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Biomedical subjects

G M Boffa

Publications and source records attributed to G M Boffa.

At least 19 recordsLinked to original sources
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

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

[Improvement of short-term prognosis in patients with refractory heart failure].

BACKGROUND: Prognosis of patients with severe heart failure is poor, despite improved results in medical therapy. Heart transplantation is the only treatment possible in end-stage heart failure. The aim of this study was to evaluate the variation in prognosis over the past six years in the patients admitted to Intensive Care Unit for heart failure in spite of optimal oral therapy. STUDY POPULATION AND METHOD: Between January 1990 and December 1995, 133 patients with heart failure were admitted to the Intensive Care Unit, despite the fact that they were on optimal oral therapy. All patients were in New York Heart Association (NYHA) functional class III to IV and required intravenous administration of sympathomimetic amines, in addition to standard heart failure treatment procedures. Cumulative survival at six months of patients observed between 1990-1992 (group A) was compared with the survival rate of patients observed from 1993 to 1995 (group B). RESULTS: Clinical and haemodynamic parameters were similar in groups A and B, but ACE-inhibitors were used more frequently in group B (75 vs 31% respectively, p < 0.05). During the follow-up period, heart transplantation was indicated in a similar percentage of patients (A 53% vs B 58%). However, mortality on the waiting list (58% group A vs 21% group B; p < 0.05) and the percentage of patients who underwent heart transplantation (41% group A vs 78% group B; p < 0.05) differed. Moreover, all patients in group A and 50% of group B were operated on as "status one" patients. The total six-month mortality rate decreased from 69% before 1992 to 48% thereafter (p < 0.05). CONCLUSION: The short-term prognosis of patients with refractory heart failure improved over time. In the latter period, ACE-inhibitors were used more frequently and the number of heart transplantations was greater. Nevertheless, our results do not allow us to identify the causes of the improved survival rate.

Adult↗
PubMed1995

Mid-term results of heart transplantation for end-stage valvular disease.

The results of heart transplantation in a consecutive series of 19 end-stage valvular disease patients (15 male, aged 48 +/- 8 years) were analysed retrospectively. The indication for heart transplantation was heart failure due to left and/or right ventricular dysfunction; 16 patients had undergone previous valve surgery, but prosthetic dysfunction was present in only one patient. All patients were in NYHA class IV, 15 were on oral anticoagulants and 5 on i.v. catecholamine support. There were four in-hospital deaths (21%) and one late death (5%), resulting in a 1-year mortality rate slightly, but not significantly, higher than that of patients transplanted for other indications (16%). Mean follow-up of the survivors was 48 +/- 33 months (range 9-95); 5-year actuarial survival was similar to that observed among the other heart transplantation patients (74 +/- 10% vs 77 +/- 3%, P = NS). The incidence of acute rejection and infection was also similar in valvular disease and non-valvular disease patients. Kidney and liver function at 1 year post-heart transplantation was preserved in all cases; cardiac catheterization revealed normal graft function in all patients and the absence of coronary lesions in all but two. In conclusion, in our limited experience morbidity and mortality in patients transplanted for end-stage valvular disease seem to be similar to those of patients undergoing heart transplantation for other etiologies.

Adult↗
PubMed1995

[Analysis of risk factors in a group of patients referred for heart transplantation for refractory heart failure: multivariate analysis].

BACKGROUND: In the absence of evident clinical controindication heart transplantation can be performed in patients with critical degree of cardiac failure. The choice of adequate candidates for transplantation is frequently based on clinical severness but also influenced by factors as body size, blood group and so on. METHODS: To identify the presence of prognostic factors for patients with high risk of death, clinical, hemodynamic and therapeutic features of 70 patients (mean age 44.8 +/- 13.8) with critical cardiac failure (64 patients in IV class N.Y.H.A.) have been reviewed. Most of them were affected by dilated or ischemic cardiomyopathy. RESULTS: All patients were admitted to Cardiac Intensive Care Unit, 13 patients received transplantation, 24 were discharged and 33 died. Univariate analysis evidenced, that in the group of patients who died, sepsis (p = 0.0003), renal (p = 0.04) and liver function impairment (P = 0.007) occurred significantly more frequent. The differentiated use of catecholamines (P = 0.0007) and of vasodilators (P = 0.007), the need of mechanical ventilation (P = 0.0001) and the need of hemodialysis (P = 0.02) resulted significantly higher in the group of dead patients where mean arterial pressure (p = 0.01) and mean systemic resistances (P = 0.04) resulted significantly lower. With multivariate analysis were identified following independent risk factors of death: male sex, infections, mechanical ventilation, differentiated use of vasodilators, mean arterial pressure and mean pressure in pulmonary artery. CONCLUSIONS: As predictive risk factors of death in patients with critical cardiac failure and awaiting transplantation resulted: sex, the presence of infection, mechanical ventilation and differentiated use of vasodilators whereas hemodynamic parameters did not result of important predictive value.

Adult↗
PubMed1994

Mid-term results of heart transplantation in diabetic patients.

BACKGROUND: Diabetes mellitus (DM) has been considered a relative contraindication to heart transplantation (HTx) because of possible increased infection rates and worsening DM with prednisone immunosuppression. The introduction of cyclosporine has allowed us to reduce the dosage of oral steroids and to accept DM patients (pts) for HTx. We reviewed the mid-term outcome of our DM pts who had undergone HTx. MATERIALS AND METHODS: Out of 257 HTx recipients, 19 males and 3 females, aged 53 +/- 8 years, were diabetic. Prior to HTx, DM was controlled with insulin in 5, with oral hypoglycemic agents in 7 and with diet in the remaining patients. Immunosuppression consisted of cyclosporine and azathioprine; low-dose prednisone was added in 10 patients because of repeated rejections. RESULTS: All patients survived HTx and were followed 2 to 75 (mean 38 +/- 21) months. There were 4 late deaths, with actuarial survival of 84% at 5 years. DM was more difficult to control after HTx (12 patients requiring insulin vs 5 pre-HTx), but comparison of diabetic vs non diabetic patients following HTx showed no significant difference in frequency of rejection or infection during the 1st year (2.6 +/- 1.6 vs 2.1 +/- 1.5; 0.5 +/- 0.4 vs 0.4 +/- 0.3 episode/patient respectively) and in 5-year actuarial survival (84% vs 77%). Renal function is preserved in all DM patients, hypertension has developed in 11/18 (61%); annual post-HTx cardiac catheterisation has shown normal graft function and coronary arteries in all but 2 patients. CONCLUSIONS: Mid-term results indicate that HTx can be safely performed in diabetic patients; a longer follow-up is required to determine whether DM is no longer a contraindication to HTx.

Actuarial Analysis↗
PubMed1994

Donor shortage in heart transplantation. Is extension of donor age limits justified?

Chronic shortage of donor organs for heart transplantation led us to extend donor age limits. To verify the effectiveness of such a policy we have compared the results of heart transplantation in 45 patients with donors more than 40 years of age (group 1) with those of 72 patients older than 50 years of age who had heart transplantation with younger donors (group 2) between November 1985 and December 1992. The two groups were comparable in terms of mean recipient age, recipient and donor sex, and indication for heart transplantation. Mean donor age was 46 +/- 4 years (range 41 to 59 years) in group 1 and 23 +/- 7 years (range 8 to 39 years) in group 2 (p < 0.001). In group 1 cerebrovascular accidents were more common as the cause of donor death (60% versus 16%, p = 0.001), and no difference was found in ischemic time (144 +/- 47 minutes versus 140 +/- 48 minutes, p = not significant). There were 6 early (< 30 days) deaths in group 1 (13%) and 10 in group 2 (14%; p = not significant). Fatal acute graft failure was more prevalent, but not significantly so, in group 1 (10% versus 5.5%, p = not significant). Mean follow-up was 29 +/- 20 months (range 3 to 78 months) in group 1 and 30 +/- 20 months (range 3 to 80 months) in group 2 (p = not significant). At 5 years actuarial survival was 80% +/- 6% in both groups with comparable graft performance at echocardiographic and hemodynamic control studies. A significant difference was found in freedom from any type of coronary artery abnormality between group 1 (49% +/- 13%) and group 2 (77% +/- 8%) at 5 years (p < 0.05); however, freedom from coronary stenotic lesions only was similar. Major conduction disturbances have occurred more frequently in patients of group 1 (37% versus 12%; p = 0.003) without any difference in the need for permanent pacing. Donors older than 40 years of age can be accepted for heart transplantation with early and long-term results comparable with those obtained with younger donors. The impact of a higher incidence of coronary abnormalities on late performance of older grafts must be assessed at longer follow-up. Our results indicate that, because of the current organ shortage, extension of donor age limits is justified, even up to the sixth decade of life in selected cases.

Actuarial Analysis↗
PubMed1993

Endomyocardial biopsy in right ventricular cardiomyopathy.

Right ventricular cardiomyopathy is characterized by a progressive myocyte loss and fibro-fatty substitution of the right ventricle. The aim of our study was to assess the diagnostic accuracy of right ventricular endomyocardial biopsy. Using an imaging analyser system, histomorphometric parameters of myocytes, interstitium, fibrous tissue and fatty tissue were evaluated on endomyocardial biopsy from 30 patients with arrhythmogenic right ventricular cardiomyopathy, 29 patients with dilated cardiomyopathy and 30 control patients. The percent area of myocytes decreased from 78.10 +/- 7.34 in control to 63.39 +/- 9.22 in dilated cardiomyopathy (P < 0.05) and to 47.28 +/- 15.01 in arrhythmogenic right ventricular cardiomyopathy (P < 0.01). Fibrous tissue increased from 8.10 +/- 3.89 in control to 21.80 +/- 9.29 in dilated cardiomyopathy (P < 0.05) and to 24.60 +/- 11.37 in arrhythmogenic right ventricular cardiomyopathy (P < 0.05). Fatty tissue varied from 0.33 +/- 1.44 in control and 0.07 +/- 0.31 in dilated cardiomyopathy to 13.30 +/- 17.30 in arrhythmogenic right ventricular cardiomyopathy (P < 0.05). Fatty tissue was a feature of arrhythmogenic right ventricular cardiomyopathy (67% of patients vs. 6% of control and dilated cardiomyopathy patients). Diagnostic values typifying arrhythmogenic right ventricular cardiomyopathy, obtained by excluding any overlapping between confidence intervals in the three groups, were: myocytes < 44.95%; fibrous tissue > 40.38%, and fatty tissue > 3.21%, with 67% sensitivity and 91.53% specificity for at least one parameter. In conclusion, a significant difference between arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy and control exists in terms of amount of myocytes, fibrous tissue and fatty tissue. Presence of fatty tissue and fibrous tissue exceeding 3.21% and 40.38%, respectively should be considered highly suspect for arrhythmogenic right ventricular cardiomyopathy in right ventricular endomyocardial biopsy.

Adipose Tissue↗
PubMed1993

Endomyocardial biopsy in heterotopic heart transplant recipients via the femoral vein.

The results of endomyocardial biopsy (EMB) via the femoral vein in heterotopic heart transplant recipients were retrospectively analyzed and compared with those obtained using the right internal jugular vein approach. A total of 139 EMB were performed in 8 patients using the femoral (35) or the jugular (104) approach. Twenty three (64.7%) of the procedures performed via the femoral vein were part of the yearly hemodynamic and coronary artery study, and 12 (35.3%) constituted a routine postoperative evaluation of the myocardium rejection state in patients with imperviousness of the right internal jugular vein. Comparing the results obtained with the femoral approach, we observed a higher overall success rate (94.3 vs 88.5%, NS) and obtained more samples that were useful for histologic evaluation (95.5 vs 85.9%, NS); with the jugular procedure, the fragments were significatively larger in diameter (1.28 +/- 0.55 vs 1.61 +/- 0.85 mm, mean +/- SD) and in area (1.49 +/- 1.16 vs 2.28 +/- 2.24 mm2, mean +/- SD). No cardiac or local complications were noted when the femoral approach was used, while two attempts to perform biopsy via the jugular vein resulted in obstruction of this vessel. Our data suggest that the femoral venous approach for endomyocardial biopsy in heterotopic heart transplant recipients is a valid alternative to the more commonly used routes.

Biopsy↗
PubMed1993

Clinical results of steroid-free induction immunosuppression after heart transplantation.

Between January 1987 and September 1991, 112 operative survivors of heart transplantation were initially immunosuppressed with cyclosporin A and azathioprine without prednisone. Eighty-eight patients (79%) remained on a regimen of double therapy for a mean follow-up of 25 +/- 15 months (range, 1 to 54 months), whereas 24 patients (21%) had oral prednisone, 5 mg/day, added to maintenance therapy for persistent or repeated rejection. There were 5 early deaths (4%) because of acute rejection (4 patients) or infection (1 patient). Only 1 patient died late after heart transplantation of chronic rejection. Actuarial survival was 95% +/- 2% and 94% +/- 3% at 12 and 48 months, respectively. Mean rate of acute rejection was 1.7 +/- 1.0 episodes per patient, with a 5% +/- 2% freedom from rejection at 48 months. Ten patients (9%) required in-hospital treatment for infection; the actuarial freedom from infectious episodes was 85% +/- 4% at 48 months. Actuarial freedom from hypertension was 43% +/- 7% at 48 months. At annual catheterization, mean left ventricular ejection fraction was 0.64 +/- 0.08 and 0.62 +/- 0.05 at 1 year and 4 years, respectively, with evidence of coronary lesions in 9 patients (8%). In conclusion, steroid-free immunosuppression after heart transplantation is associated with a high incidence of acute rejection. However, the excellent medium-term survival and the low incidence of both infection and chronic rejection seem to justify a wider use of such treatment.

Adolescent↗
PubMed1993

Hypertrophic cardiomyopathy with mitochondrial myopathy. A new phenotype of complex II defect.

Two brothers, 25 and 19 years old, were affected by asymmetrical hypertrophic cardiomyopathy. The older brother had waddling gait and weakness of the proximal girdle muscles, while the younger had a broad-based gait and weakness of selected limb girdle muscles. EMG exam was myopathic. Serum enzyme, CPK and aldolase were elevated. Histochemical reactions in muscle revealed "core-like" areas, subsarcolemmal rims of mitochondria and lipid accumulation. Succinate-dehydrogenase stain showed a lack of activity in both biopsies, with the exception of intrafusal fibers. Microphotometric quantitative measurements confirmed the defect in both biopsies. Biochemical measurements of several mitochondrial enzymes in muscle showed a reduced activity of succinate-dehydrogenase (33%) and succinate-cytochrome C reductase (36-47%) which are both components of complex II. On myocardial biopsy lipid and mitochondrial abnormalities were found. This mitochondriopathy represents a new phenotype of partial complex II defect.

Adult↗