Biomedical subjects
Publications and source records attributed to A Nava.
We describe a 47-year-old man affected with hypertrophic cardiomyopathy and frequent episodes of syncope. During ambulatory Holter monitoring and head-up tilt test, a syncopal attack was associated with sinus arrest and hypotension. This case suggests an additional mechanism of syncope in hypertrophic cardiomyopathy.
The standard enzyme linked immunosorbent assay (ELISA) currently in use for detection of anticardiolipin antibodies (ACA) was used to evaluate the influence of heat inactivation and sheep erythrocyte adsorption on individual optical density (OD) of sera from healthy blood donors or patients with primary antiphospholipid syndrome. Each sample was tested after single or combined maneuvers as follows: adsorbed, adsorbed and inactivated, only inactivated, and compared to basal readings. A significant increase of ACA titers did occur after inactivation of normal sera, but adsorption had no effect. In contrast, neither inactivation nor adsorption changed ACA titer in primary antiphospholipid syndrome sera as a group, although in certain sera there were changes. This observation may suggest the presence in normal serum of a thermolabile factor which modulates ACA binding to its antigen and the reactivity of the anticardiolipin antibodies of the primary antiphospholipid syndrome with sheep erythrocyte membrane phospholipids.
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.
The purpose of this study was to define the history and prognosis of 12 patients (8 males, 4 females) with syncope of unknown origin (5 to 15 episodes), who developed prolonged asystole or complete AV block during the upright tilt test (UTT). The mean age (+/- SD) of the patients was 29 +/- 7.4 years, and all had normal neurological and cardiological findings on evaluation. These patients were selected from a larger group of 92 cases with positive UTT out of a total of 136 subjects who were referred for recurrence of syncope. Neither clinical nor autonomic nervous system evaluation distinguished these 12 patients from those with positive UTT. Following UTT, therapy was initiated and consisted of transdermal scopolamine in four, disopyramide in two, and beta-blockers in four patients. During follow-up (mean, 17 +/- 5.4 months), four patients had recurrences but none experienced episodes of life-threatening syncope. These patients do not show an enhanced risk of sudden death, and drug therapy seems to improve their clinical course. Only long-term follow-up would correctly identify a subgroup at higher risk.
An asymptomatic 19-year-old top-level athlete had electrocardiographic evidence of intermittent cardiac preexcitation and intermittent left bundle branch block. The electrophysiologic study demonstrated the presence of a direct accessory pathway and enhanced atrioventricular node conduction that resulted in infrahisian and intraventricular conduction disturbances. The echocardiogram disclosed tricuspid valve dysplasia.
The classification of myocardial disease proposed by the WHO/ISFC task force in 1980 distinguishes specific heart muscle diseases from myocardial diseases of unknown origin, termed cardiomyopathies, and differentiated into the dilated, hypertrophic and restrictive forms. This last group includes endomyocardiofibrosis and fibroblastic parietal endocarditis. In more recent years, two new forms of heart muscle disease have been recognized: so-called "primary" restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. Primary restrictive cardiomyopathy is characterized anatomically by normally sized, non-hypertrophic ventricles with dilated atria, and functionally by impaired diastolic compliance due to myocardial stiffness. The clinical picture is that of chronic congestive heart failure; histology shows interstitial fibrosis and myocardial disarray, but not hypereosinophilia. In arrhythmogenic right ventricular cardiomyopathy, the myocardium of the right ventricular free wall is substituted by fibrous and/or adipose tissue, which results in regional dynamic alterations and ominous ventricular arrhythmias. The left ventricle is usually spared. Both forms should be classified as heart muscle diseases of unknown origin, and kept clearly distinct from the other cardiomyopathies listed in the WHO classification.
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In right ventricular cardiomyopathy the relationship between the progression of structural abnormalities and arrhythmia development is not yet well known. This report describes a case in which severe ventricular arrhythmias appeared 3 years after the demonstration of right ventricular (RV) structural and dynamic abnormalities. In this interval of time structural changes were not detectable with the commonly used diagnostic methods, but endocavitary RV late fractionated QRS potentials appeared suggesting the development of an arrhythmic component of the disease.
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.
A P wave of 7.5 mm in lead I and 12.5 in V1 was detected in a 28-year-old man, with a progressive cardiomegaly since the age of 14 years. At last admission he had minor symptoms, and a systolic murmur consistent with tricuspid regurgitation. The electrocardiogram showed an extremely tall P wave and a QRS of a very low amplitude; T waves were inverted on the precordial leads. These ECG features, and subsequent investigations, were consistent with right ventricular cardiomyopathy with massive tricuspid regurgitation, and right atrial abnormality.
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.
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.
The morphology of ventricular extrasystole (VES) in 46 cases of arrhythmogenic dysplasia of the right ventricle (ADRV) was correlated with the point of origin located by intracavitary mapping. The cases concerned 41 of left bundle-branch block (LBB) with various axes on the frontal plane (FP), 4 of right bundle-branch block (RBB), and 5 of atypical morphology (frontal plane shifted inferiorly and increased R from V1 to V6; on the horizontal plane, clockwise rotation of the loop oriented anteriorly and leftward). There is a good correlation with the site of origin: VESs which were LBB in appearance originated in the right ventricle (apex, septum, infundibulum); VESs which were RBB in appearance originated in the apex of the left ventricle, while the atypical VESs started in the upper posterior septum. A study of morphology may therefore also give an indication of the location of the disease.
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.
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The object of the study was to define spreading and quantitative criteria of left ventricular hypertrophy in echocardiography by using a "score"--for this, the left ventricle has been divided into 11 regions and a "score" attributed to each one of them--and to find the correlation with the vectocardiogram (VCG) in 42 patients with hypertrophic myocardiopathy (HM). The results obtained show the following: 1) the left ventricular hypertrophy aspect on the ECG and the VCG is very sensitive for the identification of a diffuse HM; 2) the necrosis, hemiblock or septal hypertrophy indicate a hypertrophy located in the forepart septum or the whole of the septum; 3) the giant T waves indicate a hypertrophy of the apex; 4) a left ventricular hypertrophy associated with a necrosis or a hemiblock indicate a global myocardiopathy, with the basal region of the septum largely affected.
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.