Importance of dural ectasia in phenotypic assessment of Marfan's syndrome.

BACKGROUND: Early identification of Marfan's syndrome is fundamental in the prevention of aortic dilatation, but the wide phenotypic expression of the disorder makes the clinical diagnosis very difficult. Dural ectasia has been classified as a major diagnostic criterion; however, its prevalence is not known. We aimed to identify the true prevalence of dural ectasia in Marfan's syndrome, and to investigate its relation to aortic pathology. METHODS: A magnetic-resonance-imaging (MRI) study of the thoracic aorta and of the lumbosacral spine was done in an inclusive series of 83 patients with Marfan's syndrome to assess the presence and degree of dural ectasia and aortic involvement; 12 patients were younger than 18 years. 100 individuals who underwent MRI of the lumbar spine for routine clinical indications represented the control group; none of them had any potential causes for dural ectasia. FINDINGS: Dural ectasia was identified in 76 (92%) patients and none of the control group. The severity of dural ectasia was related to age; the mean (SD) age of patients with mild dural ectasia was 26 years (14) whereas that of those with severe disease (meningocele) was 36 years (9) (p=0.038). 11 of 12 patients younger than 18 years had dural ectasia. No association was found between aortic dilatation and dural ectasia. INTERPRETATION: Dural ectasia is a highly characteristic sign of Marfan's syndrome, even at an early age.

Prolonged heparin after uncomplicated coronary interventions: a prospective, randomized trial.

BACKGROUND: Continuous heparin infusion after percutaneous transluminal coronary angioplasty (PTCA) procedures prolongs the hospital stay and could increase the occurrence of bleeding complications. The aim of this randomized trial was to evaluate whether omission of heparin infusion after uncomplicated coronary interventions in patients with stable and unstable angina with or without stent implantation increased the incidence of acute cardiac complications. METHODS AND RESULTS: A total of 191 consecutive patients who underwent successful PTCA were randomly assigned to receive either prolonged heparin (heparin group) or no postprocedure heparin (control group). The 2 treatment groups were comparable with respect to clinical and angiographic characteristics. Stents were used in 36% of the control group and in 33% of the heparin group. Cardiac complications occurred in 8 (4%) patients. Four (4%) patients in the control group and 3 (3%) patients in the heparin group had a myocardial infarction. One patient in the control group died 3 days after the intervention. No patient in either group needed a repeat revascularization during the target hospitalization. Peripheral vascular complications in the control and heparin groups occurred in 1% and 3% of the patients, respectively. CONCLUSIONS: Omission of heparin after successful PTCA with or without stent implantation in patients with stable and unstable angina did not significantly increase the incidence of acute cardiac complications. It allows for early sheath removal and patient discharge and saves costs. This study, combined with other small studies in the field, provides strong evidence that heparin should not be used routinely.

[Use and significance of intravascular ultrasonic imaging]. / Anwendung und Bedeutung der intravaskulären Ultraschallbildgebung.

Intravascular ultrasound (IVUS) imaging of coronary arteries has recently become possible in vivo with the improvements achieved in the miniaturisation of ultrasound transducers. IVUS provides informations complementary to angiography, and is considered as the gold standard for the assessment of lumen size, plaque thickness and plaque distribution. As formerly demonstrated by pathological studies, IVUS also reveals an underestimation of plaque thickness with angiography and an incomplete assessment of the true morphologic distribution of the plaque burden. Among its clinical applications, IVUS can be used to monitor revascularisation procedures, and may provide very accurate measurements of progression or regression of coronary atherosclerosis and of the extent of posttransplant vasculopathy. IVUS was employed successfully to assess and optimize the results of percutaneous transluminal coronary angioplasty. Clinical studies have shown that the risk of restenosis is inversely proportional to the size of the postprocedural lumen. With the guidance of percutaneous transluminal angioplasty by IVUS, balloon size and inflation pressures were increased, and better results were obtained with larger lumen size in comparison to the results of the procedures assessed by angiography alone. These improvements contributed to abandon the anticoagulation after stent implantation in most cases. In spite of the valuable information provided by IVUS, its role in clinical settings should still be defined IVUS prolongs the revascularisation procedure and enhances its costs and risks. Thus, the cost effectiveness of IVUS in its clinical application should be determined by prospective studies.

[Inadequate treatment compliance, patient information and drug prescription as causes for emergency hospitalization of patients with chronic heart failure]. / Ungenügende Behandlungsdisziplin, Patienteninformation und Medikamentenverschreibung als Ursachen für die Notfallhospitalisation bei chronisch herzinsuffizienten Patienten.

UNLABELLED: Causes of decompensation of treated chronic congestive heart failure in patients referred for emergency hospitalization were examined prospectively. 111 consecutive patients (76 +/- 11 years) were interviewed and their records examined on admission. The diagnosed underlying diseases were coronary artery disease (80%), hypertensive heart disease (40%), valvular heart disease (11%), and idiopathic dilated (7%) and alcoholic (5%) cardiomyopathy. The grounds for decompensation of chronic congestive heart failure were: insufficient compliance 47% (n = 52, irregular or not intake of medication [25%], salt [9%] or fluid [7%] excess, stopping medication because of side effects [6%]), uncontrolled hypertension (27%), insufficient diuretic therapy in spite of progressive symptoms (23%), treatment with negative inotropic drugs (21%), acute rhythm disturbances (14%), acute myocardial infarction or unstable angina pectoris (14%), infections (6%). 80% of the patients were treated with diuretics, 34% with digoxin, 31% with ACE-inhibitors. Insufficient basic knowledge about the disease (regular weighing, diet, behavior if symptoms worsen) was found in 78% of patients, complete lack of knowledge concerning the prescribed drugs in 29%. Only 44% were regularly followed by their physicians, 53% had either no regular follow-ups or they were set at too long intervals. CONCLUSIONS: In the majority of patients, one or more avoidable causes leading to decompensation of chronic congestive heart failure can be identified. The main potential for intervention aiming at a reduction of the hospitalization frequency lies in improving patient compliance and state of the art medication by the primary care physician. Equally unsatisfactory is the low frequency of follow-up checks to reassess and renew drug therapy.