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Pulmonary Vascular Disease |

Predictors of Outcome in Portopulmonary Hypertension

Varun Gaur, MD; Omar Minai, MD; Adriano Tonelli, MD; Raed Dweik, MD; Gustavo Heresi, MD
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Cleveland Clinic Foundation, Cleveland, OH


Chest. 2013;144(4_MeetingAbstracts):857A. doi:10.1378/chest.1703007
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Abstract

SESSION TITLE: DVT/PE/Pulmonary Hypertension Posters

SESSION TYPE: Original Investigation Poster

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

PURPOSE: Little is known about risk stratification in portopulmonary hypertension (POPH). We sought to determine baseline predictors of outcomes in a cohort of POPH patients.

METHODS: We queried the IRB approved Cleveland Clinic Pulmonary Hypertension Registry for POPH patients. We identified 54 patients satisfying the inclusion criteria diagnosed between February 1996 and March 2012. All-cause mortality was determined by review of the medical records. Survival curves and Cox models were derived starting on the date of the diagnostic right heart catheterization.

RESULTS: Baseline characteristics: age 53+-9 years, female 59%, mean pulmonary artery pressure 47 +/-11 mmHg, pulmonary vascular resistance 6.4 +/- 3.2 Wood Units, cardiac index 3.27 +/- 1.22 l/min/m2, MELD score 12 +/- 4. Forty-six (85%) patients were rejected for liver transplant due to various reasons, 19 (41%) due to POPH. Five (9%) patients were diagnosed with POPH after liver transplant. Median follow up was 32 months (IQR 12.5 - 60). Univariate predictors of mortality included use of beta blockers (HR 3.41, 95% CI 1.11- 10.46), PCWP >= 11 mmHg (HR 2.05, 95% CI 1.03-4.10), 6-minute walk distance (HR per 50 meters 0.67, 95% CI 0.46 - 0.98), heart rate recovery at 1 minute > 14 bpm (HR 0.12, 95% CI 0.02 - 0.64), MELD score (HR per 5 units 1.11, 95% CI 1.03 - 1.20), diffusion capacity for CO (HR per 5 units 0.65, 95% CI 0.43 - 0.99) and creatinine (HR per 0.1 mg/dL 1.08, 95% CI 1.04 - 1.11). The development of POPH after liver transplant conferred worse survival compared to those with POPH but rejected for liver transplant (p = 0.04).

CONCLUSIONS: Predictors of mortality in our POPH cohort include severity of liver disease, decreased walk distance, impaired heart rate recovery, compromised kidney function, and use of beta blockers. POPH after liver transplant may be a more serious form of POPH.

CLINICAL IMPLICATIONS: Other than liver disease and hemodynamics, kidney function and functional capacity impairment need to be considered as risk markers in POPH.

DISCLOSURE: The following authors have nothing to disclose: Varun Gaur, Omar Minai, Adriano Tonelli, Raed Dweik, Gustavo Heresi

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