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Biomedical subjects

G Pacileo

Publications and source records attributed to G Pacileo.

At least 19 recordsLinked to original sources

Strain rate imaging: data acquisition and postrocessing.

Several studies already demonstrated the clinical relevance of strain rate imaging. Unfortunately, so far only few echolaboratories are using this technique in their clinical practice. This is mainly due to the lack of information on how to perform a standard strain rate imaging study. Thus, the aim of the present review is to provide the bases and methodology to perform a correct strain rate study.

Echocardiography↗

Pulmonary stenosis and atresia with intact ventricular septum during prenatal life.

AIM: To identify fetal echocardiographic characteristics predictive of perinatal outcome in cases with a prenatal diagnosis of pulmonary stenosis or pulmonary atresia. PATIENTS AND METHODS: We retrospectively reviewed the records and the videotapes of all the cases of pulmonary stenosis and pulmonary atresia diagnosed at our institutions between 1990 and 1999. The following measurements were obtained: diameters of right and left atria and ventricles and ventricular wall thickness; main pulmonary artery and aortic root diameter; direction of flow through the atrioventricular, aortic and pulmonary valves and through the ductus arteriosus. Perinatal outcome and follow-up of the survivors were available in each case. RESULTS: There were 21 cases of pulmonary atresia. Eleven were diagnosed before 24 weeks and nine of them (82%) underwent termination of pregnancy. The survival rate was 50% among the 12 fetuses born at term. None of the fetuses that survived had a large right ventricle, while this was a finding in 50% of those that died. Among the fetuses that died, 83% had a hypertrophic right ventricular wall compared to 33% of the survivors. There were 12 cases of pulmonary stenosis. Three cases were diagnosed before 24 weeks but none underwent termination of pregnancy. All the fetuses with pulmonary stenosis were born at term and four died in the perinatal period. The survival rate was thus 66.6% (8/12). Three (75%) of the fetuses that died had reversed flow in the ductus arteriosus compared with one of the fetuses that survived. CONCLUSION: Our data suggest that a grossly enlarged right ventricle and/or a hypertrophied right ventricular wall in cases of pulmonary atresia and reversed flow in the arterial duct in cases of pulmonary stenosis are likely indicators of a poor prognosis.

Cardiomyopathy, Dilated↗

Subclinical hypothyroidism and Down's syndrome; studies on myocardial structure and function.

BACKGROUND: The management of subclinical hypothyroidism (SH) is still controversial, as the benefit to risk ratio of prolonged L-thyroxine therapy is not clear cut. Some authors have shown abnormalities of myocardial function and structure in adults with SH, which could be reversed by L-thyroxine therapy. As SH frequently affects children with Down's syndrome (DS), and almost one half of these are affected by congenital heart disease, a concomitant SH related impairment of cardiac function might further compromise their clinical condition. AIMS: To establish whether SH influences myocardial structure and function in children with DS. METHODS: Sixteen children with DS and untreated SH and 25 matched euthyroid controls with DS underwent echocardiographic analysis of left ventricular mechanics and tissue characterisation. RESULTS: None of the 16 patients had myocardial impairment. CONCLUSION: Results suggest that children with DS who have SH are not at risk of cardiac disease. Clinicians should consider these data in the management of SH, as the benefit to risk ratio of prolonged L-thyroxine therapy is not clear cut.

Adolescent↗

Ultrasound evaluation of aortic valve anatomy in the fetus.

OBJECTIVE: To assess the feasibility of ultrasound identification of aortic valve anatomy in the fetus, with particular emphasis on the detection of bicuspid aortic valve. METHODS: This study was a prospective analysis of 21 fetuses with prenatally diagnosed congenital left heart obstructive lesions and 45 normal fetuses undergoing routine ultrasound evaluated at a tertiary referral center. These fetuses underwent detailed echocardiography, including the study of the aortic valve on a targeted short-axis view of the right ventricle. Necropsies or postnatal echocardiograms were available for confirmation of the diagnosis in all cases. RESULTS: Aortic cusps and commissures were satisfactorily visualized in 38/45 (84%) normal fetuses and in 18/21 (86%) fetuses with congenital heart disease. The aortic valve was correctly defined as bicuspid in one normal fetus and in six fetuses with congenital heart disease. In two fetuses with a positive family history, the bicuspid aortic valve was isolated. There was one incorrect diagnosis (a unicuspid unicommissural valve diagnosed prenatally as a bicuspid aortic valve in a fetus with severe aortic stenosis) and one false-positive diagnosis in a fetus diagnosed with a coarctation and a bicuspid aortic valve late in the third trimester of pregnancy and in which both anomalies were not confirmed at neonatal echocardiography. CONCLUSIONS: This study demonstrated that aortic valve anatomy can be satisfactorily assessed in fetuses with and without left heart obstructive lesions. We believe that a detailed search for a bicuspid aortic valve should be attempted in all patients referred for a positive family history of congenital heart disease, in general, and of left ventricle outflow tract obstruction or bicuspid aortic valve, in particular. In fact, the presence of an asymptomatic bicuspid aortic valve has been demonstrated to represent an important factor predisposing to the development of bacterial endocarditis and dissecting aortic aneurysm late in adult life. Therefore, an early detection of such an anomaly may contribute to ensure a longer symptom-free lifespan of individuals with the most common cardiac anomaly at birth.

Aortic Coarctation↗

Complete transposition of the great arteries: patterns of congenital heart disease in familial precurrence.

BACKGROUND: Transposition of the great arteries (TGA) is considered to be associated only rarely with genetic syndromes and to have a low risk of precurrence among relatives of affected patients. Because most family studies have involved a relatively small number of patients and evaluated all types of TGA as a single group, we performed a large, prospective study investigating the precurrence of congenital heart disease in families of children with complete, nonsyndromic TGA. METHODS AND RESULTS: From January 1997 through December 2000, 370 patients with nonsyndromic, complete TGA were consecutively evaluated and enrolled in the study. The occurrence of cardiac and noncardiac anomalies among relatives of the probands was investigated. Relatives with congenital heart disease were found in 37 of 370 families (10%), including 5 of 37 families (13.5%) with more than one affected relative. TGA itself was the most common precurrent malformation: complete TGA occurred in 6 families and congenitally corrected TGA occurred in 5 families. Precurrence risks for congenital heart disease were calculated at 1.8% (8 of 436) for siblings, 0.5% (4 of 740) for parents, 0.5% (16 of 3261) for first cousins, 0.2% (4 of 2101) for uncles/aunts, and 0.06% (1 of 1480) for grandparents. CONCLUSIONS: The present study shows that TGA is not always sporadic in families. Precurrence of concordant cardiac defects within affected family members supports monogenic or oligogenic inheritance of TGA in certain kindreds. Moreover, the occurrence of complete TGA and congenitally corrected TGA among first-degree relatives in several different families strongly suggests an underlying pathogenetic link between these 2 malformations that has been previously unrecognized.

Adolescent↗

Left ventricular remodeling and mechanics after successful repair of aortic coarctation.

Forty normotensive patients (mean age 12.3 +/- 6.5 years) followed up after a successful repair of aortic coarctation (mean age at coarctectomy 5.1 +/- 4.8 yrs) were studied by echo-Doppler to (1) evaluate left ventricular (LV) remodeling and endocardial and midwall mechanics, and (2) identify factors that might predispose to persistent abnormalities. Sex- and age-specific cutoff levels for LV mass/height2.7 and relative wall thickness were defined to assess LV geometry. To adjust for age-and growth-related changes in ventricular mechanics, all echocardiographic variables were expressed as a Z-score relative to the normal distribution. In addition, the smallest diameter of the aorta was assessed by magnetic resonance imaging and calculated as percent narrowing compared with the diameter of the aorta at the diaphragmatic level. In the study group, 24 of 40 patients (60%) had normal LV geometry. Among the 16 patients (40%) with abnormal LV geometry, 5 (12.5%) had a pattern of concentric remodeling and 11 (27.5%) an eccentric hypertrophy. LV hypertrophy was marked (LV mass index >51 g/m2.7) in 5 of these patients. No patient had a pattern of concentric hypertrophy. LV contractility was increased (Z-score >95th percentile) in 28 patients (70%) as assessed using the endocardial stress-velocity index. In contrast, LV contractility assessed using midwall stress-velocity index remained elevated (Z-score >95th percentile) in 15 patients (37.5%). The stepwise multiple logistic regression analysis was not able to detect any significant independent predictor of abnormal LV remodeling, including sex, age at surgical repair, length of postoperative follow-up, heart rate, body mass index, systolic and diastolic blood pressure, and smallest diameter of the aorta, as well as indexes of LV geometry (shape, mass, volume, mass/ volume ratio) and function (preload, afterload, pump function, and myocardial contractility). Thus, normotensive patients after surgical repair of aortic coarctation may be in an LV hyperdynamic cardiovascular state (more frequent in those who have undergone late repair) and have multiple patterns of LV geometry.

Adolescent↗

Left ventricular outflow tract obstruction in the transposition of great arteries defined by transthoracic three-dimensional echocardiography.

We assessed the feasibility of transthoracic three-dimensional reconstruction of the pulmonary valve and subpulmonary left ventricular outflow tract in two patients with transposition of great arteries, ventricular septal defect, and obstruction to the left ventricular outflow tract. Three-dimensional reconstruction of the pulmonary valve could be displayed as "en face" through a three-dimensional generated "pulmotomy view," allowing an overview of the pulmonary aspect of the valve from a surgeon's perspective. In similar fashion, reconstruction of the subpulmonary outflow tract could be displayed along its longitudinal axis as seen through a left ventriculotomy. Unique views could be obtained equivalent to surgical or autopsy dissections, allowing more complete understanding of the morphology and severity of left-sided obstructive lesions.

Echocardiography, Three-Dimensional↗

New insights in the pathophysiology of mitral and aortic regurgitation in pediatric age: role of angiotensin-converting enzyme inhibitor therapy.

This review has been focused on the new insights in the pathophysiology of mitral and aortic regurgitation and on the role of ACE-inhibitor therapy in children with chronic volume overload due to left-sided valvular lesions. Recent clinical studies show that these drugs have favorable effects when administered orally in chronic mitral and aortic regurgitation. Interestingly, the beneficial effects of ACE-inhibition regard the basic anatomic, hemodynamic and adaptive pathologic conditions related to volume overload, namely, the regurgitant orifice area and volume and ventricular remodeling. The heart is a plastic structure, constantly being altered in size, shape and composition in response to chronic volume overload. Thus, modulation of cardiac plasticity by ACE-inhibition raises the possibility of using new therapeutic strategies specifically designed to prevent and/or antagonize the mechanical disadvantages secondary to volume overload-induced cardiac remodeling. The beneficial effects of ACE-inhibition have also been observed in growing children with asymptomatic valvular regurgitation; thus, it appears that the unloading therapy has the potential of influencing the natural history of both mitral and aortic regurgitation and possibly delays surgical valve repair or replacement. These data justify early inhibition of the renin-angiotensin system in children with left ventricular volume overload due to mitral and aortic regurgitation.

Angiotensin-Converting Enzyme Inhibitors↗

Echocardiographic assessment of ventricular filling pressure during the second and third trimesters of gestation.

AIMS: To confirm changes in the atrioventricular diastolic flow velocities (peak E, peak A, E/A ratio) with gestational age and to define whether these changes genuinely reflect variations in filling pressure in the fetal heart. METHODS: Fifty normal pregnancies were studied between the 13th and the 37th weeks of gestation. The fetal flow velocity patterns were evaluated by pulsed-wave (PW) Doppler and the annular velocities of the atrioventricular valves by PW-Doppler tissue imaging (DTI). RESULTS: All indexes evaluated (atrioventricular peak flow and annular velocities) correlated significantly with gestational age. This correlation was stronger for the early diastolic indexes (tricuspid E and EA, 0.69 and 0.78; mitral E and EA, 0.61 and 0.77, respectively) and weaker for the end-diastolic indexes (tricuspid A and AA, 0.46 and 0.37; mitral A and AA, 0.45 and 0.39, respectively). Neither mitral nor tricuspid E/Ea ratio changed significantly with gestational age. CONCLUSIONS: The lack of correlation between the Doppler-assessed ventricular filling pressures and gestational age suggests absence of significant changes of ventricular compliance during the second and third trimesters of pregnancy, and a progressive enhancement of active relaxation and/or changes in loading conditions.

Adult↗

Critical left ventricular outflow tract obstruction due to accessory mitral valve tissue.

Left ventricular outflow tract (LVOT) obstruction due to anomalous tissue tag arising from the mitral valve is a rare congenital cardiac anomaly. It generally becomes symptomatic during the first decade of life as exercise intolerance, chest pain, or syncope at effort. To date, only a few cases of critical systemic obstruction due to isolated mitral valve anomaly in neonates have been reported. We report the case of a neonate who was a few hours old and was referred in severe clinical condition due to critical left ventricular outflow obstruction resulting from an anomalous tissue tag of mitral valve origin.

Blood Flow Velocity↗

Hypertrophic cardiomyopathy in pediatric patients: effect of verapamil on regional and global left ventricular diastolic function.

OBJECTIVE: To assess the effects of treatment with verapamil on regional and global left ventricular (LV) diastolic function in paediatric patients with hypertrophic cardiomyopathy (HCM). DESIGN: Twelve patients (age range 5.1 to 12.3 years, median 8.6) with HCM were evaluated during ongoing chronic oral treatment with verapamil (4 mg/kg/day) and four days after withdrawal of therapy. Twelve age- and body surface area-matched normal children served as controls. In an echocardiographic study, global LV diastolic function was evaluated by assessing isovolumic relaxation time (IVRT) and mitral flow indexes, including peak filling rate normalized to mitral stroke volume (PFR/SV). In addition, regional LV diastolic function was assessed by pulsed-wave Doppler tissue imaging at the subendocardial portion of the middle region of the anterior and posterior interventricular septum, and anterolateral and inferior walls to measure the peak velocities and the velocity-time integrals of myocardial excursion in both early diastole and atrial systole. In addition, as an index of diastolic asynchrony (AsyI), the variation in time to peak filling rate, measured as the time from the peak of the R wave on the electrocardiogram to the peak of the regional E wave, among the four myocardial regions was defined by subtracting the smallest value from the greatest and expressing the difference as a percentage of the smallest value. RESULTS: Compared with the controls, patients with HCM without therapy showed a longer IVRT (P<0.01) and a decrease in PFR/SV (P<0.01) without a compensatory increase in filling during atrial systole. Oral administration of verapamil induced a significant shortening of the IVRT (P=0.003) and an increase in PFR/SV (P=0.02). Furthermore, patients with HCM without therapy showed a significantly longer time to peak filling rate (P<0.01) associated with a decreased peak velocity in early diastole without a concomitant increase in peak velocity during atrial systole in each of the myocardial regions. Furthermore, the AsyI was higher in the HCM group than in controls (19% versus 6%, respectively), and this index was inversely correlated with the PFR/SV (r=-0.86, P<0.001). The regional diastolic velocity of the myocardium at each of the four analyzed regions was not significantly different with verapamil, but the AsyI was significantly lower (P<0.05). CONCLUSIONS: Children with HCM show abnormalities of both global and regional LV diastolic function. In these patients, chronic administration of verapamil plays a crucial role in the improvement in global LV filling and, as a consequence, in clinical manifestations. The beneficial effects of verapamil seem to be related to a reduction in diastolic asynchrony more than to significant changes in diastolic velocities of the myocardial fibres.

Calcium Channel Blockers↗

[Epidemiologic surveillance of ischemic heart disease in the population of Pavia in 1986-1995].

BACKGROUND: In Italy mortality for coronary heart disease is continuously decreasing in males and females. However, it is not yet clear how much of this decline is attributable to a longer survival of coronary heart disease patients or to a real decreased incidence in the population. The aim of this paper was to analyze coronary heart disease mortality trends in the population of Pavia, case-fatality rates and acute myocardial infarction attack rates. METHODS: Mortality surveillance was carried out by the Epidemiological Unit of the ASL of Pavia; acute myocardial infarction attack rates were estimated from regional admission data for the Pavia population. The target population (1991 Census) was represented by two groups: the first was equal to 49,326 residents (23,627 males and 25,699 females) 45-64 years of age, the second was equal to 17,208 residents (7236 males and 9972 females) 65-74 years of age. RESULTS: The decline in mortality was mainly observed in males aged 45-64. Acute myocardial infarction attack rates showed a decline in 45-64 men and an increase in the oldest age group. CONCLUSIONS: The surveillance of coronary heart disease epidemiological data from 1986 to 1995 in this population showed a decreased mortality mainly attributable to the decline of attack rates in the youngest and only to case-fatality rates in the oldest age group.

Aged↗