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Biomedical subjects

G Thiene

Publications and source records attributed to G Thiene.

At least 235 records · Page 13Linked to original sources

[Coronary atherosclerosis and sudden death in a young athlete].

A case of juvenile sudden death, occurring during athletic activity and due to obstructive atherosclerosis of coronary arteries, is reported. Repeated maximal exercise stress tests, Holter monitoring (24 hours) and echocardiograms, performed because of recurrent episodes of palpitation during effort, gave negative results. Uncertainties regarding the diagnostic sensitivity of the protocols for competitive sport fitness in revealing latent myocardial ischemia are stressed.

Adult↗

Right ventricular cardiomyopathy and sudden death in young people.

From 1979 to 1986, we conducted postmortem studies of 60 persons under 35 years of age who had died suddenly in the Veneto Region of northeastern Italy. Unexpectedly, we found that 12 subjects--7 males and 5 females ranging in age from 13 to 30 years--had morphologic features of right ventricular cardiomyopathy. This disorder had not been diagnosed or suspected before the subjects died. In five cases, sudden death was the first sign of disease; the remaining seven subjects had a history of palpitation, syncopal episodes, or both, and in five of those seven, ventricular arrhythmias had previously been recorded on electrocardiographic examination. Ten of the subjects had died during exertion. At autopsy, the subjects' heart weights were normal or moderately increased. Two main histologic patterns were identified--a lipomatous transformation or a fibrolipomatous transformation of the right ventricular free wall (6 cases each); in all cases, the left ventricle was substantially spared. Signs of myocardial degeneration and necrosis, with or without inflammatory infiltrates, were occasionally observed. These findings indicate that right ventricular cardiomyopathy, the cause of which is still unknown, may be more frequent than previously thought. At least in this area of Italy, it may represent an important cause of sudden death among young people.

Adolescent↗

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↗

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↗

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↗

The Hancock pericardial xenograft: incidence of early mechanical failures at a medium-term follow-up.

The Hancock pericardial xenograft has been used in our Institution since August 1981 as an alternative to porcine bioprostheses. Up to July 1984, 97 Hancock pericardial xenografts have been implanted in 84 patients; of 76 operative survivors with a mean age of 55.2 +/- 13 years (range 13-75 years), 50 had undergone aortic valve replacement, 16 mitral valve replacement and 10 mitral-aortic valve replacement. Follow-up ranged from 0.5 to 5.2 years with a cumulative duration of 239 patient/years and is 99% complete. Actuarial survival is 92% +/- 4% for patients with aortic valve replacement and 84% +/- 10% for patients with mitral valve replacement at 5 years, and 77% +/- 14% for those with mitral-aortic valve replacement at 4 years. Thromboembolic episodes occurred in 2 patients (1 after aortic and 1 after mitral valve replacement). The actuarial freedom from emboli is 100% for patients with mitral-aortic valve replacement at 4 years, and 96% +/- 3% for patients with aortic and 93% +/- 6% for patients with mitral valve replacement at 5 years. Reoperation was performed in 13 patients (9 aortic, 2 mitral and 2 mitral-aortic valve replacements), because of endocarditis in 3 (2 aortic and 1 mitral valve replacement), paravalvular leak in 1 (aortic valve replacement), and primary tissue failure in 9 (6 aortic, 1 mitral and 2 mitral-aortic valve replacements). Actuarial freedom from primary tissue failure is 72% +/- 9% for aortic and 83% +/- 8% for mitral Hancock pericardial xenografts at 5 years. Eleven xenografts explanted because of primary tissue failure were studied pathologically.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Performance of the Hancock porcine bioprosthesis following aortic valve replacement: considerations based on a 15-year experience.

All patients undergoing isolated aortic valve replacement with a standard Hancock porcine bioprosthesis (PB), from 1970 to 1983, were reviewed. There were 196 patients, 162 male and 34 female patients, with a mean age of 48 +/- 12 years. Operative survivors were followed up from 3 to 15.6 years (mean follow-up, 6.6 +/- 1.5 years), with a cumulative follow-up of 1,140 patient-years, being 100% complete. Actuarial survival was 51 +/- 15% at 14 years. Eight patients sustained systemic embolic episodes (0.7 +/- 0.2%/patient-year); actuarial freedom from emboli is 89.4 +/- 4.3% at 14 years. Reoperation was performed in 53 patients: in 6 because of endocarditis (0.5 +/- 0.2%/patient-year), in 7 because of perivalvular leak (0.6 +/- 0.2%/patient-year), and in 40 because of PB primary tissue failure (3.5 +/- 0.5%/patient-year). Actuarial freedom from PB-related deaths, PB failure, and overall PB-related complications at 14 years was 66.3 +/- 19, 34.3 +/- 11, and 30 +/- 10%, respectively. This long-term experience shows that the performance of the Hancock PB appears satisfactory up to 8 years, while it progressively deteriorates beyond 10 years because of the impact of primary tissue failure on valve durability, justifying the restriction of its use in the aortic position in selected patients.

Actuarial Analysis↗

Sudden death as the first manifestation of coronary artery disease in young people (less than or equal to 35 years).

During 1979 to 1987, we collected 84 consecutive cases of juvenile sudden death which occurred in the Veneto Region, northeast Italy. Death was attributed to cardiovascular disease in 79 cases. Nineteen of these (24%), consisting of 17 males and two females, from 18 to 35 years of age, had 70% or greater atherosclerotic coronary stenosis, in the absence of other cardiac pathology and previous clinical evidence of angina pectoris or myocardial infarction. In 13 cases (68%), sudden death was the first manifestation of coronary artery disease; the remaining six patients had experienced atypical, non-diagnostic prodromal symptoms. At the moment of death, 16 patients were engaged in sedentary activity. Pathological examination disclosed that in 15 cases (79%), only one major vessel was stenosed, and in 12 cases it was the proximal descending coronary artery: the other four patients had three-vessel disease. Histologic study revealed uncomplicated, obstructive fibromatous plaques in 16 cases, and a preserved tunica media in all cases. Plaque fissuring with superimposed mural or occlusive thrombosis was present in only three cases. Overt myocardial infarction was not observed. Our findings indicate that coronary atherosclerosis is an important cause of sudden death in young persons. In this series, coronary disease was 'silent', and sudden death was its first clinical manifestation. The occurrence of death at rest, in the absence of an acute coronary lesion, with preservation of the coronary tunica media suggests that fatal outcome might be due to coronary vasomotor tone abnormalities culminating in ischaemia-induced cardiac arrest.

Adolescent↗

The standard Hancock porcine bioprosthesis: overall experience at the University of Padova.

All patients undergoing aortic (AVR, n = 196), mitral (MVR, n = 502), and mitral-aortic (MAVR, n = 71) valve replacement with a standard Hancock porcine bioprosthesis (HPB) from 1970 to 1983 were reviewed. A total of 665 patients discharged were followed for 5,099 patient-years with an actuarial survival at 15 years of 52% +/- 4.5%, for MVR, 37% +/- 14% for AVR, and at 12 years of 52 +/- 7.4% for MAVR. Embolic episodes occurred in 9 patients after AVR (0.7% +/- 0.2% pt-yr), in 61 after MVR (1.7% +/- 0.2% pt-yr), and in 6 after MAVR (1.7% +/- 0.7% pt-yr); actuarial freedom from emboli at 15 years is 91% +/- 3.5% after AVR, 79% +/- 14% after MVR, and at 12 years is 87% +/- 5% after MAVR. Reoperation because of primary tissue failure (PTF) was performed in 47 patients with AVR (3.9% +/- 0.5% pt-yr), 91 with MVR (2.6% +/- 0.3% pt-yr), and in 13 with MAVR (4.1% +/- 1.1% pt-yr); actuarial freedom from PTF at 15 years is 41% +/- 5.5% after MVR, 37% +/- 10% after AVR, and at 12 years is 49% +/- 13% after MAVR. After AVR and MVR, freedom from PTF is significantly better for patients over 50 years of age.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

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↗

Hancock bioprosthetic valve failure: causes, and results of reoperation.

Between March 1970 and the end of December 1986, 180 porcine valve recipients underwent reoperation at our institution because of bioprosthetic failure. Of these patients, 126 (70.0%) had mitral bioprosthesis replacement (MBR), which was necessitated because of primary tissue failure in 107 cases, prosthetic valve endocarditis in eight cases, and paravalvular leakage in eleven cases. Forty-six patients (25.5%) had aortic bioprosthesis replacement (ABR), owing to primary tissue failure in 37 cases, endocarditis in six cases, and paravalvular leakage in three cases. The remaining eight patients (4.5%) all underwent double (mitral and aortic) bioprosthesis replacement (DBR) because of primary tissue failure. In a total of 152 patients who underwent reoperation for primary tissue failure, the hospital mortality was 6.5% for the MBR group, 8.0% for the ABR group, and 0% for the DBR group (mean, 6.5%). In those 14 patients who required reoperation owing to endocarditis, the hospital mortality was 75% for the MBR group and 50% for the ABR group (mean, 64%). In another 14 patients who underwent reoperation because of paravalvular leakage, the hospital mortality was 9% for the MBR group and 0% for the ABR group (mean, 7%). Morphologic studies of the explanted valves revealed that tissue calcification is the most frequent cause of primary tissue failure in Hancock-valve recipients. Calcification leads to cusp stiffening with stenosis or to cuspal and commissural rupture with incompetence. Usually, primary tissue failure causes progressive dysfunction that allows for elective reoperation, which is associated with a low mortality; rarely, however, porcine xenograft failure can occur acutely and require emergency operation, which is associated with a significantly higher risk. Such operation was necessary in nine (6%) of our patients with primary tissue failure and resulted in four deaths (44%); conversely, elective reoperation was done in 143 patients with other primary tissue failure (94%) and resulted in six deaths (4%) (p<0.001). Our long-term experience with the Hancock bioprosthesis confirms that this valve's durability is limited. Therefore, we believe that the indications for the use of porcine bioprostheses should be restricted, until consistent data become available concerning the durability of the new generation of prosthetic valves.

Journal Article↗

[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↗

Primary pulmonary hypertension: an Italian multicenter study. A retrospective epidemiological survey in the period 1975-1985.

An epidemiological retrospective survey on primary pulmonary hypertension was undertaken in Italy in 1985-6. The aim of the study was to obtain clinical and laboratory data on patients observed in the major cardiological Institutions, both Universities and Hospital Clinical Centers, in the period 1975-85. Forms were sent to 67 centers and twenty-three of them communicated their data on 124 cases of primary pulmonary hypertension. The diagnosis was considered certain or probable in 91 patients by the referring center. The mean age of patients was 38.5 years, with an overall female-to-male ratio of 2:1. Data were subjected to statistical analysis which confirmed the poor prognosis of the disease particularly for patients with high pulmonary resistances, low cardiac output and in class 3-4 of the NYHA. Half of the 124 cases were treated with calcium antagonists after 1980, but the results of therapy in modifying the clinical course of the disease could not be assessed by the study. Like all retrospective studies even the Italian Multicenter Study is biased but it can be considered as an useful preliminary investigation which can form the basis for a prospective registry of primary pulmonary hypertension cases.

Age Factors↗

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↗

Pulmonary vascular disease in infants with complete atrioventricular septal defect.

In order to assess incidence and time of occurrence of pulmonary vascular disease, the lungs of 49 patients were studied histologically. Thirty-four lung specimens were obtained at autopsy, and 15 were open lung biopsies. Patients consisted of 24 males and 25 females ranging in age from 10 days to 14 years (median 9 months); 37 had Down's syndrome. Overall incidence of irreversible pulmonary vascular disease (grade 3 or more) under 1 year of age was 34.6%; grade 4 of pulmonary vascular disease was observed only in Down patients. In correlating pulmonary vascular resistance, measured during heart catheterization, with pulmonary vascular disease severity at histology, 6 out of 7 patients with pulmonary vascular resistance over 7 units per metre squared showed grade 4 of pulmonary vascular disease, and of these 4 were under 1 year of age. These findings suggest that a significant rate of pulmonary vascular disease occurs under 1 year of age, with most severe degrees in the time interval 7-12 months. They show that good correlation exists between pulmonary vascular resistance over 7 units per metre squared and grade 4 of pulmonary vascular disease. They demonstrate that the most severe pulmonary vascular disease is seen in Down's syndrome. Finally, they indicate that early surgical correction is mandatory and should be accomplished within 6 months of age.

Adolescent↗