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Pulmonary Hypertension Characteristics in Alpha-1 Antitrypsin Deficiency Patients Undergoing Lung Transplant Evaluation
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A5716 - Pulmonary Hypertension Characteristics in Alpha-1 Antitrypsin Deficiency Patients Undergoing Lung Transplant Evaluation
Author Block: R. G. Valentin, D. Kay, M. L. Brantly, H. M. Alnuaimat, A. Ataya; Pulmonary, Critical Care, and Sleep Medicine, University of Florida, Gainesville, FL, United States.
Rationale:
Patients with alpha-1 antitrypsin deficiency (A1ATD) constitute approximately 6% of lung transplantsed lungs between 1995 and 2012 in the United States, as compared to 33% for non-A1ATD COPD patients. Lung transplant for cases offor end-stage lung disease in A1ATD patients has been shown to improve long-term survival, in some studies, while other studies have shown improvement only in quality of life measures. The characteristics of A1ATD patients undergoing transplant have been recently described, but no study to date has described the pulmonary hemodynamic characteristics of A1ATD patients undergoing lung transplant evaluation.
Methods:
We performed a retrospective cohort observational study of all A1ATD patients with PI*iZZ genotype referred for lung transplantation at the University of Florida from 1994 to 2015. The primary outcome was pulmonary hemodynamic characteristics measured by right heart catheterization. Values were compared to COPD patients without A1ATD evaluated for lung transplantation using the t-tests and Levene’s’s tests.
Results:
A total of 23 A1ATD patients were identified (age 54.1±9.0, 52.2% female, FEV1 0.67±0.37, FVC 1.77±0.70) that were referred for lung transplant evaluation, of these patients 19 (82.6%) underwent lung transplant, 13 (56.5%) were already on oxygen and 8 (34.8%) were on A1AT replacement therapy. RHC hemodynamics revealed that 8 (34.8%) had precapillary PH, 6 (26.1%) had postcapillary PH and 2 (8.7%) and combined pre and post capillary PH. Among the precapillary PH group, only one patient had severe PH defined by mPAP >35mmHg. None of the patients required PAH specific therapy. Compared to an equally weightedmatched cohort of non-A1ATD COPD patients (n=52) referred to lung transplant, there was no significant difference in pulmonary hemodynamics when compared to the A1ATD patients, even among the different types of PH.
Discussion:
In this group of A1ATD patients referred for lung transplant at our center, just over a third had Group 3 PH (precapillary PH by RHC). When comparing patients with A1ATD to COPD evaluated for transplantation there was no significant difference in pulmonary hemodynamics even among the different PH subgroups. This is the first study describing the pulmonary hemodynamics of patients with A1ATD pending transplantation. Our study is limited by small sample size. In future studies, hemodynamic characteristics can be compared to transplant outcomes.
Author Block: R. G. Valentin, D. Kay, M. L. Brantly, H. M. Alnuaimat, A. Ataya; Pulmonary, Critical Care, and Sleep Medicine, University of Florida, Gainesville, FL, United States.
Rationale:
Patients with alpha-1 antitrypsin deficiency (A1ATD) constitute approximately 6% of lung transplantsed lungs between 1995 and 2012 in the United States, as compared to 33% for non-A1ATD COPD patients. Lung transplant for cases offor end-stage lung disease in A1ATD patients has been shown to improve long-term survival, in some studies, while other studies have shown improvement only in quality of life measures. The characteristics of A1ATD patients undergoing transplant have been recently described, but no study to date has described the pulmonary hemodynamic characteristics of A1ATD patients undergoing lung transplant evaluation.
Methods:
We performed a retrospective cohort observational study of all A1ATD patients with PI*iZZ genotype referred for lung transplantation at the University of Florida from 1994 to 2015. The primary outcome was pulmonary hemodynamic characteristics measured by right heart catheterization. Values were compared to COPD patients without A1ATD evaluated for lung transplantation using the t-tests and Levene’s’s tests.
Results:
A total of 23 A1ATD patients were identified (age 54.1±9.0, 52.2% female, FEV1 0.67±0.37, FVC 1.77±0.70) that were referred for lung transplant evaluation, of these patients 19 (82.6%) underwent lung transplant, 13 (56.5%) were already on oxygen and 8 (34.8%) were on A1AT replacement therapy. RHC hemodynamics revealed that 8 (34.8%) had precapillary PH, 6 (26.1%) had postcapillary PH and 2 (8.7%) and combined pre and post capillary PH. Among the precapillary PH group, only one patient had severe PH defined by mPAP >35mmHg. None of the patients required PAH specific therapy. Compared to an equally weightedmatched cohort of non-A1ATD COPD patients (n=52) referred to lung transplant, there was no significant difference in pulmonary hemodynamics when compared to the A1ATD patients, even among the different types of PH.
Discussion:
In this group of A1ATD patients referred for lung transplant at our center, just over a third had Group 3 PH (precapillary PH by RHC). When comparing patients with A1ATD to COPD evaluated for transplantation there was no significant difference in pulmonary hemodynamics even among the different PH subgroups. This is the first study describing the pulmonary hemodynamics of patients with A1ATD pending transplantation. Our study is limited by small sample size. In future studies, hemodynamic characteristics can be compared to transplant outcomes.
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