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Late Breaking Abstract - Screening of pulmonary arterial hypertension in asymptomatic BMPR2 mutation carriers (DELPHI-2 Study) 摘要:无症状BMPR2突变携带者筛查肺动脉高压(DELPHI-2研究)
Right ventricle Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3951
D. Montani, B. Girerd, X. Jaïs, L. Savale, P. Laveneziana, A. Bouchachi, S. Hascoët, Sven Gunther, L. Godinas, F. Parent, C. Guignabert, P. Hervé, Gilles Garcia, G. Simonneau, O. Sitbon, M. Humbert
{"title":"Late Breaking Abstract - Screening of pulmonary arterial hypertension in asymptomatic BMPR2 mutation carriers (DELPHI-2 Study)","authors":"D. Montani, B. Girerd, X. Jaïs, L. Savale, P. Laveneziana, A. Bouchachi, S. Hascoët, Sven Gunther, L. Godinas, F. Parent, C. Guignabert, P. Hervé, Gilles Garcia, G. Simonneau, O. Sitbon, M. Humbert","doi":"10.1183/13993003.congress-2019.pa3951","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3951","url":null,"abstract":"Introduction: Heritable PAH due to BMPR2 mutation is an autosomal dominant disease with incomplete penetrance. To date, no strategy has proven its efficacy for early PAH screening in asymptomatic relatives carrying a BMPR2 mutation. Objectives: To evaluate efficacy of PAH screening in this high risk population. Methods: We prospectively followed for 2 years a cohort of asymptomatic subjects carrying a BMPR2 mutation (DELPHI-2 study NCT01600898), continued by an optional long-term extension. Initial and yearly assessment included NYHA functional class (FC), EKG, chest X-ray, 6-min walk distance (6MWD), pulmonary function tests, cardiopulmonary exercise testing, echocardiography and biological testing. Right heart catheterization (RHC) at rest and exercise was proposed at inclusion or at follow-up if PAH was suspected based on predefined criterion. Results: 55 adult subjects (26 males, 29 females) with a median age of 37 yo (min-max 18-78) were included. At inclusion, all subjects were in NYHA FC I with normal 6MWD (median 533 m, min-max 368-693) and normal NT-proBNP. No PAH was suspected based on echocardiography. All 55 subjects accepted RHC at inclusion allowing to identify 2 females (25 and 78yo) with mild PAH (mPAP 25 and 26 mmHg) and 12 subjects (22%) with exercice PH of unclear significance. At follow-up (>200 patient-year), two additional cases were diagnosed among the subjects with exercise PH. In all detected patients, early treatment maintained a low-risk profile with oral PAH therapy. Conclusion: Screening allowed early PAH diagnosis in asymptomatic BMPR2 mutation carriers. This study will describe early biomarkers of PAH in this population.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"5 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91234313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 3
Late Breaking Abstract - Safety and efficacy of B-cell depletion with rituximab for the treatment of systemic sclerosis-associated pulmonary arterial hypertension 摘要:b细胞去除联合利妥昔单抗治疗系统性硬化相关性肺动脉高压的安全性和有效性
Right ventricle Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.rct1884
R. Zamanian, D. Badesch, L. Chung, R. Domsic, T. Medsger, A. Pinckney, L. Keyes‐Elstein, Carla D’Aveta, M. Spychala, J. White, P. Hassoun, F. Torres, J. Molitor, D. Khanna, Holden Maeker, B. Welch, E. Goldmuntz, M. Nicolls
{"title":"Late Breaking Abstract - Safety and efficacy of B-cell depletion with rituximab for the treatment of systemic sclerosis-associated pulmonary arterial hypertension","authors":"R. Zamanian, D. Badesch, L. Chung, R. Domsic, T. Medsger, A. Pinckney, L. Keyes‐Elstein, Carla D’Aveta, M. Spychala, J. White, P. Hassoun, F. Torres, J. Molitor, D. Khanna, Holden Maeker, B. Welch, E. Goldmuntz, M. Nicolls","doi":"10.1183/13993003.congress-2019.rct1884","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.rct1884","url":null,"abstract":"Background: Pulmonary arterial hypertension (PAH) is a morbid complication of systemic sclerosis (SSc). Management of SSc-PAH remains challenging and mortality is high. The postulated role of B-cells in SSc suggests a role for B-cell depletion in SSc-PAH. Methods: We conducted an NIH-sponsored proof-of-concept multicenter, double-blind, randomized controlled trial of rituximab (Rx) in SSc-PAH on stable PAH therapy. Dosing was two 1000 mg infusions 14 days apart. The primary endpoint was change in 6-minute walk distance (6MWD) at 24 weeks. Secondary endpoints included change in 6MWD at other time points, pulmonary vascular resistance (PVR), time to clinical worsening (TTCW), diffusion capacity, and SSc-specific factors. Results: Between 2010–2018, 57 subjects with similar baseline clinical characteristics were randomized 1:1 to Rx or placebo (Pc). Rx was well tolerated without unexpected toxicities. In a longitudinal analysis using data through week 24, 6MWD trended towards improvement after Rx relative to Pc, but did not differ significantly between arms (23.6±11.1m Rx, 0.5±9.7m Pc, p=0.12). A pre-specified secondary analysis using data through week 48 demonstrated improvement at week 24 (25.5±8.8m Rx, 0.4±7.4m Pc, p=0.03) with a loss of effect by week 48 (9.5±12.4m Rx, -7.0±8.6m Pc, p=0.28). Higher proportion of Pc subjects required additional PAH therapies, but changes in PVR and TTCW were not significantly different. Conclusion: Adjunctive B-cell depletion therapy is a safe and potentially effective treatment for SSc-PAH. This is the first controlled trial examining the role of immunotherapy in a serious form of PAH and warrants further study.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78883432","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 6
Late Breaking Abstract - The effect of exercise rehabilitation on cardiac function, measured by cardiac MRI, in patients with pre-capillary pulmonary hypertension 摘要-运动康复对毛细血管前肺动脉高压患者心功能的影响,通过心脏MRI测量
Right ventricle Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3946
M. McGettrick, G. Jayasekera, M. Brewis, C. Church, Martin Johnson, A. MacKenzie
{"title":"Late Breaking Abstract - The effect of exercise rehabilitation on cardiac function, measured by cardiac MRI, in patients with pre-capillary pulmonary hypertension","authors":"M. McGettrick, G. Jayasekera, M. Brewis, C. Church, Martin Johnson, A. MacKenzie","doi":"10.1183/13993003.congress-2019.pa3946","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3946","url":null,"abstract":"Introduction: Exercise rehabilitation is effective and safe in patients with pre-capillary pulmonary hypertension (PH). It has been shown to improve exercise capacity and quality of life but there is limited information available on changes in cardiac function. We assessed the effect of exercise on cardiac function, measured by cardiac MRI (CMR), in a prospective study in the Scottish Pulmonary Vascular Unit. Methods: 26 patients with stable, optimally treated PH, were recruited to a two-phase rehabilitation trial: Phase 1: 3 weeks of supervised inpatient aerobic and resistance exercise Phase 2: 12 weeks of remotely supervised exercise, based on inpatient regime. CMR was obtained at baseline and 15 weeks. Image analysis was performed using Circle Cardiovascular Imaging, version 5.1. Results: Mean age 53 years, majority female (19/26). 62% Idiopathic Pulmonary Arterial Hypertension, 23% Connective Tissue Disease related PH, 8% Heritable, 8% Chronic Thromboembolic PH. Table 1 shows changes in cardiac indices. Conclusion: Exercise rehabilitation in pre-capillary PH led to significant improvements in cardiac output, stroke volume and right ventricular ejection fraction (EF). Small numbers limited results. Further work is required to assess long term benefits.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"9 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85637740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Late Breaking Abstract - Morphine for dyspnea in advanced pulmonary arterial hypertension 摘要:吗啡治疗晚期肺动脉高压患者呼吸困难
Right ventricle Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3948
D. Christiansen, K. Wentlandt, J. Lew, J. Granton
{"title":"Late Breaking Abstract - Morphine for dyspnea in advanced pulmonary arterial hypertension","authors":"D. Christiansen, K. Wentlandt, J. Lew, J. Granton","doi":"10.1183/13993003.congress-2019.pa3948","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3948","url":null,"abstract":"Background: Whether morphine is useful for improving dyspnea and exercise capacity in advanced pulmonary arterial hypertension (PAH) is unclear. Methods: Patients with PAH with WHO FC III symptoms despite therapy were enrolled in a randomized, cross-over study of oral morphine sulfate 5mg versus no drug prior to two six-minute walk tests (6MWT) performed within 7 days. The primary outcome was peak Borg dyspnea score. Safety outcomes were symptom scores and changes in vital signs. Data were analyzed using a paired t-test. Results: 12 patients were enrolled and 2 were withdrawn (one due to syncope on a control 6MWT and one due to intercurrent illness). Among those completing the study, median disease duration was 4.6 years and 90% were on dual or triple targeted PAH therapy. The peak Borg dyspnea score did not differ with morphine versus control (4.7 vs 5.0, p=0.34). Borg scores at 2, 4 and 6 minutes of the 6MWT were lower with morphine but did not reach statistical significance. The mean 6MWD was 349m with morphine versus 342m with control (p=0.38). No significant differences in clinically important symptoms or side effects were detected with morphine. Changes in systolic blood pressure response (+22 vs +11mmHg, p=0.18), heart rate response (+38 vs +35bpm, p=0.36), and nadir oxygen saturation (86 vs 86%, p=1) with exercise did not differ (morphine vs control). Conclusion: Oral morphine did not improve peak dyspnea scores or exercise capacity in patients with advanced PAH. The improvement in submaximal dyspnea scores with morphine did not achieve statistical significance. Limitations relate to small subject numbers and absence of blinding. Morphine was not associated with side effects or worsening of vital signs.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"130 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79604289","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Late Breaking Abstract - Effects of preventive exercise in monocrotaline rat model with pulmonary hypertension 摘要:预防性运动对肺动脉高压大鼠模型的影响
Right ventricle Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3945
F. Pacagnelli, T. B. Mariano, Ana Karênina Dias de Almeida Sabela, Raissa Dias, A. Freire, L. Junior, L. Schaffer, R. Carvalho, K. Okoshi
{"title":"Late Breaking Abstract - Effects of preventive exercise in monocrotaline rat model with pulmonary hypertension","authors":"F. Pacagnelli, T. B. Mariano, Ana Karênina Dias de Almeida Sabela, Raissa Dias, A. Freire, L. Junior, L. Schaffer, R. Carvalho, K. Okoshi","doi":"10.1183/13993003.congress-2019.pa3945","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3945","url":null,"abstract":"In patients with pulmonary hypertension(PH), right ventricular failure(RVF) is the main determinant of prognosis and a common cause of death. PH leads to compensated hypertrophy that progresses to decompensated RVF. RVF is accompanied by changes in the right ventricle(RV) and extracellular matrix(ECM). Preventive exercise has been shown to be a therapeutic approach in PH. We evaluated the influence of preventive exercise in ECM in the RV compensated hypertrophy in PH. We used male Wistar rats divided into 4 groups(n=8): Sedentary control(SC); Training control(TC); Sedentary PH(SPH); and Training PH(TPH). The rats performed the exercises for 13 weeks. PH was induced by monocrotaline(60mg/kg) during the 10th week of training, and the control rats received a saline solution. At the end of the 13th week, echocardiogram were performed, and the rats were euthanized. RV hypertrophy was assessed through histomorphometric analysis. Gene expression using RT-qPCR: colIa1(collagen type I alpha 1 chain); colIa2(collagen type I alpha 2 chain); coll3a1(collagen type III alpha 1 chain);and Mmp2 and 9(matrix metalloproteinase 2 and 9). Collagen content was assessed through picrosirius red. Our results showed increases in cardiomyocyte sectional area(SC vs. SPH,p=0.0001), peak flow velocity(SC vs. SPH,p=0.001), and gene expression of the colla1(SC vs. SPH,p=0.007). The preventive exercise normalized the peak flow velocity(SHP vs. TPH, p=0.001) and decreased the area of the cardiomyocytes(SHP vs. TPH,p=0.001). This occurred without changes in the ECM. Preventive exercise exerts a cardioprotective effect in compensated hypertrophy in monocrotaline rat model with PH(function and hypertrophy), without alterations in ECM.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"32 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75137927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Late Breaking Abstract - The effect of altering gut microbiota on the development of pulmonary hypertension 摘要-改变肠道微生物群对肺动脉高压发展的影响
Right ventricle Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3950
T. Jujo, N. Tanabe, Koji Hosomi, Hiroki Shoji, Jonguk Park, Yumiko Ikubo, Asako Yanagisawa, Takayuki Kobayashi, Keiko Yamamoto, H. Miwa, A. Naito, H. Kasai, Rika Suda, Ayumi Sekine, Rintaro Nishimura, T. Sugiura, A. Shigeta, S. Sakao, K. Mizuguchi, J. Kunisawa, K. Tatsumi
{"title":"Late Breaking Abstract - The effect of altering gut microbiota on the development of pulmonary hypertension","authors":"T. Jujo, N. Tanabe, Koji Hosomi, Hiroki Shoji, Jonguk Park, Yumiko Ikubo, Asako Yanagisawa, Takayuki Kobayashi, Keiko Yamamoto, H. Miwa, A. Naito, H. Kasai, Rika Suda, Ayumi Sekine, Rintaro Nishimura, T. Sugiura, A. Shigeta, S. Sakao, K. Mizuguchi, J. Kunisawa, K. Tatsumi","doi":"10.1183/13993003.congress-2019.pa3950","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3950","url":null,"abstract":"Background: Pulmonary arterial hypertension (PAH) is an intractable disease, the development of which may be related to the inflammatory system. Recently, abnormal characteristics of gut microbiota in a PAH rat model (Su/Hx) were reported, but it has not yet been clarified whether abnormality of microbiota is a cause or result of PAH, which is therefore the purpose of this study. Methods: Su/Hx was induced in Sprague Dawley rats by subcutaneous injection of the VEGF antagonist SU 5416 (30 mg/kg) and exposure to hypoxia (10% O2). Some Su/Hx rats were treated with antibiotics (ampicillin, vancomycin, neomycin, and metronidazole) (Su/Hx+ABx group). Control rats were kept in normoxia. Alteration of pulmonary vessels within the lungs was analyzed. Preliminarily, we also collected fecal samples of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Gut microbiomes were analyzed by 16S rRNA gene sequencing. Results: Right ventricular systolic pressure (RVSP) in control, Su/Hx, and Su/Hx+ABx groups was 27.8±5.1, 52.2±13.7, and 29.1±4.6 mmHg, respectively (control vs. Su/Hx: p=0.005; Su/Hx vs. Su/Hx+ABx: p=0.02; control vs. Su/Hx+ABx: p=1.0). The percentage of obstructed pulmonary arteries in the Su/Hx+ABx group was significantly lower than in the Su/Hx group (Su/Hx: 23.6±5.3; Su/Hx+ABx: 9.5±6.3; p=0.002). 16S rRNA gene sequence analysis revealed that the percentages of several bacterial species were significantly correlated with RVSP in the rats and the percentage of a bacterial species X was positively correlated with pulmonary vascular resistance (r=0.76, p=0.049) in CTEPH patients. In conclusion, altering gut microbiota could affect the development of pulmonary hypertension.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"834 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77546214","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Late Breaking Abstract - Inflammation and metabolic syndrome exacerbate pulmonary hypertension associated with left heart disease 摘要:炎症和代谢综合征加重左心相关肺动脉高压
Right ventricle Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3949
Benoît Ranchoux, V. Nadeau, S. Breuils-bonnet, J. Omura, M. André, J. Dupuis, P. Pibarot, R. Paulin, O. Boucherat, S. Provencher, S. Archer, S. Bonnet, F. Potus
{"title":"Late Breaking Abstract - Inflammation and metabolic syndrome exacerbate pulmonary hypertension associated with left heart disease","authors":"Benoît Ranchoux, V. Nadeau, S. Breuils-bonnet, J. Omura, M. André, J. Dupuis, P. Pibarot, R. Paulin, O. Boucherat, S. Provencher, S. Archer, S. Bonnet, F. Potus","doi":"10.1183/13993003.congress-2019.pa3949","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3949","url":null,"abstract":"","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"88 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76343360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Late Breaking Abstract - Supplementation of iron in pulmonary hypertension (SIPHON): results from a randomised controlled crossover trial 摘要:肺动脉高压患者补充铁(SIPHON):一项随机对照交叉试验的结果
Right ventricle Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3947
L. Howard, Jianguo He, J. Wharton, G. Watson, H. Gall, H. Ghofrani, Li Huang, D. Kiely, Q. Luo, K. Sheares, Lan Zhao, M. Wilkins
{"title":"Late Breaking Abstract - Supplementation of iron in pulmonary hypertension (SIPHON): results from a randomised controlled crossover trial","authors":"L. Howard, Jianguo He, J. Wharton, G. Watson, H. Gall, H. Ghofrani, Li Huang, D. Kiely, Q. Luo, K. Sheares, Lan Zhao, M. Wilkins","doi":"10.1183/13993003.congress-2019.pa3947","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3947","url":null,"abstract":"Background: Iron deficiency in the absence of anaemia is common in patients with idiopathic pulmonary arterial hypertension (IPAH). It has been associated with inappropriately raised levels of hepcidin, the key regulator of iron homeostasis and correlates with disease severity and worse clinical outcomes. Oral iron absorption may be impeded by elevated hepcidin levels. The safety and benefits of parenteral iron replacement in IPAH are unknown. Methods: Iron-deficient patients (defined as ferritin 28.1nmol/L) were randomised to receive a single infusion of ferric carboxymaltose (FCM) 15 mg/kg up to 1000mg or saline placebo with cross-over after 12 weeks of treatment. The primary outcome was change in endurance time on cardio-pulmonary exercise testing (CPET) at 12 weeks. Secondary measures included resting haemodynamics, incremental CPET, iron indices, 6 minute walk distance, WHO functional class, quality of life score, NT-proBNP and cardiac anatomy and function from MRI. Results: 40 patients enrolled and 39 provided data for analysis. FCM improved iron indices, with a significant increase in ferritin (P=0.0003) and reduction in soluble transferrin receptor levels (P Conclusion: Iron repletion through a single infusion of FCM improves iron indices and is well tolerated but does not improve exercise capacity in PAH.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"63 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79336568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Young males with pulmonary arterial hypertension have a high afterload and low right ventricular ejection fraction at presentation 年轻男性肺动脉高压患者在发病时具有高后负荷和低右心室射血分数
Right ventricle Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.OA1637
J. Groeneveldt, C. V. D. Bruggen, P. Trip, N. Westerhof, B. Westerhof, Anton Vonk-Noordergraaf, F. S. D. Man
{"title":"Young males with pulmonary arterial hypertension have a high afterload and low right ventricular ejection fraction at presentation","authors":"J. Groeneveldt, C. V. D. Bruggen, P. Trip, N. Westerhof, B. Westerhof, Anton Vonk-Noordergraaf, F. S. D. Man","doi":"10.1183/13993003.CONGRESS-2018.OA1637","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.OA1637","url":null,"abstract":"Background: Gender is known to play a role in right ventricular (RV) adaption in pulmonary arterial hypertension (PAH). Sex hormone levels change with aging both in males and females. However effect of aging on RV function within sexes has not been explored. Aim: To study the effect of age on PAH disease severity and RV adaptation in both sexes. Methods: Systolic elastance (Ees), arterial elastance (Ea), diastolic elastance (Eed) and RV-arterial coupling (Ees/Ea) were determined in 88 newly diagnosed, treatment naive, idiopathic and hereditary PAH patients using the single-beat pressure volume analysis. Right ventricular ejection fraction (RVEF) was determined by CMR. Both males and females were divided in an age ≤50 and age > 50 years group, as 50 years is the mean age women reach the menopause. Results: Results are presented in figure 1. Ees (p=0.56), Ea (p=0.40), Ees/Ea (p=0.87) or Eed (p=0.67) were not related to age in women. However, young men presented with a higher afterload (p =0.0006) when compared to men>50 years old. In addition, baseline RVEF was lower in young men compared to men>50 years (21%±11.7 vs. 35%±12.4, p=0.0431). Conclusion: Young males with PAH presented with a high afterload and low RVEF. Further research in a larger cohort is warranted to elucidate whether this is a consequence of a distinct disease phenotype.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2018-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80028980","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Outcomes of surgical treatment of VSD with pulmonary hypertension 室间隔缺损合并肺动脉高压的手术治疗效果
Right ventricle Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.OA1638
Sheenam Walia, G. Puri, K. Gandhi, M. Ganesh, B. Mandal
{"title":"Outcomes of surgical treatment of VSD with pulmonary hypertension","authors":"Sheenam Walia, G. Puri, K. Gandhi, M. Ganesh, B. Mandal","doi":"10.1183/13993003.CONGRESS-2018.OA1638","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.OA1638","url":null,"abstract":"Introduction: High pulmonary pressure in uncorrected left-to-right shunt causes progressive rise in pulmonary vascular resistance and increased perioperative mortality. Aims and objectives: To assess the incidence of pulmonary hypertension (PH) in VSD patients and associated post-operative outcomes in children of age group 0-14 years. Methods: Retrospective, observational, hospital data based analysis of children ≤ 14 years undergoing cardiac surgery. Exclusion criterion was inoperability secondary to irreversibility of PH. Results: Conclusions: Presence of PH in cases of acyanotic CHD posted for surgery is associated with increased adverse perioperative outcomes and mortality. Early surgical closure prior to development of irreversible PH would be desirable.","PeriodicalId":21522,"journal":{"name":"Right ventricle","volume":"17 10","pages":""},"PeriodicalIF":0.0,"publicationDate":"2018-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91442839","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
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