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Pieroni et al. ([1][1]) recently reported a 50% prevalence of right ventricular (RV) myocarditis in a cohort of 30 patients fulfilling standardized diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC). The differential diagnosis between myocarditis and ARVC was obtained by

Human parvovirus B19 infection is occasionally associated with acute lymphocytic myocarditis (ALM). Three infants with B19 virus-associated ALM were followed up clinically, histologically, and immunovirologically. Each infant had B19 virus DNA in the blood or B19 virus-specific IgM antibodies. Two infants with postnatal infection recovered after immunosuppressive therapy. The third infant with possible prenatal infection developed chronic persistent myocarditis associated with persistent B19 virus DNA in the blood. All 3 infants had increased levels of interferon-gamma, tumor necrosis factor-alpha, and interleukins -6 and -8. Four newborns with congenital B19 virus infection and 4 infants and children who had postnatally acquired B19 virus infection without myocarditis all had normal levels of these cytokines. These observations suggest that B19 virus infection in infancy causes ALM in some infants and children.

OBJECTIVES The purpose of this study was to describe the clinical and molecular features of a large family with maternally inherited cardiomyopathy (MICM). BACKGROUND Recently, several mitochondrial deoxyribonucleic acid (mtDNA) point mutations have been associated with MICM. However, the distinctive clinical and morphologic features of MICM are not fully appreciated. This is partially due to the small size of the reported pedigrees, often lacking detailed clinical and laboratory information. METHODS Clinical and genetic analysis of the family was carried out. RESULTS Echocardiography showed mostly symmetrical hypertrophic cardiomyopathy in 10 family members. The illness had an unfavorable course. Progressive heart failure occurred in three subjects, who eventually died; one individual underwent heart transplantation. Electrocardiographic or echocardiographic signs of cardiac hypertrophy in the absence of significant clinical complaints were observed in five subjects. Neurologic examination was normal. The mutation was detected in blood from all available subjects. Abundance of mutated molecules ranged between 13% and 100% of total mtDNA genomes. The severity of the disease could not be foreseen by the proportion of mutation in blood. CONCLUSIONS This report contributes a better description of the clinical aspects of MICM and provides important clues to distinguish it from hypertrophic cardiomyopathy. We suggest that mtDNA mutations, particularly in the transfer ribonucleic acid for isoleucin, should be systematically searched in patients with MICM. The identification of an underlying maternally inherited mitochondrial DNA defect in familial cases of cardiomyopathy may considerably influence the management and genetic counseling of affected patients.

Dynamic cardiomyoplasty is a therapeutic alternative to heart transplantation in irreversible cardiac insufficiency. Little information exists about the use of muscle relaxants in patients with cardiomyoplasty. In particular, it is not clear if the muscle flap is responsive to neuromuscular blockers. The purpose of this report is to describe the safe use of vecuronium in a patient with cardiomyoplasty. A 59-yr-old man, after cardiomyoplasty for dilated cardiomyopathy two years earlier, underwent general anaesthesia with fentanyl, propofol and vecuronium during surgery for intestinal ischaemia. Intraoperative transthoracic echocardiography showed that vecuronium did not affect muscle flap motion. Two days after surgery he died in septic shock. Post-mortem histological and immunohistochemical examination showed nervous degeneration of the flap probably as a result of the chronic low frequency pacing. There was also an increase in extrajunctional receptors and an alteration in junctional receptors, as demonstrated by the negative reaction to anti-synaptophysin antibodies, used to identify the neuromuscular plate. In patients undergoing non-cardiac surgery after previous cardiomyoplasty, muscle relaxants, such as vecuronium, may be used safely. Depolarising agents, such as succinylcholine, should probably be avoided because of the possible exaggerated actions on extrajunctional receptors.

We undertook this study to determine the use of transthoracic and transesophageal echocardiography in detecting valvular perforation and the clinical impact of the latter on the outcome of left-sided infective endocarditis. Transthoracic echocardiography was performed in 58 consecutive patients with infective endocarditis. According to the study protocol, a subgroup of 42 patients also underwent transesophageal echocardiogrophy. At referral, 20 (34%) of 58 patients had echocardiographic evidence of valvular perforation (group A). No valvular perforations were found in the remaining 38 patients (group B). During a follow-up period of 27 +/- 16 months, a major complication occurred in 18 of 20 patients in group A and in 11 of 38 patients in group B (p < 0.0001). Univariate analysis indicated previous infective endocarditis, aortic involvement, and New York Heart Association functional class had a predictive value for valvular perforation (p < 0.001). Stepwise regression analysis confirmed aortic valve perforation as the only independent predictive variable for surgery and death. Valvular perforation is a common complication of infective endocarditis and is associated with an adverse outcome. Transthoracic echocardiography can detect or suggest valvular perforation in infective endocarditis, but transesophageal echocardiography better defines this complication and predicts severe heart failure or the need for early surgical management.

We aimed at investigating contractile changes after hypoxia-reoxygenation and dobutamine challenge in superfused human atrial pectinate muscle to see whether high versus low stimulation rate during hypoxia might account for outcome differences compatible with the definition of an in vitro model of myocardial stunning and whether pretreatment with the dihydropyridine Ca2+ entry blocker felodipine might afford protection.Human right atrial trabeculae obtained from adult patients were superfused in an organ bath with oxygenated (O2 content 16 ml/l) and modified (NaHCO3 25.7 mmol/l) Tyrode's solution at 37 degrees C. Dobutamine (1 nmol/l to 10 micromol/l) was superfused in 10 oxygenated preparations to select the optimal drug concentration to be used in another 22 which were randomized. Group (A) consisted of time-related controls (Tyrodes's solution for 225 min at cycle length (CL) 1600 ms and no dobutamine). There were two test groups, respectively: (B) low (1600 ms CL) and (C) high (400 ms CL) stimulation rate. After 60 min of stabilization, in groups B and C, hypoxic superfusion (O2 content 5 ml/l) lasted 60 min, then reoxygenation (60 min) and dobutamine challenge (1 micromol/l, 15 min) were performed. Analysis of variance for repeated measures with the Greenhouse-Geisser correction, and a repeated measures model with structured covariance (preparation mass, length, width and time-varying time to peak tension) matrices were used whereby grouping (G), time (T) and G x T interaction were weighted. Force-frequency relationship and post-pausal potentiation were studied after each phase. Electrophysiology, histomorphometry and electron microscopy were carried out (n=6). Felodipine (0.1 micromol/l, n=5) pretreatment (15 min before hypoxia) was given in parallel experiments.Time-related controls showed approximately 10% per hour decrease of developed tension and the Paradise test provided approximately 80% of control values. In test groups (as compared to baseline values) contractility was decreased approximately 65% after hypoxia-reoxygenation and it increased approximately 25% after dobutamine (G, 0.0065P0.0155; T, P=0.00005; G x T, P=0.00005). High stimulation rate during hypoxia worsened hypoxia-reoxygenation contractile changes, whereas reversibility after dobutamine was less. In both B and C groups during hypoxia, contractility decreased quite rapidly, although by 10 min or so a plateau (approximately 50%) was reached in group B, whereas in group C contractility decreased to20%. None of the covariates contributed significantly to predict the dependent variables investigated. Force-frequency relationship and post-pausal potentiation were repeatable, paralleled overall changes due to hypoxia, reoxygenation and dobutamine challenge and were useful to discriminate Ca2+-related diastolic processes thus helping index myocardial contractile reserve. Force-frequency relationship was negative at high stimulation rates, concomitant to an abrupt change of shape and duration of action potential with little time for Ca2+-related Ca2+ release and ensuing systolic processes. Felodipine pretreatment enabled an unblunted response to dobutamine. Histomorphometry showed an unexpected 'fibrotic core'. At electron microscopy, subendocardial and deep part of the same pectinate muscles showed identical degrees of degenerative lesions. Superfused samples showed, unexpectedly, less anoxic lesions than preparations fixed within 15 min from surgical explant, although lesions were higher than in samples fixed immediately after explant.This might be a relevant model, whereby pharmacological or physical interventions are tested. Native human atrial trabeculae might be used without dissection and/or preservatives. If high stimulation rate during hypoxia is used the power of hypothesis testing is maximized. Future studies with this material will be easier and comparatively smaller series might be investigated. Felo

Background Although muscle pain is relatively frequent in primary Sjogren Syndrome (pSS), a frank myositis is rare and reported only in 2.5-11% of patients. The most frequent symptoms are muscular weakness and myalgias and diagnosis is based on clinical, laboratory and histologic evaluation. Objectives To describe the prevalence and course of myositis in a multicenter cohort of patients with pSS. Methods Clinical and laboratory data from patients with a diagnosis of pSS were retrospectively collected. The clinical charts of patients with symptoms and/or signs of inflammatory myositis were carefully reviewed. Results One thousand one hundred seventy patients with pSS (52M, 1118 F, mean age 57.3 years; range 17–89 years) were considered. Mean age at diagnosis was 51±14 years and the mean follow up from diagnosis was 5.6 years (range 0–42 years). Ten female patients (0.85%) presented invalidating muscular weakness in the upper and lower limbs and/or severe myalgias. In 8/10 patients an increase of Creatine Phosphokinase (CK) serum level was present. A possible iatrogenic effect was excluded; in one case myositis started under statin therapy but didn’t disappear after drug discontinuation. In the 10 patients, mean age at pSS diagnosis was 47.4 years (range 14–68), while mean age of myositis diagnosis was 50.6 years (range 23–69). In six out of ten, muscular and sicca symptoms had occurred together at onset. Diagnosis of myositis was supported by clinical evidences and by the presence of increased CK serum levels. In 7/10 patients muscle biopsy was performed. In all biopsies signs of inflammation were present. In 1/7 patients (14.2%) the biopsy was typical of an inclusion body myositis, while in the remaining cases histological aspects of Dermatomyositis and Polymyositis (PM) were detected. Eight out of ten patients presented at least three criteria for inflammatory myopathy (according to Bohan and Peter criteria, 1975) suggesting a possible diagnosis of a true overlap syndrome. In one of these patients serum anti Jo-1 and anti RNP antibodies were detected. Six patients (60%) were successfully treated with DMARDs, while four (40%) did not respond. Thus, two were treated with IvIg, one showing complete remission of myositis, and the remaining two non-responder patients were treated with Rituximab, one showing complete remission. Conclusions Our retrospective study shows a lower prevalence of myositis in pSS than previously reported. Muscle involvement can occasionally represent the first symptom at disease onset. An overlap syndrome with inflammatory myopathies, above all PM, should be considered. Different therapeutic approaches can be used: DMARDs are effective in the majority of the patients, while IvIg and Rituximab may represent an available alternative therapeutic options in non-responder patients. Disclosure of Interest None Declared

To evaluate possible occult myocardial disease in patients with ventricular arrhythmias of unknown origin, over 11 years right ventricular endomyocardial biopsies (EMB) were performed on 80 consecutive such patients (29 Females, 51 Males; median age 42 years). Seventy-one (89%) had ventricular tachycardia or fibrillation, 7 (9%) had complex ventricular arrhythmias, and 2 (3%) had premature ventricular beats. None showed clinical evidence of congestive heart failure or significant coronary artery or valvular disease. Endomyocardial biopsies revealed pathologic changes in 70 out of 80 patients (88%). Of the 70 affected, 39 (56%) had nonspecific changes consistent with cardiomyopathy (e.g., myofiber hypertrophy, interstitial and perivascular fibrosis, and vascular sclerosis); 6 (9%) had active myocarditis (Myo); 7 (10%) had borderline Myo; 7 (10%) had small vessel disease; 6 (9%) had changes consistent with arrhythmogenic cardiomyopathy; 2 (3%) had amyloidosis; 2 (3%) had microfibrillar cardiomyopathy, and one (1.0%) showed intravascular organizing thrombus. Thus, EMB reveals a variety of abnormalities in the majority of patients presenting with ventricular arrhythmias without clinical evidence of structural heart disease.

A novel mtDNA mutation at position nt. 4300 in the tRNAIle gene is associated with hypertrophic cardiomyopathy inherited as a maternal trait. Interestingly, this mutation seems to cause a pure heart disease as opposed to most other mtDNA mutations, which are associated with multisystemic disorders. Hypertrophic cardiomyopathies are genetically heterogeneous, and mtDNA defects should be considered in the differential diagnosis, especially when there is evidence of maternal inheritance.

"Myofiber disarray" defines a nonparallel arrangement of cardiac myocytes. The presence of a sufficient quantity of myocardial fibers showing this change is considered to be a specific histological feature of hypertrophic cardiomyopathy (HCM). However, small zones of myofiber disarray are found in both cardiac hypertrophy and other pathological conditions. Recently, we demonstrated an altered pattern of desmin intermediate filaments in disarrayed myofibers from specimens of HCM. To test the hypothesis that desmin alterations might be specific for cardiomyopathy, we performed an immunohistochemical study on myocardial surgical samples from 11 patients with HCM and from 12 patients with tetralogy of Fallot (toF) on 14 endomyocardial biopsy specimens (EMBs) from transplant recipients with myofiber disarray surrounding areas of scarring (previous biopsy site) and on specimens of four autoptic hearts with severe acquired left ventricular hypertrophy. Disarrayed myofibers from all specimens of HCM showed the following abnormalities in the pattern of desmin intermediate filament distribution: (1) decrease or loss of labeling of intercalated discs and Z bands, (2) longitudinal arrangement of desmin intermediate filaments, and (3) intense, granular staining of several myocytes. This spectrum of desmin alterations was never observed in disarrayed myofibers in specimens of toF or acquired myocardial hypertrophy or in EMBs. Altered distribution of desmin intermediate filaments seems to be specific to myofiber disarray in HCM and it may play a role in the altered myocyte arrangement in HCM.

Cardiac transplantation for patients with idiopathic dilated cardiomyopathy (IDC) and poor left ventricular function usually is postponed until symptoms have become intolerable. However, the short-term prognosis of this subset of patients has been defined poorly. Accordingly, the 1-year outcome was investigated in 30 patients with IDC with an ejection fractionor = 25% who showed a stabilized clinical condition at assessment for transplantation and were therefore considered at low priority for surgery. During follow-up, 10 patients (group A) showed a poor outcome: 2 died suddenly, and 8 had hemodynamic failure (4 of whom underwent transplantation and 4 of whom died from heart failure while on the waiting list). The remaining 20 patients (group B) had a benign outcome. At assessment for cardiac transplantation, clinical and electrocardiographic features, left ventricular dimension, and ejection fraction were similar between the two groups. However, group A patients had higher left ventricular end-diastolic pressure (p0.03) and lower cardiac index (p0.02) and stroke volume index (p0.03) with respect to group B patients. In addition, the former had a lower myofibril volume fraction (p0.001) and a higher nuclear area (p0.001) compared with the latter. Multivariate analysis selected myofibril volume fraction (p0.001) and nuclear area (p0.005) as the only independent predictors of a poor 1-year outcome. The combination of myofibril volume fractionor = 89% and nuclear area50 microns 2 was found in all group A patients (sensitivity 100%) but in only 2 group B patients (specificity 90%). It is concluded that in patients with IDC considered at low priority for cardiac transplantation: (1) the 1-year freedom from a cardiac event is lower than that currently expected with surgery; (2) histomorphometric features, that is, the concurrency of low myofibril volume fraction and increased nuclear area, predict short-term outcome; and (3) endomyocardial biopsy at assessment for cardiac transplantation might improve the rationalization of the timing of the procedure.

A case of cystic adventitial degeneration (CAD) of the popliteal artery is presented. Histologically the cyst appeared localised in the adventitia, outside the elastic lamina. A lectin-histochemical study evaluated the following peroxidase conjugated lectins: Peanut agglutinin, Concanavalin A, Ulex Europaeus (UEA I) and Wheat Germ Agglutinin. The lack of UEA I reactivity excluded an arterial origin of the cyst. Moreover, the lectin binding pattern of CAD appeared to be similar to the reactivity pattern of normal synovia.

The goal of our study was to evaluate the incidence of heart involvement in AIDS patients during various stages of the disease. Between January 1988 to September 1991, we conducted a prospective study in 114 anti-HIV positive patients. The patients, whose mean age (+/- SD) was 34.6 +/- 5.4 years (range 20 to 54), were divided into three groups: anti-HIV positive asymptomatic (n = 31; 27%), AIDS related complex (ARC) group IV-A (n = 11; 10%), and AIDS subgroups IV-C1 (n = 62; 54%) and IV-D (n = 10; 9%). Overall, 84 patients (74%) were i.v. drug abusers, 24 (21%) were homosexuals, and six (5%) were partners at risk. Zidovudine (AZT) was administered to 94 patients (82%). Opportunistic infections and/or secondary malignancies were detected in 72 patients (63%). Electrocardiographic changes were of little clinical relevance. Of 72 AIDS patients, 47 (65.2%) presented a cardiac involvement: 12 subjects (16.6%) were affected by a dilated cardiomyopathy, 13 (18%) by pericardial effusion, three (4.1%) by mitral valve prolapse, four (5.5%) by myocarditis, five (6.9%) by valvular bacterial endocarditis, and 10 (13.8%) by alterations of left ventricle regional contractility. During a mean follow-up period of 44 months, 29 AIDS patients (40.2%) died. Death was attributed to a cardiac event in four patients; autopsy could be performed in 24 of the 29 patients who died. Our results demonstrate that heart involvement is present in 45.6% of HIV-infected patients, but only in the end-stage of the disease (AIDS) and it is presumably due to opportunistic infections and/or secondary malignancies.(ABSTRACT TRUNCATED AT 250 WORDS)

Arrhythmogenic right ventricular cardiomyopathy (also known as arrhythmogenic right ventricular dysplasia) is characterized by adipose or fibroadipose tissue replacement of the right ventricular myocardium, whereas the left ventricle is substantively spared. Two cases of the disease with evidence of extensive left ventricular involvement at pathologic examination are described. Hearts from two patients who died suddenly showed full-thickness right ventricular fatty infiltration associated with extensive left ventricular involvement (> 50% of myocardial thickness). These findings might explain the reported clinical features of left ventricle dysfunction in a subset of patients with arrhythmogenic right ventricular cardiomyopathy. In view of the biventricular involvement of the disease, it should simply be termed “arrhythmogenic cardiomyopathy.”

In the March, 2009, edition of Europace (volume 11, number 3), Ejima et al. 1 reported the case of a young woman in whom a diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) was obtained with of a novel endomyocardial biopsy (EMB) approach, guided by electroanatomic mapping of the right ventricle (RV). In order to increase the diagnostic accuracy of EMB, Ejima et al. 1 performed …

Working Formulation (WF) was recently introduced by the International Society for Heart Transplantation to grade acute cellular rejection, as well as additional lesions observed in endomyocardial biopsies (EMBs). The aim of this study was to evaluate the actual advantages of this grading system in terms of feasibility and predictive value. To this purpose, we reclassified 1037 EMBs performed in our heart transplantation units according to the WF. Our results show that multifocal mild rejection (grade IA), when worsening, tends to progress to multifocal moderate (3A), whereas diffuse mild (1B) generally worsens to diffuse moderate (3B), thus following the same focal or diffuse pattern. Unifocal moderate rejection (grade 2) has a peculiar behavior, in that it almost always resolves, though in our units it is treated the same way as is grade 113. Finally, we found a significant relationship between Quilty B effect and chronic rejection. In conclusion, this retrospective study shows that WF is effective in using both qualitative and quantitative criteria and, particularly, in separating focal and diffuse forms of rejection and devoting a distinct grade to unifocal moderate rejection.

To investigate the role of cell viability and metabolism on the myocardial kinetics of a new tracer, technetium-99m-methoxyisobutyl isonitrile (Tc-99m-MIBI), 250 microCi/l Tc-99m-MIBI was infused in isolated rat hearts under constant flow conditions. The hearts were studied after inducing irreversible damage by cytochrome c oxidase inhibitor sodium cyanide (n = 8) or sarcolemmal membrane detergent Triton X-100 (n = 8). The control hearts (n = 6) received no toxins. Mean Tc-99m-MIBI peak accumulation activity was significantly reduced after cyanide (51.1 +/- 44.2% of control, p less than 0.01) and Triton (13.8 +/- 2.7% of control, p less than 0.001) administration. Kinetic studies also showed marked reduction in accumulation rates and marked increase in clearance rates for cyanide (p less than 0.01) and Triton (p less than 0.01) groups compared with controls. Potential changes in regional flow distribution were assessed using microspheres. When peak accumulation activity was corrected for these changes, there remained significant differences between the groups. In the cyanide and Triton groups, irreversible cell injury was confirmed by creatine kinase and lactate dehydrogenase release, triphenyl tetrazolium chloride staining, and electron microscopy. All the cells were viable in the control group. We conclude that the accumulation and clearance kinetics of Tc-99m-MIBI are significantly affected by cell viability. Tc-99m-MIBI kinetics appear to be dependent on sarcolemmal integrity and to a lesser extent on aerobic metabolism.

To evaluate whether complex ventricular arrhythmias relate to presenting features and prognosis of dilated cardiomyopathy, 104 patients were studied from 1977 to 1987. At diagnosis, the 19 patients with complex ventricular arrhythmias (18%), as compared to the 85 patients without (82%), had a higher incidence of palpitation (P less than 0.01), severe dyspnea (P less than 0.001) and atrial fibrillation (P less than 0.01). They showed also higher mean right atrial pressures (10 +/- 5 vs 6 +/- 4 mm Hg, P less than 0.001) and higher right ventricular end-diastolic pressures (11 +/- 4 vs. 7 +/- 4 mm Hg, P less than 0.001) than patients without complex ventricular arrhythmias. Histologic samples were collected from the 32 patients (31%) studied since 1984 and semiquantitatively graded. The 11 patients with complex ventricular arrhythmias showed a higher frequency of severe interstitial fibrosis than the 21 patients without (64% vs. 24%, P less than 0.05), but they were otherwise similar as to the frequency of marked myocellular hypertrophy, changes of myocardial regression, endocardial fibrosis, attenuation of myocytes, hyperplasia of smooth muscle cells and infiltration by inflammatory cells. During a follow-up of 3.8 +/- 3.5 years, 35 patients (34%) died. Mortality was 58% (11 out of 19) in patients with complex ventricular arrhythmias and 28% (24 out of 85) in patients without (P less than 0.025). These results show that complex ventricular arrhythmias in dilated cardiomyopathy are associated with impairment of function of the right heart and severe interstitial fibrosis of the left ventricle, rather than with left ventricular dysfunction. Presence of complex ventricular arrhythmias also seems to identify those at high risk for death.

Recent studies have shown that a paradox occurs in ischemic heart disease, as women (W) show a higher incidence of heart failure than men (M) despite similar LV ejection fractions. Aim of this study was to verify whether such a “gender paradox” also exists in idiopathic dilated cardiomyopathy (IDC) and, if so, which are the possible underlying factors. Accordingly, we analyzed the clinical, ECG, echocardiographic, hemodynamic and LV histomorphometric features of 75 IDC patients (48 M and 27 W), consecutively referred to our Unit for invasive evaluation. W were older (55 ± 9 vs 45 ± 11 yrs, p l 0.005), more symptomatic (NYHA class III or IV in 63 vs 35%, p l 0.05), and had more frequent signs of heart failure (70 vs 42%, p l 0.04), as compared with M. At echocardiography, the two groups had similar LV diameter, volume and mass. Doppler examination, however, showed that W had a lower ratio of transmitral early to late peak filling waves than M (1.0 ± 0.3 vs 1.4 ± 0.4, p l 0.001), thus indicating a worse LV diastolic filling dynamics. At catheterization, W showed higher mean pulmonary artery pressure (25 ± 9 vs 19 ± 8 mmHg, p l 0.005). and LV end-diastolic pressure (21 ± 7 vs 16 ± 8 mmHg, p l 0.01) than M, whereas LV ejection fraction and cardiac index were similar in both genders. Comparison of LV histomorphometric features did not show any difference in myocellular area, nuclear area, and myofibril volume fraction. Conversely, W had larger interstitial fibrosis (12 ± 6 vs 7 ± 5%, p l 0.001), and endocardial fibrosis (15 ± 5 vs 8 ± 6%, p l 0.001), as compared with M. It is concluded that: (1) the paradox observed in ischemic heart disease of more frequent symptoms and signs of heart failure in W than in M occurs also in IDC; (2) such “gender paradox” relates to differences between W and M in LV diastolic function, rather than in cardiac contractility; (3) the association of female gender with a larger accumulation of cardiac fibrous tissue seems to constitute the morphologic substrate of the phenomenon.

To investigate the clinical significance of intraoperative cultures in elective abdominal aortic aneurysmectomy, we cultured the aneurysmal wall and contents in 90 patients undergoing vascular graft surgery. Prosthetic graft infection was documented in 1 out of 62 patients with negative cultures and in none of 28 patients with positive cultures (chi 2 = 0.4, p greater than 0.1). Bacterial growth was seen in neither of 2 inflammatory aneurysms, 3 of 14 atherosclerotic aneurysms and 2 of 5 aneurysms without specific features. A retrospective analysis of patients' charts aimed at finding possible risk factors failed to identify any correlation between results of cultures and length of hospitalization before surgery, time interval between angiography and surgery, route of angiography procedure or minutes of surgery before sample collection. We conclude that positive cultures may not imply clinical infection at the time of surgery and that prolonged post-operative organism-specific antibiotic therapy does not appear necessary to prevent graft contamination in patients undergoing elective abdominal aortic aneurysmectomy.

Objective . This study evaluated prospectively the frequency, clinical outcome and pathologic findings of acute global left ventricular dysfunction in human immunodeficiency virus (HIV) infection during the various stages of the disease. Background . Acute global left ventricular dysfunction in the course of HIV infection is still a poorly defined clinical entity, and little is known about the outcome after the acute onset Methods . Between January 1988 and June 1992, 136 HIV-positive (HIV + ) patients without clinical, electrocardiographic or echocardiographic evidence of cardiovascular dysfunction on admission were prospectively studied with serial echocardiograms. Patients were assigned to three groups: 1) anti-HIV + asymptomatic (17 patients, 12.5%); 2) acquired immunodeficiency syndrome (AIDS)-related complex (26 patients, 19.1%); 3) AIDS (93 patients, 68.4%). Results . During a mean follow-up period of 415 ± 220 days, seven patients, all in the AIDS subgroup, developed clinical and echocardiographic findings of acute global left ventricular dysfunction; of these, six (85%) died of congestive heart failure. Mean survival time from symptom onset was 41 ± 13 days. Necropsy findings in five patients revealed acute lymphocytic myocarditis in three, cryptococcal myocarditis in one and interstitial edema and fibrosis in one. In only one patient was left ventricular dysfunction reversible with treatment. Conclusions . Although infrequent, acute global left ventricular dysfunction is not rare in the course of HIV infection. It seems to occur exclusively during the AIDS stage. Acute global left ventricular dysfunction is often fatal but may be reversible and is mainly associated with the pathologic findings of acute myocarditis.

The authors describe a family of Sephardic Jews with progressive external ophthalmoparesis, skeletal muscle weakness, and parkinsonism. Autosomal recessive inheritance was suggested by many consanguineous marriages, although a dominant disorder could not be excluded. No linkage to known progressive external ophthalmoparesis locus was found. The presence of cytochrome c oxidase-negative ragged-red fibers, biochemically reduced respiratory chain complexes, and multiple mitochondrial DNA deletions in muscle biopsies from four patients suggested a new mitochondrial disorder of intergenomic communication.

In this study, we reveal that leptin evokes an acute hypotensive effect in 6-hydroxydopamine sympathectomized rats (response to maximal leptin dose, mean blood pressure: from 92 +/- 4 to 78 +/- 2 mmHg, P < 0.01). This hemodynamic effect is related to a direct action of the hormone on vascular tone, since in aortic and mesenteric rings increasing doses of leptin evoke a dose-dependent vasorelaxation (aorta: from 3 +/- 1 to 36 +/- 3, n = 15; mesenteric: from 6 +/- 1 to 30 +/- 5, n = 10), which is impaired by endothelial denudation. In particular, leptin-evoked vasorelaxation is impaired by nitric oxide synthase inhibition in aorta (delta% of maximal response: from 36 +/- 3 to 3 +/- 1, P < 0.01) and by endothelium-derived hyperpolarizing factor (EDHF) inhibition in mesenteric arteries (delta% of maximal response: from 30 +/- 5 to 7 +/- 2, P < 0.01), suggesting that vasorelaxation evoked by leptin is heterogeneous and related to the vascular bed. Finally, the inhibition of nitric oxide synthase by NG-nitro-L-arginine-methyl ester does not modify blood pressure response to leptin, suggesting a predominant role of the EDHF mechanism in the hypotensive effect of leptin.

Ten cases of glioblastomas showing oncocytic changes are described. The tumors showed mononuclear to multinuclear cells and abundant, granular, eosinophilic cytoplasm. The cytoplasm of these same cells was filled by strongly immunoreactive mitochondria. At ultrastructure, numerous mitochondria, some of which were large, were evidenced in the cytoplasm of neoplastic cells. Finally, 9 of 10 of these cases had a significantly high mitochondrial DNA content compared with control tissue (P < .01). It seems that, for these tumors, the designation of oncocytic glioblastoma is appropriate. To the best of our knowledge, oncocytic changes have not been previously reported in such neoplasms. Oncocytic glioblastomas have to be added to the long list of various tumors that can manifest “unexpected” oncocytic changes in different organs. Albeit failing to show statistical significance (log-rank test, P = .597; Wilcoxon test, P = .233), we observed a trend for longer median survival in oncocytic glioblastomas, when compared with “ordinary” glioblastomas (median survival of 16 versus 8.7 months). Thus, it seems that the definition of neoplasms showing oncocytic changes, currently based on classic morphological parameters (ie, histology, ultrastructure, and immunohistochemistry), can be expanded by including the quantitative assessment of mitochondrial DNA content.