Chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension — assessment by magnetic resonance imaging

“…Additionally, small-vessel hypertensive arteriopathy, similar to that seen in other forms of pulmonary hypertension, seems to develop in most CTEPH patients [3,5]. This is supported by several observations: (1) progression of pulmonary hypertension typically occurs in the absence of recurrent pulmonary embolic events or in situ pulmonary artery thrombosis; (2) there is a poor correlation between the extent of central vessel occlusion and the degree of pulmonary hypertension: this observation suggests that a component of the increased pulmonary vascular resistance results from the unobstructed, distal vascular bed; (3) histopathology demonstrates hypertensive arteriopathic changes in the resistance vessels of lung regions both involved and uninvolved in proximal vessel-organized thromboembolic disease.…”

“…Instead, they follow an aberrant path of organization and recanalization leading to characteristic abnormalities such as intraluminal webs and bands, pouchlike endings of arteries, irregularities of the arterial wall and stenotic lesions [2,3,5]. This aberrant path of obstruction and reopening occurs in repeated cycles over many years.…”

“…In combination with the retrospective gating and use of a 16-or 64-slice MD-CT scanner, the method should allow for assessment of right heart impairment [15], but data from prospective studies with CTEPH patients are still missing. Selective DSA in combination with right heart catheterization delivers essential information on mean arterial pumonary pressure (MPAP) und pulmonary vascular resistance (PVR) [2,3].…”

“…These critical values are reached after the occlusion of approximately 60% of the total diameter of the pulmonary arterial vascular bed. The longstanding increase in MPAP over 30 mmHg results in cor pulmonale ending in right heart failure with a corresponding 5-year-survival of only 30% [2,3,5].…”

Clinical Worsening During Long-Term Follow-Up in Inoperable Chronic Thromboembolic Pulmonary Hypertension

“…However, some patients are not suitable for surgical treatment due to the distal location of pulmonary thromboemboli or severe comorbidity. In addition, approximately 10% of patients who undergo PEA obtain no relief and maintain a pulmonary hypertensive state [7]. Modern specific pulmonary vasoactive medication (i.e., endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and prostacyclins) seems to improve the functional status and hemodynamics in patients with inoperable CTEPH at short-term follow-up [8][9][10][11][12][13][14][15][16][17].…”