[Congenital heart diseases in adult age and primary cardiomyopathies at the referral centers].
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Adolescent↗
Biomedical subjects
A Dolara
Publications and source records attributed to A Dolara.
Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy.
BACKGROUND: Clinical impact of atrial fibrillation (AF) in hypertrophic cardiomyopathy (HCM) is largely unresolved. Thus, we analyzed the prognostic implications of AF in a large, community-based HCM population assembled from Italian and US cohorts. METHODS AND RESULTS: Occurrence of AF and outcome were assessed in 480 consecutive HCM patients (age at diagnosis, 45+/-20 years; 61% male) who were followed up for 9.1+/-6.4 years. AF occurred in 107 patients (22%; incidence, 2%/y) and was independently predicted by advancing age, congestive symptoms, and increased LA size at diagnosis. Patients with AF had increased risk for HCM-related death (OR, 3.7; P<0.002) because of excess heart failure-related mortality but not sudden, unexpected death. This risk associated with AF was substantially greater in patients with outflow obstruction or with earlier development of AF (</=50 years of age). AF patients were also at increased risk for stroke (OR, 17.7; P=0.0001) and severe functional limitation (OR for NYHA class III or IV, 2.8; P<0.0001). Compared with those with exclusively paroxysmal AF, patients developing chronic AF showed higher combined probability of HCM-related death, functional impairment, and stroke (P<0.0001). In a subgroup of 37 patients with AF (35%), the clinical course was largely benign in the absence of stroke and severe symptoms. CONCLUSIONS: In a community-based HCM population, AF (1) was common, with 22% prevalence over 9 years; (2) was associated with substantial risk for heart failure-related mortality, stroke, and severe functional disability, particularly in patients with outflow obstruction, those </=50 years of age, or those developing chronic AF; and (3) was nevertheless compatible with benign outcome in 35% of patients.
Adult↗
Increased glutamate in CSF and plasma of patients with HIV dementia.
BACKGROUND: Experimental evidence suggests that excitotoxicity might play a major role in HIV-induced neurodegeneration. However, few studies have investigated the role of endogenous glutamate in patients with HIV dementia. OBJECTIVE: To analyze CSF and plasma glutamate levels in 30 patients with AIDS with different dementia severity compared with 10 patients with other neurologic disorders, 11 healthy control subjects, and 10 patients with Alzheimer-type dementia. METHODS: CSF and plasma glutamate levels were measured by reverse-phase high-performance liquid chromatography followed by fluorometric analysis. RESULTS: Glutamate CSF levels were increased fivefold in the patients with HIV vs normal control subjects (p = 0.001), patients with Alzheimer-type dementia (p < 0.0001), and patients with other neurologic disorders (p < 0.01). CSF glutamate levels were also related to the degree of dementia (p < 0.02) and brain atrophy (p < 0.002). Plasma levels were also higher in the patients with HIV (p < 0.0001) but did not correlate with either clinical or imaging features. CONCLUSION: Increased CSF glutamate may originate within the CNS and may play a pathogenetic role in HIV dementia, thus supporting the treatment of these patients with glutamate receptor antagonists.
AIDS Dementia Complex↗
"Natural histories" of mitral valve prolapse. Influence of patient selection on cardiovascular event rates.
BACKGROUND: In previous studies the reported incidence of cardiovascular events among mitral valve prolapse patients has differed more than 10 fold. We endeavored to determine the relation between the clinical features and mode of ascertainment of mitral valve prolapse and the resulting event rate. METHODS: Between January 1979 and August 1996, 275 patients (129-47% men, 146-53% women, mean age 43 +/- 19 years), were followed for a mean of 98 months after evaluation in a referral center for valvular heart disease. Comparative data were obtained from a separate, less selected population consisting of 316 patients. RESULTS: A total of 65 events occurred (2.9/100 patient-years): 46 (2.0/100 patient-years) mitral surgery, 12 cardiac deaths (0.5/100 patient-years), 6 neurologic ischemia (0.26/100 patient-years), and 1 infective endocarditis (0.04/100 patient-years). The overall event rate varied significantly according to demographic, clinical and echocardiographic variables (all p < 0.0001). It was higher among males (odds ratio-OR 2.1), subjects > or = 45 years of age (OR 14.7), those with a holosystolic murmur (OR 25.9), an enlarged left ventricle (OR 13.5) or left atrium (OR 34.9) and those with 3-4+ mitral regurgitation at color Doppler echocardiography (OR 40.0). It was lower in those with an audible mid-systolic click (OR 0.05). These ORs closely resembled those we reported previously in a less selected population. At multivariate analysis, male gender (p = 0.013), severe Doppler mitral regurgitation (p = 0.0048), and left atrial enlargement (p = 0.046) were all independent predictors of events. CONCLUSIONS: In a population of mitral valve prolapse patients, including many with significant mitral regurgitation at baseline, we identified similar predictors of events but an overall event rate nearly 3 times higher than that we previously reported for relatively unselected patients or family members in New York City (1/100 patient-years). Therefore, the impact of patient selection on the prevalence of mitral regurgitation, older age and male gender strongly affects the adversity of the "natural history" of mitral valve prolapse.
Adolescent↗
Cardiac contusion in blunt chest trauma: a combined study of transesophageal echocardiography and cardiac troponin I determination.
BACKGROUND: The role of cardiac troponin I (cTnI) is well established in acute myocardial ischemia. However, its role in myocardial contusion remains to be clarified. Since transesophageal echocardiography (TEE) appears, at present, to be the best method for the diagnosis of myocardial contusion, the aim of this study was to measure the concentration of cTnI in patients with blunt chest trauma studied using TEE. METHODS: Thirty-two patients (27 males, 5 females, mean age 44+/-20 years), admitted to the Trauma Center of our Institution with clinical and/or radiological signs of acute blunt chest trauma, underwent biplane TEE within 24 hours of injury; serial blood samples were taken to measure cTnI levels (normal values < 0.4 ng/ml), using fluorimetric enzyme immunoassay. RESULTS: Abnormal levels of cTnI were found in 17 patients (53%): 7 patients had levels of cTnI between 0.4 and 1 ng/ml, whereas 10 patients had levels > 1 ng/ml. Segmental wall motion abnormalities consistent with myocardial contusion could be identified by echocardiography in 6/10 patients with cTnI levels > 1 ng/ml (60%) but in no patients with normal cTnI levels or with titers between 0.4 and 1 ng/ml; mean cTnI levels showed a significant difference between the two groups of patients with and without echocardiographic signs of myocardial contusion (2.6+/-1.6 vs 0.6+/-1.4 ng/ml, p < 0.001). CONCLUSIONS: Abnormal titers of cTnI suggesting myocardial contusion may be found in more than half of patients with blunt chest trauma; however, myocardial injury can be detected by TEE only for cTnI levels > 1 ng/ml; cTnI concentrations ranging between 0.4 and 1 ng/ml might be indicative of myocardial microlesions, not detectable by echocardiography, even if TEE is used; cTnI assay could therefore be suggested as a screening test before performing TEE after blunt chest trauma.
Adult↗
[Guidelines].
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Angioplasty, Balloon, Coronary↗
Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy.
OBJECTIVES: The present study was designed to prospectively evaluate the prognostic relevance of abnormal blood pressure response to exercise (ABPR), defined as hypotension or failed blood pressure increase (<20 mm Hg) with exercise, in a community-based hypertrophic cardiomyopathy (HCM) population representative of the overall disease spectrum. BACKGROUND: Abnormal blood pressure response to exercise has been proposed as a marker for hemodynamic instability and increased risk for disease-related mortality in highly selected patient populations with HCM. METHODS: The study population comprised 126 patients (aged 42+/-14 years) who underwent maximal symptom-limited cycloergometer exercise testing as part of the standard evaluation at our institution, and who were followed systematically for 4.7+/-3.7 years after testing. RESULTS: Of the 126 study patients, 98 (78%) had a normal blood pressure response during exercise, whereas the other 28 (22%) had ABPR, including nine with hypotension and 19 with failed blood pressure rise. During the follow-up period, nine patients (7%) died of HCM-related causes (three suddenly and six heart failure-related), of whom four had ABPR. In those patients aged < or =50 years, survival analysis after exercise testing showed a significantly increased risk for cardiovascular mortality associated with ABPR compared with a normal exercise response (p = 0.04), with an odds ratio of 4.5 (95% confidence interval: 1.1, 20.1). However, ABPR showed low positive predictive accuracy for cardiovascular mortality (i.e., 14%), whereas negative predictive accuracy was high (i.e., 95%). CONCLUSIONS: A hypotensive blood pressure response during exercise occurred in over 20% of a community-based patient cohort with HCM, and was associated with adverse long-term prognosis in patients <50 years old. However, the positive predictive accuracy of this blood pressure response is too low to justify modifications of clinical management or to allow identification of the high-risk patient based solely on an abnormal test result. By virtue of its high negative predictive accuracy for HCM-related mortality, the blood pressure response to exercise appears to be most valuable (in conjunction with the absence of other well recognized risk factors) as a screening test for the identification of low-risk subsets of patients.
Adult↗
Nonsustained ventricular tachycardia as a predictor for sudden death in patients with idiopathic dilated cardiomyopathy. The role of amiodarone treatment.
BACKGROUND: Sudden death frequently occurs in patients with idiopathic dilated cardiomyopathy. Ventricular arrhythmias are encountered in almost all cases. The prognostic significance of life-threatening arrhythmias such as successfully resuscitated ventricular fibrillation and sustained ventricular tachycardia is well known, while it is controversial for ventricular arrhythmias of a lower degree. Amiodarone has been used widely in these patients but its value in preventing sudden death is still uncertain. The aim of this study was to evaluate the prognostic significance of runs of nonsustained ventricular tachycardia (NSVT) as a hallmark for sudden death and the efficacy of amiodarone in preventing sudden death and reducing overall mortality in a large series of patients with dilated cardiomyopathy. METHODS: Over the period between 1983 and 1994, a series of 151 consecutive patients with idiopathic dilated cardiomyopathy underwent ambulatory electrocardiographic monitoring for a mean period of 191 hours/patient. Seventy-nine patients (56 male, mean age 50.7 +/- 13.1 years) (group A) had ventricular arrhythmias of Lown class < or = 4A, while 72 (53 male, mean age 48.6 +/- 12.8 years) (group B) had one or more NSVT runs. The two groups were well matched in terms of clinical features. Mean follow-up period was 86.8 +/- 38.7 and 74.7 +/- 39.5 months, respectively. In group A no antiarrhythmic drug was administered, while in group B 54/72 patients were treated with amiodarone (mean dosage 300 mg/day) for a mean period of 69.7 +/- 37.8 months (group B1). The remaining 18 patients received class I antiarrhythmic drugs, mexiletine (12) and propaphenone (6) for a mean period of 46.1 +/- 29.4 months, because amiodarone was contraindicated (3) or serious side-effects occurred during amiodarone treatment (15), which was discontinued after a mean period of 3.8 +/- 3.1 months (group B2). RESULTS: The cumulative survival probability in the whole population was 86.6% at two years and 65.6% at five years. The rate of sudden death was 6.0% at two years and 18.3% at five years. No statistically significant difference was observed in terms of all-cause mortality or sudden death in the three groups (A, B1, B2). In group B1, amiodarone determined the disappearance of NSVT at Holter monitoring in 50% of patients (27), with no significant difference in the rate of sudden death between the two subgroups. CONCLUSIONS: In unselected patients with idiopathic dilated cardiomyopathy, cardiovascular mortality does not differ between those with NSVT on chronic amiodarone treatment and those without NSVT who have not undergone antiarrhythmic therapy. There was a trend towards a higher overall and sudden mortality rate in patients with NSVT treated with other antiarrhythmic drugs vs patients with NSVT treated with amiodarone, but due to the small size of the first group no significant difference could be calculated. Assuming NSVT as a potential prognostic marker for sudden death, amiodarone treatment may have exerted a beneficial effect in these patients, but this statement is only a presumption due to the limitations of our study. The disappearance of NSVT during amiodarone treatment is not predictive of a reduced rate in sudden death, so that the potential effect of the drug does not appear to be related to the suppression of NSVT at Holter monitoring.
Adolescent↗
[Should the patient with an interatrial defect recognized in adulthood always be operated on?].
BACKGROUND: Atrial septal defect (ASD) can be recognized in adult age, mostly in asymptomatic or scarcely symptomatic patients. These patients differ from patients in "historical" clinical series, in whom diagnosis was done on the basis of clinical evidence, and their natural history is probably different. AIM OF THE STUDY: Our aim was to verify retrospectively results of surgery versus medical follow-up in an adult population with ASD with age at first diagnosis > or = 30 years. PATIENTS AND METHODS: Seventy-two patients with ASD, 52 females (72%), observed at our Institution since 1978, were considered. Mean age at diagnosis was 48 +/- 12 years (range 30-79); 36 patients (50%, group A) are still on medical therapy, 36 patients (group B) were operated. As groups A and B did not differ significantly in any demographic, clinical or echocardiographic parameter, they were compared for the incidence of complications. RESULTS: During follow-up (100 +/- 70 months, range 12-240), the incidence of major clinical events showed no significant differences in the two groups, as cardiac death or cardiovascular complications (cerebral ischemic events, severe mitral insufficiency, reoperation) occurred in 4 patients in group A (11%) and in 4 patients in group B (11%). Worsening of NYHA class was observed in 3 patients from group A (8%) and 2 patients from group B (5.5%; p = ns). New onset of supraventricular arrhythmias occurred more frequently in group B (14 patients, 39%) than in group A (5 patients, 14%) (p = 0.01; OR = 3.9; CI 95%: 1.2-12.6). CONCLUSIONS: In an adult population affected with asymptomatic or mildly symptomatic ASD and age at first diagnosis > or = 30 years, surgical closure of the defect did not modify morbidity and mortality at a mid-term follow-up. We suggest that, mostly in older asymptomatic patients, surgery should not be a routine choice and clinical decision-making should be individualized in each case.
Adult↗
Coronary arteriovenous aneurysmatic fistula draining into the right atrium.
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Angiography↗
Prognostic value of non-sustained ventricular tachycardia and the potential role of amiodarone treatment in hypertrophic cardiomyopathy: assessment in an unselected non-referral based patient population.
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.
Adult↗
Cardiac troponin I in myocardial contusion.
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Adult↗
Elevated glutamate in the cerebrospinal fluid of patients with HIV dementia.
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AIDS Dementia Complex↗
Risk for atrial fibrillation in patients with hypertrophic cardiomyopathy assessed by signal averaged P wave duration.
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.
Adolescent↗
Platelet and blood clotting activation in patients with mitral valve prolapse.
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.
Adenosine Diphosphate↗
Signal-averaged P-wave duration and risk of paroxysmal atrial fibrillation in hyperthyroidism.
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.
Adult↗
[Myocardial lipofuscinosis of unknown etiology in a young asymptomatic woman].
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.
Adult↗
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↗