- Email: [email protected]
836 Pulmonary Arterial Hypertension in Sweden: First Data from a National Registry
S284
The Journal of Heart and Lung Transplantation, Vol 31, No 4S, April 2012
parenchyma, with no predominance for either the left/right lungs or upper/ lower zones. There was a moderate positive correlation to all measures of haemodynamics that was strongest for the diastolic pulmonary arterial pressure (r⫽0.415, p⬍0.01), and a positive correlation to the six-minute walk distance (r⫽0.305, p⬍0.05).
creased cardiac index and oxygen use were independently associated with increased mortality. Conclusions: Pulmonary arterial hypertension remains a progressive and fatal disease in this modern management era. 837 Echocardiographic and Clinical Predictors of Outcome in Patients with Pulmonary Arterial Hypertension F. Haddad,1 R. Doyle,1 A.Y. Denault,2 M. Skhiri,1 E. Spiekerkoetter,1 V. de Jesus Perez,1 K. Kudelko,1 I. Schnittger,1 R. Zamanian.1 1 Medicine, Stanford University, Palo Alto, CA; 2Anesthesia and Critical Care Medicine, Montreal Heart Institute, Montreal, QC, Canada.
Conclusions: This is the first study to demonstrate a correlation between neovascularity and both pulmonary arterial pressures and six-minute walk distance in patients with IPAH. The presence of this feature may be evidence of severe and longstanding pulmonary arterial hypertension leading to the development of pulmonary plexogenic arteriopathy. 836 Pulmonary Arterial Hypertension in Sweden: First Data from a National Registry N. Selimovic,1 B. Lövgren Ekmehag,6 K. Jansson,3 F. Larsen,2 S. Söderberg,5 G. Wikström.4 1Dept. of Cardiology, Sahlgrenska University Hospital, Gothenburg, Sweden; 2Dept. of Clinical Physiology, Karolinska University Hospital, Stockholm, Sweden; 3Dept. of Cardiology, University Hospital Linköping, Linköping, Sweden; 4Dept. of Cardiology, Akademiska Hospital University of Uppsala, Uppsala, Sweden; 5Dept. of Cardiology, Umeå University Hopsital, Umeå, Sweden; 6Norrtälje Hospital, Norrtälje, Stockholm, Sweden. Purpose: The aim of the present study was to characterize mortality in patients with pulmonary arterial hypertension (PAH) and to investigate factors associated with survival. Methods and Materials: Analysis of the patients entered in The Swedish National Registry for pulmonary hypertension from 1st January 2000 through 31st December 2010. We looked at medical history, hemodynamic data, functional capacity, gas exchange and demographic variables. Patients were observed until the end of September 2011. The overall survival distribution was estimated by Kaplan-Meier statistics. The relations between survival and selected variables were examined using Cox regression model. Results: A total number of 364 patients were entered in the registry (169 patients with idiopathic PAH, 13 with familial PAH, 118 patients with connective tissue disease associated PAH (CTD-APAH), 42 patients with congenital heart disease APAH and 22 patients with other forms of APAH. The mean age at diagnosis was 55.6⫾17 (18-86) years, and 67 % (n⫽245) were female. At diagnosis, 84 % of the patients were in WHO functional class III or IV. Mean six minute walking distance was 318 ⫾ 139 m. Pulmonary artery mean pressure, cardiac index and pulmonary vascular resistance were 49⫾16 mm Hg, 2.5⫾0.8 L/m2 and 9.9⫾5.7 Wood units, respectively. During the observation period 127 (35%) patients died and 28 (8%) underwent lung transplantation (Ltx). The median survival time for the entire group was 2.9 yrs (1.46 - 5.95 yrs).The observed 1-, 2-, and 3-years survival rates were 88% (85%), 79% (74%), and 73% (65%) (Ltx patients were censored/event). Prevalent cases had a significantly better survival than incident cases (p⫽0.008). CTD-APAH patients had a worse survival compared with IPAH patients (p⬍0.001). In multivariate analysis age, WHO class III-IV, reduced hemoglobin levels, incident cases, de-
Purpose: The objective of this study is to determine the predictive of quantitative echocardiographic indices in patients with pulmonary arterial hypertension (PAH). Methods and Materials: Consecutive patients with idiopathic, familial or drug and toxin associated PAH followed at Stanford University between January 2000 to January 2009 were included in the study. Echocardiographic indices included indexed RV end-diastolic area, indexed right atrial area, RV fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), right ventricular myocardiocardial performance index (RVMPI), systolic and diastolic eccentricity index, estimated RV systolic pressure (RVSP) and estimated right atrial pressure (RAP). The combined end-point consisted of death or lung transplantation. Multivariable logistic regression analysis was performed to determine independent predictors of survival. Results: Ninety five patients with PAH were included in the study.The mean age was 43⫾11 years,79% of patients were women and the six minute walking distance was 432⫾117 m. On right heart catheterization, the mean pulmonary arterial pressure was 54⫾12 mmHg, the mean capillary wedge pressure 10⫾4 mmHg, the mean RAP 10⫾ 6 mmHg and the mean PVRI 25⫾12 Wood units m2. On echocardiography, RVFAC was 27%⫾8%,TAPSE was 1.5⫾0.4 cm, RVMPI was 0.60⫾0.15 and indexed RA area was 11 ⫾ 5 cm2/m2. Patients were followed for an average of 5.0 ⫾ 2.6 years. The combined end point occurred in 28 patients and the estimated1 and 3 year event free survival were 93% and 84%. Multivariate analysis identified RVFAC [HR of 0.73 per 5% increase (0.54 to 0.90)], indexed right atrial area [HR of 1.77 per 5 cm2/m2, (1.26 to 2.58)] and systolic blood pressure [HR of 0.78 per 5 mmHg increase (0.66 to 0.92)] as independent predictors of outcome. Conclusions: Outcomes of patients with PAH with PAH have improved in the modern era of therapy. Incorporating quantitative echocardiographic indices can improve prediction of outcome in patients with PAH. 838 Differences in Mortality and Morbidity in Pulmonary Hypertension Secondary to Systolic vs. Diastolic Heart Failure J.N. Salamon,1 R. Zolty.2 1Medicine, Jacobi Medical Center/Albert Einstein College of Medicine, Bronx, NY; 2Medicine, Division of Cardiology, Montefiore Medical Center/AlbertEinstein College of Medicine, Bronx, NY. Purpose: Traditionally, left ventricular systolic dysfunction (sHF) and valvular disorders have been implicated in precipitating pulmonary hypertension (sHF-PH). Recently, heart failure with preserved ejection fraction (HFpEF), a disease whose prevalence rises with age, has been recognized as a leading cause of PH (HFpEF-PH). However, data is limited comparing the natural history of these two etiologies. We sought to elucidate morbidity and mortality through 5 years of severe HFpEF-PH compared with sHF-PH in a large medical center in New York City. Methods and Materials: In a retrospective cohort fashion we identified all patients who, from 2000-2010, were diagnosed with severe PH via echo (pulmonary artery systolic pressureⱖ65mmHg, left atrial sizeⱖ4cm) at Montefiore Medical Center. Patients were grouped based on ejection fraction (EF) ⱖ50% (HFpEF-PH) or EFⱕ35% (sHF-PH) with exclusion if they had evidence of recent surgery. Endpoints were all-cause mortality and readmission through 5 years. Results: After multivariate analysis, patients with HFpEF-PH (N⫽370, mean age 75⫾0.7) exhibited a significantly elevated all cause mortality rate
The Journal of Heart and Lung Transplantation, Vol 31, No 4S, April 2012
parenchyma, with no predominance for either the left/right lungs or upper/ lower zones. There was a moderate positive correlation to all measures of haemodynamics that was strongest for the diastolic pulmonary arterial pressure (r⫽0.415, p⬍0.01), and a positive correlation to the six-minute walk distance (r⫽0.305, p⬍0.05).
creased cardiac index and oxygen use were independently associated with increased mortality. Conclusions: Pulmonary arterial hypertension remains a progressive and fatal disease in this modern management era. 837 Echocardiographic and Clinical Predictors of Outcome in Patients with Pulmonary Arterial Hypertension F. Haddad,1 R. Doyle,1 A.Y. Denault,2 M. Skhiri,1 E. Spiekerkoetter,1 V. de Jesus Perez,1 K. Kudelko,1 I. Schnittger,1 R. Zamanian.1 1 Medicine, Stanford University, Palo Alto, CA; 2Anesthesia and Critical Care Medicine, Montreal Heart Institute, Montreal, QC, Canada.
Conclusions: This is the first study to demonstrate a correlation between neovascularity and both pulmonary arterial pressures and six-minute walk distance in patients with IPAH. The presence of this feature may be evidence of severe and longstanding pulmonary arterial hypertension leading to the development of pulmonary plexogenic arteriopathy. 836 Pulmonary Arterial Hypertension in Sweden: First Data from a National Registry N. Selimovic,1 B. Lövgren Ekmehag,6 K. Jansson,3 F. Larsen,2 S. Söderberg,5 G. Wikström.4 1Dept. of Cardiology, Sahlgrenska University Hospital, Gothenburg, Sweden; 2Dept. of Clinical Physiology, Karolinska University Hospital, Stockholm, Sweden; 3Dept. of Cardiology, University Hospital Linköping, Linköping, Sweden; 4Dept. of Cardiology, Akademiska Hospital University of Uppsala, Uppsala, Sweden; 5Dept. of Cardiology, Umeå University Hopsital, Umeå, Sweden; 6Norrtälje Hospital, Norrtälje, Stockholm, Sweden. Purpose: The aim of the present study was to characterize mortality in patients with pulmonary arterial hypertension (PAH) and to investigate factors associated with survival. Methods and Materials: Analysis of the patients entered in The Swedish National Registry for pulmonary hypertension from 1st January 2000 through 31st December 2010. We looked at medical history, hemodynamic data, functional capacity, gas exchange and demographic variables. Patients were observed until the end of September 2011. The overall survival distribution was estimated by Kaplan-Meier statistics. The relations between survival and selected variables were examined using Cox regression model. Results: A total number of 364 patients were entered in the registry (169 patients with idiopathic PAH, 13 with familial PAH, 118 patients with connective tissue disease associated PAH (CTD-APAH), 42 patients with congenital heart disease APAH and 22 patients with other forms of APAH. The mean age at diagnosis was 55.6⫾17 (18-86) years, and 67 % (n⫽245) were female. At diagnosis, 84 % of the patients were in WHO functional class III or IV. Mean six minute walking distance was 318 ⫾ 139 m. Pulmonary artery mean pressure, cardiac index and pulmonary vascular resistance were 49⫾16 mm Hg, 2.5⫾0.8 L/m2 and 9.9⫾5.7 Wood units, respectively. During the observation period 127 (35%) patients died and 28 (8%) underwent lung transplantation (Ltx). The median survival time for the entire group was 2.9 yrs (1.46 - 5.95 yrs).The observed 1-, 2-, and 3-years survival rates were 88% (85%), 79% (74%), and 73% (65%) (Ltx patients were censored/event). Prevalent cases had a significantly better survival than incident cases (p⫽0.008). CTD-APAH patients had a worse survival compared with IPAH patients (p⬍0.001). In multivariate analysis age, WHO class III-IV, reduced hemoglobin levels, incident cases, de-
Purpose: The objective of this study is to determine the predictive of quantitative echocardiographic indices in patients with pulmonary arterial hypertension (PAH). Methods and Materials: Consecutive patients with idiopathic, familial or drug and toxin associated PAH followed at Stanford University between January 2000 to January 2009 were included in the study. Echocardiographic indices included indexed RV end-diastolic area, indexed right atrial area, RV fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), right ventricular myocardiocardial performance index (RVMPI), systolic and diastolic eccentricity index, estimated RV systolic pressure (RVSP) and estimated right atrial pressure (RAP). The combined end-point consisted of death or lung transplantation. Multivariable logistic regression analysis was performed to determine independent predictors of survival. Results: Ninety five patients with PAH were included in the study.The mean age was 43⫾11 years,79% of patients were women and the six minute walking distance was 432⫾117 m. On right heart catheterization, the mean pulmonary arterial pressure was 54⫾12 mmHg, the mean capillary wedge pressure 10⫾4 mmHg, the mean RAP 10⫾ 6 mmHg and the mean PVRI 25⫾12 Wood units m2. On echocardiography, RVFAC was 27%⫾8%,TAPSE was 1.5⫾0.4 cm, RVMPI was 0.60⫾0.15 and indexed RA area was 11 ⫾ 5 cm2/m2. Patients were followed for an average of 5.0 ⫾ 2.6 years. The combined end point occurred in 28 patients and the estimated1 and 3 year event free survival were 93% and 84%. Multivariate analysis identified RVFAC [HR of 0.73 per 5% increase (0.54 to 0.90)], indexed right atrial area [HR of 1.77 per 5 cm2/m2, (1.26 to 2.58)] and systolic blood pressure [HR of 0.78 per 5 mmHg increase (0.66 to 0.92)] as independent predictors of outcome. Conclusions: Outcomes of patients with PAH with PAH have improved in the modern era of therapy. Incorporating quantitative echocardiographic indices can improve prediction of outcome in patients with PAH. 838 Differences in Mortality and Morbidity in Pulmonary Hypertension Secondary to Systolic vs. Diastolic Heart Failure J.N. Salamon,1 R. Zolty.2 1Medicine, Jacobi Medical Center/Albert Einstein College of Medicine, Bronx, NY; 2Medicine, Division of Cardiology, Montefiore Medical Center/AlbertEinstein College of Medicine, Bronx, NY. Purpose: Traditionally, left ventricular systolic dysfunction (sHF) and valvular disorders have been implicated in precipitating pulmonary hypertension (sHF-PH). Recently, heart failure with preserved ejection fraction (HFpEF), a disease whose prevalence rises with age, has been recognized as a leading cause of PH (HFpEF-PH). However, data is limited comparing the natural history of these two etiologies. We sought to elucidate morbidity and mortality through 5 years of severe HFpEF-PH compared with sHF-PH in a large medical center in New York City. Methods and Materials: In a retrospective cohort fashion we identified all patients who, from 2000-2010, were diagnosed with severe PH via echo (pulmonary artery systolic pressureⱖ65mmHg, left atrial sizeⱖ4cm) at Montefiore Medical Center. Patients were grouped based on ejection fraction (EF) ⱖ50% (HFpEF-PH) or EFⱕ35% (sHF-PH) with exclusion if they had evidence of recent surgery. Endpoints were all-cause mortality and readmission through 5 years. Results: After multivariate analysis, patients with HFpEF-PH (N⫽370, mean age 75⫾0.7) exhibited a significantly elevated all cause mortality rate