更新于：2024-11-01

Pulmonary arterial hypertension associated with congenital heart disease

与先天性心脏病相关的肺动脉高压

更新于：2024-11-01

基本信息

别名

PAH associated with congenital heart disease、Pulmonary arterial hypertension associated with congenital heart disease、Pulmonary arterial hypertension associated with congenital heart disease (disorder)

简介-

关联

项与与先天性心脏病相关的肺动脉高压相关的药物

Sildenaifl Citrate

枸橼酸西地那非

靶点

PDE5A

作用机制

PDE5A抑制剂

在研机构

原研机构

在研适应症

非在研适应症

最高研发阶段批准上市

首次获批国家/地区

美国

首次获批日期1998-03-27

项与与先天性心脏病相关的肺动脉高压相关的临床试验

CTR20243649 / RecruitingN/A

波生坦分散片人体生物等效性研究

本试验旨在研究单次空腹和餐后口服江苏天士力帝益药业有限公司研制、生产的波生坦分散片（32 mg（以C27H29N5O6S计））的药代动力学特征；以Actelion Pharmaceuticals Ltd持证、Patheon Inc.生产的波生坦分散片（Tracleer®，32 mg（以C27H29N5O6S计））为参比制剂，比较两制剂中药动学参数Cmax、AUC0-t、AUC0-∞，评价两制剂的人体生物等效性。

开始日期2024-09-18

申办/合作机构

江苏天士力帝益药业有限公司

ChiCTR2200065240 / Suspended早期临床1期IIT

A single center, open label randomized controlled study of inhaled nitric oxide (iNO) in children with pulmonary hypertension

开始日期2022-11-01

申办/合作机构-

NCT06196801 / RecruitingN/AIIT

Efficacy of Triple-Combination Therapy in Severe Pulmonary Arterial Hypertension Associated Congenital Heart Diseases

Congenital heart disease (CHD) is a leading cause of pulmonary arterial hypertension (PAH) worldwide. Treatment for PAH associated with CHD (PAH-CHD) depends on the defect's type, size, and hemodynamic impact. For those with CHD correction indications, early defect repair or interventional closure is crucial to prevent irreversible pulmonary vascular remodeling due to prolonged exposure to a left-to-right shunt.
Current guidelines recommend triple-combination therapy, including phosphodiesterase 5 inhibitors, endothelin receptor antagonist, and parenteral prostacyclin, for patients with intermediate-high or high risk. Recent studies suggest that patients with PAH-CHD and borderline hemodynamics might regain eligibility for surgery after targeted vasodilatory treatment. Consequently, early initiation of triple-combination therapy may be critical for severe PAH-CHD patients to restore their surgical or interventional closure eligibility. Therefore, we conducted this prospective study to assess the effectiveness of triple-combination therapy in severe PAH-CHD cases.

开始日期2022-06-17

申办/合作机构

广东省人民医院

100 项与与先天性心脏病相关的肺动脉高压相关的临床结果

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100 项与与先天性心脏病相关的肺动脉高压相关的转化医学

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0 项与与先天性心脏病相关的肺动脉高压相关的专利（医药）

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项与与先天性心脏病相关的肺动脉高压相关的文献（医药）

2024-07-01·Diagnostic and Interventional Imaging

4D flow cardiac MRI to assess pulmonary blood flow in patients with pulmonary arterial hypertension associated with congenital heart disease

Article

作者: Sitbon, Olivier ; Savale, Laurent ; Azarine, Arshid ; Humbert, Marc ; Valdeolmillos, Estibaliz ; Jais, Xavier ; Audié, Marion ; Montani, David ; Sakhi, Hichem ; Tortigue, Marine ; Hascoët, Sébastien ; Isorni, Marc-Antoine ; Lecerf, Florence

PURPOSEThe purpose of this study was to evaluate the accuracy of four-dimensional flow cardiac magnetic resonance imaging (4D flow MRI) compared to right heart catheterization in measuring pulmonary flow (Qp), systemic flow (Qs) and pulmonary-to-systemic flow ratio (Qp/Qs) in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD).MATERIALS AND METHODSThe study was registered on Clinical-trial.gov (NCT03928002). Sixty-four patients with PAH-CHD who underwent 4D flow MRI were included. There were 16 men and 48 women with a mean age of 45.3 ± 13.7 (standard deviation [SD]) years (age range: 21-77 years). Fifty patients (50/64; 78%) presented with pre-tricuspid shunt. Qp (L/min), Qs (L/min) and Qp/Qs were measured invasively using direct Fick method during right heart catheterization and compared with measurements assessed by 4D flow MRI within a 24-48-hour window.RESULTSThe average mean pulmonary artery pressure was 51 ± 17 (SD) mm Hg with median pulmonary vascular resistance of 8.8 Wood units (Q1, Q3: 5.3, 11.7). A strong linear correlation was found between Qp measurements obtained with 4D flow MRI and those obtained with the Fick method (r = 0.96; P < 0.001). Bland Altman analysis indicated a mean difference of 0.15 ± 0.48 (SD) L/min between Qp estimated by 4D flow MRI and by right heart catheterization. A strong correlation was found between Qs and Qp/Qs measured by 4D flow MRI and those obtained with the direct Fick method (r = 0.85 and r = 0.92; P < 0.001 for both).CONCLUSIONQp as measured by 4D flow MRI shows a strong correlation with measurements derived from the direct Fick method. Further investigation is needed to develop less complex and standardized methods for measuring essential PAH parameters, such as pulmonary arterial pressures and pulmonary vascular resistance.

2024-06-24·Korean circulation journal

A Single Center Experience of Pulmonary Arterial Hypertension Management in Korea: A 25-Year Comparative Analysis Following the Introduction of Targeted Therapy.

Article

作者: Song, Jinyoung ; Huh, June ; Jang, Shin Yi ; Cha, Ji Hyun ; Yang, Jeong Hoon ; Park, Taek Kyu ; Park, Seung Woo ; Chang, Sung-A ; Kim, Hojoong ; Kang, I-Seok ; Kim, Duk-Kyung

BACKGROUND AND OBJECTIVESThe transformation of pulmonary arterial hypertension (PAH) treatment in Korea, ushered by targeted therapy's advent, prompted our analysis of baseline attributes, treatment trends, and survival shifts within our single-center registry.METHODSWe examined 230 patients (72.6% female, mean age 40.6±17.4 years) diagnosed and/or treated between 1980 and 2021 in our PAH clinic. Given targeted therapy's introduction and active use since 2007, we compared diagnostic classification, demographics, and treatment patterns at that juncture. Survival analysis encompassed PAH types and the overall population. For historical survival comparison, 50 non-registry patients were retrospectively added, and age-sex matching enabled pooled analysis.RESULTSCongenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) constituted the largest subset (43.0%), trailed by connective tissue disease-associated PAH (CTD-PAH, 29.6%) and idiopathic PAH (IPAH, 19.1%). Post-2007, CTD-PAH proportions surged, notably with an elevated initiation rate of targeted therapy (95.4%). Overall survival rates at 1, 5, and 10 years stood at 91.3%, 77.4%, and 65.8%, respectively, with CHD-PAH exhibiting superior survival to idiopathic or CTD-PAH. Age-sex matching analysis indicated survival disparities between those starting immediate targeted therapy vs. conservative treatment upon diagnosis, especially driven by IPAH.CONCLUSIONSIn the post-introduction of the targeted therapy era, patients with PAH promptly started treatment right away, and higher survival rates of patients who started initial PAH-targeted therapy were demonstrated. The transition towards early treatment initiation might have likely contributed to the elevated survival rates observed in Korea's PAH patient cohort.

2024-04-01·Pulmonary Circulation

Left main coronary artery compression in precapillary pulmonary hypertension

Article

作者: Catana, Nicu ; Radu, Teodora ; Badea, Ruxandra ; Radu, Noela ; Deleanu, Dan ; Coman, Ioan M ; Enache, Roxana ; Olaru-Lego, Georgiana ; Iosifescu, Andrei George ; Platon, Pavel ; Popescu, Bogdan A ; Predescu, Lucian M

AbstractPulmonary hypertension (PH) is a progressive and invalidating condition despite available therapy. Addressing complications such as left main coronary artery compression (LMCo) due to the dilated pulmonary artery (PA) may improve symptoms and survival. Nevertheless, clear recommendations are lacking. The aim of this study is to analyze the prevalence, characteristics, predictive factors and impact of LMCo in a heterogenous precapillary PH population in a single referral center. Two hundred sixty‐five adults with various etiologies of precapillary PH at catheterization were reviewed. Coronary angiography (CA) was performed for LMCo suspicion. Revascularization was performed in selected cases. Outcomes were assessed at a mean follow‐up of 3.9 years. LMCo was suspected in 125 patients and confirmed in 39 (31.2%), of whom 21 (16.8%) had 50%–90% stenoses. Nine revascularizations were performed, with clinical improvement. The only periprocedural complication was a stent migration. LMCo was associated with PH etiology (p 0.003), occuring more frequently in congenital heart disease‐associated PH (61.5% of all LMCo cases, 66.6% of LMCo ≥ 50%). Predictors of LMCo ≥50% were PA ≥ 37.5 mm (Sn 81%, Sp 74%) and PA‐to‐aorta ≥1.24 (Sn 81%, Sp 69%), with increased discrimination when considering RV end‐diastolic area. LMCo ≥ 50% without revascularization presented clinical deterioration and worse survival (p 0.019). This analysis of a heterogeneous pre‐capillary PH population provides LMCo prevalence estimation, predictive factors (PA size, PA‐to‐aorta, RV end‐diastolic area and PH etiology) and long‐term impact. While LMCo impact on survival is inconclusive, untreated LMCo ≥ 50% has worse prognosis. LMCo revascularization may be performed safely and with good outcomes.