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Biomedical subjects

G Thiene

Publications and source records attributed to G Thiene.

At least 199 records · Page 11Linked to original sources

Degenerative, non-atherosclerotic cardiovascular disease in the elderly: a clinico-pathological survey.

Cardiovascular disease, the major cause of death in the elderly, is mostly ascribable to complications of coronary atherosclerosis: angina pectoris, myocardial infarction, and sudden death. However, other degenerative diseases involving several cardiac structures exist, and should be distinguished from age-related cardiac changes. Extensive dystrophic calcification determines aortic stenosis, and may affect either a normally tricuspid or a congenitally bicuspid valve. Surgical valve replacement is now a low risk option, even in elderly persons, whereas the efficacy of balloon valvuloplasty is questionable. Aortic incompetence in adults and aged persons is mostly the consequence of aortic tunica media atrophy with anular ectasia, in the setting of nearly normal aortic leaflets. Mitral valve prolapse is the main cause of mitral incompetence; spontaneous cordal rupture is a late complication in the natural history of this disease, thus warranting prompt surgical valve repair or replacement. The entire spectrum of cardiomyopathies is observed in the elderly: dilated, hypertrophic, restrictive, arrhythmogenic. Cardiac amyloidosis is by far the most frequent secondary form and leads to congestive heart failure by impairing ventricular compliance. Idiopathic fibrosis of the specialized AV junction or dystrophic calcification of central fibrous body are the usual substrates of AV block, which requires pace-maker implantation. Nonrheumatic atrial fibrillation, due to fibro-fatty degeneration of the atrial musculature or dilated left atrium, carries a high risk of thromboembolic complications and cerebral accidents; oral anticoagulants have proven to be effective in preventing stroke. Aortic dissecting aneurysm is a spontaneous laceration, and usually a complication of longstanding systemic hypertension; exceptionally, spontaneous dissection may primarily occur in the coronary arteries. In conclusion, longevity at present is mostly threatened by cardiovascular disease, among which the role of degenerative, non-atherosclerotic disorders may be greater than thought.

Aged↗

Sudden death in young competitive athletes: clinicopathologic correlations in 22 cases.

PURPOSE: To investigate the pathologic substrates of sudden death in young competitive athletes. PATIENTS AND METHODS: Twenty-two cases of sudden death in young competitive athletes occurring in the Veneto region (northern Italy) in the period January 1979 to December 1989 were studied by postmortem examination. The athletes included 19 males and three females, ranging in age from 11 to 35 years (mean, 23 years). RESULTS: In 18 cases, sudden death occurred during (16 cases) or immediately after (two cases) a competitive sport activity. In 10 subjects, sudden death was apparently the first sign of disease. Postmortem examination disclosed that this fatality was due to arrhythmic cardiac arrest in 17 cases; among these, right ventricular cardiomyopathy, also known as "right ventricular dysplasia," was the most frequently encountered cardiovascular disease (six cases), followed by atherosclerotic coronary artery disease (four cases), conduction system pathology (three cases), anomalous origin of right coronary artery from the wrong aortic sinus (two cases), and mitral valve prolapse (two cases). In two athletes, the abrupt lethal complication was "mechanical" and consisted of pulmonary embolism and rupture of the aorta; in three athletes, death was due to a cerebral cause. All athletes with right ventricular cardiomyopathy died during effort, and most had a history of palpitations and/or syncope. Whenever available, electrocardiographic (ECG) tracings showed inverted T waves in precordial leads and/or left bundle branch block ventricular arrhythmias. CONCLUSIONS: Clinicopathologic correlations indicate that in the Veneto region of Italy, right ventricular cardiomyopathy is not so rare among the cardiovascular diseases associated with the risk of arrhythmic cardiac arrest, and seems to account for the majority of cases of sudden death in young athletes; this disorder can be suspected during life on the basis of prodromal symptoms and ECG signs.

Adolescent↗

Surgical excision of intracardiac myxomas: a 20-year follow-up.

Since November 1968, 54 patients have undergone excision of an intracardiac myxoma, which was located in the left atrium in 46 (85%), in the right atrium in 6 (11%), and in the right ventricle in 2 (4%). There were 35 female and 19 male patients with a mean age of 48 +/- 14 years (range, 7 to 68 years). Four patients were asymptomatic; the others were seen mostly with exertional dyspnea, palpitation, signs of systemic illness, and syncopal episodes. Before operation, embolic episodes occurred in 13 patients with a left atrial myxoma. There were two early (3.7%) and two late deaths (3.8). Actuarial survival at 20 years is 91% +/- 4%, and most of the current survivors are asymptomatic at a mean follow-up of 6.5 +/- 5 years (range, 0.2 year to 20 years). Noninvasive reevaluation was performed with echocardiographic studies in 44 patients and 24-hour electrocardiographic monitoring in 34. No instances of tumor recurrence were observed, and there was a low incidence of major supraventricular arrhythmias late postoperatively. We conclude that excision of intracardiac myxomas is curative and long-term survival is excellent. The transseptal approach provides adequate exposure and allows complete removal of the tumor regardless of its location.

Adolescent↗

Influence of prosthetic design on durability of the Liotta porcine valve in the mitral position.

From March 1979 to December 1984, the Liotta low-profile porcine bioprosthesis was employed for mitral valve replacement in 71 patients to avoid potential left ventricle-prosthesis mismatch occasionally observed with the standard, high-profile, Hancock porcine xenograft. Follow-up of 61 operative survivors showed at 10 years an actuarial survival of 67% +/- 7%, freedom from thromboemboli of 96% +/- 2%, freedom from structural deterioration of 63% +/- 11% and freedom from all Liotta bioprosthesis-related complications of 53% +/- 10%. Complications related to excessive protrusion of the stent into the left ventricular cavity were eliminated with the Liotta bioprosthesis; the peculiar stent configuration, however, was responsible for an increased rate of structural deterioration requiring reoperation in 10 patients (2.8% +/- 0.9%/patient-year) at a mean interval of 76 +/- 18 months after mitral valve replacement (range, 45 to 106 months). Common findings in all explants were cusp prolapse, cusp tears, and commissural rupture related to various degrees of tissue calcification, constantly leading to severe prosthetic incompetence. As also shown experimentally, such structural changes have been attributed to increased systolic stresses on the closed cusps, favored by excessive reduction of the stent height. Our experience shows that the Liotta bioprosthesis used for mitral valve replacement does not provide any clear-cut advantage over standard porcine bioprostheses and that its long-term durability appears affected by the unique prosthetic design.

Adult↗

Right atrial myxoma originating from the inferior vena cava.

A patient undergoing successful excision of a right atrial myxoma arising from the inferior vena cava is reported. The rarity of this case prompted a review of the literature in which only 2 other patients with a right atrial myxoma originating from inferior vena cava tissue were found.

Adult↗

The relationship between growth of atherosclerotic plaques, variant angina and sudden death.

Clinico-pathological findings are described in two patients with typical variant angina who died suddenly during an ischemic attack. In both cases, detailed pathologic examination of the coronary arteries disclosed severe focal atherosclerosis of the anterior descending coronary artery. The only distinctive histological finding was new intimal proliferation of smooth muscle cells enmeshed within mucoid substance, superimposed on the old fibrous cap of the plaque. These findings agree with experimental and clinical data which suggest that coronary vasospasm may be related to growth of atherosclerotic plaques. This study provides histological evidence that progression of an atherosclerotic plaque may underlie variant angina and sudden death.

Angina Pectoris, Variant↗

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↗

Isomerism of the left atrial appendage and left lung in conjoined twins.

A pair of thoracophagus twins with conjoined hearts and livers are described with emphasis on the cardiac anatomy. The heart of one twin had isomerism of the left atrial appendages in association with azygos continuation of the inferior caval vein. Aortic atresia and atrioventricular septal defect was present in the heart of the other twin.

Female↗

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↗

Coronary arterial wall and atherosclerosis in youth (1-20 years): a histologic study in a northern Italian population.

Based on the working hypothesis that coronary atherosclerosis begins in childhood, a histologic study was carried out on the subepicardial coronary arterial tree of 100 young persons (1-20 years), who had died from causes unrelated to the cardiovascular system. These subjects were natives of a well-defined geographic area in northern Italy, namely the region of Veneto. Intimal proliferations (musculo-elastic and fibro-elastic layers) were observed in 95.3% of the coronary arterial segments in the age group between one and five years. The more distal the coronary segments examined, the lesser was the intimal thickening. Raised mature fibrous plaques were detected in 23 segments from 15 patients (2 from subjects aged between six and 10 years; 4 between 11 and 15 years, and 9 between 16 and 20 years). Single vessel disease was present in 9, double vessel disease in 4 and triple vessel disease in 2 cases. The site most involved by plaques was the proximal part of the left anterior descending coronary artery. Only one plaque was of sufficient dimensions to be considered stenotic (50% luminal reduction). Plaques were rarely sudanophilic, and all seemed to arise in relation to previous intimal thickening. No qualitative nor quantitative sexual differences were observed. These data give rise to much concern, and one consistent with a recently observed occurrence of sudden coronary death in young people from the same geographic area.

Adolescent↗

Surgical pathology of aortic valve disease. A study based on 602 specimens.

A consecutive series of 602 surgically excised aortic valves was evaluated by means of macroscopic and histological study. Pure aortic stenosis was diagnosed in 140 patients, pure incompetence in 254 and combined dysfunction in 208. Of the cases with pure aortic stenosis, 38% were rheumatic, 34% were calcified bicuspid valves and 23% showed dystrophic calcification. Half the patients with pure aortic regurgitation showed aortic root dilatation. Most cases of combined aortic stenosis and regurgitation were the sequelae of rheumatic fever. A male prevalence was detectable in each group (mean male: female ratio = 2.6), and was highest in infective endocarditis and aortic root dilatation. Infective endocarditis was a frequent complication of congenitally bicuspid valves. In conclusion, rheumatic disease is still a frequent cause for surgical replacement of the aortic valve. At least half the explanted aortic valves have degenerative or congenital diseases which are often the site of a superimposed infective endocarditis.

Adolescent↗

The pathology of Hancock standard porcine valve prosthesis: a 20-year span of experience.

A spectrum of events leading to tissue failure is responsible for late dysfunction of Hancock porcine valve xenografts: (a) Primary failure: dystrophic calcification, thrombosis, fibrous tissue overgrowth, primary tears, cuspal hematomas, and stent postbending. (b) Secondary failure: endocarditis and paravalvular leak. Dystrophic calcification is the main factor influencing long-term durability and accounts in our experience for 88% of primary failure, through different clinical presentations; particularly, incompetence by cusp tearing and egg-shell fragmentation is by far the most frequent mode of failure. Cusp degeneration by primary tears (in the absence of dystrophic calcification) is an uncommon event, due to lipid infiltration or to right coronary muscle shelf spontaneous or immuno-related disruption.

Bioprosthesis↗

Morphologic features of the hypoplastic left heart syndrome--a reappraisal.

The report describes the morphologic features of 102 specimens with hypoplasia of the chambers of the left heart, all with either a concordant atrioventricular connection (66 cases) or absence of the left atrioventricular connection, the right atrium being connected to a dominant right ventricle (36 cases). Either the aorta was connected to the left ventricle, having a patent valve in 23 cases and an imperforate valve in 5, or else the aortic trunk was atretic (74 cases). It was not possible to determine with certainty the ventricular origin of the aorta when its trunk was atretic, but in all hearts but 1 it seemed to be connected to the left ventricle. The atrial septum was intact in 11 hearts and grossly restrictive in a further 8. Deviation of the atrial septum relative to the infolded atrial roof (septum secundum) was seen in 32 specimens. The ventricular septum was intact in 88 hearts. The length of the left ventricle compared to the right was greatest in the 14 hearts with a ventricular septal defect. The left ventricular length was also significantly greater in hearts with a patent aortic valve. The state of the atrial septum, in contrast, bore no relationship to the dimensions of the left ventricle. Aortic coarctation was found in 44 of the hearts, and in all but 2 the shelf lesion was preductal in location. Coarctation was significantly more frequent in those hearts in which the ascending aorta was less than 3 mm in diameter. The anatomic findings are discussed in relation to recent advances in the surgical palliation of these malformations.

Aortic Coarctation↗

[Spontaneous dissecting coronary hematoma (aneurysm) and sudden death].

Two women, 34 and 54 years old, died suddenly from acute coronary occlusion due to spontaneous dissecting haematoma (aneurysm) involving the entire left coronary artery and the right coronary artery, respectively. In the older woman, cystic medial necrosis of the coronary tunica media was observed. The younger case was the only sudden death due to dissecting hematoma among 120 cases in the Registry of "juvenile sudden death" in the Veneto Region, Italy. From a review of the literature, following the first description in 1931, we collected 97 cases with the following characteristics: mean age in women, 39 years, in men, 48 years; only significant risk factor: peripartum (34% of women); coronary arteries involved: isolated left anterior descending (52.5%, equally distributed among women and men), right (24%, mainly men), left (13.5%, only women), multiple vessels (8%), left circumflex artery (2%). The clinical presentation was cardiac arrest with sudden death in 49.5%, acute, non-fatal myocardial infarct in 33%, and acute fatal myocardial infarct in 17.5% of the cases. The prognosis is very poor, and emergency medical or surgical myocardial revascularization is rarely feasible.

Adult↗

[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↗