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Pulmonary Vascular Disease |

Prevalence and Outcomes of Pulmonary Hypertension Associated With Sarcoidosis FREE TO VIEW

Alem Mehari, MD; Yohannes Mengistu, MD; Olana Molla, MD; Shubhangi Budhi, MD; Alicia Thomas, MD; Davis Wayne, MD; Alvin Thomas, MD
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Pulmonary, Howard University, Washington, DC


Chest. 2014;146(4_MeetingAbstracts):845A. doi:10.1378/chest.1994640
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Abstract

SESSION TITLE: DVT/PE/Pulmonary Hypertension Posters IV

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 29, 2014 at 01:30 PM - 02:30 PM

PURPOSE: The prevalence, outcomes and the pathogenic mechanisms of sarcoidosis related pulmonary hypertension is not well known

METHODS: This cross-sectional study included consecutive patients with biopsy proven sarcoidosis (n=79) who were followed up in an outpatient setting from 6/10/2003 through 2/20/2014. Demographic data; PFT results, CXR stage, 6 minute walk test and Doppler Echocardiography in all patients and available right heart catheterization results were reviewed. We defined pulmonary hypertension (PH) either ECHO estimated pulmonary arterial systolic pressure (PASP) > 40mmHg or mean pulmonary artery pressure (mPAP) ≥25mmHg by right heart catheterization (RHC). All-cause mortality data was obtained either from medical records and or social security death index

RESULTS: By Doppler echocardiography, 30 (38%) patients were found to have estimated pulmonary arterial systolic pressure (PASP) > 40mmHg. The mean estimated systolic PAP in the PH group compared to those without PH was (51.4±11.3 vs. 20.9±10.3mmHg; p<001) respectively. Eight out of the 30 patients underwent right heart catheterization and PH (mPAP≥25mmHg) was confirmed in all 8 patients. In these patients the hemodynamic findings were, mean mPAP of 51±12.3mmHg, mean pulmonary artery wedge pressure (PAWP) 15±10.1mmHg, mean transpulmonary gradient (TPG) 36±7.1mmHg, mean diastolic pulmonary gradient (DPG) 17±4.4mmHg, mean cardiac index (CI) 2.4±1.1L/minute/m2, and mean of pulmonary vascular resistance (PVR) of 8±4.5wood units. The mean age (58±9yrs vs.56±10yrs), PFT results and CXR stage did not differ between those with PH and those without PH. There were a total of 12 deaths during the study period. Compared to those without PH, the PH group had significantly higher mortality 8(26.6%) vs. 4(8%) ; P=0.026 and worse functional capacity; 6-minute walk distance 210±80 vs.319.6±113 meters; p=0.036 respectively.

CONCLUSIONS: We report a prevalence of PH of 38% in this study. The occurrence of PH was associated with worse functional capacity and increased mortality but not with the severity of parenchymal fibrosis or degree PFT abnormality

CLINICAL IMPLICATIONS: Early screening for pulmonary hypertension and further studies exploring the pathophysiology and therapy of sarcoidosis associated PH are urgently needed

DISCLOSURE: The following authors have nothing to disclose: Alem Mehari, Yohannes Mengistu, Olana Molla, Shubhangi Budhi, Alicia Thomas, Davis Wayne, Alvin Thomas

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