Chronic Thromboembolic Pulmonary Hypertension: An Observational Study

Abstract

Background and Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) has a high mortality. The treatment of CTEPH could be balloon pulmonary angioplasty (BPA), medical (MT) or pulmonary endarterectomy (PEA). This study aims to assess the clinical characteristics of CTEPH patients, surgically or medically treated, in a pulmonology referral center. Materials and Methods: A total of 124 patients with PH with suspected CTEPH (53 male subjects and 71 female subjects; mean age at diagnosis 67 ± 6) were asked to give informed consent and then were evaluated. The presence of CTEPH was ascertained by medical evaluations, radiology and laboratory tests. Results: After the evaluation of all clinical data, 65 patients met the inclusion criteria for CTEPH and they were therefore enrolled (22 males and 43 females; mean age at diagnosis was 69 ± 8). 26 CTEPH patients were treated with PEA, 32 with MT and 7 with BPA. There was a statistically significant age difference between the PEA and MT groups, at the time of diagnosis, the PEA patients were younger than the MT patients, whereas there was no statistically significant difference in other clinical characteristics (e.g., smoking habit, thrombophilia predisposition), as well as functional and hemodynamic parameters (e.g., 6-min walk test, right heart catheterization). During three years of follow-up, no patients in the PEA groups died; conversely, eleven patients in the MT group died during the same period (p < 0.05). Furthermore, a significant decrease in plasma BNP values and an increase in a meter at the six-minute walk test, 1 and 3 years after surgery, were observed in the PEA group (p < 0.05). Conclusions: This study seems to confirm that pulmonary endarterectomy (PEA) can provide an improvement in functional tests in CTEPH.

Keywords: chronic thromboembolic pulmonary hypertension (CTEPH); chronic thromboembolism; pulmonary artery pressure (PAP); pulmonary thromboendarterectomy; pulmonary vasodilator therapy.

Figure 1

Pulmonary endarterectomy casts from a patient, enrolled in the study, with complex and severe CTEPH.

Figure 2

Chronic pulmonary thromboembolism in a 66-y/o woman with a history of episodes of acute PE. (A) Coronal image from a pulmonary angio-CT demonstrate the presence of bilateral residual bands within the lumen of the vessel (white arrows), a typical vascular sign of chronic pulmonary thromboembolism. (B) Axial image demonstrates the enlargement of the main pulmonary artery (>30 mm), a typical indirect sign of pulmonary hypertension. (C) On high-resolution CT can be observed typical parenchymal signs of CTEP, which are represented by a diffuse mosaic perfusion pattern (*), thickening of the bronchial walls (white arrows) and peripheral parenchymal linear opacities from old infarcts (black arrow).

Figure 3

Kaplan–Meier analysis. The survival rate in the medical treatment group within 3 years.