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Study Objectives: To examine the results of right heart derivations and clinical outcomes according to preoperative characteristics and operative strategy implemented., Methods: Fontan operations were performed in 65 patients (mean age = 10.3 years, 41 males). The majority of cardiopathies were single ventricles (SV) with (49% of patients) or without (26%) tricuspid atresia. A palliative bidirectional cavo-pulmonary (BCP) anastomosis was performed prior to Fontan in 15 patients. Intra-atrial Fontan tunnelling was performed in 43 patients, Kreutzer-type operations in 10, and extracardiac tubes were used in 8 patients. The mean duration of follow-up was 6.1 +/- 0.3 years., Result: The 30-day mortality was 13.8%. Early mortality was higher among patients with SV with than without tricuspid atresia (P < 0.01), and among patients < 4 years old. Early reoperations were required in 5 patients, including dismounting in 1, BCP anastomosis after Kreutzer procedure in 1, and tube thrombosis in 1 patient. A single death occurred past 30 days, and late adverse events included protein-losing enteropathy in 1 patient, complete atrioventricular block in 1, and tube thrombosis treated with heparin in 2 patients. At the end of follow-up, 75% were in New York Heart Association functional class I., Conclusion: Our intermediate-term results of Fontan-type operations were satisfactory, and steadily improving. The prognosis was better in patients operated at age 4 or older. A prior BCP anastomosis improved the results. A higher morbidity was observed with intra- than with extra-atrial Fontan procedures. The merit of fenestration procedures with respect to morbidity remains the be evaluated.

Aortic valve sparing operations are now widely accepted for ascending aortic aneurysm surgery. We herein report our experience of the Tirone David procedure in larger indications. From January 1997 to August 2003, 50 Tirone David procedure have been performed on 36 male and 14 female (mean age: 60 +/- 15). Five patients presented a Marfan disease and 4 acute dissections. Grade III or IV aortic insufficiency was frequent (40%). Aortic diameter was not particularly dilated, ranging from 44 to 78 mm (mean: 57 +/- 10 mm). Mean ejection fraction: 57 +/- 10%. Mean left ventricular end diastolic diameter =63 +/- 7 mm. An associated mitral valve repair and 1 coronary bypass were necessary. Mean cross clamp and bypass times =94 min and 122 +/- 28 min respectively. There was one in-hospital mortality. Secondary mortality affected 2 patients (non-cardiac deaths), for a cumulative follow-up of 946 months. During follow-up continence control was always excellent, only 1 bicuspid valve had an aortic insufficiency >grade II. Tirone David procedure gave satisfactory results as regards both aortic ectasia and aortic regurgitation control. We consider it feasible even in case of aortic dissection but caution is required when facing bicuspid aortic valves.

The aim of this retrospective study was to analyse cases of infectious endocarditis (IE) of native or repaired ventricular septal defects (VSD) to determine its incidence, the circumstances of its occurrences, the outcome and prognosis of this complication. From 1966 to 2002, 36 IE occurred in 19 boys and 17 girls: the age at diagnosis was 13.4 +/- 11.8 years; 26 had an isolated VSD and 10 had VSD associated with a minor lesion. Eleven of the 36 cases (30.5%) had been previously operated: repair of an isolated VSD with a patch in 5 cases, associated with a Crafoord procedure for coarctation of the aorta in 2 cases, three times with conservative treatment of associated aortic regurgitation (AR) and with ligature of patent ductus arteriosus (PDA) in 1 case. Twenty-five of the 36 cases (69.5%) had not been operated before: 21 isolated type 1 VSD; 2 VSD + AR, 1 VSD with PDA (undiagnosed), 1 VSD with valvular pulmonary stenosis (PS). The portal of entry was post-surgical in 7 out of 36 cases (19.4%): 4 VSD patches, 2 VSD patches + Crafoord and 1 VSD patch with ligature of PDA. The source of infection was dental in 14 out of the 36 cases (38.9%): one isolated VSD repair with residual shunt, 11 native VSDs, and 2 cases of unoperated VSD + AR. The other infectious causes (15 = 41.7%) were ENT (2 cases), skin (2 cases), gastrointestinal (2 cases), pulmonary (1 case) or unknown (8 cases), on operated lesions (3 VSD patches + AR) or native lesions (12 cases: 10 isolated VSDs, 1 VSD with PSD and 1 VSD with PS). Twelve episodes occurred (33.3%) despite antibiotic prophylaxis, 7 out of 7 post-surgical and 5 out of 14 dental cases. The commonest localisation was the tricuspid valve (10 cases, always in isolated VSD). Embolism was observed in 60% of right heart endocarditis (always multiple) and in 55% of IE of the left heart (single embolism). Early surgery was required in 6 patients (16.7%). The risk of early surgery was higher in patients with VSDs associated with other lesions (4 out of 10 = 40%) than in isolated VSD (2 out of 26, 7.7%, p = 0.027). Thirteen patients underwent secondary surgery after an average interval of 2.96 years, median 0.86 years (from 4 months to 22.8 years) for VSD repair (10 cases), aortic valve replacement (2 cases) and aorto-aortic conduit (1 case). The global follow-up period was 7.4 +/- 8.3 years, from 28 days to 27.9 years (median 3.3 years). Five deaths were observed on average 3.7 +/- 6.2 years after the episode of IE (median 6 months): 2 were early, occurring less than 6 months after IE and directly related to the infective episode. The survival was 97.1% at 1 month, 94.3% at 6 months, 91.4% at 1 year and 86.6% at 5 and 10 years after IE. VSD is a benign cardiac lesion, the prognosis of which can be severely compromised by infectious endocarditis: surgical repair reduces the risk but does not totally exclude it because of minor associated abnormalities. Prophylactic antibiotic therapy and the diagnosis of latent infectious problems, particularly dental, remains essential before and after cardiac surgery.

Coarctation or isthmic stenosis of the aorta is defined as an abnormal obstruction situated at the junction of the aortic arch and the descending aorta near the site of ligamentus arteriosus. It is a common malformation representing 5 to 7.5% of all congenital heart diseases. Coarctation of the aorta is 2 to 3 times commoner in boys than in girls. Two clinical forms may be distinguished: asymptomatic isolated coarctation of childhood, the surgical treatment of which was first performed by Crafoord in 1944, and coarctation of the neonate and infant associated in over 2/3 of cases with other cardiovascular malformations, especially tubular hypoplasia of the aortic arch. The surgical correction of isolated coarctation is best performed between 6 months and 1 year of age in other to limit the incidence of residual hypertension. A modified Crafoord technique decreases the risk of restenosis and enables treatment of associated hypoplasia of the aortic arch in the same surgical procedure.

Drainage of the inferior vena cava into the left atrium during surgery for closure of an atrial septal defect is a rare complication. More common in low situated defects, it was more frequent when this type of surgery was performed without cardiopulmonary bypass. This diagnosis was made in a 45 year old woman with cyanosis operated 28 years previously. The right-to-left shunt was demonstrated by the hyperoxia test and confirmed by perfusion pulmonary scintigraphy and contrast echocardiography but only when the contrast was injected in the inferior vena cava territory, and by angiography. The surgeon confirmed the abnormality, closed the interatrial septum and reconnected the inferior vena cava to the right atrium.

Circulatory assistance in children and neonates has not been extensively reported. It poses specific problems related mainly to miniaturisation of systems. The authors report three cases of left ventricular assistance with the Biomedicus centrifugal pump followed by functional recovery in neonates operated for d-transposition of the great arteries. The assistance was installed by cannulation of the left atrial appendage and the ascending aorta, the canules being connected to the Biomedicus pump. The duration of assistance was 53, 116 and 120 hours respectively. The beginning of left ventricular recovery was observed after 24, 48 and 70 hours of assistance and the patients were weaned of assistance under stable haemodynamic conditions. One of the major problems during the period of assistance is decoagulation, 2 out of 3 patients requiring removal of thrombi during the first 12 hours of assistance. On the other hand, no cases of systemic embolism or thrombosis in the pump itself were observed. Decoagulation was maintained by continuous intravenous heparin with an initial dosage of 5 U/kg/h adapted to the activated cephalin time and anti Xa levels measured every 6 hours. No infectious complication was observed and the sternal wound healed satisfactorily despite systematic delayed closure of the thorax. The quality of results requires cooperation of a surgical infrastructure used to techniques of circulatory assistance, a well equipped neonatal intensive care unit and a permanently accessible laboratory for monitoring coagulation status.

Between December 1984 and September 1996, 43 cardiac transplantations were carried out in 40 patients aged 2 days to 21 years (one third under 10 years of age) for cardiomyopathy (21 cases), congenital heart disease (19 cases) or retransplantation (3 cases). The average waiting time for transplantation was 80 days: this delay increased by a factor of five in 2 years (from 1 month, before 1994, to 5 months at present). Twelve patients dies, including 6 before the 8th day. The 28 survivors were prescribed triple immunosuppressive therapy: the average follow-up was 4.4 years (range 3 months to 11 years). Monitoring rejection was carried out by non-invasive methods based on clinical, electrocardiographic and Doppler echocardiographic observations. Any suspicion of acute rejection led to endomyocardial biopsy for confirmation and therapeutic guidance. There was a total of 47 episodes of acute rejection (0.3 per patient), mainly in the first 3 months: acute rejection was less common in the younger children. Graft function was normal in 71% of cases. Five children have a pacemaker implanted during the first month. Despite continuous steroid therapy, 82% of patients had normal staturo-ponderal growth. The myocardial mass of the graft increased in parallel with the body surface area. Nephrotoxicity of ciclosparine was responsible for significant renal failure in 19% of patient and seemed more common in the young children. Psychological disturbances were commonest in adolescence and could result in poor treatment compliance (4 cases, with 1 death and 2 retransplantations). Despite satisfactory medium-term results, nephrotoxicity of ciclosporine, long-term graft function and psychological difficulties of adolescents remain unresolved so that transplantation is reserved for terminal cardiac disease resistant to all other forms of treatment.

The diagnostic value of coronary angiography, a widespread method of detection of transplant coronary artery disease, was studied in 17 cardiac transplant patients with reference to histological examination. In the 6 coronary segments studied, the only significant but weak correlation that was found was for the distal left anterior descending artery: the correlations were not statistically significant in the other 5 segments. Coronary angiography underestimated lesions and false negative results were frequently reported (66 and 27% respectively). The limitations of coronary angiography may be explained by the technical artefacts related to both methods of evaluation and the anatomically diffuse and distal nature of transplant coronary artery atherosclerosis. A more reliable diagnostic method would seem to be required in view of the clinical importance of this pathology.

Systematic transoesophageal echocardiography after the 10th day of mitral valve replacement with a mechanical prosthesis has enable diagnosis of several abnormal conditions: thrombi, strands and paravalvular leaks. The aim of this prospective study was to determine the prevalence of these conditions by biplane transoesophageal echocardiography in the first 24 postoperative hours. Transthoracic and transoesophageal echocardiography was performed on average 12 +/- 3 hours after coming out of the surgical block in 77 consecutive patients who underwent mitral valve replacement with a mechanical prosthesis. Nine patients (11.7%) had appearances of thrombi or strands in the left atrium or auricle. These small thrombi (1 to 1.5 cm2) were not obstructive. No embolic events were observed in the first month of these patients. Spontaneous contrast was seen in the left atrium of 31 patients (40%). The factors associated with the presence of thrombus or strands were advanced age (p = 0.02), presence of spontaneous contrast (p = 0.02) and more dilated left atrium (p = NS) Paraprosthetic leaks were seen in 11 cases (14.3%). In 10 cases, the regurgitant jets were narrow at their origin with little extension into the left atrium. Only one patient had severe regurgitation associated with raised transprosthetic pressure gradients. Cases with paravalvular leaks had a lower incidence of spontaneous contrast (3.2% vs 21.7%) and no thrombosis. Transoesophageal echocardiography demonstrated spontaneous contrast, thrombi and strands in the initial hours following implantation of a mechanical mitral valve prosthesis. The prevalence of these appearances, comparable to that of the results reported with later investigations, underlines the importance of effective anticoagulation from the fist postoperative hours.

The use of aortic angioplasty rather than surgery for restenosis of coarctation of the aorta after initial surgery remains controversial. The efficacy and complications of these two techniques have never been compared in prospective and retrospective studies. Between 1976 and 1992, 56 patients were treated for secondary restenosis of coarctation of the aorta: 29 by angioplasty and 27 by surgery. The case reports of these patients were reviewed retrospectively. The average follow-up was 1.5 +/- 0.3 years of the angioplasty group and 5.4 +/- 0.8 years for the surgical group. The reduction of systolic blood pressure in the right arm was significant and identical in the two groups (p < 0.05). The residual pressure gradient was less immediately and at term in the surgical group (p < 0.05). No fatalities were observed in either group. There were no aneurysmal complications. A neurological complication (posterior column syndrome) was observed after surgery (3.7%). The global rate of complications was higher after surgery (6.7% vs 33%). The rate of further restenosis was higher after angioplasty (18.5% vs 3.7%). The low rate of complications, the shorter hospital stay without repeat thoracotomy were in favour of the angioplasty procedure but the greater immediate and long-term efficacy of surgery on the pressure gradient was in favour of the latter solution. A prospective long-term study is necessary.

In emergency cases of babies with a single ventricle and an obstruction to aortic outflow, low pulmonary flow or, on the contrary, high pulmonary flow, only palliative surgical procedures can be proposed. The authors set out to determine the prognosis of a population with this type of lesion having undergone one of the three following procedures: systemic pulmonary shunt, pulmonary artery banding, repair of the aortic arch (usually associated with pulmonary banding). One hundred and nineteen (63%) of the 185 patients hospitalised between 1/01/1970 and 31/12/1991 in the paediatric cardiology unit of the Cardiac Hospital of Lyon with a diagnosis of single ventricle, underwent one of these three procedures as a treatment of first intention. The survival of the 22 patients who underwent pulmonary artery banding (90 +/- 6%, 85 +/- 8%, 85 +/- 8% at 1.5 and 10 years respectively) was significantly better than that of the patients undergoing systemico-pulmonary shunt (63 +/- 6%, 53 +/- 6% and 49 +/- 6% at 1.5 and 10 years respectively). On the other hand, repair of an obstacle of the aortic arch was a precarious procedure as the survival was only 23 +/- 11%, 16 +/- 11% and 16 +/- 11% and 1.5 and 10 years respectively). These results suggest, with the reserve inherent to the methodology of retrospective studies of small populations, that it is not illogical to continue to propose pulmonary artery banding for babies with single ventricle associated with high pulmonary flow. This procedure should only be envisaged after strict selection of candidates and providing there are facilities for intensive postoperative care.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors analysed the anatomical and clinical prognostic factors of single or common ventricle. This retrospective study was based on a series of 185 patients hospitalised between 1/2/70 and 31/12/91 in the paediatric cardiological unit of the Cardiological Hospital of Lyon with this condition. A number of anatomical and clinical parameters were identified in this population. For each parameter, a given patient could only relate to single modality and a survival graph determined by Kaplan-Meier analysis was established for each modality. For each variable, the survival curve of the most frequently encountered modality served as a reference and the other modalities were compared with it using a logrank test. The different modalities potentially related to patient survival were then entered into a multivariable model using logistic regression. The results of this study of multivariable analysis using the Odds-Ratio (OR) independently suggest that four variables may influence negatively survival of patients with a single ventricle: pH < or = 7.3 on admission (OR = 3.55), a non-left ventricular morphology of the main ventricular chamber (OR = 3.11), the presence of an obstacle on the aortic outflow (OR = 5.58) and a total anomalous pulmonary venous drainage (OR = 26.88).

The authors report their experience of mitral valve replacement in infants under 2 years of age. A St Jude medical mitral prosthesis was implanted in 8 children, 7 with mitral regurgitation and 1 with mitral stenosis. The average age at surgery was 9 months with an average body weight of 6.6 Kg. Two children had prior mitral valvuloplasty. All patients were in the NYHA functional Class IV. One patient died after surgery (12.5%). There was one secondary death during follow-up. Six children were followed up for an average of 61 months (range 34 to 104 months). The 6 survivors are asymptomatic and receive oral anticoagulant therapy. Regular postoperative Doppler echocardiographic assessment has shown a gradual increase of the mean transprosthetic pressure gradient in all children. To date, no reoperation on the prosthetic valve has been necessary. Mitral valve replacement is an effective option in infants with severe cardiac failure in whom mitral valve repair is impossible. In these cases, the St. Jude medical prosthesis is a good choice. Effective anticoagulation is recommended, even at this age. Growth of the child will necessitate changing the prosthesis in later years.

The evaluation of the growth of the cardiac transplant in children was studied in four young children and three infants by echocardiography after orthotopic transplantation. These children were all under 13 years of age at the time of transplantation and have been followed up for more than two years. The age of the recipients ranged from 2 days to 12.8 years (average 10.7 years) and that of the donors from 7 days to 27 years (average 6.7 years). All patients received triple immunosuppressor therapy. The follow-up was 29 to 48 months (average 39.7 months) in the infants and 28 to 71 months (average 50.25 months) in the children. In the 7 patients the global follow-up period ranged from 28 to 71 months (average 45.8 months). No episodes of acute rejection or hypertension were observed during the study period. The echocardiographic parameters studied were the left ventricular end diastolic dimension, left ventricular end systolic dimension, left ventricular mass, left ventricular mass index. The date was gathered prospectively during the study at monthly intervals after the 3rd postoperative month. The donor/recipient weight ratio varied from 0.83 to 5 (average 1.89). The growth of the recipients was normal in 5 out of 7 cases and moderately retarded in 2 cases. The left ventricular end diastolic dimension, left ventricular end systolic dimension and left ventricular mass increased linearly with the body surface area of all patients. The growth was faster in the infant patients.(ABSTRACT TRUNCATED AT 250 WORDS)

The prognosis of right ventricular failure in patients with mitral valve disease would seem to depend on its chronicity and progression according to the results of a study of 382 cases of mitral valve disease: 231 women, 151 men: average age 55 +/- 13 years, operated between January 1983 and December 1986, most (321) for mitral valve replacement, one third (108) with associated aortic valve replacement. Right ventricular failure was or had been present before surgery in 161 patients: the peri-operative mortality was 11.2% and the secondary mortality 21.7% in these cases, significantly higher than in patients without RVF (respectively 5.0%, p = 0.02, and 8.1%, p < 0.0005). The RVF was present in the immediate post-operative phase in 43 patients, 8 of whom had no signs of this complication beforehand. The cause of this "de novo" early RVF is multifactorial. Ten other patients developed secondary RVF without any obvious cause in 3 cases. The prognosis of "de novo" RVF is very poor (28% survivors at 75 months). At the end of 60.4 +/- 13.6 months 20 patients were lost to follow-up, RVF was present in 60/286 patients followed up. The mortality was high in the patients with residual RVF (38.3% vs 4.0% in patients without: p < 0.0001). This study confirms the poor prognosis of RVF in patients with mitral valve disease whatever the timing with respect to surgery. Right ventricular failure occurring for the first time after surgery is rare (4.7% of cases), of uncertain cause, and carries a particularly poor prognosis.

Accelerated atherosclerosis of cardiac grafts is one of the factors limiting long-term survival after cardiac transplantation. The authors report the case of a patient who had a cardiac arrest associated with severe atherosclerosis 18 months after transplantation. The severity of the coronary lesions was underestimated by coronary angiography. An ergometrine test induced coronary spasm, a phenomenon which has only rarely been observed in transplanted hearts. The patient died one month later despite calcium inhibitor therapy. Autopsy revealed very severe triple vessel disease. This case illustrates the possible rapid evolution of coronary artery disease in cardiac transplant recipients, the difficulty in evaluating the severity of the lesions by coronary angiography and the additional possibility of observing coronary spasm in these cases.

The poor results of palliative surgery in the hypoplastic left heart syndrome has led Bailey to propose neonatal cardiac transplantation for this condition. The authors report the case of a neonate who underwent transplantation 2 days after birth for hypoplastic left heart with mitro-aortic atresia, diagnosed in utero. The child was prescribed triple immunosuppressive therapy (steroids, azathioprine, ciclosporine) and is now 24 months old. An episode of acute rejection during the first month was treated with a short intensive course of intravenous steroids. At the fourth month, the child underwent aortic angioplasty for an isthmic stenosis. Growth is retarded (- 2SD) and he has mild renal failure but psychomotor development has been normal. The number of neonatal cardiac transplantations remains limited by parental refusal and the lack of donor organs. Despite encouraging medium term results, questions remain as to the long-term viability of the graft and the patient's renal function.

Ninety-three cardiac transplantations were carried out in 91 patients (2 retransplantations) between March 1st 1987 and November 1st 1989, in 84 adults and 7 children under 15 years of age. The indications were dilated cardiomyopathy (48%), ischemic cardiomyopathy (35%), decompensated valvular heart disease (11%), congenital heart disease (3%) and two cases of Uhl's anomaly. Twelve patients underwent transplantation after external circulatory assistance (13%), 11 patients after inscription on the list of extreme emergencies, and 68 on an elective basis (74%). The postoperative immunosuppressive protocol was triple therapy: Ciclosporine, Azathioprine and Prednisone. Three of the children died. The early adult mortality was 9 cases (10.7%). It was 8% in patients operated electively. Major infectious complications occurred in 10 patients (11%). Rejection was looked for by systematic endomyocardial biopsy and echocardiography. Three hundred and forty-nine biopsies were made. Thirty-five patients (44%) had no problems of rejection. Seventy-nine patients have now been followed up for an average of 19 months. There were 7 late deaths. Seventy seven per cent of the survivors are asymptomatic. Acute rejection and transplant dysfunction were the two main causes of early mortality after cardiac transplantation. Although the long-term prognosis is uncertain, the medium-term results are very encouraging.

A retrospective study of 150 children under 3 months of age who underwent repair of coarctation of the aorta in the same center (between 1972 and 1987) was undertaken to assess the problems posed by surgery without cardiac catheterisation or angiography. The patients were divided into two groups: Group A, comprising 104 children operated after invasive investigations, and Group B, comprising 46 children operated on Doppler echocardiographic data alone. The two populations were comparable and "hypoplastic aortic arch" type coarctation was present in over 60% of cases. However, there were more ventricular septal defects in Group B (67.5%) than in Group A (49%). There were no significant diagnostic errors in Group B (one case of interrupted aortic arch diagnosed at surgery). The 1 month survival was the same in the two groups (82%). The indications of pulmonary artery banding were less frequent in Group B although there were more ventricular septal defects in these patients. These results confirm the value of Doppler echocardiography in the context of urgent surgery of congenital heart disease.

Twenty-four patients with a Saint-Jude Medical tricuspid valve prosthesis, aged 5 to 77 years, were studied. The etiology of the tricuspid lesion was rhumatic in 17 cases, infectious in 4 cases, and congenital in the other 3. Fourteen patient (58%) had undergone previous valve surgery, 7 of whom had undergone tricuspid valve replacement (TVR) by a bioprosthesis. Three patients were operated on for the third time. The TVR was isolated (4 cases) or associated with aortic valve replacement (3 cases), mitral valve replacement (8 cases), double aortic and mitral valve replacement (7 cases), repair of a ventricular septal defect (VSD) (1 case) and radical treatment of a Wolff-Parkinson-White syndrome (WPW) in 1 case. There were 3 early deaths (12.5%). Eighteen of the 21 survivors were followed up clinically, biologically (detection of hemolysis) and by Doppler echocardiography for an average period of 45 months (range 10 to 96 months). The clinical benefit was clear cut. No embolic complications were observed and there were no cases of hemolysis. The mean resting tricuspid pressure gradient was 3.57 +/- 2.36 mmHg. The Saint-Jude Medical prosthesis would therefore seem to be a good alternative to other mechanical valve prosthesis in the tricuspid position and without the risk of valve degeneration associated with bioprosthesis.

The authors report two cases of anomalous origin of the left coronary artery from the pulmonary artery in the adult. The two patients were pauci-symptomatic and were successfully operated, the one by reimplantation of the left coronary artery in the aorta and the other by an internal mammary artery left anterior descending artery bypass. Resting and stress myocardial scintigraphy and radionuclide ventriculography were performed before and after surgery in both cases. An analysis of segmental wall motion was possible in one patient. Before surgery, there was hypo-fixation of the tracer during the stress test and an alteration of left ventricular function. Postoperative isotopic investigations confirmed the efficacy of surgery the absence of regional ischemia and the normalisation of the ventricular contraction. These results argue in favour of a surgical reconstruction of a two coronary system, given the spontaneous risk of sudden death in this condition.

Eleven patients aged 7 to 58 years were placed on assisted circulation with Pierce (2 cases) or Abiomed (9 cases) external prosthetic ventricles as a bridge to cardiac transplantation. The indications were terminal cardiac failure following cardiomyopathy (7 cases), decompensated ischemic heart disease (3 cases) and subacute post-transplantation rejection (1 case). The duration of the assisted circulation ranged from 24 hours to 11 days. All patients were transplanted but 3 died after transplantation (27%). The circulatory assistance was satisfactory in all patients as shown by the regression of clinical signs of low cardiac output and the normalisation of diuresis. The complications observed during assisted circulation and after cardiac transplantation were: haemorrhage (36%), infection (27%) and thromboembolism (9%). These preliminary results with a 72% post-transplantation survival rate, show that both systems are effective "bridges to cardiac transplantation". The Abiomen device is excellent value for money and relatively simple to install and represents a good compromise between the sophisticated techniques of circulatory assistance and the problems of the cost of health care.

Twenty-five patients aged 31 to 74 years (average 50 years) operated for type A aortic dissection (type I: 19 cases, type II: 6 cases) were included in this study. Surgical repair only concerned lesions of the ascending aorta. The hospital mortality was 20 per cent (5 cases), and usually secondary to extension of the dissection. With the exception of 2 late deaths, all patients were followed up for an average of 3.5 years. A late assessment including nuclear magnetic resonance imaging of the thoracic aorta was obtained in 17 of the 18 survivors. These investigations confirmed the good result of repair of the ascending aorta, the uselessness of systematic aortic valve replacement and the palliative nature of repair of type I dissection as 80 per cent of patients had a persistent patent false lumen in the distal aorta.

Between 1973 and 1989, 81 consecutive patients aged 2 to 42 years old, with ventriculo-pulmonary discontinuity, were treated by implantation of prosthetic conduits. The initial pathology was Tetralogy of Fallot (33%), complete transposition of the great arteries (20%), truncus arteriosus (17%), double outlet right ventricle (17%) and atrioventricular discordance with L malposition of the great arteries (10%). The overall early mortality was 22% (18 cases) and 14% (5 cases) in the 36 patients operated after 1982. Sixty three patients were followed up for 3 months to 16 years; there were 8 late deaths which occurred spontaneously or at reoperation. Postoperative catheterisation was carried out in 33 cases; the average ventriculopulmonary systolic pressure gradient was 40 +/- 26 mmHg. Six patients were reoperated to change the conduit, on average 6 years +/- 23 months after the first operation. Five other patients underwent endoluminal dilatation of a stenosed conduit which delayed reoperation to change the conduit in 3 cases. Prosthetic conduits have been extensively used in patients with ventriculo-pulmonary discontinuity because they are readily available. However, because of progressive degradation of the prostheses between the 5th and 10th postoperative years, other therapeutic solutions should be considered, i.e. endoventricular repair when possible and, in other cases, the use of aortic homografts.

Seven children or adolescents aged 8 to 20 years (average 13.5 years) underwent implantation of an apico-aortic conduit from 1980 to June 1989. Eleven previous operations under cardiopulmonary bypass had been performed. The indications were recurrence of muscular subaortic stenosis and/or congenital valvular stenosis in 3 cases, and stenotic, previously implanted aortic valve prosthesis of small calibre in 4 cases. The preoperative left ventricular-aortic systolic pressure gradient was between 70 and 130 mmHg. The first two patients had a bioprosthetic valvulation and the 5 succeeding patients a St Jude Medical mechanical prosthesis. The early and late mortality was nil. The average follow-up period is 5, 6 years at present (range 6 months to 9 years). One patient had to undergo repeat valve replacement after 5 years for degenerescence of the porcine bioprosthesis implanted in the conduit. No other complications related to the conduit or valve were observed. At the endpoint of the study all patients were asymptomatic without treatment. Control echocardiographic data showed normalisation of the indices of left ventricular function. Apico-aortic conduits would seem to be a safe and effective technique for the treatment of recurrent severe obstruction of the left ventricular outflow tract.

Fifty infants with isolated total anomalous pulmonary venous connection (TAPVC) were operated between 1/01/73 and 31/12/87. The average weight at surgery, which was performed under hypothermia with circulatory arrest in 92 per cent of cases, was 4.5 Kg. The preoperative pulmonary to systemic pressure ratios (PAP/PS) enabled identification of two groups of patients: Group I: TAPVC without severe pulmonary hypertension (PAP/PS less than 0.85) (n = 35), and Group II: TAPVC with severe pulmonary hypertension (PAP/PS greater than 0.55) (n = 15). The hospital mortality was 22 per cent (8 cases) in Group I compared with 73 per cent (11 cases) in Group II (p less than 0.05). Patients in Group II were younger (64 days compared with 137 days, p less than 0.02), lighter (p less than 0.05) and had preoperative mean pulmonary artery systolic pressures of 83 mmHg (p greater than 0.001). Three patients in Group I required early reoperation for stenosis of the pulmonary veins at the site of repair resulting in pulmonary hypertension, and all died. The global survival was 28 patients with an average follow-up of 7 years (range 1 to 15 years). Six of these patients were reoperated (2 phrenoplications, 4 atrial shunts). All survivors are asymptomatic and have no conduction defects. Control echocardiography in 15 of the 28 survivors was judged to be normal. These results show that obstructive forms of TAPVC (Group II) carry a very poor prognosis: immediate results in this group could only be improved by earlier surgery. The clinical long-term results in those who survive surgery are very satisfactory.

Ten patients aged from 60 to 73 years presenting with Horton's disease or polymyalgia rheumatica had arteritis of the upper limbs. Asymptomatic abolition of pulse in the upper limbs (1 case) or claudication at rest or exercise (9 cases) and/or Raynaud's phenomenon (5 cases) preceded (4 cases) or accompanied (1 case) the discovery of giant cell arteritis, or complicated the reduction or discontinuation of corticosteroid therapy. Diagnosis rested on the regular association of an inflammatory syndrome with multiple arterial tapered stenoses and/or arterial thrombosis in the post-vertebral subclavian, axillary or brachial arteries and, chiefly, on the demonstration (in 7 cases) of a giant cell granuloma at biopsy of the temporal artery. Corticosteroid therapy (1 mg/kg/24 h in 8 cases and 0.5 mg/kg/24 h in 2 cases) initially combined with anticoagulants in 4 cases resulted in rapid regression of ischaemic and systemic signs in all patients, thus avoiding surgical revascularization of the upper limbs.

Between 1972 and 1984, 141 infants of less than 12 months of age were operated upon for coarctation of the aorta. The abnormality was isolated in 41 cases (29 p. 100) and associated with ventricular septal defect (VSD) in 58 cases (41 p. 100), with transposition of the great vessels with or without VSD in 16 cases (11.3 p. 100), with cardiac valve disease with or without VSD in 11 cases (7.8 p. 100) or with miscellaneous intracardiac lesions in 15 cases (10.6 p. 100). Resection-anastomosis (Crafoord) was performed in 89 cases (63 p. 100), subclavian flap aortoplasty (Waldhausen) in 36 cases (26 p. 100) and dacron aortoplasty in 16 cases (11 p. 100). Cerclage of the pulmonary artery was combined with one or another of these operations in 65 cases (46 p. 100). Twenty-five patients (17.5 p. 100) died within 30 days of the operation, and 28 patients (24.7 p. 100 of those who survived surgery) died at a later stage. Three infants were lost sight of. Follow-ups ranged from 1 to 13 years (mean: 4.01 years). Fifteen infants (13.3 p. 100 of those who survived surgery) were reoperated upon for recurrence of the coarctation. Four infants (3.5 p. 100) now present with clinical signs of recoarctation and are awaiting treatment.

Among the 628 consecutive patients who underwent coronary bypasses performed by the same surgical group between January 1, 1982 and December 31, 1987, 71 (11 p. 100) were aged 70 years or more (mean: 72.5 years; range: 70-83 years). 99 p. 100 of the patients had a history of severe, disabling exertional angina (46 p. 100) or unstable angina (52 p. 100) of 55 months' duration on average; 35 patients (49 p. 100) had already experienced myocardial infarction. Coronary angiography showed a one-vessel disease in 1 case, a two-vessel disease in 1 case, a two-vessel disease in 31 cases and a three-vessel disease in 39 cases; 11 patients (15 p. 100) had stenosis of the left main coronary artery. The ventricular function was considered unaltered in 59 cases (83 p. 100). Altogether, 155 bypasses, including 25 internal mammary grafts, were performed, i.e. an average of 2.2 bypasses per patient. There was only one early (21st day) post-operative death. The post-operative period was uneventful in 57 patients (60 p. 100); 9 developed peri-operative necrosis. Seventy patients have been followed up for a mean period of 24 months: there were 3 late deaths of non-cardiac origin; 60 patients (84.5 p. 100) are now asymptomatic and 3 (4.5 p. 100) are suffering from residual angina. Early mortality excluded, the cumulative probability of survival at 5 years is 94 p. 100.(ABSTRACT TRUNCATED AT 250 WORDS)

From 1974 till November, 1986, 268 adult patients aged from 15 to 84 years (11 p. cent over 70) underwent isolated aortic valve replacement by Björk-Shiley prosthesis. 81 p. cent of the prosthetic valves implanted were size 21 or over. The initial lesion was aortic stenosis (AS) in 40 p. cent, aortic regurgitation (AR) in 35 p. cent and aortic disease (AD) in 25 p. cent of the cases. The aortic valve lesions were degenerative (46 p. cent), rheumatismal (18 p. cent), congenital (12 p. cent), infective (19 p. cent including acute infective endocarditis) or dystrophic (7 p. cent) in origin. Pre-operatively, 54 p. cent of the patients were in NYHA functional class III and 14 p. cent in NYHA functional class IV. 7 p. cent presented with permanent atrial fibrillation. Mean pre-operative cardiac index was 2.49 1/mn/m2. Peri-operative mortality (up to 30 days) was 7.09 p. cent in both the 1974-79 period and the 1980-86 period; it was 3.4 p. cent in AS, 7.4 p. cent in AD and 10.7 p. cent in AR. The 249 survivors were followed up for a mean period of 5.3 years, or 1313 patient-years. 30 patients (11 p. cent) died subsequently (11 of prosthesis-related cause), giving an actuarial survival rate of 81.7 p. cent at 5 years and 71.4 p. cent at 10 years. 12 patients were reoperated upon, including 2 for prosthesis-related reasons (2.4 p. cent patient-years).(ABSTRACT TRUNCATED AT 250 WORDS)

The cases of 73 patients undergoing valvulotomy for congenital valvular aortic stenosis between 1957 and 1982 were reviewed. Data was updated after recalling patients to the outpatient clinic and/or analysis of the results of a questionnaire sent to the patient's family doctor or cardiologist. Babies less than 12 months old at the time of surgery were excluded from the study. Operation consisted of valvulotomy under direct control with few associated procedures as the valvular lesion was isolated in 89 p. 100 of cases. 5 patients died in the first 30 postoperative days, an operative mortality of 5,4 p. 100. The follow-up period ranged from 1 to 25 years, with 15 patients having been followed up for over 10 years. 6 patients were reoperated with no operative mortality. 2 of whom have since undergone a second reoperation. Of the 59 patients not re-operated, 54 were class I and 5 class II of the NYHA. Of the latter group, 4 are candidates for aortic valve replacement for significant aortic regurgitation. The actuarial survival graph shows a 92.82 p. 100 probability of survival at 5 years, and 86.83 p. 100 at 10 years. Aortic valvulotomy remains a palliative operation which does not protect the patient from subsequent sudden death.

The limitations of medical treatment in angina pectoris depend on its efficacity and indications. 1. Efficacity of medical treatment.--Anti-anginal drugs are able to relieve anginal pain in at least 3/4 of cases. However, the ability of medical measures (diet, exercise, stopping smoking, hypolipidemic drugs, antihypertensive drugs, anticoagulants, platelet anti-aggregants, antiarrhythmics, inotropic agents, vasodilators and diuretics) to prevent coronary atherosclerosis delay its progression and prevent its complications--so increasing the life expectancy of coronary patients--remains very uncertain. 2. Frontiers and judications of medical treatment.--Coronary patients with few or no symptoms appear to be best suited for long-term anti-anginal treatment with long acting nitrate derivatives and/or betablockers. The ability of the latter group to increase the life expectancy of all coronary patients remains to be shown. The limits of the indications of medical treatment are more difficult to define either by purely subjective criteria (incapacitating angina after trials of anti-anginal drugs at adapted doses) or by "objective" criteria (ergometry, coronary angiography).

Between January 1, 1982 and January 1, 1988, 49 complete corrections of complete atrioventricular canal were performed in children aged from 4 months to 8 years. 41 were infants less than 2 years' old and 31 were less than one year old. In the last 35 patients the "composite double patch" technique was used, consisting of closure of the interventricular septal defect with a dacron patch, followed by closure of the ostium primum with a pericardial patch. The mitral cleft was left intact in the last 6 operations. The overall mortality rate was 35 p. 100 (17 patients). It was 23 p. 100 in infants under 1 year and 17 p. 100 in infants aged from 6 to 12 months at the time of surgery (p less than 0.01). Seven of the 35 children in whom the "composite double patch" technique was used died (20 p. 100). Only one early death was recorded among the last 15 children operated upon. Two reoperations were performed: one within one month of the first operation, the other 4 months later for residual mitral regurgitation with haemolysis. 32 children were followed up for periods of 2 months to 6 years, 10 of them for more than 3 years. Two late deaths occurred during the follow-up. Grade 2 or 3/4 residual regurgitation was found in 14 patients who have regular clinical and echocardiographic examinations.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary cardiac tumours are rare. They are usually benign, the most common ones being left atrial myxomas. The authors report their experience of 16 cardiac tumours operated between 1978 and 1985. The patients were 12 adults and 4 children. The tumours were benign in 14 cases and malignant in 2 cases. Complete (14 cases) or incomplete ablation of the tumour was carried out under cardiopulmonary bypass with an early mortality of 6.25 per cent (1 case) and late mortality of 12.5 per cent (the two malignant tumours). The tumours were identified as myxomas in 11 cases, rhabdomyomas in 2 cases, fibroma in 1 case and malignant sarcomas in 2 cases. The average follow-up period of the 13 survivors is 30 months (range 4 months to 5 years) with excellent clinical and anatomical results. Clinical and paraclinical diagnosis of these tumours does not pose any major problems nowadays because of the reliability of the methods of investigation. However, the nature of the tumour is not always predictable and the operative findings are fundamental, not so much for the treatment which is relatively stereotyped but for the prognosis. Age is not a significant prognostic factor as excellent results may be obtained in the very young and the very old despite a sometimes precarious preoperative clinical condition. The benign or malignant nature of the tumour is the only real factor which affects the prognosis.

The immediate and long-term results of aortic valve replacement for pure or predominant aortic valve stenosis were evaluated in 186 patients operated upon since 1975 and followed for up to 10 years. This population fell into two groups depending on whether the pre-operative cardiac index was superior (group I, n = 111) or inferior (group II, n = 75) to 2.3 l/min/m2. There was no significant difference between the two groups as regards the immediate (i.e. within 30 days) post-operative mortality rate (6.6% vs 8.1% respectively). In contrast, the cardiac index proved to be a significant post-operative prognostic factor in aortic stenosis, since the probability of survival at 5 years was 96.4% in group I and only 71.7% in group II (p less than 0.001). This high rate of mortality in group II was exclusively due to myocardial dysfunction (sudden deaths included) in these patients with low cardiac index. When late mortality was analyzed according to age (over or below 60 years) and to pre-operative cardiothoracic ratio (over or below 50), these two criteria also proved to be significant prognostic factors. However, considering the poor prognosis of unoperated aortic stenosis, these long-term results in group II should encourage surgical treatment in many cases, even those with advanced cardiopathy.