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A Picelli

Publications and source records attributed to A Picelli.

9 recordsLinked to original sources
PubMed1993

Left ventricular myocardial function in myotonic dystrophy.

Myotonic dystrophy is an autosomal dominant disease affecting many organ systems, including the heart. Abnormalities of the cardiac conduction system are a frequent and well-documented finding in this neuromuscular disease, whereas overt signs of heart failure are rarely reported. However, controversy exists about the prevalence of preclinical left ventricular (LV) dysfunction in patients with myotonic dystrophy who have no symptoms of heart failure. To address this issue, load-independent LV function indexes were compared in patients with myotonic dystrophy and in normal subjects. LV measurements were obtained with M-mode echocardiography in 43 consecutive patients with myotonic dystrophy (mean age 35 +/- 14 years) who had no clinical evidence of heart failure, and in 35 sex- and age-matched controls. A cuff sphygmomanometer was used to determine blood pressure. No difference was found between patients and control subjects in the percentage of LV ejection fraction predicted for end-systolic stress (103 +/- 7 vs 103 +/- 7%; p = 0.97) or in the end-systolic stress/volume index ratio (2.9 +/- 0.5 vs 2.7 +/- 0.4 dyne7/cm3; p = 0.09). Similarly, no significant difference in LV myocardial function was detected between the 19 patients with a mild degree of the neuromuscular disease and in the 24 with a moderate or severe degree of the disease. Finally, LV myocardial function was compared between patients with myotonic dystrophy and atrioventricular or intraventricular conduction disturbances (n = 8) and those without these disturbances (n = 35), but no significant difference in the percentage of LV ejection fraction predicted for end-systolic stress or in the end-systolic stress/volume index ratio could be detected.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

The natural course of cardiac conduction disturbances in myotonic dystrophy.

In this study we noninvasively followed for a mean period of 46 months 21 patients with different grades of myotonic dystrophy to evaluate the course of the involvement of the cardiac conducting system. Six patients (28.6%), 1 affected by a mild and 5 by a severe form of the neuromuscular disorder, showed appearance or deterioration of conduction disturbances suggesting a serious derangement of the specialized tissue; in 3 of them a permanent demand pacemaker was implanted. Conduction defects are the most frequent cardiac manifestations in myotonic dystrophy and become worse with time, mainly in patients with higher degrees of the disease. Furthermore, first-degree atrioventricular block may represent an early sign of developing of more advanced conduction defects.

Adolescent↗
PubMed1990

Electrocardiographic diagnosis of left ventricular hypertrophy in the presence of left bundle branch block: a wasted effort.

We assessed the reliability of multiple electrocardiographic variables for detecting left ventricular hypertrophy in 100 patients (aged 23 to 92 years, mean age 39 +/- 14) with complete left bundle branch block and different underlying cardiac diseases. Left ventricular hypertrophy, defined as an echocardiographically evaluated left ventricular mass greater than 241 g, was present in 66 of the 100 patients. The electrocardiographic parameters with the highest sensitivity were both the Cornell voltage criteria (RaVL + SV3 greater than 28 mm in men and greater than 20 mm in women) and the combination of criteria proposed by Kafka (any of these four indexes: RaVL greater than or equal to 11 mm, QRS axis -40 degrees or less, SV1 + RV5 or RV6 greater than or equal to 40 mm, SV2 greater than or equal to 30 mm and SV3 greater than or equal to 25 mm), with a sensitivity of 77%. Both criteria had a very low specificity (32 and 35%, respectively). The high specificities (greater than or equal to 88%) of several electrocardiographic criteria were accompanied by ineffective low sensitivities (less than 35%). Moreover, the cumulative parameters of Kafka and Cornell voltage criteria achieved a sensitivity of 84 and 89%, respectively, in hypertensive patients and in those with valvar diseases. None of the electrocardiographic indexes tested showed a significant difference in sensitivity when applied in categories of patients with left ventricular hypertrophy and different left ventricular geometry (cavity dilation or concentric hypertrophy). These data indicate that both conventional and recently proposed electrocardiographic criteria for left ventricular hypertrophy in the presence of left bundle branch block poorly recognize an augmented left ventricular mass.

Adult↗
PubMed1989

Autoantibodies in myotonic dystrophy.

We evaluated some immunological parameters in a group of 24 patients affected with myotonic dystrophy (MD). IgG, IgA, IgM immunoglobulin serum levels resulted decreased in most of the patients. Anti-smooth-muscle antibodies have been found in 10 out of 24 patients (45.5%). Moreover in some of them decreased C3 and C4 complement fraction serum levels have been found. Our data confirm the existence of some immunological abnormalities in a great number of subjects affected with the disease. Besides, these data evidence for most of the subjects the presence of autoantibodies non-organ-specific direct against myocellular antigens. These autoantibodies could be considered the expression of suffering of muscular fibres.

Adolescent↗
PubMed1988

Antimitochondrial autoantibodies in myocardial hypertrophy: comparison between hypertrophic cardiomyopathy, hypertensive heart disease, and athlete's heart.

Antimitochondrial autoantibodies (AMA) were tested by indirect immunofluorescence in three groups of subjects with different types of myocardial hypertrophy: 35 patients affected with hypertrophic cardiomyopathy (HC), 20 patients with cardiac hypertrophy secondary to essential hypertension, and 35 active endurance athletes with exercise-induced left ventricular hypertrophy. Forty-two healthy subjects served as a control group. Left ventricular hypertrophy was considered a left ventricular mass (LVM) echocardiographically calculated (Devereux formula), exceeding 244 gm or a LVM index exceeding 122 gm/m2 (greater than 2 SD from a previously studied normal population). AMA were found in 15 of 35 (43%) patients with HC and in 6 of 20 (30%) patients with hypertensive heart disease (p less than 0.01); in contrast, AMA were not present in the sera of athletes or in the sera of controls. Although the significance of AMA in subjects with pathologic myocardial hypertrophy has not yet been established, their absence in the sera of athletes strengthens the opinion that cellular changes, as a compensatory response of the myocardium to a work overload, have a physiologic fashion in these cases. Moreover, identification of AMA in the sera of athletes with disproportionate severe left ventricular hypertrophy of uncertain origin may be helpful to ensure a single diagnosis.

Adolescent↗
PubMed1988

Equivocal and borderline myocardial hypertrophy in relatives of patients with hypertrophic cardiomyopathy: possible implications in genetics of the disease.

To determine the occurrence of familial and sporadic forms of hypertrophic cardiomyopathy (HC) 74 first-degree relatives of 21 patients with proven HC were studied by M-mode and two-dimensional echocardiography. A diagnosis of HC was made in 11 relatives (15%) while it was excluded in 61 of them (82%); 2 subjects (3%) were considered neither affected nor unaffected (borderline left ventricular hypertrophy suggestive of HC). Inspection of pedigrees revealed 38% of familial forms of HC with an autosomal dominant pattern of inheritance in 5/8 families (62%). Furthermore, among those relatives judged unaffected by means of full echocardiographic criteria for HC, an attempt was made to find out whether minor changes of left ventricular geometry were present for their possible implications in genetics of HC (latent or potential forms, low phenotypic expression of the disease). Eleven out of 61 unaffected relatives had a left ventricular wall thickness radius ratio greater than 0.50 (equivocal hypertrophy), a value that was higher than two standard deviations of the control group. Assessment of clinical significance of borderline and equivocal hypertrophy in relatives of patients with HC is required for a better understanding of genetic transmission of this disease. In this view the occurrence of sporadic and familial forms of HC might be revisited.

Adolescent↗
PubMed1988

Clinical relevance of the electrocardiogram in relatives of patients with hypertrophic cardiomyopathy.

Eighty-nine first-degree relatives of 22 patients with an established diagnosis of hypertrophic cardiomyopathy underwent electrocardiographic and echocardiographic screening. Scalar electrocardiogram was abnormal in 30/89 (33.7%) relatives. Of these thirty, eleven had definite evidence of hypertrophic cardiomyopathy at echo; one had borderline hypertrophy and was considered neither affected nor unaffected; four had questionable signs of hypertrophy. The remaining 14 relatives had normal echo-cardiograms. Fifty-nine relatives (66.3%) had normal electrocardiograms; at echo 3 were considered to have borderline hypertrophy, 16 had questionable signs of hypertrophy and 40 were normal. In relatives of patients with hypertrophic cardiomyopathy an abnormal electrocardiogram may reflect different morphologic conditions: a real hypertrophic cardiomyopathy or a myocardial hypertrophy of uncertain significance. Furthermore, in these categories of subjects, an abnormal electrocardiogram with normal echo must be considered with caution.

Adult↗