Congenital pericardial defect: report of two cases and literature review.

The authors report two cases of congenital pericardial defect and emphasize the role of MRI as a diagnostic tool. A review of the development of the pericardium and of the various possible explanations dealing with these abnormalities is then presented. Finally, clinical data and diagnosis and therapeutic options are discussed.

[Transient left ventricular apical ballooning syndrome. Case report and review of the literature]. / Syndrome de ballonisation apicale transitoire du ventricule gauche. Revue de la littérature à propos d'un cas.

SUBJECT: Transient left ventricular apical ballooning syndrome, known as the japanese name of tako-tsubo was recently described and is characterised at the acute period by a large apical dyskinesia of the left ventricule apex. This syndrome usually mimics an acute coronary syndrome, sometime a cardiac failure or arrhythmias. We report a typical case of a seventy-seven years old women struck by a tako-tsubo syndrome revealed by an acute chest pain after an emotional stress. The diagnosis was proposed because angiography didn't shown any abnormality in spite of a large left ventricular dysfunction at the ventriculography. Actuality and strong points: Tako-tsubo syndrome is more and more published due to a better knowledge of this syndrome, specially in the acute coronary syndrome without coronary abnormality. His physiopathology is not well known but is clearly in relation with an acute stress. Several hypothesis are discussed, helped by some experimental animal model. With an excellent prognosis at a middle-course, his discovery allow an adapted take care especially of the complications. PROSPECT: The setting up of clinic and electrocardiographic reliable and well-tried criteria will allowed an early diagnosis to avoid harmful treatment. His specific treatment is not reached by consensus but will develop by a better knowledge of the physiopathology.

[Severity of aortic ring abscess complicated by cardiac conduction abnormalities]. / Sévérité des endocardites abcédées du manchon aortique compliquées d'anomalies de la conduction.

OBJECTIVE: To determine clinical features, management and prognosis of cardiac conduction abnormalities (CCA) complicating abscessed endocarditis. METHODS: We have analysed clinical, microbiologic and echocardiographic datas, therapies and outcome of cardiac abscesses complicated by CCA in patient hospitalized between 1995 and 2001 in our centre. RESULTS: Above 35 cardiac abscesses, six men (mean age 62 years) had CCA complicating six aortic ring abscesses (4 on native valve and 2 on prosthetic valve) with four cases of interventricular septal involvement and fistulization. Severe heart failure is present four times, a septic cerebral embolization twice. Streptococcus and Staphylococcus prevail. Complete atrioventricular block (AVB) reveals endocarditis twice and complicates the evolution three times. Trifascicular block (first degree AVB, left anterior fascicular block and complete right bundle branch block) revealed recurrence of endocarditis. Two patients were treated medically: one died quickly (complete AVB pre-mortem), and the other one had favourable issue (paroxystic complete AVB). Four patients had surgery with temporary pacemaker in three cases (one died) then definitive pacemaker in two cases. At 26.5 month (7-50), the four survivors had no recurrence of endocarditis. CONCLUSION: Severe CCA are classical in aortic ring abscessed endocarditis and associated with increased mortality. Immediate transfert in a dentre with cardiac surgery is necessary. Definitive cardiac pacing can be performed early without leads infection.

[Outcome of 30 congenital atrio-ventricular blocks]. / Devenir à long terme de 30 blocs auriculo-ventriculaires complets congénitaux isolés.

Congenital isolated atrio-ventricular block (CAVB) is a rare pathology, and its management is still rather poorly described through international literature. Within the service of pediatric cardiology leaded by Pr Choussat and Dr Jimenez (Cardiologic Hospital Haut-Lévêque of Bordeaux), we collected from 1980 to 2003, 30 isolated congenital CAVB, constituting the purpose of this retrospective study. Average follow-up is 14 +/- 8.8 years. None death occurred. CAVB are discovered at an average age of 4.8 years old; 6 cases were diagnosed in utero, half of them were associated with maternal lupus. Twenty patients on 30 were fitted with stimulator at an average age of 8.7 +/- 6.9 years old, due to symptoms or bradycardy. Epicardic fitting in VVI mode represents 65% of first approaches, it is followed by endocavitary way for 81% of cases. Cardiac stimulation does not prevent from dilated cardiomyopathy. Among 30 patients 10 were not fitted with stimulator, half of them presents chronotrop insufficiency during effort. As a conclusion, our patients show a good long-term vital prognosis; although CAVB discovered in utero lead to worse prognosis for children.

[Tachycardia-induced cardiomyopathy, unusual and reversible cause of left ventricular dysfunction: report of 9 cases]. / Tachycardiomyopathie, une cause rare et réversible d'altération de la fonction ventriculaire gauche: à propos de neuf cas.

In this study, 9 cases of tachycardia-induced cardiomyopathy have been reported, with a 7-year follow-up period. The patient population consisted of 5 males and 4 females with a mean age of 41 +/- 18 years (range: 10-70 years). It was difficult to determine the onset of the arrhythmia, due to its frequently asymptomatic course until such time as cardiac insufficiency became apparent (this was the case in 7 patients, while 2 subjects had palpitations). Four cases of AV intranodal reentry tachycardia (3 with an accessory pathway), 2 cases of atrial fibrillation, 1 case of auricular flutter, 1 case of atrial tachycardia and 1 case of ventricular tachycardia were observed. Disappearance of the symptomatology following restoration of sinus rhythm was accompanied by echocardiographically-determined normalization of systolic function on average 5 +/- 4 months post-arrhythmia (range: 1-12 months). Left-ventricular end-diastolic volumes decreased from 60 +/- 5 mm to 51 +/- 4 mm (P < 0.01), and end-systolic volumes from 50 +/- 7 mm to 35 +/- 4 mm (P < 0.001), while the left ventricular shortening fraction increased from 17 +/- 5% to 33 +/- 4% (P < 0.0001). In the differential diagnosis for primary dilated cardiomyopathy, tachycardia-induced cardiomyopathy appears very similar to the former, and only an adequate period of follow-up after the restoration of sinus rhythm can confirm the diagnosis.

[Cardiac myxoma in patients over 75 years of age. Report of 19 cases]. / Myxomes cardiaques chez le sujet âgé de plus de 75 ans. A propos de 19 observations.

Left atrial myxoma is considered to be exceptionally rare in the elderly. The authors observed and reported on 19 cases occurring in patients over 75 years of age out of a series of 100 myxomas diagnosed between 1962 and 1997, in 12 women and 7 men (mean age: 80 years, range 75 to 89 years). In 3 cases, the myxomas were chance findings at echocardiography but the 16 symptomatic patients (85%) had left ventricular failure (47%), positional symptoms (25%), pyrexia and poor general health (17%) or systemic embolism (17%). The location of the myxoma was the left atrium in all cases, with mitral valve obstruction in 13 of the 19 cases. Eighteen tumours were attached to the interatrial septum and one to the atrial surface of the anterior mitral leaflet. Calcifications were observed in 5 patients. Surgical ablation of the tumour was performed in 15 of the 19 patients. The post-operative course was usually uncomplicated: one patient died of a cerebral haemorrhage. Four patients did not undergo surgery because of patient refusal in 3 cases and major associated morbidity in the other case. These cases were included in the study because the tumours had all the characteristics of myxomas. Though the discovery of a myxoma remains a classical surgical emergency, the presence of quiescent, non-obstructive, well circumscribed and calcified myxomas with a low risk of obstruction and of embolism in elderly high risk patients may be exceptions to this traditional dogma.

[Congenital left ventricular aneurysms and diverticula. Two case reports]. / Anévrysmes et diverticules congénitaux du ventricule gauche. A propos de deux observations.

The authors report the cases of two young adults (25 and 27 years) presenting with congenital left ventricular aneurysm or diverticulum with healthy coronary arteries. This saccular evagination of the ventricular wall is rare. The authors describe a classification distinguishing muscular (contractile) diverticula, composed of the three cardiac tunics, fibrous diverticula and finally aneurysms with a dyskinetic wall. Although the limits of this classification are sometimes poorly defined, it presents a prognostic value, because it appears logical to propose nonsurgical management in the context of muscular diverticula and it seems legitimate to operate on fibrous diverticula and aneurysms due to the risks of rupture, extension, thrombosis or arrhythmia.

[Human malaria: dysglobulinemia-hypocholesterolemia syndrome]. / Paludisme. Le syndrome dysglobulinémie--hypocholestérolémie.

Comparative study of protein and lipid parameters within a homogenous sample of 144 non-immune subjects having stayed for four months in Central Africa, suggests a dysglobulinemia more significant in the infected group. This dysglobulinemia affects in particular the IgM which are negatively correlated with cholesterol, positively with apoproteins A2 and H.D.L. Such a peculiar profiling involving immunoglobulins, apoproteins and cholesterol is rapidly regressive after stopping exposure to parasitic risk. It appears as an adaptation syndrome--or resistance--to tropical environment.