Characteristics, goal‐oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry

Quan, Ruilin; Zhang, Gangcheng; Yu, Zaixin; Zhang, Caojin; Yang, Zhenwen; Tian, Hui; Yang, Yuanhua; Wang, Weifeng; Chen, Yu‐Cheng; Wang, Kun; Xianyang, Zhu; Li, Shengqing; Shen, Jieyan; Zheng, Zeqi; Zhu, Xiaoyun; Wang, Guangyi; Wang, Qian; Zhou, Daxin; Ji, Yingqun; Yang, Tao; Li, Wen; Chen, Xiaoxi; Qian, Yuling; Lin, Yangyi; Gu, Qing; Xiong, Changming; Shan, Guangliang; He, Jian‐Guo

doi:10.1111/resp.14247

Cited by 20 publications

(15 citation statements)

References 25 publications

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“…The inclusion of these parameters in risk stratification may be due to the extensive studies on the prognostic value of these parameters, and stresses their important prognostic abilities in adult PAH patients. Based on the comparable predictive strength of non-invasive models and models including invasive parameters reported in three studies ( 19 , 45 , 46 ), a fully non-invasive risk stratification may be feasible. However, data supporting fully non-invasive risk stratification models are still scarce.…”

Section: Discussionmentioning

confidence: 99%

Risk stratification in adult and pediatric pulmonary arterial hypertension: A systematic review

Lokhorst

Werf

Berger

et al. 2022

Front. Cardiovasc. Med.

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IntroductionCurrently, risk stratification is the cornerstone of determining treatment strategy for patients with pulmonary arterial hypertension (PAH). Since the 2015 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines for the diagnosis and treatment of pulmonary hypertension recommended risk assessment, the number of studies reporting risk stratification has considerably increased. This systematic review aims to report and compare the variables and prognostic value of the various risk stratification models for outcome prediction in adult and pediatric PAH.MethodsA systematic search with terms related to PAH, pediatric pulmonary hypertension, and risk stratification was performed through databases PubMed, EMBASE, and Web of Science up to June 8, 2022. Observational studies and clinical trials on risk stratification in adult and pediatric PAH were included, excluding case reports/series, guidelines, and reviews. Risk of bias was assessed using the Prediction model Risk Of Bias Assessment Tool. Data on the variables used in the models and the predictive strength of the models given by c-statistic were extracted from eligible studies.ResultsA total of 74 studies were eligible for inclusion, with this review focusing on model development (n = 21), model validation (n = 13), and model enhancement (n = 9). The variables used most often in current risk stratification models were the non-invasive WHO functional class, 6-minute walk distance and BNP/NT-proBNP, and the invasive mean right atrial pressure, cardiac index and mixed venous oxygen saturation. C-statistics of current risk stratification models range from 0.56 to 0.83 in adults and from 0.69 to 0.78 in children (only two studies available). Risk stratification models focusing solely on echocardiographic parameters or biomarkers have also been reported.ConclusionStudies reporting risk stratification in pediatric PAH are scarce. This systematic review provides an overview of current data on risk stratification models and its value for guiding treatment strategies in PAH.Systematic review registration[https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022316885], identifier [CRD42022316885].

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Section: Discussionmentioning

confidence: 99%

Risk stratification in adult and pediatric pulmonary arterial hypertension: A systematic review

Lokhorst

Werf

Berger

et al. 2022

Front. Cardiovasc. Med.

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“…По данным российских и международных регистров, до 70% пациентов на момент постановки диагноза имеют III ФК по ВОЗ и соответствуют промежуточному риску развития летального исхода [6,[18][19][20]. Сроки назначения ЛАГ-специфической терапии с момента верификации диагноза и ее объем напрямую ассоциируются с характером течения ЛАГ и прогнозом пациентов [21,22].…”

Section: цели терапии пациентов с лагunclassified

Combination therapy of newly diagnosed intermediate-risk pulmonary arterial hypertension: A review

2022

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Advancements in the specific therapy of pulmonary arterial hypertension (PAH) and new combination therapy strategies and escalation treatment over the past decade have significantly improved disease control, slowed its progression, and improved quality of life and patient survival. This review provides a detailed discussion of current strategies for PAH-specific therapy available in the Russian Federation for patients with classical and non-classical PAH who fail to achieve treatment goals and remain at intermediate risk of disease progression/mortality.

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“…Details for the inclusion and exclusion criteria are illustrated in our previous report. 23 The study protocol, which was approved by the Institutional Review Board (IRB) of Fuwai Hospital (Approval No. 2009-208), complies with the Declaration of Helsinki and is registered on ClinicalTrials.gov (Identifier: NCT01417338).…”

Section: Study Design and Participantsmentioning

confidence: 99%

Incorporation of noninvasive assessments in risk prediction for pulmonary arterial hypertension

et al. 2022

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Risk assessment for pulmonary arterial hypertension (PAH) utilizing noninvasive prognostic variables could be more practical in real-world scenarios, especially at follow-up reevaluations. Patients who underwent comprehensive evaluations both at baseline and at follow-up visits were enrolled. The primary endpoint was all-cause mortality. Predictive variables identified by Cox analyses were further incorporated with the French noninvasive risk prediction approach. A total of 580 PAH patients were enrolled. During a median follow-up time of 47.0 months, 112 patients (19.3%) died. By multivariate Cox analyses, tricuspid annular plane systolic excursion (TAPSE), TAPSE/pulmonary arterial systolic pressure (PASP), and cardiopulmonary exercise testing-derived peak oxygen consumption (VO 2 ) remained independent predictors for survival. Regarding the French noninvasive risk prediction method, substituting N-terminal pro-b-type natriuretic peptide (NT-proBNP) with the newly derived low-risk criteria of a TAPSE ≥ 17 mm or a TAPSE/PASP > 0.17 mm/mmHg, or alternating 6-min walking distance with a peak VO 2 ≥ 44 %predicted retained the discrimination power. When recombining the low-risk criteria, the combination of World Health Organization functional class (WHO FC), TAPSE and peak VO 2 at baseline, and the combination of WHO FC, NT-proBNP, and peak VO 2 at follow-up showed better discriminative ability than the other combinations. In

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Characteristics, goal‐oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry

Cited by 20 publications

References 25 publications

Risk stratification in adult and pediatric pulmonary arterial hypertension: A systematic review

Risk stratification in adult and pediatric pulmonary arterial hypertension: A systematic review

Combination therapy of newly diagnosed intermediate-risk pulmonary arterial hypertension: A review

Incorporation of noninvasive assessments in risk prediction for pulmonary arterial hypertension

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