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Biomedical subjects

L Ballerini

Publications and source records attributed to L Ballerini.

At least 73 records · Page 4Linked to original sources

Cardiomyopathy and multicore myopathy with accumulation of intermediate filaments.

A girl affected by a restrictive cardiomyopathy with neuromuscular involvement is described. Morphological examination showed a pattern of multicore myopathy and with electron microscopy a sarcoplasmic accumulation of electron dense granular and filamentous material was demonstrated both in skeletal muscle and heart. This peculiar electron dense material corresponded to increased desmin in muscle and cardiac fibres and was demonstrated immunohistochemically.

Adolescent↗

Effect of the nootropic drug oxiracetam on field potentials of rat hippocampal slices.

1. The effect of the nootropic drug oxiracetam on hippocampal neurotransmission was investigated in the CA1 region of the rat hippocampal slice in vitro by use of extracellular recordings. 2. Superfusion of oxiracetam (0.1-100 microM) produced a concentration-dependent, wash-resistant (greater than 90 min), increase in initial slope and amplitude of the dendritic field excitatory postsynaptic potential (e.p.s.p.). This increase was maximal at a concentration of 1 microM (70%). 3. Input-output curves relating the initial slope to the amplitude of the afferent volley were significantly (P less than 0.05) steeper and showed a greater maximal response in the presence of 1 microM oxiracetam than in control conditions. 4. Two trains of high frequency stimulation (100 Hz, 0.4 s, 5 min apart) delivered in the stratum radiatum 30 min after washout of oxiracetam (1 microM) still elicited a long-term potentiation (LTP) of the field e.p.s.p. However, the absolute magnitude of the LTP produced did not differ from that obtained in untreated slices. 5. After induction and establishment of LTP, oxiracetam (1 microM) had a smaller (27%) and reversible effect on the evoked field e.p.s.p. 6. D-2-Amino-5-phosphonopentanoic acid (AP-5), at the same concentration (50 microM) which in our conditions prevented the induction of LTP, blocked the action of 1 microM oxiracetam and strongly depressed the effect of higher concentrations of the nootropic drug. 7. It is concluded that oxiracetam provokes an enduring increase of neurotransmission in the CA1 rat hippocampal region. This action appears to share some features with LTP as indicated by its persistence, sensitivity to AP-5 and lack of additivity with electrically-induced LTP.

2-Amino-5-phosphonovalerate↗

Ventricular inversion with truncus arteriosus.

We report clinical, angiocardiographic, and anatomic findings of an infant with ventricular inversion (atrioventricular discordance) and truncus arteriosus. The patient was in congestive heart failure and the injections into the left ventricle, right ventricle, and truncus arteriosus allowed a precise diagnosis. The autopsy confirmed this very unusual combination of anomalies and in particular it showed a truncus type 2 completely surrounded by muscular infundibulum and mainly aligned with the right ventricle. Surgical correction of this malformation is a challenge and so far, to our knowledge, has never been reported in the literature.

Abnormalities, Multiple↗

[Non-surgical closure of patent ductus arteriosus in children with the Rashkind double disk occluder].

Since the first clinical application of transcatheter closure technique for patent ductus arteriosus in children and infants in 1977 by Rashkind and Coll., this technique is routinely performed only in a few major Pediatric Cardiology Centers. We report our successful series which is the first of its kind both in Italy and in Southern Europe. The series included 11 children aged 1.6 to 10 years (mean age 4.5 years) all affected with patent ductus arteriosus (PDA). Subaortic stenosis (1) and ventricular septal defect with pulmonary stenosis (1) were associated anomalies. In all of the cases, after the standard percutaneous cardiac catheterization, the Mullins method was attempted to advance and deliver the Rashkind PDA double-disk occluder. Successful closure was accomplished in 10 while almost complete closure was achieved in the 11th. Normal Doppler flow pattern after the procedure confirmed the successful results. No complications occurred. Although our experience is limited, non-surgical PDA closure provided an excellent alternative to surgical procedure.

Cardiac Catheterization↗

Univentricular atrioventricular connection to a dominant left ventricle with a concordant ventriculo-arterial connection.

Double inlet left ventricle with concordant ventriculo-arterial connection, first described by Holmes, is an unusual cardiac malformation which includes a right-sided rudimentary right ventricle and, frequently, subpulmonary stenosis. We have now encountered six patients, aged 1 month to 13 years, with this basic combination. In 5 cases, cross-sectional echocardiography, using parasternal, apical and subcostal views was diagnostic. Subpulmonary stenosis was seen in two of them. The sixth case shown to have atresia of the left atrioventricular valve, a concordant ventriculo-arterial connection, a left-sided rudimentary right ventricle, mild subpulmonary stenosis and a right-sided aorta. The combination of the left atrioventricular valve, left-sided rudimentary right ventricle and a concordant ventriculo-arterial connection has not, to our knowledge, been previously reported. Cross-sectional echocardiography always provided the correct morphologic diagnosis.

Adolescent↗

Percutaneous balloon dilatation of stenotic truncal valve in a newborn.

We describe the use of percutaneous balloon angioplasty of a stenotic truncal valve in a newborn with a common arterial trunk. A 12 mm balloon catheter was chosen, this representing 80% of the diameter of the ventriculo-truncal junction. The peak systolic gradient dropped from 45 to 15 mm Hg. The procedure can avoid, or certainly delay, surgical treatment of the stenotic truncal valve.

Catheterization↗

Congenital mitral valve anomalies in transposition of the great arteries.

Seventy-nine specimens with transposition of the great arteries were reviewed. Among them were 45 with ventricular septal defect, including 6 with so-called "posterior aorta transposition". The morphology of the mitral valve was carefully studied. Mitral valve anomalies were found in 24 cases (30%). The anomalies involving the mitral valve were classified into 4 groups according to the different components of the valve: A) specimens with anomalies of the leaflets--2 cases with mitral cleft; B) those with anomalies of the commissures--1 case with a mitral gap; C) those with abnormalities of the chordae tendinae--2 cases of malattachment of the chordae tendinae to the ventricular septum and 2 with bridging chordae between the papillary muscles; and D) those with abnormalities of the papillary muscles--5 cases with a parachute valve, 10 with a "form fruste" of the parachute mitral valve (hypoplasia of one papillary muscle and short chordae tendinae) and 2 with abnormally placed papillary muscles, which reached the pulmonary annulus. In 14 cases, abnormalities of the tricuspid valve were also encountered, including 8 cases with a tricuspid gap and 6 with a malattachment of tricuspid chordae on the border of a ventricular septal defect. It can be concluded that mitral valve anomalies are not uncommon in transposition of the great arteries. This pathology must be carefully evaluated at the time of surgery, particularly in those patients for whom an anatomic correction is considered, for in those cases the left ventricle will be supplying the systemic circulation and therefore, a completely normal mitral valve will be required.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗

Long-term results after extracardiac valved conduits implanted for complex congenital heart disease.

Between August 1982 and December 1986, 56 patients survived implantation of an extracardiac valved conduit for complex congenital heart disease. The mean age at operation was 4.2 years (16 days to 24 yrs) and the mean weight was 15.9 kg (2.4 to 93.0 kg). The diagnosis was pulmonary atresia (PA) with ventricular septal defect (VSD) in 13 patients, tetralogy of Fallot in 11, transposition of the great arteries (TGA) with VSD in 8, truncus arteriosus, in 7, complex left ventricular outflow tract obstruction (LVOTO) in 6, complex left atrioventricular valve obstruction in 4, double outlet right ventricle with VSD and subaortic obstruction in 3, univentricular heart with pulmonary stenosis in 2, TGA with LVOTO in 1, and PA with intact ventricular septum in 1. In 35 patients, a preclotted conventional Dacron conduit (CDC) with bioprosthetic valve was used, in 19 patients a collagen-sealed Tascon valved conduit (TC) was implanted, and in 1 patient an aortic homograft was used. In a mean follow-up of 32.5 months (9 to 64 mo), there were two deaths (2/56, 3.6%) that were not related to the conduit. All survivors have been evaluated by two-dimensional and Doppler echocardiography, and 29/56 (51.8%) underwent cardiac catheterization. Nine patients (9/56, 16.1%) underwent successful valved conduit replacement, in seven cases with a nonvalved conduit. There was a significant difference (P = .011) with regard to the incidence of conduit replacement between the group with CDC (2/36, 5.5%) and the group with TC (7/19, 36.8%). Five patients underwent percutaneous transluminal balloon dilatation of the prosthetic conduit, with adequate relief of the gradient in four patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Surgical bypass of the systemic atrioventricular valve in children by means of a valved conduit.

The surgical approach to relief of mitral stenosis in children is still a controversial problem. We describe our experience with four severely symptomatic children in whom a valved conduit from the left atrium to the left ventricle was successfully used to bypass a hypoplastic systemic atrioventricular valve. A left atrial-left ventricular extracardiac conduit was implanted in these patients with a hypoplastic mitral anulus and an adequate left ventricular chamber. There were no early or late deaths. Postoperative cardiac catheterization performed in all patients 1 month after the operation showed reduced size of the left atrium, a reduction of the left atrial-left ventricular gradient from a mean of 14 mm Hg to a mean of 5 mm Hg, and an increase of the left atrial outlet from a mean diameter of 10.7 mm to 28.7 mm (including the diameter of the native mitral valve plus the internal diameter of the valved conduit). The application of this unconventional operation in children with congenital or acquired stenosis of the systemic atrioventricular valve should be considered when the mitral valve obstruction cannot be relieved by conventional valve repair or replacement. Furthermore, the left atrial-left ventricular conduit does not preclude future alternative surgical options.

Aorta↗

Palliative Mustard operation: an expanded horizon.

From March 1982 to December 1983, five patients with a mean age 7 years (4 months-16 years) underwent a palliative Mustard operation for complex cardiac anomalies. The diagnoses were: univentricular heart with pulmonary vascular obstructive disease (PVOD) (2 cases); criss-cross heart with intact atrial septum, ventricular septal defect (VSD), transposition of the great arteries (TGA) and moderate pulmonary stenosis; isolated atrioventricular discordance with VSD; TGA, diminutive right ventricle and multiple VSDs. Cardiac catheterization showed unfavourable direction of flow: the oxygen saturation in the aorta was lower than in pulmonary artery, with a mean difference of 13.5% (range 6% to 30%). In no case was anatomical or physiological repair considered advisable because of the unfavourable intracardiac anatomy or the presence of PVOD. In two patients the atrial rerouting was accompanied by pulmonary artery banding. There were no hospital or late deaths in a mean follow-up of 36 months (range 29 to 50 months). The mean post-operative arterial oxygen saturation was 95.4% (range 92% to 99%).

Adolescent↗

Indication for systemic-pulmonary artery shunts guided by two-dimensional and Doppler echocardiography: criteria for patient selection.

From June, 1983, to February, 1987, 91 patients with pulmonary outflow tract obstruction underwent a systemic-to-pulmonary artery shunt without an invasive study. The surgical indication was based on two-dimensional (2-D) and Doppler echocardiography only. In 82 patients (90.1%), 2-D and Doppler echocardiography allowed correct assessment of the intracardiac anatomy and of the morphology of the pulmonary arteries, pulmonary veins, and aortic arch. The aortic arch anatomy was misdiagnosed in 6 patients (6.6%) who underwent a modified instead of a classic Blalock-Taussig shunt. In 3 patients (3.3%), the indication for the systemic-pulmonary artery shunt was inappropriate; in 1, operative death resulted from the incomplete diagnosis. After the preselection of patients based on knowledge of the anatomical patterns, a systemic-pulmonary artery shunt may be performed without cardiac catheterization if these rules for definitive patient selection are followed: (1) accurate clinical assessment to identify the patients with restricted pulmonary blood flow; (2) precise intracardiac diagnosis by 2-D and Doppler echocardiography; and (3) definitive 2-D echocardiographic visualization of the pulmonary venous connection and the confluence of the pulmonary arteries.

Anastomosis, Surgical↗

Injury to the left coronary artery during repair of tetralogy of Fallot: successful aorta-coronary polytetrafluoroethylene graft.

Inadvertent transection of an anomalous left coronary artery, which originated from the right coronary artery, occurred during repair of tetralogy of Fallot in a 2-year-old child. An aorta-coronary bypass graft was constructed with a size 4 polytetrafluoroethylene conduit. Early recatheterization showed patency of the polytetrafluoroethylene graft, but a myocardial scintiscan done 3 months after operation demonstrated reduced perfusion of the areas supplied by the left coronary system. Preoperative aortography or selective coronary angiography is mandatory to avoid this potentially lethal complication. Future surgical options are discussed.

Blood Vessel Prosthesis↗

Patterns of pulmonary arterial anatomy and blood supply in complex congenital heart disease with pulmonary atresia.

To identify the pattern of pulmonary anatomy and pulmonary blood supply, we analyzed the angiocardiograms of 40 patients with pulmonary atresia and complex congenital heart disease. All patients but two (95%) had confluent pulmonary arteries supplied by a patent ductus arteriosus. These two patients (5%) had nonconfluent pulmonary arteries, one with major systemic-pulmonary collateral arteries and absent patent ductus arteriosus and the other with a patent ductus arteriosus supplying the right lung and small collateral arteries supplying the left. The low incidence of pulmonary arterial abnormalities and the uniform pattern of pulmonary blood supply make the patients with pulmonary atresia and complex congenital heart disease potential candidates for palliative operation without cardiac catheterization in the neonatal period.

Adolescent↗

Anatomical-echocardiographic correlations in pulmonary atresia with intact ventricular septum. Use of subcostal cross-sectional views.

Cross-sectional echocardiographic images from 26 patients presenting pulmonary atresia with intact ventricular septum were compared with anatomical findings in specimens having the same anomaly. The echocardiographic study was based on a subcostal approach using oblique projections, and specimens were cut in the same planes. Patients were classified into three groups. In the first group the morphologically right ventricle was represented by all of its three component parts: inlet, trabecular and outlet (12 cases). The second group was made up of patients with overgrowth of the apical component. Consequently the right ventricle had only two effective components: inlet and outlet (6 cases). In the third group, muscular overgrowth of both outlet and apical portions meant that the right ventricle was represented only by an inlet portion (8 cases). The good morphologic assessment and correlation with anatomic cuts, obtained in particular by using the "right oblique equivalent" view can avoid invasive study in this malformation prior to palliative surgery.

Angiocardiography↗

Prevalence of left-sided obstructive lesions in patients with atrioventricular canal without Down's syndrome.

Ninety consecutive patients with atrioventricular canal were studied to identify the association between left-sided obstructive lesions and Down's syndrome. Three groups were recognized: Group I, 76 patients with atrioventricular canal and no left-sided obstructions; Group II, nine patients with atrioventricular canal and left-sided inlet obstructions; Group III, five patients with atrioventricular canal and left-sided outlet obstructions. Of the 90 patients studied, 47 had Down's syndrome and only one of these had a left-sided obstructive lesion. Of 43 without Down's syndrome, 13 or 30% had left-sided obstructive lesions with predominating lesions at the inlet level caused by right ventricular dominance.

Aortic Coarctation↗