[A cardiac rhabdomyome evoking the antenatal diagnosis of a Bourneville's tuberous sclerosis]. / Un rhabdomyome cardiaque évoquant le diagnostic anténatal d'une sclérose tubéreuse de Bourneville.

Rare hereditary affection, the Bourneville's tuberous sclerosis (BTS) is an autosomal dominant inherited phakomatosis. Rhabdomyomes are the most frequent cardiac tumors in children and infants, they are one of the most premature modes of revelation of the STB. They sometimes allow to envisage the diagnosis in antenatal period at the same time as the genetic and neurological explorations. We report the diagnosis of a fetal BTS evoked by the antenatal discovery of a cardiac rhabdomyome. The antenatal cerebral explorations, realized by magnetic resonance imagery (MRI), put evidence cerebral localisations confirming the diagnosis.

Uncommon cause of acute pelvic pain: isolated torsion of hydrosalpinx.

Isolated torsion of hydrosalpinx is a rare cause of acute pelvic pain. Pre-operative diagnosis is very difficult because of non specific clinical presentation. Definitive diagnosis is always made at surgical exploration performed for suspected adnexal torsion and salpingectomy is performed in the majority of cases. A 34-year-old woman was admitted for acute pelvic pain with nausea and vomiting. Vaginal examination revealed a right adnexal tender mass and ultrasound revealed a well circumscribed right adnexal cystic mass. Surgical exploration has revealed torsion of a right hydrosalpinx and right salpingectomy was performed. Differential diagnosis between adnexal and tubal torsion is very difficult, however both should be managed by rapid surgical exploration which an allow precocious diagnosis and conservative treatment.

Percutaneous mitral valvuloplasty in patients with low cardiac output at high surgical risk.

Among 1146 patients undergoing percutaneous mitral valvuloplasty for symptomatic mitral stenosis, 8 (4 men and women) were at high risk for surgery on the basis of the New York Heart Association functional class IV (n=8), severe pulmonary hypertension (n=5). All these patients aged 30+/-23.6 years had signs of right heart failure, high echocardiographic score (9.6+/-3.6) and low mitral valve area (0.50+/-0.19 cm(2)). The procedure resulted in an increase in mitral valve area (1.55+/-0.17 cm(2)) with a concomitant reduction in pulmonary artery systolic pressure (58.7+/-9.9 mm Hg) and decrease in tricuspid regurgitation. At follow-up (mean 14+/-3 months), one patient with renal failure... presented with a mitral restenosis is scheduled for mitral valve replacement, two patients with severe tricuspid regurgitation required tricuspid annuloplasty. In conclusion, percutaneous mitral valvuloplasty is feasible and safe in patients at high surgical risk and can be considered as an acceptable alternative to surgery.

[Percutaneous mitral commissurotomy and Lutembacher syndrome]. / Commissurotomie mitrale percutanée et syndrome de Lutembacher.

INTRODUCTION: Lutembacher syndrome refers to the rare combination of congenital atrial septal defect and acquired mitral stenosis. This condition is usually treated surgically by mitral valve operation with concomitant closure of the atrial septal defect. MATERIALS AND METHODS: Between 1993 and 2003, 4 patients with congenital Lutembacher syndrome had percutaneous mitral commissurotomy without closure of the atrial septal defect at our institution. The 4 patients were very symptomatic with right-sided heart failure signs and NYHA functional class III-IV. RESULTS: The procedure was carried out successfully for the four patients. Mitral valve area increased from 0.87 to 1.97 cm2 at mean; left atrial pressure decreased from 28.2 to 12.7 mmHg and the mean valve mitral gradient was reduced from 15.5 to 3.9 mmHg. Functional and clinical improvement was observed in all the cases. During a mean follow up of 55 +/- 29 months, our 4 patients remain pauci symptomatic under medical treatment. CONCLUSION: The percutaneous treatment of the Lutembacher syndrome is currently a possible alternative to the surgery among patients having an anatomy favourable to the procedure.

[Percutaneous mitral valvuloplasty in the pregnant woman Moroccan experience]. / Valvuloplastie mitrale percutanée chez la femme enceinte: expérience marocaine.

Pregnancy is one of the most important factors known to destabilize valvular heart disease. In particular, pregnancy is a major cause of aggravation in the clinical course of women with mitral stenosis (MS). Surgical treatment of MS during pregnancy may be hazardous for both mother and foetus. In this context, percutaneous mitral valvuloplasty using the Inoue balloon may constitute a particularly attractive alternative to surgery. We report the results of percutaneous mitral valvuloplasty achieved in a series of 11 pregnant women.

Difficult percutaneous mitral commissurotomy: failure of the Inoue balloon technique and successful double-balloon technique.

From December 1994 to May 1999, 487 patients underwent percutaneous transvenous mitral commissurotomy according to the Inoue balloon technique for tight mitral stenosis. In three cases, the Inoue balloon technique was not possible because the catheter balloon could not enter the mitral orifice and the double-balloon technique was performed instead. In two cases, a flow-guided balloon catheter met the mitral orifice and the double-balloon technique was effective. In the third case, passing the valve with a flow balloon catheter was impossible, a 0.032" Terumo straight wire crossed the valve, which was dilated with a single balloon the first time and a double balloon the second time. The mitral valve area increased in the three cases from 0.8 to 1.8 cm2, from 0.7 to 1.6 cm2, and from 0.5 to 1.5 cm2, respectively. There were no complications.

[Mixed form of total anomalous pulmonary venous connection: apropos of a case]. / Forme mixte du retour veineux pulmonaire anormal total: a propos d'un cas.

As opposed to partial anomalous of pulmonary venous connection, it is frequent and benigns, the total anomalous of pulmonary venous connection is extremely rare and more serious. The anomalous is severe because all pulmonary venous connection, instead of left heart it go to the right heart. The age of diagnosis is closely tied up anatomics characteristics, so various clinical cases are present. The TAPVC of new born is a surgical emergency, especially where it's block up and release++ cardiorespiratory distress syndrome secondary to OAP. For great children, the total anomlous of pulmonary venous connection can be assumed to a case of atrial septal defect. This study intend to clear up this clinical and anatomical polymorphism and to report an exceptional-form of this congenital anomaly which is mixed total anomalous of pulmonary venous connection.

[Percutaneous transluminal valvuloplasty in congenital pulmonary stenosis in adults. Apropos of 34 cases]. / Valvuloplastie transluminale percutanée des sténoses pulmonaires congénitales chez l'adulte. A propos de 34 cas.

Percutaneous pulmonary valvulotomy is the treatment of choice for isolated congenital pulmonary valvular stenosis in childhood. However, experience of this procedure in the adult is much more limited. Between January 1984 and December 1994, 34 patients with severe or moderate pulmonary valvular stenosis underwent percutaneous transluminal valvuloplasty. The age of the patients ranged from 20 to 47 years (mean 22 +/- 4 years). Cardiac catheterisation was performed using the femoral vein in 27 cases and the internal jugular vein in 7 cases. Success was obtained in 28 patients (81% of cases). Pulmonary artery-right ventricular pressure gradient decreased from 113 +/- 35 to 32 +/- 13 mmHg (p < 0.001) after valvuloplasty with one or two balloon catheters. The tolerance of transluminal valvuloplasty was generally good. The poor results were explained by cases of dysplasic valves or of infundibular reactions. There was one death which occurred 24 hours after the procedure. Clinical and echocardiographic follow-up was obtained in 20 patients, 3 to 36 months after valvuloplasty (average: 23 +/- 13 months). No cases of restenosis were observed. Percutaneous transluminal pulmonary valvuloplasty in the adult is feasible and gives good results which are maintained at medium-term; it has become the treatment of choice of valvular pulmonary stenosis and gives good results which are maintained at medium-term, thereby avoiding surgical valvulotomy.

[Association of coronary-pulmonary fistula and intercoronary fistula. Apropos of a case]. / Association fistule coronaro-pulmonaire et fistule intercoronaire. A propos d'un cas.

The authors present the case of a 30-year-old woman admitted to hospital for investigation of a systolodiastolic murmur. Assessment revealed a fistula between the trunk of the left coronary artery and the pulmonary artery, with an aneurysm on the left anterior descending artery (LAD), associated with an intercoronary anastomosis between the left conus artery and the LAD. Thallium myocardial scintigraphy demonstrated reversible decreased uptake in the anterolateroapical region. Simple coronaro-pulmonary fistulas are a rare disease. The positive diagnosis is suggested by echocardiography and confirmed by coronary angiography. The clinical course is generally favourable, except in the presence of complications such as aneurysm, heart failure or coronary insufficiency. This case raises the difficult problem of the operative indication in these young, usually asymptomatic patients.

[Mitral valve prolapse and myocardial ischemia. Apropos of 2 cases]. / Prolapsus valvulaire mitral et ischémie myocardique. A propos de deux cas.

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.