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Biomedical subjects

A Angelini

Publications and source records attributed to A Angelini.

At least 55 records · Page 3Linked to original sources
PubMed1998

Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease.

Coronary arteries anomalies may be part of complex congenital malformations of the heart or be an isolated defect. In our anatomic collection of congenital heart disease, an isolated anomalous origin of coronary arteries was observed in 27 of 1,200 specimens (2.2%): left coronary artery from pulmonary trunk in five, origin from the wrong aortic sinus in 12 (both right and left coronary artery from the right sinus in four and from the left sinus in seven, left coronary artery from the posterior sinus in one), left circumflex branch from right aortic sinus or from very proximal right coronary artery in three, high takeoff of right coronary artery in three, stenosis of the coronary ostia attributable to valvelike ridge in four. In 16 (59%) patients (12 males and 4 females, age ranging from 2 months to 53 years; median, 14), the final outcome was sudden death; it occurred in all cases of left coronary artery origin from right aortic sinus, in 43% of right coronary artery origin from left aortic sinus, and in 40% of the left coronary artery from the pulmonary trunk. Sudden death was precipitated by effort in eight (50%) and was the first manifestation of the disease in eight (50%); previous symptoms consisted of recurrent syncope in four, palpitations in three, and chest pain in one. Five patients who died suddenly during effort were athletes. In conclusion, (1) more than half of our postmortem cases with anomalous origin of coronary arteries died suddenly, (2) all but two patients with sudden death had anomalous coronary artery origin from the aorta itself, (3) the fatal event was frequently precipitated by effort, (4) palpitations, syncope, and ventricular arrhythmias were the only prodromic symptoms and signs. Recognition during life of these coronary anomalies, by the use of noninvasive procedures, is mandatory to prevent the risk of sudden death and to plan surgical correction if clinically indicated.

Adolescent↗
PubMed1998

An unusual case of myocarditis.

A 25-year-old woman was admitted to our center for left heart failure. The clinical, electrocardiographic and bioptic findings suggested an acute lymphocytic myocarditis. One month later, paroxysmal hypertension and high urinary excretion of noradrenaline revealed the presence of a pheochromocytoma.

Adrenal Gland Neoplasms↗
PubMed1998

Cardiac transplantation in a Duchenne muscular dystrophy carrier.

We report here for the first time the case of a symptomatic DMD carrier, who had a heart transplant for a severe dilated cardiomyopathy. Dystrophin immunohistochemistry, western blot and analysis of X-chromosome inactivation on leucocytes, and skeletal and cardiac muscle biopsies on the explanted heart were performed. The patient was a heterozygote for exons 50-52 deletion in the dystrophin gene. The number of dystrophin-deficient fibres in the heart was much higher than in skeletal muscle. On the other hand, the explanted heart showed a non-skewed pattern of X-chromosome inactivation, as in leukocytes and skeletal muscle. The adverse cardiac course may be explained by the absence of regeneration among cardiomyocytes.

Adult↗
PubMed1998

HIV infection in the first heart transplantation in Italy: fatal outcome. Case report.

A 46-year-old man with alcoholic dilated cardiomyopathy underwent heart transplantation on November 14, 1985. It was the first cardiac transplant in Italy and at that time no HIV antibody screening test was available in this country. The patient remained in good health for 6 years postoperatively, with only one episode of rejection (type 3A). In June 1992 he died of fulminant complications of AIDS and severe chronic rejection. Neither the patient nor the organ donor belonged to any of the known risk groups for HIV infection; a retrospective analysis revealed that perioperative blood transfusions had been the vectors of transmission.

AIDS-Related Opportunistic Infections↗
PubMed1998

Novel heart diseases requiring transplantation.

Cardiac transplantation represents an extraordinary opportunity to study in vivo the pathology of native disease in the terminal phase. The experience cumulated at the University of Padua in 418 orthotopic cardiac transplant procedures allowed to come across previously unrecognized disease: idiopathic left ventricular aneurysm, non-compacted (spongy) myocardium, restrictive cardiomyopathy. A discrepancy between pre- and post-transplant diagnosis was found in 8% of cases. Endomyocardial biopsy and coronary arteriography should be carried out in each patient with apparently dilated cardiomyopathy before proceeding to heart removal, since alternative therapeutic procedures may be available.

Journal Article↗
PubMed1998

In vivo evidence of apoptosis in arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary heart muscle disease characterized by progressive atrophy of the right ventricular myocardium with fibro-fatty replacement and the risk of electrical instability and sudden death. The disease is often familial and the aetiopathogenesis is still unknown. Recently apoptosis (genetically determined cell death) was postulated to account for progressive loss of myocardium. To establish whether apoptosis is present in ARVC, right ventricular endomyocardial biopsies from 20 patients with clinical and histological diagnosis of ARVC were examined by electron microscopy and terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end-labeling method (TUNEL). Apoptotic index was calculated as the percentage of positive nuclei in sections stained by TUNEL. Cell proliferation activity was also assessed by argyrophilic staining of the nucleolar organizer region (AgNOR) and MIB-1 antibody analysis. Twenty biopsies taken from patients during monitoring of cardiac transplantation (grade 0 rejection) served as control. Occurrence of apoptosis was correlated with clinical history duration and the presence of acute symptoms and signs like angina, pyrexia, erythrocyte sedimentation rate and creatine phosphokinase elevation, as well as ST segment elevation on basal electrocardiogram. Electron microscopy and TUNEL revealed presence of apoptotic myocytes in seven cases (35%) with a mean apoptotic index of 24.4+/-9.8. The remaining 13 patients and all of the 20 controls were negative both at the electron microscopy and TUNEL. Presence of apoptosis appeared to be significantly related to clinical history duration of less than 6 months (P < 0.001) and presence of acute symptoms and signs (P = 0.007). AgNOR staining and MIB-1 antibody analysis ruled out cell proliferation activity. In conclusion, apoptosis is present in endomyocardial biopsies of patients with ARVC, especially in the early symptomatic phase of disease. Myocardial destruction with replacement by fat may be episodic rather than gradual and continuous.

Adult↗
PubMed1997

Morphologic spectrum of primary restrictive cardiomyopathy.

A restrictive hemodynamic profile with left ventricular (LV) end-diastolic volume < 100 ml/m2 and LV end-diastolic pressure > 18 mm Hg, in the absence of endomyocardial, pericardial, and specific cardiomyopathy, is a peculiar feature of primary restrictive cardiomyopathy. From 1985 to 1994, 7 hearts of patients who met the above hemodynamic criteria and underwent endomyocardial biopsy because of heart failure, were studied through gross (5 cardiectomies and 2 autopsies), histologic, and electron microscopic investigations. Ages ranged from 9 to 48 years (mean age 29 +/- 13). Four patients (57%) had a positive family history: 2 for hypertrophic and 2 for restrictive cardiomyopathy. Three patterns were identified in the 7 hearts: (1) pure restrictive form in 4 cases with mass/volume ratio 1.2 +/- 0.5 g/ml, ejection fraction 58 +/- 5%, LV end-diastolic volume 67.5 +/- 12.6 ml/m2, LV end-diastolic pressure 26.7 +/- 3.5 mm Hg; (2) hypertrophic-restrictive form in 2 cases with mass/volume ratio 1.5 +/- 0.07 g/ml, ejection fraction 62 +/- 1%, LV end-diastolic volume 69 +/- 10 ml/m2, LV end-diastolic pressure 30 +/- 7 mm Hg; and (3) mildly dilated restrictive form in 1 case with mass/volume ratio 0.9 g/ml, ejection fraction 25%, LV end-diastolic volume 98 ml/m2, LV end-diastolic pressure 40 mm Hg. Histology and electron microscopy disclosed myocardial and myofibrillar disarray and endoperimysial interstitial fibrosis in each pattern. The familial forms suggest the presence of a genetic abnormality. Primary restrictive cardiomyopathy may present with or without hypertrophy and shares similar microscopic pictures with hypertrophic cardiomyopathy. The 2 entities may represent a different phenotypic expression of the same genetic disease.

Adolescent↗
PubMed1997

Angiographic and morphologic features of the left ventricle in Ebstein's malformation.

Quantitative and qualitative cineangiographic analysis of the left ventricle (LV) was performed in 26 patients with isolated Ebstein's malformation, having a mean age of 23 +/- 17 years. Nine autopsied hearts with isolated Ebstein's malformation were submitted to morphologic and morphometric analysis. In 4 of the cases, it was possible to make a direct correlation between the angiographic data obtained during life and the autopsy findings. On the basis of the LV end-diastolic volume we identified 3 groups of patients: 7 with volume <60 ml/m2, another 7 with volume between 60 and 80 ml/m2, and 12 with volume >80 ml/m2. The LV ejection fraction was reduced in 2 patients with normal LV end-diastolic volume and in 6 with increased LV end-diastolic volume. The ratio of ventricular mass to LV end-diastolic volume was always adequate, but a reduction of the ventricular contractive performance (end-systolic pressure to end-systolic volume ratio <3 mm Hg/ml/m2) was found only in patients with a dilated left ventricle. No correlation was demonstrated between the extent of the atrialized component of the right ventricle (mean value 67 +/- 31 cm2, range 13 to 133) and the LV dimensions. All but 2 patients showed a leftward diastolic displacement of the ventricular septum, but in only 1 did this produce an elongated shape of the left ventricle. Sixteen had anomalies of LV dynamics: 10 with hypokinesia (3 of the posterior wall, 4 of the apex, 1 of the inferior wall, 1 of the septum, and 1 global), 6 with dyskinesia (1 of the posterior wall, 2 of the apex, 1 of the posterior wall and apex, 1 of the superior part of the septum, and 1 of the anterior wall), and 8 with premature diastolic distension of the anterobasal wall. Morphometric analysis produced mean values for myocytes of 59 +/- 10%, for the interstitium of 21 +/- 4%, and for fibrous tissue of 20 +/- 9% (normal 4 +/- 1%). Five autopsied hearts had a prolapsing and/or dysplastic mitral valve.

Adolescent↗
PubMed1997

The elongation of the internal carotid artery: early and long-term results of patients having surgery compared with unoperated controls.

The purposes of this study are to (1) demonstrate the association of elongations of the internal carotid artery (ICA), that is, kinking, coiling, tortuousity, and angulation, and the neurologic symptoms with high stroke risk; (2) compare the results of the surgical treatment versus the medical treatment alone; (3) contribute to the knowledge of the natural history of these anatomical particularities. From January 1992 to December 1994, 113 patients with ICA kinking, coiling, tortuousity, and angulation were randomized either to surgery (group I, n = 55) or not (group II, n = 58). Patients, who presented a carotid hemodynamically significant lesion (>60%) at the origin and associated distal elongation were excluded. The groups were comparable with regard to sex, age, risk factors for atherosclerosis, associated diseases, symptoms and anatomic feature of the contralateral ICA. Follow-up was obtained in all patients: it consisted of clinical evaluation and Duplex scan control at 3-month intervals during the follow-up period (6-36 months; average, 23). Histologic specimens were obtained in all surgically treated arteries. Early results were excellent: in group I, no patient died, no patient presented major or minor stroke. Only one patient had an immediate transient ischemic attack (TIA) which spontaneously recovered within 24 hours. All symptomatic patients examined the complete disappearance of clinical signs. There were no late deaths due to stroke and no late major or minor neurologic deficit occurred. All reconstructed ICAs were patent. In group II, three patients experienced a major stroke with hemiplegia due to ICA occlusion. Most of the symptomatic patients (37) of group II remained stable, while seven of them had worsening of symptoms and were referred for surgery. To conclude, all surgically treated patients had the complete relief of preoperative neurologic symptoms; none of the medically treated patients had an improvement. Although there was no statistically significant difference between the two groups with regard to stroke risk, three medically treated patients progressed to total occlusion. This suggests that kinking, coiling, tortuousity, and angulations of the ICA are not merely an anatomic curiosity but a potentially disabling, even fatal condition.

Adult↗
PubMed1997

Autoimmunity in myocarditis and dilated cardiomyopathy: cardiac autoantibody frequency and clinical correlates in a patient series from Italy.

BACKGROUND: Organ- and disease-specific cardiac autoantibodies, detected by indirect immunofluorescence, represent markers of autoimmunity in a subgroup (25-35%) of patients with dilated cardiomyopathy or myocarditis from Northern Europe and the United States of America. Autoantibody frequencies, as well as associations between clinical and immunological features, may vary in patients from different countries, due to ethnically related differences in genetic susceptibility to autoimmune disease. METHODS: We assessed the frequency of cardiac autoantibodies in a series from Italy, including 91 subjects with idiopathic dilated cardiomyopathy (61 male, aged 49 +/- 11 years) and 11 with biopsy-proven (Dallas criteria) myocarditis (7 male, aged 23 +/- 16), including 2 cases of giant cell myocarditis. Controls were 160 patients with other cardiac disease, 141 with ischemic heart failure and 270 normals Cardiac antibody test was performed blindly by indirect immunofluorescence on normal human myocardium and skeletal muscle. RESULTS: The frequency of organ-specific cardiac autoantibodies was higher (p = 0.0001) in myocarditis (45%) and in dilated cardiomyopathy (20%) than in other cardiac disease (1%), in ischemic heart failure (1%), or in normals (2.5%). Cross-reactive antibodies were detected in similar proportions of study patients and controls. Both patients with giant cell myocarditis were antibody positive. Myocarditis patients with cardiac antibodies had shorter duration of symptoms compared to those who were antibody negative (0.4 +/- 0.3 vs 4 +/- 1 months, p = 0.004). In dilated cardiomyopathy, antibody status was not associated with any clinical or diagnostic feature. CONCLUSIONS: Autoimmunity is involved in a subset of patients with myocarditis and with dilated cardiomyopathy, regardless of their geographical origin or immunogenetic background. The antibody frequency in our dilated cardiomyopathy series from Italy tended to be lower than in other countries. This may reflect reduced antibody levels with disease progression and/or the recognised feature that Mediterranean populations are often less susceptible to autoimmune disease.

Acute Disease↗
PubMed1997

Causes of late failure after heart transplantation: a ten-year survey.

BACKGROUND: Little is known about the causes of death of heart transplant recipients who survive long-term. METHODS: The pathologic and clinical records of 97 patients who underwent heart transplantation in Italy from 1985 to 1995 and died (85 of 97) or underwent retransplantation (12 of 97) at least 2 years after transplantation were surveyed. Graft failures were classified as late (occurring between 2 and 5 years after transplantation) and belated (more than 5 years). RESULTS: Graft vasculopathy was the single most common cause of death (40.0%) and the only cause of late retransplantation. Tumors ranked second (23.5% of deaths), but the expected non-Hodgkin's lymphomas and Kaposi's sarcoma were accompanied by a high number of lung cancers (especially metastasizing adenocarcinomas). They were followed by the emergence or recurrence of pretransplantation diseases (9.4%), fatal infections (exclusively bacterial) (4.7%), the development of transmissible diseases (viral hepatitis and acquired immunodeficiency syndrome, 4.7%), and late acute rejection (2.3%). The distribution of failures differed in the late and belated periods: death and organ loss proportions for graft vasculopathy, respectively, fell and rose from the late to the belated period; some types of malignancy and fatal acute rejection were never observed in the belated period, whereas the emergence of pretransplantation diseases prevailed in the belated period. Graft vasculopathy was more frequent and tumors were less frequent among patients undergoing transplantation for ischemic heart disease. CONCLUSIONS: The reasons why heart transplant recipients die or undergo retransplantation, respectively, in the late and belated periods slightly differ from one another and are widely different than in short-term survivors.

Bacterial Infections↗
PubMed1996

Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis?

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a frequent cause of sudden death in young individuals and athletes. Although familial occurrence has been documented and a gene defect was recently localized on chromosome 14q23-q24 the etiopathogenesis of the disease is still obscure. METHODS AND RESULTS: A pathological study was conducted in 30 hearts with ARVC (age range, 15 to 65 years; mean, 28 years). In the 27 autopsy cases, the mode of death was sudden in 24 and congestive heart failure in 3. ECG, available in 19 cases, showed inverted T waves in the right precordial leads in 15 cases (79%) and ventricular arrhythmias in 15 (79%). Right ventricular aneurysms were present in 15 hearts (50%) and located in the inferior wall in 12. Left ventricle and ventricular septum were involved in 14 (47%) and 6 (20%) cases, respectively. Scattered foci of lymphocytes with myocardial death were observed in 20 cases (67%). Electron microscopy studies, although confirming the myocardial death and lymphocyte infiltrates, did not show any specific ultrastructural substrate. Two pathological patterns, fatty (40%) and fibrofatty (60%), were identified. The fibrofatty pattern was associated with a thinner right ventricular wall (P < .0001) and a higher occurrence of focal myocarditis (P < .001). In sections of right ventricular free wall with maximal fatty infiltration, the mean percentage area of fatty tissue was 35.9 +/- 11.1% in control versus 80.4 +/- 9.6% in the ARVC, fatty variety (P < .00001). Involvement of the left ventricle and/or ventricular septum, right ventricular aneurysms, and inflammation were found almost exclusively in the fibrofatty variety. CONCLUSIONS: In the fibrofatty variety of ARVC, the myocardial atrophy appears to be the consequence of acquired injury (myocyte death) and repair (fibrofatty replacement), mediated by patchy myocarditis. Whether the inflammation is a primary event or a reaction to spontaneous cell death remains unclear.

Adolescent↗
PubMed1996

Spin-echo nuclear magnetic resonance for tissue characterisation in arrhythmogenic right ventricular cardiomyopathy.

OBJECTIVE: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disorder characterised clinically by ventricular arrhythmias that can cause cardiac arrest and morphologically by fatty or fibro-fatty myocardial atrophy of the right ventricle. In vivo tissue characterisation without endomyocardial biopsy would be useful. The aim of this study was to investigate the diagnostic accuracy of spin-echo nuclear magnetic resonance (NMR) for tissue characterisation in ARVC. PATIENTS AND METHODS: Twenty three subjects (15 men and eight women, aged 18-49, mean 34) were studied with spin-echo T1-weighted NMR and multislice scan. Fifteen had a clinical diagnosis of ARVC and eight were controls (age and sex matched subjects). Data were independently evaluated by two expert observers. RESULTS: In the control group NMR was always negative (100% specificity). Ten of the 15 patients with ARVC had an abnormal NMR result (67% sensitivity), with areas that had a signal intensity close to that of pericardial or subcutaneous fat. In the remaining five cases the NMR signal was inadequate. Nine patients underwent both NMR and endomyocardial biopsy; biopsy was positive in eight (89%) and NMR was positive in five (56%). CONCLUSIONS: NMR is a useful non-invasive diagnostic tool in the evaluation of fatty replacement in ARVC. The technique can be used with other procedures in the initial diagnostic evaluation and is a useful alternative tool in the long term follow up of patients with ARVC.

Adolescent↗
PubMed1996

Evolution of focal moderate (International Society for Heart and Lung Transplantation grade 2) rejection of the cardiac allograft.

BACKGROUND: In the absence of clinical evidence of cardiac allograft rejection, it is still poorly defined whether the International Society for Heart and Lung Transplantation biopsy grade 2 (e.g., focal moderate rejection) should be treated. Aim of the present study was to retrospectively investigate the evolution of focal moderate rejection, diagnosed during the first postoperative year in patients who had undergone orthotopic heart transplantation. METHODS: A retrospective analysis was conducted on 256 International Society for Heart and Lung Transplantation grade 2 biopsies from 110 orthotopic heart transplantations; 125 episodes occurred within the first 3 months (group 1), 131 later (group 2). For each grade 2 diagnosis, two biopsies, one immediately before and one after, were analyzed and classified as follows: lower (grade 0 or 1), equal (grade 2), or higher (grade 3 or 4). RESULTS: Evolution of grade 2 rejection was to a lower grade in 66% of cases, an equal grade in 16.8%, and a higher grade in 17.2%, with differences between group 1 and 2 (higher: 25% versus 10%, respectively, p = 0.005). Episodes which progressed into higher grades occurred earlier compared with those which persisted or resolved (9.2 +/- 8.6 weeks versus 20.0 +/- 15.6, p < 0.001). Five-year actuarial survival and incidence of graft coronary disease were similar in patients whose conditions progressed and those whose conditions did not. However, left ventricular ejection fraction at 1 and 2 years was lower in patients whose conditions progressed compared with those whose conditions persisted or resolved (56% +/- 4% versus 66% +/- 2%, p = 0.004; 56% +/- 10% versus 64% +/- 8%, p = 0.02, respectively). CONCLUSIONS: Progression of grade 2 rejection occurred in a minority of cases and did not affect 5-year survival or incidence of coronary disease, but its relationship with long-term cardiac allograft dysfunction warrants further investigation.

Actuarial Analysis↗
PubMed1995

Arrhythmogenic right ventricular cardiomyopathy in young versus adult patients: similarities and differences.

OBJECTIVES: This study was designed to evaluate and compare the patterns of arrhythmogenic right ventricular cardiomyopathy in young people and adults. BACKGROUND: Few data are available on this cardiomyopathy in young people because clinical and morphologic findings considered pathognomonic are normally based on observations in adults. However, a familial occurrence with a probable genetic transmission led to the study of children and to early detection of this disease, in which sudden death has been reported even in young people. METHODS: Seventeen young patients with arrhythmogenic right ventricular cardiomyopathy diagnosed at a mean age +/- SD of 14.9 +/- 4.9 years were studied. Clinical, electrocardiographic, echocardiographic, cineangiographic and biopsy findings were compared with those of 19 adult patients whose condition was diagnosed at a mean age of 38.1 +/- 13.4 years. RESULTS: Syncope occurred in 23.5% of the young patients but in none of the adults (odds ratio of familial sudden death 5.54, p = 0.1). Ventricular couplets (odds ratio 16.0, p = 0.004) and subtricuspid bulging on echocardiography (odds ratio 5.95, p = 0.036) were prevalent in the young group. Cineangiographic data in the two groups were similar, except that more hypokinetic areas were found in adults (odds ratio 4.44, p = 0.05). Morphometric analysis of biopsy sections showed a greater amount of fibrous tissue in the young patients (p = 0.04) and a prevalence of fatty tissue in the adults (odds ratio 12, p = 0.005). During an equivalent follow-up time (mean 7 years), two young patients died suddenly, and two had ventricular fibrillation in the absence of antiarrhythmic therapy. CONCLUSIONS: The pathognomonic criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in adults are also valid for young people. Sudden or aborted death occurred frequently in young untreated patients.

Adolescent↗