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A case of a 12 year old girl who presented tetralogy of Fallot (T. o F.) associated with Total Anomalous Pulmonary Venous Drainage (TAPVD) is described. This association is extremely rare, only four autopsied cases being reported in literature. TAPVD is associated with complex cyanotic congenital heart diseases in about one third of cases; asplenia or polysplenia are generally present. Double outlet right ventricle (DORV) is the commonest trunco-conal malformation associated with TAPVD; extremely rare is T. o F. Pulmonary outflow tract obstruction can avoid the haemodynamic consequences due to obstruction on the venous channel, or progression to pulmonary hypertension. The association of the two malformations can masquerade the clinical and Xray picture of TAPVD and make difficult the diagnosis during cardiac catheterization.

Idiopathic restrictive cardiomyopathy is a rare myocardial disease characterized by restrictive physiology without a specific histologic basis. To assess its clinical, hemodynamic, morphologic and prognostic details we retrospectively evaluated all the patients hospitalized in our Institute from 1974 to 1988. Nine patients, aged 42 +/- 16 years, M/F ratio = 0.29, who represent 64% of all the restrictive myocardial diseases biopsied were identified. Severe cardiac heart failure (3-4 NYHA) and arrhythmias (ventricular and supraventricular) were extremely common. The electrocardiogram showed several non specific signs: low voltage of QRS in peripheral leads (4/7), pseudo-infarctional aspects (3/7), mono or biventricular hypertrophy (3/7) disturbance of ventricular conduction (3/7), aspecific abnormalities of ventricular repolarization (3/7). All patients showed a prolonged QTc. M-mode and 2-dimensional echocardiography demonstrated in 6 cases biatrial enlargement, normal or slightly enlarged ventricles, normal or moderately depressed fractional shortening; biventricular concentric hypertrophy was detected in 3 cases, asymmetrical septal hypertrophy in 1. Five patients showed pericardial effusion. Cardiac catheterization disclosed an increase of left and right ventricular end-diastolic pressures (8/8) with a dip-plateau pattern and/or characteristic W waveform in the atrial pressure tracing (9/9). Passive pulmonary hypertension was detected in 6/9 cases. The cardiac index was decreased in 4/8 cases. Left ventricular angiography showed mitral regurgitation in 5/8 patients, tricuspidal in 5/8. Ejection fraction was decreased in 3/8 cases. Endomyocardial biopsy showed interstitial fibrosis (8/9), cellular hypertrophy and/or nuclear alterations (7/9), slight endocardial thickening (2/9). At a mean follow-up of 22 +/- 15 months 3 patients died and 2 underwent heart transplantation. In conclusion idiopathic restrictive cardiomyopathy is one of the most frequent forms of restrictive myocardial diseases in our geographic area. Severe congestive heart failure and arrhythmias are extremely common. The disease can be suspected by clinical, electrocardiographic and echocardiographic features, but the final diagnosis requires cardiac catheterization and endomyocardial biopsy. Prognosis is severe and heart transplantation must be considered in the cases with severe heart failure.

The detection of myocardial depression is an important goal in the management of patients with chronic severe aortic regurgitation but may be quite difficult at an early stage by the conventional basal measures of contractility. The response to afterload stress determined by angiotensin challenge and the end-systolic pressure-volume relationship was evaluated echocardiographically in 16 asymptomatic or mildly symptomatic patients with chronic severe aortic regurgitation, ages 15 to 56 years (mean 32 +/- 12). Nine normal subjects, ages 25 to 41 years (mean 31 +/- 5), served as a control group. In the group with aortic regurgitation, end-systolic dimensions were greater than 55 mm in five of 16 patients and fractional shortening was 25% or less in two of 16. In the control group angiotensin caused a decrease of stroke volume index in six out of nine patients (15% at the most) and a mild increase in three. In the group with aortic regurgitation stroke volume index decreased by 15% or more of the basal value in nine of 16 patients and increased or decreased by less than 15% in seven of 16. Ejection fraction decreased in both groups, from 61 +/- 6% to 52 +/- 7% in the control group and from 56 +/- 6% to 45 +/- 5% in the group with aortic regurgitation. Ventricular function curves were derived by relating end-diastolic volume index to stroke work index; seven of 16 patients had abnormal responses reflecting an afterload mismatch.(ABSTRACT TRUNCATED AT 250 WORDS)

Precordial mapping of QRS complex is a reliable tool in attempts to quantitate infarct size in patients with acute myocardial infarction. However the recording of multiple precordial leads is an expansive and time-consuming process; the purpose of this study is to investigate the usefulness of Frank's ECG as a substitutive method of infarct sizing. In a group of patients with acute myocardial infarction of the anterior wall many repeated precordial mappings with simultaneous recording of x, y, z leads of Frank's ecg were obtained (71 observations as a whole). Parameters provided by precordial mapping [delta R, delta Q, delta R + delta Q, delta R/delta (Q + S)] and by Frank's ecg (spatial vector voltage deviation of QRS or VSDV-QRS; spatial vector area deviation of QRS or VSDA-QRS; spatial vector R wave deviation or VSDR) were correlated each other by means of linear regression analysis. delta R + delta Q correlated significantly with VSDA QRS (r = 0.54; p less than 0.001) and with VSDR (r = 0.55; p less than 0.001) as regards the total of observations. The correlation coefficient was higher (r = 0.8; p less than 0.001) if comparison between delta R + delta Q and VSDA-QRS was restricted to early 48 hours from the beginning of the symptoms. This could be explained by the greater entity of QRS complex changes in this period, compared to the subsequent course. We conclude that Frank's ecg is an useful alternative method to precordial mapping in sizing the extension of myocardial necrosis.

The haemodynamic effects of an intravenous amiodarone infusion (5 mg/kg for 10 minutes) were measured in ten patients with hypertrophic cardiomyopathy (two with a left ventricular outflow gradient at rest) five, 15, and 30 minutes after drug administration. Mean (SD) pulmonary capillary wedge pressure rose significantly at five and 15 minutes (from 12.3 (6.2) mm Hg to 17.6 (9.2) and to 16.2 (8.6] with a subsequent tendency to fall to control values at 30 minutes. Mean right atrial and right ventricular end diastolic pressures increased from 3.6 (1.8) mm Hg to 7.3 (3.1) and from 6.3 (2.4) to 9.8 (3.2) mm Hg respectively at 30 minutes. The increase in filling pressures was paralleled by a decrease of left ventricular max dP/dt from 1522 (414) to 1372 (327) to 1316 (338) and to 1326 (379) five, 15, and 30 minutes after infusion. Despite this slight negative inotropic effect, cardiac index and stroke volume index were unchanged or slightly increased, possibly because of the decrease in systemic vascular resistance (from 1326 (330) dyn s cm-5/m2 to 1152 (285]. In both patients with outflow gradient the pressure gradient at rest decreased (from 110 to 65 and from 85 to 65 mm Hg) through a reduction of left ventricular systolic pressure. Thus short term intravenous infusion of amiodarone is safe in patients with hypertrophic cardiomyopathy. The main changes were a mild depression of ventricular contractility, which was well tolerated and adequately compensated for by a decrease in afterload.

In order to assess left ventricular diastolic function in elite athletes we studied 24 athletes (mean age 21 +/- 5 years) with the echo-Doppler technique. The control group consisted of 14 healthy males (mean age 24 +/- 6 years) and 99 patients (mean age 50 +/- 14 years) with primary or secondary left ventricular hypertrophy. The following variables were calculated: age, systolic and diastolic blood pressure, septal and posterior wall thickness, left ventricular volume and dimensions, myocardial mass index and mass/volume ratio, left ventricular end-systolic and peak systolic stress, RR interval, isovolumic relaxation time (IVRT), early (E) and late (A) peak flow velocity, E/A ratio, time and rate of deceleration of early diastolic flow. Compared with the normal subjects significant differences were observed with regard to systolic blood pressure, left ventricular wall thickness, myocardial mass, mass/volume ratio and peak systolic stress in the athlete group. In spite of a noticeable increase in myocardial mass indexes, all the diastolic function parameters were normal in the athletes. In this group a linear positive correlation between rapid filling indexes, ventricular mass and mass/volume ratio exists. These correlations are not present in the patients group. Moreover a linear negative correlation between RR interval and late diastolic flow velocity was found. In conclusion, the global diastolic ventricular function, evaluated with the echo-Doppler technique, is normal in athletes in spite of an increase in ventricular mass. Th early diastolic flow velocity is in direct proportion with mass increase and may represent one of the physiological mechanisms of the athlete heart adaptations.

We studied ventricular volumes and ejection fraction by radionuclide angiography (equilibrium technique) in 15 patients (aged 3-48 years) with double inlet ventricle not yet submitted to corrective surgery. The end-diastolic volume (measured in nine cases) ranged from 108 to 219 ml/m2 (156 +/- 32), being lower than the normal theoretical value (right plus left ventricle) in six cases. Ejection fraction ranged from 30 to 77% (56.4 +/- 13). The value was significantly higher in the subgroup of 10 patients with a dominant left ventricle as compared to the five cases with dominant right or indeterminate ventricular morphology (63.2 +/- 8.3 versus 42.8 +/- 9, P less than 0.01). In seven of the 15 patients, measurements were obtained both at rest and during dynamic exercise in the semi-upright position. The end-diastolic and end-systolic volumes, stroke volume, ejection fraction underwent a slight non-significant reduction (from 158 +/- 29 to 147 +/- 24 ml/m2, from 58 +/- 16 to 56 +/- 24 ml/m2, from 100 +/- 27 to 90 +/- 24 ml/m2, from 64% +/- 9 to 61% +/- 13). During exercise, ventricular volumes mostly behaved as follows: slight reduction of end-systolic volume, decrease of end-diastolic volume, no increase (no change or decrease) of ejection fraction.(ABSTRACT TRUNCATED AT 250 WORDS)

To define the risk of side branch occlusion during percutaneous transluminal coronary angioplasty (PTCA), 99 consecutive procedures, performed on 92 patients, were examined. In 77 of them side branches existed, originating from the stenosed segment; analysis was performed on 65 successful procedures (success rate = 84.4%). The 121 side branches were divided as follows: 53 (43.8%) originating from the stenosis itself (group A), of which 32 small in size (less than 1 mm) and 21 "moderate" (greater than or equal to 1 mm); 68 (56.2%) originating in the immediate vicinity of the stenosis (group B) of which 23 small and 45 moderate. After PTCA 3 side branches were occluded (2.5%): a small 1 of group A and 2 (1 small and 1 moderate) of group B. Three side branches (2.5%) all of group B, 1 small and 2 moderate, became stenotic in their take-off. In one only patient who had a side branch occlusion a slight CK-MB elevation (25 mU/ml) occurred together with a Q wave appearance in the aVL lead. In conclusion, side branches at risk are frequently present (in our population they account for 83.7% of the patients) but the real incidence of damage of these branches after PTCA is quite low, without any considerable difference between groups A and B, and significant clinical consequences are usually rare.

The Authors are examing two cases of A-V canal associated with T. of Fallot. The most important clinical aspects of this rare cardiopathy result in the following: -- the frequent coexistence with Down dyndrome; -- the ECG and VCG aspects of counterclockwise rotation of the initial vectors of QRS in the frontal plane; -- the simultaneous observation of the typical echocardiographical aspects of both kinds of cardiopathy. The injection of dye into both ventricles allows the precise diagnosis. The post-mortem study in both cases shows the morphology of A-V canal associated with the one of T. of Fallot. From the surgical point of view it is important to take into account the abnormal morphology and extension of VSD. The importance of the part that the conal cushion plays in the morphogenesis of the malformation is discussed.