Therapy For Chronic Thromboembolic Pulmonary Hypertension Research Articles

Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension in Cape Town, South Africa.

Pulmonary endarterectomy (PEA) is the only definitive and potentially curative therapy for chronic thromboembolic pulmonary hypertension (CTEPH), associated with impressive improvements in symptoms and haemodynamics. However, it is only offered at a few centres in South Africa. The characteristics and outcomes of patients undergoing PEA in Cape Town have not been reported previously. To assess the difference in World Health Organization functional class (WHO-FC) before and at least 6 weeks after surgery. We interrogated the adult cardiothoracic surgery database at the University of Cape Town between December 2005 and April 2021 for patients undergoing PEA at Groote Schuur Hospital and a private hospital. A total of 32 patients underwent PEA, of whom 8 were excluded from the final analysis owing to incomplete data or a histological diagnosis other than CTEPH. The work-up of these patients for surgery was variable: all had a computed tomography pulmonary angiogram, 7 (29%) had a ventilation/perfusion scan, 5 (21%) underwent right heart catheterisation, and none had a pulmonary angiogram. The perioperative mortality was 4/24 (17%): 1 patient (4%) had a cardiac arrest on induction of anaesthesia, 2 patients (8%) died of postoperative pulmonary haemorrhage, and 1 patient (4%) died of septic complications in the intensive care unit. Among the survivors, the median (interquartile range) improvement in WHO-FC was 2 (1 - 3) classes (p=0.0004); 10/16 patients (63%) returned to a normal baseline (WHO-FC I). Even in a low-volume centre, PEA is associated with significant improvements in WHO-FC and a return to a normal baseline in survivors. What the study adds. South African patients undergoing pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) have a marked improvement in functional status, with many returning to a normal functional baseline. However, the small number of patients included in this study indicates that PEA is probably underutilised. Pre- and postoperative assessment is inconsistent, despite availability of established guidelines.Implications of the findings. More patients should be referred to specialist centres for assessment for this potentially curative procedure. Use of guidelines to standardise investigations and monitoring of patients with CTEPH may improve patient selection for surgery.

Abstract 10269: Direct Oral Anticoagulation Therapy vs Vitamin K Antagonist in Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review and Meta-Analysis

Introduction: The use of direct oral anticoagulation therapy (DOAC) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) has recently increased, despite the scarce evidence to support their use in this condition. Our systematic review and meta-analysis aim to summarize the available data on the safety and effectiveness of DOAC. Hypothesis: DOAC is a safe and effective alternative anticoagulation therapy in CTEPH. Methods: Unrestricted search of the PubMed, EMBASE, and Cochrane databases from inception till May 1, 2021, for studies comparing DOAC with Vitamin K antagonist (VKA) in CTEPH patients. Relevant data were extracted and analyzed using Revman 5.3 software. Odds Ratio (OR) and 95% Confidence interval (CI) were calculated using the random-effects model. Results: A total of 3 observational studies were included examining 1,630 patients with CTEPH (386 DOAC vs 1,244 VKA). The most used DOAC was Rivaroxaban (79% of that population). There was no difference between DOAC and VKA in the incidence of recurrent venous thromboembolism OR 1.35 (95% CI 0.40-4.52), major bleeding OR 0.60 (95% CI 0.33-1.10), and clinically relevant non-major bleeding OR 1.42 (95% CI 0.86-2.33). Conclusions: DOAC is probably non-inferior to VKA in CTEPH patients. Since rivaroxaban is the most used DOAC in the included cohort, the safety, and efficacy of other DOAC remain unclear. Our findings need further validation with randomized clinical trials.

USE OF PAH SPECIFIC THERAPY IN CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION: A META-ANALYSIS

SESSION TITLE: Pulmonary Vascular Disease 1 SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2018 01:00 PM - 02:00 PM PURPOSE: Pulmonary endarterectomy (PEA) is the definitive treatment for chronic thromboembolic pulmonary hypertension (CTEPH). However, not all patients are surgical candidates and some may be left with residual pulmonary hypertension after PEA. Treatment options for such patients are limited. The microvasculopathy of CTEPH and pulmonary arterial hypertension (PAH) are similar. A few randomized controlled trials (RCTs) have reported on the use of PAH specific therapy in CTEPH but not all have shown improvement in exercise capacity and/or pulmonary hemodynamics. METHODS: PUBMED was searched for articles published between January 1996 and February 2018, using the term “chronic thromboembolic pulmonary hypertension” and applying the filters: English language, Humans and Clinical Trials. Studies were included if they met the following criteria: randomized, placebo controlled, clinical trial AND pulmonary hypertension (PH) confirmed by right heart catheterization, AND baseline and follow up six minute walk distance (6MWD) AND baseline and follow up pulmonary hemodynamic data. RevMan 5.3 was used to perform the meta-analysis. RESULTS: Five RCTs met our inclusion criteria. There was a total of 293 patients treated for 12-16 weeks with placebo or one of the three drug classes of PAH specific therapy: Riociguat (n= 173), Endothelin receptor antagonist (n= 118), and Phosphodiesterase 5 inhibitor (n= 9). The majority of the cases was inoperable CTEPH. Overall, the placebo corrected mean change in 6MWD was 24.73 meters (95%CI 14.24, 35.21). The mean change in mean pulmonary artery pressure was -4.78 mm Hg (95%CI-6.42, -3.14), in pulmonary vascular resistance -194 dynes.sec/cm5 (95%CI -233.30, -155.09) and in cardiac index 0.39 L/min/m2 (95%CI 0.16, 0.61). CONCLUSIONS: The use of PAH specific therapy in patients with CTEPH results in significant improvement in 6MWD and pulmonary hemodynamics. CLINICAL IMPLICATIONS: PAH specific therapy is beneficial in patients with inoperable CTEPH or persistent CTEPH following PEA. DISCLOSURES: No relevant relationships by Jason Filopei, source=Web Response No relevant relationships by Adil Shujaat, source=Web Response No relevant relationships by David Steiger, source=Web Response

Recent Advances in Chronic Thromboembolic Pulmonary Hypertension

Surgical excellence in pulmonary thromboendarterectomy (PTE) for chronic thromboembolic pulmonary hypertension (CTEPH) has begun to spread around the world. The perioperative mortality for this procedure is typically under 10%. The maximal benefit from PTE is derived in those patients who have a high proximal clot burden that is surgically accessible, as outlined by the Jamieson classification. Residual pulmonary hypertension after successful PTE is common and increasingly is managed with maintenance oral pulmonary vasodilator therapy such as endothelin antagonists, phosphodiesterase inhibitors, and/or prostaglandins. The role of pulmonary vasodilator therapy in CTEPH before PTE is limited and should not delay definitive surgical therapy. Although plain deep hypothermic circulatory arrest (DHCA) is the classic technique for CTEPH, alternatives such as DHCA with antegrade cerebral perfusion are feasible as well. Prolonged mechanical ventilation after PTE remains common in part because of reperfusion pulmonary edema. Careful perioperative management can reduce the incidence of this syndrome. Because ventilator-associated pneumonia is also a common complication after PTE, it represents a major opportunity for outcome improvement, particularly because there are multiple modalities for its prevention and prompt diagnosis.