0.95+/-0.29 for TS1 and 2.05+/-0.74 for TS2). The difference in pHi 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.

This study was undertaken to determine whether there were regional variations in the access to the national waiting list for cardiac transplantation and in access to cardiac transplantation itself in France. The incidence of inscription on the waiting list for transplantation was calculated for new candidates living in each region between 1998 and 2000, indicating the level of access to the waiting list. The average interval before transplantation between 1995 and 2000 was calculated by the Kaplan-Meier method. The regional lack of transplant organs was estimated by the ratio of the difference between patients inscribed on the waiting list and those operated, over the number of patients operated. The overall national incidence of inscription of the waiting list for cardiac transplantation was 8 new patients per year per million inhabitants with a range of 1.7 in Corsica to 12.1 in Lorraine. The regional incidence was lower than the national reference in 5 regions in higher in 4 regions. There was a strong attraction of patients to the Ile-de-France, Pays de la Loire and Rhone-Alpes regions. The average waiting time for transplantation in France was 3.7 months and ranged from 1.2 months in Brittany to 15.4 months in Aquitaine (p < 0.001). The degree of regional lack of transplant organs was significantly and positively correlated with the average waiting time for transplantation. The results of this study show that there are regional differences in the incidence of inscription on the waiting list for cardiac transplantation in France and a significant variation in waiting times for cardiac transplantation, and therefore, in access to cardiac transplantation.

Despite the cardiac surgery improvements allowing the correction of the majority of congenital heart diseases with ventricle-great vessels discontinuity, some abnormalities increase the risk of bi-ventricular reparation. We herein report the case of a patient presenting a rare form of double outlet right ventricle with a ventricular loop, with moderate right ventricle hypoplasia. L-malposition of great vessels and pulmonary artery stenosis, and for whom we opted for a palliative surgical treatment including a systemic-pulmonary anastomosis followed by a upper right bi-directional cavo-pulmonary derivation. The last surgery was followed by recurrent right pleural effusions disappearing after the embolization of the systemic-pulmonary anastomosis by catheterism as it probably obstructed the draining of the cavo-pulmonary anastomosis. The relevance of this clinical case reported is, firstly the description of this ventricle loop resulted from a marked ventricular malposition which is a rare heart disease, and secondly the discussion about the surgical treatment, especially about the choice between palliative and curative surgery. Only comparative studies on long term morbidity and mortality between the bi-ventricular reparation and mono-ventricular palliation will allow the selection of the most appropriate surgical treatment.

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=0.014), the diagnosis of IAA and TAPVD (P<0.05), CBP duration over 185 min (P=0.048), clamping time over 98 min (P=0.039) and central venous saturation below 51% P=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.

We report a case of an infant presenting with the rare association of tetralogy of Fallot, hypoplasia of the pulmonary arteries, and stenotic bicuspid aortic valve. Surgical correction, performed at 16 months of age, included aortic valvular commissurotomy, opening the right ventricular outflow tract (transannular patch), and ventricular septal defect closure. The postoperative course was favorable, and the child was discharged from the hospital. Three months after the procedure, the patient is in excellent condition, without cardiac medication.

The aim of our study was to compare the clinical, echographic, and prognostic features of Streptococcus bovis (S. bovis) endocarditis with those caused by other streptococci and pathogens in a large sample of patients with definite endocarditis by Duke criteria, using transesophageal echocardiography. Two hundred six patients (149 men, mean age 57 +/- 15 years) with a diagnosis of infective endocarditis formed the study population. All patients underwent multiplane transesophageal echocardiography and blood cultures. Cerebral, thoracoabdominal computed tomographic scan was performed in almost all patients (95%). All patients with S. bovis endocarditis underwent colonoscopy. Incidence of S. bovis endocarditis in our sample was 19%. Patients with S. bovis endocarditis were older than other groups. Multiple valve involvement, native valves, and large vegetations (>10 mm) were more frequent in patients with S. bovis. There was a significantly higher occurrence of embolism in the S. bovis group. Splenic embolism and multiple embolisms were significantly more frequent in patients with S. bovis. Gastrointestinal lesions, anemia, and spondylitis were observed more frequently with S. bovis endocarditis. In addition to the requirement for gastrointestinal examination for S. bovis endocarditis, our study underlines the need for systematic screening for vertebral and splenic localizations, and suggests the use of early surgery to prevent the high risk of embolism in these patients.

Conventional treatment of pulmonary atresia with ventricular septal defect (VSD), hypoplastic pulmonary arteries (PA) and major aorto-pulmonary collaterals (MAPCAs) is controversial: from symptomatic and palliative treatment for some authors to surgery with unifocalisation of collaterals for others. These treatments never use native pulmonary arteries as only source of pulmonary flow, but create "neo-pulmonary arteries". Nine cases of pulmonary atresia with VSD, hypoplastic PA and MAPCAs were treated by rehabilitation of native PA through a staged approach: 1) surgical neonatal connection between right ventricule (RV) and hypoplastic PA, 2) evaluation and interventionnal catheterism with angioplasty of PA stenosis and closure of collaterals, 3) complete surgical correction with reconstruction of right outflow track and PA and closure of VSD. After first surgical stage of RV-PA connection at the mean age of 4.8 months (+/- 5.6 months), 8 patients were alive and underwent 22 cardiac catheterisms (mean of 2.7 per patient), with angioplasty of PA, and occlusion of MAPCAs in 6 and 2 patients respectively. Seven patients underwent complete anatomical correction at the mean age of 28.8 months (+/- 17.7 months) with one late death. The 6 remaining patients had encouraging hemodynamic status (RV pressure/LV pressure ratio at 0.6 +/- 0.26; mean left and right distal pulmonary pressure at 15.2 mmHg (+/- 9.1 mmHg)), and good functionnal status (3 in NYHA functionnal class 1, and 3 in class 2), for a mean follow-up of 79.5 months (+/- 41.4 months). One patient had reoperation on right outflow track stenosis, 6 years after correction. This small series enhances the feasibility of a staged approach with rehabilitation of small PA, allowing complete surgical correction with the native PA with good hemodynamic and functional results in pulmonary atresia, with VSD, hypoplastic PA and MAPCAs.

Background: Lung or heart-lung transplantation is a useful therapy in life-threatening pulmonary disorders during childhood. Cyclosporine A is a major immunosuppressive treatment but has a number of adverse effects including nephrotoxicity. There have been no reports on the long-term evolution of renal function in a large series of paediatric pulmonary transplantation recipients., Methods: We examined 19 patients followed up for at least 3 years after pulmonary transplantation. The mean time of follow-up was 5.36 years. Kidney function was evaluated by calculation of glomerular filtration rate (GFR) according the Schwartz formula., Results: The GFR was normal before transplantation in all patients. The short-term evolution of GFR was marked by a significant drop during the first and until the 6th month. Then, regardless of the level reached at the end of the 6th month, the GFR remained stable in all patients except one until the end of follow-up. At the end of follow-up, 31% had normal GFR, 57% had mild chronic renal failure, and 5% had advanced renal failure. Hypertension was frequent and associated with renal failure., Conclusions: Paediatric pulmonary recipients showed evidence of long-term cyclosporine A-associated nephrotoxicity. Most of this toxicity occurred during the first 6 months.

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

Background: In most cases of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, a Lecompte procedure (réparation à l'étage 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).

A case of a false aneurysm arising at the proximal suture of an aortic root replacement with a pulmonary autograft is presented. This complication did not occur in the first postoperative month but was discovered late, and the female eight-year-old patient was in an extremely serious condition. She was reoperated on an emergency basis but died of acute pulmonary artery hypertension. The mechanism of the occurrence of such a case is discussed. In the absence of infection, structural weakness of the right ventricular muscle with progressive tearing is suggested. Strict and prolonged echocardiographic surveillance after the Ross procedure and early reoperation are mandatory.

Now that the Ross procedure (RP) has been established as the best method of aortic valve replacement (AVR) in several cohorts of patients, it is appropriate to analyze the evolution, as well as the anatomic and physiologic bases for it. Reviewing the evolution of this operation, one may understand the time lapse between its inception and the universal performance of this procedure. Experimental work began as early as 1927 by Hochrein. He was followed by the Stanford group, Lower in 1960 and 1961, and Pillsbury and Shumway in 1966. Successful clinical application by Donald Ross in 14 patients, two in the mitral and 12 in the aortic positions, was accomplished in 1967. Several important developments followed, including Marcel Geens' study of the blood supply to the ventricular septum in 1971 together with the improvement in surgical results following the initial experience of Gonzalez-Lavin and Ross. Further developments included assessment of the tensile strength of the pulmonary valve (PV) by Gorczynski (1982), ability to grow by Murata (1984), a finding of low Ca++ content of the PV by Livi in 1987 and of excellent hydraulic function by Wareesena in 1994. Finally there was universal acceptance by Elkins, Duran, and others, culminating with the Ross Registry and the establishment of the Ross Colloquium by Oury et al. A review of the anatomical features of the PV are compared with those of the aortic valve (AV), including gross anatomy and relationship to the sinotubular junction, scan microscopy and anisotropic properties of both AV and PV. The blood supply to the ventricular septum will be outlined by reviewing Marcel Geens work. The hemodynamics as reported by several investigators are reviewed. The clinical evidence of growth by Elkins et al. is outlined. Based on this increasing knowledge, indications and contraindications for AVR by the RP are discussed.

Background: This study examined our experience with bilateral single-lung transplantation in pediatric patients., Methods: Between 1988 and 1995, we have performed 32 double-lung transplantations in children. The first 10 were performed en bloc, the following 22 by bilateral single-lung transplantation. Indications for bilateral single-lung transplantation were cystic fibrosis in 16 patients, primitive obliterative bronchiolitis in 1, pulmonary artery hypertension in 1, and retransplantation in 4. Patients' ages ranged from 7 to 16 years (mean, 12 years). Four patients underwent a parenchymal reduction (lobectomy or bilobectomy). Bilateral single-lung transplantation was performed with a "clam-shell" incision, normothermic cardiopulmonary bypass, and a beating heart., Results: There was one postoperative death (heart failure in a retransplantation patient). Bleeding was moderate, and 4 patients had a bloodless procedure. Bronchial healing was satisfactory, with 3 patients receiving temporary left main bronchus stenting. There were two hospital deaths (recurrent cytomegalovirus infection in a retransplantation patient and multiorgan failure at 2 months) and seven late deaths, caused by infection (mostly cytomegalovirus), obliterative bronchiolitis, or both. Actuarial survival was 75% at 1 year, 56% at 2 years, and 36% at 3 years., Conclusions: We conclude that bilateral single-lung transplantation appears to be an acceptable technique, even in small children. Bronchial healing is satisfactory, and no revascularization procedure appears necessary. Midterm and long-term results are comparable with those of heart-lung transplants, and in view of the current problems with organ donation, we think it is an adequate strategy in pediatric lung parenchymal disease.

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.

Background: After standard orthotopic heart transplantation (Sd HT), the enlarged resultant atria may promote atrial thrombosis. The purpose of this study was to compare the incidence of spontaneous echo contrast and left atrial thrombosis after Sd HT and total orthotopic (Tot HT) heart transplantation., Methods and Results: Transesophageal echocardiography (TEE) was performed in 75 patients with Sd HT and in 20 patients with Tot HT. Despite the use of antiplatelet therapy, an acute arterial embolism occurred in 11 (15%) of the 75 patients with Sd HT but in none of the 20 Tot HT patients. All patients were in sinus rhythm. Left ventricular ejection fraction was similar in Sd HT and Tot HT patients. Left atrial diameter was smaller in Tot HT patients than in Sd HT patients (41 +/- 4 versus 58 +/- 6 mm, P < .001). In Sd HT patients, spontaneous echo contrast was present in 43 patients (57%) and was associated with left atrial thrombus in 20 patients (on the left atrial appendage in 12 patients, on the posterior wall in 6, and on the suture in 2). No thrombus was detected by transthoracic echocardiography; all thrombi were detected by TEE. On the other hand, no left atrial thrombus was observed in Tot HT patients, and only 1 patient had spontaneous echo contrast. Of the 11 Sd HT patients who experienced an arterial embolism, 5 had both spontaneous echo contrast and left atrial thrombus and 5 had only spontaneous echo contrast., Conclusions: This study demonstrates a high rate of left atrial thrombus after Sd HT and emphasizes the role of TEE in the follow-up of these patients. The therapeutic implications are the need for a preventive anticoagulant therapy in the high-risk population receiving Sd HT diagnosed with TEE and the consideration of Tot HT as a better surgical approach as far as thrombotic complications are concerned.

An experimental model has been designed to evaluate the potential of growth of aortic vascular autografts and homografts. In 25 young rabbits a graft interposition was performed at the level of the infrarenal aorta, with micro-surgical techniques. Different groups included fresh autografts, fresh and cryopreserved homografts and controls. Animals were allowed to grow normally and were sacrificed when adult, at the mean weight of 2.99 kg. We studied for each case the growth of the native aorta and that of the graft, and calculated the growth-ratio (growth of the graft/growth of native vessel). Grafts and adjacent aorta were histologically studied. The growth in terms of increase in diameter of the native aorta was identical between the four groups (from 1.97 +/- 0.14 mm to 2.86 +/- 0.23 mm). Growth of the graft was normal (mean growth ratio 1.08 +/- 0.21) for autografts. Growth was absent (mean ratio 0.12 +/- 0.15) for fresh and cryopreserved homografts (p = 0.001). Histologic study showed normal optical microscopic aspects (endothelial layer, intima and media thickness and cellularity) when growth had been normal and, when growth had been absent, aspects compatible with rejection including mainly intima hyperplasia and media attenuation. In conclusion, normal growth of the arterial autografts was confirmed, the histologic features of these grafts were normal, but homologous aortic grafts, fresh or cryopreserved, never showed any growth potential.

Between may 1988 and march 1993, twenty five double lung transplants were performed and five heart/lung transplants. Lung function tests (EFR) were performed on these patients for a period of 19.2 +/- 3.4 months. The aim of this study was two-fold. First, to report our overall results and to estimate the role of the single breath nitrogen washout test (N2 slope) in the early detection of chronic rejection (RC). Secondly, to assess the diagnostic value of EFR in the discrimination of acute rejection (RA) and of cytomegalovirus pneumonitis (PCMV). There were 41 episodes of RA and 21 episodes of PCMV and they were analysed as a function of the presence or absence of RC. In the absence of RC, RA produced no change in EFR and PCMV was accompanied by a pure restrictive ventilatory defect. On the otherhand, RA and PCMV lead to a worsening of obstruction and an hypoxaemia which characterises RC. The diagnosis of RC was made, on average, 14.4 +/- 2.9 months after surgery. However, from the sixth month the nitrogen slope was significantly increased and other parameters of EFR (particular maximal flows at low lung volume) remained normal. Thus, our results suggest that the N2 slope, measured in the absence of any evidence of acute rejection, constitutes an early test for chronic rejection. When its pathological rise is compared to the results of histology (presence or absence of RC), it shows a sensitivity of 0.94 and a specificity of 0.93.

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.

Many lung transplant programs consider ventilator dependence as a contraindication for transplantation. Among 54 patients in whom bilateral lung transplantations for cystic fibrosis were performed by the Joint Marseille-Montreal Lung Transplant Program, 10 were ventilator dependent. Three of them died in the early postoperative period (30%): 2 as a result of cerebral anoxia and sepsis, 1 of Pseudomonas cepacia pneumonia. Two patients died at 15 and 19 months after transplantation of obliterative bronchiolitis and secondary bacterial pneumonitis. Another 2 patients in whom obliterative bronchiolitis developed underwent retransplantation with a heart-lung block; 1 of those was operated on at 12 months and is well at 29 months after his initial transplantation; the second was operated on at 34 months and died of primary graft failure. Three other patients are alive and well at 3, 11, and 14 months after transplantation. Actuarial survival at 1 year was 70%. The postoperative course and the infectious and rejection complications were no different from those in patients who underwent transplantation while spontaneously breathing. Obliterative bronchiolitis developed in 66% of patients at risk (2 of 6 patients surviving more than 6 months). We conclude that transplantation in mechanically ventilated patients with cystic fibrosis is not associated with an increase in morbidity or mortality after bilateral lung transplantation. Long-term survival, as in patients who undergo transplantation while spontaneously breathing, is limited by the development of obliterative bronchiolitis.

The authors review the recent changes observed in the indications for lung transplantation. Several classical contra-indications have been alleviated or even cancelled. Chronic infection presenting as cystic fibrosis has become one of the first indications for lung replacement. Respirator-dependent patients are at an operative risk comparable to the overall results. Long-term corticosteroid therapy and pleural adhesions are no longer formal contraindications but should be approached with extreme caution. In recent years, single lung transplantation has been extended to diseases, such as emphysema and pulmonary hypertension, which were formerly indications for double-lung or heart-lung transplantation. The reasons for this include donor shortage and the better 1-year survival following single lung transplantation. The most recent development is paediatric lung transplantation. The legitimacy of redo transplantation is controversial.

Coronary artery disease in cardiac transplant recipients is the main cause of mortality after the first postoperative year. This atheroma has unique anatomical features with widespread infiltration of the intima by inflammatory cells. The different physiopathological hypotheses are analysed. Immunological processes are probably responsible, suggesting chronic rejection. The cytomegalovirus could be an inductive mechanism. The classical vascular risk factors probably play a role but their action is not as clear as that of immunological and viral factors. Hyperlipidaemia is a causative mechanism and the predisposing role of steroid therapy is also recognised. From the practical point of view, correction of the classical risk factors is the only available therapeutic possibility at present.

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.

Volume concordance between donor lungs and the chest cavities of transplant recipients has important perioperative and postoperative implications. Between December 1987 and August 1991, 90 patients underwent lung transplantation in the Joint Marseilles-Montreal Lung Transplantation Program: 51 patients had double lung transplants, 19 patients had single lung transplants, and 20 patients had heart-lung transplants. There were 18 children (age range, 7 to 17 years) and 72 adults (age range, 18 to 58 years). Size matching was based on measurement of the submammary thoracic perimeter. Patient age (+/- 2 years) was also taken into consideration in children. Airway anastomoses were bronchial except for all heart-lung transplant patients and two double lung transplant patients, who had tracheal anastomoses. Occasional differences between donor and receiver bronchial diameters in children (greater donor size twice and smaller donor size once) required bronchoplasty in three instances. Healing was normal in these three instances, and no bronchial stenoses were noted. Performance of separate sutures rather than continuous running sutures on the cartilaginous anterior portion facilitated correction of airway diameter inequalities in adults. Excess volume was noted in three patients during closure of the thorax. In one patient, donor and recipient thoracic perimeters were similar. In two patients, however, donor size was greater by more than 20%. This was corrected by pneumoreduction with a surgical stapler. Lung size was decreased by 10% to 40% with use of this technique. Thoracic closure was facilitated and hemodynamic instability was thus corrected. No functional abnormalities were noted after surgery once the differences in lung size were corrected.(ABSTRACT TRUNCATED AT 250 WORDS)

We describe a simple and rapid technique for takedown of right-sided modified Blalock shunts involving a blunt approach between the pericardium and the pleura. We have used this method successfully on either side in 47 cases.

Good long-term results obtained with homologous valve grafts, specially for correction of congenital cardiac defects, prompted us to create a local cryopreserved homograft bank. A 5-year experience is described, including indications for harvesting, techniques of preparation, cryopreservation and thawing. During this period, 195 homografts entered the bank and 92 were used. Among these 92 clinical cases, 74 consisted of right ventricular outflow tract reconstruction (47 valved conduit and 27 monocusp bearing patch) and 17 were left ventricular outflow tract reconstructions (11 free hand aortic valve and 6 aortic root replacement). Only one case was a tricuspid replacement with a stented homograft. Short-term results, with these homografts, are satisfactory and are compared with long-term results reported in the literature. The authors insist on using very thorough techniques and constant quality control. New developments should include viability control and immunological studies.

The aim of this study was to evaluate spontaneous changes of Doppler and echocardiographic parameters of cardiac transplants in the absence of rejection. Based on 258 recordings representing the follow up of 30 patients after orthotopic cardiac transplantation in the absence of histological signs of rejection on biopsy within 24 hours, we tried to define: the evolution of these parameters from the first postoperative week to after the 30th month (average 12 months); the Doppler echocardiographic values of "normal" transplants: the values observed one year after surgery were compared with a control group age-matched to the donors.(ABSTRACT TRUNCATED AT 250 WORDS)

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.

Since December 1985, we have performed 38 transplantations: cardiac (CT) n: 31, cardiopulmonary (CPT) n: 1, or bipulmonary (BPT) n: 6. There were 31 male and 7 female patients, aged 7 to 62, mean 46. In the cardiac group, the cardiomyopathy was primitive in 13, ischemic in 16, valvular in 2. Five patients had undergone one or more previous operations. Three patients had a biventricular assist device (1,6 and 7 days before transplant) for acute cardiac failure. The indication of CPT or BPT was pulmonary artery hypertension (1), silicosis (1), cystic fibrosis (4). There were 4 post-operative deaths in the CT group (12.9%); failure of graft, low cardiac output, pulmonary artery hypertension by multiple pulmonary thrombosis, and 2 deaths in the CPT and BPT groups (28%). The mean post-operative hospital stay was one month. All patients with CT were treated by an initial maintenance bitherapy protocol (cyclosporine, steroids) and observed by myocardial biopsies and echocardiograms. In 40 per cent of the patients, Azathioprine was subsequently added. The patients had 2.1 rejection episode/patient/year, either spontaneously reversed of treated medically. There were two late deaths (2 and 7 months) by refractory rejection. 78 per cent of the patients were alive one year after transplant. All survivors have recovered a normal life, some of them with full-time work.(ABSTRACT TRUNCATED AT 250 WORDS)

This report presents six cases (among 43 surgical cases) of left ventricular EMF presenting as pure mitral insufficiency without any echocardiographic or hemodynamic signs of left ventricular EMF (except apical diverticulum in two cases). Five of these cases were diagnosed at operation, and the sixth was diagnosed on the basis of a characteristic right ventricular angiogram. At operation a fibrotic lesion localized to the level of the anteropapillary muscle, with severe mitral insufficiency, was found. Five patients underwent successful mitral valve replacement and one a successful conservative mitral valve procedure. Postoperative angiograms, done in two patients, showed a normal contour of the left ventricle. A review of the literature did not reveal any previous descriptions of this type of limited left ventricular EMF, mimicking rheumatic mitral insufficiency. In our surgical experience, we have encountered three types of left ventricular EMF: obliterative, extensive, and limited. We emphasize EMF as a possible cause of mitral insufficiency in African children.