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Biomedical subjects

A Nava

Publications and source records attributed to A Nava.

At least 109 records · Page 6Linked to original sources

Right ventricular dysplasia: a familial cardiomyopathy?

268 preselected subjects were extensively studied and the diagnosis of right ventricular dysplasia (RVD) was made in 108 living and 18 deceased patients, 35% of cases being familial. Subsequently we studied 72 subjects from nine families in which a case of sudden death had occurred with the autoptic diagnosis of RVD. In 42 out of 72 cases the autoptic (11 patients), clinical-echocardiographic (30 patients) and haemodynamic (15 patients) data supported the diagnosis of RVD. In all but one deceased patient, death was sudden, while in all the living family members we observed ventricular arrhythmias, mostly with left bundle branch block morphology. Both manifest and concealed forms were documented with polymorphic presentation and with clinical-pathologic findings similar to the non-familial RVD cases. This study confirms the presence of a familial form of RVD that is probably more frequent than previously thought. Preliminary data seem to indicate an autosomal dominant inheritance with incomplete penetrance and variable expression.

Arrhythmias, Cardiac↗

Relevance of subtle echocardiographic findings in the early diagnosis of the concealed form of right ventricular dysplasia.

Right ventricular dysplasia (RVD) is an important cause of arrhythmic sudden death in young people. Echocardiographic criteria suggestive of RVD were: dilation of RV, localized bulge and dyskinesia of the inferobasal wall, structural changes of the moderator band, isolated enlargement of RV outflow tract, apical dyskinesia and trabecular disarrangement. Among 136 subjects 'at risk of high incidence of RVD', 40 had a suggestive echocardiogram. 90% of these showed lipomatous transformation at biopsy and/or developed serious ventricular arrhythmias during a mean follow-up of 42 months. Thus, echocardiography is a potent method in detecting myocardial pathology due to RVD.

Adolescent↗

Toxicological profile of FCE 22101 and its orally available ester FCE 22891.

LD50 values of FCE 22101 iv were 3872 mg/kg and 4392 mg/kg in male and female mice, and 2000 mg/kg and 2201 mg/kg in male and female rats respectively. Oral LD50s of FCE 22891 were 4363 mg/kg in male and 6167 mg/kg in female mice; in the rat this value was over 5000 mg/kg in both males and females. FCE 22101, given iv for two consecutive days was less nephrotoxic in rabbits than cephaloridine and imipenem alone, but more nephrotoxic than imipenem/cilastatin. Dose ranging studies carried out in rats and 13-week studies in rats and monkeys indicated that the kidney was a target organ for both penem compounds. Renal lesions appeared beginning with doses higher than 300 mg/kg/day and were morphologically similar to those induced by cephaloridine and imipenem. Possible targets at high doses were the urinary bladder in rats and the haemopoietic system in monkeys given FCE 22101. The toxicity data available for iv FCE 22101 and oral FCE 22891 in the rat and monkey indicated an adequate tolerance of these compounds, comparable with other beta-lactam antibiotics, including imipenem/cilastatin.

Animals↗

Contribution of cross-sectional echocardiography to the diagnosis of right ventricular dysplasia at the asymptomatic stage.

The value of cross-sectional echocardiography in the early diagnosis of right ventricular (RV) dysplasia in asymptomatic patients has been assessed. Criteria that defined RV dysfunction and structural abnormalities were developed from 38 normal subjects. Of 136 patients admitted into the study program, 40 (29.4%) had an echocardiogram suggestive of RV dysplasia using the following criteria: mild dilatation of the right ventricle (normal range defined as the 95% confidence limit of the values in the control group); localized bulge and dyskinesia of the infero-basal wall; structural changes of the moderator band; isolated enlargement of RV outflow tract; apical dyskinesia and trabecular disarrangement. Holter monitoring and a maximal exercise stress test at entry into the study and during the follow-up (mean 42, range 18-82 months) demonstrated serious ventricular arrhythmias, and RV endomyocardial biopsy confirmed the diagnosis of RV dysplasia in most of these patients, characterized by an electrical instability of RV myocardium (82.5%). During the follow-up, a marked enlargement of RV cavity appeared in three other patients, and RV endomyocardial biopsy demonstrated the typical findings in all patients.

Adolescent↗

Asystole with syncope secondary to hyperventilation in three young athletes.

We describe three athletes who had syncope after (case 1) or during (cases 2, 3) hyperventilation. During the episode, ECG showed prolonged sinus arrest. Clinical data and noninvasive investigations were normal and the phenomenon was not reproducible. Electrophysiological study after autonomic blockade allowed a prolonged intrinsic heart rate in case 1, and abnormal corrected sinus node recovery time in cases 1 and 2. During follow-up, symptomatic sinus arrest provoked by deep inspiration occurred in case 3. These cases document prolonged asystole of unknown etiology, secondary to hyperventilation, and probably caused by different vagally-mediated mechanisms.

Adolescent↗

[Incidence of conduction disorders in patients who underwent surgery for hypertrophic obstructive cardiomyopathy].

Thirteen non-consecutive patients, aging 7 to 61 (average 27) years, underwent left ventricular myotomy-myectomy for a severely symptomatic idiopathic hypertrophic subaortic stenosis (IHSS). In all patients the resting ECG before surgery showed P-R less than 0.18 sec, QRS duration less than 0.11 sec, QRS axis ranging from +10 to +80 degrees. In the immediate post-surgical period 3 patients has complete heart block and 1 had 2nd degree type 2 atrio ventricular block. Lesion was infra-Hisian in 3 patients and intra-Hisian in 1 patient. In the remaining 9 patients an immediate post-surgical left bundle branch block appeared; in 3 out of these patients ECG and an electrophysiologic study documented severe infra-Hisian conduction impairments after an average period of 4 years from surgery. During follow-up 3 patients died suddenly.

Adolescent↗

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↗

Complex arrhythmias in a patient with predominantly right ventricular cardiomyopathy.

A 23-year-old man presented with ventricular tachycardia. The electrocardiogram revealed right bundle branch block plus right axis deviation. It also showed frequent episodes of asystole, 2: 1 sinuatrial block and couplets of left bundle branch block morphology. Electrophysiologic study demonstrated sinuatrial entrance block, with an HV interval of 65 msec. It was also possible to induce sustained ventricular tachycardia of left bundle branch block pattern with normal QRS axis. Subsequent investigations were consistent with the diagnosis of right ventricular cardiomyopathy with mild left ventricular involvement.

Adult↗

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↗

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↗

Two simultaneous right ventricular tachycardias in a case of arrhythmogenic right ventricular dysplasia.

A 51 year old woman with arrhythmogenic right ventricular dysplasia had two types of ventricular tachycardia--(a) a regular and sustained tachycardia and with normal frontal plane axis on electrocardiography and (b) an irregular non-sustained tachycardia with a leftward frontal plane axis. Changes in the QRS complex were sometimes seen during the sustained ventricular tachycardia. The clinical, electrocardiographic, and electrophysiological data were consistent with the diagnosis of two different and sometimes simultaneous tachycardias originating in the right ventricle. This case suggests a possible new mechanism for the multiform appearances of the ventricular tachycardia.

Cardiomyopathies↗

Ceftriaxone in the treatment of bacterial meningitis in adults.

Twenty-six adults with acute bacterial meningitis were enrolled in an open randomized comparative study. The organisms isolated from CSF were Streptococcus pneumoniae, Staphylococcus epidermidis, Haemophilus influenzae, Escherichia coli and Salmonella typhi. 13 patients (group A) were treated once daily with intravenous ceftriaxone (Rocephin). The 13 patients in group B received ampicillin or ampicillin plus chloramphenicol in 4 doses/day. The mean duration of therapy in groups A and B was 9.9 and 12.3 days, respectively. This difference in the duration of therapy was statistically significant. All patients from group A showed clinical improvement and all were bacteriologically cured. In group B only 12 patients were clinically and bacteriologically cured; 1 patient had to be withdrawn from the therapy because CSF culture remained positive after 48 h of therapy. Ceftriaxone was well tolerated in all patients; ampicillin or ampicillin plus chloramphenicol were associated with diarrhea and skin rash in 6 patients.

Adolescent↗

Bidirectional tachycardia. A sustained form, not related to digitalis intoxication, in an adult without apparent cardiac disease.

In this paper we report the first adult case of an "idiopathic" ventricular bidirectional tachycardia (BT), in a 57 year old woman. The tachycardia, at the time of our observation, was incessant in type and had a slightly irregular frequency of about 140 bpm. BT initiated and terminated abruptly, without any temporal relationship to the preceding RR interval, or the QRS morphology. The interval between the two alternating QRS patterns often varied over a wide range of values. The BT could be interrupted only by overdrive atrial and ventricular stimulation, but promptly reappeared as pacing was discontinued. Therapy with quinidine associated with propranolol was effective on a long term trial. The vectorcardiographic analysis and the electrophysiologic investigation demonstrated a ventricular origin of the BT, localizing its site of origin to common myocardial tissue, probably near the two left hemifascicles. Our data could not elucidate the electrogenetic mechanism of this ventricular arrhythmia, because of its chaotic behavior.

Digitalis↗

[Termination of idiopathic ventricular tachycardia with QRS morphology of right bundle branch block and anterior fascicular hemiblock (fascicular tachycardia) by vagal maneuvers. Presentation of 4 cases].

We present 4 patients aged 51, 19, 22 and 16 years respectively, with no overt heart disease. They complained of recurrent episodes of paroxysmal sustained tachycardia with QRS morphology of right bundle branch block and left fascicular hemiblock. The analysis of the electrocardiogram during the tachycardia and, in two cases, the electrophysiologic study showed a complete a-v dissociation and capture beats confirming the ventricular origin of the arrhythmia. In all the patients the interruption of the tachycardia was obtained by the vagal maneuvers; in two of them the tachycardia was also sensitive to verapamil iv. These cases demonstrate the efficacy of the vagal maneuvers in the termination of fascicular tachycardia and support the hypothesis of slow-response nodal-like fibers, distally displaced, as the anatomical substrate of this arrhythmia.

Adolescent↗

[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↗