PHA 2022 International Pulmonary Hypertension Conference Abstracts

Lily M. LandryDepartment of Pediatrics, Division of Pediatric Cardiology University of Mississippi Medical Center, Jackson, MS, USAAndrew C. BurksSchool of Medicine, University of Mississippi Medical Center, Jackson, MS, USAOnyekachukwu OsakweDepartment of Pediatrics, Division of Pediatric Cardiology, University of Mississippi Medical Center, Jackson, MS, USAJarrod D. KnudsonDepartment of Pediatrics, Division of Pediatric Critical Care, University of Mississippi Medical Center, Jackson, MS, USAChristopher L. JenksDepartment of Pediatrics, Division of Pediatric Critical Care, University of Mississippi Medical Center, Jackson, MS, USACategory: Clinical ScienceSelected Areas: Pediatrics, Therapeutic StrategiesBackground: Pulmonary hypertension (PH) is a rare and potentially fatal disease in children if left untreated. Emerging therapies, including bosentan, a dual endothelin receptor antagonist (ERA), have shown significant benefits in the adult PH population; however, few studies have assessed the efficacy and safety of ERAs in infants and young children.Methods: Our study was a single-center retrospective analysis of patients <2 years of age with a confirmed diagnosis of PH and started on bosentan therapy between 2017 and 2020. Twelve cases met eligibility criteria. Demographic (Table 1), laboratory, echocardiographic, and cardiac catheterization data were analyzed (Tables 2–4).Results: With treatment, there was a statistically significant decrease in mean right ventricular systolic pressure estimated by the tricuspid regurgitation jet (79 ± 23 mm Hg reduced to 52 ± 25 mm Hg; P value <0.001) and pro-brain natriuretic peptide levels (21 071 reduced to 2037; P < 0.001). Additionally, improvement and eventual normalization of right ventricular function and septal geometry was seen within the first 4 months of therapy (Figure). Patients who underwent cardiac catheterization after therapy initiation (n = 4) demonstrated hemodynamic improvements; however, only the decrease in diastolic pulmonary pressure was statistically significant (P value = 0.018). No significant difference in hemoglobin, platelet count, or liver function tests was observed between groups.Conclusions: In conclusion, these data suggest that bosentan may be an effective and relatively safe treatment option for children <2 years of age with PH. Further long-term randomized control studies are necessary to validate the potential clinical benefit of using this drug therapy in young children.Essam IbrahimJanssen Inc., Toronto, Ontario, CanadaAssem Al-AkabawiJanssen Inc., Toronto, Ontario, CanadaMoses DawoduJanssen Inc., Toronto, Ontario, CanadaJuejing LingIQVIA Solutions Canada Inc., Kirkland, Quebec, CanadaIrene WangIQVIA Solutions Canada Inc., Kirkland, Quebec, CanadaBrad MillsonIQVIA Solutions Canada Inc., Kirkland, Quebec, CanadaCategory: Clinical ScienceSelected Areas: Databases and RegistriesBackground: The current standard of care for pulmonary arterial hypertension (PAH) is combination therapy, which imposes pill burden on PAH patients who live with multiple comorbidities. This study aims to understand the concomitant medication use in treatment journey of Canadian PAH patients.Methods: Patients initiating PAH therapy (index) from September 1, 2016, to August 31, 2019, were selected in the Ontario Drug Benefits (ODB), Régie de l’assurance maladie du Québec (RAMQ), and IQVIA Canadian Private Drug Plan (PDP) databases. Medication prescriptions 5-year prior and 1-year postindex were tracked. Concomitant medication analysis used ODB and PDP; prescriber specialty analysis used PDP database.Results: A total of 1019 patients were included from ODB (N = 481), PDP (N = 422), and RAMQ (N = 116). Tadalafil (61%) and sildenafil (22%) were most prescribed at index. One year prior to index, 75% and 30% of patients with concomitant medications (N = 828) were on 5+ and 10+ concomitant medication classes, respectively. From 5 years prior to the 1-year postindex, the proportion of cardiovascular medications use declined (16% versus 13%), while that of diuretics use increased (10% versus 16%). Compared with the year prior, prescriptions of rescue treatment decreased 1 year postindex (926 versus 763). Here, 72% of patients (N = 300) were prescribed by general practitioner (GP) before index, while prescribers of cardiovascular and respiratory medications shifted from GP to specialist postindex. Among the top 3 prescribers, females had a significant reduction in the number of concomitant medications postindex, while males trended the opposite way.Conclusions: This study shows the complex treatment journey of Canadian PAH patients and highlights the need to reduce pill burden and improve disease management.Essam IbrahimJanssen Inc., Toronto, Ontario, CanadaAssem Al-AkabawiJanssen Inc., Toronto, Ontario, CanadaMoses DawoduJanssen Inc., Toronto, Ontario, CanadaJuejing LingIQVIA Solutions Canada Inc., Kirkland, Quebec, CanadaJillian MurrayIQVIA Solutions Canada Inc., Kirkland, Quebec, CanadaBrad MillsonIQVIA Solutions Canada Inc., Kirkland, Quebec, CanadaCategory: Clinical ScienceSelected Areas: Databases and RegistriesBackground: Treatment guidelines for pulmonary arterial hypertension (PAH) recommend combination therapy in most patients. This study evaluated retention of PAH drugs and time-to-initiation of combination drug therapy with macitentan and selexipag in Canada.Methods: Patients claiming PAH drugs from January 2017 to March 2021 were identified in the Ontario Drug Benefits (ODB), Régie de l’assurance maladie du Québec (RAMQ), and IQVIA Canadian Private Drug Plan (PDP) databases. Drug retention was calculated for patients submitting claims 12 months following treatment index (March 2019 to February 2020 [PDP, ODB]; January to December 2019 [RAMQ]). Treatment was considered first line if patients were treatment naïve with >180 days of claims history. Time-to-initiation of combination therapy was calculated for macitentan+PDE5i and selexipag-containing therapies.Results: Around 2150 patients claimed PAH drugs annually, with averages of 1000 (PDP), 900 (ODB), and 250 (RAMQ). Most patients were female (65%–77%) and aged 45+ years (69%–85%). The 12-month retention was greatest for macitentan in PDP (67%) and RAMQ (88%), and tadalafil (78%) in ODB. Of patients on macitentan or selexipag, approximately 79% and 95% were on combination therapy (2+ drugs) in 2020, respectively. In 2020, 12%–17% of patients were on macitentan combination therapy (3+ drugs), and 43%–69% were on selexipag combination therapy (3+ drugs). In 2020, mean time-to-initiation was 4–5 months for macitentan+PDE5i and 17–23 months for selexipag-containing therapies (Table).Conclusions: Real-world data from Canada shows that most PAH patients on macitentan or selexipag are on combination therapies. Time-to-initiation was 4–5 months for macitentan+PDE5i, and 17–23 months for selexipag-containing combination therapies. This study shows the complex treatment journey of Canadian PAH patients and highlights the need to reduce pill burden and improve disease management.Roxana SulicaPulmonary Critical Care and Sleep Division, NYU Grossman School of Medicine, NYU Langone Health, New York, NY, USAStacy MandrasAdventHealth Transplant Institute, Orlando, FL, USAChad MillerPiedmont Physicians, Pulmonary Hypertension/Pulmonary Critical Care Medicine, Piedmont Healthcare, Austell, GA, USAR. James WhiteUniversity of Rochester Medical Center, Rochester, NY, USANick H. KimDivision of Pulmonary and Critical Care Medicine, University of California San Diego, La Jolla, CA, USASameer BansilalBayer US, LLC, Whippany, NJ, USAVicki PoonBayer US, LLC, Whippany, NJ, USADaniel JohnsonBayer US, LLC, Whippany, NJ, USAValerie CarvajalBayer US, LLC, Whippany, NJ, USAMurali ChakinalaDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Washington University at Barnes-Jewish, St. Louis, MO, USACategory: Clinical ScienceSelected Areas: Databases and Registries, Quality of Life, Therapeutic StrategiesBackground: Initial pulmonary arterial hypertension (PAH) treatment has evolved from monotherapy to combinations of approved drug classes. Riociguat was approved for PAH based on the PATENT study, which studied it as monotherapy or in sequential combination with an endothelin receptor antagonist (ERA) or nonparenteral prostanoid. PATENT and REPLACE gave substantial data on riociguat combinations, but there are no US-based registry data on riociguat.Methods: ROAR (NCT04813926; funder: Bayer US, LLC) is a US-based, multicenter, prospective, observational registry of adults with PAH who are riociguat-naïve or who initiated riociguat 90 days previously. Data will be collected at standard-of-care visits (usually every 3–6 months) for 24 months or until 30 days after discontinuing riociguat. Planned enrollment: 500 patients (~50 sites). The is to study safety and of riociguat as therapy or with an prostanoid. safety and of riociguat versus from a and the of treatment from to months and 12 in clinical PAH and New and will be assessed at and safety are of selected and 24 ROAR in January patients were data will be Inc., San Diego, CA, LLC, NJ, Inc., San Diego, CA, Inc., San Diego, CA, C. University School of Medicine, USACategory: Clinical ScienceSelected Areas: Databases and RegistriesBackground: of therapies of disease are to in patients with pulmonary arterial hypertension but is in PAH are a to that or by PAH patients.Methods: A was an used by patients and to and for care were years with 1 PAH had a PAH diagnosis for and had in a clinical The was approved by a and One patients the age was from PAH diagnosis was and and of were and in 75% a with a are PAH patients are in clinical is to of current therapy and and a to study drug after Further is for patients and to University of Pulmonary Institute, Pulmonary of Pulmonary and Critical Care Medicine, School of Medicine, of Pulmonary and Critical Care Medicine, School of Medicine, Medicine, Institute, School of Medicine, School of Medicine, Medicine, of Pulmonary and Critical Care Medicine, School of Medicine, USACategory: Clinical ScienceSelected Areas: Therapeutic StrategiesBackground: The most pulmonary arterial hypertension (PAH) guidelines the of to disease and are in clinical to to and which may clinical A retrospective was to understand the of at a pulmonary hypertension (PH) specialty including a and were to use of a for on the and the of necessary for A for the the and were used to and the of from to The of patients with a in the months increased from to The of natriuretic peptide and pulmonary tests 1 and of a with improvement is an effective to use of PAH in a is to understand this increased of The of on the and of is of M. Areas: or and and with Quality of are the most of of year in the Among with 1 in 4 with a need for in In this study, the of and in pulmonary arterial hypertension (PAH) cases to in of of cardiac were in of In this clinical and of PAH cases to who were at of the cardiac and to and in cardiac The first of an female who was from the was in days of had a and was the of a after months of had an of on the first The difference in to in cases was to and and clinical in and in in in of may in diagnosis and of cardiac and diagnosis and of cardiac of children with if with of care at Inc., San Diego, CA, Inc., San Diego, CA, Clinical LLC, Inc., San Diego, CA, Inc., San Diego, CA, USACategory: Clinical ScienceSelected Areas: Therapeutic StrategiesBackground: is an for the treatment of pulmonary arterial hypertension with an that and and these may pulmonary and In by the on in and that are in evaluated the potential for with adults a of and with or and safety were mean for and of with and are is a the on on the is to with most PAH drugs that are bosentan, and these drugs may be In the of be considered based on In with of for or are was with or Inc., San Diego, CA, Institute, of Rochester, Inc., San Diego, CA, Pulmonary D. of and Sleep Medicine, FL, Inc., San Diego, CA, Inc., San Diego, CA, Inc., San Diego, CA, Inc., San Diego, CA, Inc., San Diego, CA, Inc., San Diego, CA, Clinical ScienceSelected Areas: Therapeutic StrategiesBackground: is an which and in pulmonary arterial hypertension (PAH) approximately and in studies suggest that in the is at the levels evaluated in of and of in PAH In a multicenter, study, with PAH were randomized to to 90 or for and were collected at and for 5 and A was to and were (n = or (n = was at to 90 at 5 but to with in data at identified in of following treatment was and in the that suggest by in PAH patients. is evaluated as a treatment for patients with PAH standard of care therapies in a study Rochester, of CA, L. Medical Center, Medical Center, CA, Inc., San Diego, CA, Inc., San Diego, CA, Inc., San Diego, CA, Inc., San Diego, CA, Inc., San Diego, CA, Inc., San Diego, CA, University Clinical ScienceSelected Areas: Therapeutic StrategiesBackground: is a for treatment of patients with pulmonary arterial hypertension and in PAH and is with a to the to and potentially improve efficacy and A study is is a study to the efficacy and safety of in adult with 1 pulmonary hypertension Patients with or PAH on therapy with a pulmonary are The treatment is 24 with a of patients. The is in by right catheterization from to 24 The is in from to 24 will In the the of on cardiac will be The will the of on pulmonary by in pulmonary arterial will be evaluated for and to This was at the and New NY, USACategory: Areas: and with hypertension is pulmonary arterial hypertension (PAH) in Pulmonary hypertension (PH) is in 1 is PAH with including or is PH to left 3 is PH to 4 is PH 5 is of hypertension in the of or This in the to to as and hypertension to of patients with liver A with PH on Pulmonary 1 with of February August and by and and was evaluated for liver had an which PH. was after cardiac catheterization which PH. was started on medications with had and was for of Medical Center, Rochester, University School of Medicine, Orlando, FL, of School of USACategory: Clinical ScienceSelected Areas: Databases and Registries, or and and with The pulmonary arterial hypertension (PAH) and The Pulmonary is an of the for (Table may be or The study to if with is a registry of PAH patients in collected and at and were to study and were if and the within 30 days of between and was were from patients at and PAH disease (Table of were on the (Table of and were the patients and patients with This is the first study between and Patients from with a of PAH disease and of use in clinical for L. University Medical Center, University Medical Center, of California San San CA, of Medical Center, USADaniel University School of Medicine, Inc., Inc., USACategory: Clinical ScienceSelected Areas: Therapeutic StrategiesBackground: Pulmonary arterial hypertension (PAH) is using to improve and clinical of an treatment may of by with is a drug and combination of and the that is for use and has the potential to improve and The study is a multicenter, study to the safety and efficacy of on assessed by and in patients with PAH to adult patients with right 1 PAH on therapy are in A safety will to with the or the of treatment days and for and and cardiac in and from to on and in from to to on and The of the study are to the of 3 of University University University University University University University University University University USACategory: Areas: and with pulmonary hypertension (PH) is in PH after pulmonary there is a of PH after in a of patients.Methods: A female with hypertension and for with PH and right ventricular 1), and pulmonary multiple of underwent with of sildenafil months and was on PH One year had PH combination PH therapy and underwent pulmonary and 1 PH and of PH was to have arterial and on studies to a diagnosis of was with and by with substantial improvement in and function by PH after is to it may Clinical of PH after and for disease and the of to be with Clinical ScienceSelected Areas: Quality of Life, Therapeutic StrategiesBackground: is an of in for of pulmonary arterial hypertension This evaluated in pulmonary and hemodynamic in PAH patients on dual or right Of age was ± years and was 3 or 4 In the and mean was ± ± and ± at and decreased by to to and to With and at 5 a reduction for by to to and to for the and the may the greatest pulmonary No significant in pressure or were in was to to and to for the 3 in pulmonary with were on with with and The only were and in a No respiratory reduction in for and was The effective to be is for or PAH MS, of Medical Center, H. M. of USACategory: Clinical ScienceSelected Areas: and with of and on PH Quality of of the pulmonary arterial hypertension (PAH) the of at the of care and potential is of care at pulmonary hypertension (PH) and an were January and February A of in PAH and were and analyzed for to potential a PAH (Tables 1 and and demonstrated of and on treatment and and were to and Further by and were as to significant in care and patients the there was a for patients with to this for With the and the to by that it was for to have a PH and to care and were of the of in PAH patients. that in care to care and of NJ, NJ, NJ, NJ, NJ, USACategory: Clinical ScienceSelected Areas: Therapeutic StrategiesBackground: of in pulmonary arterial hypertension (PAH) is by a and treatment is a of a the and of and of adults of or (n = or (n = or multiple of days (n = or 4 3 days (n = or days (n = of the Of (n = a versus of and were most Of (n = had a versus of versus versus versus and versus were most were in and in with no or of had in multiple was and was was after a and after multiple was safe and in with a that were and with of and Critical Care Medicine, Medical of Medical of USACategory: Areas: or and and with Therapeutic StrategiesBackground: A with and for versus disease after a for underwent a and was to have pulmonary but right catheterization had demonstrated A with study demonstrated pulmonary hypertension with a of mm (Table was with riociguat and with which demonstrated after to riociguat (Table demonstrated estimated pulmonary systolic pressure 23 mm Hg is to an of pulmonary but on it be to Data on treatment of are Our demonstrated a 3 mm to be an of reduced pulmonary this the of and pulmonary disease at disease Our a for pulmonary on WhiteUniversity of Rochester, Rochester, NY, USAR. and de de de Clinical ScienceSelected Areas: Therapeutic StrategiesBackground: is approved to pulmonary arterial hypertension 3 to disease to Data are from who to an study after a clinical the study or at study visits at of and every 12 until or study included and Of in the and and data are in the increased from in with a in the study ± decreased in with in of with from the study to were most in patients with No safety were after had a significant in and reduced after had in efficacy after initiating data are with the and suggest that was effective in a of who had had a of Pediatric of Medicine, of Pediatric of Medicine, USACategory: Areas: and with has with pulmonary hypertension but it as an in with a Here, the of an with PH in the of after and of and of was only for and for a few Of was by multiple and at to had a care for respiratory and underwent of at of included started on index, and on significant and had of the pulmonary a with PH. Initial natriuretic peptide was to was and to the care for and catheterization mean pulmonary pressure mm pressure mm and pulmonary on and was from to sildenafil therapy and started on diuretics with of the for a study which with pressure therapy, studies were of for and by A in the the diagnosis of has to tadalafil as an and most shows improvement in right ventricular as is an with a of function in the or which and of respectively. The of PH in patients with is to and for has with of decreased is In it is the to of cardiac and increased cardiac The of PH and disease is and studies are this is the first with PH. patients are to PH to and there is improvement with of the have a have of PH on or that PH in was to the of including to therapy, and to This the of patients with this for the of PH as and comorbidities. the potential for and improvement of PH with PH be considered a with of Further studies are to the between the of Pulmonary Critical Care Medicine, FL, of Pulmonary Critical Care Medicine, FL, D. of Sleep Medicine, FL, of Pulmonary Critical Care Medicine, FL, of Sleep Medicine, FL, of Medicine, FL, USACategory: Clinical ScienceSelected Areas: or and of and on PH is a control to of use has for with and retention Our was to of and arterial a in patients with pulmonary arterial hypertension patients for 1 PAH and who a and with a between 2019 and 2020 before and after of a were patients underwent a and with a calculated The were ± days patients were at was between 1 versus ± versus ± P = and the were in 1 versus ± versus ± P = ± versus ± P = a had no on the arterial and to or in patients with a to be safe in PAH Health, Health, Health, USACategory: Clinical ScienceSelected Areas: Therapeutic StrategiesBackground: Pulmonary arterial hypertension (PAH) is a disease that to right One treatment for PAH is or therapies are with that in the to or by a pulmonary hypertension (PH) in the and is The of this improvement was to improve safety of a within the of patients with PAH an the PH identified the need to care are a is an This of the by and the PH is in clinical of or the is to In with Clinical and a was (Figure). the of an with an on medication The the is a the or and the PH be The be by the prior to the was by the PH to in the Center, Department of Medicine, and The has safety and be at PH Division of Medicine, Medical Center, The of The of The of The Division of Medicine, Medical Center, The Division of Medicine, Medical Center, The USACategory: Clinical ScienceSelected Areas: Databases and RegistriesBackground: is to treatment in pulmonary arterial hypertension (PAH) are and to a using a for PAH patients.Methods: a on were for to and for function and PAH patients that years or prior to 5 years left for analysis age = of analysis was used to a of from which were P < with long-term versus were a and between the selected and of was The the were to of the to the of PAH including and and and right ventricular 1 and a were to the first PAH including that may be to the PAH of The Division, Medical Center, The of The Division, Medical Center, The of The Division, Medical Center, The USACategory: Clinical ScienceSelected Areas: and with in pulmonary arterial hypertension (PAH) are in are in for the the between and with of these to a clinical using clinical data at from adult PAH and The data age = or and clinical with a the data as the and as the the studied in to by with were selected to a in the 1-year The to were based on clinical The an the of using the In the was to PAH the Health, Health, Health, Health, Health, Health, USACategory: Clinical ScienceSelected Areas: Databases and Registries, Therapeutic StrategiesBackground: to pulmonary arterial hypertension (PAH) patients the however, improve pulmonary and and the to clinical therapies which and with care a significant in medication The of this study is to the that visits have on to PAH identified patients who an PAH drug selexipag, or by a and patients who an PAH drug or by a using data from January 1, to A and medication with (Table From January to 2019, identified patients in the study and patients in the control patients in the study versus P < an medication versus P < and of versus P < in the control was versus P = in the control and to therapy in the first months following the index = P = Patients by had of Pulmonary and Critical Care, Department of Medicine, University of Pulmonary and Critical Care, Department of Medicine, University of University of University of Pulmonary and Critical Care, Department of Medicine, University USACategory: ScienceSelected Areas: or and the increased of pulmonary arterial hypertension (PAH) while in and female are data that of may be by in cardiovascular the of on the pulmonary are by in the of naïve and female will be used in the a of the of will be in and will be to for will be to by of the the of the the will be and and collected for and right ventricular The will be and in The left will be and in will be and with and data that had by control female and female The was to be in female control female and data demonstrated and in in This may suggest in from the of Pulmonary and Critical Care, Department of Medicine, University of Pulmonary and Critical Care, Department of Medicine, University of Pulmonary and Critical Care, Department of Medicine, University USACategory: Clinical ScienceSelected Areas: Databases and Registries, and with Pulmonary disease is a rare of pulmonary Most patients need as a that patients had pulmonary arterial hypertension (PAH) patients. there are no on of in this to between and PAH patients.Methods: This is a retrospective observational Patients with a diagnosis of and PAH underwent between and were identified from data were in mean ± data were in and and tests were to between was using the patients and PAH patients who underwent were Patients with had significant and mean pulmonary pressure and cardiac at were patients had on the versus P < The proportion of patients were in the versus P < The proportion of patients

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.