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Mitral prolapse is a common condition, defined by the systolic bulging of at least one mitral leaflet into the left atrium, which is often accompanied by various degree of mitral insufficiency. While for most of the patients the prognosis is linked to the severity of the valve regurgitation and its repercussions on the left ventricle (dilation and/or dysfunction), a minority of patients present with severe ventricular arrhythmia and an increased risk of sudden cardiac death, irrespective of the severity of the mitral regurgitation. To describe this particular condition, the terms arrhythmic or malignant mitral valve prolapse have been coined. The aim of this article is to describe the clinical, electrocardiographic and morphologic characteristics, which have been associated with an increased risk of arrhythmia in patients with mitral prolapse.Le prolapsus mitral est une pathologie fréquente, définie par le bombement en systole d’au moins un feuillet mitral dans l’oreillette gauche, qui s’accompagne fréquemment d’un degré variable d’insuffisance mitrale. Dans la majorité des cas, le pronostic est lié à la sévérité de l’insuffisance valvulaire et ses répercussions sur le ventricule gauche (dilatation et/ou dysfonction). Toutefois, dans certaines formes, le prolapsus mitral se manifeste par une susceptibilité aux arythmies ventriculaires et s’associe à un risque accru de mort subite, indépendamment de la présence ou de la sévérité de l’insuffisance valvulaire. On parle alors de syndrome du prolapsus mitral arythmique ou prolapsus mitral malin. Cet article décrit les caractéristiques cliniques, électrocardiographiques et morphologiques associées au risque arythmique chez les patients atteints d’un prolapsus mitral.

The association between the mitral valve prolapse and the sudden Cardiac Death remains controversial, the high prevalence of this valvulopathy contrasting with the low incidence of sudden death in this population. We report the case of a 54-year-old woman admitted for a sudden cardiac death, revealing a bi-prolapse with low-grade leakage, leading to the implantation of a subcutaneous automatic defibrillator. Combined echocardiography and cardiac MRI can identify the mitral annular disjunction, the rolling motion of the posterior face of the mitral annulus towards the myocardium, and the myocardial fibrosis of the inferolateral wall induced by streching forces of the sub valvular apparatus, that may lead to ventricular arrhythmias. More than the conventional clinical parameters (young woman, ventricular premature beats with a right bundle branch block morphology, mitral bi-prolapse), mitral annular disjunction and myocardial fibrosis are to be considered as powerful markers of the rhythmic risk of mitral prolapse and must be systematically sought and integrated into the prognostic evaluation of these patients. In the absence of randomised trials, therapeutic management is difficult especially in primary prevention, and needs Heart Team advice.

To analyse and discuss cardiovascular manifestations of ankylosing spondylitis, evaluate the frequency and giving special attention to unusual forms.We retrospectively reviewed the medical records of 21 cases with cardiovascular manifestations among 210 cases of ankylosing spondylitis seen over a 25 years period who met the Amor criteria.Cardiovascular complications were found in 21 patients (10%): 5 (2.40%) had aortic insufficiency, 2 (0.95%) had mitral insufficiency, 1 had mitral valve prolapse, 1 had pericarditis, 10 (4.7%) had atrioventricular block and 6 (2.8%) had bundle branch block. Takayasu's disease was diagnosed in one case. Cardiovascular involvement was common In patients longer disease duration. Peripheral arthritis was found in 33% and its prevalence did not differ in patients with or without cardiac involvement.Aortic insufficiency and conduction disturbances were the most common cause of cardiovascular involvement in ankylosing spondylitis. The funding of cardiovascular manifestations in 10% of our patients suggests that in this illness evidence of cardiac manifestations should be actively investigated in ankylosing spondylitis.

The posterior mitral leaflet is usually motionless following mitral valve repair. The aim of this study was to assess (1) the geometric changes of the left ventricular base following prosthetic ring annuloplasty and (2) their impact on the anterior mitral leaflet (AML) mobility. Thirty five patients operated upon for mitral valve repair underwent an intraoperative transesophageal echographic study before and after annuloplasty. A posterior leaflet resection was achieved in 29 cases and ring annuloplasty alone in 6 cases. No repair technique was performed on the AML. Four parameters were assessed: the anteroposterior mitral annulus diameter, the aortomitral angle, the opening and closure angles of the AML. Annuloplasty resulted in a drastic reduction of the mitral annulus from 36.8 +/- 5.6 mm to 20.9 +/- 3.8 mm (systole, long axis view) (p0.0001). The aortomitral angle decreased following annuloplasty from 115.1 +/- 8.3 to 108.0 +/- 9.60 (systole, long axis view) (p0.0001). No difference was observed between systolic and diastolic measurments concerning the mitral annulus or the aortomitral angle. The opening angle of the AML remained unchanged whereas the closure angle increased from 17.8 +/- 6.10 to 26.6 +/- 6.70 (long axis view) (p = 0.0001) resulting in a displacement of the coaptation point towards the apex. Consequently, the excursion of the anterior leaflet throughout the cardiac cycle decreased following annuloplasty from 43 +/- 130 to 32.5 +/- 11 (long axis view) (p0.0001).

Steinert disease, the most common myopathy in adults, is a challenge for anaesthesiologists and critical care physicians during the perioperative time. The risk of myotonic crisis, malign hyperthermia and the increased sensitivity to anaesthetic drugs shouldn't be forgotten. On contrary, Steinert disease is rarely revealed in the postoperative period. It should be evoked in case of postoperative pulmonary complications such as difficult weaning with neurological symptoms like hypotonia or muscular weakness.

Real-time three-dimensional echocardiography is currently used in a standard echocardiographic examination. Volume-rendered images better identify and locate anatomic structures and improve our comprehensive approach to various heart diseases. The assessment of mitral valve disease and congenital cardiopathies and the measurement of left ventricular mass, volume, and ejection fraction are the three main applications of three-dimensional echocardiography. Three-dimensional vascular imaging is an emerging and promising application of three-dimensional echography. The near future of three-dimensional echography requires the integration of all modalities of conventional echography in three dimensional probes, a higher image resolution compared to the current situation, as well as the development of real-time three-dimensional probes dedicated to transesophageal cardiac or vascular examination.

There is only a few data on sudden unexpected death (SUD) in the pediatric population, as well as the role of sport.Between 1980 and 2003, 2220 autopsies were performed at the Lyon's forensic institute for SUD (all ages) as defined by world health organization.Fifty-seven cases of SUD of cardiac origin concerned 6 to 18-year-old children. Hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy remained the main causes of SUD. Mitral valve prolapse were frequently observed and for the most part of the cases associated with other diseases. Twenty-four were observed during physical activity.Our study confirms the low incidence of SUD and its male predominance in the pediatric population (47 males, 10 females). It seems that physical activity is not the decisive factor for SUD. The authors emphasized the necessity to get a rapid access to cardiac resuscitation devices, as well as the formation of collaborators. Some preventive measures could be done.

There are few literature data on the localization and extent of mitral valve prolapse zones with transesophageal echocardiography (TEE).To assess a standardized imaging technique for the localization and extent determination of prolapse zones, based on 3 easily reproducible views with multiplane TEE.Seventy patients with severe mitral regurgitation due to valve prolapse requiring a multiplane TEE prior to surgery (valve repair or replacement) have been retrospectively assessed. Data of TEE on the localization and extent of prolapse zones have been confronted to per-operative anatomical observations (gold standard).The sensitivity of TEE for the identification of isolated P2 prolapse, prolapse with commisural extension, isolated rupture of the posterior commisure and bi-valvular prolapses were respectively at 96%, 88%, 86% and 80%. The corresponding specificities were from 98% to 100%.The use of a standardized technique with the use of 3 easily reproducible incidences with multiplane TEE allows a precise definition of the localization and extent of mitral valve prolapse zones, in order to potentially indicate valve repair.

Mitral valve prolapse should be considered as a disease when superior displacement of the mitral leaflets during systole is more than 2 mm with a maximal leaflet thickness of at least 5 mm. Using these criteria, the prevalence of mitral valve prolapse is 1.3% in the general population, nearly the same in men and women. Serious complications may occur during follow-up with a 1 to 3% patient-year risk. The most important complication is mitral regurgitation, mainly due to rupture of the chordae tendineae, which must be quickly corrected by surgical repair. Second is infective endocarditis, a complication which may occur particularly in men older than 45 years of age with systolic murmur. Arrhythmias are not infrequent but ischemic neurologic events are unusual, especially in young patients. Cases of sudden death have occasionally been reported.

Mitral valve prolapse (MVP) is a common disorder that, in general, has a good prognosis. Rare occasions of sudden death have been reported in patients with MVP and it is presumed that the basis of sudden death is arrhythmias. We report a case of a 47 years old men affected by MVP complicated by ventricular arrhythmias and sinoatrial block; who died suddenly from ventricular tachycardia. The pathophysiology and risk factors of sudden cardiac death in MVP are discussed.

Type II mucopolysaccharidosis (Hunter's disease) is a hereditary condition due to a deficit of a lysosome specific hydrolase (iduronate sulfatase) inducing an accumulation of dermatane-sulphate and heparane-sulphate in certain organs. Cardiac involvement is constant in this disease and manifests itself essentially by aortic valve stenosis and insufficiency and/or mitral insufficiency which is progressive, irreversible and life-threatening. Two brothers of Portuguese nationality suffering from a so-called slight form of this disease had classical mucopolysaccharide infiltration of their aortic valves. The elder brother, aged 11, had severe aortic insufficiency associated with mild stenosis requiring treatment with vasodilator drugs. The younger, aged 8, had asymptomatic mild aortic regurgitation. Curiously, mitral valve prolapse with regurgitation was present in both children. The association of mitral valve prolapse and type II mucopolysaccharidosis, without other typical cardiac involvement, has only been reported once in the literature.

Some of the classical concepts of mitral valve prolapse (MVP) should be reviewed in the light of recent publications. It is a condition, according to strict echocardiographic criteria excluding near physiological abnormalities, which affects 2 to 3% of the adult population in the industrialised world. Only repetitive atrial arrhythmias and complex ventricular arrhythmias are more common in this condition than in control groups, the differences being more pronounced in cases of mitral regurgitation. The risk of syncope or sudden death is 0.1% per year, hardly any different to that of the rest of the general adult population (0.2%). However, this risk may attain 0.9 to 2% in cases with mitral regurgitation. The causes of sudden death are unclear (haemodynamic, neurohumoral, arrhythmic, etc...), although there is evidence in favour of malignant ventricular arrhythmias. Detailed clinical, electrophysiological, isotopic and anatomopathological studies have raised doubts as to the direct responsibility of the vascular malformation (or its eventual consequences on the atrial and ventricular chambers) in this mode of fatal outcome. On the other hand, localised or diffuse myocardial disease is often observed, usually a- or pauci-symptomatic, associated with MVP, the responsibility of which is more plausible. Therefore, the physician should adopt a flexible attitude towards these patients, reassuring those with benign symptoms at low risk and following up or actively treating the rarer malignant forms (especially familial, syncopal with mitral regurgitation and/or severe arrhythmias).

The concept of mitral valve repair is discussed, emphasizing the basic knowledge on which it relies, and the breakthrough it has produced in modern cardiac surgery. This concept challenges the myth of the artificial prosthesis, showing the superiority of autologous biological structures submitted to plastic repair. The foundations of valvular repair and the techniques that have contributed to its success are reviewed, and the advantages for the patients in terms of quality of life, and preservation of the left ventricular function are discussed.

Advances in cardiac imaging techniques have resulted in the recognition of several potential cardioembolic sources (such as patent foramen ovale, atrial septal aneurysm, strands, mitral valve prolapse), but it is often not clear whether the findings, which often have a substantial prevalence in patients without stroke, are merely an associated abnormality or a direct cause of the stroke. Overall, these cardiopathies are associated with a low risk of stroke. Studies are needed to better define the risk and mechanisms of stroke associated with these cardiac abnormalities, to determine which patients are at increased risk of stroke and to assess the potential benefits from therapeutic interventions. Unless we can define strict indications for therapeutic interventions, we risk ending up with exposing some patients to unnecessary complications of treatment.

We report the case of a 73-year-old woman who presents an acute mitral regurgitation secondary to fibro-elastic degenerescence. A mitral valvuloplasty is rapidly performed with complete disappearance of valvular insufficiency and the patient leaves the hospital ten days later. Degenerative mitral regurgitation is commonly seen in elderly patients. This constitutes an excellent indication for mitral valvuloplasty, rather than valve replacement. In our experience, mitral valvuloplasty leads to a complete correction of valvular regurgitation without neither stenosis, nor subvalvular obstruction. Left ventricular function is preserved, as subvalvular apparatus is conserved. This approach allows, in the absence of specific indication, anticoagulation withdrawn two to three months later.

Ischemic cerebro-vascular events are in 15% of cases secondary to a cardiac embolus. The prevalence of echocardiographic anomalies, susceptible of being at the origin of a cerebral embolus may reach 50%. Transesophageal echocardiography (TEE) is superior to transthoracic echocardiography (TTE) in the detection of a thrombus of the left atrial appendage, spontaneous echocontrast, intra-aortic atherosclerotic plaque lesion, patent foramen ovale or an atrial septal aneurysm. The high prevalence of these anomalies in a population who presented an ischemic cerebrovascular accident does not suffice to establish a causal relationship between the anomaly and the cerebral event. Further studies seem to be necessary to establish the responsibility of these different anomalies and determine their embolic risk, by defining certain risk factors. Pending the results of such studies, TEE should be indicated more systematically in the case of an ischemic cerebrovascular accident of the young patient without a clinically evident cardiopathy, independently of the results of TTE.

Three-dimensional echocardiography is based on two methods of retrospective reconstruction from two-dimensional echocardiographic images. The acquisition of the two-dimensional images may be free or imposed, the transducer either carrying an emission-reception system or fixed to an articulated support providing data about its position. In the first system, manual tracing of the contours of the region of interest performed on each frame are superimposed after time sequencing (using the ECG) and spatial repositioning, so enabling three-dimensional visualisation of the contours of the cardiac structures: this approach provides reliable quantitative information (volumes, mass and ejection fractions) and has led to the redefinition of the echocardiographic criteria of mitral valve prolapse. The second system is based on equidistant sections obtained by progressive, controlled two-dimensional scanning (parallel, arc of a circle and rotational) of the structure of interest: a value of grey scale is assigned to the space between two adjacent pixels, enabling the formation of voxels which, when superimposed, give the required effects of volume and surface for three-dimensional imaging. It is then possible to obtain any section of the volume and simulate surgical views of the beating heart. This approach may significantly improve diagnostic accuracy compared with two-dimensional echocardiography and provides access to new quantitative and qualitative parameters.

Left and right ventricular wall motion was studied in mitral valve prolapse with or without ventricular arrhythmias. Regional and global ventricular wall motion was evaluated by isotopic methods, based in ejection fraction and Fourier phase analysis representing the progression of wall contraction. The synchronisation of the ventricles was characterized by the difference of the mean phase of each ventricle. The heterogeneity of contraction of each ventricle was defined by the dispersion around the mean (standard deviations of the phases). Fifteen of the 36 patients had complex ventricular arrhythmias (Lown gradeor = III). 12 had LVP and 16 had mitral regurgitation. In mitral valve prolapse, the RV EF was decreased compared with normal controls (30 +/- 9% vs 40 +/- 10% ; p0.001), especially in patients with mitral regurgitation (26 +/- 7% vs 30 +/- 10%; p = NS) and complex ventricular arrhythmias (26 +/- 7% vs 32 +/- 10%; p0.01). The SDP of the LV was greater than those of controls (18 +/- 11 degrees vs 11 +/- 5 degrees ; p = NS) whereas the SDP of the RV was greater (27 +/- 17 degrees vs 12 +/- 5 degrees ; p0.05) especially in those with complex ventricular arrhythmias (36 +/- 21 degrees vs 21 +/- 10 degrees : p0.01). The SDP of LV and RV were greater in patients with mitral regurgitation: 20 +/- 11 degrees versus 17 +/- 10 degrees (NS) and 35 +/- 21 degrees versus 20 +/- 8 degrees (p0.01). Heterogenous ventricular contraction, more marked in the right ventricle in mitral valve prolapse suggests severe myocardial disruption in this valvular disease, reflected by the high incidence of LVP and complex ventricular arrhythmias.

Prolapsed mitral valve prolapse (PMV) is classically associated with disorders of ventricular excitability whose significance is unclear. However, syncope can suggest the possibility of a serious ventricular arrhythmia. The objective of this study was to try to identify the mechanisms of dizziness and syncope associated with PMV. We report the results of programmed atrial and ventricular stimulation performed under baseline conditions and after administration of Isuprel in 56 patients with PMV: 27 patients had a history of presyncope or syncope (group I), 14 had spontaneous atrial or supraventricular tachycardias without dizziness or syncope (group II) and 15 were asymptomatic and investigated for VEBs or conduction disorders (group III). The following results were obtained: In group I, 6 patients experienced sustained inducible ventricular tachycardia (VT); an atrial tachycardia (atrial tachycardia and/or atrial fibrillation) (AT) was also induced in 5 of them. In another 19 patients, a supraventricular tachycardia (SVT) and/or AT was induced. A total of 24 atrial or junctional tachycardias were triggered in this group. In group II, AT and/or SVT were reproduced in 13 out of 14 cases (93%). In group III, AT was triggered in 3 patients (20%). SVT were induced by Isuprel while AT were triggered prior to administration of Isuprel, under baseline conditions, and 3 of them were reproduced during vagal manoeuvres. A ventricular arrhythmogenic effect was observed in two cases in group II while taking class I antiarrhythmics. In conclusion, spontaneous AT and SVT of PMV are easily inducible with a sensitivity of 93%, but are difficult to induce in asymptomatic subjects. The high incidence of TA and SVT in the case of unexplained presyncope in subjects without documented tachycardia therefore appears to be suggestive of a relationship between these presyncopes and AT or SVT. However, the search for VT should take precedence. SVT appear to be catecholaminergic while AT tend to be vagal.

The authors report the case of a 67-year-old woman, with mitral valve prolapse, for more than 20 years. She recently complained of attacks of syncope and clinical ventricular tachycardia; ventricular fibrillation was induced during programmed stimulation. The patient seemed to be at high risk for sudden cardiac death, and was therefore treated with an automatic implantable defibrillator. The pathophysiology and risk factors of sudden cardiac death in mitral valve prolapse are discussed.

From January 1993 to December 1994 twelve patients were evaluated for sudden hearing loss. The median age was 49 years with a range of 18 to 71. All had severe or profound initial hearing loss. The incidence of bilateral disease was 25%. Total deafness occurred in five (33%) ears. Nine (75%) patients had vestibular symptoms and eight (67%) admitted experiencing tinnitus. Two-dimensional echocardiography revealed mitral prolapse in eight (67%) patients; another patient showed moderate to severe ischemic left ventricular dysfunction with apical aneurysm.

Type IV Ehlers-Danlos syndrome, a rare disease caused by abnormal synthesis of type III collagen, often leads to vascular fragility. We report 9 cases (6 men and 3 women, mean age 35 years). For 7 of the patients, the inaugural signs were arterial complications including haemoperitoneum in 2 patients with multiple aneurysmal dystrophy of the abdominal arteries, one case of ruptured subclavian artery, two dissections of the renal artery, one case with rupture of a cerebral aneurysm, one rupture of the mesenteric artery and a haematoma after arterial puncture. Other vascular manifestations were acrosyndrome (n = 4), varicose veins (n = 3), and prolapsus of the mitral valve (n = 2). In addition, 8 of the 9 patients presented extravascular signs. There was a history of familial disease in 5 cases. Pregnancy was completed to term in three patients: a cesarean section was required in one case and intra-uterine growth retardation was seen in 2. Morbidity was important with hemiparesia, blindness and paraparesis sequellae. One patient died from haemorrhage. This series of patients with type IV Ehlers-Danlos syndrome illustrates the severity of this disease whose prevalence is often underestimated. The disease is transmitted by autosomal dominant inheritance, underscoring the importance of familial testing for early diagnosis. Clinicians should be aware of the vascular manifestations and avoid invasive punctures or operations except in exceptional indications.

Ruptured mitral chordae tendinae is a classical complication of myxomatous mitral valves or Barlow's syndrome. This complication is controversial in non-myxomatous mitral valve. Of 91 consecutive patients with mitral valve prolapse examined over an 18 months period by transthoracic and transesophageal echocardiography, 42 (18 women and 24 men) with an average age of 76 +/- 8 years (60-93 years) had ruptured mitral chordae tendinae. The thickness of the anterior mitral leaflet measured at the distal third of the valve by transesophageal echocardiography enabled the identification of two groups of patients; group I:3 mm (24 patients), average 4.8 +/- 0.8 mm and group II:or = 3 mm (18 patients), average 2.6 +/- 0.3 mm. The diameter of the mitral ring and left atrium, the length of the anterior mitral leaflet, the left ventricular end diastolic dimensions and fractional shortening, were measured by transthoracic 2D echocardiography (mitral ring) and M mode (other parameters). Ruptured chordae were detected in only 13 cases (31%) by transthoracic echocardiography; 38% were asymptomatic and a chance finding at transesophageal echocardiography. No significant difference was observed between the two groups with respect to age, gender presence of hypertension, dimensions of the cardiac chambers, fractional shortening or localisation of the prolapse related to the ruptured chordae. Fifty-eight per cent of patients in group I were in NYHA functional classes 3-4 as compared to 16% in group II (p0.02). The size of the left atrium was significantly greater in group I, 51 +/- 8 mm vs 38 +/- 7 mm (p0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

The authors report two cases of severe myocardial ischaemia with healthy coronary arteries associated with mitral valvular prolapse (MVP). The first case was a 43-year-old woman treated with beta-blockers following the discovery of MVP. This patient was admitted to hospital six months later with persistent chest pain in a context of cardiogenic shock. The response to treatment was rapid and spectacular. The second case was a 44-year-old hypertensive smoker man in whom assessment of chest pain revealed several signs of myocardial infarction as well as MVP. This rare combination of MVP and myocardial ischaemia raises pathophysiological as well as therapeutic problems.

We conducted this study to evaluate the role of transoesophageal echocardiography in the aetiologic diagnosis of ischaemic cerebral vascular events in young subjects.Over a 16-month period, 70 consecutive patients under 55 (mean age 49 years; range 32-55; 34 females, 36 males) underwent transoesophageal echocardiography as part of a complete work-up after a recent (1 month) cerebral event considered to be ischaemic in nature. Exclusion criteria were age over 55, vascular stenosis or ulcerated plaque, embologenic heart disease and valve prosthesis. 24-h Holter recordings were also obtained in all patients.The cerebral event was temporary in 11 subjects and permanent in the other 59. The ischaemic nature of the event was confirmed in all patients with computed tomography or magnetic resonance imagery. All patients were in sinus rhythm at examination and supraventricular paroxysmal arrhythmia was observed in 7 during the Holter recordings. The transoesophageal echocardiography was normal in 37 patients (52%) and pathologic in 33 (48%). The source of the embolus was identified in 4 cases (6%) and the probable cause of the cerebral event was found in 29 (42%). Among the abnormal structures observed were permeable oval foramens, inter-atrial septum aneurysms and plaques on the ascending aorta.In our selected population of patients under 55 years of age, transoesophageal echocardiography detected the certain or probable source of the ischaemic cerebral event in 48% of the cases, a diagnostic yield better than most other methods. Nevertheless, there is no known therapeutic response to several of the potential sources of embolus observed.

In order to determine the predictive value for ventricular arrhythmias of ventricular late potentials (LP) in mitral valve prolapse (MVP) the authors performed high amplification signal-averaging ECG (SA) and 24 hours ambulatory ECG (Holter) monitoring in 68 consecutive patients (34 men, 34 women, average age 48 +/- 17.7 years) with echocardiographically diagnosed MVP. Patients with bundle branch block or associated cardiac disease were excluded. Echocardiography showed 26 patients to have floppy mitral valves (38.2%), 50 patients to have posterior deplacementor = 5 mm of the mitral valves in systole (73.5%) and 35 patients to have mitral regurgitation (51.4%). Holter monitoring showed 17 patients without ventricular extrasystoles (VES), 15 had Lown Grade I, 6 had Lown Grade II, 3 had Lown Grade III, 15 had Lown Grade IV A and 12 had Lown Grade IV B ventricular arrhythmias. Therefore, 30 patients had complex ventricular arrhythmias (or = Lown Grade III) and 13 patients had spontaneous non-sustained ventricular tachycardia (NSVT) (one patient had NSVT on resting ECG but not on Holter monitoring). Eighteen patients had LP (26.5%). The incidence of complex ventricular arrhythmias was higher in patients with mitral regurgitation (62.8% versus 27.7%; p0.005) whereas the incidence of NSVT was not significantly different (25.7% versus 17.1%; p = 0.15). On the other hand, the frequency of complex ventricular arrhythmias was not significantly different in the presence or absence of LP (61.1% versus 40%: NS) whereas the incidence of NSVT was higher in patients with LP (44.4% versus 10%; p0.005).(ABSTRACT TRUNCATED AT 250 WORDS)

Mitral valve prolapse (MVP) a common condition easily recognised by echocardiography with, however, strict criteria to avoid diagnosis by excess, may be complicated by arrhythmias. Two very different situations oppose severe decompensated mitral regurgitation due to myxoid dystrophy and quasi-asymptomatic MVP with a good prognosis and a low risk of complications. The important question is therefore to detect risk criteria of sudden death in patients with few symptoms. Unfortunately, no isolated factor or association of factors resolves this problem in a given patient. However, it is usually young women without severe mitral regurgitation but with thickened valves and, sometimes, a long QT interval, who are involved. Programmed ventricular stimulation and the detection of later ventricular potentials do not seem to be useful in the present state of our knowledge.

Transoesophageal echocardiography (TOE) is a new modality of cardiac investigation allowing examination of cardiac structures and anomalies previously inaccessible to ultrasound. TOE is performed under local anaesthesia after fasting the subject for more than 4 hours and after premedication when the patient is admitted to hospital. In the assessment of an ischaemic cerebral vascular accident, TOE may reveal potential sources of emboli such as intracavitary thrombi and tumours (left atrium and left atrial appendage), atherosclerosis of the aortic arch, vegetations. Certain indirect causes may also be more clearly identified: aneurysm of the interatrial septum, patent foramen ovale for which the causal relationship is more difficult to establish. Intra-atrial blood stasis ("spontaneous echo contrast") is perfectly analysed in the form of intra-atrial "smoke" observed in the presence of atrial dilatation, particularly in the presence of atrial fibrillation and mitral valve obstruction (mitral stenosis, valve prosthesis). The indication for TOE in this context is currently under evaluation, particularly in young patients. It is routinely indicated in patients with known heart disease when peripheral embolism is suspected, looking for intracavitary thrombosis, generally not seen on transthoracic echocardiography.

Tearing of the chordae is a common complication of prolapse of the mitral valve; which becomes increasingly common with age. In contrast, aneurysm of the Valsalva sinus is rare in patients over 65 years of age. The antero-left sinus is an unusual location, but may be complicated by compression of the left coronary artery, which is reported in 16 cases in the literature. The case reported is unusual due to the combination in a 66-year old subject of acute mitral incompetence, due to tearing of the chordae following prolapse of the mitral valve, and an aneurysm pressing on the coronary trunk whilst remaining asymptomatic. Complete correction of the lesions was successfully achieved by surgery.

Sustained ventricular tachycardia in the absence of coronary artery disease is mainly observed in diffuse left ventricular cardiomyopathy especially dilated but sometimes hypertrophic, and in right sided cardiomyopathies such as ventricular dysplasia, more difficult to diagnose. More rarely, other localised abnormalities, mitral valve prolapse, cardiac tumour and idiopathic aneurysm, may give rise to this arrhythmia. Irrespective of the case, sustained ventricular tachycardia carries a poor prognosis during the most advanced stages of a myocardial disease. The therapeutic strategy remains the same as that of other cases of sustained ventricular tachycardia, drug therapy generally orientated by the results of programmed ventricular stimulation with the exception of Class I antiarrhythmics when the ejection fraction is under 30%, and non-pharmacological methods when drug therapy fails.

The relationship between anxiety and cardiovascular function and symptoms has long been of interest, culminating in the recent emphasis on the modulation of cardiovascular response in patients with panic disorder. The relationship between panic disorder and mitral valve prolapse remains controversial. Panic disorder appears to be significantly associated with increased incidence of cardiovascular morbidity. The detection and treatment of panic disorder in patients with cardiovascular risk or diseases could have an important impact on prognosis and quality of life of the patients.

Signal averaged electrocardiogrammes were recorded in 100 consecutive patients with echocardiographic mitral valve prolapse (MVP) and compared with 50 normal control subjects. Criteria of normality were defined in the reference population: QRS duration after averaging113 ms, Simson's vector of the last 40 ms (RMS40)or = 17 microV, and duration of the terminal potential within the 40 microV range38 ms, using a high-pass bidirectional 40 Hz filter. Of the 100 patients with MVP, late ventricular potentials (LP) were recorded in 38 cases with at least 2 criteria of positivity, compared to only 3 out of 50 (6%) in the control group (p0.01). The prevalence of LP was very dependent on the appearances of the valve; when the valve was of normal thickness and only showed slight systolic bulging there were only 5/46 cases of LP (10.9%) which did not differ significantly from the control group. On the other hand, when the valve showed myxoid changes, the prevalence of LP was very high: 33/54 = 61% (p0.001 versus controls). Six patients had severe ventricular arrhythmias (sustained ventricular tachycardia or ventricular fibrillation): all had LP. In cases of MVP with frequent ventricular extrasystoles (VEs) the prevalence of LP was the same with normal valves (2/23 = 9% and 2/22 = 9%, NS) whereas LPs were more common in cases of VEs with myxoid valve (23/33 = 70% versus 5/16 = 31%; p = 0.01). The high prevalence of LP in MVP with myxoid valves is a convincing argument in favour of myocardial abnormality in this disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic dissection after cardiac surgery is a rare complication. The prognosis is often poor: 33-78% of mortality. The study of two cases out of 2100 patients operated upon during the last three years, and the review of the literature, allows to recall the mechanism, the anatomic origin and the different surgical techniques. Early diagnosis is essential. Some preventive techniques may reduce the incidence of the iatrogenic dissections.

Mitral valve prolapse (MVP) screening using bidimensional echocardiography was performed on 171 healthy subjects from Lorraine of both sexes, aged 20 to 60 years. Six men and seven women presented this valvular disease. In the Lorraine population, the estimated prevalence of MVP was 7.6% (95% confidence interval: 3.6-11.6%). In this study, only seven cases of MVP had a cardiac auscultation suggestive of MVP. As compared with echocardiography, clinical screening of MVP had a low validity (sensibility 54%, specificity 69%) and a poor positive predictive value (12.5%). Because MVP has severe complications, and because clinical screening has a low performance, a strategy of screening with bidimensional echocardiography could be interesting.

The potential thrombotic risk of mitral valve prolapse may, in certain circumstances, require preventive treatment. This study was aimed at determining whether the presence of angina in patients with mitral valve prolapse and healthy coronary vessels was accompanied by a high-risk thrombogenic profile. Forty two patients (19 women and 23 men) with anginal chest pain and angiographically normal coronary vessels were divided into two populations according to the presence (18 patients) or absence (24 patients) of mitral valve prolapse (MVP) shown by angiography. Before angiography, all patients underwent laboratory studies to detect any possible abnormality of plasma coagulation and of prothrombotic physiological fibrinolysis. Study of subgroups, according to sex and/or the presence of MVP, revealed no significant difference in the profile of laboratory parameters. Thus the presence of angiographic MVP in symptomatic patients free of atherosclerosis is not associated with the existence of any particular thrombotic profile and, theoretically, does not require preventive anti-thrombotic treatment.

Mitral valve prolapse should be considered a benign condition as there are few complications. The natural history is not significantly different to that of a matched control population. There are potentially severe forms of the condition which are mainly observed in specialised centres and this introduces a bias in patient selection when compared with the general population. These forms are relatively rare in clinical practice and can be easily identified by simple echocardiographic criteria of valvular thickness. In any case, patients with mitral valve prolapse should be reassured in order not to create or unduly increase anxiety which in itself may cause incapacity.

The reputation of mitral valve prolapse being a benign condition is based to a great extent on the fact that complications are rare in minor forms, but also because a number of studies of the condition included normal subjects, especially of the female sex. The prevalence of mitral valve prolapse in the general population is 4 to 5%. Approximately 20% of these patients have marked redundancy of valve tissue and are particularly exposed to complications. The incidence of infective endocarditis in cases with an audible murmur is 0.05% per year. The incidence of mitral regurgitation increases with age, so that the annual probability of surgical correction is 0.03%. The risk of sudden death in cases without mitral regurgitation is low (2/10,000 per year) but it is 50 to 100 times greater when mitral regurgitation is present. The frequency of arrhythmias is also higher in cases with mitral regurgitation and that of cerebro-retinian ischemia is estimated to be 0.02% per year. Therefore, a serious complication (endocarditis, sudden death, surgical mitral regurgitation, cerebral or retinian ischemia) occurs each year for every 1,000 mitral valve prolapses, or for a population of 25,000 inhabitants.

The investigation of a cardiac murmur in a 9 month old infant led to the clinical, echo and angiographic diagnosis of congenital mitral regurgitation due to isolated mitral valve prolapse, whereas the electrocardiogram showed signs of inferior myocardial ischaemia which regressed in a week. The valvular dysfunction of mitral valve prolapse has been held responsible for coronary spasm ischaemia and infarction in adults. This complication has not been previously reported to our knowledge in infancy.

Mitral valve repair surgery, in presence of a pure mitral leakage or one associated to a stenosis, is not only possible but has been well codified for a decade. According to damage, there are two methods of operation: valvular mobilization surgery and valvular motion amplitude reduction surgery. They are usually associated to annuloplasty with a Carpentier prosthetic ring. The incidence of late mortality is of 0.6 p. 100 pt/yr, that is to say 91.7 p. 100 at 13 years. This late survival rate is about 20 p. 100 better than for a valvular replacement. Reoperations rate is 1.6 p. 100 pt/yr. The incidence of thromboembolic event occurrence is low: 0.5 p. 100 pt/yr. The ideal indications for mitral valve repair are represented by damage of prolapse from a degenerative origin for which results are better and more constant. For rheumatic damage, the valvular repair indication depend on the valvular tissue elasticity and area. The presence of calcification and extensive fibrosis remain on principle counter-indications.

One-hundred patients with unexplained cerebral ischaemic events were explored by two-dimensional echocardiography. Thirty-nine patients showed abnormalities considered causative of embolic stroke. The most frequent cardiopathy found was mitral valve prolapse (38.5 per cent). This study suggests that unexplained cerebral ischaemic events should be evaluated by two-dimensional echocardiography and that the most frequent source of emboli to be expected is mitral valve prolapse.