Cardiovascular Disease |

A Case of Chronic Thromboembolic Pulmonary Hypertension Treated Successfully With Bosentan and Sildenafil: A Salvage Regimen FREE TO VIEW

Ashima Sahni, MD; Shashvat Sukhal, MD; Najamul Ansari, MD; Nancy Quesada, MD
Author and Funding Information

john H Stroger Jr Hosp of Cook County, Chicago, IL

Chest. 2013;144(4_MeetingAbstracts):157A. doi:10.1378/chest.1703874
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SESSION TITLE: Cardiovascular Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Chronic thrombo embolic pulmonary hypertension (CTEPH) is associated with high morbidity and mortality. Pulmonary endarterectomy (PEA) is the treatment of choice. We report a case of successful improvement in hemodynamics and six minute walk distance (6MWD) in a patient receiving combination of bosentan and sildenafil as an attempt at salvage therapy of CTEPH, who was not a candidate for surgical intervention, due to financial limitations.

CASE PRESENTATION: A 60-year-old female with a history of pulmonary embolism (PE), presented with exertional dyspnea for two months. Physical exam revealed exertional desaturation. Investigations showed hypoxia with high A-a gradient, and moderately reduced diffusion capacity on the lung function test. Echocardiography showed severe right ventricular (RV) strain with estimated pulmonary artery systolic pressure (PASP) of 90 mm of Hg. CT chest showed patchy areas of mosaic pattern in bilateral lungs, no acute PE. Right heart catheterization showed a mean pulmonary artery pressure (mPAP) of 67 mm of Hg with no vasodilator response, a normal wedge pressure and left ventricular end diastolic pressure. An autoimmune & HIV workup was negative. A ventilation/perfusion scan was consistent with chronic PE. The patient declined evaluation for PEA due to financial limitations. She was started on bosentan ,sildenafil, anticoagulation and oxygen. On follow up after six months, the patient’s 6MWD improved from 60 to 283 meters, as did her physiological responses to exercise. Echocardiography one year after treatment showed normalization of the PASP, and no RV strain. These improvements were sustained on subsequent follow ups as well.

DISCUSSION: CTEPH is now considered “dual vasculopathy”. There is increased vascular resistance in the pulmonary vessels affected with embolism and vascular remodeling in spared vessels which are histopathological indistinguishable from classical pulmonary arteriopathy observed in pulmonary arterial hypertension (PAH) [1]. In addition to PAH, all forms of pulmonary hypertension, regardless of etiology, have proven to have common defects in the downstream signaling pathways, and common underlying molecular mechanism [2]. This may suggest some benefit in using pulmonary vasodilators in CTEPH even though PEA is the treatment of choice.

CONCLUSIONS: Further Randomized Controlled Trails need to be done in patients with CTEPH who are not surgical candidates to assess the combined effects of pulmonary vasodilators.

Reference #1: Moser KM et al , Pulmonary vascular lesions occurring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest, 1993. 103(3): p. 685-92.

Reference #2: Du L, et al., Signaling molecules in nonfamilial pulmonary hypertension. N Engl J Med, 2003. 348(6): p. 500-9.

DISCLOSURE: The following authors have nothing to disclose: Ashima Sahni, Shashvat Sukhal, Najamul Ansari, Nancy Quesada

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