Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan

Ogawa, Aiko; Ejiri, Kentaro; Matsubara, Hiromi

doi:10.1016/j.lfs.2014.01.077

Cited by 67 publications

(69 citation statements)

References 19 publications

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“…Nowadays, considering the fact that in real-life population, access for LTx is still limited as well as the long-term survival after LTx has not improved much, being still not satisfactory [19], there is a strong need for alternative strategy to prolong patient's life. Outstanding outcome in a population of patients with IPAH and heritable PAH (HPAH) treated with high doses of epoprostenol, either as monotherapy or in combination with phosphodiesterase inhibitors and/or endothelin-receptor antagonists gives some hope for the future [26]. In this patients' cohort, decrease in PAP was the independent determinant of more than ten years survival.…”

Section: Treatment Goals Of Pahmentioning

confidence: 84%

“…The risk factors for mortality in univariate analysis are B-type natriuretic peptide (BNP), RAP, and arterial blood saturation (SpO 2 ) [26]. Patients with a BNP level ≥ 350 pg/mL and SpO 2 ≤ 96% at baseline had a significantly worse prognosis.…”

Section: Lowering Pap To Improve Survivalmentioning

confidence: 99%

“…Patients with a BNP level ≥ 350 pg/mL and SpO 2 ≤ 96% at baseline had a significantly worse prognosis. Also, the cut-off value for RAP of 10 mmHg in receiver operating characteristic (ROC) curves analysis is of importance but it cannot be used as a predictor of prognosis [26]. All these data suggest, that patients with IPAH/HPAH cannot be treated successfully after development of severe heart failure, even at a referral center and with a use of parenteral prostanoids.…”

Section: Lowering Pap To Improve Survivalmentioning

confidence: 99%

“…All these data suggest, that patients with IPAH/HPAH cannot be treated successfully after development of severe heart failure, even at a referral center and with a use of parenteral prostanoids. Regarding the treatment effect, a decrease in mean PAP (mPAP) below 42.5 mmHg during treatment is a most important determinant of good prognosis in epoprostenol treated individuals [26]. In a population treated to achieve as low PAP as possible, unexpectedly good long-term survival of IPAH/HPAH patients were observed.…”

Section: Lowering Pap To Improve Survivalmentioning

confidence: 99%

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Pulmonary artery pressure matters – how to efficiently improve survival in pulmonary arterial hypertension (RCD code: II‐1A.1)

Matsubara

Błaszczak

Podolec

et al. 2017

Journal of Rare Cardiovascular Diseases

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Please cite this article: Matsubara H, Blaszczak P, Podolec P, et al. Pulmonary artery pressure matters -how to efficiently improve survival in pulmonary arterial hypertension. AbstractPulmonary arterial hypertension is a disease characterized by poor prognosis despite treatment. Even in a modern era of pharmacotherapy there is a strong need to further improve survival of patients. The current therapeutic strategies do not offer a real break-through in terms of reducing mortality. In a search for better efficacy of treatment we discuss a strategy based on lowering pulmonary artery pressure as much as possible with epoprostenol in monotherapy or in combination. Epoprostenol with bosentan is an effective tool in achieving this goal. A rapid up-titration regardless of maximum epoprostenol dose achieved gives additional long-term benefit. Practical issues related to such modality of treatment are also discussed. JRCD 2017; 3 (4): 110-115

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Section: Treatment Goals Of Pahmentioning

confidence: 84%

Section: Lowering Pap To Improve Survivalmentioning

confidence: 99%

Section: Lowering Pap To Improve Survivalmentioning

confidence: 99%

Section: Lowering Pap To Improve Survivalmentioning

confidence: 99%

See 2 more Smart Citations

Pulmonary artery pressure matters – how to efficiently improve survival in pulmonary arterial hypertension (RCD code: II‐1A.1)

Matsubara

Błaszczak

Podolec

et al. 2017

Journal of Rare Cardiovascular Diseases

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“…2,3) Additionally, the therapeutic outcome of PH has notably improved, 2,3) and long-term survival can be expected. 4) However, PH is still a life-threatening disease.…”

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confidence: 99%

Impairment of Iodine-123-Metaiodobenzylguanidine (<sup>123</sup>I-MIBG) Uptake in Patients with Pulmonary Artery Hypertension

et al. 2018

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SummaryAccording to recent studies, lung uptake of iodine-123-metaiodobenzylguanidine ( 123 I-MIBG) is impaired in many lung diseases and low lung uptake of 123 I-MIBG suggests endothelial dysfunction of the pulmonary artery. The 21 patients with PH were categorized into those with pulmonary artery hypertension (PAH, n = 12) and those with chronic thromboembolic pulmonary hypertension (CTEPH, n = 9). The mean pulmonary artery pressure was not significantly different between patients with CTEPH and PAH (37.7 ± 6.8 versus 32.3 ± 5.3 mmHg respectively; P = 0.054). There were no significant differences in any other hemodynamic parameters between the two groups. The lung uptake of 123 I-MIBG in PAH patients (early image: 1.54 ± 0.18, delayed image: 1.41 ± 0.16) was significantly lower than that of CTEPH patients (early image: 2.17 ± 0.25, P < 0.0001; delayed image: 1.99 ± 0.20, P = 0.0001, adjusted for age and World Health Organization classification) and controls (early image: 2.32 ± 0.27, P = 0.0007; delayed image: 1.92 ± 0.19, P = 0.0007). In conclusion, we found for the first time that the lung uptake of 123 I-MIBG in patients with PAH is lower than that in patients with CTEPH and controls.(Int Heart J 2018; 59: 112-119)

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Inhaled iloprost induces long‐term beneficial hemodynamic changes in patients with pulmonary arterial hypertension receiving combination therapy

et al. 2022

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Inhaled iloprost is an established treatment for pulmonary arterial hypertension (PAH). However, the long-term hemodynamic changes that inhaled iloprost induces are unclear. Here, we retrospectively enrolled 18 patients with PAH who received inhaled iloprost as add-on to oral combination therapy from December 2016 to January 2021 at our institute in Japan. We then examined the changes in hemodynamic parameters induced by iloprost in these patients during right heart catheterization (RHC). To examine the long-term effects of iloprost, we repeated the RHC examination at follow-up (median time to follow-up, 8.5 months). During both catheterization procedures, iloprost was administered by using an Ineb AAD system (Philips NV). In a comparison of pre-inhalation values at the first and follow-up RHCs, inhaled iloprost significantly improved mean pulmonary artery pressure (mPAP; 39.9 ± 7.8 to 32.5 ± 7.2 mmHg, p = 0.016) and pulmonary vascular resistance (PVR; 588.5 ± 191.7 to 464.4 ± 188.5 dyn s cm −5 , p = 0.047). During the follow-up RHC, in a comparison of the pre-inhalation and best recorded values out to 30 min after the end of iloprost inhalation, iloprost significantly decreased mPAP (32.5 ± 7.2 to 30.0 ± 6.6 mmHg, p = 0.007) and PVR (457.8 ± 181.4 to 386.2 ± 142.8 dyn s cm −5 , p = 0.025) and significantly increased cardiac output (4.19 ± 0.91 to 4.64 ± 1.01 L/min, p = 0.035). Iloprost may have not only acute vasodilation effects but also long-term hemodynamic benefits in PAH patients receiving combination therapy.

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Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan

Abstract: The study revealed a long-term survival of Japanese patients with I/HPAH. Hemodynamic parameters improved significantly after treatment, which might be related to high prescription rates of PAH-targeted drugs. Multicenter studies are needed to reveal the prognostic factors for I/HPAH.

Cited by 67 publications

References 19 publications

Pulmonary artery pressure matters – how to efficiently improve survival in pulmonary arterial hypertension (RCD code: II‐1A.1)

Pulmonary artery pressure matters – how to efficiently improve survival in pulmonary arterial hypertension (RCD code: II‐1A.1)

Impairment of Iodine-123-Metaiodobenzylguanidine (<sup>123</sup>I-MIBG) Uptake in Patients with Pulmonary Artery Hypertension

Inhaled iloprost induces long‐term beneficial hemodynamic changes in patients with pulmonary arterial hypertension receiving combination therapy

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