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. 2023 Sep;42(9):1275-1285.
doi: 10.1016/j.healun.2023.04.016. Epub 2023 May 16.

Natural history of chronic thromboembolic pulmonary disease with no or mild pulmonary hypertension

Sathineni A Reddy  1 , Emilia M Swietlik  2 , Lucy Robertson  2 , Alice Michael  2 , Sonja Boyle  2 , Gary Polwarth  2 , Nick J Screaton  3 , Alessandro Ruggiero  3 , Sarah L Nethercott  4 , Dolores Taboada  2 , Karen K Sheares  2 , Charaka Hadinnapola  2 , John E Cannon  2 , Katherine Bunclark  2 , David Jenkins  5 , Choo Ng  5 , Mark R Toshner  6 , Joanna Pepke-Zaba  2
Affiliations

Natural history of chronic thromboembolic pulmonary disease with no or mild pulmonary hypertension

Sathineni A Reddy et al. J Heart Lung Transplant. 2023 Sep.

Abstract

Background: We describe baseline characteristics, disease progression and mortality in chronic thromboembolic pulmonary disease patients as a function of mean pulmonary artery pressure (mPAP) according to new and previous definitions of pulmonary hypertension.

Methods: All patients diagnosed with chronic thromboembolic pulmonary disease between January, 2015 and December, 2019 were dichotomized according to initial mPAP: ≤ 20 mmHg ('normal') vs 21-24 mmHg ('mildly-elevated'). Baseline features were compared between the groups, and pairwise analysis performed to determine changes in clinical endpoints at 1-year, excluding those who underwent pulmonary endarterectomy or did not attend follow-up. Mortality was assessed for the whole cohort over the entire study period.

Results: One hundred thirteen patients were included; 57 had mPAP ≤ 20 mmHg and 56 had mPAP 21-24 mmHg. Normal mPAP patients had lower pulmonary vascular resistance (1.6 vs 2.5WU, p < 0.01) and right ventricular end-diastolic pressure (5.9 vs 7.8 mmHg, p < 0.01) at presentation. At 3 years, no major deterioration was seen in either group. No patients were treated with pulmonary artery vasodilators. Eight had undergone pulmonary endarterectomy. Over 37 months median follow-up, mortality was 7.0% in the normal mPAP group and 8.9% in the mildly-elevated mPAP group. Cause of death was malignancy in 62.5% of cases.

Conclusions: Chronic thromboembolic pulmonary disease patients with mild pulmonary hypertension have statistically higher right ventricular end-diastolic pressure and pulmonary vascular resistance than those with mPAP ≤ 20 mmHg. Baseline characteristics were otherwise similar. Neither group displayed disease progression on non-invasive tests up to 3 years. Mortality over 37 months follow-up is 8%, and mainly attributable to malignancy. Further prospective study is required to validate these findings.

Keywords: health outcomes; pulmonary vascular disease; thromboembolic disease.

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