Efficacy of bosentan in the treatment of a patient with mixed connective tissue disease complicated by pulmonary arterial hypertension.

This study aimed to investigate the efficacy of bosentan in the treatment of severe pulmonary hypertension in a young female patient with mixed connective tissue disease (MCTD) associated with antiphospholipid syndrome. A 27-year-old woman presented with sudden onset of dyspnea. She had not experienced any dyspnea before this period, and she was known to have MCTD. Laboratory tests showed positive results for antinuclear antibodies, anti-RNP antibodies, anticardiolipin antibodies, beta(2)-glycoprotein I, and lupus anticoagulant. A complete echocardiographic study was performed demonstrating a pulmonary artery systolic pressure (PAPs) of 85 mmHg. Treatment with bosentan was initiated. After 12 days, the patient improved clinically. After 6 months of therapy, the follow-up echocardiography showed a near-normalization of PAPs. Patients who develop PAH secondary to an underlying systemic disease often have a poor survival rate. In this report, we describe a correlation between anticardiolipin antibodies and rapidly progressive pulmonary hypertension. Indeed, the patient in this study very likely improved secondary to the effect of bosentan which produces systemic and pulmonary vasodilatation associated with pulmonary vascular remodeling as well as possible antifibrotic, anti-inflammatory and antiatherothrombotic effects on cells of lungs damaged by an aPL-antibody mediated mechanism.

Antiarrhythmic drug therapy after radiofrequency catheter ablation in patients with atrial fibrillation.

OBJECTIVES: The use of antiarrhythmic drugs after ablation is a controversial issue when evaluating the efficacy of atrial fibrillation (AF) ablation. This study compares in a prospective and randomized fashion the impact of an antiarrhythmic drug in preventing AF recurrence after AF ablation. METHODS: From February 2004 to May 2005, 107 consecutive patients (mean age 57 +/- 10 years, 69 men), with paroxysmal (60%) or persistent (40%) drug refractory AF, were randomly assigned to ablation alone (Group A, 53 patients) or combined with the best antiarrhythmic therapy, preferably amiodarone (Group B, 54 patients). All patients underwent cavo-tricuspid and left inferior pulmonary vein (PV)-mitral isthmus ablation plus circumferential PV ablation, using a guided electro-anatomical approach. Standard electrocardiograms (ECG), and ambulatory and transtelephonic ECG monitoring were used to assess AF recurrences. Recurrences during the first month after ablation were excluded from this analysis. RESULTS: At 12 months of follow-up, no significant difference was observed in the rates of AF recurrences between Group A (18/53 patients, 34%) and Group B (16/54 patients, 30%). The percentage of patients with >/= 1 asymptomatic AF episode was higher in Group B than in Group A (10/16 patients, 63%, vs 5/18 patients, 28%, P = 0.04). CONCLUSIONS: Continuing antiarrhythmic drug therapy in patients who undergo catheter ablation for AF did not lower the rate of AF recurrences. Antiarrhythmic drugs increased the proportion of patients with asymptomatic AF episodes.