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Biomedical subjects

G Pacileo

Publications and source records attributed to G Pacileo.

At least 37 records · Page 2Linked to original sources
PubMed1999

Arrhythmogenic substrate in young patients with repaired tetralogy of Fallot: role of an abnormal ventricular repolarization.

Ventricular repolarization analysis has been shown to be effective in the identification of electrical myocardial instability leading to ventricular arrhythmias. The aim of the present study was to examine ventricular repolarization time indexes, in terms of both absolute measures and dispersion across the myocardium, in young patients with repaired tetralogy of Fallot (41 pts; 28M/13F, age 11.7+/-3.6 years), assessing, furthermore, the possible influence of known negative prognostic factors relative to the surgical operation and residual haemodynamic abnormalities. The data of the study group were compared with those of 33 aged-matched asymptomatic control subjects (22M/11F, age 11.7+/-2.3 years). Ventricular depolarisation, as expressed by QRS duration, resulted significantly longer in total Fallot group than in the Control group (P<0.0001). Particularly, patients operated through a right ventricular approach showed higher values of QRS interval (P<0.0001) than those operated through a combined transatrial-transpulmonary approach. All the patients operated on for tetralogy of Fallot exhibit, with respect to control subjects, an inhomogeneous prolongation of ventricular repolarization across the myocardium, as showed by the significant increase in the absolute indexes of ventricular repolarization, JTc (P<0.001), QT (P<0.0001) and QTc (P<0.0001) with a concomitant prolongation of the indexes of dispersion of ventricular recovery time, QTcD (P<0.0001), JTcD (P<0.0001), 'adjusted' QTcD (P<0.001) and Tp-Te interval (P<0.0001). A temporal and regional variation in the ventricular repolarization across the myocardium in patients with repaired tetralogy of Fallot, could create the pathophysiological substrate for an increased cardiac electrical instability. The presence of negative prognostic factors, relative to the surgical intervention or residual haemodynamic abnormalities, even if not influencing the arrhythmic substrate, invariably present, could determine 'trigger' conditions essential for the development of ventricular arrhythmias.

Adolescent↗
PubMed1999

Hemodynamic effects of a single oral dose of enalapril among children with asymptomatic chronic mitral regurgitation.

BACKGROUND: Angiotensin-converting enzyme inhibitors have been shown to have beneficial effects in the short- and long-term treatment of adult patients with chronic mitral regurgitation. The safety and efficacy of such treatment have not been established for children. The objective of this study was to assess the effect of the angiotensin-converting enzyme inhibitor enalapril on the severity of valvar mitral regurgitation and the systolic performance of overloaded left ventricle of children. METHODS: Ten patients 3 to 16 years of age (mean age 9.6 +/- 3.8 years) with moderate to severe chronic mitral insufficiency were examined by means of Doppler echocardiography before and 2 hours after receiving a single oral dose of enalapril (0.40 mg/kg). Effective regurgitant orifice area, regurgitant volume and fraction, left ventricular end-diastolic and end-systolic volumes indexed for body surface area, left ventricular pump function (total ejection fraction), left ventricular contractility (stress-adjusted velocity of shortening) and afterload (peak systolic and end-systolic circumferential wall stress), and systemic vascular resistance were calculated before and after treatment. RESULTS: The following values decreased significantly compared with baseline values: effective regurgitant orifice area (36.2 +/- 17.4 versus 25.9 +/- 16.5 mm(2), P =.00008), regurgitant volume (53.6 +/- 27.4 versus 36.1 +/- 24.5 mL, P =.0002), regurgitant fraction (56.7 +/- 14.5% versus 39.9 +/- 17.0%, P =. 0009), left ventricular end-diastolic volume indexed for body surface area (81.3 +/- 17.4 versus 76.1 +/- 16.1 mL/m(2), P =.005), left ventricular end-systolic volume indexed for body surface area (26.7 +/- 9.1 versus 22.6 +/- 8.9 mL/m(2), P =.02), afterload (peak systolic circumferential wall stress 135.8 +/- 15.3 versus 123.5 +/- 19.7 g/cm(2), P =.005; end-systolic circumferential wall stress 57.8 +/- 12.4 versus 48.3 +/- 12.8 g/cm(2), P =.005), and systemic vascular resistance (2012.2 +/- 536.1 versus 1622.7 +/- 389 dyne. sec. cm(-5), P =.005). Left ventricular pump function increased (total ejection fraction 67.6 +/- 5.7% versus 71.7 +/- 6.5%, P =. 005) without significant changes in left ventricular contractility (stress-adjusted velocity of shortening -0.35 +/- 0.8 versus -0.21 +/- 1.3 SD, P not significant). CONCLUSIONS: The data showed that for pediatric patients single-dose treatment with oral enalapril reduces the severity of mitral regurgitation and improves left ventricular loading conditions and systolic performance without impairment of myocardial contractility. Persistence of these unloading effects in long-term therapy might slow the evolution of left ventricular dysfunction caused by overload-induced myocardial damage and possibly delay the time at which surgical repair or replacement of the mitral valve becomes necessary.

Administration, Oral↗
PubMed1999

Repeat syncopal attacks due to postsurgical right ventricular pseudoaneurysm.

Pseudoaneurysm of the right ventricular outflow tract is a rare lesion caused by disruption of the ventricular wall that allows the blood to leak into the surrounding space. It often complicates surgery involving right ventriculotomy and progressively increases in size, therefore causing airway compression, pulmonary perfusion asymmetry, thromboembolism, and rupture. We report on a patient who developed right ventricular pseudoaneurysm early after surgery for atrio-ventricular septal defect with tetralogy of Fallot and needed emergency surgical repair due to low cardiac output and repeat syncopal attacks.

Adolescent↗
PubMed1999

Left ventricular midwall mechanics in healthy children and adolescents.

The assessment of left ventricular function in pediatric age generally has been made by measuring the extent and velocity of fiber shortening at the endocardium and relating these parameters to systolic wall stress. It has been suggested from animal and human studies that this "endocardial method" can result in an overestimation of myocardial function when healthy hearts and those with hypertrophy are compared. Thus an assessment of left ventricular pump function and contractility that takes into account the epicardial migration of the midwall circumferential fibers during systole (midwall analysis) is warranted. Although the normal range for midwall indexes of left ventricular mechanics has been established in adult subjects, up to now it has not been studied in pediatric subjects. To establish normal values for left ventricular midwall mechanics, 70 healthy children ranging in age from 3 days to 18 years were evaluated with 2-dimensional and M-mode echocardiography. Midwall fractional shortening (FSmw), rate-corrected mean velocity of circumferential fiber shortening (VCFcmw), and the relation between these indexes and left ventricular end-systolic wall stress (ESS) were calculated separately for the children younger than 2 (group 1, 20 subjects) and older than 2 (group 2, 50 subjects) years of age. Group 1 had significantly higher FSmw and VCFcmw compared with group 2. An inverse linear relation between FSmw and ESS and between VCFcmw and ESS was found in both age groups. The y-intercept was higher in group 1, and the slope of the mean regression line was steeper than in group 2 for both the relationships, suggesting an age-dependent midwall left ventricular pump function and contractility. These normative data can be used to assess the left ventricular midwall mechanics in pediatric patients with pressure or volume overload.

Adolescent↗
PubMed1998

Left ventricular mechanics after closure of ventricular septal defect: influence of size of the defect and age at surgical repair.

To evaluate the influence of the size of the defect and the age of surgical repair on left ventricular mechanics, including geometry, shape, diastolic and systolic function as well as myocardial contractility, we used cross-sectional echo-Doppler to study 20 patients (12 males, 8 females) who had undergone successful surgical closure of a ventricular septal defect. The patients were divided in two groups, corrected early and late, on the basis of the degree of left-to-right shunting (ratio of pulmonary to systemic output of greater or less than 2.5/1) and the age at the surgical repair (older or younger than 2 years of age). The group undergoing early correction included 11 patients, mean age 7.1+/-1.8 years (range 4.2-11.8 years), having surgery at mean age of 1.3+/-0.6 years for a large ventricular septal defect (mean ratio of pulmonary to systemic output of 3.1/1; range 3.4-2.7/1) with a mean postoperative follow-up 4.6+/-1.9 years. The group of nine patients undergoing late correction had a mean age of 11.3+/-4.9 years (range 6.7-17.2 years), with a later surgical repair (mean age 4.7+/-2.7 years) for a moderate-sized ventricular septal defect (mean pulmonary/systemic output ratio 2.1/1; range 2.3-1.7) and a mean postoperative follow-up of 7+/-4.2 years. Each group of surgically repaired patients was compared with a control group matched for age, body surface area and gender. No significant differences were found between the normal controls and those undergoing early correction for any assessed functional index regarding left ventricular geometry (normalized volumes and mass for body surface area, mass/volume and thickness/radius ratios), shape (long axis-short axis ratio), diastolic (mitral and pulmonary venous flow patterns) and systolic (fractional shortening and rate-corrected mean velocity of circumferential fibre shortening) function. In addition, the data points for each patient for the rate-corrected mean velocity of circumferential fibre shortening to end-systolic stress relationship were within the 95% confidence limits of normal, suggesting normal left ventricular contractility. On the other hand, the patients undergoing surgery at a later age showed a persistent increase of the normalized left ventricular end-diastolic volume and mass, with an higher mass/volume ratio and reduced end-systolic stress compared with normal controls. Furthermore, left ventricular shape (long axis-short axis ratio) was abnormal at end-diastole but with its normal values at end-systole. Our data suggest that, in the presence of a large ventricular septal defect, early successful surgical repair <2 years of age results in complete recovery of left ventricular mechanics in the postoperative follow-up. In contrast, surgical closure at > 2 years of age, even for a moderately sized ventricular septal defect, deleteriously affects postoperative left ventricular geometry and shape. Since prolonged volume overload may be detrimental to myocardial function, earlier surgical repair should be recommended.

Adolescent↗
PubMed1998

Prenatal diagnosis of 22q11 microdeletion in a fetus with a conotruncal heart defect.

A case of prenatal diagnosis of 22q11 microdeletion in a fetus with a conotruncal heart defect is described. This type of chromosomal aberration has been shown to be present in up to 30% of isolated conotruncal anomalies and in most cases of DiGeorge and velocardiofacial syndromes. The implications of such a diagnosis on prenatal counselling are discussed.

Amniocentesis↗
PubMed1998

Prenatal diagnosis of aortic insufficiency.

The prenatal diagnosis of various forms of left ventricular outflow tract obstruction has been reported in detail. However, the appearance and the characteristics of fetal aortic insufficiency have been described only in association with complete absence of the value. We report here a case of aortic insufficiency associated with severe aortic stenosis. Features characteristic of insufficiency were severe cardiomegaly due to left ventricle dilatation and the hypocontractility of the myocardium. The aortic root was enormously dilated and the semilunar valve showed thick and dysplastic cusps. On color Doppler, the retrograde high-velocity regurgitant jet was clearly visible.

Adult↗
PubMed1998

[Dispersion of ventricular recovery time following surgery for tetralogy of Fallot: correlation with negative prognostic factors].

Malignant ventricular arrhythmias have been reported in patients with repaired tetralogy of Fallot. The aim of this study was to examine ventricular repolarization time indexes, in terms of both absolute measures and dispersion across the myocardium, in young patients operated on for tetralogy of Fallot (32 patients; 19 males and 13 females, mean age 11.1 +/- 3.4 years); these electrocardiographic parameters have been shown to be effective in the identification of electrical myocardial instability and hence of risk for ventricular arrhythmias too. The electrocardiographic data of the study group were compared with those of 22 age-matched asymptomatic control subjects (14 males and 8 females, mean age 12 +/- 1.5 years). Furthermore it has also been investigated the possible influence on ventricular repolarization of known negative prognostic factors relative to the surgical approach, age at intervention, and presence of pulmonary obstruction and/or regurgitation. No patients in the study group revealed at the Holter recordings and/or at the exercise test severe ventricular arrhythmias. From the analysis of ventricular depolarization, expressed by QRS duration, emerged that it resulted significantly longer in total Fallot group (p < 0.0001), and in each subgroup (p < 0.05) compared to the control group. Particularly, patients operated through a right ventricular approach showed higher values of QRS interval (p < 0.05) than those operated through combined transatrial-transpulmonary approach. All patients operated on for tetralogy of Fallot showed, compared to control subjects, a non homogeneous prolongation of ventricular repolarization across the myocardium, as confirmed by the significant increase in the absolute indexes of ventricular repolarization, JTc (p < 0.001), QT (p < 0.0001) and QTc (p < 0.0001) with a concomitant prolongation of the indexes of dispersion of ventricular recovery time, QTc dispersion (p < 0.0001), JTc dispersion (p < 0.0001), "adjusted" QTc dispersion (p < 0.05) and T peak-T end interval (p < 0.0001). The non homogeneous ventricular repolarization across the myocardium, preceding the development of arrhythmic events, could be the effect of the right ventricular morphological and functional changes of tetralogy of Fallot predisposing to the development of ventricular reentry tachyarrhythmias.

Adolescent↗
PubMed1997

Myocardial dysplasia in a 3rd-trimester fetus. An ultrasound and pathologic study.

Arrested myocardial development, often described as spongiosum heart, has been reported in association with obstructive semilunar valve disease and, much more rarely, as a primary disease in adolescents and adults. To our knowledge, this condition has never been diagnosed in utero. We describe the echocardiographic and pathoanatomic findings of the 1st case of myocardial dysplasia detected in utero by ultrasound. A 28-year-old woman, gravida 2, para 1, was referred to our unit at 34 weeks of gestation due to severe fetal hydrops. On echocardiography, we observed gross fetal cardiomegaly (particularly of the septal and ventricular myocardium), an unusually bright myocardial echostructure, thick trabeculations in both ventricular chambers, and severe loss of myocardial contraction. There were normal ventriculoarterial connections and no signs of obstructive semilunar valve disease. After fetal death, necropsy confirmed the presence of spongiosum heart and the diagnosis of myocardial dysplasia--which term best describes this disorder in its various temporal expressions. Because this condition has never before been observed prenatally, no consideration has been given to intrauterine management. We recommend that fetal cardiac function be monitored echocardiographically whenever a pregnant patient has a positive family history of this disease. There is a possibility that the life of the affected fetus might be prolonged beyond the gestational period by avoiding intrauterine cardiac decompensation, through early delivery. We recommend further that the parents of these children be advised of the risks associated with future pregnancies. Little is known about the pattern of inheritance of myocardial dysplasia, but the disorder appears to be familial. Therefore, the possibility that it may recur within the same generation must be taken into account.

Adult↗
PubMed1996

Myocardial ultrasonic tissue characterization in pediatric and adult patients with hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) has different clinical and prognostic aspects in young than in adult patients. This study was undertaken to determine whether these reported differences are reflected by changes in ultrasonic backscatter parameters and whether oral treatment with verapamil modifies backscatter variables in children with HCM. METHODS AND RESULTS: Sixty-eight subjects underwent backscatter analysis to assess the ultrasonic myocardial reflectivity and the amplitude of the cardiac cycle-dependent variation of the backscatter power curve. Subjects were divided into four groups: 10 HCM and 23 normal subjects < 10 years old and 17 HCM and 18 normal adults. Myocardial reflectivity and amplitude of the cyclic variation were assessed in the septum and in the posterior wall of all subjects. The children with HCM were restudied after long-term oral administration of verapamil. Both children and adult patients, compared with the normal control groups, showed a significant reduction in the amplitude of the cyclic variation both in the septum and in the posterior wall. In contrast, myocardial reflectivity, although significantly increased in adult HCM patients, was unchanged in the young HCM group. Verapamil administration did not significantly alter the results in the children with HCM. CONCLUSIONS: These data demonstrate that in young HCM patients, the ultrasonic myocardial reflectivity is normal, in contrast to the significant increase observed in adult patients affected by the same disease. This observation is in accord with the different clinical manifestations reported in young HCM patients and indicates an age-dependent difference in the echogenic structure of the hypertrophied myocardium in HCM.

Adult↗
PubMed1992

Tetralogy of Fallot in three siblings: a familial study and review of the literature.

We report a family in which three out of four siblings had tetralogy of Fallot (TOF). The family history showed TOF in the daughter of a maternal cousin, while no other congenital heart diseases were discovered. Although no teratogenic environmental agent was discovered, the absence of parental consanguinity and the presence of another affected relative suggest multifactorial inheritance. Autosomal recessive inheritance cannot be ruled out.

Female↗
PubMed1992

[The evaluation of atherosclerotic lesions of the extracranial carotid arteries in hypertensive subjects. A study by B-mode and pulsed Doppler echotomography].

Arterial hypertension is considered an independent atherosclerotic risk factor. In hypertensive patients it increases cardiovascular morbidity and mortality risk. The aim of the study was to emphasize the presence of atherosclerotic lesions at the level of extracranial carotid tree in patients with essential arterial hypertension. In 110 hypertensive patients (63 males, 47 females) mean age 66 +/- 16 years, and in 100 normotensive patients (66 males, 34 females) mean age 65 +/- 15 years, high-resolution B-mode pulsed wave Doppler echotomography of internal, external and common carotid artery of both sides was performed. The value of the intimal-medial complex thickness of common carotid was measured, and the stenosis percentage evaluated by B-mode pulsed wave Doppler echotomography. Atherosclerotic plaques were classified according to their echogenic characteristic and surface; their localization was then evaluated. In hypertensive patients we observed the presence of common carotid intimal-medial thickening in 75.4%, atherosclerotic plaques in 60.9% and stenosis in 17.9%; 58.3% of these ones were localized at internal carotid, 33.3% at common carotid and 8.3% at external carotid. We observed that 36% of the 100 normotensive patients presented common carotid intimal-medial thickening (p < 0.001 versus hypertensives), 25% atherosclerotic plaques (p < 0.001 versus hypertensives) and 8% stenoses; 50% of these stenosis were localized at internal carotid, the second half at external carotid. In conclusion, high-resolution B-mode echotomography study with pulsed wave Doppler spectral analysis represents a necessary method to evaluate the condition of the extracranial carotid tree in patients with hypertension.

Adult↗
PubMed1991

Truncus arteriosus and double aortic arch associated with DiGeorge syndrome.

We report the 1st known case in which truncus arteriosus and double aortic arch have been associated with DiGeorge syndrome. The association of these 2 cardiovascular anomalies lends support, by itself, to speculation that truncus arteriosus and double aortic arch have a common embryonic pathogenesis; and the presence of these anomalies in a patient with DiGeorge syndrome strengthens the contention that the common causative factor is pathologic development of the neural crest cells.

Journal Article↗