Long-Term Outcome of Chronic Thromboembolic Pulmonary Hypertension at a Single Japanese Pulmonary Endarterectomy Center

Miwa, Hideki; Tanabe, Nobuhiro; Jujo, Takayuki; Kato, Fumiki; Anazawa, Rie; Yamamoto, Keiko; Naito, Akira; Kasai, Hajime; Nishimura, Rintaro; Suda, Rika; Sugiura, Toshihiko; Sakao, Seiichiro; Ishida, Keiichi; Masuda, Masahisa

doi:10.1253/circj.cj-17-1242

Cited by 22 publications

(28 citation statements)

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“…22 Our group has recently identified that a Jamieson classification of III or IV and a high PVR were associated with poor survival, although similar survival rates were recently observed in patients with CTEPH treated by BPA, PEA, and medication between 2009 and 2016. 23 The findings of the present study are similar in that a Jamieson classification of III or IV was a poor prognostic factor, providing evidence that having a PD may be an additional important risk factor for perioperative death in patients with CTEPH.…”

Section: Discussionsupporting

confidence: 80%

Clinical characteristics and prognosis in patients with chronic thromboembolic pulmonary hypertension and a concomitant psychiatric disorder

et al. 2019

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Chronic thromboembolic pulmonary hypertension (CTEPH) can cause right heart failure. A concomitant psychiatric disorder (PD) is thought to increase the risk of acute pulmonary thromboembolism; however, whether PDs are associated with deterioration in CTEPH pathophysiology is unclear. In this study, we evaluated the clinical characteristics and prognoses in patients with CTEPH and a co-existing PD. We retrospectively identified 229 consecutive patients (mean age = 58.7 ± 12.5 years; 160 women) with CTEPH and categorized them according to whether they had a PD (PD group; n = 22, 9.7%) or not (non-PD group; n = 207, 90.3%). We compared the clinical characteristics, respiratory function, hemodynamics, and clinical courses in the two groups. Those in the PD group had significantly lower exercise tolerance compared to the non-PD group (6-min walk test, 309.5 ± 89.5 m vs. 369.4 ± 97.9 m, P = 0.008, percent vital capacity 85.5% ± 17.3% vs. 96.0% ± 15.5%, P = 0.003) and partial pressure of oxygen (PaO2) (54.4 ± 8.6 mmHg vs. 59.3 ± 10.7 mmHg, P = 0.039). Three-year survival was significantly poorer in the PD group compared to the non-PD group (66.1% vs 89.7%, P = 0.0026, log-rank test), particularly in patients who underwent surgery (62.2% vs 89.5%, P < 0.001, log-rank test). A concomitant PD was associated with low exercise tolerance and impaired respiratory function in patients with CTEPH and predicted poor survival, especially in those who underwent a pulmonary endarterectomy.

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Section: Discussionsupporting

confidence: 80%

Clinical characteristics and prognosis in patients with chronic thromboembolic pulmonary hypertension and a concomitant psychiatric disorder

et al. 2019

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“…Interestingly, a recent study showed that even though in patients treated before 2008 PEA enabled significantly better survival than medication, in a group of CTEPH patients who initiated treatment in 2009–2016, no difference in survival between PEA, BPA and medication was observed. 33 With recent cases of patients treated with both PEA and BPA, 34,35 it has been suggested that in the future some patients may benefit most from various combinations of surgical therapy, percutaneous therapy and MT. 5…”

Section: Discussionmentioning

confidence: 99%

Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team

Siennicka

Darocha²,

Banaszkiewicz³

et al. 2019

Ther Adv Respir Dis

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Background: Chronic thromboembolic pulmonary hypertension (CTEPH) may be treated with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapy (MT). Assessment in a multidisciplinary team of experts (CTEPH team) is currently recommended for treatment decision making. The aim of the present study was to report the effects of such an interdisciplinary concept. Methods and results: A total of 160 patients were consulted by the CTEPH team between December 2015 and September 2018. Patient baseline characteristics, CTEPH team decisions and implementation rates of diagnostic and therapeutic procedures were analysed. Change in World Health Organization (WHO) functional class and survival rates were evaluated by treatment strategy. A total of 51 (32%) patients were assessed as operable and 109 (68%) were deemed inoperable. Thirty-one (61% of operable patients) underwent PEA. Patients treated with PEA, BPA(+MT) and MT alone were 50.9 ± 14.7, 62.9 ± 15.1 and 68.9 ± 12.7 years old, respectively. At the follow-up, PEA patients had the highest WHO functional class improvement. Patients treated with BPA(+MT) had significantly better survival than PEA (p = 0.04) and MT patients (p = 0.04; 2-year survival of 92%, 79% and 79%, respectively). Conclusions:The CTEPH team ensures that necessary diagnostic procedures are performed. A relatively low proportion of patients was assessed by the CTEPH team as operable and underwent surgery, which in survivors resulted in the best functional improvement. Although patients undergoing BPA(+MT) were older than patients treated with PEA, their survival was better than patients subjected to PEA or MT alone. The reviews of this paper are available via the supplemental material section.

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“…Idiopathic PAH (IPAH) patients admitted for RHC were also enrolled for a comparison during the same period. The definition of CTEPH was described previously ( 10 ). IPAH was defined as follows: ( 1 ) a mean pulmonary arterial pressure (mPAP) of ≥25 mmHg and normal pulmonary arterial wedge pressure (PAWP) on RHC; ( 2 ) no perfusion defect on lung scintigraphy; and ( 3 ) no identifiable cause of pulmonary hypertension (connective tissue disease, congenital heart disease, portal hypertension, HIV infection, chronic lung disease) on further diagnostic work-up.…”

Section: Methodsmentioning

confidence: 99%

Section: Study Populationmentioning

confidence: 99%

Nocturnal Hypoxemia and High Circulating TNF-α Levels in Chronic Thromboembolic Pulmonary Hypertension

et al. 2020

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Objective Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension caused by persistent thromboemboli of the pulmonary arteries, and one of its etiological factors may be inflammation. Sleep disordered breathing (SDB) is reportedly an important complication of pulmonary hypertension. However, the association between SDB and inflammation in CTEPH has been undefined. This prospective observational study analyzed the association between the severity of SDB, pulmonary hemodynamic parameters and the systemic inflammation level in patients with CTEPH. Methods CTEPH patients admitted for a right heart catheter (RHC) examination were consecutively enrolled from November 2017 to June 2019 at the pulmonary hypertension center in Chiba University Hospital. Patients with idiopathic pulmonary arterial hypertension (IPAH) were also enrolled as a control group. All patients underwent a sleep study using a WatchPAT 200 during admission. Results The CTEPH patients showed worse nocturnal hypoxemia, oxygen desaturation index (ODI), and apnea-hypopnea index than the IPAH patients. Among these factors, only the nocturnal mean percutaneous oxygen saturation (SpO2) was negatively correlated with the pulmonary hemodynamic parameters. The circulating tumor necrosis factor-alpha (TNF-α) level was also high in the CTEPH group, and a multivariate analysis showed that the nocturnal mean SpO2 was the most important predictive factor for a high TNF-α level. Conclusion We showed that CTEPH patients had high serum TNF-α levels and that the nocturnal mean SpO2 was a predictive factor for serum TNF-α levels. Further investigations focused on nocturnal hypoxemia and the TNF-α level may provide novel insight into the etiology and new therapeutic strategies for CTEPH.

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Long-Term Outcome of Chronic Thromboembolic Pulmonary Hypertension at a Single Japanese Pulmonary Endarterectomy Center

Abstract: Survival in CTEPH in the recent era has significantly improved, especially in non-PEA patients. BPA and selective pulmonary vasodilators could improve survival in the non-PEA group. In the present study, no difference in survival was found between PEA and non-PEA.

Cited by 22 publications

References 22 publications

Clinical characteristics and prognosis in patients with chronic thromboembolic pulmonary hypertension and a concomitant psychiatric disorder

Clinical characteristics and prognosis in patients with chronic thromboembolic pulmonary hypertension and a concomitant psychiatric disorder

Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team

Nocturnal Hypoxemia and High Circulating TNF-α Levels in Chronic Thromboembolic Pulmonary Hypertension

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