MiscellaneousSurvival of Japanese Patients With Idiopathic/Heritable Pulmonary Arterial Hypertension
Section snippets
Methods
We conducted a retrospective chart review of patients with I/HPAH. Patients were treated at 3 pulmonary hypertension centers in Japan (National Hospital Organization Okayama Medical Center, Kyorin University Hospital, and Keio University Hospital) between November 1992 and August 2012. Diagnosis was performed using a standard approach for the diagnosis of PAH including physical examination and right heart catheterization.7, 8 The study protocol was approved by the institutional review board of
Results
We conducted a retrospective chart review of 141 consecutive patients with I/HPAH. Patients' characteristics are listed in Table 1. Patients were predominantly women and in their 30s at diagnosis. At baseline, 86.5% of patients were in WHO functional class III or IV. Hemodynamic parameters were severely impaired, with mPAP >60 mm Hg and PVR >1,500 dyn⋅s/cm5.
Data of 130 patients who underwent follow-up right heart catheterization were collected. At follow-up, WHO functional class, 6MWD, and BNP
Discussion
This is the first multicenter report on survival of Japanese patients with I/HPAH treated at 3 referral centers. The results confirmed our previous report on the high survival rate of Japanese patients with I/HPAH and significant improvement in hemodynamic parameters after treatment.6 In patients receiving epoprostenol therapy, concomitant use of ERA was beneficial and warfarin was related to worse survival.
Survival of patients with PAH improved after progress in the use of PAH-targeted drugs,
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Outcome of mean pulmonary arterial pressure-based intensive treatment for patients with pulmonary arterial hypertension
2022, Journal of CardiologyCitation Excerpt :I/HPAH patients who achieved mPAP <42.5 mmHg using PAH-targeted drugs showed a 10-year survival rate of 100%. We also demonstrated that mPAP after treatment determined the prognosis of I/HPAH patients at three Japanese pulmonary hypertension centers [11]. Based on those results, Japanese Circulation Society 2017/Japanese Pulmonary Circulation and Pulmonary Hypertension Society 2017 guidelines recommend to treat PAH patients with mPAP ≥40 mmHg by parenteral prostacyclin (PGI2) [12].
Increased levels of platelet-derived microparticles in pulmonary hypertension
2020, Thrombosis ResearchCitation Excerpt :The use of anticoagulation in patients with pulmonary hypertension had been recommended based on small number of observational studies and was recently reconsidered, and has become controversial [23,24]. We previously reported that in patients with IPAH on epoprostenol therapy, anticoagulation resulted in a poor prognosis [25], which might be due to the inhibitory effect of epoprostenol on platelet function. PDMP in patients with PAH express high levels of soluble CD40 ligand, which is a proinflammatory mediator contained inside platelets and is expressed on the surface of activated platelets [16].
Age-related differences in hemodynamics and functional status in pulmonary arterial hypertension: Baseline results from the Pulmonary Hypertension Association Registry
2020, Journal of Heart and Lung TransplantationCitation Excerpt :On average, patients with incident PAH in PHAR were diagnosed with PAH at a mean cardiac index of 2.3 liters/min/m2 regardless of age. Prior registries have reported similar cardiac indices of incident patients (i.e., 2.1–2.4 liters/min/m2), suggesting that this level of cardiac dysfunction may be a threshold at which patients tend to present to care.6–8,28–30 The criteria for diagnosing PAH require mPAP > 20 mm Hg and PVR > 3 WU.31
Long-term efficacies of selective vasodilators in pulmonary arterial hypertension: a comprehensive comparison using a spontaneous reporting database
2024, Naunyn-Schmiedeberg's Archives of Pharmacology
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