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Background: The natural history of idiopathic dilated cardiomyopathy (IDC), once a disease with a dire prognosis, is thought to be changing. Aim of this study was to describe the clinical characteristics, long term course and prognostic factors of IDC patients followed up prospectively since the late eighties., Methods: Patients with a diagnosis of IDC confirmed by normal coronary angiography, non specific endomyocardial biopsy findings and a left ventricular ejection fraction below 50% were consecutively enrolled in a multicenter registry and followed up at 6-months intervals., Results: From January 1986 till January 1994, 441 IDC patients with a mean age of 43 +/- 13 years (range 8-68) entered the registry. Thirty per cent of patients were women and 8% had familial dilated cardiomyopathy. NYHA class was I-II in 77% and 35% of patients were asymptomatic at the time of diagnosis. Treatment included digitalis in 235 patients (53%), diuretics in 239 (54%), angiotensin converting enzyme inhibitors in 269 (61%), betablockers in 108 (24%). Chronic atrial fibrillation was detected in 10% of patients and left bundle branch block in 24%. Mean cardiothoracic ratio was 0.54 +/- 0.06. Mean left ventricular end diastolic dimension was 38 +/- 6 mm/m2; 48% of patients had minimal or mild left ventricular dilatation. Mean left ventricular ejection fraction was 30 +/- 10%. At Holter monitoring 67% of cases had complex ventricular arrhythmias, 37% had ventricular tachycardia and 4% had advanced atrioventricular block. Mean exercise stress test duration was 9 +/- 4 minutes. After a mean follow up of 31 +/- 24 months, 337 patients were alive without transplantation and 5 were lost to follow up; 60 patients (14%) had died of cardiac causes, namely heart failure (6%), sudden death (7%) and pulmonary embolism (< 1%) and 30 had been transplanted (7%), while 4 had died of unclear causes. Survival and transplant-free survival were 94% and 90% at 2 years and 82 and 76% at 5 years, respectively. At multivariate analysis pulmonary capillary wedge pressure (p = 0.0001, odds ratio, for values > 15 mm Hg, 2.05) and betablocker treatment (p = 0.002, odds ratio 0.26) were independent predictors of survival., Conclusions: In this large, multicenter prospective study, prognosis of IDC in the eighties appears to be improved. Early diagnosis, together with improved medical treatment, probably bears a causal relation to these changes.

We studied the hemodynamic effects of dobutamine in 10 patients with severe chronic heart failure due to congestive cardiomyopathy in 5, to ischemic heart disease in 4 and to hypertensive cardiomyopathy in 1. Dobutamine was injected during three periods of 30 min each alternated with equal periods of placebo at doses of 2,5-5 and 7,5 mcg/Kg/min respectively. The most favorable hemodynamic effects, obtained at an infusion rate of 5 mcg/Kg/min, was characterized by a significant inn 4 and to hypertensive cardiomyopathy in 1. Dobutamine was injected during three periods of 30 min each alternated with equal periods of placebo at doses of 2,5-5 and 7,5 mcg/Kg/min respectively. The most favorable hemodynamic effects, obtained at an infusion rate of 5 mcg/Kg/min, was characterized by a significant increase of the cardiac index and of the left ventricle stroke work index, accompanied by a significant decrease of the left ventricular filling pressure and of the systemic and pulmonary vascular resistance. The hemodynamic monitoring showed that the pharmacological effects of the drug subsided about 5-10 min after the interruption of infusion. At the infusion rate of 7,5 mcg/Kg/min we observed a significant increase of premature ventricular beats in 3 patients. We conclude that dobutamine at the dose of 5 mcg/Kg/min shows a powerfull positive inotropic action not accompanied by apparent side effects.

The study of 46 patients with frequent anginal episodes characterized by S-T elevation (so called "variant angina pectoris") demonstrated that this type of electrocardiographic pattern does not characterize a homogeneous group of patients. In fact, while in some patients angina occurred only at rest, in others it occurred also on exercise. Sometimes ecgraphic alterations characterized by S-T depression were observed on the same leads which on other occasions had shown S-T elevation. The angiographic picture revealed: absence of significant coronary alterations in 10% of cases, stenosis greater than 75% in one main branch in 29%, in two branches in 39% and in three branches in 22% of cases. The hemodynamic monitoring carried out on 14 of these patients demonstrated that the ecgraphic modifications occur before the onset of the hemodynamic parameters which control myocardial O2 consumption. This suggests a primitive reduction of regional myocardial blood supply as a cause of the ischaemic episodes. The study of the regional myocardial perfusion with 201Tl technique in 6 patients confirmed this hypothesis. Coronary angiography carried out during an ischemic episode showed that the reduction of myocardial blood supply was caused by a spasm of a large coronary artery involving a long segment of the vessel, reversible by nitroglycerin administration. Aorto-coronary by-pass operation performed on 6 patients was followed by the disappearance of pain in two patients, even though the "by-pass" patency was angiographically proved in two patients.

Purpose of this study was to evaluate the capability of M-mode echocardiography (M-mode) and of 201Thallium myocardial scintigraphy (201T1) to detect transient myocardial ischemia at rest in man. Fourteen male patients (pts) were studied by the two techniques under basal conditions and during episodes of angina at rest. Data were obtained in 2 pts in rapid succession during the same episode, in the remaining 12 pts in different episodes with electrocardiographic changes similar in site, type, entity and duration. Echocardiographic criteria of transient ischemia (reversible asynergies and transient reduction of systolic thickening) were present in 9/11 pts during episodes of ST segment elevation; the scintigraphic ones (reversible perfusion defects) in 10/11 pts. In 3 pts during episodes of ST segment depression M-mode was positive in 2, 201T1 in all three. The poor acoustic window in 2 pts and the involvement in one of left ventricular lateral wall not seen by M-mode on parasternal approach, may account for the 3 false negatives of M-mode. The single false negative of 201T1 can be related to the short duration (1') of the episode. A complete correspondence of site of ischemia, detected by ECG, M-mode and T1, was observed. In conclusion, M-mode appears a reliable technique for detecting transient ischemic episodes, being the main limits the acoustic window and the incapacity in exploring all ventricular walls and the main advantage the high temporal resolution, which permits to study continuously the full sequence of the ischemic event. On the other hand 201T1 does not allow detection of episodes of short duration. This two techniques, when combined in anginal patients, provide complementary information on perfusion and contraction respectively. The reliability of these techniques in the assessment of transient myocardial ischemia is the basis for their possible diagnostic use in pts with transient specific ECG changes or with typical anginal pain without ECG evidence of ischemia.

Radiolabeled human albumin microspheres (HAM) have been used in the past, in order to visualize coronary flow distribution in man. In order to obtain information both on myocardial perfusion and contractility, we applied R-wave synchronized reconstruction of the cardiac cycle in cine mode to HAM imaging. In 20 patients with angina pectoris and coronary stenosis of variable severity and in 1 patient with congestive cardiomyopathy we injected 4 millions of HAM (mean diameter 15 microns) labeled with 15-20 mCi of 99mTc, into the left ventricle during routine cardiac catheterization. Data were collected by a gamma camera, and by the use of a high resolution collimator and were electronically restricted to the cardiac area (64 X 64 matrix). The reconstruction of the cardiac cycle was made according to the technique developed in our laboratory for gated blood pool imaging. From the displayed images we evaluated myocardial flow distribution at the time of injection and regional wall motion (by cine mode) at any subsequent time, with a temporal resolution of 2 to 4 mins. The patterns of contractility of myocardial walls with normal or reduced perfusion were in agreement with those observed by means of contrast ventriculography. Advantages of the technique are: a) simultaneous information on flow and contractility; b) prolonged time for wall motion observation (4 to 6 hours); c) high quality pictures, since the signal to background ratio is much more favourable than with other isotope techniques. The technique appears to be promising from the results obtained in the number of patients included in this study.

Unlabelled: In the past ten years we studied 80 patients with angina at rest by 201-Thallium perfusion scintigraphy. According to ECG changes during episodes of transient ischemia at rest, the patients were divided into three groups. Thirty six patients showed transient ST segment elevation (Group 1); 33 ST segment depression (Group 2) and 11 normalization of negative T waves (Group 3). 201-TI scintigraphy was performed during spontaneous or ergonovine induced episodes of ischemia and at redistribution. Group 1 showed localized and severe perfusion defects, well correlated to the site of ECG changes. Group 2 showed more diffuse and less severe perfusion defects, less correlated to the site of ECG changes. Group 3 showed perfusion defects similar to those observed in Group 1 and associated in 54% with basal perfusion defects due to previous myocardial infarction., In Conclusion: A) three main perfusion patterns are associated with the three types of ECG changes; B) relative to ECG, myocardial scintigraphy provides a more accurate definition of the site and extension of ischemia, particularly in Group 2 patients.