Coronary arteriovenous aneurysmatic fistula draining into the right atrium.
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Biomedical subjects
Publications and source records attributed to A Dolara.
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BACKGROUND: Amiodarone has been reported to reduce the likelihood of sudden death in patients with hypertrophic cardiomyopathy (HCM). However, data regarding the clinical course in HCM have traditionally come from selected referral populations biased toward assessment of high risk patients. AIMS: To evaluate antiarrhythmic treatment for sudden death in an HCM population not subject to tertiary referral bias, closely resembling the true disease state present in the community. METHODS: Cardiovascular mortality was assessed in relation to the occurrence of non-sustained ventricular tachycardia (NSVT) on 24 or 48 hour ambulatory Holter recording, a finding previously regarded as a marker for sudden death, particularly when the arrhythmia was frequent, repetitive or prolonged. 167 consecutive patients were analysed by multiple Holter ECG recordings (mean (SD) 157 (129) hours) and followed for a mean of 10 (5) years. Only patients with multiple repetitive NSVT were treated with amiodarone, and in relatively low. doses (220 (44) mg/day). RESULTS: Nine HCM related deaths occurred: 8 were the consequence of congestive heart failure, but only 1 was sudden and unexpected. Three groups of patients were segregated based on their NSVT profile: group 1 (n = 39), multiple (> or = 2 runs) and repetitive bursts (on > or = 2 Holters) of NSVT, or prolonged runs of ventricular tachycardia, included 4 deaths due to heart failure; group 2 (n = 38), isolated infrequent bursts of NSVT, included 1 sudden death; group 3 (n = 90), without NSVT, included 4 heart failure deaths. Kaplan-Meier survival analysis showed no significant differences in survival between the three groups throughout follow up. CONCLUSIONS: In an unselected patient population with HCM, isolated, non-repetitive bursts of NSVT were not associated with adverse prognosis and so this arrhythmia does not appear to justify chronic antiarrhythmic treatment. Amiodarone, administered in relatively low doses, did not carry an independent and additive risk for cardiac mortality. Amiodarone may have contributed to the absence of sudden cardiac death in patients believed to be at higher risk because of multiple repetitive NSVT.
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OBJECTIVE: To assess the relation between P wave duration and the occurrence of atrial fibrillation in hypertrophic cardiomyopathy (HCM). DESIGN: High resolution signal averaged electrocardiography was used in 110 patients with HCM to determine whether patients at risk for paroxysmal atrial fibrillation could be detected during sinus rhythm by measuring P wave duration. Filtered P wave duration was measured manually, over an average of 300 beats per patient. RESULTS: During follow up, 18 patients (16%) had at least one one clinical episode of paroxysmal atrial fibrillation, including four patients whose initial episode followed the signal averaged electrocardiogram. The 18 patients with overt atrial fibrillation had greater P wave duration than the 69 patients who did not develop atrial fibrillation: 150 (SD 20) v 126 (14) ms; P < 0.001. Another 23 patients without clinically evident atrial fibrillation had prolonged bursts of the arrhythmia on Holter recording, and showed a P wave duration (138 (15) ms) intermediate between patients with and without clinical atrial fibrillation. In assessing risk for atrial fibrillation, P wave duration > or = 140 ms was associated with sensitivity, specificity, and positive predictive accuracy values of 56%, 83%, and 66%. Multivariate analysis showed that duration of the P wave was the only independent variable associated with occurrence of atrial fibrillation; if P wave duration > or = 140 ms was combined with left atrial enlargement > 40 mm, the specificity and positive predictive accuracy for atrial fibrillation increased to 93% and 78%. CONCLUSIONS: Measurement of P wave duration in sinus rhythm by high resolution signal averaged electrocardiography may be a useful non-invasive clinical tool for identifying patients with HCM likely to develop electrical instability and atrial fibrillation.
In patients with mitral valve prolapse (MVP) a high incidence of valvular abnormalities with a history of previous cerebrovascular disease has been reported and an embolic mechanism has been proposed. Aim of this study is the study of platelet and coagulation activation in patients with MVP. Fifty-four patients affected by MVP (mean age 46 +/- 15 yrs, 22 males, 32 females) and 50 control subjects, age- and sex-matched, were tested for platelet activation [P-selectin and GpIIb-IIIa platelet surface expression at rest and after stimuli by flow cytometric analysis, Beta-Thromboglobulin (TG) and Platelet Factor 4 (PF4) plasma levels by ELISA, platelet-rich-plasma (PRP) and whole blood spontaneous platelet aggregation (SPA)] and for activation of blood coagulation (Prothrombin activation fragment F1+2 plasma levels by ELISA). P-selectin, GpIIb-IIIa expression, Beta-TG, PF4 and SPA were found similar in MVP patients and in controls. However, in patients with severe mitral regurgitation (MR) the percentage of activated platelets which express P-selectin after stimuli was slightly but significantly (p < 0.05) lower in comparison to MVP patients without or with mild to moderate MR and to controls. Moreover, in patients with severe MR F1+2 levels (median 1.6 nmol/L, range 0.6-2.6 nmol/L) were significantly higher (p < 0.001) than both in controls (median 0.95 nmol/L, range 0.2-1.4 nmol/L) and in patients without or with mild to moderate MR (median 1.0 nmol/L, range 0.4-2.3 nmol/L). Our findings suggest that MVP is not responsible per se for blood clotting activation, but in patients with severe mitral insufficiency an increase in thrombin generation can occur. These alterations in hemostatic system may represent a mechanism by which MR increases the risk of thromboembolic events in patients with MVP.
The onset of atrial fibrillation (AF) in hyperthyroid patients constitutes an unfavorable clinical event associated with high risk of cardiovascular complications, occurring in approximately one fifth of patients. Therefore, it is advantageous to define noninvasive markers that may identify patients at risk. The high-resolution, signal-averaged electrocardiogram was used to evaluate the relation between P-wave duration and occurrence of paroxysmal AF in a group of 50 patients with hyperthyroidism, of whom 24 had a history of paroxysmal AF and 26 did not. Filtered signal-averaged P-wave duration was measured over an average of 300 beats/patient while in sinus rhythm, both at the time of first diagnosis of hyperthyroidism and after restoration of euthyroidism by medical treatment. The 24 patients with paroxysmal AF had significantly greater P-wave duration than the 26 patients without it (135 +/- 7 vs 124 +/- 9 ms; p = 0.001). A P-wave duration cut-off value of 130 ms held specificity, sensitivity, and positive predictive accuracy values of 79%, 85%, and 83%, respectively. Of several variables, multivariate analysis showed P-wave duration to be the only independent variable significantly associated with the occurrence of paroxysmal AF. Thus, the high-resolution signal-averaged electrocardiogram may be a useful noninvasive clinical tool for the identification of electrical instability associated with paroxysmal AF in hyperthyroid patients.
An asymptomatic 32-year-old woman was examined because of a family history of unexplained sudden deaths and a left ventricular strain pattern of the ECG during a routine examination. No abnormal finding of the cardiovascular system was present but an endomyocardial biopsy showed focal interstitial fibrosis, myocyte hypertrophy with heavy perinuclear accumulation of lipofuscin. The cause of these abnormalities remains unknown.
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.
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.
BACKGROUND: Mitral valve prolapse (MVP) is generally regarded as a benign condition, but serious complications (including severe mitral insufficiency, cerebral ischemia, infective endocarditis, complex arrhythmias and sudden death) have been described in a minority of patients and have been correlated to demographic, clinical and echocardiographic characteristics. Both a lack of standardized definition of MVP in earlier studies and the different ways of recruitment of MVP patients may explain the variability in reported complication rates. METHODS: As an offspring of a larger prospective study this paper focuses on the profile of patients who were found to have MVP by M-Mode and two-dimensional echocardiography in several outpatient hospital departments. A total of 8252 consecutive subjects, examined since March 1990 to February 1991 in the Echo laboratories of the Florence area are considered; according to the presence or absence of structural changes (anterior mitral leaflet thickness > 5 mm, leaflet redundancy and/or anulus dilatation) two groups of patients with MVP (A and B) were identified. RESULTS: A MVP was diagnosed in 288 subjects (3.5%), 170 females (59%) and 118 males (41%), mean age 41 +/- 18 years (range 7-84). 110 (38%) were in Group A, 178 (62%) in Group B. The following parameters differed significantly in the two groups: age (45 +/- 17 vs 39 +/- 17 years; p < 0.003); male gender (50% vs 35%; p < 0.01); auscultatory findings (midsystolic click: 31% vs 68%; p < 0.00001; holosystolic murmur: 22% vs 3%; p < 0.00001); left ventricular diameter (53 +/- 7 vs 48 +/- 5 mm; p < 0.00001) and left atrial diameter (38 +/- 8 vs 33 +/- 5 mm; p < 0.00001). Among patients with mitral regurgitation detected by Color Doppler Echocardiography 65% were in Group A (p < 0.00001). CONCLUSIONS: These patients with MVP are obviously selected by the modality of recruitment; hence there is a higher prevalence of subjects with morphologic abnormalities and mitral regurgitation who are older and more likely to be male if compared to individuals with MVP who are found in the general population. A long-term follow-up of these patients is ongoing: owing to the data of the literature about prognostic predictors, a higher incidence of complications with a different prognosis between the two groups (with or without structural changes of the mitral valve) is expected.
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Atrial and ventricular arrhythmias have been reported with variable incidence in symptomatic patients with mitral valve prolapse (MVP). The role of clinical and echocardiographic parameters as predictors for arrhythmias still needs to be clarified. One hundred nineteen consecutive patients (56 women and 63 men, mean age 40 +/- 17 years) with echocardiographically diagnosed MVP were examined. A complete echocardiographic study (M-mode, two-dimensional, and Doppler) and 24-hour electrocardiographic monitoring were performed in all patients. Complex atrial arrhythmias (CAAs) included atrial couplets, atrial tachycardia, and paroxysmal or sustained atrial flutter or fibrillation. Complex ventricular arrhythmias (CVAs) included multiform ventricular premature contractions (VPCs), VPC couplets, and runs of three or more sequential VPCs (salvos of ventricular tachycardia). The relation between complex arrhythmias and clinical parameters (age and gender) and echocardiographic parameters (left atrial and left ventricular dimensions, anterior mitral leaflet thickness [AMLT], and presence and severity of mitral regurgitation) was evaluated by multiple logistic regression analysis. CAA were present in 14% of patients and CVA in 30%. According to multiple logistic modeling, CAA correlated separately in the univariate analysis with age, presence of MR, and left ventricular and left atrial diameters; age was the only independent predictor (p < 0.001). CVA, in the univariate analysis, correlated with age, female gender, left ventricular end-diastolic diameter, and AMLT; only female gender and AMLT were independent predictors in the multivariate analysis (p < 0.01). The incidence of mitral regurgitation (59%) was higher than expected in a general population of MVP patients.(ABSTRACT TRUNCATED AT 250 WORDS)
We report a case of a patient operated on for total anomalous pulmonary venous drainage at the age of six months. Seven years later an angiographic examination showed a obstruction of superior vena cava at Juxta right atrial. The patient, asymptomatic until the age of fifteen, was then subjected to further hemodynamic and angiocardiographic examination due to the appearance of an incessant and therapy-resistant cough. After the diagnostic procedure was completed, percutaneous balloon angioplasty was performed on the obstruction, which marked the remission of the symptoms.
The term mitral valve prolapse is used to describe either one of the pathophysiological mechanisms of mitral regurgitation or a distinct biological condition with a defined inherited basis, with or without mitral incompetence. In the former case, association with rheumatic fever is implied by the definition itself; in the latter it's questionable (rheumatic fever could act as an environmental factor in the expression of a genetically determined mitral valve prolapse). Three hundred and twenty patients with mitral valve prolapse, diagnosed as a primary one, were studied in order to assess the prevalence of a well-documented history of rheumatic fever: this resulted higher versus a control population (5.6% vs 0.9%-p < 0.01). The retrospective nature of the study does not allow a definite conclusion. Familial as well as long-term follow-up studies are necessary to better define the association between rheumatic fever and mitral valve prolapse; a potentially different prognosis, mainly accounting to the development of a clinically relevant mitral regurgitation in patients with mitral valve prolapse and previous rheumatic fever, has to be searched.
In a series of 55 patients with dilated cardiomyopathy, the presence of noninvasive recordings of late ventricular potentials (LVP) was correlated to ventricular tachycardia (VT), as detected by a 24-hour Holter monitoring obtained one week within LVP recording. LVPs were found in 12/55 patients (21.8%) and in 2 of a series of 66 normal subjects of the same age and sex. In all patients with LVP either non sustained (11 cases) or sustained (1 case) VT was present at Holter monitoring. In the other 43 patients without LVP only 13 (30%) had non sustained VT (p < 0.01). During the follow-up period (mean 17 months) six patients died suddenly; three of them had LVP and VT (sustained in one); two had non sustained VT, but no LVP; one had neither. This study suggests that the presence of LVP predicts VT recording in ambulatory ECGs. On the contrary, VT may be recorded in patients without LVP. Further studies are necessary to ascertain the value of LVP as a marker of sudden death in patients with dilated cardiomyopathy.
In order to get information on the number of patients with acquired valvular heart disease in Italy and about changes in the etiology of valvular involvement, we have examined death certificates regarding chronic endocardial and rheumatic heart disease; epidemiologic data of some congenital-degenerative valve disease; the results of a survey of valvular replacements in 40 Italian cardiac surgery centres in the periods 1970-1979 and 1980-1988. Deaths due to chronic endocardial and rheumatic heart disease (ISTAT source) were 8,129 in 1958 and 3,547 in 1986. These data suggest the presence of about 70,000 patients with acquired valvular heart disease due to chronic and rheumatic heart disease in Italy. Mitral valve prolapse and bicuspid aortic valve, complicated by degenerative changes, defined as congenital-degenerative valve diseases with clinical expression in adulthood, may be included in the group of acquired valvular heart disease. By extrapolation from epidemiological data the number of patients with acquired mitral insufficiency due to mitral valve prolapse might be about 28,000 and patients with aortic valve disease about 30,000 with an incidence of 1,500 per year. The survey performed in 45 Italian cardiac surgery centres revealed that the overall number of prosthetic valvular replacements was 7,695 in the period 1970-1979 and 25,467 in the period 1980-1988; the increase was maximal for aortic valve replacement (ratio 2.5:1 between the 2 periods) and and minimum for the combined mitral and aortic valve replacement (1.1:1). These data indicate that valvular heart disease of rheumatic origin is declining in Italy while degenerative valvular disease is increasing.
BACKGROUND: To evaluate the prevalence of congenital heart disease in a homogeneous population we examined 46,895 liveborns in the period from January 1975 to December 1984 in the Careggi Maternity Hospital in Florence. METHODS: The diagnosis of congenital heart disease was made in 579 newborns within five days from birth. All newborns were examined clinically by two neonatologists and referred to the pediatric cardiologist in case of cardiac abnormalities. An ECG was recorded in each of them, chest x-ray in 87% and echo in those who were born after 1980. The children were followed up until December 1989. Mean follow-up period was 6 years. RESULTS: The annual incidence ranged from 9.5% to 15.7% (average 12.3%). Chromosomic anomalies and extra-cardiac malformations occurred in 102 children (17.6%), respectively in 50 (8.6%) and 52 (9.0%) cases. Ventricular septal defect (VSD) and the patent ductus arteriosus (PDA), isolated or associated, were the most frequently recognized congenital heart diseases. 52 children (9.0%) were lost at follow-up. The initial diagnosis was changed in 11/579 cases. In 187 children (32.3%) there was a spontaneous disappearance of clinical and/or instrumental findings that had suggested the presence of congenital heart disease at birth: in 144 the diagnosis was VSD, and in 43 PDA. The prevalence of VSD was 4.7 per thousand in the period 1975-80 and 8.6 in the period 1981-84. 131 children (22.6%) died, 127 (96.9%) of them in the first year of life. 52 children were operated on and pulmonary valvuloplasty was performed in 5. CONCLUSIONS: The prevalence of congenital heart disease does not change during a long observation period, while the number of VSDs increases. VSD and PDA spontaneously close in a high percentage of cases. The study suggests the usefulness of an intensive care unit for newborns with severe congenital heart disease, especially for those in the first year of life.