PubMed Health⌕ Search

Biomedical subjects

Maurizio Pieroni

Publications and source records attributed to Maurizio Pieroni.

26 records · Page 2Linked to original sources
PubMed2004

Clozapine-induced hypersensitivity myocarditis.

A rare, but frequently fatal, side effect of the antipsychotic drug clozapine is myocarditis. We report a case of hypersensitivity myocarditis secondary to clozapine administration that was diagnosed in vivo for the first time through endomyocardial biopsy and was successfully treated with corticosteroids. Histologic diagnosis was based on the evidence of eosinophilic infiltration of the endomyocardium and eosinophil degranulation. Endomyocardial biopsy was performed in order to establish or exclude a clear-cut relationship between cardiac dysfunction and clozapine, and was crucial to establish a correct diagnosis and appropriate treatment. Clozapine withdrawal and targeted 8-day, low-dose corticosteroid therapy resolved the symptoms and restored cardiac function.

Clozapine↗
PubMed2003

Hypertrophic cardiomyopathy: two homozygous cases with "typical" hypertrophic cardiomyopathy and three new mutations in cases with progression to dilated cardiomyopathy.

About 10% of cases of hypertrophic cardiomyopathy (HCM) evolve into dilated cardiomyopathy (DCM) with unknown causes. We studied 11 unrelated patients (pts) with HCM who progressed to DCM (group A) and 11 who showed "typical" HCM (group B). Mutational analysis of the beta-myosin heavy chain (MYH7), myosin-binding protein C (MYBPC3), and cardiac troponin T (TNNT2) genes demonstrated eight mutations affecting MYH7 or MYBPC3 gene, five of which were new mutations. In group A-pts, the first new mutation occurred in the myosin head-rod junction and the second occurred in the light chain-binding site. The third new mutation leads to a MYBPC3 lacking titin and myosin binding sites. In group B, two pts with severe HCM carried two homozygous MYBPC3 mutations and one with moderate hypertrophy was a compound heterozygous for MYBPC3 gene. We identified five unreported mutations, potentially "malignant" defects as for the associated phenotypes, but no specific mutations of HCM/DCM.

Adult↗
PubMed2003

Early detection of Fabry cardiomyopathy by tissue Doppler imaging.

BACKGROUND: Fabry cardiomyopathy is diagnosed by detection of left ventricular hypertrophy (LVH) in patients with alpha-Galactosidase A deficiency. Conventional noninvasive tools are unable to provide a preclinical diagnosis allowing prompt institution of enzymatic therapy. METHODS AND RESULTS: We studied three groups of patients: 10 patients with causal mutations for Fabry disease and LVH, 10 mutation-positive patients without LV, and 10 healthy relatives without causal mutations and no LVH. All patients with LVH and 6 patients with Fabry disease without LVH with complex repetitive ventricular arrhythmias underwent biventricular endomyocardial biopsy to assess cardiac involvement. In all patients 2-dimensional echocardiography with tissue Doppler analysis in the pulsed Doppler mode was performed: systolic (Sa), early diastolic (Ea), and late diastolic (Aa) velocities were measured, and the Ea/Aa ratio and the dimensionless parameter E/Ea were computed at both corners of the mitral annulus. Histology and electron microscopy studies showed glycosphingolipid deposits in all cases. All mutation-positive patients had significant reduction of Sa, Ea, and Aa velocities at both corners of the mitral annulus compared with normal control subjects. Ea/Aa ratio was significantly lower and E/Ea ratio significantly higher in mutation-positive patients than in control subjects. Patients with LVH showed significantly lower contraction and relaxation tissue Doppler velocities, lower Ea/Aa ratio, and higher E/Ea ratio in comparison with mutation-positive patients with no LVH. CONCLUSIONS: Fabry cardiomyopathy is characterized by reduced myocardial contraction and relaxation tissue Doppler velocities, detectable even before development of LVH. Tissue Doppler imaging can provide a preclinical diagnosis of Fabry cardiomyopathy, allowing early institution of enzyme replacement therapy.

Adult↗
PubMed2003

Immunosuppressive therapy for active lymphocytic myocarditis: virological and immunologic profile of responders versus nonresponders.

BACKGROUND: The beneficial effect of immunosuppressive treatment on myocarditis is still controversial, possibly because the immunologic and virological profile of potential candidates is largely unknown. METHODS AND RESULTS: Out of 652 biopsied patients, 112 had a histological diagnosis of active lymphocytic myocarditis; 41 of these 112 patients were characterized by progressive heart failure despite conventional therapy and were treated with prednisone and azathioprine for 6 months. All were resubmitted to cardiac catheterization, angiography, and endomyocardial biopsy at 1 and 6 months and followed-up for 1 year. A total of 21 patients responded with prompt improvement in left ventricular ejection fraction from 25.7+/-4.1% to 47.1+/-4.4% and showed evidence of healed myocarditis at control biopsy. Conversely, 20 patients failed to respond and showed a histological evolution toward dilated cardiomyopathy: 12 remained stationary, 3 underwent cardiac transplantation, and 5 died. We retrospectively performed a polymerase chain reaction on frozen endomyocardial tissue for the most common cardiotropic viruses and assessed circulating serum cardiac autoantibodies. Viral genomes were present in biopsy specimens of 17 nonresponders (85%), including enterovirus (n=5), Epstein-Barr virus (n=5) adenovirus (n=4), both adenovirus and enterovirus (n=1), influenza A virus (n=1), parvovirus-B19 (n=1), and in 3 responders, who were all positive for hepatitis C virus. Cardiac autoantibodies were present in 19 responders (90%) and in none of the nonresponders. CONCLUSIONS: In patients with active lymphocytic myocarditis, those with circulating cardiac autoantibodies and no viral genome in the myocardium are the most likely to benefit from immunosuppression. The beneficial effect of immunosuppression in hepatitis C virus myocarditis suggests a relevant immunomediated component of damage.

Adolescent↗
PubMed2002

Celiac disease associated with autoimmune myocarditis.

BACKGROUND: Both celiac disease (CD) and myocarditis can be associated with systemic autoimmune disorders; however, the coexistence of the 2 entities has never been investigated, although its identification may have a clinical impact. METHODS AND RESULTS: We screened the serum of 187 consecutive patients with myocarditis (118 males and 69 females, mean age 41.7+/-14.3 years) for the presence of cardiac autoantibodies, anti-tissue transglutaminase (IgA-tTG), and anti-endomysial antibodies (AEAs). IgA-tTG-positive and AEA-positive patients underwent duodenal endoscopy and biopsy and HLA analysis. Thirteen of the 187 patients were positive for IgA-tTG, and 9 (4.4%) of them were positive for AEA. These 9 patients had iron-deficient anemia and exhibited duodenal endoscopic and histological evidence of CD. CD was observed in 1 (0.3%) of 306 normal controls (P<0.003). In CD patients, myocarditis was associated with heart failure in 5 patients and with ventricular arrhythmias (Lown class III-IVa) in 4 patients. From histological examination, a lymphocytic infiltrate was determined to be present in 8 patients, and giant cell myocarditis was found in 1 patient; circulating cardiac autoantibodies were positive and myocardial viral genomes were negative in all patients. HLA of the patients with CD and myocarditis was DQ2-DR3 in 8 patients and DQ2-DR5(11)/DR7 in 1 patient. The 5 patients with myocarditis and heart failure received immunosuppression and a gluten-free diet, which elicited recovery of cardiac volumes and function. The 4 patients with arrhythmia, after being put on a gluten-free diet alone, showed improvement in the arrhythmia (Lown class I). CONCLUSIONS: A common autoimmune process toward antigenic components of the myocardium and small bowel can be found in >4% of the patients with myocarditis. In these patients, immunosuppression and a gluten-free diet can be effective therapeutic options.

Adolescent↗
PubMed2002

Lone hepatitis C virus myocarditis responsive to immunosuppressive therapy.

OBJECTIVES: This study analyzes the causal role of hepatitis C virus (HCV) in patients with lone myocarditis, and its susceptibility to immunosuppression. BACKGROUND: Prevalence of HCV in lone myocarditis, its mechanism of damage, and possible treatment are still unknown. METHODS: Among 48 consecutive patients with myocarditis serologically screened for HCV and other cardiotropic viruses, 3 patients had anti-HCV antibodies. Clinical manifestation was heart failure in two cases, and left bundle-branch block with moderate cardiac dysfunction was present in patient 3. The three patients underwent two-dimensional echocardiography, coronary angiography, and endomyocardial biopsy. Nested polymerase chain reaction (PCR) for positive and negative strands of HCV on sera and myocardial samples, and PCR for the most common cardiotropic viruses were performed. HCV in the myocardium was detected by TORDJI-22 antibody. RESULTS: At histology, a lymphocytic myocarditis associated with myocytes positively stained by TORDJI-22 was shown in all. Cardiac autoantibodies were detected in all cases. Nested PCR showed both positive and negative strands of HCV RNA in serum and myocardium; other viral genomes were absent. Patients were treated with prednisone and azathioprine for 6 months, with recovery of cardiac volumes and function. At 4-week control biopsy, myocarditis progressed to a healed phase, though HCV RNA was still detectable in the serum and myocardium. Cardiac improvement was maintained at the 12-month overall follow-up. CONCLUSIONS: HCV can be detected in the myocardium of as many as 6% of patients with lone myocarditis; HCV myocarditis can benefit from immunosuppression despite persistence of viral genome, suggesting an immunomediated mechanism of damage.

Adult↗
PubMed2002

The role of endomyocardial biopsy in the diagnosis of cardiomyopathies.

Though many publications in the field of myocarditis and cardiomyopathies have renewed interest in the value of endomyocardial biopsy, its role in the work-up of patients with cardiomyopathies, idiopathic arrhythmias and even ischemic heart disease, is still debated. Since its introduction in 1963 the technique has been developed with current routine use of a biventricular and even atrial approach. At the same time immunohistochemistry and molecular biology studies have greatly enhanced the information obtainable from myocardial samples. The authors report their experience and briefly review the pertinent literature on the current use of endomyocardial biopsy in the diagnosis and treatment of dilated, hypertrophic and restrictive cardiomyopathy, of idiopathic arrhythmias and in ischemic heart disease.

Arrhythmias, Cardiac↗