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Outcomes of Patients with Pulmonary Hypertension Associated with Sarcoidosis Treated with Epoprostenol
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A5700 - Outcomes of Patients with Pulmonary Hypertension Associated with Sarcoidosis Treated with Epoprostenol
Author Block: S. Hon, E. D. Abston, L. Bajpayee, J. S. Berman, P. Govender, H. W. Farber; Pulmonary Center, Boston University School of Medicine, Boston, MA, United States.
RATIONALE:Sarcoidosis is a granulomatous disease of unknown etiology which commonly affects the lung. Pulmonary Hypertension (PH) has been observed in a subset of these patients and can occur via several different mechanisms including advanced lung disease, cardiac disease, and intrinsic pulmonary arterial hypertension. The latter group of patients is currently classified as Group 5 PH; however, many of these patients have hemodynamic profiles similar to those observed in Group 1 PH (PAH). Sarcoidosis Associated Pulmonary Hypertension (SAPH) has been associated with worse respiratory symptoms, diminished quality of life, and poorer outcomes. Optimal treatment of PH in patients with sarcoidosis is not well defined. Epoprostenol was the first drug found to be effective in PH, and it continues to be a mainstay of treatment in severe PAH. We have previously reported the effectiveness of Epoprostenol in SAPH, and the current study reports our experience in 9 additional patients with SAPH treated with epoprostenol.
METHODS:In this IRB-approved study we identified 9 subjects in our sarcoidosis database diagnosed with SAPH who were treated with epoprostenol. Patient demographics, hemodynamics, medications, and treatment outcome data were collected via chart review. A paired T-test was used to compare measurements pre- and post- epoprostanol.
RESULTS:Treatment with epoprostenol was associated with significantly improved hemodynamics acutely. Pulmonary Vascular Resistance (PVR) was significantly reduced ([Mean±SD] Pre 1138±438, Post 560±176 dynes, p=0.002), Mean right atrial pressure was significantly reduced (Pre 15.7±8.4 vs Post 8.3±2.8 mmHg p=0.02), and Cardiac Output (CO) was significantly increased (Pre 3.7±1.2 vs Post 5.5±1.2 p=0.003) following acute epoprostenol titration. Long term follow up (25±14 months) of these patients also demonstrated improved clinical status; WHO Functional Class (FC) improved from 3.7±0.5 to 2.8±0.5 (P=0.004). Survival at 10 months was 100%.
CONCLUSIONS:In this series of 9 patients with severe, FC III-IV SAPH, treated with epoprostenol, we found that subjects responded to acute epoprostenol titration with an improvement in mRAP, CO and PVR. With continued treatment, these subjects demonstrated improved symptoms (FC to a lower risk category) and improved outcome (compared to historical controls). These observations suggest that epoprostenol is an effective treatment to improve hemodynamic parameters in sarcoidosis-associated PH acutely and can mitigate symptoms and improve outcome when used chronically. Further studies are needed to identify populations of patients with severe sarcoidosis-associated PH who will benefit from treatment with epoprostenol or other systemic prostanoids.
Author Block: S. Hon, E. D. Abston, L. Bajpayee, J. S. Berman, P. Govender, H. W. Farber; Pulmonary Center, Boston University School of Medicine, Boston, MA, United States.
RATIONALE:Sarcoidosis is a granulomatous disease of unknown etiology which commonly affects the lung. Pulmonary Hypertension (PH) has been observed in a subset of these patients and can occur via several different mechanisms including advanced lung disease, cardiac disease, and intrinsic pulmonary arterial hypertension. The latter group of patients is currently classified as Group 5 PH; however, many of these patients have hemodynamic profiles similar to those observed in Group 1 PH (PAH). Sarcoidosis Associated Pulmonary Hypertension (SAPH) has been associated with worse respiratory symptoms, diminished quality of life, and poorer outcomes. Optimal treatment of PH in patients with sarcoidosis is not well defined. Epoprostenol was the first drug found to be effective in PH, and it continues to be a mainstay of treatment in severe PAH. We have previously reported the effectiveness of Epoprostenol in SAPH, and the current study reports our experience in 9 additional patients with SAPH treated with epoprostenol.
METHODS:In this IRB-approved study we identified 9 subjects in our sarcoidosis database diagnosed with SAPH who were treated with epoprostenol. Patient demographics, hemodynamics, medications, and treatment outcome data were collected via chart review. A paired T-test was used to compare measurements pre- and post- epoprostanol.
RESULTS:Treatment with epoprostenol was associated with significantly improved hemodynamics acutely. Pulmonary Vascular Resistance (PVR) was significantly reduced ([Mean±SD] Pre 1138±438, Post 560±176 dynes, p=0.002), Mean right atrial pressure was significantly reduced (Pre 15.7±8.4 vs Post 8.3±2.8 mmHg p=0.02), and Cardiac Output (CO) was significantly increased (Pre 3.7±1.2 vs Post 5.5±1.2 p=0.003) following acute epoprostenol titration. Long term follow up (25±14 months) of these patients also demonstrated improved clinical status; WHO Functional Class (FC) improved from 3.7±0.5 to 2.8±0.5 (P=0.004). Survival at 10 months was 100%.
CONCLUSIONS:In this series of 9 patients with severe, FC III-IV SAPH, treated with epoprostenol, we found that subjects responded to acute epoprostenol titration with an improvement in mRAP, CO and PVR. With continued treatment, these subjects demonstrated improved symptoms (FC to a lower risk category) and improved outcome (compared to historical controls). These observations suggest that epoprostenol is an effective treatment to improve hemodynamic parameters in sarcoidosis-associated PH acutely and can mitigate symptoms and improve outcome when used chronically. Further studies are needed to identify populations of patients with severe sarcoidosis-associated PH who will benefit from treatment with epoprostenol or other systemic prostanoids.
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