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Biomedical subjects

M G Russo

Publications and source records attributed to M G Russo.

At least 37 records · Page 2Linked to original sources
PubMed2000

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↗
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

[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↗
PubMed1996

Conotruncal anomalies in prenatal life.

This retrospective multicenter study represents an analysis of the intrauterine determinants of the prognosis for conotruncal anomalies. Data regarding reason for referral, presence of chromosomal or extracardiac anomalies, pregnancy and surgical outcome were recorded in 67 cases of conotruncal anomalies from three Italian referral units. Chromosomal aberrations effected 11 of the 60 (18.3%) fetuses in which a karyotype was available. Extra-cardiac malformations were present in 25/67 cases (37.3%). No chromosomal anomalies were present in fetuses with complete or corrected transposition of the great arteries. However, tetralogy of Fallot and double-outlet right ventricle were associated with chromosomal anomalies in 22% and 38% of cases, respectively, and with extracardiac anomalies in 45% and 46% of cases, respectively. Only 20 of the 67 (31%) cardiac malformations were associated with an abnormal four-chamber view. There were 28 (41.7%) terminations of pregnancy, six (8.9%) intrauterine deaths and 16 (23.8%) neonatal deaths. Seventeen neonates (25.3%) are currently alive, and 15 of these have undergone reparative surgery. The prognosis of conotruncal anomalies is poorer when the conditions is diagnosed in utero. This is mainly due to the frequent association with chromosomal and/or extracardiac anomalies, often leading to intrauterine or early neonatal death.

Chromosome Aberrations↗
PubMed1995

Correlation between infection and the onset of the giant cell (temporal) arteritis syndrome. A trigger mechanism?

OBJECTIVE: To assess for a correlation between infection and the onset of the giant cell (temporal) arteritis (GCA) syndrome. METHODS: A matched case-control study design was used. Records of 100 patients with biopsy-proven GCA and 100 patients undergoing corrective surgery for hip fracture who did not have GCA were retrospectively reviewed. Non-GCA patients were sex-matched with GCA patients and were as old or older in age. The review period for GCA patients was up to 4 months before and during the occurrence of symptoms (median 2 months), and for non-GCA patients, it was up to 7 months before hip fracture. The prevalence of infection was compared using matched-pairs odds ratios and their 95% confidence intervals. RESULTS: Infections were 3 times more likely to occur in GCA patients than in non-GCA patients (P < 0.05). CONCLUSION: A correlation between the occurrence of infection and the onset of GCA is strongly suggested. We speculate that infection may act as a trigger mechanism in the pathogenesis of this syndrome.

Aged↗
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↗
PubMed1991

Multiple sclerosis in childhood. Longitudinal study in 14 cases.

The Authors review the literature on the occurrence of multiple sclerosis in children and add 14 personal cases below the age of 15.5 years, the youngest being 6.4 years old. Modality of onset, clinical course, clinical classification according to the criteria proposed by McDonald and Halliday, paraclinical evidence of lesions and disability grade at the last control are widely discussed. Eleven cases were scheduled as clinically definite multiple sclerosis. In the youngest children the recovery may be often complete or the disability grade may be low.

Adolescent↗