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In a series of 252 patients (47 with arteriovenous malformation, 205 with aneurysms), two patients had a coexisting intracranial aneurysm and an arteriovenous malformation. In one patient, the aneurysm was proximal to the main feeding arteries of the arteriovenous malformation. In the other patient, there was no anatomical relationship between the two lesions. These findings illustrate different views on the pathogenesis of the rare association of the two conditions.

Three accessory middle cerebral arteries are described in two out of 75 brains dissected under the operating microscope. Each artery originated from the anterior cerebral artery, lateral to its junction with the anterior communicating artery, followed the proximal segment (A1, pars precommunicalis) of the anterior cerebral artery, then the horizontal portion (M1, pars sphenoidalis) of the middle cerebral artery towards the lateral sulcus. Each supplied the lateral orbital gyrus, the gyrus longus of the insula, part of the putamen, the head of the caudate nucleus and the anterior limb of the internal capsule. The presence of the accessory middle cerebral artery is discussed with regard to the recurrent artery of Heubner (A. recurrens, A. centralis longa).

One case is presented whose characteristics features were breast hypertrophy, normal immune functions and the efficacy of cyclophosphamide alone : A complete remission is present 31 months after the granuloma started, after 7 and a half months of treatment by cyclophosphamide, 14 months after its interruption by the patient. It is proposed that lethal midline granuloma is a localised form of Wegener's granulomatosis ; between these extreme forms are the limited (pulomonary) form of Wegener's granulomatosis, and the lethal midline granuloma with cutaneous, glandular, digestive or urethrogenital involvements. Therapeutic is briefly reviewed and the efficacy of cyclophosphamide claimed.

A double-blind, randomized study was designed to evaluate the efficacy of oral propafenone and oral quinidine in suppressing premature ventricular complexes (PVCs). Twenty-five men were studied for 3 weeks. Twelve were randomized to the quinidine group and 13 to the propafenone group. Small doses of the drugs were administered for 1 week (200 mg of quinidine every 6 hours or 300 mg of propafenone every 12 hours) and large doses were administered for another week (400 mg of quinidine every 6 hours or 300 mg of propafenone every 8 hours). Strict criteria were used to define responders to antiarrhythmic therapy. For more than 85% reduction in total PVCs per hour: During the low-dose week, 36% in the quinidine group and 50% in the propafenone group were responders (difference not significant [NS]), while during the high-dose week 33% and 64% were responders (NS). For more than 95% reduction of ventricular couplets per hour: During the low-dose week, 45% in each group were responders, while during the high-dose week, 56% and 60% were responders (NS). For 100% abolition of ventricular tachycardia (VT) beats per 24 hours: During the low-dose week, 60% in the quinidine group and 56% in the propafenone group were responders (NS); during the high-dose week 80% and 67% were responders (NS). There was no significant difference in the 2 groups in incidence of side effects. This study shows comparable efficacy and tolerance of propafenone and quinidine for the control of ventricular arrhythmias in ambulatory patients with diverse forms of heart diseases.

Although the left ventricle is traditionally viewed as the heart's main pumping chamber, no correlation has been shown between left ventricular (LV) ejection fraction (EF) at rest and exercise capacity in patients with chronic LV failure. Because vasodilators with venodilating activity increase exercise capacity more than predominant arterial dilators in patients with LV failure, right ventricular (RV) function may relate to exercise capacity in these patients. In 25 patients with chronic LV failure, caused by coronary artery disease in 12 patients and idiopathic dilated cardiomyopathy in 13 patients, RVEF and LVEF at rest were measured by radionuclide angiography. Maximal upright bicycle exercise testing was also performed to determine maximal oxygen consumption, which averaged only 13 +/- 4 ml/min/kg. The LVEF at rest was 26 +/- 10% and did not correlate with maximal oxygen consumption (r = 0.08). However, the RVEF was 41 +/- 12% and correlated with maximal oxygen consumption (r = 0.70, p less than 0.001) in the same patients. The correlation was stronger (r = 0.88) in patients with coronary artery disease than in those with idiopathic dilated cardiomyopathy (r = 0.60). Thus, RVEF at rest is more predictive of exercise capacity than LVEF in the same patients with chronic LV failure. These results are consistent with the clinical observation that only venodilating agents increase exercise capacity of patients with chronic LV failure.

The absence of the internal carotid artery (ICA) is a very rare anomaly. Since the first description of the condition in 1787, only 60 cases have been reported in the literature. We report two cases of absence of the left ICA amongst 2195 carotid angiograms performed in the Department of Neuroradiology at the Royal Brisbane Hospital over a period of 4 years. The first patient was suffering from obstructive hydrocephalus; the second from right cerebellopontine angle syndrome. Cerebral angiography by catheterization of the aorta revealed the absence of the left ICA. The collateral circulation to the left cerebral hemisphere was provided by the vertebrobasilar system via a dilated left posterior communicating artery. In the first patient, the absence of the ICA was coincidental with other congenital anomalies of the cervico-occipital junction. In the second patient, the dilated vertebrobasilar system was responsible for compressing the right cranial nerves VII and VIII. It is emphasized that in some cases of cerebellopontine angle syndromes, thorough investigation of the cerebral vessels is necessary.

A case of gastric tuberculosis associated with villous atrophy is reported in a sixty-three years old female. Fifteen years before she had a skin-disease whose histology was compatible with a dermatitis herpetiformis. Under treatment by gluten exclusion and anti-tuberculous drugs, a cure of gastric tuberculosis and a dramatic relapse of jejunal morphology were observed.

Pneumocystis carinii pneumonia can only be diagnosed once the microorganism is demonstrated. The authors review and illustrate various staining technics, and discuss for each of them specific advantages. All samples must by systematically examined both by phase contrast microscopy and by light microscopy after Gomori-Grocott and Gram-Weigert stains. This morphological study is completed by ultrastructural photographs.

Chronic cor pulmonale is defined as right heart hypertrophy and/or chronic right heart failure. There are many etiologies, but the common cause is increased right heart work from pulmonary hypertension. Etiology can be conveniently discussed by assuming two prototypes, the asphyxial or hypoxic type and the vascular obliterative type. A common cause of the asphyxial type is chronic obstructive pulmonary disease, and the obliterative type is represented by chronic pulmonary thromboembolic disease or primary pulmonary hypertension. Pathology is discussed, emphasizing the cardiac manifestations of chronic cor pulmonale including data of specific cardiac chamber size. An overview of hemodynamics is given, and the use and limitation of electrocardiography and chest x-rays are discussed. The exciting potential use of echocardiography for the serial non-invasive measurement of anatomical and pathophysiological features is outlined, along with the value of a careful physical examination and the proper utilization of laboratory tests in the diagnosis of chronic cor pulmonale. In the patient with the asphyxial type, the treatment of pulmonary infectious exacerbations, the role of corticosteroids, digoxin, diuretics, phlebotomy, bronchodilators (theophylline, beta adrenergic agonists, and anticholinergics), and long-term oxygen therapy is noted. The controversy surrounding the use of vasodilators and calcium blockers in these patients is discussed. Treatment aspects of the vascular obliterative type, including the role of vasodilators, calcium blockers, prostacyclin, anticoagulants, and overall strategy are discussed. A brief note is mentioned of the promising role of surgical therapy in chronic thromboembolic disease causing chronic cor pulmonale.

Thirty-two men with chronic ventricular arrhythmias responded to propafenone, a new potent antiarrhythmic agent, in short-term trials with 85% or greater reduction of total ventricular premature complexes (VPCs) per hour, 95% or greater reduction of ventricular couplets (VCs) per hour, and 100% abolition of ventricular tachycardia (VT) beats per 24 hours. These patients were continued on long-term propafenone therapy to assess sustained therapeutic efficacy and safety. Thirty patients completed 1 year and 26 patients completed 2 years of testing with this agent; one patient died of sudden death and another died of a noncardiac cause. Although there were significantly fewer patient responders at 1 and 2 years, the majority of patients (greater than 79%) continued to respond optimally to propafenone. Side effects were minor and included bitter taste, dizziness, congestive heart failure, fatigue, and significant prolongation of the PR and QRS intervals. Propafenone has sustained antiarrhythmic efficacy after 2 years without serious toxicity.

The effects of orally administered propafenone on ejection fraction (EF) determined by radionuclide angiography were studied in 2 groups of patients receiving different dosing regimens. Fourteen group A patients had no clinical evidence of left ventricular (LV) dysfunction and were not receiving digoxin therapy. In this group a mean daily dosage of 879 mg resulted in a decrease in resting LVEF from 52 +/- 9% to 48 +/- 11% (p less than 0.05). Eight group B patients had clinical radionuclide evidence of LV dysfunction and were receiving digoxin therapy. In this group, a daily dosage of propafenone of 600 mg/day resulted in no significant change in LVEF. No clinically significant effects on cardiac compensation were evident in either group. These data suggest a negative inotropic effect that is either related to propafenone dosage or at least partially attenuated by digoxin therapy. Further studies are necessary to define precisely the effects of propafenone on LV function.

The second observation of hemoglobin Pyrogos is reported. This abnormality was initially described in a Greek family, in our case it concerns an African negro originating from the Republic of Mali. The abnormal hemoglobin was without clinical or hematological consequences. The structural defect is a substitution of an Asp for a Gly in the immediate vicinity of lysine beta 82. This leads to a large inhibition of the corresponding tryptic cleavage and therefore to difficulties in the determination of the mutation. A second feature is a slight modification occurring near one of the 2.3 DPG binding site. As a consequence, the regulatory effect of this organic phosphate is smaller in the purified and stripped component than on hemoglobin A.

Use of total 12-lead QRS electrocardiographic voltage as a criterion for left ventricular (LV) hypertrophy has been of recent interest. Although upper and lower limits of QRS voltage for individual electrocardiographic leads have been reported in clinically healthy men and women, the upper limit of total 12-lead QRS voltage has not been established in adults free of cardiopulmonary disease by clinical and necropsy criteria. Therefore, the total QRS voltage from all 12 electrocardiographic leads was determined in 30 autopsied men known to be free of cardiopulmonary disease by clinical assessment and by a special cardiac examination using postmortem coronary angiography and chamber partition determination of LV weight. Gross heart weight, LV weight and total QRS voltage are reported. Comparisons were made between disease-free patients and previously reported patients with aortic valve stenosis, aortic regurgitation and cardiac amyloidosis with respect to total QRS voltage and gross heart weight. Total QRS voltage and gross heart weight were significantly greater in patients with severe aortic stenosis (mean 245 mm) and severe aortic regurgitation (mean 274 mm) than in our patients (mean 127 mm). Total QRS voltage was significantly less, whereas gross heart weight was significantly greater in patients with cardiac amyloidosis (mean 101 mm) than in our normal subjects (mean 127 mm). These data provide a basis for evaluating the total 12-lead QRS voltage as a criterion for LV hypertrophy.

The present investigation was designed to determine if acute ischemic cardiac injury causes the release of atrial natriuretic factor (ANF). Seventeen patients with acute myocardial infarction but without clinical evidence of congestive heart failure had their circulating concentration of ANF and creatine phosphokinase monitored daily for 14 days. All 17 patients had an elevated plasma ANF concentration at time of presentation. Maximal increase in ANF was on day 2 and 3 post-infarction. This maximal increase correlated with the size of infarction estimated by the maximal creatine phosphokinase concentration (r = 0.475; p less than 0.05), but did not correlate with the amount of left ventricular dysfunction. ANF began to decrease by day 4 post-infarction and was normal at 10 days post-infarction in 14 of the 17 (82%) patients. At 12 days post-infarction, all 17 patients had normal ANF levels. Another three patients with acute myocardial infarction were treated with tissue plasminogen activator (tPA). The measured ANF levels in these patients were within our normal range and were significantly lower (p less than 0.001) than those seen in patients with acute myocardial infarction not given thrombolytic therapy. Six patients with unstable angina likewise had normal circulating ANF concentrations during prolonged episodes of chest pain. These levels were also significantly lower (p less than 0.001) than the 17 patients with acute infarcts not given tPA. The distinct pattern of release of ANF may be useful as an adjunct to serum cardiac enzymes in determining if a myocardial infarction has occurred.

The surface electrocardiogram remains an insensitive method for detection of ventricular hypertrophy. Technical problems related to body size and habitus and distance from the heart cannot be overcome. Coronary arterty disease and amyloidosis, although frequently associated with hypertrophy, tend to obscure the electrocardiographic changes because of the attendant loss of voltage. The progress made in the last 20 years is due primarily to re-evaluation of traditional criteria in terms of careful anatomic correlation. The studies cited have the advantage of using specific clinical diagnoses in a defined population, specific chamber weights, and a 97.5 percentile confidence level for distinguishing normal pathologic and electrocardiographic data from abnormal. They are limited because the results may not apply to females or patients with mitral stenosis and congenital heart disease. In general, the electrocardiogram can be expected to detect left ventricular hypertrophy in six out of ten patients with the disease, and will misdiagnose the problem in about one out of every ten without the disease. Methodology using multiple criteria will achieve the best sensitivity and specificity. Several methods are available and of comparable accuracy. Simplicity of these methods varies widely and will be a factor in the choice of the method selected. The electrocardiogram will perform best in the population of patients with hypertension and aortic stenosis or regurgitation and have its greatest limitation in patients with coronary artery disease and myocardial infarctions. Echocardiography is proven to be more sensitive than the electrocardiogram for detection of left ventricular hypertrophy. Sensitivity is around 90 per cent with 95 per cent specificity. Its major limitations lie in the expense as compared to the electrocardiogram and in inadequate image resolution in a small proportion of patients. In order to achieve the results reported by centers proficient in this technique, careful attention must be paid to precise standardization of measurements and selection of images to be measured. When this is done the echocardiogram certainly offers a distinct advantage over the electrocardiogram in detecting left ventricular hypertrophy. We recommend the use of left atrial abnormality as a criterion to diagnose left ventricular hypertrophy when there is right bundle branch block. When left bundle branch block is present on the electrocardiogram, traditional criteria are probably no more accurate than the bundle branch block itself.(ABSTRACT TRUNCATED AT 400 WORDS)