pulmonary artery hypertension
pulmonary artery hypertension |
- Pulmonary Artery Denervation Can Improve CpcPH Patients’ Outcome, Trial Shows - Pulmonary Hypertension News
- Soluble Neprilysin Not Linked to Prognosis in Pulmonary Hypertension - The Cardiology Advisor
- Children with IPH Have Similar Transplant Survival Rates as Those with Other Indications, Study Says - Pulmonary Hypertension News
- Should Macitentan Plus Riociguat Be Used as First-Line Therapy in PAH? - Pulmonology Advisor
| Posted: 15 Feb 2019 12:00 AM PST Invasive pulmonary artery denervation is safe and can improve the outcome of patients with combined pre- and post-capillary pulmonary hypertension associated with left heart failure, compared with standard of care, results of a randomized trial show. The findings were reported in the study "Pulmonary Artery Denervation Significantly Increases 6-Min Walk Distance for Patients With Combined Pre- and Post-Capillary Pulmonary Hypertension Associated With Left Heart Failure: The PADN-5 Study," published in the journal JACC: Cardiovascular Interventions. In many patients, pulmonary hypertension (PH) is caused by left heart disease (LHD), including left ventricular dysfunction, valve disease, or congenital left heart disease. In recent years, an additional form of the disease with significant vascular remodeling was identified as reactive or combined pre-capillary and post-capillary PH (CpcPH). CpcPH is defined by 7 mmHg or higher of diastolic pressure gradient (DPG), more than 3 Wood units for pulmonary vascular resistance (PVR), or both. This new entity has a different clinical presentation than PH and, despite its potential poor outcome, it remains unclear whether PH-targeted therapies are suitable to manage CpcPH. Interested in PH research? Check out our forums and join the conversation!Previous studies have suggested that a pulmonary arterial vasoconstrictor component is involved in the development of CpcPH. This could mean that pulmonary artery denervation (PADN) — a procedure to reduce the overactivation of nerves controlling the vasoconstrictor mechanism— could be a potential strategy to treat people with CpcPH. Results of a previous Phase 2 clinical trial (chiCTR-ONC-12002085) demonstrated that PADN was safe and feasible to manage the symptoms of patients with idiopathic pulmonary arterial hypertension (PAH). Patients showed significant improvements in overall blood flow pressure, exercise capacity, and cardiac function, with less frequent PAH-related events and death after PADN treatment. Now researchers evaluated in a new clinical study (NCT02220335) the potential of PADN to manage the symptoms of patients with CpcPH associated with LHD. The study enrolled 98 patients across four sites. They were randomized to undergo PADN treatment or to take Revatio (sildenafil, marketed by Pfizer) and undergo a sham PADN procedure (a faked surgical intervention without the therapeutic steps). After six months of follow-up, patients treated with PADN showed an improvement of 21.4%, compared to baseline, in the 6-minute walk distance test (6MWD) — a test assessing exercise capacity. Patients treated with Revatio had an improvement of only about 4.9%. Still, 18 patients showed a reduction in exercise capacity, including six in the PADN group. Additional analyses showed that a reduction in 6MWD in these patients was associated with an increased risk of clinical worsening. PADN-treated patients also showed significantly lower PVR than those treated with Revatio at six months, 29.8% compared to 3.4%. In general, the PADN procedure was found to be associated with significant improvements in all hemodynamic (blood flow) parameters, except for right atrial pressure, which tended to be lower. Also, "clinical worsening was less frequent in the PADN group compared with the sildenafil [Revatio] group (16.7% vs. 40%)," the researchers said. At the end of the study there were seven all-cause reported deaths, two of which were in the PADN group and five in the Revatio-treated group. Overall, "the present randomized sham-controlled study is the first to our knowledge (…) to demonstrate the hemodynamic and functional benefits of PADN in patients with CpcPH," researchers said. Additional randomized studies are still needed to further compare the long-term impact of PADN in the outcome of this particular patient population.  |
| Soluble Neprilysin Not Linked to Prognosis in Pulmonary Hypertension - The Cardiology Advisor Posted: 14 Feb 2019 12:00 AM PST  Among individuals with pulmonary hypertension, no associations have been found between circulating soluble neprilysin and prognosis, natriuretic peptide, or right heart catheterization parameters, according to study results recently published in ESC Heart Failure. This prospective observational study included 79 individuals with pulmonary hypertension whose pulmonary arterial pressure was ≥25 mm Hg and whose pulmonary artery wedge pressure was ≤15 mm Hg. Right heart catheterization was used to determine these values. The study sample's mean pulmonary arterial pressure was 42.9±14.5 mm Hg. Associations were investigated between levels of soluble neprilysin and parameters of natriuretic peptide, right heart catheter, or echocardiography. Participants were followed for incidence of all-cause mortality. To compare categorical variables, the chi-squared test was used. The Student's t-test was used to compare parametric variables whereas the Mann–Whitney U test was used for nonparametric variables. Soluble neprilysin was not correlated with B-type natriuretic peptide (P =.86), high-sensitivity troponin I (P =.11), N-terminal pro B-type natriuretic peptide (P =.87), right ventricular fractional area change (P =.06), left ventricular ejection fraction (P =.76), tricuspid valve pressure gradient (P =.77), or parameters related to the right heart catheter. All-cause mortality did not differ significantly between patients with higher and patients with lower neprilysin levels (P =.53) and was not predicted by soluble neprilysin (P =.49). Limitations to this study included a small cohort, use of exclusively hospitalization-related variables, and measurement of certain peptides but not others such as angiotensin II, endothelin-1, adrenomedullin, substance P, or bradykinin. The study researchers concluded that "[soluble neprilysin] does not correlate with any parameters of [right heart catheterization], echocardiography, or laboratory data, including natriuretic peptides and troponin I, and is not associated with prognosis in [pulmonary hypertension] patients." Several authors reported associations with organizations supported by pharmaceutical companies. For a full list of author disclosures, see the reference. Reference Yoshihisa A, Yokokawa T, Misaka T, et al. Soluble neprilysin does not correlate with prognosis in pulmonary hypertension [published online January 25, 2019]. ESC Heart Fail. doi: 10.1002/ehf2.12404  |
| Posted: 04 Mar 2019 04:00 AM PST Children with idiopathic pulmonary hypertension (IPH) who underwent lung or combined heart-lung transplants show similar survival rates to children who underwent these procedures for other indications, according to a study. Data also indicated that children who had a transplant at an older age had better outcomes. The study, "Transplant outcomes for idiopathic pulmonary hypertension in children," was published in The Journal of Heart and Lung Transplantation. IPH is an indication for lung transplant or combined heart‒lung transplant in children. Prior studies of lung transplant outcomes in pediatric populations have analyzed a small number of cases, which limits the accuracy of the data. A team at Nationwide Children's Hospital in Ohio used the United Network for Organ Sharing (UNOS) registry to evaluate outcomes in children with IPH undergoing lung or combined heart-lung transplant, and to assess clinical characteristics that could be associated with post-transplant survival. In total, 65 children (26 boys, 39 girls, mean age of 10 years) were included in the study. They had transplants between May 2005 and December 2015, with follow-up data collected through December 2016. Forty-seven patients underwent a double lung transplant, and 18 underwent heart and lung transplant. Analyzed clinical data included patients' age, lung allocation score (LAS; used to prioritize waiting list candidates by estimating the severity of illness and chance of post-transplant survival), Status 1A designation for heart‒lung transplant (which refers to patients with the highest priority based on medical urgency), and mean pulmonary artery pressure (mPAP). Results showed a total of 28 deaths, 19 in the group undergoing lung transplants and nine in children having a combined heart-lung transplant. Five-year survival rates were 61% for lung and 48% for combined heart-lung transplants, although this difference was not statistically significant. In addition, overall survival in the two groups was not different from that of children undergoing the same procedures for other indications. Researchers also observed that older age at the time of the transplant correlated with a lower risk of death. In contrast, LAS in patients older than 12 years, Status 1A in patients undergoing combined transplant, and mPAP before the procedure did not significantly influence survival. Fifty-four additional children were listed for but did not receive a transplant; of these, 22 (40.7%) died, 14 experienced clinical improvement and no longer required a transplant, three refused to have the procedure, and four were too sick. These non-transplanted patients had lower mPAP (63 mmHg vs. 77 mmHg), and had Status 1A less frequently (4% vs. 20%) than children who went through with the procedure. These results confirmed data from The International Society for Heart and Lung Transplantation Registry, showing that "post-transplant outcomes for IPH appear to be similar to children transplanted for other indications," the researchers wrote. "In conclusion, the very small number of children transplanted for a primary indication of IPH (65 over a 10-year period) suggests that transplant may be an underutilized treatment modality in this population, and should strongly be considered for appropriate pediatric candidates, especially older children," they said. Results also suggest that measures of disease severity, such as mPAP and LAS, "may not accurately reflect chances of long-term survival in this patient population," according to the team.  |
| Should Macitentan Plus Riociguat Be Used as First-Line Therapy in PAH? - Pulmonology Advisor Posted: 07 Feb 2019 12:00 AM PST  Upfront combination therapy with macitentan plus riociguat has demonstrated effectiveness in patients with pulmonary arterial hypertension (PAH), according to the results of a small retrospective, open-label cohort study conducted between 2014 and 2016. Findings from the study were published in Pulmonary Circulation. Investigators sought to assess the hemodynamic and clinical effects of combined macitentan and riociguat as first-line treatment for newly diagnosed patients with PAH from their practice at the Mount Sinai Beth Israel Pulmonary Hypertension Program in New York City. Clinical and hemodynamic data were collected from 15 consecutive patients with PAH. All data were obtained at baseline, at visit 1 (median, 4 months), and at visit 2 (median, 12 months). Patient survival and transplantation status were analyzed during a 36-month period. Overall, 73.3% (11 of 15) of the patients were women; the mean patient age was 55.8 years (range, 27-82 years). At treatment initiation, 14 of the patients belonged to World Health Organization functional class III (WHO FC III). The last patient, who had systemic lupus erythematosus-PAH, was classified as WHO FC IV because of a history of exertional syncope. Study results demonstrated a significant increase in 6-minute walk distance from 281.6 m at baseline, to 315.7 m at visit 1, to 313.9 m at visit 2, representing a 34-m and 32-m change, respectively, associated with improvements in Borg score (P <.05). Moreover, significant decreases in brain natriuretic peptide were reported: from 318.2 pg/mL at baseline to 122.0 pg/mL at visit 1, and to 98.6 pg/mL at visit 2 (P <.05 for visit 2 compared with baseline). Additional findings showed WHO FC improvements in 8 patients (53%; 95% CI, 27%- 79%). Further, pulmonary vascular resistance (from 9.2-5.7 Wood units) and mean pulmonary artery pressure (from 47.3-38.9 mm Hg) decreased significantly from baseline to visit 2 (P <.05). Cardiac index, in contrast, increased significantly from 2.3 L/min/m2 to 3.0 L/min/m2 from baseline to visit 2. At baseline, 14 of the participants were at intermediate risk and 1 of the participants was at high risk. At visit 2, a total of 7 patients experienced a reduction in risk, 6 patients had a decrease in risk from intermediate to low, and 1 patient had a decrease in risk from high to low. In 8 patients, no change in risk was reported, with these individuals maintaining intermediate risk status. Some adverse effects were reported, but none were serious or unexpected. Overall, 3 patients died from unrelated causes and 1 patient underwent lung transplantation. The transplant-free survival rate was 85.1% at 36 months. This small study is the first of its kind to show that dual-treatment combinations other than ambrisentan plus tadalafil may be used in patients with PAH. The researchers concluded that additional research with a more definitive study design is warranted to further delineate the role of combined macitentan plus riociguat in patients with PAH. Reference Sulica R, Sangli S, Chakravarti A, Steiger D. Clinical and hemodynamic benefit of macitentan and riociguat upfront combination in patients with pulmonary arterial hypertension [published online January 14, 2019]. Pulm Circ. doi:10.1177/2045894019826944  |
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