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Biomedical subjects

A Frustaci

Publications and source records attributed to A Frustaci.

At least 37 records · Page 2Linked to original sources
PubMed1998

Necrotizing myocardial vasculitis in Churg-Strauss syndrome: clinicohistologic evaluation of steroids and immunosuppressive therapy.

Treatment of cardiac dysfunction associated with Churg-Strauss syndrome (CSS) is empiric since the histologic findings provided by endomyocardial biopsy are rare and often nondiagnostic. Myocardial necrotizing vasculitis presenting as restrictive cardiomyopathy has not been reported before. A case of CSS, presenting with fever and progressive heart failure due to pericarditis, eosinophilic endomyocarditis, and myocardial necrotizing vasculitis, is reported. Cardiac involvement assessed by noninvasive (cardiac two-dimensional echocardiogram and nuclear magnetic resonance [NMR] imaging) and invasive (cardiac catheterization, angiography, and biopsy) studies showed a moderate degree of pericardial effusion and left ventricular (LV) dysfunction (ejection fraction 0.40), severe diastolic dysfunction (increased right and LV filling pressure with a dip and plateau pattern) and a severe reduction of cardiac index (1.6 L/min/m2). Histologic characteristics showed marked eosinophilic infiltration of the endocardium and myocardium with myocitolysis and fibrinoid necrosis of arterioles, venules, and capillaries. Combination therapy of steroids and cyclophosphamide resulted in both a clinical (regression of pericardial effusion, normalization of systolic and diastolic dysfunction, and increase of cardiac index to 2.8 L/min/m2) and histologic (sequential endomyocardial biopsies at 1, 3, and 6 months of follow-up) resolution of cardiac involvement. No recurrences were registered at 12-month follow-up with the patient receiving a maintenance drug regimen.

Cardiomyopathies↗
PubMed1997

Histological substrate of atrial biopsies in patients with lone atrial fibrillation.

BACKGROUND: Lone atrial fibrillation (LAF) is a common clinical syndrome, but its origin remains unknown. METHODS AND RESULTS: We performed endomyocardial biopsies of the right atrial septum (2 to 3 per patient; mean, 2.8) and of the two ventricles (6 per patient) in 12 patients (10 men, 2 women; mean age, 32 years) with paroxysmal LAF refractory to conventional antiarrhythmic treatment. As controls, we used endomyocardial biopsies (3 to 5 per patient; mean, 4.4) from the right atrial septum of 11 patients with Wolff-Parkinson-White syndrome (WPW) undergoing resection of the abnormal AV pathway. The weight of the biopsies ranged from 2.8 to 4.5 mg. Biopsy samples were processed for histology and electron microscopy and were read by a pathologist blinded to clinical data. All patients underwent two-dimensional Doppler echocardiography; cardiac catheterization; coronary angiography; and hormonal, virologic, and electrophysiological studies. All tests and WPW biopsies were normal, but all LAF atrial biopsy specimens (average, 2.8 per patient) showed abnormalities (P<.0001). The type of abnormalities varied: Two patients had a severe hypertrophy with vacuolar degeneration of the atrial myocytes and ultrastructural evidence of fibrillolysis occupying >50% of the areas assessed morphometrically (P=.50), 8 had lymphomononuclear infiltrates with necrosis of the adjacent myocytes (5 with fibrosis and 3 without; P<.003), and 2 had only nonspecific patchy fibrosis (P=.50). Biventricular biopsies were abnormal in only 3 patients and showed inflammatory infiltrates similar to those found in atrial biopsies. CONCLUSIONS: Abnormal atrial histology was uniformly found in multiple biopsy specimens in all patients with LAF. It was compatible with a diagnosis of myocarditis in 66% of patients (active in 25%) and of noninflammatory localized cardiomyopathy in 17% and was represented by patchy fibrosis in 17%. The cause of the pathological changes, which were found only in atrial septal biopsies but not in biventricular biopsies, in 75% of patients remains unknown.

Adult↗
PubMed1997

Brief report: healed myocarditis as a cause of ventricular repolarization abnormalities in athlete's heart.

In the past myocarditis has been suggested as a possible cause of repolarization abnormalities in sportsmen, but, to our knowledge, no direct in-vivo demonstration of this relationship has so far been found. We report the cases of three professional athletes with repolarization changes at rest and/or during exercise and mild segmental wall motion anomalies in the left ventricle on echocardiography, in whom myocarditis was diagnosed by non-invasive and invasive clinical investigations, including endomyocardial biopsy. We think that probably the frequency with which myocarditis is responsible for electrocardiographic and echocardiographic abnormalities in athletes has so far been underestimated, and that caution must be employed when interpreting minor segmental wall motion abnormalities on resting and exercise echocardiograms in trained subjects as being due to athlete's hart, especially when they present with repolarization changes.

Adult↗
PubMed1997

Idiopathic myocardial vasculitis presenting as restrictive cardiomyopathy.

A previously unreported case of small-vessel myocardial vasculitis presenting as restrictive cardiomyopathy and congestive heart failure is described. The hemodynamic study, showing severely increased and equalized diastolic pressures in atrial and ventricular chambers, and cardiac MRI, showing normal pericardium and ventricular endomyocardial biopsy, not including myocardial vascular component, were insufficient to make a diagnosis. This made a thoracotomy and surgical cardiac biopsy necessary. Steroids and cyclophosphamide, introduced after histologic evidence of necrotizing vasculitis, unassociated with a systemic disease, became available and improved the clinical profile and the diastolic dysfunction at two-dimensional echocardiographic Doppler analysis.

Alkylating Agents↗
PubMed1996

Paradoxical inhibition of cardiac lipid peroxidation in cancer patients treated with doxorubicin. Pharmacologic and molecular reappraisal of anthracycline cardiotoxicity.

Anticancer therapy with doxorubicin (DOX) and other quinone anthracyclines is limited by severe cardiotoxicity, reportedly because semiquinone metabolites delocalize Fe(II) from ferritin and generate hydrogen peroxide, thereby promoting hydroxyl radical formation and lipid peroxidation. Cardioprotective interventions with antioxidants or chelators have nevertheless produced conflicting results. To investigate the role and mechanism(s) of cardiac lipid peroxidation in a clinical setting, we measured lipid conjugated dienes (CD) and hydroperoxides in blood plasma samples from the coronary sinus and femoral artery of nine cancer patients undergoing intravenous treatments with DOX. Before treatment, CD were unexpectedly higher in coronary sinus than in femoral artery (342 +/- 131 vs 112 +/- 44 nmol/ml, mean +/- SD; P < 0.01), showing that cardiac tissues were spontaneously involved in lipid peroxidation. This was not observed in ten patients undergoing cardiac catheterization for the diagnosis of arrhythmias or valvular dysfunctions, indicating that myocardial lipid peroxidation was specifically increased by the presence of cancer. The infusion of a standard dose of 60 mg DOX/m(2) rapidly ( approximately 5 min) abolished the difference in CD levels between coronary sinus and femoral artery (134 +/- 95 vs 112 +/- 37 nmol/ml); moreover, dose fractionation studies showed that cardiac release of CD and hydroperoxides decreased by approximately 80% in response to the infusion of as little as 13 mg DOX/m(2). Thus, DOX appeared to inhibit cardiac lipid peroxidation in a rather potent manner. Corollary in vitro experiments were performed using myocardial biopsies from patients undergoing aortocoronary bypass grafting. These experiments suggested that the spontaneous exacerbation of lipid peroxidation probably involved preexisting Fe(II) complexes, which could not be sequestered adequately by cardiac isoferritins and became redox inactive when hydrogen peroxide was included to simulate DOX metabolism and hydroxyl radical formation. Collectively, these in vitro and in vivo studies provide novel evidence for a possible inhibition of cardiac lipid peroxidation in DOX-treated patients. Other processes might therefore contribute to the cardiotoxicity of DOX.

Adenosine Diphosphate↗
PubMed1996

Acute myocarditis and left ventricular aneurysm as presentations of systemic lupus erythematosus.

A case of systemic lupus erythematosus (SLE) associated with fever, heart failure, and left ventricular (LV) aneurysm is reported. A diagnosis of SLE was suspected owing to the presence of active lymphocytic myocarditis and fibrinous endocarditis at LV endomyocardial biopsy and was confirmed by identification of 4 of the 11 criteria proposed by the American Rheumatism Association for the definition of SLE. A 2-month period of steroid therapy was followed by a remarkable recovery of LV function and progression of endomyocarditis to a healed phase at control LV biopsy. The LV aneurysm disappeared, likely because thrombosis occurred as a result of the hypercoagulable state accompanying the presence of anticardiolipin antibodies. This is the first reported case of LV aneurysm induced by SLE and is a rare clinicohistologic documentation of the effectiveness of steroid treatment on lupus endomyocarditis.

Acute Disease↗
PubMed1996

Recurrent perimyocarditis following a non penetrating chest trauma. A case report.

A young man, after a non penetrating chest trauma, developed recurrent episodes of fever, chest pain, pleural and pericardial effusion, without laboratory evidence of viral infections, or positivity for conventional autoimmunity markers. A clearcut positivity for more specific cardiac autoantibodies, against Beta 1 adrenoceptors (AB1AA), was found (at all dilutions from 1:20 to 1:160). A full dosage of Prednisone rapidly relieved all symptoms, whereas antibiotic therapy had been previously uneffective. At a follow-up control after three months, the patient was healed and AB1AA were positive only at dilutions 1:20 and 1:40. As silent viral myocarditis was apparently ruled out by serological negativity for viral infections, it is possible that autoimmunity could have played a primary pathogenetic role for the development of pericarditis in this patient. Further work is needed to ascertain whether or not AB1AA detection could be a specific marker of cardiac autoimmunity phenomena.

Adult↗
PubMed1995

A novel variant of transthyretin, 59Thr-->Lys, associated with autosomal dominant cardiac amyloidosis in an Italian family.

BACKGROUND: Amyloidosis is a disorder of protein metabolism characterized by extracellular accumulation of abnormal protein fibrils. Different proteins form the fibrils in different forms of the disease, and the condition can be acquired or hereditary. Involvement of the heart is quite common, producing a serious and usually fatal cardiomyopathy. Cardiac amyloidosis is often diagnosed late, and cardiac biopsy together with proper histological examination is essential. Contrary to previous perceptions, there is much recent evidence of effective treatment for several different types of systemic and cardiac amyloidosis, including the most common hereditary form caused by mutations in the transthyretin gene. Chemical and genetic typing of amyloid is therefore of considerable clinical importance. METHODS AND RESULTS: Seven members in two generations of an Italian family presented with cardiac disease inherited as an autosomal dominant and were found to have systemic amyloidosis. Angina pectoris-like pain, an unusual feature in cardiac amyloidosis, was a prominent symptom, possibly related to partial obliteration of the distal coronary arteries by amyloid infiltration. There were also cases of sudden cardiac death. Peripheral and autonomic neuropathy, which are the usual features of hereditary amyloidosis, were present in only two cases, and a diagnosis of acquired, immunoglobulin light chain (AL type) amyloidosis was suspected in the index case before the family history emerged. In fact, the amyloid fibrils were composed of transthyretin, and the two affected individuals from whom DNA was available were both heterozygotes for a single base change in exon 3 of the transthyretin gene, encoding substitution of Lys for the wild-type Thr residue at position 59 in the mature protein. This mutation has not previously been reported. CONCLUSIONS: We have identified a novel mutation in the transthyretin gene encoding 59Thr-->Lys associated with autosomal dominant hereditary systemic amyloidosis in an Italian kindred in whom cardiac involvement was the major feature. This family illustrates the difficulty in diagnosis of cardiac amyloid, the variable clinical phenotype in hereditary amyloidosis even within a family, and the importance of precise fibril typing for correct management in this condition.

Amyloidosis↗
PubMed1995

Biopsy evidence of atrial myocarditis in an athlete developing transient sinoatrial disease.

Atrial myocarditis causing transient sinoatrial disease (incessant atrial tachycardia alternating with sinoatrial pauses of up to 6 s in duration) in an athlete is reported. Diagnosis was undertaken by endomyocardial biopsy; biventricular and right atrial specimens were obtained. After a 6-month rest period, the atrial arrhythmias disappeared, and the athlete was able to resume his professional sporting activities.

Adult↗
PubMed1994

Results of biventricular endomyocardial biopsy in survivors of cardiac arrest with apparently normal hearts.

Seventeen young patients (10 males and 7 females, aged 14 to 38 years, mean 26.4) without overt organic heart disease, who had been resuscitated from sudden cardiac arrest and referred to our institution during the period 1984 to 1993 for diagnostic evaluation and electrophysiologic study-guided antiarrhythmic therapy, were studied. Patients underwent noninvasive (electrocardiography, echocardiography [2-dimensional and Doppler], and magnetic resonance imaging) and invasive (left ventricular [LV], right ventricular [RV], and coronary angiography, ergonovine testing, electrophysiologic study, and biventricular endomyocardial biopsy) cardiac studies. Six to 8 biopsy fragments per patient were processed for histology and electron microscopy and read by a pathologist blinded to clinical data. Antiarrhythmic drug testing included amiodarone, propafenone, and metoprolol. A cardioverter-defibrillator was implanted in patients with persistently inducible sustained ventricular tachycardia or ventricular fibrillation. Sequential cardiac biopsy specimens were obtained in patients with active myocarditis undergoing immunosuppressive treatment. Periodic 3-month follow-ups included echocardiography and Holter monitoring. Two groups of patients were distinguished by invasive and noninvasive examinations: group I consisted of 9 patients with entirely normal parameters; group II consisted of 8 patients with structural, nonspecific cardiac abnormalities. In this latter group, mild to moderate dilatation and hypokinesia of the left ventricle were documented in 4 patients, concentric LV hypertrophy was seen in three patients, and RV dysfunction was noted in 1 patient. Histologic examination was abnormal in in all patients and revealed specific lesions in 65% of them; LV biopsy specimens allowed a diagnosis in 3 of 7 myocarditic patients with normal RV histology.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1994

Role of myocarditis in athletes with minor arrhythmias and/or echocardiographic abnormalities.

We report the clinical and instrumental data, including the endomyocardial biopsy findings, of six young athletes presenting with minor arrhythmias and/or echocardiographic abnormalities. In one of them, a left ventricular dilation with moderate depression of the systolic function had been attributed to an athlete's heart. A diagnosis of arrhythmogenic right ventricular dysplasia had been made in three others, one with right ventricular dilation and apical hypokinesia, and two with ventricular arrhythmias with QRS morphology of left bundle branch block. A myocarditis could be unequivocally established in four athletes (two with and two without fibrosis). In the remaining two, with a clinical history strongly suggesting a previously acute myocarditis, the endomyocardial biopsy specimen revealed a nonspecific fibrosis compatible but not definitely pathognomonic of a healed myocarditis. Our report suggests that a myocarditis may be a cause of minor rhythm disturbances and/or echocardiographic abnormalities in athletes. A prevalent localization of the inflammatory process in the right ventricle with or without the occurrence of ventricular arrhythmias with left bundle branch block morphology can mimic an arrhythmogenic right ventricular dysplasia. An early diagnosis of myocarditis in athletes is useful to avoid the risk of fatal arrhythmias, also considering that rest still keeps on being one of the most effective strategies in myocarditis management.

Adolescent↗
PubMed1993

Cardiomyopathy may be the only clinical manifestation in female carriers of Duchenne muscular dystrophy.

Cardiomyopathy was reported in a few Duchenne muscular dystrophy (DMD) carriers with clinical evidence of myopathy. We report two carriers with dilated cardiomyopathy, increased serum CK, and no symptoms of muscle weakness. In heart biopsies of both patients, dystrophin-the protein product of DMD locus--was absent in many fibers. Dilated cardiomyopathy may be the only manifestation of dystrophin gene mutation in carriers.

Adult↗
PubMed1992

Reversible dilated cardiomyopathy due to growth hormone deficiency.

An unusual case of severe dilated cardiomyopathy is described in a woman with postpartum hypopituitary function, which was characterized by marked decrease of cardiac myofibrils (decreased ratio of myofibrillar volume:cell volume) and somatomedin C deficiency. The patient was treated with a daily intramuscular dose of 4 UI of recombinant human growth hormone for 3 months. Clinical and laboratory assessment and follow-up consisted of noninvasive (echocardiograms, Doppler study, and clinical laboratory tests) and invasive procedures (angiography and endomyocardial biopsy). Morphologic study included optical and electron microscopic examinations and morphometric analysis. The patient exhibited a relevant improvement of cardiac function after recombinant human growth hormone administration. The clinical improvement was confirmed by normalization of electrocardiographic voltages, increased myocardial mass, angiography, and optical and electron microscopic examination, which showed a dramatic recovery of myofibrillar content in myocardiocytes. Furthermore, withdrawal of growth hormone was followed by reduction of electrocardiographic voltages and myocardial mass as well as by impairment of left ventricular function. The authors suggest that there are forms of dilated cardiomyopathy that may benefit dramatically by growth hormone administration. These forms should be identified by growth hormone/somatomedin C axis deficiency and by decrease in myofibrillar content at endomyocardial biopsy.

Cardiomyopathy, Dilated↗