[Circadian rhythm of the heart rate and autonomic nervous system stimulation tests in patients with systemic lupus erythematosus]. / Ritmo circadiano della frequenza cardiaca e prove di stimolazione autonomica in pazienti con lupus eritematoso sistemico.

We studied 19 women (mean age 35 +/- 13 years) with systemic lupus erythematosus (SLE), in order to evaluate whether or not alterations in the circadian rhythm of heart rate (HR) occur in patients with pathologic responses to stimulation tests of the autonomic nervous system (ST-ANS). The duration of SLE was 5.3 +/- 5 years. None of the patients had clinical signs of cardiopathy or dysautonomy, nor were any of them taking drugs with known effects on the heart or ANS. Nine patients (47%, group A) had normal ST-ANS and 10 (53%, group B) had an abnormal response to at least 1 ST-ANS (5 to sympathetic ANS, 3 to parasympathetic and 2 to both ST-ANS). Age, duration of disease and therapy were not different between the 2 groups. All patients underwent 24-hour ambulatory ECG monitoring, and chronobiologic analysis of hourly HR was carried out by single and mean cosinor methods. A significant circadian rhythm was found both in the total sample (mesor 80 b/min, acrophase h 13:12; p less than 0.01), and, separately, in group A (mesor 82 b/min, acrophase h 13:11; p less than 0.01) and group B (mesor 78 b/min, acrophase h 13:12; p less than 0.01). No difference existed between the HR circadian rhythms of the 2 groups. Thus, our data show the possibility of ANS involvement in SLE patients without clinical signs of dysautonomy; the analysis of the HR circadian rhythm does not appear to be a sensitive method to identify early involvement of the ANS in these patients.

Reproducibility in circadian rhythm of ventricular premature complexes.

To evaluate the existence and reproducibility of a circadian rhythm of ventricular premature complexes (VPCs), 38 patients (mean age 57 +/- 17 years) with greater than or equal to VPCs/hour were studied with 24-hour electrocardiogram Holter monitoring. Nineteen patients had coronary artery disease and 19 had structurally normal hearts. A second Holter electrocardiogram was recorded in all patients from 2 to 47 days (mean 11) after the first. Chronobiologic analysis was made by single and mean cosinor methods. A significant and similar circadian rhythm of VPCs was found in the total sample both on the first (mesor 399, acrophase at 15:08, p less than 0.01) and the second day (mesor 306, acrophase at 14:47, p less than 0.05), with 2 main peaks, the first in the late morning and the second in the afternoon. However, only 18 patients (47%, group A) had a significant individual circadian rhythm of VPCs on both days, whereas 20 (53%, group B) did not have a significant rhythm in greater than or equal to 1 day. A high reproducibility of the circadian rhythm of VPCs was found in group A patients, with a difference of 2.1 +/- 1.8 hours between the acrophases of the 2 days, whereas the difference was 4.4 +/- 3.3 hours in group B patients (p less than 0.01). Among group A patients, 14 (78%) had a VPC rhythm with acrophase occurring during waking hours, whereas the acrophase of 4 (22%) occurred during the night. The reproducibility of the circadian rhythm of VPCs was not influenced by gender, presence of coronary disease, medical therapy, basal VPC number, or day-to-day variability of VPCs, although group A patients were older than group B patients (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Accuracy in clinical arrhythmia detection of a real-time Holter system (Oxford Medilog 4500).

The accuracy of a real-time analysis Holter system (Oxford Medilog 4500) in detecting ventricular and supraventricular arrhythmias was evaluated. Hand-counted data from randomly selected hours of 152 ECG monitorings of 152 patients were used as the control standard. Accuracy of the system was evaluated on 606 hours for premature ventricular complexes (PVC), PVC couplets and supraventricular extrasystoles (SVE), and on 1,789 hours for ventricular tachycardia (VT), accelerated idioventricular rhythm (AIVR), and supraventricular tachycardia (SVT). Sensitivity and positive predictive accuracy for the Oxford system were (1) 92.9% and 94.9% for PVC; (2) 90.1% and 87.8% for PVC couplets; (3) 98.1% and 56% for AIVR; (4) 80% and 82.3% for VT; (5) 88.6% and 56.5% for SVE, and (6) 43.7% and 60.2% for SVT. Furthermore, negative predictive accuracy, the ability to predict the total absence of an arrhythmic event in an hour, was determined. It was 91.7% for PVC, 99.5% for PVC couplets, 99.9% for AIVR, 99.7% for VT, 95% for SVE, and 98% for SVT. ventricular arrhythmias, whereas significant inaccuracies appear to exist in the analysis of supraventricular arrhythmias.

[Cardiovascular aspects of Martin-Bell syndrome]. / Aspetti cardiovasculari della sindrome di Martin-Bell.

Cardiovascular anomalies have been studied in 13 subjects (8 males and 5 females, average age 15 +/- 7 years) affected from fragile X syndrome. This group has been examined by standard-ECG, Holter-ECG, echocardiography (M-mode, B-mode, Doppler and color-Doppler). The results have been compared with a control group of 39 subjects (20 males and 19 females, average age 15 +/- 5 years), with non genetic mental retardation. Clinical examination, ECG and Holter did not show any significant pathological alteration compared with the results of the control group. In the study group echocardiography showed the following results: 10 subjects (77%) had mitral valve prolapse of the anterior leaflet (arching); 4 of which (31%) with associated posterior leaflet prolapse; 2 subjects (15%) with posterior aortic leaflet prolapse; 2 subjects (15%) with tricuspid septal leaflet prolapse; 3 subjects (23%) had mild pulmonary artery dilatation; 1 subject (8%) had a mild aortic regurgitation; in 9 subjects (69%), 3 of whom with pulmonary artery dilatation, has been found pulmonary valve regurgitation; 10 subjects (77%) had tricuspid valve regurgitation. In all subjects cardiac dimensions were within the normal range. The most important result, in accordance with literature, is the high prevalence of mitral valve prolapse. The prolapse is asymptomatic and silent. We have never found aortic root dilatation that was described by other Authors. The described anomalies could be ascribed at the dysfunction of the connective tissue. This theory has been confirmed by necropsy studies. Therefore, we suppose that these alterations, particularly the anterior mitral leaflet prolapse, are non casually associated with the fragile X syndrome.

Opposite effects of propafenone and flecainide in a patient with reciprocating supraventricular tachycardia.

A 46 year-old woman with Wolff-Parkinson-White syndrome (postero-septal accessory pathway), symptomatic for recurrent episodes of nonsustained paroxismal supraventricular tachycardia (PSVT), was empirically treated with propafenone (600 mg/day). After a week of therapy the patient returned to the hospital after an episode of syncope. She referred a significant increase in duration and frequency of "palpitations". Under treatment with propafenone a sustained PSVT could be induced during transesophageal testing. During the electrophysiologic study performed off drugs, only a nonsustained PSVT could be induced. After flecainide infusion (1 mg/kg) anterograde block of the accessory pathway was observed and only few beats (less than 8) of PSVT could be induced. The patient was discharged on flecainide (200 mg/day) and 1 month later a transesophageal testing was repeated showing an anterograde block of the accessory pathway at a pacing cycle length of 500 ms; no arrhythmias were induced. The patient has been asymptomatic on chronic oral therapy with flecainide during a follow-up period of 8 months. This case shows that 2 1c class antiarrhythmic drugs may have opposite effects (proarrhythmic and antiarrhythmic). Failure, or even the proarrhythmic effect of one drug, does not necessarily exclude the efficacy of another drug of the same subclass in preventing recurrence of PSVT.

Coenzyme Q10 in dilated cardiomyopathy.

The authors have tried to study the therapeutic efficacy of coenzyme Q10 (CoQ10) in patients with dilated cardiomyopathy (DCM). In fact, CoQ10 has been shown to be deficient in myocardial tissue biopsies taken from DCM hearts, compared to normal hearts. Thirty patients with histological diagnosis of DCM were orally treated with CoQ10 (100 mg/die) for 2 months. Before and after treatment a clinical examination with determination of NYHA class and an echocardiographic examination with determination of ejection fraction (EF) and of telediastolic (TDV) and telesystolic (TSV) volumes were performed, and blood was drawn for plasma CoQ10 determination. In seven patients the pretreatment endomyocardial level of CoQ10 was also assayed. Seven patients left the study because of poor therapeutic compliance. In 47% of patients the clinical symptomatology regressed, with improvement of NYHA class. The EF improved from 0.31 +/- 0.09 to 0.37 +/- 0.11 (p less than 0.001). The TDV passed from 262.2 +/- 85 ml to 203.3 +/- 83 ml (p less than 0.05), and the TSV from 166.13 +/- 75 ml to 126.9 +/- 56 ml (ns). The CoQ10 plasmatic levels improved in 95% of the patients: from 0.74 +/- 0.37 micrograms/ml to 2.27 +/- 0.99 micrograms/ml (p +/- 0.0001). The CoQ10 myocardial levels did not show univocal values, but the patients with lower myocardial levels seemed to have a better therapeutic response. These data suggest that the CoQ10 deficiency in DCM may be reversible and that the therapeutic effects depend on the basal plasmatic and myocardial levels. Therapy with coenzyme Q10 may be considered to be an efficacious aid in the traditional treatment of chronic cardiac failure.

Persisting hypereosinophilia and myocardial activity in the fibrotic stage of endomyocardial disease.

An unusual case of endomyocardial fibrosis is reported complicating an idiopathic hypereosinophilic syndrome. Persisting hypereosinophilia, degranulated eosinophils in the blood, and myocardial activity have been found accompanying the fibrotic phase of endomyocardial disease. This occurrence supports the unitarian theory on tropical and temperate endomyocardial disease and suggests in such a condition the use of steroids or cytotoxic drugs in addition to surgery.

Rhythm of carnitine levels in serum and urine of normal subjects.

A study of the diurnal serum and urine levels of L-carnitine and acetylcarnitine was carried out in eleven healthy volunteers. No significant difference was found between the levels in the morning and in the afternoon, although a higher carnitinaemia was shown in the waking hours when the energy demands were higher.

[Prevalence of pulmonary valve insufficiency in healthy children: a Doppler color study]. / Prevalenza dell'insufficienza polmonare in soggetti sani di età pediatrica: studio con color-doppler.

One hundred patients, institutionalized for mental retardation, aged between 3 and 14 years (mean age 12.2 +/- 3) and free from cardiovascular and pulmonary diseases, were studied using Doppler technique (pulsed wave-continuous wave and color-coded Doppler), to evaluate the prevalence of pulmonary regurgitation. The authors, utilizing a triple method (diastolic turbulence above pulmonary valve detected by pulsed wave Doppler or diastolic flow detected by continuous wave Doppler, presence of regurgitant pulmonary color-jet, from short axis view, toward the right ventricular outflow tract, and presence of the same feature in the color m-multigate) to detect the presence or absence of pulmonary regurgitation found 73% positivity. There were no differences between the two sexes and the size of the pulmonary artery was in the normal range. The characteristics of regurgitation were: No holodiastolic. The regurgitant max velocity jet was not greater than 1.50 m/s. Beat to beat variability. Max length of color-jet was not more than 2 cm. Rapidly decreasing Doppler profile. We can conclude that pulmonary regurgitation is very frequent in children and is not significant if it has the above-named characteristics. This latter fact is further confirmed by other authors.

Circadian variation of ventricular tachycardia in acute myocardial infarction.

Circadian rhythms have been described both for acute myocardial infarction (AMI) and sudden death. In this study the diurnal distribution of ventricular tachycardia (VT) in patients with AMI was analyzed. Ninety-four AMI patients with greater than or equal to 1 VT on Holter electrocardiographic monitoring who were not taking antiarrhythmic drugs were studied. Forty-seven patients had a recent AMI (group A) and 47 an old AMI (group B). Chronobiologic analysis was made by single cosinor method. There were 157 VTs (mean 1.67 VTs/patient, range 1 to 10) in the 94 patients: 70 in group A and 87 in group B. A significant circadian rhythm of VT was found in the total population with acrophase at 2:29 P.M. The hourly distribution of VT showed a tendency to bimodality, which seemed due to a different time of peak VT occurrence in group A (significant rhythm with acrophase at 4:40 P.M.) and group B (significant rhythm with acrophase at 12:39 P.M.). Thus, the hourly VT frequency in patients with AMI has a significant circadian variation with the highest occurrence in the awake hours, similar to the rhythms described for AMI and sudden death.

A comparison of the assessment of mitral valve area by continuous wave Doppler and by cross sectional echocardiography.

Transmitral pressure half time (PHT) was assessed by continuous wave Doppler in 44 patients with rheumatic mitral valve stenosis (14, pure mitral valve stenosis; 15, combined mitral stenosis and regurgitation; and 15 with associated aortic valve regurgitation). The mitral valve area, derived from transmitral pressure half time by the formula 220/pressure half time, was compared with that estimated by cross sectional echocardiography. The transmitral pressure half time correlated well with the mitral valve area estimated by cross sectional echocardiography. The correlation between pressure half time and the cross sectional echocardiographic mitral valve area was also good for patients with pure mitral stenosis and for those with associated mitral or aortic regurgitation. The regression coefficients in the three groups of patients were significantly different. Nevertheless, a transmitral pressure half time of 175 ms correctly identified 20 of 21 patients with cross sectional echocardiographic mitral valve areas less than 1.5 cm2. There were no false positives. The Doppler formula significantly underestimated the mitral valve area determined by cross sectional echocardiography by 28(9)% in 19 patients with an echocardiographic area greater than 2 cm2 and by 14.8 (8)% in 25 patients with area of less than 2 cm2. In thirteen patients with pure mitral valve stenosis Gorlin's formula was used to calculate the mitral valve area. This was overestimated by cross sectional echocardiography by 0.16 (0.19) cm2 and underestimated by Doppler by 0.13 (0.12) cm2. Continuous wave Doppler underestimated the echocardiographic mitral valve area in patients with mild mitral stenosis. The Doppler formula mitral valve area = 220/pressure half time was more accurate in predicting functional (haemodynamic) than anatomical (echocardiographic) mitral valve area.