Cardiac events in Costello syndrome: one case and a review of the literature

Costello syndrome is a rare syndrome associated with de novo mutations in the HRAS gene. It is mostly revealed during in the first months of life by growth retardation, facial dysmorphic features, skin and cardiac abnormalities and subsequent cognitive deficit of varying severity. We report a case of Costello syndrome in a 3-month-old infant. The initial cardiac investigations were normal except frequent premature atrial complexes. After few months, worsening arrhythmia with bursts of ventricular tachycardia were noted as well as the secondary progressive obstructive left ventricular hypertrophic cardiomyopathy [HCM]. Cardiac involvement is determinant for the prognosis of Costello syndrome. It frequently consists of hypertrophic cardiomyopathy [one third of patients], with involvement of the left ventricle in half of the cases. It is often asymmetrical and associated with obstruction of the outflow recalling family hypertrophic cardiomyopathy. The natural history of HCM in Costello syndrome and its management remains poorly known because of paucity of reported cases. Progression of the HCM can be very rapid like the reported case. On the other hand, the spontaneous regression of the HCM in some patients has been reported. In addition, cardiac threatening arrhythmias may be noted. So that, cardiac assessment and monitoring with regular echocardiography and electrocardiogram follow up is mandatory

[Contrast-induced nephropathy after cardiac catheterization: a prospective study of 180 patients]

Contrast-induced nephropathy [CIN] is associated with an increased cardiovascular morbi-mortality. Little is known about the incidence and risk factors of CIN after cardiac catheterization in Tunisian patients. To determine the incidence of CIN and its predictors after coronary angiography as well as its prognostic and therapeutic repercussions in a Tunisian patients' cohort. In this prospective single center study, 180 consecutive patients who underwent cardiac catheterization were enrolled; all patients were followed-up for 3 months. The incidence of CIN defined as an absolute increase in serum creatinine >/= 5 mg/l [44micromol/l] and/or a relative increase in serum creatinine >/= 25%, was 17.2%. In multivariate logistic regression, independent predictors of CIN were: diabetes mellitus [Odds Ratio [OR]=2.26 ; 95% confidence interval [95%CI]: 1.29-3.98, p=0.005], creatinine clearance < 80ml/mn [OR=2.87 ; 95%CI: 1.59-5.19, p<0.001], left ventricular ejection fraction [LVEF] < 45% [OR=2.03 ; 95%CI: 1.22-3.39, p=0.007] and use of a volume of contrast media > 90ml [1.72 ; 95%CI: 0.99-2.99, p=0.05]. Perprocedural hypotension was the strongest independent predictor of CIN in our study [OR=3.99; 95% CI: 1.65-9.66, p=0.002]. CIN was totally regressive within one month in 27 patients [86.7%] while 3 patients [10%] had a residual renal dysfunction at the end of the follow-up period [3 months]. More than one angiocoronarography on 6 resulted in CIN in our population. CIN affects cardiovascular prognosis even if renal function normalization is usually obtained within one month after the investigation. Besides identifying risk factors of CIN in order to apply preventive measures in risky patients, we stress the necessity of insuring a good hemodynamic status while achieving the procedure

[Large ostium secundum atrial septal defect discovered after severe mitral regurgitation: a case report]

Mitral regurgitation is associated with ostium secundum atrial septal defect in about 22% of cases. mitral valve prolapse induced by atrial shunt is the main cause of this regurgitation. Ususually, atrial septal defect discovery precedes that of mitral regurgitation. The aim of this paper is to focus on clinical, hemodynamic and evolutive details of atrial septal defect and mitral regurgitation association. We report the case of large atrial septal defect in 37 years old girl referred for hemodynamic investigation of mitral regurgitation. The divergence of clinical data, electrocardiogram and echocardiography findings has led to atrial septal defect discovery. Hemodynamic data showed severe pulmonary arterial hypertension [medium pulmonary arterial pressure: 45 mmhg]. Hence, mitral valve substitution by mechanical prosthesis and closure of atrial septal defect have been carried out. Ten hours after surgery, death occurred because of severe pulmonary arterial hypertension and heart failure. Association of severe mitral regurgitation and large ostium secundum atrial septal defect is an original anatomo-clinic entity characterized by mitral valve lesions diversity and severe secondary pulmonary arterial hypertension. Danger of such a hypertension is due to progressive and infra clinical rise of pulmonary resistances and association of increased pulmonary blood flow and capillary pulmonary hypertension

Evoluation after 5 years ago of percutaneous pulmonary valvulotomy report

47 patients aged from 2 to 59 years affected by valvular congenital or mixed pulmonary stenosis including three fallot trilogies and one patient with right congestive cardiac failure are treated by percutaneous pulmonary valvulotomy between October 1986 and December 1990. All patients have been controlled with a mean follow-up of 6.5 +/- 1.1 years. The total gradient rate between pulmonary artery and right ventricule decrease from 112 +/- 55 mm Hg to 20 +/- 8 mm Hg on the last control with disappearance of infundibular inflammation and their auricular shunt in all the concerned cases. We observe the degression of right cardiac failure symptoms with disappearance of tricuspid insufficiency in the cases of advanced pulmonary stenosis with right ventricular dysfunction. Percutaneous pulmonary valvulotomie by its simplicity and inocuity, its long term efficiency is a good method of treatment of pure valvular or mixed pulmonary stenosis

Percutaneous mitral commissurotomy report of a new simplified method

We report a new simplified method of mitral dilatation done on 146 patients. The technique use the lnoue balloon with progressive diameters under ultrasonographic control, avoiding the arterial part of the dilatation. The population include 9 pregnant women, 13 children, 13 with restenosis and closed heart and 12 restenosis after dilatation. Mean age is 32 +/- 16 years [9 to 72] and sex ratio 3.7 [114 F/32 M]. Three groups are individualized according to histopathologic exam of metal struclure, group [26 percent] concern good indication and III [32 percent] bad indication for this technique, group II [42 percent] contain intermediate indications. Commissurolomy increase the mean mitral square for 0.2 to 1.9 +/- cm'. We observe 3 cases of chirurgical mitral insufficiency needing remplacement recuited for 2 patients from the group III. Mean fluoroscopic exposition time is 5.2 +/- 3.3 mn and procedure duration 18 +/- 11 mn. Hospitalisation duration is one day for 95 percent of the patients. This method remaining simple, efficient and safe permit a gain in time of 70 percent with a good confort for patient and a return to work on the 4th day