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We present the case of a patient who came to our attention for enlargement of the cardiac silhouette on chest X-ray. Echocardiography showed moderate diastolic overload of both ventricles with enhanced cardiac output without valvular disease or cardiac shunt that could account for this cardiomegaly. A subsequent abdominal echocardiographic exploration showed an angiomatous transformation of the liver due to diffuse lacunar enlargement of hepatic portal vein branches and arterial-venous shunts. Computed tomography and magnetic resonance imaging confirmed the echocardiographic findings. The imaging findings coupled with cutaneous and nasopharyngeal lesions were suggestive of hereditary hemorrhagic telangiectasia (HHT) and the diagnosis was confirmed after the identification of a mutation in the ACVRL1 gene on chromosome 12. HHT is a rare but underestimated vascular disease that can affect different organs, in particular the liver, leading to organ failure requiring transplantation as occurred in our patient. Echocardiography is a useful imaging tool to exclude cardiac abnormalities as a cause of cardiomegaly and to guide the correct diagnosis of a peripheral origin of high cardiac output.

The echocardiographic examination is generally performed in patients with heart failure and it often gives a significant contribution to the differential diagnosis. Firstly, the evaluation of left ventricular pump function by measuring the ejection fraction (EF) can distinguish patients with heart failure into two different groups, with depressed or preserved EF. The most frequent causes of heart failure and depressed EF are coronary artery disease, idiopathic dilated cardiomyopathy (DCM) and hypertensive heart disease. Although the echocardiographic features of coronary artery disease versus idiopathic DCM may be similar, the demonstration of inducible ischemia at dobutamine echocardiographic test suggests the presence of significant coronary artery disease and may be useful in the selection of cases for coronary arteriography. The association of left ventricular hypertrophy, hypokinesis and, sometimes, significant dilation is compatible with hypertensive heart disease or end-stage hypertrophic cardiomyopathy. No useful echocardiographic findings can identify the patients with genetic DCM or affected by myocarditis from other cases with idiopathic DCM. Some advanced cases of right ventricular dysplasia/cardiomyopathy may show a biventricular involvement and mimic DCM; these patients are usually characterized at echo by predominant right ventricular dilation and multiple a-dyskinetic bulges in the absence of pulmonary hypertension. Very difficult to manage are the patients with significant left ventricular dysfunction and severe valvular heart disease (such as aortic stenosis or mitral regurgitation). According to the literature, the left ventricular systolic function is relatively preserved (EF > 40%) in 30-40% of patients with heart failure. In these cases a diastolic dysfunction may be hypothesized. Echo-Doppler evaluation can be helpful in the recognition of signs of increased left ventricular stiffness ("restrictive filling pattern") and of increased filling pressures. In the differential diagnosis one must first consider the most frequent heart disorders that may present with this clinical syndrome, coronary artery disease and hypertensive heart disease. Furthermore, other less common diseases characterized by heart failure due to predominant diastolic dysfunction are the following: hypertrophic and restrictive cardiomyopathies, infiltrative heart diseases, such as amyloidosis, and constrictive pericarditis. Restrictive cardiomyopathy is characterized by heart failure and preserved left ventricular EF in the absence of significant ventricular dilation and hypertrophy; typical, although not pathognomonic, echocardiographic features are atrial enlargement ad restrictive filling pattern. In distinguishing constrictive pericarditis from restrictive cardiomyopathy useful Doppler signs are the wide respiratory variability in flow velocities at mitral and tricuspid levels, due to increased ventricular interdependence caused by the presence of an abnormally rigid pericardium.

Forty-three patients (40 men and 3 women, mean age 54 +/- 9 years) with coronary artery disease underwent 99mTc methoxy isobutyl isonitrile (sestamibi) myocardial scintigraphy and coronary arteriography. Sestamibi uptake and wall thickening index (WTI) were quantitatively evaluated in each myocardial segment. Segments were divided into group 1 (normal coronary arteries, no. = 94), group 2 (coronary artery stenosis 50-99%, no. = 79), and group 3 (coronary artery stenosis 100%, no. = 42). Group 3 segments were subdivided into group 3A (with collaterals, no. = 18) and group 3B (without collaterals, no. = 24) segments. Both sestamibi uptake and WTI were significantly lower (p < 0.01) in group 3 than in groups 1 and 2. However, only WTI was significantly reduced (p < 0.01) in group 3B vs group 3A. Diagnostic capabilities (i.e. identification of segments supplied by stenosed coronary arteries) of sestamibi uptake, WTI, and a combination of both variables with a discriminant function were compared by analysis of receiver operator characteristic curve (ROC) areas. The diagnostic capabilities of sestamibi uptake (ROC area = 0.65 +/- 0.04) were significantly lower (p < 0.05) than those of WTI (ROC area = 0.81 +/- 0.03) and discriminant function (ROC area = 0.83 +/- 0.03). In conclusion, our data suggest that combined analysis of myocardial perfusion and regional ventricular function may increase the diagnostic accuracy of sestamibi myocardial scintigraphy in identifying myocardial segments supplied by stenosed coronary arteries.

A case report of glycogenosis II with early infantile onset (type a) is presented and discussed. Some unusual features were observed. Pathological signs, involving the urinary tract, developed during the first days of life. Early instrumental evidence of myocardial disease and early onset of accompanying clinical signs were also seen. A further argument is proposed about guidelines for differential diagnosis of disease which can cause hypertrophic cardiomyopathy in infantile age.

The extensive use of ultrasound imaging in cardiology has greatly contributed to expand both its diagnostic possibilities and its utility in the interpretation of physiopathologic mechanisms. Conventional echocardiography is the more specific technique for the diagnosis of myocardial hypertrophy which, in turn, is one of the most important cardiovascular risk factors. The improvement of ultrasound technology may expand the possibility of noninvasive characterization of left ventricular hypertrophy by adding to the already known information about left ventricular mass and function, and that relative to the degree of hypertrophy-related fibrosis. In the present paper the authors reviewed the knowledge about biological and hemodynamic factors which contribute to the development and regression of myocardial hypertrophy. The possible role of new ultrasonic technology in the tissue characterization of myocardial hypertrophy is also discussed.

Background: Patients with hypertrophic cardiomyopathy are at increased risk of sudden death. Recently it has been investigated whether late potentials can be useful markers of that risk in such patients, with discordant results. The aim of our study was to detect a correlation, if any, between late potentials, ventricular tachycardia and the hypertrophy extension., Methods: Twenty-five patients were enrolled, 19 males and 6 females, mean age 40.40 +/- 15.02 years. Each underwent 24h Holter monitoring, averaged ECG, M-Mode and 2-D Echo. The hypertrophy was considered extensive (septum and free wall) or non extensive (septum and/or apex)., Results: Late potentials of a mean duration of 37 +/- 14.83 ms were recorded in 5/7 patients with documented ventricular tachycardia vs in none of the non tachycardia group. Sensibility was 100%, specificity 71% and predictive value 100%. Late potentials were present in 8.3% of patients with non extensive and in 30.8% of patients with extensive hypertrophy (p = NS)., Conclusions: From our data it seems that extensive hypertrophy is present in about half of patients with hypertrophic cardiomyopathy, and this roughly correlates with a more frequent ventricular tachycardia and with late potentials. Furthermore, late potentials are strictly related to the incidence of ventricular tachycardia in this disease, just as in ischemic heart disease or in idiopathic dilated cardiomyopathy. The small number of our cases may not be sufficient to reach a significant statistical correlation between late potentials and hypertrophy, but we believe that the trend is very promising.

Left atrial enlargement may occur sometimes through only the increase of the supero-inferior (S-I) diameter, with normality of the antero-posterior (A-P) and latero-medial dimensions. In this study, both the largest dimensions of the left atrium and S-I and transversal dimensions of the left atrium and S-I and transversal dimensions of the right atrium were investigated, among the 98 pts suffering from recurrent paroxysms of atrial fibrillation (FAP). On the basis of the clinical, ECGraphic and echocardiographic data, a subgroup of 78 pts has been found, with FAP reliable to heart disease, which mostly appeared as accompanying a finding of atrial enlargement--left or right or both--. The remaining 20 pts distinguished, by means of the echocardiographic findings, as following: a) "idiopathic" FAP, neither dependent on heart disease nor on atrial enlargement (no. 11 pts); b) FAP dependent on "unexplained" atrial enlargement, i.e. unreliable to definite cardiac pathology (no. 9 pts). Among the b) pts, 7 showed the only, isolated S-I dimension increased. Therefore, the determination of the all largest dimensions of the atria, in pts with recurrent FAP, appeared able to more carefully distinguish the true cases of "idiopathic" FAP.

To investigate whether the level of clinical blood pressure (BP) may serve to stratify the risk of left ventricular (LV) hypertrophy in essential hypertension regardless of the level of ambulatory BP, we performed 24-hour noninvasive ambulatory BP monitoring and echocardiography in 115 consecutive hypertensive patients who had never been treated before and in 92 normotensive subjects. Hypertensive patients were grouped according to the difference between the observed clinical BP and the predicted value of clinical BP, defined by regressing the observed clinical BP on the 24-hour average of the ambulatory BP: "low" clinical BP group (clinical systolic BP less than = 10 mmHg, diastolic BP less than = 6 mmHg than predicted values), "high" clinical BP group (systolic greater than = 10 mmHg, diastolic greater than = 6 mmHg than predicted values), "intermediate" clinical BP group (values within the above mentioned limits). Ambulatory BP did not show any statistically significant differences between the three groups. LV mass index was higher in hypertensive patients in each of the three groups (including the "low" clinical BP group) as compared with the normotensive group (all p less than 0.01), but did not show any statistically significant difference among the three groups of hypertensive patients, either defined by systolic BP or by diastolic BP. Other indexes of LV anatomy (relative wall thickness, cross-sectional area) showed a similar pattern.(ABSTRACT TRUNCATED AT 250 WORDS)

To evaluate the difference between the sexes in the echocardiographic indices of left ventricular hypertrophy in essential hypertension, we analysed the results of 24-hour non invasive ambulatory blood pressure monitoring and echocardiography in 195 consecutive untreated hypertensive patients (101 males, 94 females). Patients were divided into 3 age groups: up to 40 years, 41-60 years, and greater than 60 years of age. Clinical blood pressure did not differ between sexes in any of the three groups, while average 24-hour ambulatory systolic and diastolic blood pressure levels were higher in males than in females up to 40 years of age (140/93 vs 129/87 mmHg, p less than 0.01), but not between 41 and 60 years (142/93 vs 141/90 mmHg) nor in those over 60 years of age (151/92 vs 145/91 mmHg). LV mass index was higher in males than in females up to 40 years (93 vs 68 g/m2, p less than 0.01), and between 41 and 60 years (115 vs 90 g/m2, p less than 0.01), but not dissimilar between the sexes over 60 years of age (102 vs 107 g/m2, p = n.s.). In females, LV mass index increased with age (r = 0.33) and body weight (r = 0.38; both p less than 0.01), but none of these relationships was significant in males (r = 0.01 and r = 0.19, respectively). Moreover, all the relations of LV mass to either clinical or ambulatory BP were closer in females than in males. None of the echocardiographic indices of left ventricular systolic function showed statistically significant differences between the sexes in any of the three age groups.(ABSTRACT TRUNCATED AT 250 WORDS)

The feasibility of two-dimensional echocardiographic visualization of the coronary artery was re-evaluated in adults in the light of technological advances and development of new imaging planes. Athletes are a good model for this type of study. The aim of our study was to visualize in athletes the coronary arteries, particularly the left main artery, and to see if a correlation exists between left-ventricular mass and coronary diameter. Twenty-one endurance athletes, aged between 17 and 30 years, and 21 control subjects, matched for age, sex and body surface area, were examined. All the subjects were examined with mono- and two-dimensional echocardiography, with annular array (3.5 and 5 MHz), with parasternal and apical projections modified in order to visualize the left main coronary artery. Wall thickness, left ventricular internal dimension and left ventricular mass were calculated. Interventricular septum thickness was 10.8 +/- 1.5 mm for athletes (A) versus 8.2 +/- 0.9 mm for controls (C); p less than 0.01. Posterior wall thickness was 10.4 +/- 1.5 mm (A) versus 8.2 +/- 0.6 mm (C); p less than 0.01. The left ventricular diastolic diameter was 54.6 +/- 5.1 mm (A) versus 49.5 +/- 3.4 mm (C); p less than 0.01. The mean left ventricular mass was 278.2 +/- 85.2 g (A) versus 165.6 +/- 35.4 g (C); p less than 0.01. The mean diameter of the left main coronary artery was 4.9 +/- 0.8 mm (A) versus 3.1 +/- 0.4 mm (C); p less than 0.01.(ABSTRACT TRUNCATED AT 250 WORDS)

The aim of this study was to evaluate diastolic function in athletes and in young borderline hypertensives with mild left ventricular hypertrophy. To compare the effects on left ventricular filling of these two conditions we studied 15 borderline hypertensives (age 21 +/- 3 yrs, left ventricular mass index 137 +/- 9 g/m2), 18 soccer players (age 22 +/- 4 yrs, left ventricular mass index 136 +/- 12 g/m2) and 20 normotensive subjects (age 22 +/- 4 yrs, left ventricular mass index 94 +/- 10 g/m2) using the echo-Doppler technique. Left ventricular mass (LVM) was calculated with Devereux's formula. The criterion for left ventricular hypertrophy was left ventricular mass index greater than or equal to 120 g/m2. The following parameters of left ventricular filling were evaluated by Doppler transmitral flow analysis: the early (E) and late (A) peak velocity, the ratio E/A, the integral of early (Ei) and late (Ai) diastolic velocity. We concluded that the left ventricular filling profile was similar in borderline hypertensives, in athletes and in normotrophic subjects. These findings suggest that at least in its early stages, mild cardiac hypertrophy secondary to pathological stimulus is characterized by indices of diastolic function which are no different to those found in athletes with physiological hypertrophy.

Clinical, electrocardiographic and echocardiographic 5-year follow-up was performed in our institution on 61 patients with Friedreich's ataxia. Cardiac failure was evident in 5% of the patients, and was the most common cause of death. Cardiac arrhythmias, most commonly supraventricular in origin, usually occurred together with the onset of cardiac failure and in 1 case resulted in sudden death. ST-T abnormalities were present in 91% of the cases, and were independent from other clinical parameters. On the contrary, pseudonecrotic (5%) and right ventricular hypertrophy pattern were associated with a poor prognosis. Left ventricular hypertrophy was evident at the echocardiogram in 75% of cases and remained unchanged throughout the entire follow-up period. In 1 case left ventricular hypertrophy turned to dilative cardiomyopathy. Autopsy was performed in 2 out of 4 decreased patients and revealed massive interstitial fibrosis with cellular degeneration in the absence of coronary lesions.

An invasive (left ventricular pressure recording) and a noninvasive technique (M-mode echocardiography) were combined during the hemodynamic study of 15 patients with various degrees of left ventricular impairment, in order to provide left ventricular function parameters independent from contrast studies. Left ventricular pressure, dimension and wall thickness changes were continuously digitized on a computer system, which was able to derive the ventricular pressure-dimension loop and to calculate the midwall equatorial stress at end-diastole and peak-systole. The parameters derived from the loop (diastolic work, net systolic work, cycle efficiency) and midwall meridional and circumferential stresses were compared with the results of contrast ventriculography and with the wall hypertrophy index. Diastolic work appeared to be correlated with the systo-diastolic volume change; systolic work correlated with the ejection fraction; the cycle efficiency was inversely correlated with both end-diastolic and end-systolic volumes and directly correlated with the hypertrophy index. Midwall stress, both end-diastolic and peak-systolic, was strongly and positively correlated with ventricular volumes, while it decreased with increasing hypertrophy. Systolic stress increased with decreasing ejection fraction. The combined echo-pressure technique, through the evaluation of the pressure-dimension loop and the calculation of wall stress, can generate data of left ventricular function that allow a more complete assessment of left ventricular impairment.

One case of enlarged coronary sinus with a total anomalous pulmonary venous connection (TAPVC) is described. The echocardiographic findings are compared to those seen in cases with persistence of the left superior vena cava (PLSVC). The diagnosis has been established by single crystal and two-dimensional echocardiography, by cardiac catheterization and angiography and was confirmed at surgery; post-operative echocardiographic control was performed. The differential diagnosis between TAPVC or PLSVC and other anomalies which may simulate a dilated coronary sinus is discussed. The differences between TAPVC and PLSVC by contrast echocardiography are reported.

Some ECG criteria and the radiologic method of Westcott are evaluated against echocardiography in the left atrial enlargement. ECG shows generally a very good sensitivity (72%); particularly when the diameter is more than 5 cm. The radiologic findings show significant correlative indexes in normal and in softly enlarged atria. In atria greater than or equal to 5 cm the method is not so precise; the reasons are discussed on an anatomical basis. We acknowledge an important place to the ECG described criteria and to the Westcott method in the depistage of the left atrium enlargement.

Right ventricular arrhythmogenic dysplasia is a rare cardiomyopathy which involves the right ventricle either totally or partially. Up to now diagnoses have been based on Ecg, hemodynamics, angiography and echocardiography. This paper deals with the first 7 patients examined also by means of Computed Tomography. The CT picture is well defined and rather accurate. The most important elements are: total (6/7) or partial (1/7) enlargement of the right ventricle; thinning of right ventricular myocardium (6/7); marked increase in subepicardial fat limited to the right ventricular wall (7/7); right ventricle hypokinesia (7/7). A good correlation exists between CT, echographic and traditional methodologies findings. Because of its densitometric evaluation, CT is much more precise in the demonstration of fatty degeneration. Moreover, it can be very useful in differentiating intracardiac thrombi from hypertrophic papillary muscles and trabeculae.

In nine closed chest mongrel dogs 42 left ventricular cineangiographies for determination of left ventricular muscle mass were carried out. Buetterich's method was verified and a different procedure of calculation of myocardial wall thickens was proposed. By this method a better correlation line between calculated and weighed left muscle mass and a lower standard error of estimate is obtained.

Eight normotensive patients with electrocardiographic criteria for left ventricular hypertrophy with giant negative T waves were studied with Thallium-201 imaging and M-mode echocardiography. In all the patients Thallium scanning demonstrated increased thickness of the left ventricular walls. In five of the above cases areas of increased uptake were noted in the apical region which had increased thickness as compared to the rest of the left ventricular wall. Echocardiography showed in one subject obstructive hypertrophic myocardiopathy and in another two asymmetric septal hypertrophy. In the remaining patients there was always present septal and posterior wall hypertrophy. Reliable echocardiograms of the apex were done in five subjects and in these hypertrophy was noted. The results of the two techniques were compared and correlated clinically and with the literature. The authors conclude in agreement with other studies that the picture of electrocardiographic left ventricular hypertrophy with giant negative T waves is indicative of hypertrophic myocardiopathies. Specifically, for us, the apical hypertrophy may be the only feature of the myocardiopathy or be part of a generalized left ventricular hypertrophy which is usually asymmetric septal hypertrophy.

A radiographic and hemodynamic investigation has been performed in 53 patients with pure isolated aortic regurgitation, in order to evaluate the most reliable method to detect left ventricular enlargement in this condition. Twelve plain chest film measurements have been compared to the end diastolic volume of the left ventricle calculated by left ventricular angiography. The most sensitive detector of left ventricular enlargement was the plain film total heart volume ( r = 0.754); the cardiothoracic ratio, the cardiac perimeter and the frontal area were less sensitive measurements, but still statistically significant (p less than 0.001). The results of our investigation support the conclusion that plain film total heart volume is the most reliable single measurement for evaluating the left ventricular enlargement in patients with isolated pure aortic incompetence.

Radiology has an important part to play in Goodpasture's syndrome. The radio-sympatomatological value of lung changes are clarified and subdivided into primary, secondary and mixed, and mention made of the possible significance of cardiomegaly, where present. Special attention is paid to the importance of radiological exploration of the kidneys for the recognition of changes whose concomitance with lung changes may suggest diagnosis of Goodpasture's syndrome.

At present, selective coronaro-angiography offers the only means of establishing the seriousness, extent and site of coronary sclerosis. It has led to the development of coronary surgery, itself a considerable advance in the treatment of coronary disease. The method is also of considerable assistance in the differential diagnosis of inexplicable ECG changes and other heart diseases. It is relatively free from risk if performed by experienced staff with suitable equipment and in line with the correct indications.

The aim of this study was to evaluate the value and limitations of Cross-sectional Echocardiography (CSE) in the diagnosis of Arrhythmogenic right ventricular dysplasia (ARVD). Diagnosis was based on accepted clinical, electrocardiographic, electrophysiologic and angiographic criteria. CSE criteria for the diagnosis are segmental right ventricular wall motion abnormalities of unknown cause, usually associated with localized or diffuse dilatation of right ventricular (RV) chamber and with the presence of localized anomalies consisting of sacculation or bulging of RV wall. Comparison of CSE and RV angiographic findings was performed in 8 patients with ARVD (6 men and 2 women, aged 10 to 37 years, mean 28 years). CSE and angiography compared closely when diffuse RV enlargement and wall motion abnormalities were identified by both techniques. Bulging and sacculation of the RV wall at CSE predicted the presence of similar lesions at angiography, but agreement for specific location was poor and, in addition, CSE showed low sensitivity in their detection. The inherent different information provided by the two methods added to the subjectivity of the qualitative analysis probably accounts for the inconsistencies. Therefore in patients with diagnosed ARVD RV enlargement, otherwise unexplained, associated with wall motion abnormalities and localized anomalies at CSE strongly supports the diagnosis and avoids the need for angiography. By other hand, in patients with high clinical suspicion of ARVD a negative CSE study can not exclude the diagnosis and angiography should be indicated.