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Four patients referred for surgical treatment of aortic stenosis presented an unexpected extremely calcified (porcelain) ascending aorta at the intraoperative epiaortic ultrasonography scanning. In each patient, replacement of the aortic valve was successfully performed using a sutureless implantable bioprosthesis during a short period of hypothermic circulatory arrest. In the era of transcatheter aortic valve implantation procedures, the sutureless valve may be a valuable option for surgical units that do not dispose of transcatheter technology or a hybrid operative theatre.

Unlabelled: Dilated cardiomyopathy is a heart muscle disease of unknown aetiology, characterized by left ventricular dilatation and impaired systolic function. Data on the incidence and prevalence of the disease is ambiguous, due to geographic variations, patient selection and the diagnostic criteria adopted., Methods: All the post-mortem and clinical cases observed in a consecutive series of 5252 patients resident in Trieste during the period November 1987-November 1989 were studied., Results: Incidence of the disease discovered at autopsy was estimated at 4.5/100,000/year (24 cases), while clinical incidence in the same period was 2.45/100,000/year (13 cases). This is a total incidence of 6.95/100,000 new cases a year. A possible family history of heart muscle disease was found in three patients (12.5%). In 15 patients (62.5%) deaths were due to cardiological complications. Endocardial thickening (P = 0.03), fatty infiltration (P = 0.01) and arterial involvement (P = 0.04) were found more frequently in older patients (> 65 years)., Conclusions: The study confirms that dilated cardiomyopathy in Europe has a higher incidence than previously suggested and emphasizes the need for greater diagnostic sensitivity, particularly since pharmacological treatment is now so effective.

The application of molecular genetics in cardiology is currently producing important results in the study of the pathogenetic mechanisms underlying cardiomyopathies. Recent clinical surveys have indicated that genetic factors play a major pathogenetic role in idiopathic dilated cardiomyopathy (IDC). Familial IDC is frequent (20-30%) and is probably a heterogeneous entity, as suggested by the clinical variability and the different pattern of inheritance in the affected families. Molecular genetic studies have demonstrated the existence of heterogeneity also at the genetic level. In a series of families with X-linked IDC, the disease gene has been identified as the dystrophin gene. In familial right ventricular cardiomyopathy (or right ventricular dysplasia), a new nosological entity characterized by isolated right ventricular involvement that can mimic IDC, the disease gene has been localized in the long arm of chromosome 14. In families with matrilineal transmission, the cardiomyopathy could be linked to mitochondrial DNA alterations. Autosomal dominant familial IDC, considered to be the most frequent form, is currently under active investigation. Our preliminary data have excluded a large series of candidate genes, among which are the cardiac beta-myosin heavy chain and several other genes encoding for cardiac contractile proteins, genes of the HLA region, and about 60 genes involved in the immune regulation.

In a prospective study on 340 cases with primary myocardial disease, eight patients (six males, two females, mean age 36 years, range 24-47) with an idiopathic left ventricular aneurysm were observed. All patients had normal coronary arteries, no angina or history of myocardial infarction; all but one had no risk factors for ischaemic heart disease; all had normal right ventricles; one patient had a history of familial dilated cardiomyopathy, two of 'flu-like' syndrome at the time of first symptom and two of alcohol abuse. All patients had ventricular tachycardia (VT), five sustained (of right bundle branch block morphology in three, and of different morphologies in two), three non-sustained. Patients with sustained VT had inducible VT (resembling the clinical one) on electrophysiological study. Electrocardiogram (ECG) showed an infarction pattern in three cases. Aneurysms were of limited size (2.1 +/- 1/11 segments on echocardiography) and were located in the septum, apex or posterior wall. Left ventricular ejection fraction (LVEF) was reduced (< 0.50) in six patients and was not correlated with the aneurysm size. The duration of illness was inversely correlated with LVEF (P < 0.05). Endomyocardial biopsy showed evidence of diffuse pathological changes in all cases (cell hypertrophy, myofibrillar lysis, mitochondriosis). During follow-up (64 +/- 32 months), patients were successfully treated with anti-arrhythmic drugs: no patients required surgical treatment to control ventricular arrhythmia. Considering the clinical and pathological features of idiopathic left ventricular aneurysm, this primary myocardial disease could be classified as a novel peculiar form of cardiomyopathy.

Right ventricular cardiomyopathy is generally considered a disease with isolated right ventricular involvement, although there are some case reports of concomitant abnormalities of the left ventricle. In order to evaluate the prevalence, characteristics and natural history of left ventricular involvement in this disease, 39 patients with right ventricular cardiomyopathy were studied by 2-dimensional echocardiography. At first evaluation 25 patients (group 1) showed right ventricular abnormalities and a normal left ventricle. In the remaining 14 patients (group 2) the right ventricular abnormalities were associated with left ventricular involvement (asynergic areas and/or mild diffuse hypokinesia). During the follow-up, left ventricular involvement appeared in 6 patients of group 1 and worsened in 4 patients of group 2. Five patients died. Pathological examination (available in 4 cases) showed, at the level of the right ventricle, marked atrophy of myocytes and fibro-fatty replacement, and predominant fibrosis at left ventricular level. Left ventricular involvement is thus frequent in right ventricular cardiomyopathy. At least in some cases the disease appears to be progressive.

Many studies on the natural history of dilated cardiomyopathy show high probability of death or of cardiac transplantation in a large percentage of patients. These studies have several methodological limitations. Our prospective study, carried out from 1971, and which evaluated 120 patients, showed improved survival in more recent years. Survival 3 years after diagnosis changed from 30% (1971-6/1981) to 88.4% (7/1986-1/1989). Thirty patients were investigated by haemodynamic exercise test to assess their haemodynamic behaviour during exercise, to evaluate the effects of pharmacological treatment and to define parameters of prognostic value. Different haemodynamic behaviours were observed. Haemodynamic investigation during exercise is useful to assess the effect of treatment and may have prognostic value. Myocarditis presents a spectrum of clinical symptoms and echocardiographic abnormalities. In patients with congestive heart failure left ventricular dysfunction is common. Patients with atrioventricular block or chest pain usually have good left ventricular function. During follow-up, improvement is possible but persistent left ventricular dysfunction is associated with a high mortality rate. When left ventricular function is good at presentation and does not deteriorate during follow-up the prognosis is good.

The characteristics of left ventricular (LV) involvement in 31 patients with right ventricular cardiomyopathy (RVCMP) were studied by two-dimensional echocardiography (2D echo). Two groups were identified: in group 1 (19 patients) the LV appeared normal initially, but became abnormal in four patients during follow-up. LV involvement was present at diagnosis in the 12 patients of group 2; LV function worsened in one of them. In conclusion, LV involvement is frequent in RVCMP. At least in some cases the disease appears to be progressive.

While coronary reperfusion arrhythmias are well defined in animal models, their prevalence and predisposing factors are still uncertain during pharmacologic coronary reperfusion in patients with acute myocardial infarction. In 80 patients, who underwent this procedure and had a continuous electrocardiographic monitoring (associated with a Holter recording in 34), 6 patients developed ventricular fibrillation. Ventricular fibrillation was the terminal event in 2/19 non-reperfused patients. The other episodes of ventricular fibrillation (controlled with DC countershock) occurred within 5 minutes and 3 minutes after angiographic documentation of coronary reperfusion. Ventricular fibrillation was observed in 4/10 patients with a residual stenosis less than or equal to 80% but in no case among the 39 patients with a residual stenosis greater than 80% (P = 0.01). Holter recordings in 19 reperfused patients showed ventricular tachycardia or accelerated idioventricular rhythm at reperfusion in 8/10 patients with a coronary stenosis less than or equal to 80% and only in 3/9 with a higher degree stenosis (P less than 0.05). Reperfusion ventricular fibrillation was found to occur in 8.1% of patients. A residual stenosis of 80% or less was found to be a predisposing factor in the occurrence of ventricular fibrillation, ventricular tachycardia or accelerated idioventricular rhythm at reperfusion.

In order to investigate the anatomic substrate of 'idiopathic' ventricular tachycardia (VT) 10 patients with chronic recurrent VT and no apparent sign of heart disease underwent an echocardiographic, haemodynamic and histologic study (5 males, 5 females: mean age = 40 +/- 11 years). In the patients with a left bundle branch block morphology of VT (7 cases), four showed findings compatible with an arrhythmogenic right ventricular dysplasia or a right ventricular cardiomyopathy. In the other three all examinations were normal with the exception of endomyocardial biopsy, which showed slight non specific changes in two. Of the remaining 3 cases (characterized by a right bundle branch block morphology of VT or by the presence of polymorphic VT) one had histologic evidence of myocarditis while another developed dilated cardiomyopathy. Macroscopic and/or microscopic ventricular abnormalities are frequently found in patients with VT which appears idiopathic. In these cases myocardial disease is frequently progressive, despite optimal control of VT.

To determine whether afterload reduction with nifedipine is effective both at rest and during stress tests (rapid atrial pacing and contrast material overload), 14 patients with chronic severe isolated aortic insufficiency (10 asymptomatic) underwent right and left cardiac catheterization. Forty-five minutes after 20 mg of nifedipine (sublingually), left ventricular end-diastolic pressure, peak aortic pressure, systemic vascular resistance and double product decreased significantly at rest, at peak paced rate, and after angiography (P values from less than 0.05 to less than 0.001). Cardiac index increased at resting heart rate (P less than 0.01) but was unchanged during pacing. The reduction of systemic vascular resistances was inversely correlated with its initial value (r = -0.69). After nifedipine, average regurgitant fraction did not change; however its variations were significantly correlated with those of systemic vascular resistance (r = 0.69). It is concluded that in severe aortic insufficiency, nifedipine induces an effective reduction of left ventricular pre- and afterload, accompanied by an enhanced mechanical efficiency (unchanged or increased cardiac index with lower double product), both at rest and during stress tests.

Sixty-five patients with dilated cardiomyopathy underwent 24 hour electrocardiographic monitoring: 62 (95.4%) showed ventricular arrhythmias and 52 (80%) complex ventricular arrhythmias (multiform ventricular extrasystoles, paired ventricular extrasystoles and ventricular tachycardia). Complex ventricular arrhythmias correlated significantly with some haemodynamic indices of ventricular dysfunction: patients with multiform and paired ventricular extrasystoles and with ventricular tachycardia had lower values of ejection fraction (31.9 +/- 11.8%, P = 0.002) and of cardiac index (2.9 +/- 0.7 litres/min/m2, P = 0.029) than the others (41.1 11.1% and 3.5 +/- 0.9 litres/min/m2 respectively). Patients were followed for a period of 30 +/- 18 months (20 days to 64 months). During follow-up 19 died and mortality was higher among patients with multiform and paired ventricular extrasystoles and/or ventricular tachycardia. Complex ventricular arrhythmias are frequent in dilated cardiomyopathy: ventricular tachycardia and multiform and paired ventricular extrasystoles seem to be related to a more depressed ventricular function and to a poor prognosis. The importance of antiarrhythmic treatment in these patients has still to be evaluated.