- Email: [email protected]
Reversal of Cardiac Allograft Dysfunction with Medical Therapy
S88
Journal of Cardiac Failure Vol. 24 No. 8S August 2018
232 Optimizing Pulmonary Artery Pressures with use of Ivabradine in Cardiomyopathy Stephanie Preister, Ladene Case, Julie Deibert, Orvar Jonsson; Sanford Cardiovascular Institute, Sioux Falls, SD Case report: Patient is a 42 yr old male with past medical history of chronic systolic heart failure, nonischemic cardiomyopathy since 2011, felt to be secondary to chronic prescription anabolic steroid use, EF 10-15%, history of cardiac arrest due to hyperkalemia, ICD single-chamber, hypertension, and chronic kidney disease stage III. Based on right heart cath and cardiopulmonary stress test, the patient is too good for advanced heart failure therapies. He is a NYHA II and is managed on carvedilol 25mg po BID, lisinopril 5 mg po daily, ivabradine 7.5mg po BID. On 2/13/18, patient ran out of his ivabradine. The following day his heart rate started to increase to the mid 90s from a baseline of 70s . It took two days for his diastolic pulmonary artery pressure (PAd) to increase above his set threshold of 15-20mmHg after stopping the ivabradine. He was able to restart ivabradine on 2/20/18 and his heart rate came back to the 70s and his PAd dropped back below 20 mmHg. Conclusion: Optimizing PAd can be influenced by many factors including hypervolemia, hypovolemia, blood pressure, and heart rate. In many cases, optimizing heart failure medications such as the betablockers and angiotension converting enzyme inhibitors can help optimize PAd, however controlling blood pressure and heart rate are important also. In this case study, using ivabradine to reduce heart rate shows a significant improvement in PAd without adjusting diuretics or other heart failure medications.
233 Isolated Right Ventricular Cardiac Sarcoidosis Presenting as Complete Heart Block Katherine Dodd; The Ohio State Wexner Medical Center, Columbus, OH Introduction: Sarcoidosis is a multi-organ disease with formation of granulomas. Involvement of the heart causes inflammation, leading to atrioventricular
block (AVB) or ventricular arrhythmias, and portends a poor prognosis. Cardiac sarcoidosis is detected in only 2% of patients, and most have left ventricular (LV) involvement. Isolated right ventricular (RV) involvement is rare. High suspicion is necessary to make the diagnosis, and advanced testing is required. Recognizing cardiac sarcoid is imited to the RV and determining presence of pulmonary hypertension (PH), is imperative for management. Case Presentation: A 53 yo woman presented with dyspnea and palpitations. She has a history of complete heart block (CHB) requiring permanent pacemaker placement, and atrial arrhythmias. On evaluation, BP 153/81 mmHg and HR 71 bpm. Physical exam was normal. Given clinical history and refractory dyspnea, there was concern for an inflammatory process. Cardiac monitoring revealed frequent premature ventricular contractions (PVCs) and non-sustained ventricular tachycardia (NSVT). An echocardiogram demonstrated normal valvular and LV function, but the RV was severely dilated with moderate dysfunction. A right -heart catheterization (RHC) demonstrated normal pulmonary artery pressures and pulmonary capillary wedge pressure. A nuclear PET scan showed areas of increased FDG uptake within the RV, distal anteroseptal LV and pericardium, concerning for sarcoidosis. An endomyocardial biopsy (EMB) demonstrated multinucleated giant cells, confirming sarcoidosis. The patient underwent radiofrequency ablation for AFL. Her device was upgraded to a defibrillator. She was initiated on Cellcept. Serial echocardiograms revealed no PH and stable RV size and function. Case Discussion: The diagnosis of cardiac sarcoidosis is elusive due to non-specific signs and symptoms. It often presents with CHB, ventricular arrhythmias or sudden death, atrial arrhythmias, or heart failure. Our patient was diagnosed with CHB, then went on to develop atrial and ventricular arrhythmias, and RV failure. The majority of patients with cardiac involvement have LV involvement. There are only a few case reports of isolated RV involvement. Since lone RV cardiac sarcoidosis is uncommon, diagnosis is delayed. Fortunately in our patient, once sarcoidosis was confirmed to be primarily involving the RV, a RHC and serial transthoracic echocardiograms were performed to rule out PH, which would influence treatment. Conclusion: Cardiac sarcoidosis is rare, identified in only 25% of patients with systemic sarcoidosis, and has a poor prognosis. A high degree of suspicion is warranted, especially among young patients with arrhythmias of unclear etiology. Further investigation with serologic ACE levels, imaging such as cardiac magnetic resonance imaging (CMR) or nuclear PET scan, and EMB should be done. Finally, periodic echocardiograms and RHCs are necessary to ensure PH does not develop, which has implications for management.
234 Reversal of Cardiac Allograft Dysfunction with Medical Therapy Luke Cunningham, Shelley Hall, Joost Felius, Parag Kale; Baylor University Medical Center, Dallas, TX Background: Acute cellular rejection (ACR) among heart transplant recipients is common with up to 20% to 40% of patients developing an episode in the first year. Yet, stability is the norm after the first year. Late graft dysfunction is less common and can result in the need for re-transplantation. We present a case in which medical therapy was used in lieu and resulted in resolution of graft dysfunction. Case report: A 48 year old man who underwent orthotopic heart transplant in July 2014 had an uncomplicated early transplant course with negative endomyocardial biopsy (EMBx) for allograft rejection through 21 months. At that time he presented with dyspnea, lower extremity swelling and excess sinus tachycardia (140 bpm). Given new heart failure symptoms, rejection evaluation was completed, including EMBx. Pathology revealed International Society for Heart and Lunt Transplantation (ISHLT) grade 2R ACR. Staining for antibody mediated rejection was negative. An echocardiogram (TTE) revealed a decline in allograft function from 70% to 40% with restrictive filling pattern. Treatment with anti-thymocyte globulin, high dose prednisone taper, plasmapheresis and bortezomib was given. Despite treatment the patient returned for heart failure hospitalization 3 months later. Right heart catheterization (RHC) showed pulmonary capillary wedge pressure of 24 mmHg, mean right atrial pressure of 12 mmHg and cardiac index of 1.9 L/min/m^2. Due to reluctance on the part of the patient to immediately consider re-transplantation, intravenous milrinone and titration of guideline-directed medical therapy was completed. Fortunately, the graft ejection fraction normalized on subsequent TTE and he was successfully weaned off milrinone over 5 months. Repeat RHC showed normal filling pressures and normal cardiac index. Conclusions: Patients who develop graft dysfunction with cellular rejection may continue to experience residual graft dysfunction after treatment. Continued medical therapy with standard heart failure regimen and inotropes helped support this patient during decompensation. This case suggests the possibility of late allograft recovery with medical support and avoidance of re-transplantation. Further research is needed regarding effective medical management of graft dysfunction.
The 22nd Annual Scientific Meeting HFSA
S89
required HD after LVAD implantation. These 7 patients had a total of 92 HD sessions during their hospitalizations. The average age of the patients was 46 +/- 11.9 years old. Two patients had ESRD and were on HD prior to LVAD implantation. The remaining five patients had CKD but were not on HD. The pre-LVAD creatinine average for all 7 patients was 2.55 +/- 1.8. After LVAD implantation, all of the patients were initially on continuous renal replacement therapy before being transitioned to HD. HD was started anywhere from within 2 to 57 days after LVAD implantation. Results: LVAD settings were typically not adjusted during HD sessions. Additionally, during HD all patients were on midodrine, vasopressors, or fludrocortisone to help increase blood pressure. There were seven HD sessions that required early termination due to symptomatic hypotension (3), asymptomatic hypotension (2), sinus tachycardia and cramping and discomfort. The average duration of all HD sessions was 172 minutes and the average duration of HD sessions that were terminated early was 148 minutes. Low flow LVAD alarms did not activate during any of the sessions. Conclusion: 7 out of 92 sessions required early termination and the most common reasons (5 out of 7) were related to hypotension. Complications related to blood pressure was also found to be the most common reason for termination of HD in previous study of HD sessions in Heartmate LVAD patients. As a result of continuous-flow technology, in most LVAD recipients, there is an absence of pulse, precluding the standard assessment of blood pressure, making assessment of blood pressure during dialysis difficult. There needs to be more research on how to address this in addition to how to optimize and change LVAD settings during HD sessions.
237 The Bionic Man: Surgical and Percutaneous Advances in Heart Failure and Dtransposition of the Great Arteries Siu-Hin Wan, Peter A. Noseworthy; Mayo Clinic, Rochester, MN
235 Lethal Combination of Clenbuterol and Taurine in a Young Body-Builder Siu-Hin Wan, Yong-Mei Cha; Mayo Clinic, Rochester, MN Background: Sudden cardiac death risk from stimulants clenbuterol and taurine for athletes is unknown. Description: A 30-year-old body-builder who took clenbuterol and taurine for one week and was otherwise healthy without any cardiovascular disease was found unresponsive, requiring five external defibrillations for ventricular fibrillation (VF) to restore sinus rhythm. The left ventricular ejection fraction was 22% immediately after resuscitation and improved to 49% in 3 days. Coronary angiogram did not show any coronary artery disease and cardiac-magnetic-resonance was unremarkable. ECG showed no abnormal findings. Discussion: Clenbuterol, a performance-enhancing drug, is a beta-2 agonist that increases fat metabolism and aerobic capacity and can cause hypertension and tachycardia. Taurine, found in energy drinks, can cause ventricular arrhythmias. Clenbuterol depletes taurine, so athletes take these in combination. While many people take sports supplements, few have sudden death. The VF is likely attributed to unknown underlying myocardial vulnerability and is precipitated by the combination of clenbuterol and taurine. Subcutaneous implantable cardioverter defibrillator (ICD) was placed in this patient for secondary prevention of sudden cardiac death. Conclusion: Clenbuterol and taurine, used as performance-enhancing drugs in athletes, may trigger VF and heart failure in those with predisposing substrate.
236 Adverse Events During Hemodialysis in LVAD Recipients Tapati Stalam, Ami Patel; University of Maryland Medical Center, BALTIMORE, MD Introduction: Over the past decade, implantable left ventricular assist devices (LVAD) have become an acceptable alternative to support patients with advanced heart failure, either as a bridge to transplantation or as destination therapy for patients who are not candidates for heart transplantation. As the number of LVAD implantation increases, greater number of LVAD recipients requiring long term renal replacement therapy (RRT), usually chronic hemodialysis (HD), will be seen. This is due to common simultaneous chronic kidney disease in patients with heart failure, but more frequently due to irreversible acute kidney injury occurring in the peri-implantation period. There though is limited data on HD in LVAD recipients. We sought to assess adverse events that required early termination of HD sessions in HeartWare LVAD recipients while they were hospitalized after LVAD implantation. Methods: Between 2015 and 2017 at the University of Maryland Medical Center, 7 patients
Background: Modern advances in surgery and percutaneous interventions have resulted in new therapeutic possibilities for end-stage heart failure in congenital heart disease. We present a case of a patient with D-transposition of the great arteries undergoing multiple surgeries and percutaneous interventions for advanced heart failure. Description: A 44-year-old man with D-transposition of the great arteries underwent a Blalock-Hanlon septectomy at age 9 days and a Mustard atrial switch procedure at age 3 years. He received an epicardial ventricular pacemaker at age 12 years for high grade atrioventricular (AV) block. He developed supraventricular tachycardia ten years ago, received electrophysiology ablation, and was found to have an inferior vena cava (IVC) baffle leak for which he underwent Amplatzer occluder device placement. Four years ago, he developed superior vena cava (SVC) and IVC baffle stenosis and underwent dilation and covered stent placements in the SVC and IVC baffles. His pacemaker was upgraded to a biventricular implantable cardioverter defibrillator (ICD) utilizing the epicardial ventricular lead and an endocardial lead through the covered stent in the SVC baffle for primary prevention of sudden cardiac death. Due to worsening heart failure despite inotropic support, the patient underwent systemic HeartWare ventricular assist device (VAD) implantation as a bridge to transplantation. Discussion: The chest X-ray shows a HeartWare ventricular assist device attached to the systemic right ventricle. The biventricular ICD includes endocardial right-atrial lead, ICD lead in the non-systemic left-ventricle, and two epicardial ventricular leads. Multiple sternotomy wires, endotracheal tube and right internal jugular catheter are seen. The stented superior and inferior vena cava baffles and Amplatzer closure device for baffle leak project over the mediastinum. Conclusion: Significant advances in surgery and percutaneous interventions allow for complex therapeutic options for patients with congenital heart disease and advanced heart failure.
Journal of Cardiac Failure Vol. 24 No. 8S August 2018
232 Optimizing Pulmonary Artery Pressures with use of Ivabradine in Cardiomyopathy Stephanie Preister, Ladene Case, Julie Deibert, Orvar Jonsson; Sanford Cardiovascular Institute, Sioux Falls, SD Case report: Patient is a 42 yr old male with past medical history of chronic systolic heart failure, nonischemic cardiomyopathy since 2011, felt to be secondary to chronic prescription anabolic steroid use, EF 10-15%, history of cardiac arrest due to hyperkalemia, ICD single-chamber, hypertension, and chronic kidney disease stage III. Based on right heart cath and cardiopulmonary stress test, the patient is too good for advanced heart failure therapies. He is a NYHA II and is managed on carvedilol 25mg po BID, lisinopril 5 mg po daily, ivabradine 7.5mg po BID. On 2/13/18, patient ran out of his ivabradine. The following day his heart rate started to increase to the mid 90s from a baseline of 70s . It took two days for his diastolic pulmonary artery pressure (PAd) to increase above his set threshold of 15-20mmHg after stopping the ivabradine. He was able to restart ivabradine on 2/20/18 and his heart rate came back to the 70s and his PAd dropped back below 20 mmHg. Conclusion: Optimizing PAd can be influenced by many factors including hypervolemia, hypovolemia, blood pressure, and heart rate. In many cases, optimizing heart failure medications such as the betablockers and angiotension converting enzyme inhibitors can help optimize PAd, however controlling blood pressure and heart rate are important also. In this case study, using ivabradine to reduce heart rate shows a significant improvement in PAd without adjusting diuretics or other heart failure medications.
233 Isolated Right Ventricular Cardiac Sarcoidosis Presenting as Complete Heart Block Katherine Dodd; The Ohio State Wexner Medical Center, Columbus, OH Introduction: Sarcoidosis is a multi-organ disease with formation of granulomas. Involvement of the heart causes inflammation, leading to atrioventricular
block (AVB) or ventricular arrhythmias, and portends a poor prognosis. Cardiac sarcoidosis is detected in only 2% of patients, and most have left ventricular (LV) involvement. Isolated right ventricular (RV) involvement is rare. High suspicion is necessary to make the diagnosis, and advanced testing is required. Recognizing cardiac sarcoid is imited to the RV and determining presence of pulmonary hypertension (PH), is imperative for management. Case Presentation: A 53 yo woman presented with dyspnea and palpitations. She has a history of complete heart block (CHB) requiring permanent pacemaker placement, and atrial arrhythmias. On evaluation, BP 153/81 mmHg and HR 71 bpm. Physical exam was normal. Given clinical history and refractory dyspnea, there was concern for an inflammatory process. Cardiac monitoring revealed frequent premature ventricular contractions (PVCs) and non-sustained ventricular tachycardia (NSVT). An echocardiogram demonstrated normal valvular and LV function, but the RV was severely dilated with moderate dysfunction. A right -heart catheterization (RHC) demonstrated normal pulmonary artery pressures and pulmonary capillary wedge pressure. A nuclear PET scan showed areas of increased FDG uptake within the RV, distal anteroseptal LV and pericardium, concerning for sarcoidosis. An endomyocardial biopsy (EMB) demonstrated multinucleated giant cells, confirming sarcoidosis. The patient underwent radiofrequency ablation for AFL. Her device was upgraded to a defibrillator. She was initiated on Cellcept. Serial echocardiograms revealed no PH and stable RV size and function. Case Discussion: The diagnosis of cardiac sarcoidosis is elusive due to non-specific signs and symptoms. It often presents with CHB, ventricular arrhythmias or sudden death, atrial arrhythmias, or heart failure. Our patient was diagnosed with CHB, then went on to develop atrial and ventricular arrhythmias, and RV failure. The majority of patients with cardiac involvement have LV involvement. There are only a few case reports of isolated RV involvement. Since lone RV cardiac sarcoidosis is uncommon, diagnosis is delayed. Fortunately in our patient, once sarcoidosis was confirmed to be primarily involving the RV, a RHC and serial transthoracic echocardiograms were performed to rule out PH, which would influence treatment. Conclusion: Cardiac sarcoidosis is rare, identified in only 25% of patients with systemic sarcoidosis, and has a poor prognosis. A high degree of suspicion is warranted, especially among young patients with arrhythmias of unclear etiology. Further investigation with serologic ACE levels, imaging such as cardiac magnetic resonance imaging (CMR) or nuclear PET scan, and EMB should be done. Finally, periodic echocardiograms and RHCs are necessary to ensure PH does not develop, which has implications for management.
234 Reversal of Cardiac Allograft Dysfunction with Medical Therapy Luke Cunningham, Shelley Hall, Joost Felius, Parag Kale; Baylor University Medical Center, Dallas, TX Background: Acute cellular rejection (ACR) among heart transplant recipients is common with up to 20% to 40% of patients developing an episode in the first year. Yet, stability is the norm after the first year. Late graft dysfunction is less common and can result in the need for re-transplantation. We present a case in which medical therapy was used in lieu and resulted in resolution of graft dysfunction. Case report: A 48 year old man who underwent orthotopic heart transplant in July 2014 had an uncomplicated early transplant course with negative endomyocardial biopsy (EMBx) for allograft rejection through 21 months. At that time he presented with dyspnea, lower extremity swelling and excess sinus tachycardia (140 bpm). Given new heart failure symptoms, rejection evaluation was completed, including EMBx. Pathology revealed International Society for Heart and Lunt Transplantation (ISHLT) grade 2R ACR. Staining for antibody mediated rejection was negative. An echocardiogram (TTE) revealed a decline in allograft function from 70% to 40% with restrictive filling pattern. Treatment with anti-thymocyte globulin, high dose prednisone taper, plasmapheresis and bortezomib was given. Despite treatment the patient returned for heart failure hospitalization 3 months later. Right heart catheterization (RHC) showed pulmonary capillary wedge pressure of 24 mmHg, mean right atrial pressure of 12 mmHg and cardiac index of 1.9 L/min/m^2. Due to reluctance on the part of the patient to immediately consider re-transplantation, intravenous milrinone and titration of guideline-directed medical therapy was completed. Fortunately, the graft ejection fraction normalized on subsequent TTE and he was successfully weaned off milrinone over 5 months. Repeat RHC showed normal filling pressures and normal cardiac index. Conclusions: Patients who develop graft dysfunction with cellular rejection may continue to experience residual graft dysfunction after treatment. Continued medical therapy with standard heart failure regimen and inotropes helped support this patient during decompensation. This case suggests the possibility of late allograft recovery with medical support and avoidance of re-transplantation. Further research is needed regarding effective medical management of graft dysfunction.
The 22nd Annual Scientific Meeting HFSA
S89
required HD after LVAD implantation. These 7 patients had a total of 92 HD sessions during their hospitalizations. The average age of the patients was 46 +/- 11.9 years old. Two patients had ESRD and were on HD prior to LVAD implantation. The remaining five patients had CKD but were not on HD. The pre-LVAD creatinine average for all 7 patients was 2.55 +/- 1.8. After LVAD implantation, all of the patients were initially on continuous renal replacement therapy before being transitioned to HD. HD was started anywhere from within 2 to 57 days after LVAD implantation. Results: LVAD settings were typically not adjusted during HD sessions. Additionally, during HD all patients were on midodrine, vasopressors, or fludrocortisone to help increase blood pressure. There were seven HD sessions that required early termination due to symptomatic hypotension (3), asymptomatic hypotension (2), sinus tachycardia and cramping and discomfort. The average duration of all HD sessions was 172 minutes and the average duration of HD sessions that were terminated early was 148 minutes. Low flow LVAD alarms did not activate during any of the sessions. Conclusion: 7 out of 92 sessions required early termination and the most common reasons (5 out of 7) were related to hypotension. Complications related to blood pressure was also found to be the most common reason for termination of HD in previous study of HD sessions in Heartmate LVAD patients. As a result of continuous-flow technology, in most LVAD recipients, there is an absence of pulse, precluding the standard assessment of blood pressure, making assessment of blood pressure during dialysis difficult. There needs to be more research on how to address this in addition to how to optimize and change LVAD settings during HD sessions.
237 The Bionic Man: Surgical and Percutaneous Advances in Heart Failure and Dtransposition of the Great Arteries Siu-Hin Wan, Peter A. Noseworthy; Mayo Clinic, Rochester, MN
235 Lethal Combination of Clenbuterol and Taurine in a Young Body-Builder Siu-Hin Wan, Yong-Mei Cha; Mayo Clinic, Rochester, MN Background: Sudden cardiac death risk from stimulants clenbuterol and taurine for athletes is unknown. Description: A 30-year-old body-builder who took clenbuterol and taurine for one week and was otherwise healthy without any cardiovascular disease was found unresponsive, requiring five external defibrillations for ventricular fibrillation (VF) to restore sinus rhythm. The left ventricular ejection fraction was 22% immediately after resuscitation and improved to 49% in 3 days. Coronary angiogram did not show any coronary artery disease and cardiac-magnetic-resonance was unremarkable. ECG showed no abnormal findings. Discussion: Clenbuterol, a performance-enhancing drug, is a beta-2 agonist that increases fat metabolism and aerobic capacity and can cause hypertension and tachycardia. Taurine, found in energy drinks, can cause ventricular arrhythmias. Clenbuterol depletes taurine, so athletes take these in combination. While many people take sports supplements, few have sudden death. The VF is likely attributed to unknown underlying myocardial vulnerability and is precipitated by the combination of clenbuterol and taurine. Subcutaneous implantable cardioverter defibrillator (ICD) was placed in this patient for secondary prevention of sudden cardiac death. Conclusion: Clenbuterol and taurine, used as performance-enhancing drugs in athletes, may trigger VF and heart failure in those with predisposing substrate.
236 Adverse Events During Hemodialysis in LVAD Recipients Tapati Stalam, Ami Patel; University of Maryland Medical Center, BALTIMORE, MD Introduction: Over the past decade, implantable left ventricular assist devices (LVAD) have become an acceptable alternative to support patients with advanced heart failure, either as a bridge to transplantation or as destination therapy for patients who are not candidates for heart transplantation. As the number of LVAD implantation increases, greater number of LVAD recipients requiring long term renal replacement therapy (RRT), usually chronic hemodialysis (HD), will be seen. This is due to common simultaneous chronic kidney disease in patients with heart failure, but more frequently due to irreversible acute kidney injury occurring in the peri-implantation period. There though is limited data on HD in LVAD recipients. We sought to assess adverse events that required early termination of HD sessions in HeartWare LVAD recipients while they were hospitalized after LVAD implantation. Methods: Between 2015 and 2017 at the University of Maryland Medical Center, 7 patients
Background: Modern advances in surgery and percutaneous interventions have resulted in new therapeutic possibilities for end-stage heart failure in congenital heart disease. We present a case of a patient with D-transposition of the great arteries undergoing multiple surgeries and percutaneous interventions for advanced heart failure. Description: A 44-year-old man with D-transposition of the great arteries underwent a Blalock-Hanlon septectomy at age 9 days and a Mustard atrial switch procedure at age 3 years. He received an epicardial ventricular pacemaker at age 12 years for high grade atrioventricular (AV) block. He developed supraventricular tachycardia ten years ago, received electrophysiology ablation, and was found to have an inferior vena cava (IVC) baffle leak for which he underwent Amplatzer occluder device placement. Four years ago, he developed superior vena cava (SVC) and IVC baffle stenosis and underwent dilation and covered stent placements in the SVC and IVC baffles. His pacemaker was upgraded to a biventricular implantable cardioverter defibrillator (ICD) utilizing the epicardial ventricular lead and an endocardial lead through the covered stent in the SVC baffle for primary prevention of sudden cardiac death. Due to worsening heart failure despite inotropic support, the patient underwent systemic HeartWare ventricular assist device (VAD) implantation as a bridge to transplantation. Discussion: The chest X-ray shows a HeartWare ventricular assist device attached to the systemic right ventricle. The biventricular ICD includes endocardial right-atrial lead, ICD lead in the non-systemic left-ventricle, and two epicardial ventricular leads. Multiple sternotomy wires, endotracheal tube and right internal jugular catheter are seen. The stented superior and inferior vena cava baffles and Amplatzer closure device for baffle leak project over the mediastinum. Conclusion: Significant advances in surgery and percutaneous interventions allow for complex therapeutic options for patients with congenital heart disease and advanced heart failure.