Multiple cardiac rhabdomyomas, wolff-Parkinson-white syndrome, and tuberous sclerosis: an infrequent combination.

Cardiac rhabdomyomas are benign cardiac tumours and are often associated with tuberous sclerosis. They are often asymptomatic with spontaneus regresion but can cause heart failure, arrhythmias, and obstruction. There have also been a few isolated reports of Wolff-Parkinson-White syndrome occurring in association with tuberous sclerosis and the great majority has been detected in patients with concomitant rhabdomyomas. We report a 12-day-old infant girl with tuberous sclerosis who presented with intraparietal and intracavitary rhabdomyomas with a Wolff-Parkinson-White syndrome (WPW). She represents one of the few published cases of WPW syndrome and tuberous sclerosis and particularly interesting because of intramural rhabdomyomas regression with persistent intracavitary rhabdomyomas after two years of followup.

Sustained benefit of left ventricular remodelling after valve replacement for aortic stenosis.

BACKGROUND: Valve replacement for aortic stenosis (AS) determines negative ventricular remodelling. We used cross sectional and Doppler echocardiography to check how rapidly it occurs and to assess if these changes are sustained over time. METHODS: We evaluated in 34 patients subjected to aortic valve replacement for AS morphological and functional (ejection fraction and E:A ratio) left ventricular data by echocardiography prior to surgery and 2 postoperative studies: early after surgery (pQ1) and at mid-term evolution (pQ2). RESULTS: Left ventricular mass index was reduced at pQ1 (from 152 +/- 47 g/m2 to 113 +/- 31 g/m2; p < 0.01) as well as end-diastolic (from 51.3 mm to 48.3 mm; p < 0.03), end-systolic (from 32.2 mm to 29.4 mm; p < 0.02), interventricular septum (from 12.9 mm to 10.3 mm; p < 0.01), and posterior wall (from 12.5 mm to 11 mm; p < 0.01) dimensions. Left ventricular ejection fraction (from 61.2% to 65.2%; p < 0.04) and E:A ratio (from 0.94 to 0.98; p < 0.01) increased significantly at pQ1. There were no significant differences in measurements between pQ1 and pQ2. CONCLUSIONS: Aortic valve replacement surgery leads to a rapid negative left ventricular remodelling during the first 7 months, including a decrease in myocardial hypertrophy and an improvement in systolic and diastolic function. These beneficial hemodynamic changes are sustained for at least 3 years.

Adaptation of left ventricular morphology to long-term training in sprint- and endurance-trained elite runners.

Long-term studies on left ventricular (LV) adaptation have not been reported. The echocardiograms of 41 top-class runners (8 males and 6 females sprint-trained, 15 males and 12 females endurance-trained) were recorded at the beginning and after 1, 2, and 3 years of training. A one-way ANOVA and a linear regression analysis were conducted to determine changes and association between performance and LV values. Training resulted in an increase in performance and LV internal diameter at end-diastole (LVIDd) and decreases in end-diastolic interventricular septal wall thickness, and posterior wall thickness (PWTd). There were no significant differences in LV mass and LV ejection fraction (LVEF, %). The changes in PWTd were linked to enlargement of the LV. In athletes with unusual LV dilatation (>60 mm), LVIDd was related to performance and LVEF was >50%. Maximal wall thickness was <13 mm in all athletes. LV adaptations were independent of sex and type of training and related to the initial level of performance. We believe that LV enlargement in elite runners is a physiological adaptation and that the LVIDd is a predictor of running performance.

Desinserción protésica mitral recidivante / Recurrent desinsertions of a prosthetic mitral value

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Echocardiography to measure fitness of elite runners.

We evaluated the echocardiograms of 134 male and 54 female elite runners who competed over different distances to observe cardiac enlargement and differences among athletes engaged in different types of training, and to relate the echographic dimension with running performance. In male runners, left ventricular (LV) internal diameter at end diastole (LVIDd), LV mass, and left atrial diameter were increased by the length of distance ( P < .001) whereas right ventricular diameter was increased only in marathon runners. In female runners, this association was found when LVIDd, LV mass, and left atrial diameter were adjusted for body surface area ( P < .001). Wall thickness was not related to distance running. Abnormal LV dilation was associated with a low LV ejection fraction for the higher values of LVIDd, but not for the higher indexed LVIDd values. In male runners, LVIDd was associated with 100 m ( r = -0.67, P < .01), 10,000 m ( r = -0.70, P < .001), and marathon ( r = -0.54, P < .001) and in female runners with a 400-m ( r = -0.84, P < .001) race time. These results show that sprint- and endurance-trained runners had an eccentric hypertrophy and that it is necessary to establish a new upper limit of abnormal cavity dilatation as indexed LVIDd. The normal systolic function and the relationship between LVIDd and performance indicate that LV enlargement in elite runners is a physiologic adaptation and that echocardiographic assessment can be used to determine the fitness of runners.