Transthyretin amyloid polyneuropathy in France: A cross-sectional study with 413 patients and real-world tafamidis meglumine use (2009-2019).

OBJECTIVE: We aimed to describe characteristics of patients with ATTR variant polyneuropathy (ATTRv-PN) and ATTRv-mixed and assess the real-world use and safety profile of tafamidis meglumine 20mg. METHODS: Thirty-eight French hospitals were invited. Patient files were reviewed to identify clinical manifestations, diagnostic methods, and treatment compliance. RESULTS: Four hundred and thirteen patients (296 ATTRv-PN, 117 ATTRv-mixed) were analyzed. Patients were predominantly male (68.0%) with a mean age of 57.2±17.2 years. Interval between first symptom(s) and diagnosis was 3.4±4.3 years. First symptoms included sensory complaints (85.9%), dysautonomia (38.5%), motor deficits (26.4%), carpal tunnel syndrome (31.5%), shortness of breath (13.3%), and unexplained weight loss (16.0%). Mini-invasive accessory salivary gland or punch skin and nerve biopsies were most common, with a performance of 78.8-100%. TTR genetic sequencing, performed in all patients, revealed 31 TTR variants. Tafamidis meglumine was initiated in 156/214 (72.9%) ATTRv-PN patients at an early disease stage. Median treatment duration was 6.00 years in ATTRv-PN and 3.42 years in ATTRv-mixed patients. Tafamidis was well tolerated, with 20 adverse events likely related to study drug among the 336 patients. CONCLUSION: In France, ATTRv patients are usually identified early thanks to the national network and the help of diagnosis combining genetic testing and mini-invasive biopsies.

[Transthyretin cardiac amyloidosis]. / L'amylose cardiaque à transthyrétine.

Transthyretin (TTR) cardiac amyloidosis results from the dissociation of the tetrameric, liver-synthetized transport protein, either because of a mutation (hereditary CA), or spontaneously due to ageing (wild type CA). Monomers self-associate into amyloid fibrils within the myocardium, causing heart failure, arrhythmias and conduction defects. This overlooked disease must be recognized in case of unexplained increased thickness of the myocardium, particularly in subjects of African descent, in patients with heart failure and preserved ejection fraction, and in those with aortic stenosis. Some extra-cardiac symptoms must also be considered as red flags: carpal tunnel syndrome, lumbar canal stenosis, recent deafness, peripheral neuropathy, or dysautonomia. Medical assessment includes an electrocardiogram, biological assessment including troponin, natriuretic peptide and monoclonal protein assay, echocardiography with 2-D strain study, MRI and bone scintigraphy. Once the diagnosis established, cardiologic management must avoid beta-blockers and other rate-slowing drugs, which are deleterious in restrictive cardiomyopathy, and restrain the use of renin-angiotensin system inhibitors, of little use and often poorly tolerated. Congestion must be treated with diuretics. Anticoagulants are often necessary due to the risk of arrhythmias and stroke. Pacemaker or defibrillator implantation should be determined in patients with high risk of sudden death. Until now, etiologic treatments were liver and/or heart transplantation in some rare cases. Tafamidis, a TTR stabilizer has recently been approved, and new therapeutic approaches targeting TTR at the transcriptional level are under investigation.

Measurement of interatrial dyssynchrony using tissue Doppler imaging predicts functional capacity and cardiac involvement in systemic sclerosis.

OBJECTIVES: We aimed to assess the prevalence of interatrial electromechanical dyssynchrony in systemic sclerosis (SSc) patients, and to study the correlation between interatrial delay and standard follow-up parameters. METHODS: Forty consecutive patients with SSc were studied. Classical echocardiographic measurements were obtained, including indices of left ventricular (LV) systolic and diastolic function, right ventricular function, and pulmonary artery pressure (PAP). Left atrial (LA) function was studied using volume measurements. The interatrial mechanical (IAMD) delay was obtained by measuring the time delay between the peak atrial velocities at the lateral tricuspid and mitral annuli using tissue Doppler imaging. A cut-off value of 35 ms was chosen to define the presence of a significant interatrial delay. The IAMD was compared to NYHA class, six-minute walking test (6MWT), NT proBNP levels, and the carbon monoxide diffusion capacity over alveolar volume ratio (DLCO/VA), as well as to classical echocardiographic parameters. RESULTS: Forty percent of patients were found to have significant interatrial dyssynchrony with an IAMD of 35 ms or more. Patients with interatrial dyssynchrony were more symptomatic, had a shorter 6MWT, higher NT proBNP levels, and a lower DLCO/VA compared with those without dyssynchrony. Regarding conventional echocardiographic parameters, increased IAMD was associated with more pronounced LV diastolic dysfunction, LA enlargement and dysfunction, altered RV function, and higher PAP. CONCLUSIONS: IAMD correlated with all of the standard follow-up parameters in SSc, and is probably a sensitive marker of LA involvement. This easy to measure parameter should be added to the routine echocardiographic assessment of these patients.

Severe cardiomyopathy revealing antineutrophil cytoplasmic antibodies-negative eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic vasculitis in which cardiac involvement is frequent and severe, and accounts for half of EGPA-related deaths. ANCA-positive EGPA differs from ANCA-negative EGPA in that the former is significantly associated with renal involvement, peripheral neuropathy and biopsy proven vasculitis, whereas the latter is associated with cardiac involvement. Herein, we report a case of EGPA with myocarditis in a woman, who was successfully treated with steroids and cyclophosphamide. This report highlights the importance of diagnosing cardiac involvement in EGPA early, especially in ANCA-negative patients.

[Cardiovascular manifestations of eosinophilia: clinical and echocardiographic presentation]. / Atteintes cardiaques au cours des hyperéosinophilies: une présentation clinique et échocardiographique polymorphe.

INTRODUCTION: Endomyocardial fibrosis with apical obliteration is the best known involvement among heart lesions induced by hypereosinophilia. However, hypereosinophilic heart disease may involve all three heart layers, with a polymorphic clinical and echocardiographic presentation. METHODS: Retrospective descriptive study of five patients highlighting the various manifestations of hypereosinophilic heart disease. RESULTS: We report five cases illustrating the variety of hypereosinophilic heart disease and review the pathophysiology of this potentially severe illness: cytotoxicity of eosinophils is mediated by the release of granular proteins that primarily damage the endocardium, leading to thrombosis and embolic complications, then to fibrosis and valvular complications; myocardial involvement may induce a dreadful acute eosinophilic myocarditis; finally, pericardial involvement may cause tamponade. CONCLUSION: These observations highlight the whole spectrum of the heart manifestations of hypereosinophilia, remind that the vital prognosis may be involved in the acute phase and underline that functional prognosis depends on early detection and treatment to reduce the risk of early thromboembolic and late fibrotic complications.

Low-frequency electromyostimulation and chronic heart failure.

Low-frequency electromyostimulation (EMS) acts on the skeletal muscle abnormalities that aggravate intolerance to effort in patients with chronic heart failure (CHF). It improves the oxidative capacity of muscles and thus enhances aerobic performance and physical capacity to almost the same degree, as does conventional physical training. No local or hemodynamic intolerance has been reported, even in cases of severe CHF. However, the presence of a pacemaker is one of the relative contra-indications (prior evaluation of tolerance is required), while that of an implanted defibrillator is one of the absolute contra-indications. EMS is an alternative to physical effort training when the latter is impossible due to a high degree of deconditioning or because there is a contra-indication, which may be temporary, due to the risk of acute decompensation and/or rhythm troubles. EMS can also be used in patients waiting for a heart transplant or in CHF patients who are unwilling to engage in physical activities. As EMS is not expensive and easy to set up, its use is likely to develop in the future.

[Acute heart failure and preserved systolic function: can we explain all only by the diastolic dysfunction? A prospective study on 145 patients hospitalized for acute pulmonary edema]. / Insuffisance cardiaque aiguë et fonction systolique préservée: peut-on tout expliquer par la seule dysfonction diastolique? Etude prospective sur 145 patients hospitalisés pour OAP.

INTRODUCTION: Heart failure with conserved systolic function is frequent and attributed to the diastolic dysfunction. The diagnosis of diastolic heart failure requires the association of clinical signs of heart failure, a conserved left ventricular systolic function and a diastolic dysfunction. OBJECTIVE: To determine the proportion of cases of isolated diastolic heart failure among patients hospitalized for acute pulmonary edema. METHODS: The left ventricular ejection fraction (LVEF), the diastolic function and levels of NT-proBNP have been assessed at admission of 145 patients hospitalized for acute pulmonary edema. RESULTS: 49% of patients included were older than 80 years (mean age 78.6 + 0.9 years). Among the 83 patients with conserved LVEF, 25% had an ischemic heart disease, 24% a severe valvular disease, 22% an atrial fibrillation, 5% a severe bradycardia, 2% a severe hypertrophic obstructive cardiomyopathy. Only 15 patients presented an isolated diastolic heart failure. The level of NT-proBNP was correlated to LVEF but was not able to identify those with isolated diastolic heart failure in the group with "conserved systolic function". CONCLUSION: Among patients hospitalized for acute pulmonary edema, the prevalence of heart failure with conserved systolic function is high, but only 10% of them presented an isolated diastolic heart failure. The NT-proBNP levels do not permit to identify them.

Hypoxaemia associated with an enlarged aortic root: a new syndrome?

OBJECTIVE: To assess the mechanisms through which an enlarged aortic root may facilitate right to left shunting through a patent foramen ovale. PATIENTS: 19 patients with the platypnoea-orthodeoxia syndrome (POS) were compared with 30 control patients without platypnoea. INTERVENTIONS: Multiplane transoesophageal echocardiography. MAIN OUTCOME MEASURES: The aortic root diameter, atrial septal dimension behind the aortic root, and amplitude of the phasic oscillation of the septum were measured. Four groups of patients were compared: 12 platypnoeic patients with a dilated aortic root (POS-D), 7 platypnoeic patients with a normal aortic root (POS-N), 15 control patients with a dilated aortic root (CONT-D), and 15 control patients with a normal aortic root (CONT-N). RESULTS: In POS-D and CONT-D patients, the apparent atrial septal dimension was 16.3 (2.7) mm and 17.4 (5.9) mm respectively, compared with 24.4 (5.2) mm in POS-N patients and 25 (4) mm in CONT-N (p < 0.005). Furthermore, the amplitude of septal oscillation was 14.7 (2.5) mm in the POS-D group versus 5.8 (2.4) mm in CONT-N (p < 0.001) compared with 23.3 (3) mm in seven patients with an atrial septal aneurysm (p < 0.001). CONCLUSION: Patients with an enlarged aorta have an apparently smaller dimension and increased mobility of the atrial septum. These findings appear to result from compression by the aortic root and decreased septal tautness. Consequently, a "spinnaker effect" with the inferior vena caval flow may take place, opening the foramen ovale and leading to sustained right to left shunting.

Melatonin protects against ischemia-reperfusion injury and inhibits apoptosis in isolated working rat heart.

INTRODUCTION: Melatonin (MEL), a pineal hormone, is well known as a potent antioxidant in a variety of ischemia-reperfusion models. Recent studies have assumed a pivotal role of reactive oxygen species (ROS) in the development of apoptosis. There are few pieces of information concerning a possible protective role of MEL against apoptosis in ischemia-reperfusion injury of myocardium. METHODS: We conducted an in vitro experiment: (1) to study the effect of MEL in the model of isolated and perfused working rat heart; (2) to evaluate the antioxidant capacity of MEL by a simple fluorescence test; and (3) to analyze the extent of apoptosis inhibition by MEL. Four groups of male Wistar rat were used: (a) group 'MEL 50 muM' (n=8); (b) group 'ischemia 30 min' (n=8); (c) group 'controls' (n=8); and (d) group 'controls+MEL 50 muM' (n=8). The perfusion medium was an oxygenated Krebs-Henseleit buffer (KHB). Hearts in groups (a) and (b) underwent 30 min of global normothermic ischemia and 45 min of reperfusion; 3 min before ischemia the hearts of group (a) received KHB with MEL 50 muM (and MEL 50 muM was also present in KHB solution during reperfusion). Hearts of group (c) were only perfused by KHB, and hearts of group (d) perfused by KHB+MEL 50 muM throughout the experiment. Registered were basic hemodynamic parameters: coronary, aortic, cardiac output and heart rate. At the end of each experiment, a left ventricle samples were taken for in situ detection of apoptosis using a TUNEL in-situ detection kit (POD) and quantitative analysis was performed. Malonedialdehyde concentrations were evaluated from heart homogenate to determine the severity of oxidative damage. To study the antioxidant capacity of MEL, a fluorescence test with allophycocyanin as an indicator was performed. A peroxyl radical generator, 2,2'-azobis(2-amidinopropan)-4-hydrochloride (AAPH) was used, and the antioxidant effect of MEL was expressed in oxygen-radical absorbing capacity (ORAC) units. RESULTS: Treatment by MEL resulted in a significant improvement of hemodynamic parameters and reduction of postischemic arrhythmias during reperfusion. All hearts in group 'ischemia 30 min' developed fatal ventricular fibrillations. MEL significantly reduced the incidence of apoptotic cells (14+/-4.3%; **P<0.01) vs. group 'ischemia 30 min' (58+/-2.1%). No apoptotic cells were detected in both control groups (c) and (d). In the fluorescence test, MEL exhibited a significant dose-dependent protective effect against peroxyl radical; MEL also reduced significantly the level of lipoperoxidation (MDA; *P<0.05). Analysis of hemodynamic parameters in both control groups (c) and (d) did not show any significant differences; the presence of MEL 50 muM in KHB solution did not have any important influence on cardiac performance in this type of experiment. CONCLUSION: We confirmed the previously reported beneficial effects of MEL against ischemia-reperfusion injury, presumably via its antioxidant properties. A significant suppression of apoptosis and the peroxyl radical scavenging properties of MEL in our study could contribute to the hypothesis of a close link between oxidative stress and apoptosis promotion.

["Spontaneous" rupture of the left iliac vein complicating Cockett's syndrome]. / Rupture "spontanée" de la veine iliaque gauche compliquant un syndrome de Cockett.

The case history reported concerns a female patient aged 42 years for whom the clinical picture was that of a blue phlebitis (phlegmatia caerulea dolens), associated with a state of shock evoking a severe pulmonary embolus. The absence of echocardiographic dilatation of the right cavities, and the appearance of a left iliac fossa mass, steered the diagnosis towards internal haemorrhage. Emergency laparotomy allowed diagnosis and treatment of a so-called spontaneous rupture of the left iliac vein, a rare condition for which 20 cases have been reported in the literature. Re-operation performed 24 hours afterwards for the absence of venous return allowed the discovery of Cockett's syndrome with ascending thrombosis, requiring cross-venous bypass associated with the creation of an arterio-venous fistula in order to maintain permeability. One year afterwards the appearance of signs of cardiac insufficiency led to the closure of this fistula.

Prevention of apoptosis by deferoxamine during 4 hours of cold cardioplegia and reperfusion: in vitro study of isolated working rat heart model.

INTRODUCTION: Heart transplantation is often accompanied by multiple functional alterations, especially in reperfusion period. These are probably related to the reactive oxygen species (ROS) formation catalyzed by transition metals such as iron and copper, and thus the preservation time of the donor hearts is limited. Metabolic protection of the heart grafts is a permanent objective of numerous experiments. Recently, an iron chelator deferoxamine (DFX) was proposed as antioxidant agent for storage solutions in heart grafts. Oxidative stress is also known to mediate the apoptotic cell death in different tissues during ischemia-reperfusion. METHODS: The aim of this study was to evaluate a possible role of DFX in prevention of apoptosis using in vitro model of isolated working rat heart and cold cardioplegia. Two groups of rats were evaluated: (a) group 'DFX 50 &mgr;M' (n=8) and (b) group 'controls' (n=8). Isolated rat hearts were perfused by Krebs-Henseleit buffer (KHB) for 30 min, arrested by cardioplegic solution and stored for 4 h in B21 solution at 4 degrees C. Then, the hearts were reperfused by KHB for 45 min. DFX was added to the cardioplegic and storage solutions and in KHB in reperfusion. Basic functional parameters were evaluated: coronary, aortic, cardiac outputs and heart rate. At the end of reperfusion period a tissue samples were taken from left ventricle and in situ detection of apoptotic cells was performed using an ApopTag kit. RESULTS: DFX significantly reduced the occurrence of apoptotic cells in myocardium (*P<0.05). Hearts treated by 50 &mgr;M of DFX showed also a better recovery of the cardiac output (***P<0.001). The presence of DFX in KHB, cardioplegic and storage solution reduced also the incidence of postischemic arrhythmias and fibrillation's but without statistical significance. CONCLUSIONS: Our results give evidence of the protective potential of DFX during cold ischemia and reperfusion, presumably due to its antioxidant properties. The significant decrease of apoptosis in hearts treated by DFX could be considered as an existence of close link between oxidative stress and apoptotic death promotion in ischemia-reperfusion injury.

[Reversible cardiomyopathy induced by psychotropic drugs: case report and literature overview]. / Myocardiopathie réversible induite par les médicaments psychotropes à propos d'un cas, revue de la littérature.

A number of psychotropic drugs, including tricyclic antidepressants, phenothiazine and lithium, have a well demonstrated risk of cardiotoxicity. Each individual therapeutic class has potentially deleterious effects on electrophysiology and myocardial function. The authors report a case showing how serious side effects may result from the association of these different classes in the presence of a coexistent heart disease, even when the underlying disease is mild.

[Cardiogenic shock complicating extensive infarction with ventricular septal defect. Circulatory assistance and heart transplantation]. / Choc cardiogénique sur infarctus étendu, compliqué d'une communication interventriculaire. Assistance circulatoire et greffe cardiaque.

A 47 year old man had a massive anterior myocardial infarction with cardiogenic shock with a left parasternal murmur. Coronary angiography showed occlusion of the left anterior descending artery for which angioplasty resulted in failure. There was antero-lateral-apical akinesia and a ventricular septal defect (VSD) with a left-right shunt (Qp/Qs = 1.54). Persistence and aggravation of haemodynamic instability led to intra-aortic balloon pumping with inotropic pharmacological support followed by biventricular assistance with a MEDOS device. Under transoesophageal echocardiographic monitoring, the outcome was marked over 7 days by the progressive increase in the shunt volume of the VSD, a decrease of drainage and injection flow, progressive increase in spontaneous contrast echos followed by the presence of fibrin in the cardiac chambers and canulae, the presence of thrombus in the external ventricles, blockage of the right external valve which only opened after increasing the degree of anticoagulation, and, finally, cardiac tamponade which required drainage before the patient's state improved. On the 8th day, the patient being stable with a normal neurological status, the availability of a donor heart led to the decision to transplant, which was carried out without complications. This case poses the problem of cardiac assist devices and their daily monitoring, and then that of cardiac transplantation in this indication.

[Long-term hemodynamic function of the transplanted heart]. / Fonction hémodynamique du coeur transplanté à long terme.

The aim of this study was to identify the long-term haemodynamic changes of the transplanted heart. Between 1987 and 1997, 136 patients required cardiac transplantation at Dijon hospital. During follow-up, 76 patients aged 51.2 +/- 9.46 years underwent catheter studies (12 women, 15.8%; and 64 men, 84.2%). Right and left heart catheterisation was performed at 3 months, 1, 2, 3 and 5 years after transplantation. Right heart catheterisation included measurement of mean pulmonary artery and pulmonary capillary pressures and pulmonary arteriolar resistances. During left heart catheterisation, cardiac output, mean aortic pressure, the ejection fraction, the dp/dt max of the left ventricular wall, systemic arterial resistances and left ventricular end diastolic pressures were measured. At each catheter study, the indexed myocardial mass, indexed end systolic and end diastolic left ventricular volumes, the mass/volume ratio, the residual serum cyclosporine concentrations and the serum creatinine were analysed. In addition, an endomyocardial biopsy was also performed. Initially raised, the mean pulmonary artery and pulmonary capillary pressures decrease from the 3rd month to the 2nd year. From the 3rd year onwards, they readjust to the upper limits of normal. The pulmonary artery resistances underwent the same changes. The left heart parameters remained constant over the period of follow-up but with a heart rate, mean aortic pressure and left ventricular end diastolic pressure higher than normal. The indexed myocardial mass was increased at all periods. The indexed left ventricular end systolic and diastolic volumes decreased with a M/V ratio which increased. Cyclosporine concentrations decreased whereas serum creatinine increased. The frequency of severe rejection and of coronary atherosclerosis was low. Significant correlations were observed between different parameters at different periods. In the long-term, the function of the transplanted heart is not normal in the strict sense of the term. The apparent normality is obtained by anti-hypertensive treatment. The transplanted heart adapts to the increase in cyclosporine-induced afterload by permanent myocardial hypertrophy, and increased diastolic pressure probably relates to diastolic dysfunction without noticeable intracardiac fibrosis.

[Randomized comparison of 4F and 6F catheters for diagnostic coronary angiographies via the femoral approach]. / Comparaison randomisée des cathéters 4 F versus 6 F pour la réalisation des coronarographies diagnostiques par voie fémorale.

The use of 6F catheters has been validated for coronary angiography. The use of small-caliber catheters is a more recent development. The aim of this study was to assess the feasibility, the cost and complications of coronary angiography using the femoral approach with 4F catheters. The authors undertook a randomized prospective study of 4F Care Infiniti catheters (N = 100) and 6F Spertorque Plus catheters (N = 100) in hospitalised patients. Criteria of non-inclusion were valvular pathology, acute myocardial infarction, aorto-coronary bypass or aorto-femoral bypass procedures. No statistical difference was observed between the two groups with respect to feasibility, to duration of the procedure, or of irradiation or to cost. The quality of the angiograms was good except in one patient in the 4F group; 4 patients in the 6F group required a 4F catheter to complete their examination. Left ventricular catheterisation was more difficult with 4F catheters (p = 0.016). Use of 4F catheters was associated with injection of significantly less contrast (p = 0.00007), reduced the duration of compression (p < 10(-6)) and its complications (p = 0.004). The authors conclude that 4F catheters are safe and well tolerated. They are associated with less patient morbidity, without any loss in quality of the angiogrammes. Other studies in valvular heart disease and after coronary bypass surgery should lead to the generalisation of their use in all coronary patients.

Antioxidative properties of pyruvate and protection of the ischemic rat heart during cardioplegia.

Formation of oxygen free radicals during heart transplantation seems to be related to the alterations occurring during ischemia and reperfusion and could explain the short preservation time of donor hearts. The aim of our study was (a) to analyze the protective effects of pyruvate during cold cardioplegia and ischemia/reperfusion sequence, and (b) to investigate in vitro the radical scavenging properties of this compound. After 30 min of perfusion, isolated working rat hearts were arrested by cardioplegic solution, stored 4 h in B21 solutions at 4 degrees C, and reperfused with Krebs-Henseleit buffer for 45 min. Pyruvate (2 mM) was added to Krebs-Henseleit, cardioplegic, and storage solutions, and functional parameters were recorded throughout the experiments. In a second part, control hearts and hearts treated with pyruvate were cannulated via the aorta and perfused for 30 min by the Langendorff method, arrested by cardioplegic solution, stored 4 h in B21 solutions at 4 degrees C, and reperfused for 45 min by the Langendorff method. Malonedialdehyde and alpha-tocopherol levels were determined on heart homogenate. In situ detection of apoptotic cells also was performed on tissue samples (left ventricle) at the end of the ischemia/reperfusion sequence. To demonstrate in vitro the antioxidant effects of pyruvate, we monitored (a) its hydroxyl radical scavenging properties by using electron paramagnetic resonance (EPR) spectroscopy, and (b) the decrease of fluorescence of allophycocyanin, in the presence of a Fenton system (H2O2/Cu2+). Ischemia for 4 h, followed by myocardial reperfusion, resulted in substantially reduced mechanical function. Hearts subjected to this ischemia and pretreated with pyruvate showed a significant improvement in the function recovery. After the ischemia/reperfusion protocol, no significant decrease of malonedialdehyde levels was shown on hearts treated with pyruvate. However, alpha-tocopherol levels were higher in the pyruvate group compared with the control group. At the end of the reperfusion period, levels of apoptotic cells were significantly lower in hearts treated with pyruvate compared with control hearts. EPR studies showed that pyruvate was an efficient hydroxyl scavenger, with a median inhibitory concentration (IC50) of 8 mM. The allophycocyanin assay also showed a dose-dependent effect of pyruvate against hydroxyl radicals. In conclusion, these findings showed that pyruvate could prevent reperfusion injuries in the isolated heart, probably by its antioxidative properties. The application of pyruvate may contribute to the preservation of hearts for organ transplantation.

[Ten-year experience with heart transplantation (1987-1997)]. / Bilan de dix années de transplantation cardiaque (1987-1997).

OBJECTIVES: Analyze ten years experience with heart transplantation at the Dijon University Hospital and determine which parameters control mid and long term outcome. PATIENTS AND METHODS: One hundred thirty six heart transplantations were performed over a 10 year period (1987-1997) in 118 men and 18 women aged 51-87 years. Heart transplantation was indicated on the basis of the following criteria: ejection fraction *20%, pulmonary arteriole resistance < 6 Wood units, peak oxygen uptake < 14 l/kg/min. The Shumway or anatomic technique was used. The triple immunosuppressive protocol combined corticosteroids, azathioprine and cyclosporin. The same team conducted the post-transplantation follow-up with regular programmed consultations in addition to those requested by the general practitioner, the cardiologist or the patient. Follow-up was oriented according to the clinical situation (blood chemistry, cell counts, cyclosporinemia, search for infection, echocardiography, endomyocardial biopsy, coronarography). RESULTS: Five patients (3.6%) died when still on the waiting list. Absolute emergency transplantation was performed for patients (28.1%) including 8 (5.9%) after circulatory assist. Hospital mortality was 11.7% and late mortality was 16.1%. Actuarial survival was 78% at 1 year, 71% at 5 years and 69% at 10 years. Among the survivors, 94% were taking two, three or even four drugs for hypertension. Cyclosporin levels decreased and creatinine levels increased. Episodes of rejection were minimal: 86.57% of the biopsies were * grade 1 and 4.45% * grade 2. Cytomegalovirus infection was documented and treated in 7.55% of the cases. Incidence of graft coronary artery disease was 3.4% at 1 year, 6.5% at 2 years and 7.9% at 3 years. CONCLUSION: Our follow-up structure where the same small team conducts regular examinations together with our approach to heart transplantation appears to be the main factor leading to the quality results obtained in this series.

[Long-term follow-up of anatomic heart transplantation. Apropos of 60 patients with a mean follow-up of 36 months]. / Suivi à long terme de la transplantation cardiaque anatomique. A propos de 60 patients dont le suivi moyen est de 36 mois.

The aim of this study was to collect different problems seen in long-term evolution of patients who had anatomical cardiac transplantation and to compare with those seen in patients with standard transplantation. During the mean follow-up of 36 months, we analysed different data of 60 patients mean aged 51, who underwent anatomical cardiac transplantation. Six patients (10%) died within the 30 days after surgery. No patient needed the use of permanent pacemaker. Echocardiographic examination found normal atrial shape. One month after surgery, echocardiography described 16 tricuspid regurgitations (22.66%) and 8 mitral regurgitations (13.33%), 1 year later, there was respectively 13.33 and 6.66% tricuspid and mitral regurgitation. We had 8 late deaths: 1 sudden death, 2 chronic rejections, 1 pancreatitis and 4 cancers. The survival analysis pointed out 84% at 1 year, 80 at 2 years, 78 at 3 years and 73 at 5 years. Six months after surgery, 80% of patients were treated for high blood pressure; 85% had serum creatinine level equal or superior to 13 mg/L, with mean serum ciclosporin at 130 ng/mL. At the 3rd month, 6 endomyocardial biopsies were equal or superior to grade 2 rejection (International Society for Heart Transplantation). Between the 3rd and 12th month, 3 endomyocardial biopsies were equal or superior to grade 2 rejection, and the same between the 12th and 24th month. The infections rate was 0.8 episode per patient. Long term follow-up of anatomical cardiac transplantation faces the same problems as in standard cardiac transplantation. It is better to perform anatomical cardiac transplantation because of its early postsurgical advantages. Long term care is the same as in standard cardiac transplantation.