Impact of the establishment of a multidisciplinary national chronic thromboembolic pulmonary hypertension board on a monocentric surgical endarterectomy program

Abstract

Objectives: Chronic thromboembolic pulmonary hypertension is a rare disease, characterized by delays in diagnosis and curative surgical treatment. After establishing a surgical pulmonary endarterectomy centre in Switzerland and due to a historically low resection rate of 14%, a national multidisciplinary evaluation board was established in January 2018. Herein, we summarize the impact of the board on our programme.

Methods: Patients discussed in the national chronic thromboembolic pulmonary hypertension board from January 2018 to December 2023 were included. Clinical characteristics, treatment allocation and survival were compared between patients undergoing surgery, patients refusing surgery and non-operable patients. Fisher's exact test or three-way ANOVA and Kaplan-Meier analyses were used.

Results: 188 patients were discussed at our national chronic thromboembolic pulmonary hypertension board; 131 (70%) presented with operable disease, 77 (41%) were referred for pulmonary endarterectomy and 34 (18%) of operable patients declined surgery. There is a significant difference in survival between these groups (P = 0.048). One- and 2-year survival in the subgroup undergoing pulmonary endarterectomy was 97% and 79%, respectively, while 1- and 2-year survival in the subgroup refusing pulmonary endarterectomy was 91% and 76%, respectively. The pulmonary endarterectomy rate has increased from a historical low of 14-41% since establishing the board.

Conclusions: Establishing an interdisciplinary board is essential to address diagnostic and management challenges in chronic thromboembolic pulmonary hypertension patients. The Swiss national chronic thromboembolic pulmonary hypertension board played an important role in substantially increasing the rate of curative surgery.

Keywords: chronic thromboembolic pulmonary hypertension; multidisciplinary board; pulmonary endarterectomy.

Graphical abstract

Figure 1:

Geographical map of Switzerland indicating cantons, from which patients with CTEPH were referred. Number of referrals for the corresponding canton is indicated

Figure 2:

(A) Pie-chart illustrating the reasons why patients with CTEPH were classified as non-surgical candidates (proportions represented in percentages). (B) Pie-chart showing different alternative types of treatments for patients with operable CTEPH who refused PEA (proportions represented in percentages)

Figure 3:

Bar-chart showing NYHA classification for patients with CTEPH who received PEA and who refused the surgery

Figure 4:

Kaplan–Meier survival curve and numbers at risk comparing survival of PEA candidates who refused the operation, PEA candidates who underwent PEA and patients who were non-operable after diagnosis. Using log-rank test, the differences in OS between these subgroups (P = 0.048) was significant. Using Cox proportional hazards model, the difference between surgery conducted and non-operable was significant (P = 0.02), the difference between surgery conducted and refused (P = 0.5) and between refused and non-operable (P = 0.3) not significant. The timeline is presented in months, and survival curves were truncated when less than 10% of the initial sample size remained at risk

Figure 5:

Sankey plot displaying the multimodal treatment of patients with CTEPH discussed at the Swiss national CTEPH Board. One hundred thirty-one (69.6%) patients were operable, 57 (30.3%) patients were non-operable. Thirty-nine (20.74%) patients received PH-targeted medication prior to PEA. Seventy-seven (40.95%) patients underwent PEA. Eighteen (9.57%) operable patients received BPA, including 8 (4.25%) that received a BPA post-PEA. Thirty-seven (19.68%) operable patients received long-term PH-targeted medication. Fifteen (7.97%) patients received PH-targeted medication post-PEA, 5 (2.6%) post-BPA and 17 (9.04%) patients received PH-targeted medication only. Sixty-four (34.04%) operable patients had no further PH-targeted specific treatment*, including 54 (28.72%) patients post-PEA, 3 (1.59%) patients post-BPA and 7 (3.6%) who did not receive either intervention. At the time of this analysis, 20 (10.63%) PEAs are pending

Figure 6:

Histogram portraying resection rates for each year since the establishment of the board. 2018 (57.1%), 2019 (48.3%), 2020 (48.2%), 2021 (56.3%), 2022 (30.3%) and 2023 (42.1%). The overall resection rate is 41%. We included a line at the historic resection rate of 14%