A neonate with cyanosis and tortuous great arteries.

This report describes an unusual case with tortuosity of the great vessels in a neonate who presented at birth with cyanosis. The diagnosis was made with magnetic resonance imaging (MRI), then confirmed by genetic analysis.

Congenital heart disease: a retrospective study of their frequency.

AIM: An early diagnosis of congenital heart disease (CHD) is necessary for the obstetrical management. METHODS: One thousand five hundred and fifty-six pregnant women underwent a fetal 2-D echocardiography from 1991 to 2002. We assessed patients who had the most common risk factor (RF): family history of CHD, diabetes mellitus, teratogen drugs, polyhydramnios, abnormal fetal growth, fetal arrhythmia, maternal age over 40 years, maternal autoimmune disease, maternal disease contracted during pregnancy, oligohydramnios, only umbilical artery, uncorrected visualization in the first level 2-D fetal echocardiography. RESULTS: In 110 morphological anomalies found 54 were complex. The malformations are not equally distributed among the different RF. The most common RF is the family history of CHD where 24 patients showed complex malformations. Another high percentage of complex malformations was found in patients with no apparent presence of RF: the diagnosis was done after an uncorrected cardiac visualization in the first level 2-D echocardiography. In the minor cardiomyopathies we did not find the prevalence of any RF. After diagnosis of complex malformations we had 15% of termination of pregnancies, 37% of the newborns are alive and in good health. CONCLUSIONS: The first RF is the family history of CHD, but a correct first level fetal 2-D echocardiography is necessary because a very high percentage of complex malformations is seen in infant whose mother did not have any RF. Ninety percent of cardiac malformations where seen in a 4 chamber view, and it is of the utmost importance also for a correct diagnosis of minor cardiomyopathies where we did not find a predominant RF.

[Neurologic outcome in infants surgically treated for congenital cardiopathy: preliminary data]. / Outcome neurologico di lattanti operati per cardiopatia congenita: dati preliminari.

INTRODUCTION: Both surgical techniques for correction of congenital heart diseases (CHD) and intraoperatory neurologic protection improved during the last 20 years. Nevertheless cardiac surgery is still a risk for neurologic morbidity. METHODS AND PATIENTS: Analysis of the postoperative neurologic status of infants younger than 6 months who underwent cardiac surgery from January 1998 to December 1999. We reviewed the EEG tracings, cranial ultrasound reports (CUS) and CT scans of 48 patients. Diagnoses were: ventricular septal defect = 15, Fallot (TOF) = 9, patent ductus arteriosus (PDA) = 5, coarctation of aorta = 4, atrio-ventricular septal defect = 4, transposition of great arteries (TGA) = 3, hypoplastic left heart syndrome = 2, pulmonary atresia = 2, total anomalous pulmonary veins drainage = 2, double outlet right ventricle = 1, cor triatriatum = 1. Mean age (range) at intervention was 54 days (2-150), 44 infants (91.7%) survived at follow-up: 23 EEG, 22 CUS and 2 CT were performed in the recent postoperative. Among survivors 5/44 had neurologic complications. EEG was altered in 4: two of them (1 TOF, 1 TGA) had pathologic CUS and CT as well (ischemic pattern in the former, atrophy in the latter). Finally a preterm newborn with PDA had mild abnormalities at CUS. After a mean follow-up of 16 +/- 6 months 3/5 patients had mild-to-moderate psychomotor delay and 2 recovered. CONCLUSIONS: According to our preliminary data the prevalence of neurologic complications in infants who undergo cardiac surgery seems to be low. The pathological findings of the recent postoperative seem to recover up to normalization in some cases at mid-term follow-up. As expected, permanent complications effect more often complex CHD. Further follow-up studies to school age will be mandatory to check the very final results of cardiac surgery performed during early infancy.

Left atrial filling volume can be used to reliably estimate the regurgitant volume in mitral regurgitation.

OBJECTIVES: The objective was to analyze the accuracy and diagnostic value of the estimated regurgitant volume of mitral regurgitation using 1) left atrial volume variation during ventricular systole (left atrial filling volume) and 2) the percent of systolic pulmonary vein velocity integral compared with its total. BACKGROUND: Left atrial filling volume (LAfill), which represents the atrial volume variation during ventricular systole, has been used for the assessment of mitral regurgitation severity. A good correlation with invasive semiquantitative evaluation was found, but with an unacceptable overlapping among grades. The reason could be the absence of information concerning the contribution of blood entering into the left atrium from the pulmonary veins. METHODS: Doppler regurgitant volume (Dpl-RVol) (mitral stroke volume - aortic stroke volume) was measured in 30 patients with varying degrees and etiological causes of mitral regurgitation. In each patient atrial volumes were measured from the apical view, using the biplane area-length method. The systolic time-velocity integral of pulmonary vein flow was expressed as a percentage of the total (systolic-diastolic) time-velocity integral (PVs%). These parameters were used in this group of patients to obtain an equation whose reliability in estimating Dpl-RVol was tested in a second group of patients. RESULTS: In the initial study group, with linear regression analysis the following parameters correlated with Dpl-RVol: end-systolic left atrial volume (R2=0.37, p=0.0004); LAfill (R2=0.45, p < 0.0001); PVs% (R2=0.56, p < 0.0001). In multiple regression analysis the combination of LAfill and the percent of the systolic pulmonary vein velocity integral (PVs%) provided a more accurate estimate of regurgitant volume (R2=0.88; SEE 10.6; p < 0.0001; Dpl-RV=6.18 + (1.01 x LAfill) - (0.783 x PVs%). The equation was subsequently tested in 54 additional patients with mitral regurgitation with a mean Dpl-RVol 27+/-37 ml. Estimated regurgitant volume and Dpl-RVol correlated well with each other (R2=0.90; SEE 12.1; p < 0.0001). In the test population, the equation was 100% sensitive and 98% specific in detecting a regurgitant volume higher than 55 ml. CONCLUSIONS: Left atrial filling volume and pulmonary vein flow give a reliable estimate of regurgitant volume in mitral regurgitation.

Opposite effects of the remodeling of infarcted and non-infarcted myocardium on left ventricular function early after infarction in humans. An echocardiographic study in patients examined before and after myocardial infarction.

OBJECTIVE: The purpose of this study was to evaluate infarction-related changes in the infarcted and the non-infarcted myocardium using a baseline assessment of ventricular function obtained prior to the infarction. BACKGROUND: Experimental studies have shown that both infarcted and non-infarcted myocardium contribute to the process of left ventricular dilatation soon after the infarction, but no data exist on the effect that the infarct has on the pre-infarct ventricular morphology in humans. METHODS AND RESULTS: 10 patients, out of 721 admitted to our coronary care unit with a first acute myocardial infarction over a 3-year period, had had an echocardiographic examination performed before (354 +/- 407 days) and after (10 +/- 9 days) the infarction which were adequate for quantitative evaluation. Ventricular volume (Simpson) and regional wall motion (Centerline method) were evaluated by biplane apical sections and the endocardial length of the infarct and the non-infarct segments, imaged in a cross-sectional view at the papillary muscle level, were measured. After the infarction end-diastolic and end-systolic ventricular volume increased (P = 0.0003 and P < 0.0001, respectively); diastolic and systolic infarct segment length increased (P = 0.011 and P = 0.0008, respectively), while non-infarct segment had only diastolic lengthening (P = 0.019), without systolic changes. The ejection fraction decreased after the infarction (P < 0.0001), in inverse relation to infarct size and in direct relation to diastolic non-infarct segment lengthening. In the five patients in whom there was a significant diastolic lengthening of non-infarct segment (larger than mean +/- 2 S.D. of the interobserver variability) the decrease in ejection fraction was less than in the patients without significant lengthening of this segment (P = 0.017), despite a similar echocardiographic infarct size index. CONCLUSION: Ventricular enlargement early after myocardial infarction is due to both infarct expansion and lengthening of non-infarct segment. However, while systolic stretching of the infarct segment is a deleterious process that accounts for the increase in end-systolic volume, diastolic non-infarct segment lengthening is the expression of a functional compensatory mechanism that counteracts the reduction of the ventricular pump function secondary to the infarction.

Does prostaglandin E1 infusion affect the left ventricular filling pattern of end-stage dilated cardiomyopathy? A combined hemodynamic-echo Doppler study.

Prostaglandin E1 improves hemodynamics in patients with severe dilated cardiomyopathy and pulmonary hypertension through it's reducing action on pulmonary resistances. However, few data are available to indicate whether these beneficial effects on right heart hemodynamics translate into any improvement of the altered left ventricular filling pattern that characterizes this condition. We studied 12 patients with dilated cardiomyopathy during preoperative evaluation for cardiac transplantation before and after prostaglandin E1, 30-50 ng/kg/min i.v. Patients underwent catheterization of the right heart and left ventricle by Swan-Ganz catheter, giving simultaneous assessment of pressure by micromanometer and of volume derived from two-dimensional echo-guided Doppler mitral flow velocity, where volume equals mitral velocity integral x valvular area. Prostaglandin E1 induced a significant reduction in mean pulmonary (from 38 to 30 mm Hg; p = 0.0001) and aortic (from 79 to 75 mm Hg, p = 0.05) pressures but no change in heart rate or tau. Peak A wave increased from 28 to 33 cm/s (p = 0.02), along with a reduction in end-diastolic pressure from 29 to 26 mm Hg (p < 0.04), whereas peak E wave did not change. E/A ratio decreased slightly (from 2.5 to 2.1; p < 0.0007) but did not reverse. Systolic volumes decreased (from 231 to 212 ml; p < 0.05), and cardiac index increased from 2.1 to 2.6 L/min/m2 (p = 0.0002) because of a reduction in pulmonary and systemic vascular resistances. The diastolic pressure-volume relation shifted downward along the same curve. Prostaglandin E1 infusion in patients with severe dilated cardiomyopathy and pulmonary hypertension reduces pulmonary and systemic resistances without affecting heart rate, relaxation, or passive diastolic left ventricular properties. Systolic right and left ventricular unloading increases cardiac index, facilitating ventricular emptying. E/A ratio does not reverse, although it decreases slightly, with mechanisms, however, that appear independent of any direct effect of the drug on the ventricular diastolic properties.

Reperfusion reduces left ventricular dilatation by preventing infarct expansion in the acute and chronic phases of myocardial infarction.

Reperfusion reduces left ventricular dilatation in patients with acute myocardial infarction, but it is unclear to what extent this is a primary effect or only a consequence of the limiting effect of reperfusion on infarct size. To address this issue, 56 consecutive patients were examined by means of two-dimensional echocardiography on day 1, on day 3, before discharge, and at 6 months after an acute myocardial infarction. From this population two groups of 12 patients each, perfectly matched for site of myocardial infarction, extent of ventricular asynergy at two-dimensional echocardiography (akinesis + dyskinesis), and clinical characteristics were identified according to the creatine kinase (CK) time to peak, which was regarded as a marker of spontaneous or induced reperfusion: (1) CK time to peak of 12 hours or less (reperfused patients, n = 12), and (2) CK time to peak of more than 12 hours (nonreperfused patients, n = 12). In these two groups of patients end-diastolic and end-systolic left ventricular volumes and endocardial lengths of asynergic and normal ventricular segments, imaged in a cross-sectional view at the level of the papillary muscles, were then computed. At the first examination end-diastolic volume, end-systolic volume, and endocardial segment lengths of normal and asynergic segments were similar in the two groups of patients. Patients with late CK time to peak, however, showed a progressive increase in left ventricular systolic volumes and in asynergic endocardial segment lengths between the first and third (predischarge) examinations (p < 0.05 for both), with no change in systolic length of the normal myocardium. The left ventricular end-systolic volume and the asynergic endocardial segment length of patients with early CK time to peak, however, did not increase during hospitalization. The increment in end-systolic volume and in systolic infarct segment length from the first to the third examinations was higher in nonreperfused patients (p = 0.018 and p = 0.04, respectively). Changes similar to those detected in systole were found for diastolic volume and diastolic infarcted and noninfarcted segment length in both groups, but they did not reach statistical significance. After 6 months, an increases in volume and endocardial length were found in both groups of patients. Relative to the first examination, however, the increase in systolic volume and in asynergic systolic endocardial lengths remained greater for nonreperfused patients (p = 0.077 and p = 0.01, respectively).(ABSTRACT TRUNCATED AT 400 WORDS)

Usefulness of transesophageal atrial pacing combined with two-dimensional echocardiography (echo-pacing) in predicting the presence and site of residual jeopardized myocardium after uncomplicated acute myocardial infarction.

The usefulness of transesophageal atrial pacing combined with 2-dimensional echocardiography (echo-pacing) in predicting the presence and site of jeopardized myocardium, defined as areas of myocardium perfused by a vessel with a stenosis > or = 75% or by a collateral circulation if the supplying vessel was occluded, was evaluated in 31 patients with uncomplicated acute myocardial infarction who underwent coronary angiography. All 5 patients without jeopardized myocardium had a negative test, whereas 24 of 26 with jeopardized muscle had a positive test (sensitivity 92%; specificity 100%). To identify the site of jeopardized myocardium, tests that were positive for development of new asynergies were analyzed further, distinguishing those positive in the infarct or remote zone. Seven of 8 patients with new asynergies in the remote zone had areas of jeopardized myocardium outside the territory of distribution of the infarct-related vessel, whereas only 2 of 12 with new asynergies in the infarct zone had areas of jeopardized myocardium outside that territory (p < 0.01), correctly predicting the site of jeopardized myocardium in 17 of 20 cases. In conclusion, echo-pacing is useful for detecting the presence and site of jeopardized myocardium after an acute myocardial infarction.

[Ventricular remodelling and modifications in ventricular filling in the acute phase of a myocardial infarct]. / Rimodellamento ventricolare e modifiche del riempimento ventricolare nella fase acuta dell'infarto miocardico.

The importance of infarct expansion in determining global ventricular remodelling and prognosis after myocardial infarction is well known, whereas how infarct expansion affects left ventricular filling dynamics is not defined. To address this issue two-dimensional and Doppler echocardiography was performed in 28 consecutive patients admitted to our Coronary Care Unit for a first acute transmural myocardial infarction 1) within 24 hours of symptoms' onset and 2) at predischarge. A semiquantitative echocardiographic infarct size index was computed, while the infarct and non-infarct segment length was measured in a short-axis papillary muscle section. Peak velocity of early (E) and late (A) transmitral Doppler curves were also measured. An increment in infarct segment length > or = 1.2 cm between baseline and predischarge examination was chosen as target to divide patients with (N = 8) and without (N = 20) infarct expansion. Patients with expansion had a higher echocardiographic infarct size index (3.5 +/- 1.4 versus 2.3 +/- 0.6 segments, p < 0.0001) and a higher CK-MB infarct size (336 +/- 235 versus 129 +/- 87 UI, p = 0.002), while ejection fraction was lower (36 +/- 8% versus 48 +/- 7%, p < 0.001). A linear correlation was found between the increment in infarct segment length and in left ventricular volume between the baseline and the predischarge examination (r = 0.58, p < 0.01). Doppler parameters were not different at baseline examination between patients with and without expansion.(ABSTRACT TRUNCATED AT 250 WORDS)

[Modifications in left atrial function in response to changes in left ventricular filling]. / Modificazioni della funzione atriale sinistra in risposta alle alterazioni del riempimento ventricolare sinistro.

In order to investigate the effects of increasing degrees of left ventricular filling impairment on left atrial function, in 9 A-fillers (E/A ratio less than 1, E wave deceleration time greater than 170 ms) and 9 E-fillers (E/A ratio greater than 1, E wave deceleration time less than 150 ms) we constructed the left ventricular and the left atrial volume curves according to a previously validated Doppler 2-dimensional echo method which combines mitral and pulmonary venous flow. Eight normals served as control. The left atrial reservoir (defined as maximum-minimum atrial volume), pump (defined by the volume of blood that enters the left ventricle with the atrial contraction) and conduit functions (defined as left ventricular filling volume--the reservoir and the pump volume) expressed as % of the left ventricular filling volumes, varied significantly between normals (37 +/- 9%, 25 +/- 3%, 37 +/- 11%), A-fillers (48 +/- 9% p less than 0.05, 39 +/- 5% p less than 0.05, 14 +/- 10% p less than 0.001) and E-fillers (27 +/- 6% p less than 0.05, 19 +/- 7% p less than 0.05, 54 +/- 10% p less than 0.01). Also maximum left ventricular and left atrial volumes differed significantly (normals 165 +/- 31 ml, 76 +/- 20 ml; A-fillers 174 +/- 33 ml, 100 +/- 20 ml p less than 0.05; E-fillers 322 +/- 34 ml p less than 0.001, 136 +/- 41 ml p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Echocardiographic contrast imaging of the human right heart: a multicenter study of the efficacy, safety, and reproducibility of intravenous SHU-454.

The contrast agent SHU-454 was intravenously injected in 103 patients during echocardiography: 37 mL/patient +/- 7 ml/patient. The quality of the contrast effect was optimal in 13, good in 51, sufficient in 30, and poor in 9 patients; reproducibility was optimal in 38, good in 46, sufficient in 17, and poor in 2 patients. Taste sensation, arm discomfort, or atypical chest pain occurred in 5 patients, premature ventricular contractions in 3. An isolated anginal attack occurred in 1 patient with frequent episodes at rest. A slight reduction in hematologic indices was attributed to hemodilution. Thus, SHU-454 produces a good and reproducible contrast effect with tolerable side effects.

[Variations in left ventricular function during chronic therapy with verapamil in angina patients. Influence of the plasma concentration of the drug. Quantitative M-mode echocardiographic analysis]. / Variazioni della funzione ventricolare sinistra durante terapia cronica con verapamil in pazienti anginosi. Influenza delle concentrazioni plasmatiche del farmaco. Analisi quantitativa ecocardiografica M-mode.

In order to assess the influence of orally administered verapamil on left ventricular function, 12 anginal patients were studied using M-mode echocardiography prior to and following two weeks of treatment with verapamil, 120 mg t.i.d. Baseline measurements were obtained by averaging the three measurements from the three different echocardiographic recordings; measurements during treatment were obtained by averaging three measurements on a single recording. The following parameters were considered: end-diastolic (dD) and end-systolic diameters of the left ventricle, fractional shortening, peak ejection rate and peak filling rate. On baseline examination, the end-diastolic diameter was 52.3 +/- 7.9 mm, the end-systolic diameter was 32.4 +/- 7.2 mm, the fractional shortening was 38.5 +/- 5.2%, peak ejection rate was 2.46 +/- 0.41 and peak filling rate was 4.87 +/- 1.44 1/sec. After verapamil treatment the end-diastolic diameter was 54.5 +/- 8.1 mm and the end-systolic diameter was 34.0 +/- 6.6 mm: both parameters showed a significant increase (p less than 0.05 for both). Fractional shortening (37.9 +/- 4.8%), peak ejection rate (2.29 +/- 0.39 1/sec) and peak filling rate (4.94 +/- 1.64 1/sec) remained unchanged. Verapamil plasma level was 149 +/- 1076 ng/ml; there was no significant correlation with the percentage variations of the echocardiographic parameters. In conclusion, in patients with normal cardiac function, chronic treatment with verapamil does not affect ventricular performance. In fact, a slight but significant cardiac dilatation appears to be the mechanism adopted to maintain adequate cardiac performance.

[Angiographic morphology of coronary lesions in various syndromes of ischemic cardiopathy]. / Morfologia angiografica delle lesioni coronariche nelle diverse sindromi della cardiopatia ischemica.

The angiographic morphology of coronary lesions is often completely ignored in the prognostic and decision-making process related to patients with coronary disease. We performed this study to evaluate the possibility of identifying complex or complicated atherosclerotic lesions by means of routine diagnostic coronary arteriography, and to assess their prevalence in the different syndromes of ischaemic heart disease. From an overall group of 200 successive cases studied using coronary angiography, 111 patients with significant coronary artery disease in whom a "culprit lesion" could be identified were retrospectively selected. The angiographic morphology of coronary lesions was defined according to an original classification as: 1) simple stenosis, 2) complex lesion, 3) thrombosis. Of the 111 patients, 36 had been studied for stable angina, 31 for unstable angina, 10 for a non-Q wave myocardial infarction, 34 for transmural infarction. The clinical groups did not show any significant differences when compared on the basis of number of vessels involved and degree of narrowing of the ischaemia-producing artery. Significant differences were found when angiographic morphology was analyzed. In stable angina 78% of ischaemia producing lesions appeared as simple stenoses, while 92% of the unstable or more severely ischaemic patients exhibited complicated lesions (p less than 0.001). In unstable angina and non-Q infarction a complex lesion was present respectively in 71% and 60% of the cases; clear-cut intraluminal thrombosis was demonstrated in 23% of unstable angina, in 30% of non-Q wave infarction and in 39% of transmural infarction (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Temporal reproducibility of diastolic filling parameters derived from Doppler left ventricular inflow time velocity curves. Studies in normal subjects.

Doppler left ventricular inflow time-velocity curves have been extensively used to evaluate left ventricular filling. The reproducibility of the technique, however, has been rarely assessed, its temporal variability being, presently, unknown. In order to define the temporal reproducibility of Doppler parameters of left ventricular filling, 10 normal subjects were studied at 3 different times (baseline, after 24 hours, and after 1 week). No parameter changed significantly during 1 week follow-up. When variability, however, was expressed as percent changes relative to baseline, values ranging from 5.7% to 25% were found, the largest variability being associated with acceleration-deceleration parameters (p less than 0.001). In conclusion temporal variability of parameters obtained from Doppler diastolic mitral time-velocity curves is acceptable in homogeneous groups of subjects. When the technique, however, is used for serial evaluation of ventricular filling in a single patient, the specific temporal variability of the parameter considered must be taken into account before any variation can be ascribed to real hemodynamic changes.

[The Italian Group for the Study of Streptokinase in Myocardial Infarct: Echocardiographic study]. / G.I.S.S.I.: Studio ecocardiografico.

Seventeen of the centres participating to the G.I.S.S.I. trial performed also, before discharge from the Hospital, an echocardiographic examination of patients (pts) included in the study. 561 pts were included, 280 assigned to the streptokinase (SK) treatment, and 281 to the control (CT) group. The echocardiographic asynergic area score index was lower in the SK pts than in the CT group (p less than 0.01). The difference was more evident in pts treated within 6 hours from the onset of symptoms (p less than 0.005), in pts without previous infarct (p less than 0.005), and in pts aged over 65 (p less than 0.005). The end diastolic (EDV) and the end-systolic (ESV) volumes were lower in SK pts (p less than 0.01 and p less than 0.025 respectively) than in the CT group; the ejection fraction (EF) did not differ. The reduction of EDV and ESV was more evident in pts treated within 6 hours, in pts without previous infarct, in pts aged over 65, and in anterior infarcts. At the 6-month follow-up examination, in SK pts the asynergic area score index, the EDV, the ESV and the EF were unmodified; in CT pts, on the contrary, the EDV and the ESV were significantly increased (p less than 0.05 and p less than 0.025 respectively).