L-arginine plasma levels and severity of idiopathic pulmonary arterial hypertension

VASA ◽  
2008 ◽  
Vol 37 (1) ◽  
pp. 61-67 ◽  
Author(s):  
Beyer ◽  
Kolditz ◽  
Ewert ◽  
Rubens ◽  
Opitz ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Plasma Levels ◽  
High Performance ◽  
Functional Class ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Pulmonary Arterial ◽  
Minute Walk

Background: Idiopathic pulmonary arterial hypertension (iPAH) is a rare disease of unknown aetiology characterized by a poor prognosis. Impairment of nitric oxide (NO) synthesis or NO-induced vasorelaxation has been suspected to play a role in the development of iPAH. This study was performed to investigate possible correlations between the plasma levels of the NO-related aminoacids L-arginine, L-citrulline and N-hydroxy-L-arginine (L-NHA) and the severity of iPAH. Methods: In twelve iPAH patients hemodynamics were measured by right heart catheterization, and plasma levels of L-arginine, L-citrulline and L-NHA were determined in blood samples from the pulmonary artery, peripheral artery and peripheral vein by high-performance liquid chromatography analysis. In eight of twelve patients a six minute walk test was performed. Results: Plasma levels of L-arginine strongly correlated to right atrial pressure, cardiac output, cardiac index, mixed-venous oxygen saturation, six minute walk data and NYHA functional class at all sites of blood sampling (p < 0.05). Conclusions: The results suggest a possible role of the NO precursor L-arginine in the pathogenesis of iPAH.

scholarly journals Evaluation of effectiveness and safety of the first Russian-manufactured generic bosentan use in patients with pulmonary arterial hypertension

2018 ◽  
Vol 15 (4) ◽  
pp. 53-58
Author(s):  
O A Arkhipova ◽  
S E Gratsianskaya ◽  
T V Martynyuk
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Systolic Pressure ◽  
Right Atrial ◽  
Pulmonary Artery Systolic Pressure ◽  
Heart Catheterization ◽  
Walk Distance ◽  
Pulmonary Arterial ◽  
Minute Walk Distance ◽  
Minute Walk

Objective. To study effectiveness and safety of generic bosentan use for 24 weeks in patients with pulmonary arterial hypertension (PAH). Materials and methods. The study included 42 patients. In 22 patients Bosentan therapy (Bosenex®, Sotex, Russia) was used for the first time. In 20 patients switching therapy from original bosentan (Tracleer, Аctelion Pharmaceuticals Ltd., Switzerland) was performed. The patients were followed up for 24±2 weeks. Results. After 24 weeks of treatment percent of patients with functional class (FC) III decreased from 55 to 30%, percent of patients with FC II increased from 45 to 55%, some patients (15%) achieved FC I, and the 6-minute walk distance increased on 52.1 meters. In the group of therapy change heart failure FC stabilization was observed, 6-minute walk distance increased on +14.8 meters (р>0.05). Echocardiography in the first group showed significant decrease of pulmonary artery systolic pressure (PASP) at -4 mm Hg and of right atrium area on 0.9 cm2. In the switched therapy group the difference was not significant. According to chest X-ray examination change of cardio-thoracic ratio, Murray and Lupi indexes was not significant in both groups. According to results of right heart catheterization improvement of mean pulmonary arterial pressure (-6.7 mm Hg), mean right atrial pressure (-1.6 mm Hg with reference value reached), and pulmonary vascular resistance (-293.2 dynes×sec/cm-5) was achieved; р

scholarly journals Triple combination therapy with macitentan, riociguat, and selexipag in a patient with idiopathic pulmonary arterial hypertension (functional class III)

Kardiologiia ◽  
2021 ◽  
Vol 61 (10) ◽  
pp. 104-107
Author(s):  
A. A. Proshkina ◽  
N. A. Tsareva ◽  
G. V. Nekludova ◽  
S. N. Avdeev
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Clinical Manifestations ◽  
Functional Class ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Triple Combination ◽  
Class Iii ◽  
Triple Combination Therapy ◽  
Pulmonary Arterial

The article presents a clinical case of successful triple combination therapy in a female patient with functional class III idiopathic pulmonary arterial hypertension. Supplementing the previous macitentan and riociguat treatment with selexipag reduced the severity of clinical manifestations of pulmonary hypertension. Also, the treatment efficacy was demonstrated by improvement of laboratory and instrumental indexes. Time-related changes were evaluated at 3 months after initiation of the selexipag treatment.

scholarly journals Acute vasoreactivity testing in pediatric idiopathic pulmonary arterial hypertension: an international survey on current practice

2019 ◽  
Vol 9 (4) ◽  
pp. 204589401985753
Author(s):  
Lina Caicedo ◽  
Rachel Hopper ◽  
Humberto Garcia Aguilar ◽  
Dunbar Ivy ◽  
Dora Haag ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Clinical Criteria ◽  
First Line Therapy ◽  
Specific Protocol ◽  
The World ◽  
Pulmonary Arterial

The aim of this study was to determine practice patterns and inter-institutional variability in how acute vasoreactivity testing (AVT) is performed and interpreted in pediatrics throughout the world. A survey was offered to physicians affiliated with the Pediatric & Congenital Heart Disease Taskforce of the Pulmonary Vascular Research Institute (PVRI), the Pediatric Pulmonary Hypertension Network (PPHNET), or the Spanish Registry for Pediatric Pulmonary Hypertension (REHIPED), from February to December 2016. The survey requested data about the site-specific protocol for AVT and subsequent management of pediatric patients with idiopathic pulmonary arterial hypertension (IPAH) or heritable PAH (HPAH). Twenty-eight centers from 13 countries answered the survey. AVT is performed in most centers using inhaled nitric oxide (iNO). Sitbon criteria was used in 39% of the centers, Barst criteria in 43%, and other criteria in 18%. First-line therapy for positive AVT responders in functional class (FC) I/II was calcium channel blocker (CCB) in 89%, but only in 68% as monotherapy. Most centers (71%) re-evaluated AVT-positive patients hemodynamics after 6–12 months; 29% of centers re-evaluated based only on clinical criteria. Most centers (64%) considered a good response as remaining in FC I or II, with near normalization of pulmonary arterial pressure and pulmonary vascular resistance, but a stable FC I/II alone was sufficient criteria in 25% of sites. Protocols and diagnostic criteria for AVT, and therapeutic approaches during follow-up, were highly variable across the world. Reported clinical practice is not fully congruent with current guidelines, suggesting the need for additional studies that better define the prognostic value of AVT for pediatric IPAH patients.

scholarly journals Is hyponatremia associated with mortality in pulmonary arterial hypertension?

2018 ◽  
Vol 8 (2) ◽  
pp. 204589401877688 ◽  
Author(s):  
Anastasiia A. Rudkovskaia ◽  
Adriano R. Tonelli ◽  
Youlan Rao ◽  
Jeffrey P. Hammel ◽  
Gregory K. Buller ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Sodium Level ◽  
Continuous Variable ◽  
Functional Class ◽  
Right Atrial ◽  
Left Heart Failure ◽  
Tertiary Referral Center ◽  
Pulmonary Arterial ◽  
One Year

Hyponatremia is associated with poor prognosis in left heart failure and liver disease. Its prognostic role in pulmonary arterial hypertension (PAH) is not well defined. We investigated the association between hyponatremia and one-year mortality in two large cohorts of PAH. This study is a secondary analysis evaluating the association between hyponatremia and one-year mortality in patients treated with subcutaneous treprostinil (cohort 1). The results are validated using a PAH registry at a tertiary referral center (cohort 2). Eight-hundred and twenty patients were enrolled in cohort 1 (mean age = 47 ± 14 years) and 791 in cohort 2 (mean age = 55 ± 15 years). Sodium level is negatively correlated with mean right atrial pressure (r = −0.09, P = 0.018; r = −0.089, P = 0.015 in cohorts 1 and 2, respectively). In unadjusted analyses of cohort 1, the sodium level (as a continuous variable) is associated with one-year mortality (hazard ratio = 0.94; P = 0.035). Hyponatremia loses its significance (as a continuous variable and when dichotomized at ≤ 137 mmol/L; P = 0.12) when adjusted for functional class (FC), which is identified as the variable whose presence turns the effect of sodium level into non-significant. Secondary analyses using a cut-off value of < 135 mmol/L showed similar results. These results are validated in cohort 2. Although the sample size for patients with sodium < 130 mmol/L is small (n = 31), severe hyponatremia is associated with higher overall mortality (47% versus 23%; P = 0.01), even when adjusting for age, FC, and baseline 6-min walk distance ( P < 0.001). Although baseline hyponatremia is associated with one-year mortality, it loses its significance when adjusted for FC.

scholarly journals Growth differentiation factor-15 levels in the blood around the pulmonary artery is associated with hospitalization for heart failure in patients with pulmonary arterial hypertension

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402096294
Author(s):  
Wei-Ting Chang ◽  
Jhih-Yuan Shih ◽  
Yu-Wen Lin ◽  
Zhih-Cherng Chen ◽  
Jun-Neng Roan ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Atrial ◽  
Heart Catheterization ◽  
Growth Differentiation Factor 15 ◽  
Arterial Blood ◽  
Differentiation Factor ◽  
Group I ◽  
Pulmonary Arterial

Despite no significant differences of growth differentiation factor-15 expressions in peripheral, right atrial, and right ventricular blood, in the pulmonary arterial blood, there was a significantly high level of growth differentiation factor-15 in Group I pulmonary arterial hypertension patients subsequently developing heart failure. During right heart catheterization, collecting pulmonary blood samples is suggested to measure growth differentiation factor-15.

scholarly journals The Modified Borg Dyspnea Scale does not predict hospitalization in pulmonary arterial hypertension

2017 ◽  
Vol 7 (2) ◽  
pp. 384-390 ◽  
Author(s):  
Debasree Banerjee ◽  
Jane Kamuren ◽  
Grayson L. Baird ◽  
Amy Palmisciano ◽  
Ipsita Krishnan ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
World Health ◽  
Pulmonary Vascular Disease ◽  
Common Symptom ◽  
Class Iii ◽  
Patient Reported ◽  
Pulmonary Arterial ◽  
Minute Walk

Background Breathlessness is the most common symptom reported by patients with pulmonary arterial hypertension (PAH). The Modified Borg Dyspnea Scale (MBS) is routinely obtained during the six-minute walk test in the assessment of PAH patients, but it is not known whether the MBS predicts clinical outcomes such as hospitalizations in PAH. Methods We performed a retrospective study of World Health Organization (WHO) Group 1 PAH patients followed at our center. The dates of the first three MBS and hospitalizations that occurred within three months of a documented MBS were collected. Marginal Cox hazard regression modeling was used to assess for a relationship between MBS and all-cause as well as PAH-related hospitalization. Results A total of 50 patients were included; most (92%) were functional class III/IV, 44% and 65% were treatment-naïve prior to their first MBS and hospitalization, respectively. The first recorded MBS was inversely correlated with the first recorded six-minute walk distance (6MWD) (r = –0.41, P < 0.01) but did not track with WHO functional class (r = 0.07, P = 0.63). MBS did not predict all-cause (hazard ratio [HR], 0.91; 95% confidence interval [CI], 0.76–1.08; P = 0.28) or PAH-related hospitalization (HR, 1.04; 95% CI, 0.89–1.23; P = 0.61), though there was a strong relationship between 6MWD and PAH-related hospitalization ( P = 0.01). These findings persisted after multivariable adjustment. Conclusions Breathlessness as assessed by MBS does not predict all-cause or PAH-related hospitalization. Robust and validated patient-reported outcomes are needed in pulmonary vascular disease.

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