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A Dolara

Publications and source records attributed to A Dolara.

At least 19 recordsLinked to original sources

Hypertrophic cardiomyopathy in Tuscany: clinical course and outcome in an unselected regional population.

OBJECTIVES: Our aim was to study a population of patients with hypertrophic cardiomyopathy from the well defined geographic region of Tuscany in central Italy, a group virtually free of selective referral bias and therefore probably closely representative of the true patient population with this disease. BACKGROUND: Most available information on clinical course, natural history and prognosis of hypertrophic cardiomyopathy is based on data generated from tertiary referral centers and therefore constitutes a potentially biased perspective of the disease process in this complex and diverse condition. METHODS: The study group comprised 202 patients aged 1 to 74 years (mean +/- SD 41 +/- 17) at initial diagnosis and followed up for 1 to 30 years (mean 10 +/- 5). RESULTS: Largely with the use of single or multiple drug therapy, the vast majority of patients (n = 154 [76%]) were asymptomatic or mildly symptomatic and in stable or improved condition over the period of follow-up, whereas the remaining patients (n = 48 [24%]) experienced deterioration, had substantial functional impairment or died. Of the 13 patients (6%) who died of cardiovascular causes related to hypertrophic cardiomyopathy, 11 had progressive congestive heart failure (including 6 in the end-stage phase) and only 2 died suddenly. The annual mortality rate for cardiovascular disease was 0.6% and that due to sudden cardiac death was only 0.1%; the cumulative survival rate was 97%, 95% and 92%, respectively, at 5, 10 and 15 years of follow-up. Atrial fibrillation proved to be a relatively common (n = 57 [28%]) and particularly unfavorable clinical feature, with premature death occurring in 9 of the 57 patients. The cumulative survival rate after 15 years was 76% for patients with atrial fibrillation versus 97% for patients with sinus rhythm. Syncope occurred in 33 patients (16%) but did not appear to be of prognostic significance. CONCLUSIONS: In an unselected regional population, hypertrophic cardiomyopathy had a relatively benign prognosis inconsistent with its prior characterization as a generally progressive disorder, based primarily on the experience of selected referral institutions. Sudden unexpected cardiac death was distinctly uncommon, although a sizable proportion of patients (particularly the subset prone to atrial fibrillation), did experience clinical deterioration.

Adolescent

[Oral anticoagulant therapy in dilated cardiomyopathy. Warfarin treatment results in subgroups of patients at risk of embolic complications].

BACKGROUND: The aim of the study was to assess the role of oral anticoagulant therapy in patients with dilated cardiomyopathy at risk of embolic complications. METHODS: We studied retrospectively two hundred thirty-six patients with dilated cardiomyopathy; sixty-eight cases, with intracavitary thrombosis at cross-sectional echocardiography (17 patients), embolic episodes (22), N.Y.H.A. functional class IV (38), chronic atrial fibrillation (23) or with a combination of such conditions, were treated with warfarin. The cumulative period of exposure to embolic events during follow-up was 814 years in the whole population in the absence of anticoagulant treatment and 213 years during treatment. RESULTS: The rate of new embolic events was 1.6 and 0 for 100 patients-years for the two periods respectively. The difference was not statistically significant. No clinically relevant haemorrhagic complication was seen during treatment. CONCLUSIONS: Oral anticoagulant therapy may be safely given to subgroups of patients with dilated cardiomyopathy at risk of embolic episodes, following empirical guidelines, provided a careful clinical and laboratory monitoring is carried on, even if no definite conclusion may be obtained about the efficacy of this treatment from a non-randomized study with low rates of new embolic events.

Administration, Oral

[Familial pulmonary stenosis: considerations on genetic aspects].

A family with 4 cases of pulmonary valvular stenosis with under-developed or normal right ventricle, is reported. The father and his three daughters were affected. In two of the girls, who were twins, a prenatal diagnosis was made using 2D-echo. The importance of genetic counseling and prenatal diagnosis in the families at risk are underlined.

Adult

[Mitral valve prolapse. A prevalence study using bidimensional echocardiography in a young population].

The results of many studies on the prevalence of mitral valve prolapse have been greatly influenced by the diagnostic methods and criteria adopted as well as by population selection. The method of choice today is 2d-echocardiography because of its ability to highlight both movement anomaly (i.e. functional prolapse) and any eventual morphological variations of the mitral valve (i.e. anatomic prolapse). The latter (chordae lengthening, thickening and overabundance of the leaflets, dilation of the valvular ring) are, nowadays, considered especially important even as predictive factors of complications. Therefore we studied the prevalence of these two types of prolapse in a population of 420 university students. Functional mitral valve prolapse was found in 27/420 (6.4%) and anatomical prolapse in 2 cases (0.5%). No auscultatory finding was present in 24/27 patients with functional prolapse. There was no correlation between the two types of mitral valve prolapse and the body mass index, the fractional shortening of the left ventricle and symptoms (dyspnea, palpitations, precordial pain, dizziness). We think that the distinction between the two types of mitral valve prolapse should prove very useful for the comparison of results in future epidemiological studies. Follow-up of both groups of patients will hopefully clarify the usefulness of such distinction from the clinical point of view.

Adult

Lack of correlation between intracavitary thrombosis detected by cross sectional echocardiography and systemic emboli in patients with dilated cardiomyopathy.

The correlation between intracavitary thrombosis detected by cross sectional echocardiography and systemic embolism was studied in 126 consecutive patients with idiopathic dilated cardiomyopathy who were examined from January 1980 to September 1987. A total of 1041 serial echocardiograms were obtained with 3.5 and 5 MHz transducers. The mean follow up period was 41.2 months. The survival rate was 88% at two years and 56% at five years. Echocardiography showed intracavitary thrombi in 14 (11.1%) patients; 13 were mural and 11 were localised at the apex of the left ventricle. Twelve patients (8.4%) had systemic emboli; this corresponded to an incidence of new embolic events of 1.4 for 100 patient-years. Patients with intracavitary thrombi or systemic emboli were treated with oral anticoagulants, as were nine in functional class IV of the New York Heart Association, for 61 patient-years. The cumulative observation period for the whole population study was 418 patient-years. None of the patients with intracavitary thrombosis had embolic complications and none of those with embolism had intracavitary thrombi. Rates of intracavitary thrombosis and systemic embolism in this series were low and there was no overlap between the two events. This may have been because the patients did not have severe dilated cardiomyopathy, because echocardiography did not detect all the thrombi, or because patients were treated with oral anticoagulants. The presence of intracardiac thrombosis detected by cross sectional echocardiography is not predictive of systemic embolism in patients with idiopathic dilated cardiomyopathy. Criteria for the use of the anticoagulant treatment remain largely empirical in these cases.

Adult

[Cardiomyopathy in Italy today: extent of the problem].

Since the number of patients suffering from cardiomyopathy in Italy is unknown at present, a multicentre survey was carried out in 15 University and Hospital cardiological centres. Each centre was asked to report the number of cases of dilated, hypertrophic or restrictive cardiomyopathy observed between 1970 and 1987. Information was also sought regarding the residence of the patient at the time of diagnosis. Diagnostic criteria for each type of cardiomyopathy, based on those published in the current literature, were sent to the participating centres. For the entire period, a total of 2,229 patients with dilated, 913 with hypertrophic, and 64 with restrictive cardiomyopathy were reported. The number of cases observed between 1980-87 was about four times greater than that observed between 1970-79 for dilated cardiomyopathy and three times greater for hypertrophic cardiomyopathy. In the period 1970-79, a minimal annual incidence of dilated cardiomyopathies was calculated. In the centres this ranged from 0.005 to 1.8 per 100,000 inhabitants, although no systematic evaluation was made. On the contrary, minimal prevalence for hypertrophic cardiomyopathy ranged from 0.4 to 5.6 between 1970-79. These rates increased to 0.09-3.4 for dilated cardiomyopathy and to 1.0-23.0 for hypertrophic cardiomyopathy in the period 1980-87. On the basis of this survey the number of patients with a diagnosis of dilated and hypertrophic cardiomyopathy seems to be on the increase in our country. This increase may be due to the diagnostic facilities available, such as echocardiography, and possibly to a better knowledge and a greater interest in cardiomyopathies.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiomyopathy, Dilated

[Echocardiographic study of heart rhabdomyoma in tuberous sclerosis].

In order to study the prevalence of cardiac rhabdomyoma in tuberous sclerosis using non invasive methods, 11 consecutive patients affected by tuberous sclerosis (age: 3 months-22 years, mean 6 years) were examined between January 1984 and April 1987. In each patient clinical examination, 12 lead electrocardiogram, M-mode and two-dimensional echocardiogram were performed: on 7 of them a 24-hour ambulatory electrocardiogram was also performed. Clinical examination revealed a systolic murmur (grade 3/6) in two cases. In one of them it was associated with cyanosis and cardiac failure. None of other patients showed signs or symptoms related to the heart during clinical examination. ECG was abnormal in 3 cases (ventricular pre-excitation in 2 cases and left ventricular strain in 1). Two-dimensional echocardiogram showed single or multiple intracardiac masses suggestive of cardiac rhabdomyoma in 8 cases; in 3 of them masses were isolated, intramural, and in the interventricular septum, from 5 to 16 mm in diameter; in the other 5 cases they were multiple, intramural and endoluminal, in the interventricular septum, in the right or left ventricle, from 7 to 20 mm in diameter. Ambulatory electrocardiogram revealed only isolated ectopic supraventricular and ventricular beats in 2/7 cases. Each patient was clinically controlled every six months using ECG standard and two-dimensional echocardiogram. The mean follow-up period was 32 months (range 9-53). This study confirms the usefulness of the two-dimensional echocardiography to visualize intramural or intracavitary masses due to cardiac rhabdomyoma in patients with tuberous sclerosis, especially when they appear clinically asymptomatic. Two-dimensional echocardiography is probably the ideal method to use in order to evaluate potential increase in cardiac rhabdomyoma, when prolonged follow-up studies are performed.

Adult

Primary pulmonary hypertension: an Italian multicenter study. A retrospective epidemiological survey in the period 1975-1985.

An epidemiological retrospective survey on primary pulmonary hypertension was undertaken in Italy in 1985-6. The aim of the study was to obtain clinical and laboratory data on patients observed in the major cardiological Institutions, both Universities and Hospital Clinical Centers, in the period 1975-85. Forms were sent to 67 centers and twenty-three of them communicated their data on 124 cases of primary pulmonary hypertension. The diagnosis was considered certain or probable in 91 patients by the referring center. The mean age of patients was 38.5 years, with an overall female-to-male ratio of 2:1. Data were subjected to statistical analysis which confirmed the poor prognosis of the disease particularly for patients with high pulmonary resistances, low cardiac output and in class 3-4 of the NYHA. Half of the 124 cases were treated with calcium antagonists after 1980, but the results of therapy in modifying the clinical course of the disease could not be assessed by the study. Like all retrospective studies even the Italian Multicenter Study is biased but it can be considered as an useful preliminary investigation which can form the basis for a prospective registry of primary pulmonary hypertension cases.

Age Factors

[Pulmonary atelectasis and massive pleural effusion. Echocardiography].

Pulmonary atelectasis can be detected by Two Dimensional Echocardiography (2D-E) when massive pleural effusion is present. A triangular mass, base toward the mediastinum and apex moving freely in the pleural cavity, is shown by 2D-E either in an apical modified view in left pleural effusions or in a subcostal modified view in right pleural effusions. The texture of the mass is liver-like. Three cases are reported by way of example. Differential diagnosis with other masses, such as pleural or pericardial metastasis, is discussed.

Child