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Riociguat for Chronic Thromboembolic Pulmonary Hypertension - Initial Experience
The 19th Annual Scientific Meeting noncompliance rate documented at approximately 80%. Purpose: The Refractory Heart Failure Clinic (RHFC) was established with the mission to provide comprehensive, multidisciplinary medical care to patients with severe or refractory heart failure requiring hospital admission for HF exacerbation. The goals are to improve patients’ quality of life and minimize HF exacerbations readmissions by enhancing patients’ adherence to diet and medications; daily Home Telehealth monitoring of HF sign and symptoms; and increase access to provider care. Clinic admission criteria include: patients with New York Heart Association Class III or IV CHF with one to two hospital admissions for CHF exacerbation within 6 months and ejection fraction (EF) of less than 40%. The RHFC consist of a medical director, a nurse practitioner, a clinical pharmacy specialist, a clinical dietitian and a home telehealth nurse. Appointments are provided in two half day clinics per week with walk-in clinic appointments available. Results: Data collected include age, gender, ethnicity, HF hospital readmission prior to clinic enrollment and post clinic enrollment, number of HFC visits, and patients’ adherence to clinic visits. Clinical data included EF, B-type Natriuretic Peptide (BNP) and pharmacotherapy prescribed. Sixty patients have been enrolled since clinic establishment. Patients were all male, age range 46-92 years (average 69.9 years); BNP range 119 to 28533 pg/ml (average 7800pg/ml). The majority of the patients, 75 percent, had an EF less than 30%. Medication classes prescribed and titrated included Beta Blockers, Angiotensin Converting Enzyme inhibitors and Angiotensin Receptor Blockers (ACE-I/ARB), Aldosterone Antagonists, diuretics, digoxin, nitrates and hydralazine. Fifteen patients had biventricular implantable cardioverter defibrillators. Prior to enrollment the mean time for HF readmission was 5.7 months and post clinic enrollment the mean time was 7.7 months, a decrease of 2 months. Prior to enrollment there were a total of 136 HF admissions; after enrollment there were a total of 62 HF admissions, an overall reduction of 46% in readmission rate and a cost reduction of approximately $1 million to Hines VA. Conclusion: Per Centers for Medicare and Medicaid Service (CMS) data, the mean cost per CHF readmission is $13,000. The cost of a hospital bed at the VA averages $2,000-$5,000 per day. Our review of data has shown we have been able to extend mean time to readmission by 35%. HF readmissions affect patients and their caregivers, and is a financial problem for the institution. Although ours is a small clinic, the outcomes data have shown a reduction in HF readmissions and significant cost savings.
155 Inpatient Diuretic Dose as a Predictor of Mortality and Re-Admission in Heart Failure Katherine Picel, Peter Eckman; University of Minnesota, Minneapolis, MN Background and Objective: Diuretics are the cornerstone of decongestion therapy for heart failure patients, but increased dose has been associated with increased mortality in the chronic, outpatient setting. There is little data on the association of diuretic dose and mortality in patients admitted for acute decompensated heart failure. We hypothesized that increased diuretic dose would be associated with increased inpatient mortality and rate of readmission for acute decompensated heart failure. Methods: Electronic medical charts for patients admitted with a heart failure exacerbation diagnosis from April 2011 through March 2012 at a single, tertiary academic medical center were examined. The primary end points were re-admission within 30 days, and mortality at 1 year from admission. Total diuretic dose administered were
Figure 1.
HFSA
S71
Figure 2.
summed and converted to furosemide equivalents in mg/kg/day. Results: There were 197 admissions (104 male, 93 female) with average length of stay 6.4 days, average furosemide equivalents 1.45 mg/kg/day, average EF 35%, average admission creatinine 1.6, average admission blood urea nitrogen 37, and average age 66.7 years. There were 64 deaths at 1 year from admission, and 64 patients re-admitted within 30 days of discharge. When diuretic dose was plotted against each of the primary end points, no significant correlation was found. Conclusion: The results of this retrospective chart review do not support the hypothesis that higher inpatient doses of diuretic correlate with increase in mortality or re-admission. However, multivariate analysis to control for confounding baseline characteristics such as renal function might provide a clearer view of diuretic dose as an independent factor. Advancing understanding of the role diuretics play in heart failure exacerbation would help to provide more insightful discussion and appropriate follow-up for this high-risk population of patients.
156 Riociguat for Chronic Thromboembolic Pulmonary Hypertension - Initial Experience Ruba Haddad1, Lisa Mielniczuk1, Carolyn Pugliese1, George Chandy2, Duncan Stewart3, Vladimir Contreras-Dominguez2, Ross Davies1; 1University of Ottawa Heart Institute, Ottawa, ON, Canada; 2The Ottawa Hospital, Ottawa, ON, Canada; 3 The Ottawa Hospital Research Institute, Ottawa, ON, Canada Background: Riociguat is a member of a new class of compounds called soluble guanylate cyclase stimulators. Randomized clinical trials established its role in the treatment of chronic thromboembolic pulmonary hypertension (WHO type 4 PH or CTEPH) either inoperable or with residual pulmonary hypertension after pulmonary thromboendarterectomy (PTE) and also in pulmonary arterial hypertension (WHO type 1 PAH) as published in the New England Journal of Medicine in July 2013. Riociguat (Adempas, Bayer Canada Inc.) was approved by Health Canada in October 2013. We describe our initial experience in 12 patients with CTEPH treated with riociguat. Methods and Results: Patients were followed in the pulmonary hypertension clinic at the University of Ottawa Heart Institute. Baseline characteristics included age of 53 6 13.8 years (mean + SD, range 33-81), male gender in 7, previous PTE in 3 and New York Heart Association Functional Class (NYHA FC) class III in 11 and class II in 1. Baseline 6 minute walk test distance was an average of 324.8 6 117 meters. All patients were on anticoagulants with warfarin in 10 patients, dalteparin in 1 patient and apixaban in 1 patient; 1 was on a calcium channel blocker; and 8 were on furosemide at a mean dose of 45 6 29.7 mg/day. The starting dose of riociguat was 0.5 mg and was up titrated by 0.5 mg every 2 weeks to an average dose of 5.3 6 3.2 mg/ day. The average duration of riociguat therapy was 53.8 6 41.5 weeks. On follow up, 11 patients demonstrated significant clinical improvement to NYHA functional class II and 1 patient remained in functional class II (p50.0001). Although there was improvement in the 6 minute walk test, this was not statistically significant. Side effects encountered included diarrhea in one patient. At this point we have not encountered headache, hypotension, anemia, dyspepsia, vomiting or constipation. Conclusion: Riociguat is a member of a promising new class of medications for PH. Our initial clinical experience with riociguat in CTEPH patients, although the number of patients is small, looks promising with improvement
S72 Journal of Cardiac Failure Vol. 21 No. 8S August 2015 in functional class and is consistent with initial publications. Riociguat has been well tolerated so far and we have only encountered early mild side effects in one patient. Table 1.
Parameter Riociguat dose (mg/day, mean 6 SD) Duration (weeks) New York Heart Association Functional Class 6 minute walk test (meters)
Pre Riociguat Post Riociguat P Value 5.3 6 3.2 Class III: 11 Class II: 1 324.8 6 117
53.8 6 41.5 Class ll: 1 Class II: 11 355 6 96
0.0001
patients with a high edema index had poorer outcomes than those with a low edema index, unadjusted hazard ratio (HR) 1.80, (95% CI 1.07-3.04). This was true whether edema index was looked at by low versus high values (Figure 1) or by quartiles. However, in multivariate analyses, a high edema index was not an independent predictor of outcomes, adjusted HR 0.87 (95% CI 0.40-1.90). In this cohort, the only independent predictors of event survival free survival were lower NYHA Class and BNP. Conclusions: A high edema index by BIA using a bioimpedance scale in a HF clinic correlates with adverse patient outcomes. Once other measures of HF severity such as BNP and NYHA Class are accounted for however, this index is no longer a significant predictor of outcomes. As a non-invasive measure of volume status, use of BIA in a HF clinic may be useful in determining patient prognosis and treatment when other outcome predictors are not immediately available.
Non significant
157 Increased Risk of LVAD Thrombosis in Patients With a Previous History Thromboembolic Event Scott Lundgren; Univeristy of Nebraska Medical Center, Omaha, NE Introduction: Heart failure is a clinical syndrome that affects more than 5.1 million Americans and over 23 million individuals worldwide. With the likely substantial growth in the incidence and prevalence of heart failure patients over the next 2040 years, there will also likely be a corresponding increasein the need for left ventricular assist devices (LVADs). Complications occur and consist primarily of infection, stroke or other thromboembolic event, device malfunction, bleeding, and pump thrombosis. Since LVAD thrombosis can have devastating consequences, including central or peripheral thromboembolism, LVAD malfunction or failure with resultant cardiogenic shock, and death, early identification of pump thrombosis as well as identification of those patients at increased risk for thrombosis prior to implantation is exceedingly important. In this study, we evaluate whether previous history of thromboembolic event increases the risk for LVAD pump thrombosis. Methods: This study was a single center, retrospective analysis of 45 patients who underwent implantation of a HeartMate II (Thoratec Corp., Pleasanton, California) left ventricular-assist device between January 2009 and January 2015. Of the 45 patients, 15 patients with known or suspected pump thrombosis were identified from a total of 172 patients who have been implanted with a LVAD at The University of Nebraska Medical Center since January 2009. The other 30 patients were controls matched to the thrombosed patients based off of sex, age, and days post-LVAD insertion. Outcomes were compared between groups using multivariate analysis. Results: A total of 15 patients out of 172 (8.7%) were identified as known or suspected LVAD thrombus. Of the 15 with thrombosis, 10/15 (67%) had a previous history of thromboembolic event compared to 10/30 (33%) in the control group (OR 4.0; 95% CI 1.07-14.90; p ! 0.05). Of the 15 patients with thrombosis, 5 underwent device exchange, 3 underwent heart transplant, 6 received medical management with IV anticoagulation and/or intra-pump thrombolytics, and 1 patient had their device explanted. History of atrial fibrillation, history of diabetes mellitus, subtherpeutic INR, BMI, or pump speed were not significantly different between groups. Conclusions: With the rapid increase in the utilization of left ventricular assist devices in the management of advanced heart failurepatients, the identification of patients at high risk for adverse events priorto implantation is crucial. This study indicates that patients who have had a previous thromboembolic event of any kind are at increased risk for LVAD thrombosis. These patients would likely benefit from a hypercoagulable workup prior to implantation, higher levels of anticoagulation/antiplatelets (ie INR target 2.5-3.5, high antiplatelet doses, or bridging these patients when their INRs become sub-therapeutic), or a combination of both.
158 Non-Invasive Bioelectrical Impedance for Predicting Clinical Outcomes in Outpatients with Heart Failure Kristin J. Lyons, Michelle K. Bischoff, Gregg C. Fonarow, Tamara B. Horwich; University of California Los Angeles, Los Angeles, CA Purpose: To assess if an abnormal (high) edema index measurement is a predictor of mortality in an outpatient population with HF. Methods: BIA assessment of edema index was performed in 359 outpatients with HF using InBody 520 (Biospace Inc, CA), an 8-point tactile electrode system employing 5, 50, and 500 kHz. Edema index was calculated from dividing extracellular water values by total body water values. Patients were stratified into those with a low (#0.39) and those with a high (O0.39) edema index. Cox regression was used to examine differences in patient outcomes after adjustment for key variables. The outcome of interest was survival free from urgent transplant or ventricular assist device (VAD). Results: Mean follow up was 25 months. The mean patient age was 56 (614) years, the mean left ventricular ejection fraction (LVEF) was 36% (616), and 28% of patients were female. Compared to patients with a low edema index, those with a high edema index were older, had lower body weights and body mass indices, had higher BNPs and New York Heart Association (NYHA) Class and were more likely to have ischemic cardiomyopathy. There were no differences in sex, medication use, or LVEF. When comparing cumulative survival free from urgent transplant or VAD placement,
Figure 1. Cumulative Patient Event Free Survival.
159 Role of Iron Chelation Therapy in Cardiac Hemosiderosis: A Case Series Abdullah AlMasoud, Ahmad Younes, Sadeer AlKindi, Chantal Elamm, Guilherme Oliveira, Robert Schilz, Jane Little, Mahazarin Ginwalla; University Hospitals Case Medical Center, Cleveland, OH Background: Cardiac hemosiderosis has been observed in adult sickle cell patients after multiple blood transfusions. It is not clear if aggressive iron chelation therapy can reverse the iron deposition and improve left ventricular function in patients with systolic dysfunction. Methods: Cardiovascular magnetic resonance (CMR) imaging was used as a quantitative tool to evaluate for iron deposition by T2-weighted imaging sequences. We identified three patients with sickle cell disease who had cardiac hemosiderosis by CMR. Two of these patients also had left ventricular systolic dysfunction as determined by echocardiography. These patients were treated with iron chelation therapy. All three had elevated ferritin levels O1000. These patients were evaluated by echocardiography and/or CMR post iron chelation. Results: Patient 1 had an improvement in left ventricular ejection fraction from 27% to 45% after 4 months of iron chelation therapy with deferiprone. This was associated with a decrease in ferritin levels from 1905 ug/L to 1292 ug/L. Patient 2 had an improvement in left ventricular ejection fraction from 53% to 66% after 12 months of iron chelation therapy with Deferasirox. Her T2* on CMR improved from 25 ms to 33 ms, and her ferritin levels decreased from 1534 ug/L to 855 ug/L. Patient 3 had advanced sickle cell disease with evidence of multiorgan failure and was initiated on exchange transfusions in addition to iron chelation with deferoxamine. She had heart failure with preserved ejection fraction with a left ventricular ejection fraction of 57%. However, her T2* on CMR showed minimal change from 16 ms to 15 ms after 20 months of iron chelation therapy with deferoxamine. Her ferritin levels decreased from 12736 ug/L to 5658 ug/L. Conclusions: Aggressive early iron chelation therapy can reverse cardiac hemosiderosis, improve left ventricular systolic function, and improve symptoms in patients with sickle cell disease. CMR can be used to document presence of cardiac iron, and to monitor for improvement in cardiac function and iron deposition after chelation therapy. Patients with severe hemosiderosis and very high ferritin levels may not respond favorably to iron chelation therapy.
155 Inpatient Diuretic Dose as a Predictor of Mortality and Re-Admission in Heart Failure Katherine Picel, Peter Eckman; University of Minnesota, Minneapolis, MN Background and Objective: Diuretics are the cornerstone of decongestion therapy for heart failure patients, but increased dose has been associated with increased mortality in the chronic, outpatient setting. There is little data on the association of diuretic dose and mortality in patients admitted for acute decompensated heart failure. We hypothesized that increased diuretic dose would be associated with increased inpatient mortality and rate of readmission for acute decompensated heart failure. Methods: Electronic medical charts for patients admitted with a heart failure exacerbation diagnosis from April 2011 through March 2012 at a single, tertiary academic medical center were examined. The primary end points were re-admission within 30 days, and mortality at 1 year from admission. Total diuretic dose administered were
Figure 1.
HFSA
S71
Figure 2.
summed and converted to furosemide equivalents in mg/kg/day. Results: There were 197 admissions (104 male, 93 female) with average length of stay 6.4 days, average furosemide equivalents 1.45 mg/kg/day, average EF 35%, average admission creatinine 1.6, average admission blood urea nitrogen 37, and average age 66.7 years. There were 64 deaths at 1 year from admission, and 64 patients re-admitted within 30 days of discharge. When diuretic dose was plotted against each of the primary end points, no significant correlation was found. Conclusion: The results of this retrospective chart review do not support the hypothesis that higher inpatient doses of diuretic correlate with increase in mortality or re-admission. However, multivariate analysis to control for confounding baseline characteristics such as renal function might provide a clearer view of diuretic dose as an independent factor. Advancing understanding of the role diuretics play in heart failure exacerbation would help to provide more insightful discussion and appropriate follow-up for this high-risk population of patients.
156 Riociguat for Chronic Thromboembolic Pulmonary Hypertension - Initial Experience Ruba Haddad1, Lisa Mielniczuk1, Carolyn Pugliese1, George Chandy2, Duncan Stewart3, Vladimir Contreras-Dominguez2, Ross Davies1; 1University of Ottawa Heart Institute, Ottawa, ON, Canada; 2The Ottawa Hospital, Ottawa, ON, Canada; 3 The Ottawa Hospital Research Institute, Ottawa, ON, Canada Background: Riociguat is a member of a new class of compounds called soluble guanylate cyclase stimulators. Randomized clinical trials established its role in the treatment of chronic thromboembolic pulmonary hypertension (WHO type 4 PH or CTEPH) either inoperable or with residual pulmonary hypertension after pulmonary thromboendarterectomy (PTE) and also in pulmonary arterial hypertension (WHO type 1 PAH) as published in the New England Journal of Medicine in July 2013. Riociguat (Adempas, Bayer Canada Inc.) was approved by Health Canada in October 2013. We describe our initial experience in 12 patients with CTEPH treated with riociguat. Methods and Results: Patients were followed in the pulmonary hypertension clinic at the University of Ottawa Heart Institute. Baseline characteristics included age of 53 6 13.8 years (mean + SD, range 33-81), male gender in 7, previous PTE in 3 and New York Heart Association Functional Class (NYHA FC) class III in 11 and class II in 1. Baseline 6 minute walk test distance was an average of 324.8 6 117 meters. All patients were on anticoagulants with warfarin in 10 patients, dalteparin in 1 patient and apixaban in 1 patient; 1 was on a calcium channel blocker; and 8 were on furosemide at a mean dose of 45 6 29.7 mg/day. The starting dose of riociguat was 0.5 mg and was up titrated by 0.5 mg every 2 weeks to an average dose of 5.3 6 3.2 mg/ day. The average duration of riociguat therapy was 53.8 6 41.5 weeks. On follow up, 11 patients demonstrated significant clinical improvement to NYHA functional class II and 1 patient remained in functional class II (p50.0001). Although there was improvement in the 6 minute walk test, this was not statistically significant. Side effects encountered included diarrhea in one patient. At this point we have not encountered headache, hypotension, anemia, dyspepsia, vomiting or constipation. Conclusion: Riociguat is a member of a promising new class of medications for PH. Our initial clinical experience with riociguat in CTEPH patients, although the number of patients is small, looks promising with improvement
S72 Journal of Cardiac Failure Vol. 21 No. 8S August 2015 in functional class and is consistent with initial publications. Riociguat has been well tolerated so far and we have only encountered early mild side effects in one patient. Table 1.
Parameter Riociguat dose (mg/day, mean 6 SD) Duration (weeks) New York Heart Association Functional Class 6 minute walk test (meters)
Pre Riociguat Post Riociguat P Value 5.3 6 3.2 Class III: 11 Class II: 1 324.8 6 117
53.8 6 41.5 Class ll: 1 Class II: 11 355 6 96
0.0001
patients with a high edema index had poorer outcomes than those with a low edema index, unadjusted hazard ratio (HR) 1.80, (95% CI 1.07-3.04). This was true whether edema index was looked at by low versus high values (Figure 1) or by quartiles. However, in multivariate analyses, a high edema index was not an independent predictor of outcomes, adjusted HR 0.87 (95% CI 0.40-1.90). In this cohort, the only independent predictors of event survival free survival were lower NYHA Class and BNP. Conclusions: A high edema index by BIA using a bioimpedance scale in a HF clinic correlates with adverse patient outcomes. Once other measures of HF severity such as BNP and NYHA Class are accounted for however, this index is no longer a significant predictor of outcomes. As a non-invasive measure of volume status, use of BIA in a HF clinic may be useful in determining patient prognosis and treatment when other outcome predictors are not immediately available.
Non significant
157 Increased Risk of LVAD Thrombosis in Patients With a Previous History Thromboembolic Event Scott Lundgren; Univeristy of Nebraska Medical Center, Omaha, NE Introduction: Heart failure is a clinical syndrome that affects more than 5.1 million Americans and over 23 million individuals worldwide. With the likely substantial growth in the incidence and prevalence of heart failure patients over the next 2040 years, there will also likely be a corresponding increasein the need for left ventricular assist devices (LVADs). Complications occur and consist primarily of infection, stroke or other thromboembolic event, device malfunction, bleeding, and pump thrombosis. Since LVAD thrombosis can have devastating consequences, including central or peripheral thromboembolism, LVAD malfunction or failure with resultant cardiogenic shock, and death, early identification of pump thrombosis as well as identification of those patients at increased risk for thrombosis prior to implantation is exceedingly important. In this study, we evaluate whether previous history of thromboembolic event increases the risk for LVAD pump thrombosis. Methods: This study was a single center, retrospective analysis of 45 patients who underwent implantation of a HeartMate II (Thoratec Corp., Pleasanton, California) left ventricular-assist device between January 2009 and January 2015. Of the 45 patients, 15 patients with known or suspected pump thrombosis were identified from a total of 172 patients who have been implanted with a LVAD at The University of Nebraska Medical Center since January 2009. The other 30 patients were controls matched to the thrombosed patients based off of sex, age, and days post-LVAD insertion. Outcomes were compared between groups using multivariate analysis. Results: A total of 15 patients out of 172 (8.7%) were identified as known or suspected LVAD thrombus. Of the 15 with thrombosis, 10/15 (67%) had a previous history of thromboembolic event compared to 10/30 (33%) in the control group (OR 4.0; 95% CI 1.07-14.90; p ! 0.05). Of the 15 patients with thrombosis, 5 underwent device exchange, 3 underwent heart transplant, 6 received medical management with IV anticoagulation and/or intra-pump thrombolytics, and 1 patient had their device explanted. History of atrial fibrillation, history of diabetes mellitus, subtherpeutic INR, BMI, or pump speed were not significantly different between groups. Conclusions: With the rapid increase in the utilization of left ventricular assist devices in the management of advanced heart failurepatients, the identification of patients at high risk for adverse events priorto implantation is crucial. This study indicates that patients who have had a previous thromboembolic event of any kind are at increased risk for LVAD thrombosis. These patients would likely benefit from a hypercoagulable workup prior to implantation, higher levels of anticoagulation/antiplatelets (ie INR target 2.5-3.5, high antiplatelet doses, or bridging these patients when their INRs become sub-therapeutic), or a combination of both.
158 Non-Invasive Bioelectrical Impedance for Predicting Clinical Outcomes in Outpatients with Heart Failure Kristin J. Lyons, Michelle K. Bischoff, Gregg C. Fonarow, Tamara B. Horwich; University of California Los Angeles, Los Angeles, CA Purpose: To assess if an abnormal (high) edema index measurement is a predictor of mortality in an outpatient population with HF. Methods: BIA assessment of edema index was performed in 359 outpatients with HF using InBody 520 (Biospace Inc, CA), an 8-point tactile electrode system employing 5, 50, and 500 kHz. Edema index was calculated from dividing extracellular water values by total body water values. Patients were stratified into those with a low (#0.39) and those with a high (O0.39) edema index. Cox regression was used to examine differences in patient outcomes after adjustment for key variables. The outcome of interest was survival free from urgent transplant or ventricular assist device (VAD). Results: Mean follow up was 25 months. The mean patient age was 56 (614) years, the mean left ventricular ejection fraction (LVEF) was 36% (616), and 28% of patients were female. Compared to patients with a low edema index, those with a high edema index were older, had lower body weights and body mass indices, had higher BNPs and New York Heart Association (NYHA) Class and were more likely to have ischemic cardiomyopathy. There were no differences in sex, medication use, or LVEF. When comparing cumulative survival free from urgent transplant or VAD placement,
Figure 1. Cumulative Patient Event Free Survival.
159 Role of Iron Chelation Therapy in Cardiac Hemosiderosis: A Case Series Abdullah AlMasoud, Ahmad Younes, Sadeer AlKindi, Chantal Elamm, Guilherme Oliveira, Robert Schilz, Jane Little, Mahazarin Ginwalla; University Hospitals Case Medical Center, Cleveland, OH Background: Cardiac hemosiderosis has been observed in adult sickle cell patients after multiple blood transfusions. It is not clear if aggressive iron chelation therapy can reverse the iron deposition and improve left ventricular function in patients with systolic dysfunction. Methods: Cardiovascular magnetic resonance (CMR) imaging was used as a quantitative tool to evaluate for iron deposition by T2-weighted imaging sequences. We identified three patients with sickle cell disease who had cardiac hemosiderosis by CMR. Two of these patients also had left ventricular systolic dysfunction as determined by echocardiography. These patients were treated with iron chelation therapy. All three had elevated ferritin levels O1000. These patients were evaluated by echocardiography and/or CMR post iron chelation. Results: Patient 1 had an improvement in left ventricular ejection fraction from 27% to 45% after 4 months of iron chelation therapy with deferiprone. This was associated with a decrease in ferritin levels from 1905 ug/L to 1292 ug/L. Patient 2 had an improvement in left ventricular ejection fraction from 53% to 66% after 12 months of iron chelation therapy with Deferasirox. Her T2* on CMR improved from 25 ms to 33 ms, and her ferritin levels decreased from 1534 ug/L to 855 ug/L. Patient 3 had advanced sickle cell disease with evidence of multiorgan failure and was initiated on exchange transfusions in addition to iron chelation with deferoxamine. She had heart failure with preserved ejection fraction with a left ventricular ejection fraction of 57%. However, her T2* on CMR showed minimal change from 16 ms to 15 ms after 20 months of iron chelation therapy with deferoxamine. Her ferritin levels decreased from 12736 ug/L to 5658 ug/L. Conclusions: Aggressive early iron chelation therapy can reverse cardiac hemosiderosis, improve left ventricular systolic function, and improve symptoms in patients with sickle cell disease. CMR can be used to document presence of cardiac iron, and to monitor for improvement in cardiac function and iron deposition after chelation therapy. Patients with severe hemosiderosis and very high ferritin levels may not respond favorably to iron chelation therapy.