scholarly journals Diagnostic advances and opportunities in chronic thromboembolic pulmonary hypertension

2015 ◽  
Vol 24 (136) ◽  
pp. 253-262 ◽  
Author(s):  
Andrea M. D'Armini
Keyword(s):  
Pulmonary Hypertension ◽  
Delayed Diagnosis ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Standard Technique ◽  
Pulmonary Angiography ◽  
Computed Tomographic ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension ◽  
Computed Tomographic Pulmonary Angiography ◽  
Operability Assessment

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterised by the presence of thromboembolic material in the pulmonary circulation, and patients have a poor prognosis without treatment. Patients present with nonspecific symptoms, such as breathlessness and syncope, which means that other more common conditions are sometimes suspected before CTEPH, leading to delayed diagnosis and treatment. This is problematic because CTEPH is potentially curable with surgical pulmonary endarterectomy (PEA); indeed, CTEPH should always be considered in any patient with unexplained pulmonary hypertension (PH).Several key evaluations are necessary and complementary to confirm a diagnosis of CTEPH and assess operability. Echocardiography is initially used to confirm a general diagnosis of PH. Ventilation/perfusion scanning is then essential in the first stage of CTEPH diagnosis, with a wedge-shaped perfusion deficit indicative of CTEPH. This should be followed by right heart catheterisation (RHC) which is mandatory in confirming the diagnosis and providing haemodynamic parameters that are key predictors of the risk associated with PEA and subsequent prognosis. RHC is ideally coupled with conventional pulmonary angiography, the gold-standard technique for confirming the location and extent of disease, and thus whether the obstruction is surgically accessible. Computed tomographic pulmonary angiography is also now routinely used as a complementary technique to aid diagnosis and operability assessment.Recent improvements in the resolution of other noninvasive techniques, such as cardiac magnetic resonance imaging, allow for detailed reconstructions of the vascular tree and imaging of vessel defects, and interest in their use is increasing.

Feasibility of a noninvasive operability assessment in chronic thromboembolic pulmonary hypertension under real-world practice.

2020 ◽  
Author(s):  
adriana Rodriguez Chaverri ◽  
Yolanda Revilla Ostolaza ◽  
Maria Jesus Lopez-Gude ◽  
María Teresa Velazquez ◽  
Ines Ponz de Antonio ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Survival Rates ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Computed Tomographic Angiography ◽  
Pulmonary Angiography ◽  
Second Phase ◽  
Implementation Phase ◽  
Initial Decision ◽  
Thromboembolic Pulmonary Hypertension ◽  
Operability Assessment

Abstract Background – The development of an optimized assessment of operability in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is crucial. This study aimed to evaluate the feasibility of a noninvasive operability assessment of CTEPH based on multidetector computed tomographic angiography (MCTA). Methods –176 patients with CTEPH were evaluated from January 2016 to April 2018. Throughout the first phase, operability was assessed with MCTA and pulmonary angiography (PA): initial surgical decision was made based on MCTA with further analysis of PA to evaluate which cases initial decision was not modified by PA. During the second phase, PA was limited to patients judged inoperable based on MCTA or in those whose assessment was not possible. Results – Patients deemed operable (50%) based on MCTAalong the first phase, had been adequately classified, as PA did not modify the initial decision in all but one patient. Comparable results were obtained throughout the implementation phase, in which decision of operability was based on MCTA in 49% of the patients. Regarding operated patients, decision of operability had been based solely on MCTA in 94% of those with level I disease regarding intraoperative CTEPH classification; in 75% with level II disease and 54% with level III. This approach enabled shorter time periods to complete surgical assessment and the avoidance of PA-related morbidity. Baseline parameters, postoperative measures and survival rates at 1 year after surgerywere comparable in both phases. Conclusions - Noninvasive operability assessment is feasible in a subset of CTEPH patients and optimizessurgical candidacy evaluation.

scholarly journals Feasibility of a Noninvasive Operability Assessment in Chronic Thromboembolic Pulmonary Hypertension under Real-World Practice

Diagnostics ◽  
2020 ◽  
Vol 10 (10) ◽  
pp. 855
Author(s):  
Adriana Rodriguez Chaverri ◽  
Yolanda Revilla Ostolaza ◽  
Maria Jesus Lopez-Gude ◽  
María Teresa Velazquez ◽  
Ines Ponz de Antonio ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Survival Rates ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Computed Tomographic Angiography ◽  
Pulmonary Angiography ◽  
Second Phase ◽  
Implementation Phase ◽  
Initial Decision ◽  
Thromboembolic Pulmonary Hypertension ◽  
Operability Assessment

This study aimed to evaluate the feasibility of a noninvasive operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH) based on multidetector computed tomographic angiography (MCTA). Up to 176 patients were evaluated from January 2016 to April 2018. Throughout the first phase, the initial surgical decision was made based on MCTA with further analysis of pulmonary angiography (PA) in order to evaluate in which cases the initial decision was not modified by PA. During the second phase, PA was limited to patients judged inoperable based on MCTA or those whose assessment was not possible. Patients deemed operable (50%) based on MCTA along the first phase had been adequately classified, as PA did not modify the initial decision in all but one patient. Comparable results were obtained throughout the implementation phase. Regarding operated patients, the decision of operability was based solely on MCTA in 94% of those with level I disease, in 75% with level II, and 54% with level III. This approach enabled shorter periods of time to complete surgical assessment and the avoidance of PA-related morbidity. Baseline parameters, postoperative measures, and survival rates at 1 year after surgery were comparable in both phases. Noninvasive operability assessment is feasible in a subset of CTEPH patients and optimizes surgical candidacy evaluation.

scholarly journals Chronic thromboembolic pulmonary hypertension: a review

2021 ◽  
pp. 318-325
Author(s):  
CZ Chong ◽  
ELW Tay ◽  
CH Sia ◽  
KK Poh
Keyword(s):  
Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
World Health ◽  
Definitive Treatment ◽  
Pharmacological Agents ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension resulting from obstruction of the pulmonary artery by fibrotic thromboembolic material, usually initiated by recurrent or incomplete resolution of pulmonary embolism. This distinct form of pulmonary hypertension is classified under Group 4 of the World Health Organization classification. Further investigations are usually initiated, with transthoracic echocardiography followed by right heart catheterisation and pulmonary angiography as the gold standard. Definitive treatment is usually in the form of surgical pulmonary endarterectomy. Inoperable CTEPH is medically treated with pharmacological agents such as phosphodiesterase Type 5 inhibitors, endothelin receptor antagonists, soluble guanylate cyclase stimulators and prostacyclin. Recent developments have made balloon pulmonary angioplasty a viable option as well.

scholarly journals Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: the UK experience

Open Heart ◽  
2020 ◽  
Vol 7 (1) ◽  
pp. e001144 ◽  
Author(s):  
Stephen P Hoole ◽  
John G Coghlan ◽  
John E Cannon ◽  
Dolores Taboada ◽  
Mark Toshner ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Functional Status ◽  
Minute Ventilation ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Carbon Dioxide Production ◽  
Functional Class ◽  
Right Heart Catheterisation ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Uk

ObjectiveInoperable chronic thromboembolic pulmonary hypertension (CTEPH) managed medically has a poor prognosis. Balloon pulmonary angioplasty (BPA) offers a new treatment for inoperable patients. The national BPA service for the UK opened in October 2015 and we now describe the treatment of our initial patient cohort.MethodsThirty consecutive, inoperable, anatomically suitable, symptomatic patients on stable medical therapy for CTEPH were identified and offered BPA. They initially underwent baseline investigations including Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) quality of life (QoL) questionnaire, cardiopulmonary exercise test, 6 min walk distance (6MWD), transthoracic echocardiography, N-terminal probrain natriuretic peptide (NT pro-BNP) and right heart catheterisation. Serial BPA sessions were then performed and after completion, the treatment effect was gauged by comparing the same investigations at 3 months follow-up.ResultsA median of 3 (IQR 1–6) BPA sessions per patient resulted in a significant improvement in functional status (WHO functional class ≥3: 24 vs 4, p<0.0001) and QoL (CAMPHOR symptom score: 8.7±5.4 vs 5.6±6.1, p=0.0005) with reductions in pulmonary pressures (mean pulmonary artery pressure: 44.7±11.0 vs 34.4±8.3 mm Hg, p<0.0001) and resistance (pulmonary vascular resistance: 663±281 vs 436±196 dyn.s.cm-5, p<0.0001). Exercise capacity improved (minute ventilation/carbon dioxide production: 55.3±12.2 vs 45.0±7.8, p=0.03 and 6MWD: 366±107 vs 440±94 m, p<0.0001) and there was reduction in right ventricular (RV) stretch (NT pro-BNP: 442 (IQR 168–1607) vs 202 (IQR 105–447) pg/mL, p<0.0001) and dimensions (mid RV diameter: 4.4±1.0 vs 3.8±0.7 cm, p=0.002). There were no deaths or life-threatening complications and the mild-moderate per-procedure complication rate was 10.5%.ConclusionsBPA is safe and improves the functional status, QoL, pulmonary haemodynamics and RV dimensions of patients with inoperable CTEPH.

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