PurposeTo 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.MethodsTwenty 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.ResultsRiociguat 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.ConclusionThese 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.Electronic supplementary materialThe online version of this article (10.1007/s00408-018-0160-4) contains supplementary material, which is available to authorized users.
The aim of the work is to compare vectorcardiographic (VCG) variables - spatial QRS-T angle and electrocardiographic ventricular gradient (VG) with echocardiography (EchoCG) data in patients with idiopathic pulmonary hypertension (IPH) and chronic thromboembolic pulmonary hypertension (CTEPH). Materials and methods. In 40 patients with IPH and 40 patients with CTEPH at the age of 45±12 years, systolic pulmonary artery pressure (SPAP); the sizes of heart chambers, parameters of RV systolic and diastolic function were evaluated with EchoCG. The QRS-T and VG angles were calculated on the VCG, derived from 12-lead digital ECG. Results. In all patients SPAP was greater than 40 mm Hg (mean 83±18 mm Hg), EchoCG data indicated hypertrophy and dilatation of RV, its systolic and diastolic function; dilatation of the right atrium (RA). Prognostically unfavorable changes in EchoCG were observed: the presence of pericardial effusion in 35 (44%) patients, RA area greater than 26 cm2 in 18 (23%) patients; TAPSE less than 1.5 cm in 37 (46%) patients. EchoCG and VCG variables had statistically significant differences in patients with III-IV functional class in comparison with I-II functional class. Statistically significant moderate correlations between VCG and EchoCG variables were revealed. VCG variables allowed to separate patient groups with the presence and absence of prognostically unfavorable changes in EchoCG with sensitivity from 54 to 78% and specificity from 66 to 87%. Conclusion. In patients with IPH and CTEPH, changes of QRS-T angle and VG correlate with SPAP, the size of RV and RA, parameters of RV systolic and diastolic function. The possibility of the use of QRS-T angle and VG for the detection of patients with prognostically unfavorable echocardiographic changes in the general group of patients with IPH and CTEPH has been shown.
Aim. To assess the clinical course of the disease and the features of the treatment goals achievement in patients with IPAH and inoperable CTEPH depending on gender and age at the time of diagnosis verification. Materials and methods. The study included 88 patients with IPAH and 38 patients with inoperable CTEPH with a PAWP 12 mm Hg and the duration of PAH-specific therapy treatment more than 12 months. IPAH/CTEPH patients were divided into groups depending on age at the time of diagnosis verification: age 50 years (n=69)/ 50 years (n=57), and gender: 106 women/20 men. Results. Patients with age 50 years at the time of diagnosis verification have significantly more severe functional class (WHO). In IPAH/CTEPH male patients a significant hemodynamic disorder also as significantly higher level of NT-proBNP at the time of diagnosis verification were observed. In men with IPAH/CTEPH and patients aged 50 years more pronounced deterioration of right ventricular systolic function was observed (ECHO). Patients with IPAH younger than 50 years were significantly more likely to achieve the treatment goals by the median 26.5 months of treatment compared to the patients aged 50 years (21% vs 6.45%). Men with IPAH/CTEPH were significantly more likely to have a high risk of death (90%) at baseline compared to the women (61%). Conclusion. IPAH/inoperable CTEPH patients with male sex, as well as the age 50 years and older at the time of diagnosis verification, compared with younger ones, are associated with a less favorable course of the disease.
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