Outcome of Early Introduction of Hypertransfusion/Chelation Program in Children with Thalassemia Intermedia

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3260-3260
Author(s):  
Yasser Wali ◽  
A. Hakim Al-Rawas ◽  
Shoaib Al Zadjali ◽  
Murtadha K. Al-Khabori ◽  
Wafa Bashir ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Conflicts Of Interest ◽  
Iron Chelation ◽  
Self Esteem ◽  
Thalassemia Intermedia ◽  
Transfusion Therapy ◽  
Early Introduction ◽  
The Mean ◽  
The Impact

Abstract Abstract 3260 Background: The standard of care for patients with thalassemia intermedia (TI) is regular follow up and iron chelation therapy. Patients with TI are usually transfusion independent and receive irregular transfusion when they develop low hemoglobin levels as in case of infection. However, with the current policy, these patients are liable to many complications; thalassemic facies, growth retardation, splenomegaly, hypersplenism, hypercoagulability, pulmonary hypertension, heart failure, cholelithiasis, diabetes mellitus, hypothyroidism, osteoporosis, and hypogonadism. We planned to investigate the impact of early introduction of hypertransfusion on delaying these complications. Material and Methods: All newly diagnosed children with TI at the Pediatric Thalassemia Day Care Centre, Sultan Qaboos University Hospital, Oman, starting from August 2006 were included in the study. They were diagnosed as intermedia based on clinical grounds of late age presentation (>5 years), maintaining mean baseline hemoglobin of 7 g/dL (range 6.1–7.9 g/dL), and transfusion independence. In addition, eight patients had a definitive TI mutation Hb Dhofar [β29(GGC–GGT)gly-gly, β58(CCT–CGT)pro-arg]. They were 10 females and 10 males included in the study. The mean age at presentation was 7.17 ± 3.9 years. At diagnosis, they were started on monthly hypertransfusion program to maintain a pretransfusion hemoglobin level above 9.5 g/dl and a post transfusion level of 14 g/dl. The mean duration of transfusion therapy was 6.2 ± 3.7 years and the mean follow up duration was 7.1 ± 3.2 years. Results: None of the 20 patients developed thalassemic facies, splenomegaly or hypersplenism. Sixteen patients maintained a normal weight (above 5th centile) and height (above 10thcentile), while 4 patients were at or below the 3rd centile. Of the 10 children above eleven years of age, 7 children reached normal puberty at their expected age. All 10 patients above eleven years of age, had a normal thyroid function test (TSH 2.27 ± 1.07 mIU/L, T4 11.04 ± 2.65 pmol/L) with no clinical or laboratory evidence of diabetes. Eight of the elder patients who had echocardiography had no evidence of pulmonary hypertension. Baseline bone densitometry done in 2 patients revealed a low Z-Score (below −2.5), with no improvement on follow-up. The mean total transfusion requirement was 162 ± 23 ml/kg/year. Patients were maintained on either deferiprone (n=14, dose 87 ± 12 mg/kg/day) or deferasirox chelation (n=6, dose 32 ± 4 mg/kg/day). Preliminary results of a self esteem questionnaire of these patients were better than our older patients who had not received hypertransfusion/chelation. Conclusion: TI children who were started at presentation on hypertansfusion/chelation regimen, have improved growth, better self esteem and they did not develop many of the complications known to occur in the non-transfused ones. However, this regimen needs to be evaluated in a larger prospective cohort study and to confirm the cost-effectiveness given the regular blood transfusion and continuous iron chelation. Disclosures: No relevant conflicts of interest to declare.

Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study

Blood ◽  
2010 ◽  
Vol 115 (10) ◽  
pp. 1886-1892 ◽  
Author(s):  
Ali T. Taher ◽  
Khaled M. Musallam ◽  
Mehran Karimi ◽  
Amal El-Beshlawy ◽  
Khawla Belhoul ◽  
...  
Keyword(s):  
Heart Failure ◽  
Iron Chelation ◽  
Common Disease ◽  
Complication Rates ◽  
Leg Ulcers ◽  
Thalassemia Intermedia ◽  
Transfusion Therapy ◽  
Hydroxyurea Treatment ◽  
Increased Risk ◽  
The Impact

Abstract Despite recent advances in understanding the pathophysiologic mechanisms behind the thalassemia intermedia (TI) phenotype, data on the effects of treatment are deficient. To provide such data, we evaluated 584 TI patients for the associations between patient and disease characteristics, treatment received, and the rate of complications. The most common disease-related complications were osteoporosis, extramedullary hematopoeisis (EMH), hypogonadism, and cholelithiasis, followed by thrombosis, pulmonary hypertension (PHT), abnormal liver function, and leg ulcers. Hypothyroidism, heart failure, and diabetes mellitus were less frequently observed. On multivariate analysis, older age and splenectomy were independently associated with an increased risk of most disease-related complications. Transfusion therapy was protective for thrombosis, EMH, PHT, heart failure, cholelithiasis, and leg ulcers. However, transfusion therapy was associated with an increased risk of endocrinopathy. Iron chelation therapy was in turn protective for endocrinopathy and PHT. Hydroxyurea treatment was associated with an increased risk of hypogonadism yet was protective for EMH, PHT, leg ulcers, hypothyroidism, and osteoporosis. Attention should be paid to the impact of age on complications in TI, and the beneficial role of splenectomy deserves revisiting. This study provides evidence that calls for prospective evaluation of the roles of transfusion, iron chelation, and hydroxyurea therapy in TI patients.

scholarly journals the Effects of Deferasirox on Iron in Pituitary, Pancreas and Thyroid Glands: An Observational Case-Control Study

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4892-4892
Author(s):  
Sule Unal ◽  
Munevver Bas ◽  
Tuncay Hazirolan ◽  
A. Murat Tuncer ◽  
Mualla Cetin ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Iron Chelation ◽  
Thalassemia Major ◽  
Iron Accumulation ◽  
Iron Chelators ◽  
Time Interval ◽  
Iron Loading ◽  
Thyroid Glands ◽  
The Mean

Abstract In the absence of adequate chelation therapy, cardiomyopathy caused by iron overload is the leading cause of death in patients with β-thalassemia major (BTM). Additionally, more than half of the adult patients with BTM suffer from hypogonadism (HG), osteoporosis, diabetes mellitus (DM) or hypothyroidism. The use of iron chelators is the mainstay of treatment in patients with BTM to ameliorate these complications. In this study, we aimed to compare the chelation effects of deferasirox (DFX) and other iron chelators on iron in heart, liver, in addition to pituitary, pancreas and thyroid glands. The study included a total of 37 patients with BTM, who were on the same iron chelator for at least 1 year of duration and above 7 years of age. All of the patients were on iron chelation therapies with either monotherapy with DFX (n=29), desferrioxamine (n=4), deferiprone (n=1) or combination therapy of desferrioxamine and deferiprone (n=3). The mean dose of DFX was 30.8 ± 6.3 mg/kg/day (20-40), the mean dose of desferrioxamine 43.1 ± 5.3 mg/kg/day (39-50) and mean dose of deferiprone was 73.26 ± 9.45 mg/kg/day (70-90). All of the patients were compliant to chelation treatment. Cardiac T2*, hepatic T2*, thyroid T2 and R2, pituitary T2 and R2, pancreas T2* and R2* MRI were ordered twice to the patients in order to measure the accumulation of iron. The median time interval between the two MRI was 6 months (range 6-11 months). The effect of DFX (n=29) on iron measurements in different organs were compared to the effects of other chelators group (OCG) (n=8). The mean age of patients participating in the study was 20.8 ± 6.3 years (7.1-36.8). Of the study group, 7.1% of the patients had DM, 8.1% had hypothyroidism and 13.5% had HG at enrollment. According to our previous study for the cut-off value determinations for iron accumulation in BTM with comparison to healthy controls (data unpublished), all of the patients in both groups were found to have pituitary iron accumulation at initial MRI. The changes in iron measures in various organs were summarized in Table 1, indicating a decrease in cardiac, pituitary and pancreas iron loading in both drug groups in follow-up MRI’s (p>0.05). On the other hand δ Liver T2* was negative direction indicating a decrease in hepatic iron loading in DFX group, wheras positive in OCG indicating an increase in follow-up, although insignificant (Table 1, p=0.9). In both groups iron loading in thyroid was found to increase in follow-up and there was no difference between drug groups (Table 1). In conclusion, DFX is as effective as other drugs in chelation of iron from cardiac, hepatic, pituitary, pancreas and thyroid. The increase in iron in thyroid gland during follow-up in both groups may indicate that iron chelation may not be as efficient in thyroid as it is in other organs. Although, all patients had pituitary iron accumulation, only 13.5% were found to have HG, indicating that patients become symptomatic only occur after a threshold of accumulation was achieved. Our study is initiative for the measurements of iron accumulation with MRI in thyroid. Table 1. δT2* and δR2 change values between first and second MRI assessments Chelation type Mean±SD Median Range p δ Liver T2 * a (ms) Deferasirox -0.06 -8.5-7.20 0.90 Other chelators 0.79 -0.98-4.40 δ Cardiac T2 * b (ms) Deferasirox -3.83±9.5 0.88 Other chelators -3.2±8.82 δ Pituitary T2 b (ms) Deferasirox -0.7±11.3 0.09 Other chelators -1.4±6.4 δ Pituitary R2 a (Hz) Deferasirox 0.10 -6,20-3,10 0.25 Other chelators 0.20 -4,60-1,30 δ Thyroid R2 a (Hz) Deferasirox -1.4 -6,1-12,7 0.06 Other chelators -0.1 -3,80-8,1 δ Thyroid T2 a (ms) Deferasirox 4.8 -59,8-14,6 0.08 Other chelators 0.4 -20,6-20,1 δ Pancreas T2* b (ms) Deferasirox -7.46±21.6 0.99 Other chelators -7.52±9.63 δ Pancreas R2 * b (Hz) Deferasirox 9.24±45.23 0.11 Other chelators 56.4±73.3 SD: Standard Deviation; aNon-parametric variable, median values were provided; bParametric variables, mean±SD were provided. Disclosures No relevant conflicts of interest to declare.

Risk Factors for Pulmonary Hypertension In Patients with Thalassemia Intermedia

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2069-2069
Author(s):  
Mehran Karimi ◽  
Khaled M Musallam ◽  
Maria Domenica Cappellini ◽  
Amal El-Beshlawy ◽  
Khawla Belhoul ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
United Arab Emirates ◽  
Conflicts Of Interest ◽  
Previous History ◽  
Multivariate Logistic Regression Analysis ◽  
Iron Chelation ◽  
Thalassemia Intermedia ◽  
Group I ◽  
History Of ◽  
Group Ii

Abstract Abstract 2069 Introduction: Mechanisms leading to pulmonary hypertension (PHT) in patients with thalassemia intermedia (TI) are still controversial. Moreover, clinical and laboratory characteristics of patients who eventually develop PHT have not been identified. Our aim was to identify factors that characterize TI patients who develop PHT. Methods: Data was retrospectively retrieved from the Thalassemia Intermedia Registry, a database of 584 TI patients currently registered at six comprehensive care centers in Lebanon, Italy, Iran, Egypt, United Arab Emirates, and Oman. Institutional review boards at each center approved the study protocol. Two Groups of patients were identified: Group I, TI patients with documented PHT (n=64; mean age 37.3 ± 10.6; 44% males); and Group II, age- and sex-matched TI patients without PHT (n=64; mean age 37.9 ± 11.4; 44% males). Collected data included demographics, laboratory parameters, disease-complications, and received treatments that may influence PHT development and reflected the period prior to PHT occurrence. Results: There were no statistically significant differences in mean platelet counts, total or fetal hemoglobin levels between the two Groups. However, mean serum ferritin level was higher in Group I compared to Group II (1233.2 ± 499.2 vs. 654.7 ± 234.5 ng/ml; P=0.01). Moreover, mean nucleated red blood cell (NRBC) count was higher in Group I compared to Group II (354.2 ± 199.2 vs. 214.7 ± 94.5 ×10⋀6/l; P=0.03). A higher proportion of patients were splenectomized (84.4% vs. 46.9%; P<0.001) or had a previous history of thromboembolic events (40.6% vs. 7.8%; P<0.001) in Group I compared to Group II. Conversely, a higher proportion of patients received transfusion (78.1% vs. 56.2%; P<0.001), iron chelation (62.5% vs. 37.5%; P<0.001), or hydroxycarbamide (34.4% vs. 17.2%; P<0.001) therapy in Group II compared to Group I. There were no statistically significant differences in the proportion of patients with heart failure, prothrombotic mutations, or receiving antiplatelet or anticoagulant therapy between the two Groups. Multivariate logistic regression analysis revealed that patients in Group I are more likely to be splenectomized (OR:4.9, 95%CI:1.9-8.5); transfusion-naive (OR:3.5, 95%CI:2.1-6.25); on no hydroxycarbamide (OR:2.6, 95%CI:1.1-5.25) or iron chelation therapy (OR:2.3, 95%CI:1.2-4.25); and have NRBC count >300 ×10⋀6/l (OR:2.59, 95%CI:1.69-6.05) or a previous history of thromboembolism (OR:3.69, 95%CI:2.38-7.05). Conclusion: Splenectomy, previous history of thromboembolism, and a high NRBC count characterize TI patients who develop PHT. Transfusion, iron chelation, and hydroxycarbamide therapy deserve evaluation for a protective role against PHT in TI. Disclosures: No relevant conflicts of interest to declare.

Sound Production Treatment for Acquired Apraxia of Speech: An Examination of Dosage in Relation to Probe Performance

2020 ◽  
pp. 1-16
Author(s):  
Julie L. Wambaugh ◽  
Lydia Kallhoff ◽  
Christina Nessler
Keyword(s):  
Retrospective Analysis ◽  
Sound Production ◽  
Apraxia Of Speech ◽  
Practice Schedule ◽  
Practice Schedules ◽  
Baseline Performance ◽  
The Mean ◽  
Number Of Treatment ◽  
The Impact

Purpose This study was designed to examine the association of dosage and effects of Sound Production Treatment (SPT) for acquired apraxia of speech. Method Treatment logs and probe data from 20 speakers with apraxia of speech and aphasia were submitted to a retrospective analysis. The number of treatment sessions and teaching episodes was examined relative to (a) change in articulation accuracy above baseline performance, (b) mastery of production, and (c) maintenance. The impact of practice schedule (SPT-Blocked vs. SPT-Random) was also examined. Results The average number of treatment sessions conducted prior to change was 5.4 for SPT-Blocked and 3.9 for SPT-Random. The mean number of teaching episodes preceding change was 334 for SPT-Blocked and 179 for SPT-Random. Mastery occurred within an average of 13.7 sessions (1,252 teaching episodes) and 12.4 sessions (1,082 teaching episodes) for SPT-Blocked and SPT-Random, respectively. Comparisons of dosage metric values across practice schedules did not reveal substantial differences. Significant negative correlations were found between follow-up probe performance and the dosage metrics. Conclusions Only a few treatment sessions were needed to achieve initial positive changes in articulation, with mastery occurring within 12–14 sessions for the majority of participants. Earlier occurrence of change or mastery was associated with better follow-up performance. Supplemental Material https://doi.org/10.23641/asha.12592190

Morphological Analysis of the Right Ventricular Endocardial Wall in Pulmonary Hypertension

2021 ◽  
Vol 143 (7) ◽  
Author(s):  
Alifer Bordones-Crom ◽  
Sourav S. Patnaik ◽  
Prahlad G. Menon ◽  
Srinivas Murali ◽  
Ender Finol
Keyword(s):  
Pulmonary Hypertension ◽  
Mean Curvature ◽  
Main Pulmonary Artery ◽  
Magnetic Resonance Images ◽  
Pulmonary Vasculature ◽  
Heart Catheterization ◽  
L2 Norm ◽  
The Mean ◽  
The Right

Abstract Pulmonary hypertension (PH) is a chronic progressive disease diagnosed when the pressure in the main pulmonary artery, assessed by right heart catheterization (RHC), is greater than 25 mmHg. Changes in the pulmonary vasculature due to the high pressure yield an increase in the right ventricle (RV) afterload. This starts a remodeling process during which the ventricle exhibits changes in shape and eventually fails. RV models were obtained from the segmentation of cardiac magnetic resonance images at baseline and 1-year follow-up for a pilot study that involved 12 PH and 7 control subjects. The models were used to create surface meshes of the geometry and to compute the principal, mean, and Gaussian curvatures. Ten global curvature indices were calculated for each of the RV endocardial wall reconstructions at the end-diastolic volume (EDV) and end-systolic volume (ESV) phases of the cardiac cycle. Statistical analysis of the data was performed to discern if there are significant differences in the curvature indices between controls and the PH group, as well as between the baseline and follow-up phases for the PH subjects. Six curvature indices, namely, the Gaussian curvature at ESV, the mean curvature at EDV and ESV, the L2-norm of the mean curvature at ESV, and the L2-norm of the major principal curvature at EDV and ESV, were found to be significantly different between controls and PH subjects (p &lt; 0.05). We infer that these geometry measures could be used as indicators of RV endocardial wall morphology changes. Two global parameters, the Gaussian and mean curvatures at ESV, showed significant changes at the one-year follow-up for the PH subjects (p &lt; 0.05). The aforementioned geometry measures to assess changes in RV shape could be used as part of a noninvasive computational tool to aid clinicians in PH diagnostic and progression assessment, and to evaluate the effectiveness of treatment.

scholarly journals Patient Satisfaction With Extended International Normalized Ratio Follow-up Intervals in a Veteran Population

2019 ◽  
Vol 54 (5) ◽  
pp. 442-449
Author(s):  
Rebecca R. Schoen ◽  
Michael W. Nagy ◽  
Andrea L. Porter ◽  
Amanda R. Margolis
Keyword(s):  
Patient Satisfaction ◽  
Focus Group ◽  
International Normalized Ratio ◽  
Limited Data ◽  
Participant Satisfaction ◽  
Small Effect Size ◽  
The Mean ◽  
The Impact ◽  
Improve Patient

Background: For highly stable warfarin patients, limited data exists regarding patient satisfaction on extended international normalized ratio (INR) follow-up intervals and how this population compares with patients on a direct oral anticoagulant (DOAC). Objective: To assess the impact on patient satisfaction of extending INR follow-up intervals. Methods: Veterans on stable warfarin doses had extended INR follow-up intervals up to 12 weeks in a single-arm prospective cohort study for 2 years. This analysis included participants who completed at least 2 Duke Anticoagulation Satisfaction Scales (DASS). The primary outcome was the change in the DASS. A focus group described participant experiences. Participant satisfaction was compared to patients on a DOAC. Results: Of the 51 participants, 48 were included in the warfarin extended INR follow-up group. Compared with baseline, the mean DASS score (42.9 ± 12.08) was worse at 24 months (46.82 ± 15.2, P = 0.0266), with a small effect size (Cohen’s d = 0.29). The 8 participants in the focus group were satisfied with the extended INR follow-up interval but would be uncomfortable extending follow-up past 2 to 3 months. The extended INR follow-up interval study had similar DASS scores as the 33 participants included on DOAC therapy (46.8 ± 15.1, P = 0.9970) but may be limited by differing populations using DOACs. Conclusion and Relevance: For patients currently stable on warfarin therapy, extending the INR follow-up interval up to 12 weeks or changing to a DOAC does not appear to improve patient satisfaction.

scholarly journals Exercise right heart catheterisation before and after pulmonary endarterectomy in patients with chronic thromboembolic disease

2018 ◽  
Vol 52 (3) ◽  
pp. 1800458 ◽  
Author(s):  
Stefan Guth ◽  
Christoph B. Wiedenroth ◽  
Andreas Rieth ◽  
Manuel J. Richter ◽  
Ekkehard Gruenig ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Thromboembolic Disease ◽  
Pulmonary Endarterectomy ◽  
Right Heart ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation ◽  
Before And After ◽  
The Mean ◽  
Chronic Thromboembolic Disease ◽  
The Impact

Symptomatic patients with chronic thromboembolic disease (CTED) without pulmonary hypertension often show an excessive increase in mean pulmonary arterial pressure (MPAP) during exercise.We report on the impact of pulmonary endarterectomy (PEA) on pulmonary haemodynamics in a prospective series of 32 consecutive CTED patients who underwent PEA. All patients had a comprehensive diagnostic work-up including right heart catheterisation at baseline and 12 months after PEA. Furthermore, in 12 patients exercise right heart catheterisation was performed before and after PEA.After PEA, MPAP was lower at rest (20±3 versus 17±3 mmHg; p=0.008) and during maximal exercise (39±8 versus 31±6 mmHg; p=0.016). The mean total pulmonary resistance (TPR) decreased from 3.6±0.8 Wood Units (WU) pre-operatively to 2.7±0.7 WU 1 year after PEA (p=0.004) and the mean slope of the MPAP/cardiac output (CO) relationship decreased from 3.6±1.0 to 2.3±0.8 WU (p=0.002). Peak oxygen uptake increased from 1.2±0.4 to 1.5±0.3 L·min−1 (p=0.014) and ventilatory equivalents of carbon dioxide decreased from 39±2 to 30±2 (p=0.002). There was a significant improvement in quality of life assessed by the Cambridge Pulmonary Hypertension Outcome Review questionnaire.In CTED patients, PEA resulted in haemodynamic and clinical improvements. The means of TPR and MPAP/CO slopes decreased to <3.0 WU.

Discordant GH and IGF-1 Results in Treated Acromegaly: Impact of GH Cutoffs and Mean Values Assessment

2020 ◽  
Author(s):  
Claudia Campana ◽  
Francesco Cocchiara ◽  
Giuliana Corica ◽  
Federica Nista ◽  
Marica Arvigo ◽  
...  
Keyword(s):  
Characteristic Curve ◽  
Area Under The Curve ◽  
Receiver Operator Characteristic Curve ◽  
Mean Values ◽  
Pituitary Diseases ◽  
Discordance Rate ◽  
Values Assessment ◽  
The Mean ◽  
The Impact

Abstract Context Discordant growth hormone (GH) and insulin-like growth factor-1 (IGF-1) values are frequent in acromegaly. Objective To evaluate the impact of different GH cutoffs on discordance rate. To investigate whether the mean of consecutive GH measurements impacts discordance rate when matched to the last available IGF-1 value. Design Retrospective study. Setting Referral center for pituitary diseases. Patients Ninety acromegaly patients with at least 3 consecutive evaluations for GH and IGF-1 using the same assay in the same laboratory (median follow-up 13 years). Interventions Multimodal treatment of acromegaly. Main Outcome Measures Single fasting GH (GHf) and IGF-1 (IGF-1f). Mean of 3 GH measurements (GHm), collected during consecutive routine patients’ evaluations. Results At last evaluation GHf values were 1.99 ± 2.79 µg/L and age-adjusted IGF-1f was 0.86 ± 0.44 × upper limit of normality (mean ± SD). The discordance rate using GHf was 52.2% (cutoff 1 µg/L) and 35.6% (cutoff 2.5 µg/L) (P = 0.025). “High GH” discordance was more common for GHf &lt;1.0 µg/L, while “high IGF-1” was predominant for GHf &lt;2.5 µg/L (P &lt; 0.0001). Using GHm mitigated the impact of GH cutoffs on discordance (GHm &lt;1.0 µg/L: 43.3%; GHm &lt;2.5 µg/L: 38.9%; P = 0.265). At receiver-operator characteristic curve (ROC) analysis, both GHf and GHm were poor predictors of IGF-1f normalization (area under the curve [AUC] = 0.611 and AUC = 0.645, respectively). The prevalence of disease-related comorbidities did not significantly differ between controlled, discordant, and active disease patients. Discussion GH/IGF-1 discordance strongly depends on GH cutoffs. The use of GHm lessen the impact of GH cutoffs. Measurement of fasting GH levels (both GHf and GHm) is a poor predictor of IGF-1f normalization in our cohort.

scholarly journals P298 The long-term impact of persistent pulmonary hypertension in patients undergoing TAVR with a self-expanding valve

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Drakopoulou ◽  
S Soulaidopoulos ◽  
G Oikonomou ◽  
P Toskas ◽  
M Xanthopoulou ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Aortic Valve ◽  
Mitral Regurgitation ◽  
Ejection Fraction ◽  
Confidence Intervals ◽  
Hazard Ratio ◽  
Cumulative Mortality ◽  
Risk Of Death ◽  
The Impact

Abstract Background Persistent severe pulmonary hypertension (PH) is considered to negatively affect early and late outcomes of patients undergoing aortic valve surgery. There is limited data however, cincerning the incidence of persistent PH after transcatheter aortic valve replacement (TAVR) and its impact on outcome is limited. Purpose We sought to investigate the impact of persistent PH on clinical outcomes of patients undergoing TAVR with a self-expanding valve. Methods Consecutive patients with severe symptomatic aortic stenosis scheduled for TAVR in our tertiary center were included in the study. Prospectively collected data before and after TAVR were retrospectively analyzed in all patients. Severe PH was defined as systolic pulmonary arterial pressure (sPAP) ≥45mmHg as assessed by echocardiography. For analysis purposes, patients with a sPAP decrease after TAVR to below 45mmHg were compared to patients with persistent PH following TAVR. All outcomes were evaluated according to the VARC-2 criteria. Results In total, 258 patients were included in this study (mean age 80.06 ± 7.50 years old, logEuroscore 24.50 ± 9.70%, NYHA III/IV Class 98.6%). Of these, 149 (57.8%) had sPAP less than 45mmHg and 109 (42.2%) had sPAP above or equal to 45mmHg at baseline. Patients with severe PH were older (81.1 ± 7.0 vs 79.1 ± 7.7, p = 0.034), presented with higher logEuroscore (26.9 ± 9.3% vs 22.5 ± 9.9%, p&lt; 0.001), lower ejection fraction (47.9 ± 9.3% vs 52.2 ± 8.5%, p&lt; 0.001) and higher rates of at least moderate mitral regurgitation (36.7% vs 16.2%, p = 0.002) compared to the group without PH. After TAVR, 161 (62.4%) patients had sPAP less than 45mmHg and 97 (37.6%) had sPAP above 45mmHg. There was a significant decrease of 2.4 ± 12.2mmHg in sPAP post TAVR (p &lt; 0.01). Multivariable analysis (univariate analysis: age, logEuroscore, pre TAVR mitral regurgitation, pre TAVR ejection fraction below 40%) identified pre TAVR ejection fraction below 40% to be the most powerful predictor for persistent PH after TAVR (odds ratio 2.4, 95% confidence interval 1.0.9 – 5.26, p = 0.028). During a mean follow up period of 26.6 ± 26.8, the presence of pre TAVR severe PH was not found to be predictive of cumulative mortality[Hazard Ratio(HR) : 1.57, 95% Confidence Intervals (CI) 0.92 – 2.66, p = 0.09). However, in the same follow up period, patients with persistent PH after TAVR had higher cumulative risk of death compared to patients with sPAP &lt; 45mmHg after TAVR (Hazard Ratio 0.49, 95% Confidence Intervals 0.29-0.82, p = 0.007) (Figure). Conclusions Our data suggest that TAVR is associated with a significant reduction in sPAP. Persistent PH post TAVR seems to be a predictor of higher cumulative mortality post TAVR. Abstract P298 Figure.

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