Cardiac involvement in Ribbing's disease.

Cardiac involvement was assessed in 14 patients with Ribbing's disease, a rare hereditary sclerosing bone dysplasia. When compared to age-, sex- and blood pressure-matched controls, the patients with Ribbing's disease had significant alterations in left ventricular systolic and diastolic function and an impaired exercise tolerance. Supraventricular and ventricular arrhythmias also tended to be more frequent in these patients than in controls. In conclusion, Ribbing's disease, initially described as a skeletal disease only, also involves the cardiovascular system. Despite its rarity, the expression of the disease is easily followed and identification of the genetic defect could shed some new light on the pathophysiological mechanisms linking hypertension, myocardial hypertrophy and diastolic dysfunction.

Dose-titration study of alfuzosin, a new alpha 1-adrenoceptor blocker, in essential hypertension.

An open, dose-titration study of alfuzosin, a new selective post-synaptic alpha 1-adrenoceptor antagonist with additional direct vasodilator properties has been performed. After a 3-week run-in placebo period, 12 patients with essential hypertension received alfuzosin 5 mg oral b.d., and then the dose was doubled every week, up to a maximum of 20 mg q.i.d. if the supine diastolic blood pressure was greater than 90 mm Hg. The study lasted for 4 weeks. Supine blood pressure (SBP) decreased from 160/102 (Day 0) to 148/89 mm Hg and upright blood pressure (UBP) from 151/102 (Day 0) to 137/84 mm Hg. Alfuzosin did not cause any significant change in supine or upright heart rate. In addition, after the first dose of alfuzosin, supine and upright blood pressure and heart rate (SHR and UHR) were measured every 30 min for 5 h. The fall in blood pressure was significant after 90 min and it lasted up to the 5th hour; the maximum effect was observed after 3 h: SBP decreased from 159/103 (time 0) to 137/84 mm Hg and UBP from 150/102 (time 0) to 123/79 mm Hg. SHR was increased from 72 (time 0) to 81 beats/min at the 5th hour and UHR from 87 to 101 beats/min at the 4th hour. A weak but significant correlation was observed between the hypotensive effect 12 h after drug intake and the plasma concentration of the drug at that time. A 10% decrease in supine diastolic blood pressure was found at a drug plasma concentration higher than 7 ng/ml.(ABSTRACT TRUNCATED AT 250 WORDS)

Antiarrhythmic use of cibenzoline, a new class 1 antiarrhythmic agent with class 3 and 4 properties, in patients with recurrent ventricular tachycardia.

Cibenzoline is a new antiarrhythmic agent with class 1 properties, and additional class 3 and 4 effects. We treated 28 patients with drug-refractory and recurrent ventricular tachycardia with up to 700 mg/day cibenzoline for periods up to 5.5 months. Cibenzoline prevented the recurrence of ventricular tachycardia in five patients (18%). In three patients (11%) the arrhythmia may have been worsened, in 23 patients (82%) cibenzoline was ineffective. Cibenzoline increased the PR interval by 18% and the QRS duration by 33%; the effect on the QT was variable and the corrected QT interval did not change significantly. Side-effects were observed in 21% of patients. We conclude that cibenzoline does not appear to be superior to conventional class 1 antiarrhythmic agents and that it cannot be recommended for general use in patients with ventricular tachycardia. Additional pharmacokinetic and electrophysiologic studies are required before cibenzoline is used in outpatients with severe ventricular arrhythmias.

Vasodilator treatment for mild to moderate heart failure. Effect of Ro 12-4713, a minoxidil analogue.

18 patients (14 men, 4 women) with a mean age of 64.3 years (range 41-75) and with chronic cardiac failure, NYHA functional class 1-2 (12 cases) or 2-3 (6 cases), insufficiently responsive to digitalis and diuretics, were treated for 4 weeks with Ro 12-4713, a minoxidil-like drug. The effects were assessed at rest and during maximal exercise, each patient was his own control. All patients completed the study. The treatment did not affect the body weight, the heart rate or the blood pressure, but it should be stressed that all patients were normotensive. The ejection fraction (by 99mTc pertechnetate scintigraphy) increased from 42.0 +/- 10.0 (resting means +/- SD) to 48.9 +/- 8.7%, and from 44.7 +/- 10.1 to 57.9 +/- 8.7% (end-exercise values), both changes being significant (p less than 0.0001). The ejection rate increased in a similar manner. The resting stroke volume increase from 53.5 +/- 13.6 to 60.2 +/- 16.5 ml (p less than 0.005), but the exercise stroke volume did not increase significantly. The cardiac output increased both at rest and during exercise, but the changes did not reach statistical significance. The end-diastolic volume decreased from 132.6 +/- 126.9 +/- 47.0 to 126.9 +/- 42.3 ml (resting values), and from 151.5 +/- 44.4 to 120.6 +/- 36.6 ml (exercise), the changes being significant (p less than 0.005). The physical work capacity increased, i.e. the duration of exercise increased from 7.8 +/- 3.2 to 9.9 +/- 1.9 min (p less than 0.05) and the 'quality of life' (symptoms related to heart failure) improved (fewer symptoms) in 14/18 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Tiapamil in the management of supraventricular arrhythmias occurring after acute myocardial infarction.

18 patients with acute myocardial infarction and sustained supraventricular arrhythmias were treated with tiapamil, in a dose of 1 mg/kg i.v. The drug was effective, i.e., the heart rate was reduced to less than 90 beats/min in 9 of 10 patients with atrial fibrillation, in 3 of 4 patients with atrial flutter, and in 3 of 4 patients with atrial tachycardia. The peak effect was observed within 2-5 min. In cases with recurring tachyarrhythmias, tiapamil was also effective during successive administrations, the systolic blood pressure was reduced by 10-15%, but severe hypotension was not observed. In these patients, no major changes in atrioventricular or intraventricular conduction were observed. In the dose used, tiapamil was effective and well tolerated.