Initial Riociguat Monotherapy and Transition from Sildenafil to Riociguat in Patients with Idiopathic Pulmonary Arterial Hypertension: Influence on Right Heart Remodeling and Right Ventricular-Pulmonary Arterial Coupling.

PURPOSE: To evaluate the influence of riociguat on World Health Organization functional class (WHO FC), 6-min walk distance (6MWD), right heart remodeling, and right ventricular-pulmonary arterial (RV-PA) coupling in patients with idiopathic pulmonary arterial hypertension (IPAH) who are treatment-naïve or who have failed to achieve treatment goals with sildenafil therapy. METHODS: Twenty patients with IPAH were enrolled: 12 had not previously received PAH-targeted therapy (treatment-naïve subgroup) and 8 had been receiving sildenafil therapy but failed to achieve treatment goals; on entering this pilot study these 8 patients were switched from sildenafil to riociguat therapy (treatment-switch subgroup). Patients received riociguat individually dose-adjusted up to a maximum of 2.5 mg three times daily. After 12 weeks, patients were assessed for WHO FC, 6MWD, right heart remodeling, and RV-PA coupling. RESULTS: Riociguat significantly improved WHO FC in treatment-naïve patients (from 0/4/8/0 patients in WHO I/II/III/IV at baseline to 1/6/5/0 at week 12) and in treatment-switch patients (from 0/4/4/0 patients in WHO I/II/III/IV at baseline to 1/4/3/0 at week 12). Additionally, treatment-naïve and treatment-switch patients showed significant improvements at week 12 versus baseline in 6MWD (increases of + 76.8 m and + 71.6 m, respectively), RV systolic function, and RV-PA coupling. CONCLUSION: These results support the proven efficacy of riociguat in patients with IPAH, including treatment-naïve patients and those switching to riociguat following failure to achieve treatment goals with sildenafil, and suggest that it may be possible to delay disease progression in this patient group.

Lipoprotein(a) and Its Autoantibodies in Association with Calcific Aortic Valve Stenosis.

Aortic valve stenosis is the most common valvular heart disease in the Western world. Lipoprotein(a) (Lp(a)) is an independent risk factor of coronary heart disease (CHD) and calcific aortic valve stenosis (CAVS). The aim of this study was to assess the role of Lp(a) and its autoantibodies [autoAbs] in CAVS in patients with and without CHD. We included 250 patients (mean age 69 ± 3 years, males 42%) and divided them into three groups. There were two groups of patients with CAVS depending on the presence (group 1) or absence of CHD (group 2). The control group included the patients without CHD or CAVS. According to logistic regression analysis, levels of Lp(a), IgM autoAbs to oxidized Lp(a) (oxLp(a)), and age were independent predictors of CAVS. A concomitant increase in Lp(a) level (≥30 mg/dL) and a decrease in IgM autoAbs concentration (<9.9 lab. Units) are associated with CAVS with an odds ratio (OR) of 6.4, p < 0.01, and with CAVS and CHD with an OR of 17.3, p < 0.001. IgM autoantibodies to oxLp(a) are associated with calcific aortic valve stenosis regardless of Lp(a) concentration and other risk factors. Higher Lp(a) and lower IgM autoantibodies to oxLp(a) levels are associated with a much higher risk of calcific aortic valve stenosis.

Rare Cause of Wide QRS Tachycardia.

Cardiac involvement is a well-known feature of neuromuscular diseases. Most commonly cardiac manifestations occur later in the course of the disease. Occasionally severe cardiac disease, including conduction disturbances, life-threatening arrhythmias, and cardiomyopathy, with its impact on prognosis, may be dissociated from peripheral myopathy. We report a case of bundle branch reentrant ventricular tachycardia as primary manifestation of myotonic dystrophy and discuss associated diagnostic and treatment challenges.

Ig apheresis for the treatment of severe DCM patients.

BACKGROUND: Autoantibodies against ß1-adrenoreceptor (AR) are considered by many authors to be the most significant in autoimmune process during DCM. Immunoadsorption (IA) of immunoglobulins (Ig apheresis) is a logic approach to remove autoantibodies against ß1-AR and other antibodies. The effect of Ig apheresis and the role of anti-ß1-AR in DCM are still an issue for discussion. METHODS: We have performed a prospective case-control study in 16 patients with DCM, NYHA Class II-IV congestive heart failure, positive and negative for anti-ß1-AR. RESULTS: We observed a clinically significant mean change of exercise tolerance compared with controls (6 MWT distance increased from 420 ± 130 m to 550 ± 150 m, p < 0.05). Systolic function improved rapidly by increase in LVEF from 28.6 ± 5.2% to 33.0 ± 10.3%, LV end-systolic and end-diastolic volumes decreased from 166 ± 58 mL to 148 ± 50 mL and from 235 ± 73 mL to 220 ± 73 mL, respectively, whereas in the control group there was no significant change in clinical variables. The improved quality of life and cardiac function in apheresis group as well as negative changes in control group didn't correlate with the presence of anti-ß1-AR. CONCLUSIONS: Ig apheresis for the treatment of DCM patients is associated with the improvement of quality of life and cardiac function regardless of the presence of anti-ß1-AR. We suggest that IgG apheresis is a safe and effective method for DCM patients.