Chronic Thromboembolic Pulmonary Hypertension (CTEPH): Experience From a Single Center in Central Valley of California

Abstract

SESSION TITLE: Venous Thromboembolism Posters SESSION TYPE: Original Investigation Poster PRESENTED ON: Wednesday, October 28, 2015 at 01:30 PM - 02:30 PM PURPOSE: Chronic Thromboembolic Pulmonary Hypertension (CTEPH - WHO Group IV) is characterized by non-resolving pulmonary thrombo-emboli resulting in increased pulmonary vascular resistance that can be treated by surgical pulmonary thrombo-endarterectomy (PEA). It is a uncommon condition with an unknown true prevalence. Our institution's experience with this condition is presented in this study. METHODS: An ongoing electronic registry of patients with Pulmonary Arterial Hypertension (PAH) was created in the year 2011 at UCSF Fresno (PHASE - “Pulmonary Hypertension - A Single Center's Experience”). 11 patients from 2008 to date with CTEPH were identified from this registry. A retrospective analysis was then performed. RESULTS: Of the 11 patients, 10 were incident and 1 was prevalent. Mean age was 59.1 ± 14.1 years. Male to female ratio being 5:6. 9 of 11 patients were a FC-III at the time of diagnosis 2 were FC-II. 7 of 11 (67%) had prior Venous thromboembolism (VTE) episode. 5 of 11(45%) had Hypercoagulability states. 10 of 11 were treated with pulmonary vasodilator therapies. 5 of 11 (36%) underwent PEA and 4 had a cure. 5 declined surgery. 3 subsequently passed away. All of the cases had a VQ scan and were positive for perfusion defects. 8 of 11 had Pulmonary angiography. The mean Cardiac Index (CI) was 2.66 ± 0.9 L/m2 and the mean PVR was 9.46 ± 3.51 woods units. Mean REVEAL Risk score was 10.4 ± 2.2. 3/11(27%) passed away. The average change in 6MWD and BNP after 12 months of treatment in 6 of 11 was 42.3m, and 101 with p-values of 0.039 and 0.02 respectively. CONCLUSIONS: 1) Healthy response to medical therapy was observed. 2) PEA resulted in a cure in > 90% of patients 3) VQ scan was diagnostic for CTEPH in all cases. CLINICAL IMPLICATIONS: 1) All patients must be considered for PEA given its efficacy in resulting in a cure 2) Medical therapy appears to be effective in improving patients symptoms 3) VQ scan is an important tool in diagnosis of CTEPH. DISCLOSURE: The following authors have nothing to disclose: Reza Ronaghi, Aysha Imtiaz Khan, Vijay Balasubramanian No Product/Research Disclosure Information