[Cardiac metastasis from malignant melanoma]. / Métastase cardiaque d'un mélanome.

The authors describe a case of cardiac metastasis associated with a malignant melanoma, which was removed in its totality. Malignant melanoma is a tumour which metastases frequently at the cardiac level. The mechanism of implantation and the symptomatology of this metastatic localisation are discussed.

[Isolated tricuspid valve endocarditis as a cause of fever of unknown origin]. / Endocardite infectieuse tricuspidienne comme cause d'une fièevre d'origine indéterminée.

Isolated tricuspid valve endocarditis is the least common endocarditis with an incidence of 5 to 10% in the literature. It is usually described in drug abusers and as a complication of nosocomial infections (catheter, post-surgery...). We present the case of a 70 year-old patient admitted for a fever of unknown origin lasting for 2 months. He had an isolated tricuspid endocarditis with Enterococcus faecalis. We review the diagnostic criterias predisposing factors and treatment of right-sided valvular endocarditis, particularly the endocarditis produced by Enterococcus faecalis, a rare etiopathogenic agent of tricuspid endocarditis.

Atrial flutter in the perinatal age group: diagnosis, management and outcome.

OBJECTIVES: The aim of this retrospective study was to evaluate perinatal atrial flutter (AF) and the efficacy of maternally administered antiarrhythmic agents, postpartum management and outcome. BACKGROUND: Perinatal AF is a potentially lethal arrhythmia, and management of this disorder is difficult and controversial. METHODS: Forty-five patients with documented AF were studied retrospectively. RESULTS: Atrial flutter was diagnosed prenatally in 44 fetuses and immediately postnatally in 1 neonate. Fetal hydrops was seen in 20 patients; 17 received maternal therapy, 2 were delivered and 1 was not treated because it had a severe nontreatable cardiac malformation. In the nonhydropic group of 24 patients, 18 were treated and the remaining 6 were delivered immediately. In the hydropic group, 10 received single-drug therapy (digoxin or sotalol) and 7 received multidrug therapy. In the nonhydropic group, 13 received a single drug (digoxin or sotalol) and 5 received multiple drugs. One patient with rapid 1:1 atrioventricular conduction (heart rate 480 beats/min) died in utero and another died due to a combination of severe hydrops because of the AF, sotalol medication, stenosis of the venous duct and hypoplastic placenta. Of the 43 live-born infants, 12 were in AF at birth. Electrical cardioversion was successful in eight of nine patients. No recurrences in AF have occurred beyond the neonatal period. Four patients with fetal flutter and hydrops showed significant neurological pathology immediately after birth. CONCLUSIONS: Fetal AF is a serious and threatening rhythm disorder, particularly when it causes hydrops, it may be associated with fetal death or neurological damage. Treatment is required and primarily aimed at reaching an adequate ventricular rate and preferably conversion to sinus rhythm. Digoxin failed in prevention of recurrence at time of delivery in a quarter of our patients, whereas with sotalol no recurrence of AF has been reported, suggesting that class III agents may be the future therapy. Once fetuses with AF survive without neurological pathology, their future is good and prophylaxis beyond the neonatal period is unnecessary.

Monosomy 11q: report of two familial cases and review of the literature.

We present four children from two families with the typical 11q- phenotype resulting from an unbalanced segregation of a parental translocation. In the first family, the father had a 46,XY,t(5;11)(q24;q23.3) constitution. The father of the three other children had a 46,XY,t(11;17)(q23;p13) translocation. Despite associated partial deletion, three of the children had a typical 11q- phenotype. The fourth one, whose pregnancy was terminated in the second trimester, had a hypoplastic left heart but no other considered gross anomalies. A review of 36 previous cases, including 5 due to translocations (4 familial rearrangements, and 1 of unknown origin) is given with emphasis on the relationships between break-points and phenotype. Undescribed manifestations in our patients include agenesis of corpus callosum adactyly and malrotation of the gut.

Nosology of lysosomal glycogen storage diseases without in vitro acid maltase deficiency. Delineation of a neonatal form.

We describe a boy with an early lethal hypertrophic vacuolar cardiomyopathy of neonatal onset. Abnormal intra- and extralysosomal glycogen storage disease was demonstrated in heart and skeletal muscles. Glycogen content was twice the normal in muscles and over 3-fold the normal in the heart. In this organ, over 50% of the intracellular space was occupied by glycogen and possibly oligosaccharides, as demonstrated by the quantitative morphometric analysis of electron micrographs. The activity of acid alpha-glucosidase was increased in the heart, skeletal muscles, and liver, but was normal in leukocytes. A review of the 11 previously published pedigrees of lysosomal glycogen storage disease with normal in vitro alpha-glucosidase activity allows the delineation of three clinical entities: juvenile and neonatal pseudo-Pompe diseases and partial Pompe disease. Partial Pompe disease, due to the tissue-specific absence of acid alpha-glucosidase, was observed in a single patient. The most common form is the late-onset pseudo-Pompe disease, which is characterized by severe cardiomyopathy and mild myopathy appearing in the second or third decade, prominent arrhythmia with Wolf-Parkinson-White syndrome, and sometimes mental retardation. Patients reported as suffering from Antopol disease probably belong to this group. Dominant inheritance (autosomal or X linked) is likely in most families. The present report appears to be the first one to describe a rapidly fatal neonatal form of lysosomal glycogenosis without acid maltase deficiency. The mode of inheritance of this form is not known. Differential diagosis includes Pompe disease (similar histology) and cardiac phosphorylase b kinase deficiency (similar clinical course). The delineation of neonatal pseudo-Pompe disease makes enzymatic confirmation mandatory in each case suspected of Pompe disease.

Two-dimensional echographic localization of intracardiac cerebrospinal fluid shunt catheters.

This study evaluated the ability of two-dimensional echocardiography (2D echo) to detect and localize the distal end of ventriculoatrial shunt catheters in the cardiac chambers of hydrocephalic patients. Twenty-eight patients were studied, and the performances of 2D echo and standard chest x-ray filming were compared. Although standard chest x-ray filming allowed accurate determination of the catheter position in only half of the patients, 2D echo afforded clear visualization and accurate localization of the catheter in all instances. By its precise definition of intracardiac anatomical landmarks and its excellent time determination, 2D echo can provide valuable information on the movements of the catheter's distal end during the cardiac cycle, during head flexion or rotation, and during deep breathing movements. Some interesting observations were made with 2D echo. A to-and-fro motion of the catheter tip through the tricuspid orifice during the cardiac cycle was visualized in two instances. The combination of head movements and deep respiratory movements induced a variation in the position of the catheter tip. The magnitude of this displacement was 6 cm at the most. Two patients underwent reoperation for lengthening or shortening of the catheter. The operative findings confirmed the ultrasonic localization in both cases where the chest x-ray film had failed to identify or adequately locate the catheter tip. It is concluded that 2D echo is a safe, innocuous, and accurate method with which to locate the distal end of a ventriculoatrial shunt. It is the only available technique that provides visualization of intracardiac structures and cardiac cycle reference.(ABSTRACT TRUNCATED AT 250 WORDS)

Relative value of clinical and transesophageal echocardiographic variables for risk stratification in patients with infective endocarditis.

BACKGROUND: Infective endocarditis remains a life-threatening disease, and its optimal management is of paramount importance. Transesophageal echocardiography (TEE) is useful for the diagnosis of endocarditis-induced lesions, but the prognostic significance of the method remains controversial. HYPOTHESIS: The purpose of this study was to relate clinical and TEE characteristics to the occurrence of mortality and/or systemic embolization in a consecutive series of 45 patients with a diagnosis of infective endocarditis. METHODS: All patients underwent at least one monoplane TEE. Clinical data, episodes of embolization, and echocardiographic characteristics were prospectively recorded. Stepwise logistic discriminant analysis was performed to identify the independent variables that best predicted three binary outcomes: systemic embolization, death, and systemic embolization and/or death. RESULTS: Twelve of the 45 patients (27%) died from the endocarditis. Significant univariate predictors of death were the presence of paravalvular abscess (p = 0.025), number of vegetations (p = 0.021), Staphylococcus aureus isolated in blood cultures (p = 0.002), medical treatment alone (p < 0.002), and systemic embolism (p < 0.001). In multivariate analysis, systemic embolism (chi 2 = 29.3; p < 0.01), echocardiographic evidence of paravalvular abscess (chi 2 = 5.6; p = 0.018), Staphylococcus aureus endocarditis (chi 2 = 5.5; p = 0.016), and medical treatment alone (chi 2 = 5.11; p = 0.024) emerged as optimal predictors of death. Systemic embolization occurred in 12 patients. Independent variables predicting systemic embolization were a total length of vegetations > 14 mm (p = 0.01), greater age (p = 0.02), and medical treatment alone (p = 0.03). When two or more vegetations were observed, the total length is the sum of the individual sizes. Independent risk factors for the development of systemic emboli and/or death as a combined end point were total length of vegetations on TEE (chi 2 = 6.4; p = 0.003) and medical treatment alone (chi 2 = 4.1; p = 0.047). CONCLUSIONS: High-risk patients may be identified by the combination of clinical variables and TEE characteristics.

[Study of a case of "pacemaker syndrome" by echocardiography and phonomechanocardiography]. / Exploration d'un cas de "pacemaker syndrome" par échocardiographie et phonomécanoardiographie.

This report is concerned with the observation of a woman aged 61 with sino-atrial disorders in whom near-syncopal episodes recurred after implantation of a pacemaker. The attacks were associated with paroxysmal lowering of blood pressure. The echocardiogram and the phonomecanocardiogram indicated that a shortening of the left ventricular ejection time and a decrease of the duration and the amplitude of the separation of the aortic valve leaflets abruptly appeared whenever the patient was under pacing. This data further stresses the importance of the so-called "pacemaker syndrome". The phonomecano- and echocardiogram may permit to select among paced patients with recurrent symptoms those who deserve consideration of replacement of their ventricular pacemaker by a bifocal unit.

[Complete obstruction of the common trunk of the left coronary vessel. Observation of a case]. / Obstruction complète du tronic commun de la coronaire gauche. Observation d'un cas.

A case of complete left main coronary artery obstruction is described. After an anterolateral and inferior subendocardial myocardial infarction, the patient remained symptomatic and underwent an angiographic investigation. Complete left main artery obstruction was disclosed and an important coronary collateral circulation was evidenced. This collaterality explains the absence of extensive transmural myocardial infarction of some patients with left main coronary artery thrombosis.

[Anatomy of the conduction system in an unusual case of a heart ventricle with double inlet (straddling tricuspid valve)]. / Anatomie du système de conduction dans un cas particulier de ventricule gauche à double entrée

The histological study of the conduction system has been done in a case of "straddling tricuspid valve". The main anomalies observed were an AV node located more anteriorly but at the left of the posterior interventricular septum and an hypertrophic bifasicular right bundle branch with an unusual anterior radiation. These morphological features allow to discuss the embryology of the conduction system and of the interventricular septum in that congenital cardiopathy.

Reversible hypertrophic cardiomyopathy in congenital hyperinsulinism.

A neonate with congenital focal hyperinsulinism developed hypertrophic cardiomyopathy that progressed until partial pancreatectomy was performed. Surgery was followed by complete resolution of the condition. Hyperinsulinism may be a cause of treatable cardiomyopathy.

[Hemodynamic complication of ventricular stimulation in a patient with moderate aortic stenosis]. / Complication hémodynamique de la stimulation ventriculaire chez un patient porteur d'une sténose aortique modérée.

This report is concerned with a patient aged 76 with mild to moderate aortic stenosis and transient A-V block who was treated by stand-by intracardiac pacing. In spite of this, the patient continued to present syncopal attacks. It is demonstrated that these episodes were related to dramatic decreases of systolic blood pressure and cardiac output occurring whenever the right ventricle was paced. These hemodynamic alterations were secondary to the loss of atrial contribution to ventricular filling. They were suppressed by insertion of a sequential auriculoventricular pacemaker.

[Involvement of the common trunk of the left coronary artery]. / Les atteintes du tronc commun de la coronaire gauche.

This work describes the observations made in a group of 32 patients suffering from left main coronary stenosis (narrowing of 70% or more). 28 patients underwent surgical treatment: they represent 14% of 200 consecutive surgical cases. The diagnosis of left main narrowing is not possible on clinical ground only. However, several common features can be noted. Patients without myocardial infarction commemoratives all experienced unstable angina pectoris. When a bicycle ergometric test could be realized, it was positive for a low work load. It showed a mean 3 mm ST depression for a mean charge of 75 watts. The repolarisation disturbance lasted long after interruption of effort (more than 7 minutes). Coronarography very often showed multiple vessel disease. the anterior descending artery was stenosed in 25 patients, the circumflex artery artery in 23 and the right coronary artery in 27 patients out of the 32. In the majority of the patients, an anastomotic circulation was evidenced. The direction of the vicarious flow was determined by the relative degree of stenosis on the arteries implied in this collaterality. The mortality of coronarography was 1/32. Among the 28 operated patients, 1 died shortly after operation, giving an operative mortality of 3.6%. During a mean follow-up of 15 months, 1 death during the first postoperative year, 2 failures, 2 partial successes and 22 clinical recoveries were noted. Out of 18 patients socially susceptible to work again, 12 are effectively working, 3 are retired and 3 are still totally unable to work at the present time.

Physiologic response to dobutamine infusion during cardiac stress testing of dogs.

OBJECTIVE: To evaluate response of various cardiovascular variables after administration of incremental doses of dobutamine in healthy conscious dogs, using standardized dobutamine stress echocardiography (DSE). ANIMALS: 8 healthy dogs. PROCEDURE: A DSE was performed twice on each dog within 24 hours. Dobutamine was infused at a rate of 12.5 to 42.5 microg/kg/min, using incremental increases of 10 microg/kg/min. Doppler sphygmomanometry, electrocardiography, and echocardiography were performed. Left ventricular size, global ventricular performance, and left ventricular systolic myocardial function were measured by means of echocardiography. RESULTS: At the highest dosage, dobutamine induced an increase of 20+/-3% and 109+/-12% in systolic blood pressure and cardiac index, respectively. The latter was associated with a significant increase in heart rate and stroke index. Fractional shortening of the left ventricle, fractional thickening of the left ventricular free wall and interventricular septum, ejection fraction, and mean velocity of fiber shortening had a progressive and significant increase during dobutamine infusion. Preejection period and left ventricular ejection time had a progressive and significative decrease during the stress test. CONCLUSIONS: The technique used was feasable, safe, and repeatable in healthy conscious dogs. Control values were determined. CLINICAL RELEVANCE: Data for these healthy dogs might be useful for comparison with results obtained from dogs with known or suspected cardiovascular disease.

Doppler echocardiographic study of left and right ventricular function during dobutamine stress testing in conscious healthy dogs.

OBJECTIVE: To evaluate left and right ventricular filling and ejection performances by use of Doppler echocardiography in healthy, conscious dogs submitted to dobutamine stress testing. ANIMALS: 10 unsedated, healthy adult Beagles. PROCEDURE: Doppler echocardiography was performed during cardiac stress testing on each dog twice at 24-hour intervals. Dobutamine was infused in 10 micrograms/kg of body weight/min incremental dosages, from 12.5 to 42.5 micrograms/kg/min. Duration of each step was 15 minutes. Doppler measurements were recorded at baseline and at each stage of dobutamine infusion, whereas aortic diameter was measured at baseline and at peak dosage by use of two-dimensional echocardiography. RESULTS: Dobutamine infusion induced a significant increase in velocity time integrals and in peak flow velocities at the aortic, pulmonic, mitral, and tricuspid valves. Acceleration time-to-deceleration time ratio at the aortic wave also was increased significantly. On the other hand, ejection time, acceleration time, and deceleration time at the aortic and pulmonic valves and peak flow velocity of the E wave-to-peak flow velocity of the A wave ratio at the mitral and tricuspid valves decreased significantly during the test. The acceleration time-to-deceleration time ratio at the pulmonic wave was unchanged. A significant, progressive increase in cardiac index also was observed during dobutamine infusion, with a maximal increase of 104% from baseline. This was mediated initially by an increase in stroke index and, at higher dosages, by an increase in heart rate. CONCLUSIONS AND CLINICAL RELEVANCE: Doppler echocardiography performed during dobutamine stress testing may be a reliable method of assessing myocardial function in dogs with cardiovascular disease.

Usefulness of dobutamine stress tests for detection of cardiac abnormalities in dogs with experimentally induced early left ventricular dysfunction.

OBJECTIVE: To determine whether dobutamine stress tests (DST) can be used to detect cardiac dysfunction in dogs with early left ventricular dysfunction (ELVD) induced by rapid right ventricular pacing (RRVP). ANIMALS: 7 adult male Beagles. PROCEDURE: A pacemaker was surgically implanted in each dog at the level of the right ventricular apex. Electrocardiography, Doppler sphygmomanometry, and Doppler echocardiography were performed before and during a DST prior to activation of the pacemaker and every 3 to 4 days during the period of RRVP. Dobutamine stress tests were performed by infusing dobutamine at incremental dosages ranging from 12.5 to 42.5 microg/kg of body weight/min. RESULTS: Clinical signs of congestive heart failure were not observed during the pacing period. However, all dogs developed ELVD associated with significant changes in values for most Doppler echocardiographic variables obtained prior to DST Adverse cardiac effects were not detected during DST. Most Doppler echocardiographic indices of cardiac function were significantly altered in response to dobutamine infusion during the pacing period, compared with prepacing values. However, a dobutamine-induced 2-fold increase in cardiac output was maintained. CONCLUSIONS AND CLINICAL RELEVANCE: Dobutamine stress tests can be safely performed in dogs with experimentally induced ELVD. Dobutamine stress tests may be a sensitive, noninvasive diagnostic method, complementary to standard clinical examinations, for detection of early cardiac dysfunction in dogs asymptomatic for dilated cardiomyopathy.

Successful tricuspid valve replacement and pulmonary valvulotomy for carcinoid heart disease.

A case of successful tricuspid valve replacement with a mechanical prosthesis and pulmonary valvulotomy for carcinoid heart disease is reported. The patient was a 61 years old women. The primary tumor was in the terminal ileum. Liver metastasis and carcinoid syndrome were present since 8 years. After cardiac surgery, the patient survived 38 months and late death was related to disseminated metastasis. Even in case of metastasis, carcinoid tumor is slow growing. Without cardiac operation for correction of valvular lesions, terminal symptoms and death may often be related to cardiac operation for correction of valvular lesions, terminal symptoms and death may often be related to cardiac failure rather than to tumoral growth. Thus, even in presence of metastasis, cardiac surgery may be mandatory to improve both quality of life and survival.

[How I treat...congenital cardiopathies in adults]. / Comment je traite.... les cardiopathies congénitales de l'âge adulte.

Nowadays, the diagnosis of congenital heart disease is generally made in the infant, or even at times, in utero. Neonatal surgery has in this field made remarkable progresses. In this article, we describe the situation of patients who reach the adult age without having their congenital abnormality corrected. Late surgery or interventional catheterisation can offer improvement in terms of survival and quality of life. It is not always too late to influence the patient's prognosis significantly.

[Pre-competition health screening of young high-level athletes]. / Examens d'aptitude à envisager chez le jeune candidat à la pratique d'un sport de haut niveau.

The unexpected death of an athlete during the practice of his sport is generally an event largely covered by the newspapers. It raises the problem of screening the athletic population in the aim to disclose anomalies (generally cardiac in origin) that predisposes to sudden death during exercise. In young people, most of these episodes are due to cardiac disorders that can be detected before initiation of the training program. The low prevalence of these disorders, the large number of people to screen and the cost of the examinations however appear to be limiting factors for this type of mass screening. Despite this notion, a scheme of a screening program is proposed in order to prevent as much as possible the occurrence of sudden death in young athletes.

[Atherosclerotic pathology of the aortic arch and cerebrovascular accidents]. / Pathologie athéroscléreuse de la crosse aortique et accidents vasculaires cérébraux.

Over the last 10 years, transoesophageal echocardiography has allowed the visualisation of the particularly frequent atheromatous involvement of the initial part of the aorta in patients suffering from cerebral as well as peripheral embolism. Recent studies have shown that atherosclerosis of the initial, precarotid, part of the aorta is both a marker of general as well as coronary atherosclerosis and an important, accident independent risk factor of recurrence of stroke. The therapy of these aortic lesions is still currently under debate and there are no published guidelines about this issue in the literature. We therefore have applied the Evidence-Based Medicine information gathering method described earlier in this journal in order to better define the best therapeutic approach of this type of pathology.