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Biomedical subjects

L Daliento

Publications and source records attributed to L Daliento.

At least 19 recordsLinked to original sources
PubMed1992

Clinical profile of concealed form of arrhythmogenic right ventricular cardiomyopathy presenting with apparently idiopathic ventricular arrhythmias.

In 24 subjects presenting with apparently idiopathic ventricular arrhythmias, a final diagnosis of arrhythmogenic right ventricular cardiomyopathy was formulated following global evaluation of the clinical, cross-sectional echocardiography and angiographic findings, and the observation of myocardial atrophy with fibrous-fatty substitution in right ventricular endomyocardial biopsy. All patients had good effort tolerance, and a normal cardiac silhouette. Ventricular arrhythmias with a left bundle branch block pattern were present in 23 cases (sustained ventricular tachycardia, nonsustained ventricular tachycardia, ventricular couplets, and ventricular premature complexes); 1 patient experienced an episode of ventricular fibrillation. A nearly constant electrocardiographic feature was T wave negativity in the right precordial leads. Cross-sectional echocardiography and hemodynamic studies showed that right ventricular impairment consisted only of localized structural and dynamic abnormalities; in a few cases the left ventricle was segmentally involved. Familial occurrence was present in 29% of the cases. No case of sudden death was observed during follow-up. These findings confirm that the concealed form of arrhythmogenic right ventricular cardiomyopathy is a cause of so-called "idiopathic" ventricular arrhythmias in subjects with apparently "normal hearts". Echocardiographic and angiographic investigations may lead to the correct diagnosis.

Adolescent↗
PubMed1992

Does Down syndrome affect prognosis of surgically managed atrioventricular canal defects?

The appropriateness of surgical correction of complete atrioventricular canal defect in patients with Down syndrome has been questioned on a cost-benefit basis. Our experience with nonselective correction of all patients with atrioventricular canal defects gave us the opportunity to evaluate the impact of Down syndrome on postoperative survival. Between January 1, 1975, and December 31, 1989, we operated on 94 patients with Down syndrome and on 127 genetically normal patients. One hundred thirty-four patients had partial or intermediate atrioventricular canal defect (28% Down patients) and 87 had complete atrioventricular canal defect (74% Down patients). Thirty-two patients died perioperatively and 10 patients died during the 15-year follow-up. The actuarial survival was 90% in the genetically normal patients and 57% in patients with Down syndrome (p < 0.0001). Nonetheless, when the confounding effects of pulmonary vascular resistance and the prevalence of more severe anatomic forms in patients with Down syndrome were eliminated with a multivariable analysis in the hazard domain, Down syndrome was not a significant independent incremental risk factor. This was verified by fitting parametric survival to actuarial survival graphically and by a testing of fit. Patients with Down syndrome underwent fewer reoperations and fared as well as or even better, on clinical and echocardiographic investigation, than their genetically normal counterparts.

Adolescent↗
PubMed1991

Electrical instability in patients undergoing surgery for atrioventricular septal defect.

Postoperative 24-hour Holter monitoring was performed in 106 patients with atrioventricular septal defect in order to identify the incidence of atrial and ventricular arrhythmias. Of the patients, 72 had separate atrioventricular orifices, including 13 with a small ventricular component to the defect, and 34 patients had a common atrioventricular orifice. Two groups of abnormal patients were found. First, patients with good electrical stability characterized by isolated atrial (9 patients) and ventricular (25 patients) extrasystoles falling in classes I and II of Lown. Second, patients with marked electrical instability characterized in one patient by repetitive atrial extrasystoles, in another by atrial flutter, in 2 by polymorphic ventricular extrasystole and in 8 by couplets or triplets. Electrical instability in individual patients was then compared, by means of logistic regression analysis, with operative, surgical and postoperative variables. There was no incidence of sudden death in our series. After surgical repair, ventricular arrhythmias were more frequent than atrial arrhythmias (33% vs. 10%) and were unrelated to the type of atrioventricular septal defect. Cardiac electrical instability after operation was significantly related to larger operative body size, higher postoperative end diastolic diameter of the right ventricle, larger size of the ventricular septal defect, coexistence of postoperative right bundle branch block and left anterior hemiblock. Conversely, the risk of arrhythmias was reduced by more recent operative data and by greater shortening fraction of the left ventricle.

Arrhythmias, Cardiac↗
PubMed1991

Is lung biopsy useful for surgical decision making in congenital heart disease?

Sixty patients with congenital heart disease (CHD), age range 3 months-45 years (median 2 years), underwent lung biopsy to exclude pulmonary vascular disease (PVD): 25 had an atrioventricular (AV) septal defect, 14 a ventricular septal defect (VSD), 7 a complete transposition of the great arteries (TGA) + VSD, 3 an atrial septal defect, and 2 a patent ductus arteriosus; 9 had other malformations. Scoring of histological section (0-4) according to a modified Heath-Edwards classification disclosed that 30 patients had severe "irreversible" PVD (greater than or equal to grade 3) (11 AV septal defect, 8 VSD, 6 TGA + VSD and 5 others); 8 patients were younger than 1 year. Pulmonary vascular resistance (PVR) was calculated in 51 patients and exceeded 7 U/m2 in 1 of 2 patients with grade 0, in 9 of 18 with grade 1, in 1 of 4 with grade 2, in 11 of 18 with grade 3, and in 8 of 9 with grade 4. PVR was also calculated after 100% oxygen administration in 19 patients, 14 of whom had a resting PVR greater than or equal to 7 U/m2. PVR persisted greater than or equal to 7 U/m2 in 5 patients: 4 had PVD greater than or equal to grade 3 and died after surgery. PVR fell to under 7 U/m2 in 14 patients, 3 of whom had PVD greater than or equal to grade 3:1 patient with grade 4 was not operated upon and 2 with grade 3 were operated upon: all are alive. Follow-up in discharged living patients was 100%, with a mean of 50 months. Fifty-five patients underwent surgery with 10 early and 2 late deaths. Among the 12 subjects with a fatal outcome, 10 had irreversible lesions. The 5 non-operated patients all had irreversible lesions at biopsy, and are still alive.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

[Congenital malformations of the mitral valve: clinical and morphological aspects].

The clinical, anatomic and functional features of 23 pts with congenital mitral valve malformation, seen at the University of Padua over a period of 10 years, are described. All pts underwent hemodynamic investigation and echo-Doppler evaluation. Diagnosis was confirmed at surgery in 17 cases, and at necropsy in six. In 9 pts mitral anomalies determined prevalent stenosis; the remaining 16 pts had moderate to severe mitral regurgitation. Associated cardiac malformations were present in 19 cases (82.6%). The clinical presentation occurred earlier in pts with mitral stenosis (16.9 months vs 6.55 years). The morphologic and echocardiographic features of each malformation are described. The term "valve dysplasia", as proposed by Becker et al to define the non-Ebstein malformations of the tricuspid valve, is herein applied to the congenital anomalies of the mitral valve in order to obtain a uniform and homogeneous approach to this vast spectrum of malformations.

Adolescent↗
PubMed1990

Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy.

Diagnostic sensitivity and specificity of cineangiography were evaluated by multivariate logistic discriminant analysis in 32 patients with arrhythmogenic right ventricular (RV) cardiomyopathy, 27 patients with biventricular dilated cardiomyopathy, 28 patients with atrial septal defect and 18 normal subjects. In patients with arrhythmogenic RV cardiomyopathy and biventricular dilated cardiomyopathy, the diagnosis was confirmed by endomyocardial biopsy. All RV values overlapped for the diagnosis of atrial septal defect and arrhythmogenic RV cardiomyopathy; overlapping extended to dilated cardiomyopathy for end-diastolic volume and infundibular dimensions. RV ejection fraction appeared reduced in all the diseases; in particular, mean values in dilated cardiomyopathy and arrhythmogenic RV cardiomyopathy were 38 and 53%, respectively (p less than 0.05). Left ventricular quantitative studies showed a significant difference between dilated and arrhythmogenic RV cardiomyopathy, both in terms of pumping indexes (mean end-diastolic volumes 180 vs 91 ml/m2 and mean ejection fraction 33 vs 60%), and indexes of contractility (stress/end-diastolic volume 3.7 vs 6.7). Multivariate analysis disclosed that transversally arranged hypertrophic trabeculae, separated by deep fissures, were associated with the highest probability of arrhythmogenic RV cardiomyopathy (p less than 0.001). Posterior subtricuspid and anterior infundibular wall bulgings were the only other independently significant variables. Coexistence of these signs was associated with 96% specificity and 87.5% sensitivity. Thus, arrhythmogenic RV cardiomyopathy presents quantitative volumetric and hemodynamic as well as qualitative features that clearly distinguish it from dilated cardiomyopathy and confirm its nosographic autonomy among the primary diseases of the myocardium.

Adult↗
PubMed1990

Monomorphic repetitive rhythms originating from the outflow tract in patients with minor forms of right ventricular cardiomyopathy.

We studied in detail 17 patients presenting with monomorphic repetitive ventricular rhythms having left bundle branch block morphology and right axis deviation. All had an apparently normal heart at physical examination. At chest radiography, three patients had mild cardiomegaly, and at electrocardiography, five patients had inverted T waves beyond V2. Five patients had syncope or near syncope. In seven patients the tachycardia occurred on effort. One patient died suddenly. The patients were extensively investigated, using cross-sectional echocardiography, complete haemodynamic and angiographic studies, electrophysiology and histology, to search for any structural basis of the arrhythmias. Tachycardia was sustained in 8 patients, nonsustained in 3, and consistent with accelerated idioventricular rhythm and repetitive paroxysmal ventricular tachycardia in 5 and 1 patients, respectively. Despite the differences in clinical and arrhythmologic features, similar abnormalities of right ventricular structure and/or wall motion were detected in all patients, consistent with localized forms of right ventricular cardiomyopathy. Different antiarrhythmic drugs were successfully used in twelve patients (the four patients with accelerated idioventricular rhythm were not treated). The patient who died suddenly had previously had a sustained ventricular tachycardia and was being treated by beta-blockade. Postmortem study revealed massive fibro-adipose substitution of the right ventricular free wall and pulmonary infundibulum.

Adolescent↗
PubMed1990

Solitary aorta with bilateral ductal origin of non-confluent pulmonary arteries in pulmonary atresia with intact ventricular septum.

We report an exceptional case of pulmonary atresia with intact ventricular septum in which non-confluent pulmonary arteries were supplied by bilateral arterial ducts, the existence of which had been diagnosed in life. One should be aware of this arterial pattern, even when the ventricular septum is intact in the setting of pulmonary atresia, because it requires a different surgical approach than when atresia is associated with a ventricular septal defect, namely construction of bilateral shunts for palliation followed by reconstitution of the pulmonary arterial confluence for definitive repair.

Aorta↗
PubMed1990

The concept of preload and its evaluation in the intact left ventricle.

Preload affects left ventricular pump function through the Frank-Starling curve by changing sarcomere length. However, common indices of preload, such as end-diastolic volume, or pressure, or stress, do not necessarily reflect sarcomere length. Ultimately, this depends on the elastic stiffness constant (k) and end-diastolic stress (sigma), which are not in simple relation with the above mentioned indices. An index of preload is proposed, (k sigma)(1/k). This index has been evaluated in 148 patients with different degrees of hemodynamic overload, and in 24 normal subjects. The preload index was found to be 1.448 +/- 0.034 in normal subjects. However, in the other patients evaluated preload index increased in mitral insufficiency (1.490 +/- 0.035), in decompensated aortic insufficiency (1.490 +/- 0.89) and in dilated cardiomyopathy (1.52 +/- 0.125), and markedly decreased in aortic stenosis (1.367 +/- 0.039) and in hypertrophic cardiomyopathy (1.41 +/- 0.034). It was always positively related to the afterload, measured as peak systolic stress. No positive relationship was found with end-diastolic volume nor pressure. Therefore, preload as a compensatory mechanism is differently recruited in response to various degrees of hemodynamic overload and parallels the afterload, in agreement with the concept of preload-afterload mismatch.

Aortic Valve Insufficiency↗
PubMed1990

[Analysis of the mode of transmission of right ventricular dysplasia].

A formal analysis of the mode of transmission of right ventricular cardiomyopathy was performed in seven families with this condition. Ninety-six subjects (81 family members, 15 connected) were studied. The index cases were family members who had died suddenly in their youth with autopsy evidence of massive fibrous-adipose right ventricular myocardial replacement. Pedigree analysis showed that 58 per cent of the family members were affected, with a male predominance (63% of men vs 53% of women). The kindreds were all normal and in none of the families were both parents affected. Carrier states were observed in both males and females and vertical transmission was demonstrated. Clinically, the disease was very variable with some cases showing widespread right ventricular involvement with or without cardiomegaly, and other cases showing localised right ventricular abnormalities. These data are consistent with a congenital disease with an autosomal dominant mode of inheritance with incomplete penetrance and variable expression.

Angiocardiography↗
PubMed1989

Ventricular fibrillation without apparent heart disease: description of six cases.

Since 1977, six patients (five males and one female), aged 14 to 35 years, resuscitated from ventricular fibrillation, were referred to our department for detailed evaluation, after exclusion of major cardiac pathologic conditions. Four patients had a family history of heart disease. Basic ECGs showed sinus rhythm in all of them. PR interval was prolonged in one. Two patients had complete and one had incomplete right bundle branch block. One patient had inverted t waves in V1-3 and late potentials. Three had an upsloping ST-T segment elevation in V1-2. The cardiothoracic index was less than 0.5 in five and 0.50 in one. In one of the five patients studied, the clinical episode of ventricular fibrillation was reproduced by stimulation of the right ventricular outflow tract during electrophysiologic study. Results of cross-sectional echocardiography and angiography showed predominantly structural and wall motion abnormalities of the right ventricle in five patients and slight wall motion abnormalities of the left ventricle in two. Two patients also had mitral and tricuspid valve prolapse. Coronary arteries were normal in all five patients examined. Results of endomyocardial biopsy showed no abnormalities in one patient, fibrosis in two, and fibrolipomatosis in one. Two patients died during follow-up: autopsy was performed in one and results showed right ventricular cardiomyopathy. Thus in five of these selected patients with apparent idiopathic ventricular fibrillation, some abnormalities, predominantly of the right ventricle, were documented only after detailed investigation; however, clinical history and some nonspecific ECG abnormalities were factors in the diagnostic procedure.

Adolescent↗
PubMed1989

Late results after resection of discrete and tunnel subaortic stenosis.

From May 1969 to June 1988, 84 consecutive patients ranging in age from 6 months to 61 years (mean 18 years) underwent surgery for fixed subaortic stenosis (SAS). A discrete fibrous or fibromuscular structure was present in 81 patients, while 3 presented with a tunnel type of obstruction. SAS was treated by sharp resection of the tissue and routine myotomy or myectomy of the hypertrophied left ventricular (LV) muscle (57 patients, group 1), while more recently, the lesion was treated by simple fibrous tissue enucleation (27 cases, group 2). There were 3 hospital deaths (3.6%) and 3 late deaths (overall mortality 7.1%). Eight patients required late reoperation because of recurrent SAS [3], aortic valve stenosis [2], aortic incompetence (AI) [2] and persistent mitral incompetence [1]. Seventy of 78 late survivors were reevaluated 3 months to 110 months after surgery (mean 75 +/- 48 months) by means of a complete cardiac catheterization or by 2-D echo and Doppler. The transaortic peak pressure gradient decreased from 97 +/- 43 (range 20-205 mmHg) to 11 +/- 16 mmHg (range 0-60 mmHg) in group 1 and from 72 +/- 38 mmHg (range 18-160 mmHg) to 3 +/- 7 mmHg (range 0-25 mmHg) in group 2 (P = NS). In 55 patients who have not undergone surgery on the aortic valve, AI remained unchanged in 31, decreased from mild to nil in 21 and from moderate to mild in 3. We conclude that simple blunt enucleation of SAS is an effective procedure in relieving LV outflow obstruction even if a myotomy or myectomy of the underlying hypertrophied muscle is not routinely used.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

Valve replacement in children: early and late results.

In order to analyze the problems associated with prosthetic valve replacement (PVR) in the pediatric population, we have reviewed 29 children, 3.5 to 15 years of age, who were operated upon from 1970 to 1986. The indications were congenital valve malformations in 16 patients, rheumatic valve disease in 7, degenerative disease in 2 and bacterial endocarditis in 4. Ten children underwent aortic valve replacement (AVR), 14 mitral valve replacement (MVR), 1 tricuspid valve replacement and 4 double valve replacement (mitral-aortic in 3 and mitral-tricuspid in 1). Twenty-one mechanical (11 aortic and 10 mitral) and 12 biological prostheses (8 mitral, 2 aortic, and 2 tricuspid) were implanted. There was 1 hospital death (3.4%). Follow-up data were available for all survivors from 3 to 184 months. There were 8 late deaths (5.9% patient-year), 4 among children with mechanical and 4 among those with biological prostheses. Overall actuarial survival at 14 years is 62.5 +/- 10.2% with no significant difference in survival between AVR (77.8 +/- 14%) and MVR (44.4 +/- 16%) (p = 0.31) at 10 years and between recipients of mechanical (65.7 +/- 13%) or biological prostheses (60.0 +/- 15%) (p = 0.62) at 6 years. No documented embolic or anticoagulant-related complications occurred in the entire series.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗
PubMed1988

Electrovectorcardiographic study of negative T waves on precordial leads in arrhythmogenic right ventricular dysplasia: relationship with right ventricular volumes.

In 24 cases of arrhythmogenic right ventricular (RV) dysplasia, the electrovectorcardiographic (ECG-VCG) behavior of T horizontal (wave and loop) was analyzed and the data compared with RV angiographic volumes. Arrhythmogenic RV dysplasia was diagnosed on the basis of echocardiographic and angiographic data in all subjects. At ECG, T wave was negative in V1 in nine subjects (37%), in V1-V2 in six (25%), in V1-V3 in two (8%), in V1-V4 in one (4%), in V1-V5 in two (8%), and in V1-V6 in four (16%). Nine subjects (37%) presented a bifid T wave in V2-V4. At VCG, T horizontal loop showed three morphologic characteristics: (1) counterclockwise rotation with a mean axis range of +15 degrees to -10 degrees (average, +5 degrees); (2) a figure-eight pattern with a mean axis range of +10 degrees to -40 degrees (average, -17 degrees); and (3) clockwise rotation with a mean axis range of -40 degrees to -110 degrees (average, -70 degrees). T wave changes seem to be primary and independent from QRS changes. RV and diastolic volumes ranged from 100 to 320 m1/m2 (average, 169 +/- 69). The extension of T wave negativity on precordial leads has a direct relationship with RV enlargement (r = 0.89, p less than 0.01). T changes are probably caused by dislocation of the left ventricle backwards secondary to RV dilatation, asynchronous RV repolarization, or intraparietal RV conduction defects.

Adolescent↗
PubMed1988

Left ventricular size and function in double inlet left ventricle.

Using quantitative cineangiography we studied left ventricular size and function in 15 patients (aged 1 day to 15 years) with double inlet left ventricle. Our purpose was to assess how ventricular volume and performance change with age and palliative surgery in patients with and without restriction to pulmonary flow. Ventricular volumes were calculated using Simpson's rule method; end-diastolic volume was also stated as a percentage of predicted total (combined right and left) ventricular volume. Ejection fraction and systolic pressure to end-systolic volume ratio were calculated as indexes of ventricular function. Our results suggest that ventricular size is inadequate for a septation procedure in patients with restriction to pulmonary flow but increases after shunting operation. In patients without restriction to pulmonary flow, ventricular volume is adequate for a septation procedure during the first months of life, but it tends towards reduction, along with obstruction of the interventricular communication, after pulmonary artery banding. Ejection fraction is slightly lower than in left ventricle of heart with biventricular atrioventricular connexion, but it does not decrease with age, at least during the first 15 years of life. Systolic pressure to end-systolic volume ratio also does not decrease with age, but it is lower in patients with excessive pulmonary flow or atrioventricular valvar regurgitation.

Adolescent↗
PubMed1988

Juvenile sudden death and effort ventricular tachycardias in a family with right ventricular cardiomyopathy.

A family with occurrence of juvenile sudden death and effort polymorphous ventricular tachycardias is reported. Nineteen members aged 9 to 63 years were investigated. Four of them died suddenly in their youth. Postmortem investigation performed in 2 deceased subjects disclosed an apparently normal heart at macroscopy but fibro-fatty substitution of the right ventricular free wall was noted at histologic examination. The 14 living members underwent physical examination, resting electrocardiography, chest X-radiography, Holter monitoring, exercise stress testing, and M-mode and cross-sectional echocardiography. Four patients underwent hemodynamic and electrophysiologic studies. All 14 subjects had normal physical examination as well as normal electrocardiographic and cardiothoracic indices. Localized right ventricular structural and dynamic abnormalities were noted at cross-sectional echocardiographic and angiographic investigation of 9 of the patients. The right ventricular volumes in these subjects were normal or slightly increased. In 7 of them, polymorphous ventricular tachycardias were induced by exercise stress testing. The arrhythmias which were responsive to beta-blockade, do not seem to depend on reentry. Enhanced automaticity appeared to be the more likely mechanism of their production. These data demonstrate that right ventricular cardiomyopathy may occur in an occult form with life-threatening electrical instability.

Adolescent↗