著者

Yuichi Tamura Hiraku Kumamaru Shiori Nishimura Yasuo Nakajima Hiromi Matsubara Yu Taniguchi Ichizo Tsujino Ayako Shigeta Koichiro Kinugawa Kazuhiro Kimura Koichiro Tatsumi

出版者

International Heart Journal Association

雑誌

International Heart Journal (ISSN:13492365)

巻号頁・発行日

vol.64, no.4, pp.684-692, 2023-07-29 (Released:2023-07-29)

参考文献数

27

被引用文献数

2

---

Upfront combination therapy including intravenous prostaglandin I2 (PGI2-IV) is recognized as the most appropriate treatment for patients with severe pulmonary arterial hypertension (PAH). This retrospective study aimed to determine reasons why this therapy is not used for some patients with severe PAH and describe the hemodynamic and clinical prognoses of patients receiving initial combination treatment with (PGI2-IV+) or without (PGI2-IV−) PGI2-IV.Data for patients with severe PAH (World Health Organization Functional Class III/IV and mean pulmonary arterial pressure [mPAP] ≥ 40 mmHg) were extracted from the Japan Pulmonary Hypertension Registry. Overall, 73 patients were included (PGI2-IV + n = 17; PGI2-IV− n = 56). The PGI2-IV+ cohort was younger than the PGI2-IV− cohort (33.8 ± 10.6 versus 52.6 ± 18.2 years) and had higher mPAP (58.1 ± 12.9 versus 51.8 ± 9.0 mmHg), greater prevalence of idiopathic PAH (88% versus 32%), and less prevalence of connective tissue disease-associated PAH (0% versus 29%). Hemodynamic measures, including mPAP, showed improvement in both cohorts (post-treatment median [interquartile range] 38.5 [17.0-40.0] for the PGI2-IV + cohort and 33.0 [25.0-43.0] mmHg for the PGI2-IV − cohort). Deaths (8/56) and lung transplantation (1/56) occurred only in the PGI2-IV − cohort.These Japanese registry data indicate that older age, lower mPAP, and non-idiopathic PAH may influence clinicians against using upfront combination therapy including PGI2-IV for patients with severe PAH. Early combination therapy including PGI2-IV was associated with improved hemodynamics from baseline, but interpretation is limited by the small sample size.

著者

Iwao Shimomura Mitsuhiro Abe Yu Li Kenji Tsushima Seiichiro Sakao Nobuhiro Tanabe Masatomi Ikusaka Koichiro Tatsumi

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

vol.58, no.7, pp.965-968, 2019-04-01 (Released:2019-04-01)

参考文献数

15

被引用文献数

7 8

---

The patient was a 71-year-old man with severe idiopathic pulmonary fibrosis (IPF) and who demonstrated a slow deterioration of his respiratory condition. After nintedanib administration, his forced vital capacity and chest high-resolution computed tomography (HRCT) findings were stable, but his dyspnea on exertion were worsened. He was diagnosed with pulmonary hypertension (PH) by right heart catheterization (mean pulmonary arterial pressure: 30 mmHg). In this case, we suspected that nintedanib caused his PH, as his IPF had not progressed.

著者

Fumiaki Kato Nobuhiro Tanabe Keiichi Ishida Rika Suda Ayumi Sekine Rintaro Nishimura Takayuki Jujo Toshihiko Sugiura Seiichiro Sakao Koichiro Tatsumi

出版者

日本循環器学会

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-15-1208, (Released:2016-02-16)

参考文献数

21

被引用文献数

1 7

---

Background:The postoperative changes in the coagulation-fibrinolysis system and the association between the system and postoperative course of patients with chronic thromboembolic pulmonary hypertension (CTEPH) who have undergone pulmonary endarterectomy (PEA) remain unclear.Methods and Results:Between 1986 and 2013, 117 patients (55.1±11.2 years, preoperative mean pulmonary arterial pressure 46.5±10.5 mmHg) underwent PEA, and 15 patients died during the perioperative period. We studied the association between the preoperative coagulation-fibrinolysis markers and surgical outcomes of all patients, and the long-term outcomes of the 102 survivors from the date of PEA. We also investigated the postoperative changes in coagulation-fibrinolysis markers and their association with residual pulmonary hypertension (PH) in 20 consecutive patients. Only an elevated factor VIII level was associated with perioperative death. Thrombomodulin and plasminogen values were significantly increased after PEA. Univariate logistic regression analysis revealed that D-dimer positivity at follow-up was a risk factor for residual PH. Patients with both an elevated fibrinogen level (≥291 mg/dl [median]) and decreased plasminogen activity (<100% [median]) had significantly worse disease-specific survival than the other patients (5-year disease-specific survival: 84.0% vs. 100%, respectively; P=0.0041 [log-rank test]).Conclusions:Preoperatively high fibrinogen and low plasminogen values in patients with CTEPH are associated with poor long-term postoperative outcome. PEA benefited not only the pulmonary hemodynamics but also the coagulation-fibrinolysis system of patients.

著者

Hajime Kasai Akane Matsumura Toshihiko Sugiura Ayako Shigeta Nobuhiro Tanabe Keiko Yamamoto Hideki Miwa Ryogo Ema Seiichiro Sakao Koichiro Tatsumi

出版者

日本循環器学会

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-15-1080, (Released:2016-03-11)

参考文献数

15

被引用文献数

2 8

---

Background:Mean pulmonary arterial pressure (MPAP) is an important pulmonary hemodynamic parameter used in the management of patients with chronic thromboembolic pulmonary hypertension (CTEPH). We compared echocardiography-derived estimates of MPAP with right heart catheterization (RHC) to identify reliable noninvasive methods of estimating MPAP-derived RHC (MPAPRHC) in these patients.Methods and Results:Echocardiography and RHC were performed in 56 patients with CTEPH (60.5±12.0 years; 44 females). We measured the tricuspid regurgitation (TR) pressure gradient (TRPG) using echocardiography. The mean systolic right ventricular (RV)-right atrial (RA) gradient was calculated by tracing the TR time velocity flow. Systolic and mean pulmonary artery pressures (SPAPTRand MPAPTR) estimated from TRPG and mean systolic RV-RA gradient were calculated by adding RA pressure based on the inferior vena cava. MPAPChemlawas calculated using Chemla’s formula: 0.61×SPAPTR+2 mmHg. MPAPRHCand pulmonary vascular resistance were 35.9±11.3 mmHg and 6.6±3.6 Wood units, respectively. The mean difference from MPAPRHCand limits of agreement were −1.5 mmHg and −19.6 to 16.5 mmHg for MPAPTR, and −4.6 mmHg and −24.5 to 15.2 mmHg for MPAPChemla. Accuracy within 10 mmHg and 5 mmHg of MPAPRHCwas 80.4% and 46.4% for MPAPTR, and 71.4% and 48.2% for MPAPChemla, respectively.Conclusions:MPAPTRand MPAPChemlaare reliable estimates for MPAPRHCin patients with CTEPH.

著者

Yudai Tamura Yuichi Tamura Yu Taniguchi Ichizo Tsujino Takumi Inami Hiromi Matsubara Ayako Shigeta Yoichi Sugiyama Shiro Adachi Kohtaro Abe Yuichi Baba Masaru Hatano Satoshi Ikeda Kenya Kusunose Koichiro Sugimura Soichiro Usui Yasuchika Takeishi Kaoru Dohi Saki Hasegawa-Tamba Koshin Horimoto Noriko Kikuchi Hiraku Kumamaru Koichiro Tatsumi on behalf of the Japan Pulmonary Hypertension Registry Network

出版者

The Japanese Circulation Society

雑誌

Circulation Reports (ISSN:24340790)

巻号頁・発行日

pp.CR-22-0098, (Released:2022-10-08)

参考文献数

21

被引用文献数

3

---

Background: Portopulmonary hypertension (PoPH) is one of the major underlying causes of pulmonary arterial hypertension (PAH). However, PoPH, especially treatment strategies, has been poorly studied. Therefore, this study evaluated current treatments for PoPH, their efficacy, and clinical outcomes of patients with PoPH.Methods and Results: Clinical data were collected for patients with PoPH who were enrolled in the Japan Pulmonary Hypertension Registry between 2008 and 2021. Hemodynamic changes, functional class, and clinical outcomes were compared between patients with PoPH treated with monotherapy and those treated with combination therapies. Clinical data were analyzed for 62 patients with PoPH, including 25 treatment-naïve patients, from 21 centers in Japan. In more than half the patients, PAH-specific therapy improved the New York Heart Association functional class by at least one class. The 3- and 5-year survival rates of these patients were 88.5% (95% confidence interval [CI] 76.0–94.7) and 80.2% (95% CI 64.8–89.3), respectively. Forty-one (66.1%) patients received combination therapy. Compared with patients who had received monotherapy, the mean pulmonary arterial pressure, pulmonary vascular resistance, and cardiac index were significantly improved in patients who had undergone combination therapies.Conclusions: Combination therapy was commonly used in patients with PoPH with a favorable prognosis. Combination therapies resulted in significant hemodynamic improvement without an increased risk of side effects.