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Biomedical subjects

L Daliento

Publications and source records attributed to L Daliento.

At least 73 records · Page 4Linked to original sources
PubMed1988

Familial occurrence of right ventricular dysplasia: a study involving nine families.

Right ventricular pathologic involvement, with autopsy evidence of fibrous and fatty infiltration of the right ventricle, was investigated in members of families in which cases of juvenile sudden death had occurred. Seventy-two subjects from nine families were studied. Sixteen died at a young age and 56 are living. Postmortem investigation in 11 cases (mean age at death 24 years) revealed massive replacement of the right ventricular free wall by fat or fibrous tissue. In the 56 living patients clinical examination included an electrocardiogram (ECG) at rest, ambulatory ECG recording, posteroanterior and lateral chest roentgenograms, M-mode and two-dimensional echocardiograms and exercise stress tests. In 14 patients, hemodynamic, angiographic and electrophysiologic studies were also carried out; right ventricular endomyocardial biopsy was performed in four. Structural and dynamic right ventricular impairment was detected in 30 living patients (mean age 25 years), and concomitant mild left ventricular abnormalities were present in 4. In eight of the nine families studied at least two members were affected. Ventricular arrhythmias (Lown grade greater than or equal to 4a) were recorded in more than half of the cases. The data reveal that right ventricular dysplasia shows a familial clustering and causes electrical instability that may place affected subjects at risk of sudden death. The mean age of these subjects suggests that the disease is manifested at a young age with a polymorphic clinical and arrhythmic profile. Finally, because this disease is a primary disorder of the ventricular myocardium, it should be included among the cardiomyopathies.

Adolescent↗
PubMed1988

Accelerated idioventricular rhythm of infundibular origin in patients with a concealed form of arrhythmogenic right ventricular dysplasia.

Five apparently healthy people (aged 16-47) presented with recurrent episodes of accelerated idioventricular rhythm characterised by left bundle branch block and right axis deviation. Clinical history, physical findings, basic electrocardiogram, chest x ray, and blood tests were within normal limits in all. Holter monitoring, exercise stress test, and electrophysiological study (in three patients) showed that accelerated idioventricular rhythm was mainly bradycardia dependent, easily suppressed by effort and overdrive pacing, and originated from the outflow tract of the right ventricle. The mechanism could be enhanced automaticity. Data from cross sectional echocardiography (in all patients) and from haemodynamic evaluation (in three) identified structural or wall motion abnormalities of the right ventricle or both without appreciable dilatation of the ventricle. Biopsy specimens of the right ventricular endomyocardium showed fibrosis in one patient, fibrosis and fatty infiltration in the second, and pronounced fatty infiltration in the third. These results show that some patients with accelerated idioventricular rhythm have right ventricular abnormalities that are typical of the localised and concealed forms of arrhythmogenic right ventricular dysplasia.

Adolescent↗
PubMed1988

[Arrhythmogenic right ventricular dysplasia. Study of a selected population].

268 patients (pts) aged between 4 and 63 (average block 33.6) years were examined in an effort to detect structural and/or wall motion abnormalities of the right ventricle, consistent with a diagnosis of Arrhythmogenic Right Ventricular Dysplasia (ARVD). The patients included in this study had some of these features: 1) sudden juvenile death (age less than 35 years) due to heart disease; 2) relatives of pts died suddenly of pathologically proven ARVD; 3) pts with ventricular arrhythmias grade Lown greater than 3, and with QRS morphology mainly of left bundle branch block; 4) pts between the ages of 18 and 40, with negative T waves beyond V2; 5) pts with ventricular arrhythmias of left bundle branch block morphology, and grade Lown greater than 1, and negative T waves beyond V1. ARVD was recognized in 108 living and 18 deceased pts. Our data confirm that ARVD is a wide spectrum disease, going from the classical form described by Marcus and Fontaine to concealed forms characterized mainly by premature ventricular complexes.

Adolescent↗
PubMed1988

Repair of congenitally malformed mitral valve in children.

We report our experience with 30 children under 15 years of age treated surgically for congenital mitral valve disease from March 1972 to July 1986. Valve reconstruction was possible in 26 patients (87%), whereas in four the valve was replaced with a mechanical prosthesis. Five patients died in the hospital (16.6%), four after conservative operations and one after mitral valve replacement. There was only one late death in a child in chronic congestive heart failure. Three patients, treated conservatively, required valve replacement 2, 22, and 24 months, respectively, after the operation. Follow-up data reveal that 22 of 24 patients are asymptomatic 5 months to 15 years after operation. Two-dimensional echocardiographic studies were performed in 19 patients treated conservatively, 17 of whom are asymptomatic. Eleven of them have no signs of mitral regurgitation or stenosis, six show only mild mitral incompetence, and two have moderate mitral regurgitation or stenosis. Peak pulmonary artery pressure is within normal limits in all. Our results indicate that mitral valve reconstructive procedures for congenital valve dysplasia may be effective and reliable in children despite the frequent severity of valve malformation. Although no major prosthesis-related complications were observed in the present series, we believe that mitral valve repair should always be attempted in the pediatric population to avoid the drawbacks of the currently available prostheses.

Adolescent↗
PubMed1987

A polymorphic form of familial arrhythmogenic right ventricular dysplasia.

Thirty-two members of a family were studied. Three of them died in their youth and had evidence of arrhythmogenic right ventricular (RV) dysplasia. The other 29 members underwent clinical examination, electrocardiography, chest x-ray and M-mode and 2-dimensional echocardiography. Fourteen patients found to have structural abnormalities of the right ventricle underwent 24-hour ambulatory electrocardiographic recording and symptom-limited bicycle stress testing. Hemodynamic and angiographic studies were performed in 6 of these patients. In this family the arrhythmogenic RV dysplasia showed a wide variation of abnormalities, ranging from mild, local alterations to generalized involvement of the right ventricle. The patients were separated into 3 groups on the basis of both the clinical profile and noninvasive/invasive studies: 3 subjects who died suddenly; 3 subjects who had severe ventricular arrhythmias; and 8 subjects in whom RV impairment was not associated with any significant arrhythmias. There was no close relation between the severity of the RV abnormality and presence of ventricular arrhythmias. The variability of the RV abnormality and the high prevalence of this condition in this family is consistent with a genetic pattern of autosomal dominance with incomplete penetrance.

Adolescent↗
PubMed1987

[Ebstein disease and pregnancy].

Three patients with Ebstein's disease became pregnant 11 times, 6 of which ended as spontaneous abortion, 2 newborn deaths and 3 successful term pregnancies. Ventricular overloading, arterial hypoxemia and arrhythmias are the major physiopathologic determinants. The ventricular overloading is the most important risk factor for the mother, so as the hypoxemia and the arrhythmias for the fetus. Pregnancy in these patients must be considered at high risk, and a close cardiologic assess with continuous hormonal controls of fetus maturity during the last trimester should be done. A team including obstetricians, cardiologists and anesthesiologists is suggested for the care of these patients in the view of a successful outcome.

Abortion, Incomplete↗
PubMed1987

[Double outlet right ventricle. Angiographic study].

Cineangiographic study in 41 patients with double outlet right ventricle was performed. Segmental approach was utilized to describe the anatomical features. Atrial situs was inversus in 5 cases, ambiguous in 3 and solitus in the other cases. Atrio-ventricular (a-v) connections were biventricular in 34 and univentricular in 7. A common a-v valve was found in 2 cases. Mitral valve was imperforated in 1 and straddling in 1. The most frequent relationship between aorta and pulmonary artery was side by side (41.1%). Ventricular septal defect was subaortic in 14, subpulmonary in 9, double committed in 5 and non committed in 6. In 27 cases a double infundibulum was present; in 7 patients we described a subpulmonary conus only. Some nosological aspects of this congenital heart disease are discussed particularly in conformity with the surgical needs.

Adolescent↗
PubMed1986

Rhythm and conduction disturbances in isolated, congenitally corrected transposition of the great arteries.

The incidence and types of rhythm and conduction disturbances in 11 male and 6 female patients with congenitally corrected transposition of the great arteries and no other intrinsic complicating anomalies were studied. Patient age ranged from 5 to 54 years; follow-up ranged from 5 to 37 years. Surface electrocardiograms were recorded in each patient; 15 also underwent 24-hour Holter monitoring and 10 underwent electrophysiologic study. The conduction system of a 54-year-old woman who died suddenly, with complete atrioventricular (AV) block since age 30 years, was studied by serial histologic sections. Fifteen patients were asymptomatic and 2 reported repeated episodes of palpitation since childhood. Ten patients presented with a normal PR interval and 2 with first-degree AV block (12%). Five patients had complete AV block (29%), but none had had it at birth; first- and second-degree AV block preceded complete AV block in 2 patients. The morphologic pattern and duration of QRS suggested a junctional rhythm in 4 patients and an idioventricular pacemaker in 1 patient. Electrophysiologic studies confirmed that the complete AV block site was supra-Hisian in 2 patients and proximal to the His bundle bifurcation in 1 patient. However, histologic investigation disclosed fibrosis and disruption of the proximal nonbifurcating His bundle in the patient who died suddenly. In 2 patients with recurrent supraventricular tachycardia, electrophysiologic studies suggested reentry through James fibers (or dual AV nodal pathway) in 1 and the presence of a left lateral accessory AV pathway in the other. Holter monitoring showed a high incidence of ventricular arrhythmias.

Adolescent↗
PubMed1986

Pulmonary atresia with intact ventricular septum: a quantitative cineventriculographic study of the right and left ventricular function.

Quantitative cineventriculographic measurements (ventricular volumes, ejection fraction, and myocardial mass) were obtained in 15 neonates with pulmonary atresia and intact ventricular septum. There was a wide dimensional range for the right ventricle, from a reduced through normal to enlarged. A restrictive tricuspid valve (less than 12 mm) was associated with a small or diminutive right ventricle. A normal function of the right ventricle was present only in those cases with normal-sized chambers. The left ventricular end-diastolic volume was always greater than normal. The ejection fraction was normal only if a normal myocardial mass was present. Additional abnormalities were frequent in the myocardium of both ventricles, such as extreme thinning of the wall of the right ventricle and hypoplasia or fibroelastosis of the left ventricle. All these factors can lead to a poor surgical prognosis despite good anatomical correction.

Cardiomegaly↗
PubMed1986

Ventricular development and function in complete transposition: angiocardiographic evaluation.

We studied 50 left ventricular cineangiograms and 41 right ventricular cineangiograms of 40 patients with usual atrial arrangement (situs solitus) together with concordant atrioventricular and discordant ventriculo-arterial connexions (complete transposition), catheterized between 1 day and 12 months of age. Our purpose was to evaluate ventricular development and function. The patients were subdivided on the basis of associated lesions into groups with intact ventricular septum; with ventricular septal defect; with ventricular septal defect together with pulmonary stenosis and with pulmonary stenosis in isolation. Each group was further separated according to age into those patients below and above 60 days. Ventricular volumes, ejection fraction and the ratio between systolic pressure and end-systolic volume were evaluated for both ventricles. The left ventricular mass, stress, and the ratio of stress to end-systolic volume were also calculated. A volume overload leads to increased left ventricular volume even at birth. With an intact ventricular septum, the left ventricle in patients with complete transposition is normal at birth and also during the first weeks of life. Myocardial mass, however, does not increase proportionately with increase in volume as the patient grows and it remains inadequate by the age of one year. The left ventricular mass is also inadequate in patients with associated anomalies when the left ventricular pressure is less than 60 mm Hg. Moreover, the left ventricle in presence of an intact ventricular septum presents a decrease in contractility during the first year of life despite the finding of a satisfactory arterial oxygen saturation. The right ventricle has a normal volume at birth which increases during the first year due to a greater diastolic filling following atrial septostomy. We noted a progressive decrease in ejection fraction, however, which is related to various factors including a reduction in contractility.

Angiocardiography↗
PubMed1986

[Complete atrioventricular block and sudden death in isolated corrected transposition].

The rate of av conduction disturbances in corrected transposition of the great arteries is high, possibly as consequence of mechanical injury by ventricular output along with the mis-positioned specialized av junction. A sudden death occurred in a 54 year old female affected by a chronic "idiopathic" complete av block, who showed at autopsy a heart with av and ventricular arterial discordance in the absence of other anomalies (isolated corrected transposition). The cardiac arrest had the ecg features of ventricular fibrillation, preceded by sustained ventricular tachycardia. Histologic study of the conduction system by serial sections confirmed the anterior position of the connected av node, with a long subendocardial course of the bundle in the pulmonary outflow tract. The histologic basis of the block consisted of sclero-atrophy of the common bundle, quite similar to the substrate of acquired av block in heart with normal connections. Possible etiologies, responsible of the relation between the av block and onset of ventricular arrhythmias, are discussed.

Death, Sudden↗
PubMed1986

[Tachycardia and ventricular fibrillation in the arrhythmogenic right ventricle (arrhythmogenic dysplasia of the right ventricle). Clinical and electrocardiographic spectrum].

In this paper we report the arrhythmias recorded on basal ECG, on Holter monitoring or on exercise test, in 32 pts affected by arrhythmogenic right ventricle (ARV). A sustained ventricular tachycardia (VT) was present in 11 pts a non sustained VT in 15 pts, a slow VT in 2 pts, a ventricular fibrillation (VF) in 3 pts and both sustained VT and VF in 1 pt. All but 1 case of sustained VT showed a LBBB like pattern. The heart rate during VT ranged between 170 and 280 beats/min. The frontal axis of the VT showed a wide range of deviation. Among non sustained VT, 9 cases had LBBB like pattern and 6 cases had polymorphic configuration. The 2 cases of slow VT showed LBBB like pattern with right axis deviation. A comparison between ventricular arrhythmias and RV impairment was made. The data obtained suggest that the effort plays an important role in the induction of VT in pts with localized RV impairment. In conclusion a wide spectrum of ventricular tachyarrhythmias is present in the ARV. Probably the RV "arrhythmogenic" zones and the electrophysiological mechanism causing the arrhythmias are various.

Adolescent↗
PubMed1985

Uncommon pattern of tricuspid stenosis in Ebstein's anomaly.

The echocardiographic and cineangiographic features of an unusual type of tricuspid stenosis in Ebstein's anomaly are described. The displaced septal and mural leaflets of the tricuspid valve are inserted to an anomalous apical muscle shelf and cause a restrictive communication between the atrialized portion and the trabecular zone of the right ventricle. The antero-superior leaflet is not involved.

Adult↗
PubMed1985

Natural and post-surgical history of mitral stenosis and mitral stenosis and insufficiency: an observational study.

Natural and post-surgical history has been investigated in 410 pts with mitral stenosis and 209 pts with mixed mitral stenosis and regurgitation. They had undergone cardiac catheterization in the years 1968-1980. Hemodynamic data and clinical status (NYHA class) have been statistically analyzed in order to obtain prognostically useful parameters. In mitral stenosis peak pulmonary artery pressure is the most important parameter for natural history, whereas cardiac index is the leading parameter in the operated patients. Commissurotomy has a very low surgical mortality, largely due to the better conditions of the patients undergoing this type of conservative surgery. Results are similar in mixed mitral stenosis and regurgitation. Surgery markedly improves survival in comparable patients. Therefore, intervention seems to be indicated especially in patients with elevated pulmonary artery pressure, because they can get the maximum advantage at a minimal risk.

Adult↗
PubMed1984

Dysplasia of the atrioventricular valves associated with conduction system anomalies.

Clinical, vectorcardiographic, and echocardiographic data from two siblings with atrial septal defects and dysplasia of the mitral and tricuspid valves are reported. Vectorcardiograms showed that both siblings had abnormal ventricular activation with initial electrical forces directed posteriorly. One sibling died after surgery, and necropsy showed incomplete differentiation of the leaflets and tensor apparatus producing anomalies resembling "mitral arcade." Serial histological examination of the conducting tissue showed that the atrioventricular node was located on the left side of the atrial septum, that the central fibrous body and the membranous septum were hypoplastic, and that an accessory nodoventricular pathway originating in the compact node joined the left side of the ventricular septum. This accessory pathway was probably the cause of the unusual ventricular activation. Dysplasia of the mitral and tricuspid valves together with hypoplasia of the central fibrous body and the presence of accessory pathways are probably part of a malformative complex caused by incomplete differentiation of both the cardiac atrioventricular valves and the junctional area.

Child↗