Chronic thromboembolic pulmonary hypertension: an enigma

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary arterial hypertension (PAH) in which the pulmonary thrombus fails to resolve, resulting in occlusion and remodelling of pulmonary arteries. Timely diagnosis is critical since it is potentially curable by pulmonary thromboendarterectomy. Twenty five per cent of cases do not have a history of thromboembolic event. The diagnosis should be considered in the diagnostic work-up of PAH despite lack of history of episodes of thromboembolism. Here we are reporting a case of CTEPH with multiple systemic to pulmonary collaterals delineated by angiogram and CT.

10-year success story in CTEPH treatment: breaking the myth of rareness

“…Residual PH could arise from small-vessel arteriopathy or a blend of residual proximal disease and arteriopathy, and these underlying mechanisms could justify the report of a few cases of recurrence a decade postsurgery. In those reported cases, ongoing mosaic changes were detected via CT in the initial re-evaluation after PEA, emphasising the importance of regular and long-term follow-up at specialised centres 1–8. Over a 10-year follow-up period, the patient did not have a recurrence of CTEPH or related complications, with normalisation of haemodynamic parameters and sustained improvement (table 1).…”

“…CTEPH is a distinct form of pulmonary vascular disease categorised under group 4 of the WHO clinical classification of PH 1–6. It arises as a consequence of mechanical obstruction caused by thromboembolic material adhering to the PA branches, leading to increased PVR and subsequent PH 1 4.…”

“…The long-term outcomes of CTEPH patients are influenced by various factors. Surgical interventions, such as PEA and medical therapy, have shown evidence of improving survival 1–7. A majority of experts agree that a favourable goal includes attaining a good functional class (WHO-FC I-II) and/or achieving normalisation or near normalisation of resting haemodynamic within 3 to 6 months after the procedure (PEA or last balloon pulmonary angioplasty).…”

Chronic Thromboembolic Pulmonary Hypertension: How to Diagnose? A Case Report

“…Dyspnea, hypoxemia, and right ventricular (RV) dysfunction are symptoms that become evident as PAH worsens, ultimately resulting in mortality from decompensated right heart failure. 3 Current research has shown that 25% of patients do not have a history of PE. 4 This poses a difficulty in differentiating between a de novo acute event and an acute embolic event worsening of undetected CTEPH.…”

“…Study shows a pooled sensitivity for total arteries, main-lobar arteries, and segmental arteries was 88%, 95%, and 88%, respectively, while pooled specificity was 90%, 96%, and 89%, respectively. 3,8 Depending on the severity of PH and vascular blockage, the imaging results in individuals with CTEPH might be very different. CTPA has the highest sensitivity for identifying abnormalities in the main and lobar pulmonary arteries and progressively lower sensitivity in the segmental and subsegmental arteries.…”