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The Atlas of Genetics and Cytogenetics in Oncology and Haematology (http://AtlasGeneticsOncology.org) is a peer-reviewed internet journal/encyclopaedia/database focused on genes implicated in cancer, cytogenetics and clinical entities in cancer and cancer-prone hereditary diseases. The main goal of the Atlas is to provide review articles that describe complementary topics, namely, genes, genetic abnormalities, histopathology, clinical diagnoses and a large iconography. This description, which was historically based on karyotypic abnormalities and in situ hybridization (fluorescence in situ hybridization) techniques, now benefits from comparative genomic hybridization and massive sequencing, uncovering a tremendous amount of genetic rearrangements. As the Atlas combines different types of information (genes, genetic abnormalities, histopathology, clinical diagnoses and external links), its content is currently unique. The Atlas is a cognitive tool for fundamental and clinical research and has developed into an encyclopaedic work. In clinical practice, it contributes to the cytogenetic diagnosis and may guide treatment decision making, particularly regarding rare diseases (because they are numerous and are frequently encountered). Readers as well as the authors of the Atlas are researchers and/or clinicians.

Unlabelled: Carvedilol can induce important clinical and hemodynamic improvements in patients with chronic heart failure resulting from severe left ventricular (LV) dysfunction. This study examines the impact of carvedilol on cardiac neuronal function using 123I-metaiodobenzylguanidine (MIBG) scintigraphy in dilated cardiomyopathy., Methods: Twenty-two patients with chronic heart failure (19 men, 3 women; mean age, 54 y; age range, 34-64 y) assessed as New York Hospital Association (NYHA) class II or III and with initial resting radionuclide LV ejection fractions (LVEF) < 0.40 were enrolled in the study. Patients had long histories of symptomatic LV dysfunction despite optimal diuretics and angiotensin-converting enzyme inhibitor treatment. Over a 6-mo period, 50 mg/day carvedilol was administered to these patients. Planar 123I-MIBG scintigraphy provided measurements of cardiac neuronal uptake (as heart-to-mediastinum count activity ratio [HMR]), 4h after intravenous injection of 185 MBq MIBG. Hemodynamic, clinical, radionuclide LVEF and HMR data measured at the outset and after 6 mo of carvedilol were compared., Results: Resting heart rate decreased from 81 +/- 13 to 71 +/- 9 bpm (P = 0.003). After carvedilol therapy NYHA functional classification for these patients improved from 2.6 +/- 0.5 to 2.3 +/-0.5 (P = 0.04), LVEF improved from 22% +/- 9% to 30% +/- 13% (P = 0.005), and HMR improved from 145% +/- 23% to 170% +/- 25% (P = 0.0001)., Conclusion: Carvedilol induces improvements of clinical symptoms and cardiac neuronal and systolic functions in patients with dilated cardiomyopathy and chronic optimal treatment.

Background: This prospective, observational study was designed to evaluate the feasibility and the safety of a new strategy for stenting in acute coronary syndromes: direct stent implantation without predilation of the culprit lesion. This strategy might reduce both the cost of the procedure and the rate of no-reflow, a phenomenon that is more frequently observed during dilation of unstable plaques., Methods and Results: Between September 1997 and March 1998, 122 carefully selected patients with unstable angina or acute myocardial infarction were included in this study. Highly calcified lesions and vessels with excessive proximal tortuosity were excluded. The procedure was successful in 96% of cases. In 5 cases the stent failed to pass through the stenosis and was successfully retrieved in the guiding catheter in 3 cases. In 2 cases the stent was lost in the peripheral circulation. Transient no-reflow occurred in only 3 cases and was rapidly reversed by rescue use of an intracoronary bolus injection of a glycoprotein IIb/IIIa receptor inhibitor in 2 cases. A patient treated by primary angioplasty with cardiogenic shock on admission died 48 hours after the initial procedure because of irreversible cardiac failure. One-month clinical follow-up was obtained by telephone for all patients; no major coronary events occurred during this period., Conclusions: Direct coronary stenting without balloon predilation can be safely performed in acute coronary syndrome-related lesions in selected patients. A randomized, controlled study is warranted to confirm the promising results of this pilot study, especially regarding the low rate of the no-reflow phenomenon.

Purpose: Twelve patients with proved pheochromocytoma evaluated in our institution between 1991 and 1997 are described., Methods: The patients' urinary excretion rates and their metabolites (vanylmandelic acid and metanephrines) were significantly greater than normal before surgery. Echocardiography showed normal left ventricular ejection fractions (72% +/- 8%). All of the patients underwent planar I-123 metaiodobenzylguanidine (MIBG) scintigraphy to assess cardiac neuronal uptake 4 hours after and locate a pheochromocytoma 24 hours after intravenous injection of 185 MBq (3 mCi) I-231 MIBG. Ten patients with pheochromocytoma had positive tumoral findings with I-123 MIBG scintigraphy. Twelve patients had significant impairment of cardiac neuronal uptake of MIBG, with a heart:mediastinum ratio averaging 142% +/- 18% (normal value, 230% +/- 30%, P < 0.05). Postoperative cardiac MIBG imaging was performed in all patients (at 6 +/- 3 months)., Results: After surgical removal of the pheochromocytoma, cardiac MIBG uptake and the heart:mediastinum uptake ratios improved significantly (197% +/- 20%, P < 0.05) in all the patients. Urinary excretion rates and metabolites returned to the normal range. However, no significant correlation was found between cardiac MIBG uptake and urinary excretion rates and metabolites after the tumors were removed., Conclusion: Removing a pheochromocytoma reversed cardiac neuronal function as assessed by MIBG scintigraphy.

Intracardiac tumors occur infrequently and are difficult to diagnose with CT and MRI. The authors describe the successful imaging of a right atrial myxoma with F-18 FDG PET.

The management of heart failure that has become refractory to conventional drug treatment is an increasingly frequent problem for clinicians. Peritoneal dialysis, by ultrafiltration, is an effective method to treat refractory oedema, thereby improving the functional symptoms of these patients. The objective of this article is to review the data, of the literature and to define the results and indications of this treatment, which still remains and exceptional modality.

Unlabelled: Abnormalities of norepinephrine uptake have been found to reflect impairment in adrenergic nerve function that has influenced the cardiac outcome of patients with heart failure. The aim of this study was to explore the cardiac neuronal function by using 123I-metaiodobenzylguanidine (MIBG) scintigraphy in patients with acute myocarditis., Methods: We studied 15 patients (age range 42 +/- 10 yr) with clinical, biological, electrocardiographic and radionuclide left ventricular ejection fraction (LVEF) (41% +/- 7%) data indicating myocarditis and 10 normal subjects (age range 36 +/- 7 yr, mean radionuclide LVEF 69% +/- 8%, p < 0.05). Fourteen patients had positive histologic findings of myocarditis and 1 had nonspecific histological data. All patients underwent planar cardiac imaging after intravenous injection of 185 MBq 123I-MIBG and right ventricular biopsy within 7 days. A chest anterior view was acquired 4 hr later. Heart-to-mediastinum ratio activity was measured, as previously described in our laboratory., Results: Significant impairment of cardiac neuronal uptake of MIBG was observed and based on a reduction of heart-to-mediastinum ratio (148% +/- 16% versus 234% +/- 36%, p < 0.05). A significant correlation was observed between LVEF and MIBG uptake in patients (y = 1.58x +/- 83.7, r = 0.72, p < 0.01)., Conclusion: Acute myocarditis is associated with an injury of the cardiac adrenergic neuronal function. In addition to the inflammatory injury of the myocytes, the impairment of adrenergic function may be involved in the cardiac pump failure induced by myocarditis.

Restenosis in the months following a successful percutaneous transluminal coronary angioplasty (PTCA) remains the main limitation to this technique for myocardial revascularisation. Despite intensive investigation in this area, no pharmacological therapy has yet been found to be useful in preventing restenosis after conventional balloon angioplasty. The occurrence of restenosis, which is now known to be caused by both vessel remodelling and neointimal hyperplasia, might be reduced in the future by a combined mechanical and pharmacological approach. Although systemic administration of 'antirestenosis' drugs has not yet been tested to prevent restenosis after coronary stenting, it is very likely that pharmacological inhibition of neointimal hyperplasia within coronary stents will take advantage of local delivery techniques. In addition to local drug delivery catheters that are available, the stent itself may be coated with polymers and serve as a platform for drug delivery. The continued attractiveness of PTCA, as an alternative to medical treatment or bypass surgery for patients with coronary artery disease, will depend upon our ability to control the restenotic process.

Despite improved management of myocardial infarction, the incidence of cardiogenic shock remains constant at about 7.5% of cases. Patients who develop cardiogenic shock are older and much more often women. The site of infarction is usually anterior, transmural with greater left ventricular systolic dysfunction and a higher increase in cardiac enzymes. Diastolic intra-aortic balloon pumping is the usual treatment but survival only seems to be increased in patients undergoing revascularisation. Surgical revascularisation and circulatory assist devices have been proposed but patient selection in these studies was biased and optimised the results. The effect of thrombolysis was analysed in a subgroup of patients in the GISSI study. The 30 day mortality was 69.9% in 146 patients treated by streptokinase and 70.1% in patients given placebo. Thrombolysis only benefitted patients with an anterior myocardial infarction aged less than 65 and treated within 6 hours of the onset of pain. Although there are no randomised studies of the value of angioplasty in patients in cardiogenic shock. Several uncontrolled series suggest that this type of procedure reduces short and long-term mortality. However, the prognosis is not as good when unselected consecutive groups of patients are studied in whom infarction is complicated by cardiogenic shock immediately and who undergo "aggressive" revascularisation: 72% of patients die in hospital and there are few long-term survivors. The prognosis appears to be particularly poor in patients over 70 years of age with a previous history of myocardial infarction or who undergo prolonged pre-hospital cardiopulmonary resuscitation. However, the implantation of coronary stents associated with circulatory assist devices seems to improve the prognosis of infarction complicated by cardiogenic shock by ensuring adequate coronary flow and reducing the risk of reocclusion.

Leiomyosarcomas (LMS) of the heart are exceptional primary malignant tumours with a catastrophic prognosis and a mean survival measured in months. Extensive radical surgical resection clearly remains the most appropriate treatment. We report three cases observed over a 3-year period, consisting of an LMS of the inferior vena cava, an LMS of the pulmonary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous vein graft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifurcation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart transplantation for local recurrence at the sixth month. The survical was significantly improved compared to other treatment options (chemotherapy, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedure and the medium-term follow-up of the transplanted patient revealed cerebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignant tumours. Recent progress of diagnostic investigations, such as spiral CT with reconstruction, MRI, positron emission tomography (PET), are now able to establish the diagnosis more rapidly and therefore allow more radical surgical resection. This resection, possibly combined with venous reconstruction, must be associated with adjuvant therapies. Heart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognosis of these lesions affections a young population.

We present the case of 44-yr-old man who presented syncope with ventricular tachycardia in the setting of Brugada syndrome. In addition to the electrocardiographic evidence of the syndrome and the absence of apparent structural heart disease, clear defects of myocardial neuronal metaiodobenzylguanidine (MIBG) uptake on MIBG SPECT imaging also were found in inferior, apical and septal walls. Thallium-201 SPECT distribution was homogeneous along the left ventricle. Thus, cardiac MIBG scintigraphy provides information about left ventricular dysinnervation in a patient with Brugada syndrome, enhancing the clinical utility of myocardial MIBG SPECT imaging in life-threatening ventricular arrhythmias.

Background: Bupivacaine may have toxic cardiovascular effects when accidentally administered by intravascular injection. However, its electrophysiologic effects in the presence of myocardial ischemia remain unknown. The authors evaluated the electrophysiologic and anti- and proarrhythmic effects of bupivacaine in an in vitro model of the ischemic and reperfused myocardium., Methods: In a double-chamber bath, a guinea pig right ventricular muscle strip was subjected partly to normal conditions and partly to simulated ischemia followed by reperfusion. The electrophysiologic effects of bupivacaine were studied at 1, 5, and 10 microM concentrations., Results: Bupivacaine (5 and 10 microM) decreased the maximal upstroke velocity of the action potential (Vmax) in normoxic conditions and further decreased (10 microM) the Vmax decrease induced by ischemic conditions. Bupivacaine reduced the mean occurrence time to the onset of myocardial conduction blocks (9 +/- 3 min; mean +/- SD; P < 0.005 with 5 and 10 microM, compared with 17 +/- 6 min during simulated ischemia with no drug or control), and it increased the number of preparations that became inexcitable to pacing (55% of preparations, with 1 microM and 100% with 5 and 10 microM, compared with 17% for the control group). The incidence of spontaneous arrhythmias was reduced by 5 and 10 microM bupivacaine during ischemia and reperfusion and was enhanced by 1 microM bupivacaine during the ischemic phase., Conclusions: In guinea pig myocardium under ischemic conditions, bupivacaine induced a loss of excitability at concentrations of 5 and 10 microM. Proarrhythmic effects observed at 1 microM were considered as lower than the cardiotoxic range in normoxic conditions. The incidence of reperfusion arrhythmias was decreased at all concentrations.

Exercise training can induce important haemodynamic and metabolic adaptations in patients with chronic heart failure due to severe left ventricular dysfunction. This study examined the impact of exercise rehabilitation on cardiac neuronal function using iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy, Fourteen patients (11 men, 3 women; mean age 48 years; range: 36-66 years) with stable chronic heart failure of NYHA class II-III and an initial resting radionuclide left ventricular ejection fraction (LVEF) < 50% were enrolled in the study. Patients underwent progressive, supervised endurance training (treadmill test, Bruce protocol) during a 6-month period (60 sessions, 3 sessions per week) at a cardiac rehabilitation referral centre in order to measure exercise parameters. Planar 123I-MIBG scintigraphy provided measurements of cardiac neuronal uptake (heart-mediastinum ratio activity, 4 h after intravenous injection of 185 MBq of MIBG). Radionuclide LVEF was also assessed at the outset and after 6 months of exercise training. Workload (801 +/- 428 vs 1229 +/- 245 kpm.min-1, P = 0.001), exercise duration (504 +/- 190 vs 649 +/- 125 s, P = 0.02), and myocardial MIBG uptake (135% +/- 19% vs 156% +/- 25%, P = 0.02) increased significantly after rehabilitation. However, LVEF did not change significantly (23% +/- 9% vs 21% +/- 10%, p = NS). It is concluded that exercise rehabilitation induces improvement of cardiac neuronal function without having negative effects on cardiac contractility in patients with stable chronic heart failure.

Dissection of the aorta is a serious condition but rare in young women, and occurring during the 3rd trimester of pregnancy. The main risk factors are hypertension and diseases of the connective tissue or of collagen (Marfan's syndrome and Ehlers-Danlos disease). The authors report a case of dissection of the aorta managed in a pluridisciplinary manner by the anaesthetists, cardiologists, obstetricians and cardiothoracic surgeons, which resulted in a favourable outcome for both mother and baby. The diagnosis of Ehlers-Danlos disease was made from the onset and, over a period of 10 years with CT scan and annual echocardiographic follow-up, total replacement of the supra-coronary aorta was performed in several stages.

Heart failure is accompanied by major disturbances of the functioning of the sympathetic nervous system: global overactivation and local modifications of the adrenergic system. 123I-MIBG cardiac scintigraphy is an isotope technique investigating presynaptic adrenergic function. The cardiac uptake of MIBG is decreased during heart failure, reflecting a reduction of norepinephrine reuptake by cardiac presynaptic nerve endings. Alteration of presynaptic function occurs early and plays an important role in the pathogenesis of the deterioration of heart failure. 123I-MIBG cardiac scintigraphy allows in vivo assessment of the myocardial adrenergic reserves of patients with congestive heart failure. It should be proposed in all patients with severe ventricular dysfunction to help define the indications for heart transplantation.

This prospective nonrandomized study enrolled 16 patients with congestive heart failure [NYHA (New York Heart Association) III and IV] refractory to a maximal well-tolerated drug therapy. The aims were to evaluate if peritoneal ultrafiltration (PUF) could improve clinical conditions and to determine morbidity secondary to resistant congestive heart failure (RCHF) and PUF. There were 16 patients (12 male, 4 female) with a mean age of 65.4 years (56-81 years) and follow-up of 15.6 months (4-33 months). Thirteen patients had RCHF without end-stage renal disease. Patients were classified as NYHA class IV (n = 11) or class III (n = 5). One anuric patient had been on previous hemodialysis and switched to APD. PUF was obtained with a 2-L hypertonic dialysis solution, once a day (n = 7) or every 2 days (n = 4). Clinical improvement was obtained for all the patients. Weight decreased from 72.2 to 66.7 kg with a weekly ultrafiltration of 3.74 L (2.2-6.5 L). Sodium removal was 79 mmol/day (urinary 43%, peritoneal transport 57%). During the follow-up period, 2 patients received a cardiac transplant since 7 died due to cardiac reasons. Mean hospitalization time was 4.4 and 1.20 per patient per day before and after PUF, respectively. Hospitalization was in keeping with either RCHF (36%), dialysis complications (16%), or miscellaneous causes (48%). Our experience showed that a functional improvement and a better quality of life were achieved for all these patients with a low rate of hospitalization.

The authors studied 18 patients (15 men, 3 women) with an average age of 67 +/- 8 years with refractory cardiac failure. In order to determine the potential of pacing to raise cardiac output in severe cardiac failure. The average ejection fraction was 26 +/- 6.5%. All patients were in sinus rhythm:resting cardiac output was 3.35 l/min. Two temporary pacing catheters were positioned in the right atrium and at the apex of the right ventricle for dual-chamber mode pacing triggered by the spontaneous P waves. Changes in cardiac output were measured by Doppler echocardiography at different values of atrioventricular delay. Patients were considered to be responders if their cardiac outputs rose by 15%. In 7 patients meeting this criterion, the average increase in cardiac output was 27% (2.99 +/- 0.7 to 3.81 +/- 0.86 l/mn; p < 0.01); all had dilated cardiomyopathies with left bundle branch block and the optimal AV delay was 103 +/- 21 ms (80-140 ms); the duration of diastolic filling increased from 212 +/- 98 to 292 +/- 116 ms (p = 0.02). In the non-responding group (11 patients with an increase of cardiac output of only 3.6 +/- 0.09 to 3.9 +/- 0.92 l/mn; p < 0.01), the underlying disease process was mainly ischaemic. Two predictive factors of efficacy of dual-chamber pacing were identified: a short ventricular filling period (29 +/- 8% of the RR interval in the responders vs 44 +/- 9% in the non-responders; p < 0.01) and the presence of 1st degree atrioventricular block. Dual-chamber pacing could be a valuable method of increasing resting cardiac outputs in a selected group of patients with severe, refractory, cardiac failure.

Objectives: The global results of various series of heart transplantation (HT) are essential to assess the life expectancy provided by this technique. Due to the increasing graft shortage, it appears essential to very strictly candidates for HT., Methods: From March 8, 1989 to December 7, 1994, 75 orthotopic Hts were performed in 62 men and 12 women (1 case of retransplantation). The mean age was 47.46 +/- 15.02 years (range: 2.5-66 years). Four patients were younger than 10 years and 22 were older than 60 years. Our series included more cases of ischaemic heart disease (36) than dilated cardiomyopathies (33), with a history of cardiac surgery in almost one quarter (20) of patients with ischaemic heart disease., Results: The immediate postoperative survival rate was 94.7% with 3 deaths attributable to refractory pulmonary hypertension associated with graft failure and one death related to postoperative tamponade. Five other patients died during the following 3 months, increasing the mean global survival to 88%. After a mean follow-up of 2.1 years (maximum 5.8 years), the actuarial 5-year survival rate was 56.8%. Eleven patients died between 4 and 38 months (mean: 18.2 months). Two deaths were due to cancers, 4 were due to septicaemia, another 4 were due to rejection and finally 1 was due to meningeal haemorrhage. The frequency (19) of reoperations for clot removal was due to the large number of patients with a history of previous heart surgery (20)., Conclusion: Strict recipient selection, possibly based on 123I-MIBG scintigraphy, the use of pulsatile circulatory assistance systems, improved CMV, morphometry and donor-recipient age matching, should optimize the results of a technique, whose efficacy is confirmed in this series.

The physiopathologic role of thrombosis in the genesis of myocardial infarction, began to be suspected early in the 20th century but its logical treatment, thrombolysis, was first used on a large scale only ten years ago. Today, it is well established that short, middle and long-term mortality is correlated to coronary permeability, the delay in the revascularization treatment start-up, its efficacy, its swiftness of action, and to the maintaining of permeability following reperfusion. The importance of time elapse before reperfusion is obtained was demonstrated as early as 1986 by the GISSI study. According to this study, the administration of streptokinase (compared to a conventional treatment) reduced mortality at 21 days respectively by 47%, 23%, and 17%, depending on whether patients were treated within one hour, three hours, or between 3 and 6 hours following the onset of the painful symptoms. One of the major teachings of the GUSTO study, reported at the end of 1993, was the confirmation of the so-called "open artery" theory: mortality at 30 days was of 4.5% among patients whose coronary circulation was restored at the 90th minute, whatever thrombolytic treatment was used, compared to 8.9% when the coronary artery remained occluded. The value of aspirin in preserving coronary permeability following thrombolysis was demonstrated by the ISIS-2 study: mortality at 5 weeks was reduced by 23% in the group of patients randomised to receive only aspirin, while it was reduced by 25% in the group of patients randomised to be treated with streptokinase, and by 42% in the group randomised to receive both aspirin and streptokinase, compared to the group who received neither aspirin nor streptokinase. However, mortality during the first days following randomisation was identical among the groups, with or without aspirin, which suggested its action was rather one of prevention against reocclusion than one of accelerating dissolution of the thrombus. However, in spite of improved therapeutical protocols, a normal flow, which is the major criteria for a reduced mortality, is only obtained at the 90th minute in 54% of the patients who were administered the up-to-date treatment ie aspirin-accelerated t-PA-heparin in combination.(ABSTRACT TRUNCATED AT 400 WORDS)

The incidence of endocarditis due to Cardiobacterium hominis is probably underestimated because clinical presentations vary greatly and culture of this Gram negative germ is difficult. A 48-year-old man with a past history of post-streptococcic aortic regurgitation was hospitalized twice within 1 week for fever (38 degrees C) and junctional tachycardia which responded to amiodarone. Subsequently, infero-apical necrosis was documented. Based on the result of the laboratory tests, coronary embolism was suspected although 12 blood cultures were negative. The patient recovered well with a standard antibiotic treatment. Fifteen days later, the blood cultures revealed Cardiobacterium hominis. Antibiotic therapy was adapted and aortic valve replacement was programmed. Two months later the patient died from uncontrollable left heart failure. A 63-year-old man who had had mitral valve replacement 10 years earlier for Streptococcus mitis endocarditis was hospitalized for fever (38 degrees C) and a painful left calf. Phlebocavography eliminated deep vein thrombosis and a complete cardiac work-up was inconclusive. Endocarditis was suspected although blood cultures were negative. The patient was given oral penicillin and discharged after one week. Three months later, the patient was again febrile (38 degrees C) and suffered a cerebral vascular event. Fourteen days after blood sampling, cultures revealed Cardiobacterium hominis. The patient's haemodynamic status worsened and valve replacement with atrioplasty was performed. Outcome after 4 years follow-up has been favourable. Endocarditis due to Cardiobacterium hominis, a saprophitic germ of the upper airway and the female genital tract, has been reported in 64 cases in the literature. The clinical picture is often limited to fever and a heart murmur and laboratory tests show an accelerated erythrocyte sedimentation rate and hyperleukocytosis. Inflammatory type anaemia is often found due to the latency of the endocarditis. Complications are frequent and can be fatal due to massive pulmonary emboli, cerebral vascular events or irreversible heart failure. C. hominis is sensitive to amoxycillin and netilmicin. Surgical treatment of the valvulopathy is indicated.

Three distinct forms of rupture of the heart may be identified after myocardial infarction: sudden rupture with massive intrapericardial haemorrhage, and sudden death with clinical signs of electromechanical dissociation; rupture into the pericardium resulting in a false aneurysm, the treatment of which is surgical; subacute rupture which accounts for 30% of cases in which bleeding into the pericardium is slow and/or repeated. Over an 8 year period and in a series of 2,400 consecutive infarcts admitted to the intensive care unit, 10 cases of subacute rupture of the heart were diagnosed. They were 6 men and 4 women, with a mean age of 73.6 years. The clinical presentation was isolated chest pain in 5 cases, syncope alone in 2 cases and the association of pain and syncope in 3 cases. Six patients were in shock on admission. In two cases, shock developed after admission. The infarction was confirmed biologically by a significant elevation of creatinine kinase in 9 out of 10 cases. Transmural infarction was observed in 9 cases: the infarct was electrocardiographically non-transmural in 1 case. Emergency echocardiography showed pericardial effusion in all cases, usually moderate, but sometimes compressive with an intrapericardial echogenic mass suggesting a thrombus. Haemodynamic improvement was obtained by medication allowing cardiac catheterisation which showed adiastole in 3 cases. Coronary angiography was performed in 7 cases. In 5 of the 7 cases, apart from occlusion of the artery presumed to be responsible for the infarct, the coronary vessels were diffusely infiltrated without significant stenosis. Left ventriculography was performed in 7 cases. In 6 of the 7 cases regional akinesis was demonstrated: the 7th case showed dyskinesia of the anterior wall. In two cases, contrast medium was observed to fill the pericardium during ventriculography, indicating myocardial rupture. The diagnosis of subacute rupture, suggested by clinical and paraclinical (particularly echocardiography), was confirmed in 9 cases at surgery and in the 10th case at autopsy. Surgery consisted of repairing the rupture. In the last two cases, biological glue was used to reinforce the surgical repair. The clinical outcome was good after surgery in 6 cases with a follow-up of 5 months to 8 years. The diagnosis of subacute rupture should therefore be made on clinical and echocardiographic criteria, as these results suggest that surgery is often possible, with a good prognosis.

The biological diagnosis is usually late with respect to clinical and electrocardiographic signs of acute myocardial infarction. Recent laboratory methods have stimulated renewed interest in very early marker of infarction: myoglobin and CPK isoforms. The authors undertook a prospective study of the diagnostic sensitivity of myoglobin (MG), CPK and CPK-MM isoforms in 30 consecutive patients undergoing intravenous thrombolytic therapy in the acute phase of myocardial infarction. Blood samples were obtained on admission and every 30 minutes for 1 h 30. The Mb contributed to diagnosis of infarction on admission in 89% of patients. This percentage fell to 44% for the ratio MM3/MM1 > 0.5 to 27% for the CPK and to 24% for the ratio MM3/MM1 > 1. The sensitivity of all tests increased in the later blood samples but it remained low in the CPK and MM3/MM1 > 1 criteria. The diagnostic sensitivity of the first sample was also better in patients admitted after the 3rd hour (Mb = 100%; MM3/MM1 > 0.5: 58%; CPK: 41%) compared with those admitted before the 3rd hour (Mb = 82%; MM3/MM1 > 0.5: 35%; CPK: 17%). These results show that the diagnostic sensitivity of biological markers of myocardial infarction is very different. The serum myoglobin is an earlier and more sensitive marker than the CPK or CPK-MM isoforms.

Between May 1991 and February 1992, 31 consecutive patients were included in a prospective study, the aims of which were to determine the criteria of early coronary revascularisation after intravenous thrombolysis in the acute phase of myocardial infarction. The rise in serum myoglobin, the ST segment elevation, accelerated idioventricular rhythm and the evolution of chest pain were analysed. All patients underwent coronary angiography. Twenty-six were revascularized and 5 remained with coronary occlusion. Two types of serum myoglobin curves were demonstrated. Those with a sudden , decrease and a well defined peak in the first 4 hours were specific for revascularisation and easily identified (Group A: 16 patients). The graphs with a progressively rising slope to a peak after the 4th hour were observed in patients with coronary occlusion, but also in 10 patients with recanalized arteries (Group B). No significant difference was demonstrated with regards to the clinical and coronary angiographic parameters between patients in Group A and Group B. On the other hand, the time between the onset of chest pain and peak myoglobin was shorter in Group A (298 +/- 81 min) than in recanalised patients in Group B (380 +/- 54 min) (p < 0.05). The difference in the profile of the serum myoglobin could therefore reflect restoration of arterial flow in myocardial cells which had not suffered the same period of ischemia. ST segment elevation may increase, decrease of remain stable at 120 minutes in patients revascularised and those remaining occluded. In 9 patients, the ST elevation increased compared with the initial electrocardiogram .(ABSTRACT TRUNCATED AT 250 WORDS)

Five to ten per cent of survivors of acute myocardial infarction die within two years. The majority of these deaths are sudden and are attributed to a lethal ventricular arrhythmia. This is usually ventricular tachycardia degenerating to ventricular fibrillation. These post-infarction tachycardias are generally due to reentry. They require an anatomic arrhythmogenic substrate, a zone of delayed conduction. This can be detected as late potentials on signal averaged ECG. The triggering of a significant ventricular arrhythmia by ventricular stimulation is closely correlated to the occurrence of a severe ventricular arrhythmia in the months following infarction. Programmed ventricular stimulation could, therefore, help to identify patients requiring close follow-up and/or preventive antiarrhythmic therapy, but cannot be offered to all patients because of its invasive nature. Seventy nine post-infarction patients were studied prospectively. All underwent coronary angiography, signal averaging electrocardiography, and programmed ventricular stimulation at least 15 days after infarction. Fifty five patients had at least one criterion of late potentials (QRS duration > or = 110 ms and/or amplitude of the last 40 ms < 27 microV and/or duration of potentials of under 40 microV > 37 ms). Twenty four patients had no late potentials. The results of programmed stimulation were estimated to be positive when sustained or unsustained monomorphic ventricular tachycardia was triggered, and negative when ventricular fibrillation, ventricular flutter unsustained polymorphic ventricular tachycardia or no arrhythmia could be induced. Programmed ventricular pacing triggered 15 significant events, 17 unsustained polymorphic ventricular tachycardias, 13 ventricular flutters and 11 ventricular fibrillations. The exploration was negative in 23 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

The frequency of cardiac lesion in patients with other signs of Lyme disease has been estimated at 8 percent. The usual manifestation of myocardial involvement is a varying degree of atrioventricular block or more diffuse signs of myocarditis. Autopsy or intramyocardial biopsy provides a histological diagnosis of myocarditis. Microscopy shows a diffuse lympho-plasmocytic infiltrate with presence of macrophages in the myocardium, associated with a varying number of necrotic myocytes. Structures resembling spirochetes have been found in some cases. Exceptionally, the cardiac lesion may be isolated, presenting as an acute atrioventricular block and/or an acute myocarditis; in such cases the diagnosis of cardiac lesion caused by Lyme disease is made on serological grounds. We report the case of a 30-year old man admitted for acute myocarditis which turned out to be totally regressive. Intramyocardial biopsy showed interstitial congestion associated with inflammatory lympho-histiocytic infiltrates and eosinophilic polymorphonuclears; the myocardial fibres in contact with these infiltrates appeared to be altered. The diagnosis of Lyme disease was subsequently confirmed by serological tests. Patients with myocarditis caused by Lyme disease must be treated with antibiotics. Recent reports have demonstrated the presence of spirochetes in the myocardium of patients with dilated cardiomyopathy, suggesting that the spirochete Borrelia burgdorferi might be associated with, or play a part in, the subsequent occurrence of dilated cardiomyopathy.

In a series of more than 700 patients admitted over a 2-year period to an intensive care unit, 3 patients with isolated right ventricular infarction were observed. At electrocardiography the condition was characterized by isolated ST elevation in the anterior precordial leads. Infarction was due to obstruction of a right marginal coronary artery in 2 cases and of a small right coronary artery feeding only the right ventricle in one case. In the absence of electrocardiographic signs in the inferior territory, this ST elevation is suggestive of an anterior infarction in constitution, but the characteristic "dome-like" shape of the elevation, its decrease from V2 or V3 to V4 or V5 and the progressive regression of repolarization disorders without occurrence of Q wave suggest a diagnosis of right ventricular infarction. This diagnosis can rapidly be confirmed by a normal left ventricular function at echocardiography.

Dissection of the inferior wall of the right ventricle during the acute phase of myocardial infarction with right ventricular involvement is a mechanical complication which has been recently identified, the diagnosis being almost exclusively post-mortem. The authors report the clinical, echocardiographic and pathological features of myocardial dissection in four patients. Between 1985 and 1988, the diagnosis of myocardial dissection was made by echocardiography in 4 patients aged 77 to 80 years, admitted to hospital for an acute inferior wall myocardial infarction. All 4 patients had signs of acute right ventricular failure indicating right ventricular necrosis and a loud systolic murmur at the left sternal border; 2 patients were in shock. The ECG showed signs of inferior wall infarction with, in 2 patients, electrical changes suggestive of right ventricular involvement. Echocardiography showed dissection of the inferior wall of the right ventricle as a pulsatile, echo-free space in the diaphragmatic wall of the right ventricle which appeared to obstruct right ventricular ejection in end systole to a variable degree. The outcome was fatal in all cases with death resulting from refractory myocardial failure. Pathological analysis confirmed biventricular inferior wall infarction also involving the posterior part of the interventricular system, the site of a small tear on the left side which communicated with a neo-cavity dissecting the RV posterior wall. The right coronary artery was totally occluded in all cases. The anatomical lesions were fully concordant with the echocardiographic data: the dissection filled with blood from the left ventricle at each systole creating a pulsatile space in the diaphragmatic wall of the ventricle obstructing ejection.

Coronary artery-cardiac fistulae are uncommon and usually congenital abnormalities. The majority of these fistulae communicate with the right heart chambers. Acquired coronary-left heart fistulae are very rare: secondary to trauma, cardiac surgery, angioplasty or endomyocardial biopsy. Fistulae secondary to myocardial infarction are rare but have been described. The authors report the case of a man with anterior wall infarction treated by fibrinolysis and by angioplasty of the left anterior descending artery (LAD) 48 hours later. This dilatation was complicated by repeated occlusion but a good result was obtained after in situ and then systemic fibrinolysis. Control coronary angiography after angioplasty performed 6 months later showed the presence of micro-fistulae between the distal part of the LAD, the left diagonal entry and the left ventricular chamber which were not present on the coronary angiogram before the angioplasty. The fistulae appeared to be secondary to a localised infarct which occurred after distal thrombosis of the LAD during angioplasty.

The authors report a case of myocardial infarction complicating an exercise stress test performed 48 hours after successful angioplasty of a stenosed left anterior descending artery in a patient with unstable angina. Three similar cases have been previously reported. The probable mechanisms of coronary obstruction in this context are rupture of an atheromatous plaque, thrombosis and coronary spasm.

ST segment elevation in the anterior precordial chest leads may be observed in some cases of right ventricular infarction alone or associated with left ventricular inferior wall infarction. Six out of 700 patients admitted to our Coronary Care Unit over a 2 year period had right ventricular infarction with these electrocardiographic changes. In three cases, isolated right ventricular infarction was due to occlusion of a right marginal artery (N = 2) or of a small right coronary artery (N = 1) which only vascularised the right ventricle. In 2 cases, right ventricular infarction was associated with a recent or chronic left ventricular inferior wall infarct. This type of ST segment elevation may suggest a left ventricular anterior wall infarct especially when there are no changes in the inferior leads, as was the case in our first patient. However, the dome-like appearance of the ST segment, the reduction in amplitude of ST elevation from V2 to V5, the progressive regression of the ST changes without the appearance of Q waves, are more suggestive of the diagnosis of right ventricular infarction. In addition, normal left ventricular dilatation on echocardiographic examination rapidly confirms the diagnosis.

Primary sarcomas arising from the trunk or branches of the pulmonary artery are exceedingly rare and usually diagnosed at autopsy. The authors report the case of a 31 year old man referred for investigation of recurrent syncope. Echocardiography, right ventriculography and the thoracic computed tomography led to early diagnosis of a pulmonary artery tumour and surgical resection. Histology revealed a primary sarcoma. The early postoperative course was uncomplicated but one month after surgery a local recurrence was diagnosed at routine echocardiographic examination. The syncopal symptoms recurred seven months after surgery and echocardiography showed tumour recurrence on the interventricular septum and in the pulmonary artery. A second palliative operation was attempted but was unsuccessful because of the size of the tumour.

In Carney's syndrome, the association of cardiac myxomas, spotty pigmentation and endocrine over activity, the myxomas are usually multiple and have atypical locations. The authors report a case in which an accurate diagnosis of these multiple myxomas was made by transoesophageal echocardiography, although transthoracic echocardiography had missed the diagnosis.

The authors report 4 cases of aortic dissection after aortic valve replacement. The aorta was dilated in all four cases before surgery. Two patients died within hours of the dissection, illustrating the poor prognosis of this complication and the difficulties of surgical management. The features of these four cases were compared to those of 29 other cases reported in the last ten years. The initial surgical indication for valve replacement was equally divided between aortic stenosis and regurgitation. The onset of dissection after surgery was very variable, ranging from a few hours to several years after valve replacement. Abnormalities of the aortic wall and peroperative trauma play an important role in the pathogenesis of this complication. The prognosis is very poor, which underlines the importance of preventive measures and regular follow-up of these patients.

Nine patients with supraventricular rhythm disorders were treated during 5-day periods with different oral doses (300, 450, 600, and 900 mg daily) of propafenone concomitantly to long-term digoxin treatment. A poor correlation (r = .398; P less than .05) was obtained when the difference between the mean digoxin serum level (calculated with the Cmin data determined each of the 5 days) observed during a given propafenone dose and the mean digoxin serum level observed before propafenone treatment, was correlated with the dose of propafenone; but an evident correlation (r = .778; P less than .01) was found when the difference in digoxin level was correlated with the plasma propafenone concentration. The propafenone effect of increasing digoxin blood levels was thus concluded to be poorly dose dependent but strongly concentration dependent. The association of propafenone to a long-term digoxin treatment can be considered with a low risk of toxicity when plasma propafenone concentration does not exceed about 1000 ng/mL. Propafenone plasma levels are unpredictable in view of their wide interindividual variation for a given dose, so their measurement is advised to detect high levels and consequently to prevent a rise in digoxin serum concentrations with the possibility of toxicity. In clinical practice, when propafenone concentration determinations are not readily available, digoxin serum levels at least have to be carefully monitored.