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We examined 166 patients with advanced chronic obstructive pulmonary disease (COPD) treated with long-term oxygen therapy (LTOT) in order to evaluate the prognostic factors of such patients. The mean observation period was 24 months (range 2-50 months) and the following variables were considered: age, forced expiratory volume in 1 s (FEV1), arterial oxygen tension (PaO2), arterial carbon dioxide tension (PaCO2), hematocrit, right ventricular systolic pressure (RVSP; evaluated by Doppler echocardiography), number of hospitalizations in the 2 years prior to prescription of LTOT and body mass index. The overall survival rate was 78.3% at 24 months and 67.1% at 36 months. A univariate analysis identified three variables as significant predictors of survival: FEV1, PaO2 and RVSP. A multivariate analysis, using Cox's model, showed an independent predictive power for RVSP, age and FEV1. RVSP higher than 35 mm Hg, age greater than 70 years and FEV1 lower than 30% of the predicted value were associated with shortened survival. The importance of pulmonary hypertension as a predictor of death suggests that LTOT could be prescribed earlier for COPD patients with cor pulmonale, as oxygen has been shown to be the only effective therapy for improving the survival probability of these patients.

The first studies about fertility and nutrition date back to the 70ies and already showed a strict relation among female fertility, weight and body composition. However, the mechanisms of this connection started to be explained only after leptin's discovery. According to some authors' opinion, leptin could interact with reproductive axis at multiple sites with stimulatory effects at the hypothalamus and pituitary and stimulatory or inhibitory actions at the gonads. Leptin could play a role in other physiologic processes such as menstruation and pregnancy, and could initiate the complex process of puberty. It has been showed that conditions in which nutritional status is suboptimal, such as eating disorders, exercise induced amenhorrea, functional hypothalamic amenhorrea and polycystic ovarian syndrome, are associated with abnormal leptin levels. These conditions, are characterized by severe changes in body composition and dietary habits. Since leptin is regulated by body composition and dietary factors, (such as energy intake and macronutrient composition), a strict connection between nutritional intake and fertility regulated by leptin is confirmed. This review focuses on the current knowledge about nutritional factors that influence leptin levels. Since clinical and subclinical nutritional imbalance can determine the development and the maintenance of neuroendocrine and metabolic aberrations, studies on fertility need a deeper attention about dietary habits and nutritional status.

In chronic pulmonary vascular thrombotic disease, pulmonary thromboendarterectomy lure proved to be effective in reducing pulmonary hypertension and improving gas exchange. However, persistent pulmonary hypertension and unrelenting reperfusion edema are the main causes of death. We report a case of pulmonary thromboendarterectomy followed by an immediate unfavorable postoperative course with acute and persistent pulmonary hypertension, gas exchange impairment, and heart dysfunction. In this particular case, inhaled nitric oxide was successfully administered.

The authors report the case of a young woman (47 yrs old) who underwent cardiac evaluation for recurrent unexplained cerebral transient ischemic attacks. In the search for a source of embolization, a transesophageal echocardiography was performed and this revealed an atherosclerotic complex plaque of the ascending aorta as the sole potential source of cerebral embolism, while the remaining aortic wall was normal. The atheroma showed a calcific portion inserted on the aortic wall and a mobile hypoechogenic portion protruding into the aortic lumen. Furthermore, we found increased levels of cholesterol, fibrinogen and plasmatic homocysteine after methionine loading. Atherosclerotic lesions of the aortic arch are a rare cause of embolism in young patients with stroke, but they can lead to important complications such as thrombosis and embolism, similar to atherosclerotic lesions in elderly patients. The mechanisms that predispose for atherosclerosis of the aorta in young patients are still unknown. It was recently reported that not only hypercholesterolemia but also elevated levels of fibrinogen and homocysteine are independent risk factors for cerebrovascular disease. It is possible that these factors may be important predictors of atherosclerosis of the thoracic aorta in young patients, but more clinical data are still necessary. This case report confirms the importance of performing a TEE study and examining the cholesterol, fibrinogen and homocysteine plasmatic concentrations in all of young patients with unexplained stroke or transient ischemic attacks.

To report our personal experience with the locoregional treatment of breast cancer.Eighteen patients aged 33-67 years (mean: 54 years) were treated with 31 sessions of intra-arterial antiblastic infusion, 5 of them for neoadjuvant purposes (Group 1), 5 for palliation in unresectable tumors (Group 2) and 8 for cutaneous recurrences after mastectomy (Group 3).2/5 CR and 3/5 PR were obtained in Group 1; 2/5 lesions were made resectable and 3/5 RP obtained in stage III or unresectable lesions (Group 2). Finally, 3/8 CR, 3/8 PR, 1/8 SD and 1/8 PD were observed in Group 3. An objective response according to WHO criteria was demonstrated in 15/18 cases (88%). We had no post-treatment hematologic complications, but one patient presented focal subcutaneous sclerosis and one cutaneous necrosis.The morphological efficacy of intraarterial antiblastic infusion in our series was similar to that of other series. No definitive conclusions can be drawn yet about clinical results and long-term survival. This poorly invasive and low risk procedure, which should be combined with other treatments, permits to reduce the extent of surgery and to treat skin recurrences.

Three children with familial hypophosphatemic rickets showed, in the untreated state, active rickets of the metaphyseal areas of long bones. All were treated with combined phosphate /1-2 g daily by mouth in 4-5 divided doses) and vitamin D2 therapy for 2-5 years. The radiological appearance of the metaphyses became practically normal in two, whereas little change took place in the endosteal bone surface. Bone biopsies demonstrated osteomalacia. Therefore, two subjects were given phospate (1-2 g daily) and 1-alpha-hydroxyvitamin D (0.25-0.50 microgram daily) for 2 years; then phosphate (1 g daily) and 1.25 dihydroxyvitamin D (0.25-0.50 microgram daily) combined therapy for 5 years; the third patient had phosphate (1 g daily) and 1.25 dihydroxyvitamin D (0.25 microgram daily) combined therapy for 2 years. After therapy with low doses of 1.25 dihydroxyvitamin D, X-rays showed a nearly normal bone aspect, without nephrocalcinosis in all patients. All subjects were submitted to osteothomies with positive results.

Focal myocardial necrosis reported in patients who died of brain lesions and in donor hearts soon after insertion has been attributed to catecholamine-related injury induced before operation, or in the perioperative period. Interpretation of the morphofunctional type of myocardial injury observed and its quantification may help understand both its pathophysiology and clinical relevance.In 27 patients without heart disease who died of intracranial brain hemorrhage after berry aneurysm rupture, terminal clinical signs were correlated with the presence of absence of myocardial injury. All hearts were systematically examined, and the total histologic area was measured in square millimeters, with both the number of foci and myocardial cells showing necrosis, normalized to 100 mm2. Forty-five cases of fatal head trauma (26 "instantaneous" and 19 "rapid" deaths) in normal subjects and 38 cases of acquired immunodeficiency syndrome with (14 cases) or without (24 cases) severe brain damage were used as control subjects.Contraction band necrosis was the only form of myocardial necrosis found in 89% of patients with acute brain hemorrhage. Its extent was 26 +/- 34 foci and 67 +/- 104 necrotic myocardial cells x 100 mm2. In patients with acquired immunodeficiency syndrome, its frequency was 58% in those without and 78.5% with severe brain lesions, with foci and myocardial cell values of 1 +/- 1.5 and 10 +/- 22 and 7 +/- 16 and 17 +/- 32, respectively. In head trauma cases with instantaneous death, the frequency was 4% (one case only with foci 0.5 and myocardial cells 35), whereas with a rapid death it was 40% (foci 12 +/- 18 and myocardial cells 21 +/- 33).The observed myocardial injury was present in all groups examined, being maximal in patients with intracranial brain hemorrhage with longer survival and minimal in patients with head trauma who died instantaneously. In this setting, this lesion is typical of catecholamine myotoxicity and may express a sympathetic overstimulation either in the agonal period and independent of therapy or be caused by brain injury, especially intracranial brain hemorrhage. However, the extent of myocardial injury observed was minimal and should not jeopardize cardiac function if hearts from such subjects are transplanted.

Therapy of patients presenting with cardiogenic shock refractory to medical treatment can be undertaken with uni or biventricular circulatory assist devices. Pre implantation evaluation of patients is aimed at defining the etiology as well as the extent of uni versus biventricular heart failure, the possibility of recovery of myocardial function improvement of vital organ function and the possibility of cardiac transplantation. Circulatory assist devices must provide efficient support of the failing ventricles, allow recovery of myocardial function or cardiac transplantation under optimal circumstances. The choice of uni-biventricular support of total artificial heart is discussed as well as criteria useful in defining a therapeutic strategy.

Primary malignant myocardial tumours are rare and essentially sarcomas. The authors report a case of primary left ventricular liposarcoma which is the 18th reported case. The presenting signs were of cardiac failure. Metastases are common by the time of diagnosis. Surgical ablation, though rarely complete because of its myocardial localisation, is justified for precise histological diagnosis. The prognosis of these lesions is poor. Complementary treatment is rarely used. However, it should be proposed as surgery alone has been shown to have limited curative applications.

ST-segment depression on exercise stress test (EST) is an independent predictor of future cardiac events. Nevertheless, in apparently healthy subjects without angina the occurrence of false positive results is frequent. Thallium myocardial imaging (TMI) may improve diagnostic and prognostic accuracy of EST. The aim of the present study was to assess the role of a normal exercise TMI for excluding a coronary artery disease in subjects with asymptomatic abnormal EST.Subjects referred for TMI from 1/1980 to 5/1991 with an abnormal EST and without history of ischemic, congenital, or valvular heart disease or abnormal resting ECG were included into the study. 137 subjects (98 men, 39 women), mean age 53 +/- 8 yrs (range 37-74 yrs) were enrolled and followed-up for 6.4 yrs (range 3-13 yrs). Clinical indications for EST were: atypical chest pain in 56 (41%) cases, check-up in 52 (38%) cases, sport activity in 29 (19%) cases. All subjects had a maximal symptom-limited EST. Abnormal EST was defined by a horizontal or downslopingor = 1 mm or upslopingor = 1.5 mm ST-segment depression at 0.08 sec. from J-point, in at least 2 leads. EST was discontinued for fatigue in 129 (94%) cases, for ST-segment depressionor = 3 mm in 8 (6%) cases. None had chest pain during EST. All subjects selected for the study had normal TMI. Criteria for normal TMI were homogeneous Thallium uptake on postexercise images and a normal washout in the delayed images by qualitative analysis. Planar images were obtained in 118 (86%) cases, and tomographic SPECT images in 19 (14%).During the follow-up period no subject died for cardiac causes and only 9 subjects (1%/yr) had non fatal cardiac events: 4 (0.45%/yr) had a non fatal myocardial infarction (one subject had coronary angiography for postinfarction angina and subsequent 3 coronary bypass graft for multivessels disease), 2 subjects (0.2%/yr) became symptomatic for unstable angina (both had coronary angiography and subsequent PTCA for critical left main coronary artery stenosis) and 3 (0.34%/yr) developed stable angina (one had coronary angiography and subsequent bypass graft for a critical stenosis of left main coronary artery). Four further subjects died for non cardiac events. Comparing clinical data and TE results of subjects with and without coronary events, we found that some parameters were related to a higher incidence of cardiac events: hypertension (78% vs 31% respectively in subjects with and without cardiac events, p0.01), hypercholesterolemia (33% vs 4.7%, p0.01);or = 2 conventional coronary risk factors (56% vs 17%, p0.02); and a slow regression of abnormal ST-segment depression during recovery (2.8 +/- 2 vs 1.5 +/- 1 min, p0.01).In conclusion, in subjects without typical chest pain and with abnormal asymptomatic EST, a normal exercise TMI identifies subjects with very low risk of future cardiac events (1%/yr). Our data suggest that subjects with abnormal asymptomatic EST should be routinely submitted to exercise TMI.

Between 1985 and 1992, the gastroepiploic artery was used for coronary bypass surgery in 80 consecutive patients. Revascularisation was totally arterial in 62.5% of cases, the gastroepiploic being associated with single or double internal mammary artery grafts. The gastroepiploic graft was used to revascularise the inferior myocardial regions. The postoperative morbidity was essentially due to respiratory complications: 6 patients had objective pulmonary complications and 11 patients had relative transient postoperative hypoxia. The early gastrointestinal complications were related to ulceration (5 patients) complicated by haemorrhage in 2 patients. This morbidity was responsible for a significant increase in the period of intensive care. The hospital mortality was 6.2%, observed mainly in the first third of the author's experience and in patients with preoperative poor prognostic factors. These relatively high morbidity and mortality rates have led to a cautious use of the right gastroepiploic artery for coronary bypass surgery in patients over 70 years of age, in those with unstable angina operated as an emergency and in those with associated pathology, especially respiratory problems. The choice of a complex surgical option should not be synonimous with an increased postoperative mortality.

Valvular tumors are uncommon and usually benign lesions, discovered accidentally or when neurological or cardiological complications occur. We report a case of mitral valve papillary fibroelastoma measuring less than 1 cm and revealed by stroke. Transesophageal echocardiography was the best method to establish the diagnosis as it provided higher discriminative power than the transthoracic echocardiography or nuclear magnetic resonance. The embolic risks justify the surgical treatment of these lesions while anticoagulation therapy can be suggested as a substitute to surgery for the high risk patients.

The authors report their experience of implantable defibrillators over a 5 year period. Between February 1988 and July 1992, 36 patients (25 men, 11 women, average age 51 +/- 11 years, range 18 +/- 71 years) underwent implantation of an automatic defibrillator with epicardial (n = 13, Group I) or endocardial leads (n = 23, Group II) without patch electrodes (n = 7), with subcutaneous patch electrodes (n = 12) or epicardial patch electrodes (n = 4). Three serious early complications were observed: 2 cardiogenic shocks in Group I, one of which died on Day 1 and one case of infection which required explanation of the defibrillator on Day 23 in Group II. Late complications in Group I included one case of disactivation of the defibrillator, 2 losses of output, one of which required replacement of the defibrillator and 2 increases of threshold treated by implantation of an endocardial lead. In Group II, 2 patients had inappropriate shocks due to overdetection (n = 1) and double counting (n = 1). During an average follow-up period of 28.5 +/- 9 months in group I and 13 +/- 6 months in Group II, 4 patients died, 2 from sudden death. Ninety seven shocks were delivered in 19 patients (56%), 5.1 shocks per patient. In the 17 patients with an antitachycardia function, 14 (82%) developed 947 episodes of VT treated successfully by antitachycardia pacing in 917 cases. This retrospective study confirms the efficacy of implantable defibrillators in the treatment of malignant ventricular arrhythmias. The efficacy of endocardial and epicardial leads seems to be the same but there seems to be a lower immediate mortality and morbidity with the endocardial system.

The purpose of this study was to compare the early postoperative effects of heart and heart-lung transplantation on the secretion of atrial natriuretic peptide (alpha-ANP), renin, aldosterone, and vasopressin. This was carried out from the first to the eighth postoperative day in ten heart and five heart-lung recipients. The changes in the release of these hormones were similar in both groups. Vasopressin release remained stable while that of the renin-angiotensin-aldosterone system progressively returned to more normal levels. Grafted heart tissue was capable of high alpha-ANP release early on in both heart and in heart-lung recipients. This sustained alpha-ANP release was not a function of the resulting overall atrial tissue mass. Our findings suggest that it might be the consequence of an intrinsic hypersecretion of alpha-ANP resulting from the loss of normal heart innervation occurring in both heart and heart-lung transplantation.

Due to the discovery and elaboration of alpha ANP, the heart can be considered a veritable endocrine gland involved in fluid and electrolyte homeostasis as well as blood pressure regulation. Nevertheless, the mechanisms regulating the synthesis and release of this new hormone are far from being understood. Heart-lung transplantation provides an interesting research model for evaluating the consequences of allograft denervation on alpha ANP synthesis and release, without the disadvantage of significantly increasing the overall atrial tissue mass, as observed in orthotopic heart transplantation. To appreciate better these consequences, we studied the changes in alpha ANP release following heart-lung transplantation. Five patients were included in the study. It began during the immediate postoperative period and went on to the 8th postoperative day. Alpha ANP levels were determined using radioimmunoassay. The postoperative course was characterized by a rapid significant increase in hormone levels as of the 6th postoperative day (45.6 +/- 5.5 fmol/ml). These findings were comparable to those found in a previous study involving orthotopic heart transplantation alone. Thus, the heart-lung group was also capable of high levels of alpha ANP release from the onset. However, this rapidly increasing release was not found to be correlated with the changes in hemodynamic parameters observed postoperatively (blood and cardiac filling pressures). Moreover, we observed no episodes of rejection which might explain the increased release of this hormone. Finally, the fact that increased alpha ANP release occurs despite a smaller increase in the overall tissue mass is more than noteworthy. In conclusion, as we found in our initial study involving heart transplant recipients, the sustained high levels of alpha ANP observed following heart-lung transplantation are in favour of a possible modulating role played by cardiac innervation on the release of this hormone.

Etiology and long-term prognosis were prospectively investigated in 155 consecutive patients (96 men and 59 women), aged 16 to 45 years, referred to our Neurosurgical Unit with cerebral transient ischemic attacks or infarction during the period 1978-1988. All patients underwent neurological and medical-cardiological evaluation, cerebral computerized tomography scanning, electrocardiogram, and laboratory tests. Two-dimensional echocardiography was performed in 123 cases (79%), cerebral angiography in 147 (95%). Atherosclerosis was the leading etiology occurring in 48 patients (31%). A cardioembolic disorder was considered the probable cause of ischemia in 8 cases (5.1%). Further possible etiologies were contraceptive pill assumption (5.8% of the total, but 15.3% within the female group), spontaneous arterial dissection (4.5%), migraine (4%), puerperium (2.6%), cervical trauma (2.6%), and other, more uncommon conditions. Despite extensive evaluation, the cause of cerebral ischemia remained unknown in 40% of cases. All patients received antiplatelet medication and 16 underwent surgery. The long-term outcome at a mean follow-up of 5.8 years was favorable: 91% of subjects resumed their work on a full or part-time basis.Etiology and longterm prognosis were prospectively investigated in 155 consecutive patients (96 men, 50 women) ages 16-45 years who were referred to the Neurosurgical Unit with cerebral transient ischemic attacks or infarction during the period 1978-88. All patients underwent neurological and medical-cardiological evaluation, cerebral computerized tomography scanning, electrocardiogram, and laboratory tests. 2-dimensional echocardiography was performed in 123 cases (79%), cerebral angiography in 147 (95%). Atherosclerosis was the leading etiology, occurring in 48 patients (31%). A cardioembolic disorder was considered the probable cause of ischemia in 8 cases (5.1%). Further possible etiologies were though to be: oral contraceptives (5.8% of the total, but 15.3% within the females), spontaneous arterial dissection (4.5%), migraine (4%), puerperium (2.6%), cervical trauma (2.6%), and other, more uncommon conditions. Despite extensive evaluation, the cause of cerebral ischemia remained unknown in 40% of the cases. All patients received antiplatelet medication and 16 underwent surgery. The longterm outcome at a mean followup of 5.8 years was favorable in that 91% of the subjects resumed their workload on a full or parttime basis.

Visual evoked potential recordings were examined in 45 liver cirrhosis patients with (n = 29) and without (n = 16) encephalopathy, in 15 normal volunteers, and in one patient with an opioid induced stupor state. Visual evoked potential parameters were classified on the basis of EEG recordings. Plasma concentrations of amino acids, octopamine, and ammonia were assayed in order to document the metabolic change of hepatic encephalopathy. Latencies and wave patterns recorded after flash stimulation differentiated the four degrees of the coma one from another according to EEG classification in the 29 patients with encephalopathy. In the group of 16 patients without clinical and EEG evidence of encephalopathy the visual potential recordings discriminated a group of patients (n = 10) in a preclinical stage of encephalopathy. Biochemical parameters and subsequent clinical observation of patients confirmed our judgement of a preclinical stage of encephalopathy. These results suggest that visual evoked potentials are a simple, suitable and objective method for differentiating the degrees of encephalopathy and for identifying the preclinical stage of encephalopathy.

Many cardiac disorders can cause acute cerebrovascular insufficiency. The spectrum of potentially embolic cardiac conditions is wide; early recognition may determine a definite change in the management and prognosis of patients. In recent years the relevance of echocardiography in the screening of patients with cerebral ischemia has been emphasized. In order to identify potentially embolic cardiac conditions, 180 consecutive non selected patients with cerebrovascular insufficiency, underwent a clinical cardiological evaluation and an echocardiogram. The study population included 132 men and 48 women; the mean age was 51.7 years (range 19 to 72 years). A technically adequate echocardiogram was obtained in 153 patients. In 131 patients echocardiography was negative; cardiac lesions were detected in 22 patients (14.4%): mitral stenosis in 2, calcified aortic stenosis in 1, valvular endocarditis vegetations in 3, dilatative cardiomyopathy in 2, hypertrophic cardiomyopathy in 4, mitral valve prolapse in 4, regional left ventricular diskynesia in 5, mitral anulus calcification in 1. Patients were divided into 3 groups according to the results of cerebral angiography: 68 patients with normal angiography (Group I), 54 patients with atheromasic lesions on cerebral angiography (Group II), 31 patients in whom cerebral angiography was not performed (Group III). A higher incidence of cardiac diseases was found in the patients of Group I. The lack of lesions on cerebral angiography and the presence of embolic high-risk cardiac conditions strengthened a causal relationship of the cardiac disorder with cerebrovascular insufficiency in 10 of the 23 patients. In the mean follow-up period of 18 months of these 10 patients who underwent cardiac surgery or anticoagulation, no further attacks of cerebrovascular insufficiency were observed.(ABSTRACT TRUNCATED AT 250 WORDS)

We report the occurrence of syncope in three healthy athletes during exercise stress testing. In two subjects the syncope occurred in the late recovery stage and was due to inhibition of sinus activity. In the third case a Mobitz type II A-V block developed during hyperventilation, which was followed by a complete A-V block with ventricular asystole and subsequent sinoatrial arrest. We discuss the probable pathophysiological mechanisms of these arrhythmias and their implications in sports practice.

Alcohol decreases myocardial contractility through direct, toxic effect. Ingestion of more than 150 g per day for more than 10 years carries a high risk of developing alcoholic cardiomyopathy. The discontinuance of alcohol intake--if put into effect early in the natural history of patients with alcoholic cardiomyopathy--commonly but not invariably results in remission of heart failure. In order to evaluate the left ventricular (LV) function and to find out a possible correlation between the degree of cardiac dysfunction and the severity of the morpho-functional aspects of alcoholic liver disease, 20 chronic alcoholic patients without clinical evidence of heart disease were examined. Echocardiography, systolic time intervals, mechanical polygraphic recordings and liver biopsy were obtained. According to the morphological alterations showed by the needle biopsy of the liver, we separated 12 patients with liver steatosis (Group I) from 8 subjects with alcoholic hepatitis and fibrosis. In Group I LVET, ICT, PEP/LVET indices and LV fractional shortening (delta %) were not statistically different from control subjects. Patients of Group II showed marked impairment of myocardial function, as revealed by significant ICT, PEP, PEP/LVET prolongation and by an equally significant reduction of fractional shortening of the LV. The noninvasive method has proved to be quite useful in detecting early LV dysfunction in asymptomatic chronic alcoholics and has revealed a correlation between the severity of the morphological involvement of the liver and the impairment of cardiac performance.

The Authors studied 11 uremic patients for whom it was necessary to make an arterio-venous (A-V) fistula for dialysis purposes. Before and after creation of the fistula, the following parameters were determined: arterial pressure (PA) heart rate (HR), peripheral vascular resistances (PVR), renin, adrenalin and nor-adrenalin blood levels. The patients were divided in two groups according to the increase of the systolic output (SO) and o the cardiac index (CI) after the fistula (1st group of 5 patients: less than 30% increase of these values as compared to those before the fistula; 2nd group: more than 30% increase. By using Wilcoxon distribution free test it was possible to demonstrate the existence of a different condition, statistically significant in the two groups. In the 1st group of older patients (P0.05) where the systolic pressure values were greater (P0.05) and the indexes of the mechanical events of the left ventricle were altered, there was an increase of heart rate (P0.05), a moderate reduction of PVR (P0.01) and a very small increase of the systolic output and cardiac index (P0.01). Furthermore, in the 1st group, a left ventricular hypertrophy was observed, except in one case whereas this condition was constantly absent in the patients of the 2nd group. Patients of the 1st group had high nor-adrenalin blood levels, which did not change after the significantly after the creation of the fistula. In conclusion, an A-V fistula provokes two different hemodynamic behaviours which are related to age, pressure values, myocardial performance, presence of a left ventricular hypertrophy, PVR variations, and renin and nor-adrenalin blood levels.

Emery-Dreifuss muscular dystrophy is an X-linked recessive condition characterized by mild muscular weakness predominantly in a humero-peroneal distribution with variable facial involvement. Onset is in childhood with slow progression of weakness. The disease is often associated with cardiac involvement, mainly with bradyarrhythmias which might be responsible for sudden death. The most striking finding derived from the literature is the high incidence of sudden death; in the 7 large families described, out of the 79 reported patients 32 died suddenly at a young age (between 25 and 56 years). We performed a cardiologic evaluation of 11 subjects of a large italian family with affected males in four generations: 5 affected males (3 adults and 2 boys), 3 carriers and 3 healthy relatives (2 females and 1 male). Supraventricular arrhythmias were documented either in the dystrophic males or in the carriers. There was no correlation between the severity of cardiac rhythm abnormality and the severity of muscular weakness in the affected males, 3 of whom required pacemaker insertion. All the carriers were free of muscular involvement, but showed arrhythmias of variable degree, in one case requiring pacemaker insertion. In conclusion our data indicate an extremely high incidence of bradyarrhythmias, sometimes serious, in patients with Emery-Dreifuss muscular dystrophy. Holter monitoring is therefore mandatory and electrophysiological study is sometime necessary. Because of the high risk of sudden death in adult patients, we recommend permanent pacemaker implantation even in asymptomatic subjects, as soon as bradyarrhythmias are detected.

Sixty-one consecutive patients, less than or equal to 40 years old, were hospitalized for cerebral infarction between 1977 and 1985. Evaluation included computed tomographic brain scan, arteriography, echocardiography, and blood tests. A probable migrainous infarction was diagnosed in six patients (10%) (all women with a history of migraine) who survived the initial stroke and were followed-up for an average of four years. In five patients the stroke occurred during a common migraine attack and in one patient during a classic migraine attack. The site of infarction was invariably the occipital lobe. During the follow-up, no subject had a further stroke. All six women had a permanent hemianopic deficit.

In patients with cerebral transient ischemic attacks or stroke myocardial infarction is the leading long-term cause of death. Despite the importance of coronary artery disease, patients with cerebrovascular insufficiency are seldom evaluated for the detection of ischemic heart disease and usually the cardiological evaluation is limited to the patients with angina or previous myocardial infarction. In order to identify asymptomatic coronary artery disease 74 consecutive patients with cerebral ischemia, and without symptoms or electrocardiographic signs of ischemic heart disease, underwent a maximal exercise treadmill test according to the Bruce protocol. An exercise Thallium myocardial scintigraphy was performed in patients with positive exercise test. A control group of 74 asymptomatic subjects underwent the same study protocol. The study population (Group I) included 57 men and 17 women; the age ranged from 22 to 72 years (mean age 54 years). An adequate exercise test was obtained in 67 patients. Exercise test was positive (ST-segment depression greater than or equal to 1.5 mm) in 19 cases (28%). The end points were exhaustion in 15 patients, ST-segment depression greater than 3 mm in 2 and systolic blood pressure greater than 240 mmHg in 2. The exercise Thallium myocardial scintigraphy was normal in 2 and abnormal in 17: reversible perfusion defects were detected in 12 cases and fixed defects in 5. In the control group (Group II), comparable for age and sex, exercise test was positive in 4 cases (5%; p less than 0.01 percentage of positive exercise tests in Group I vs Group II); the exercise myocardial scintigraphy was normal in 1 and abnormal in 3 subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

Prognosis of asymptomatic myocardial ischemia is largely unknown and the opportunity is still controversial of seeking for patients with silent ischemia. Aim of the present study is to evaluate the prognosis of painless myocardial ischemia documented by exercise test and myocardial scintigraphy. From June 1981 through November 1986, 206 patients without angina, history or ECG signs of old myocardial infarction, presenting a positive (decreases ST greater than or equal to 1.5 mm) exercise treadmill test, underwent exercise Thallium 201 myocardial imaging. Myocardial scintigraphy showed a normal scan in 85 cases and a reversible or fixed perfusion defect in 121. Patients with abnormal scan presenting ischemia at a low to moderate ergometric work-load were treated with betablockers or calcium-antagonist drugs. Out of patients with positive myocardial scintigraphy a sample of the first 100 consecutive subjects was considered. They were 87 men and 13 women aged 28-72 years (mean 54.8) observed during a mean follow up period of 33.1 +/- 1.6 months. Seven patients underwent coronary angiography which showed 3-vessel critical stenosis in 3 cases, 3-vessel lesions plus critical stenosis of the left main coronary-artery in 1 and 2-vessel lesions in 3. Two patients underwent coronary artery bypass surgery. A non fatal myocardial infarction occurred in 1 and 1 became symptomatic for angina, 11 and 20 months respectively after the diagnosis of ischemia. Three patients with ischemia at a low work-load and extensive scintigraphic perfusion defects died of sudden death and one of cancer.(ABSTRACT TRUNCATED AT 250 WORDS)

Electrocardiographic abnormalities associated with intracranial diseases, especially subarachnoid hemorrhage, are well known, while there is hardly mention of cardiac arrhythmias in the neurological and cardiological literature. In order to assess the incidence of arrhythmias 52 consecutive patients with subarachnoid hemorrhage secondary to ruptured aneurysm were investigated with 24-hour Holter recordings. Bradyarrhythmias and tachyarrhythmias were found in 46 patients (88%); premature ventricular beats in 25 pts (12 of these in 3rd-5th Lown classes), ventricular tachycardia in 2, premature supraventricular beats in 14, paroxysmal atrial fibrillation in 1, sinoatrial blocks and arrests in 18, atrioventricular dissociation in 2 and idioventricular rythm in 2. Moreover in 5 pts ST segment changes were found, suggestive of transitory acute myocardial ischemia. The presence and severity of arrhythmias were correlated with the time elapsed from the episode of bleeding, with the QT interval, and with the hemorrhage extent. Our results indicate an high incidence of arrhythmias in subarachnoid hemorrhage, sometimes serious mainly in early stage. Continuous electrocardiographic monitoring is therefore extremely useful and provides data for therapeutic consideration.