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End-Stage Biventricular Failure from Necrotizing Autoimmune Myopathy
The 22nd Annual Scientific Meeting HFSA
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Performance Improvement Initiatives / Case Studies 212 Progressive Cardiomyopathy in TTR Amyloidosis after Liver Transplant Jenna Kay1, Jonathan Menachem2, Rupal O’Quinn1, Brian Drachman1; 1Hospital of the University of Pennsylvania, Philadelphia, PA; 2Vanderbilt University, Nashville, TN Introduction: Familial transthyretin (TTR) amyloidosis is a rare disorder characterized primarily by progressive neuropathy and cardiomyopathy. The source of amyloid protein is the liver, thus early liver transplantation can significantly slow disease progression. Unfortunately, for some patients without significant cardiomyopathy at the time of transplant, cardiac disease can subsequently progress due to enhanced deposition of wild-type amyloid protein. Promising new medical therapies may soon replace liver transplant as the gold standard treatment. Case report: A 67 year-old male with bilateral carpal tunnel syndrome presented with hematuria and was found to have bladder masses that stained positive for amyloidosis. A work-up for systemic amyloidosis included a normal bone marrow biopsy and an echocardiogram with LVH in the absence of increased voltage on ECG. Two years later he had a cardiac arrest in the setting of sustained VT. A left heart catheterization revealed normal coronary arteries. His endomyocardial biopsy Congo red stain was positive. He was diagnosed with a previously undiscovered mutation of familial TTR amyloidosis (Glu61Gly) and five years later underwent liver transplantation. He was NYHA Class I and did well for ten years before his functional capacity gradually declined. Serial echocardiograms showed a slow progression of LV wall thickness from 1.3 to 1.7 cm and a decline in LVEF from 50% to 30%. In addition, the defibrillation threshold of his ICD progressively increased. He underwent heart transplantation due to end stage amyloid cardiomyopathy. Postoperatively he developed ulcerative esophagitis and ultimately expired due to fungal pericarditis. Discussion: Although liver transplantation can slow the progression of TTR amyloidosis and is an appropriate treatment for select individuals, new alternative medical therapies may replace early transplantation. Diflunisal, an anti-inflammatory drug, stabilizes the TTR tetramer in vitro and in a randomized trial slowed progression of neuropathy in familial TTR. Combined doxycycline and tauroursodeoxycholic acid disrupt TTR amyloid fibrils and appear to slow disease progression. Tafamadis, a small molecule that inhibits the dissociation of TTR tetramers has been shown in phase 2 and 3 trials to slow neuropathy progression and decrease mortality and heart failure hospitalizations. Patisiran is a small interfering RNA that blocks TTR synthesis and has demonstrated stabilization of neuropathy. Inotersen is an antisense oligonucleotide that has also shown promise in familial TTR patients with polyneuropathy. Most recently, an investigational monoclonal antibody has been designed to target and remove amyloid deposits. Conclusion: Familial TTR amyloidosis is characterized by progressive neuropathy and cardiomyopathy for which patients may undergo liver transplant, though cardiac disease can subsequently progress. Promising new medical therapies may soon replace liver transplant as the gold standard.
213 Don’t Go Breaking My Heart Again: A Case of Recurrent Myocarditis Tishangi Kumar, Christina L. Dunbar Matos; Carilion Clinic, Roanoke, VA Background: Myocarditis refers to inflammation of the heart muscle due to infectious and non-infectious triggers.The most common infectious pathogens are coxsackievirus, adenovirus and parvovirus B-19. Myocarditis is the third most common cause of sudden cardiac death - identified in 8.6% to 12% of autopsies. We present a case of recurrent myocarditis, in a patient with a history of coxsackie myocarditis and the importance of cardiac magnetic resonance (CMR) in its diagnosis. Case report: 45-year-old female with a history of coxsackie myocarditis - occurred in 2012 and again in 2014. During both episodes patient underwent a coronary angiogram that revealed normal coronary arteries (FIG 1).In 2014, the angiogram noted hypokinesis of the mid-ventricle with an estimated left ventricular function of 40%. Prior treatments included Aspirin and Colchicine. In 2017, she presented to the emergency department with sharp chest pain radiating to the back. Associated symptoms included two-week history of chills, sore throat and cervical lymphadenopathy. Initial vital signs and physical exam were unremarkable. A 12 lead electrocardiogram showed sinus rhythm with septal q waves. Laboratory results were notable for mildly elevated troponin at 0.134, erythrocyte sedimentation rate at 32 and C-reactive protein at 1.05. Cardiac magnetic resonance (CMR) revealed left ventricular systolic function of 40% with hypokinesis and T2 enhancement of the mid-septum and the mid lateral segments (FIG 2) - there was no late gadolinium enhancement (LGE). Based on this information a diagnosis of focal myocarditis was made. She was successfully treated with Aspirin 81mg, Colchicine 0.6mg twice per day, Metoprolol Succinate 25mg daily and a two week tapering dose of Prednisone 20mg daily (for suspected underlying pericarditis component). Discussion: Myocarditis is an under diagnosed cardiac disease that carries high morbidity and mortality. Diagnosis remains challenging due to nonspecific clinical signs and symptoms and low accuracy of endomyocardial biopsy. However, CMR can provide information about cardiac anatomy and tissue characterization. The presence and extent of LGE and abnormal T2 weighted imaging carry prognostic implication in predicting major cardiovascular events. This case highlights the importance of CMR in diagnosis and risk stratification of chronic myocarditis.
214 End-Stage Biventricular Failure from Necrotizing Autoimmune Myopathy Janet I. Ma, Nicholas Wettersten, Paul Kim, Eric Adler, Marcus A. Urey; University of California San Diego, San Diego, CA Necrotizing autoimmune myopathy is a rare neuromuscular disorder characterized clinically by severe proximal limb muscle weakness and pathologically by necrotic muscle fibers with minimal inflammation. It is often associated with statin therapy, connective tissue diseases, and malignancy. Most have characteristic autoantibodies although a small subset present without myositis-specific autoantibodies. While cardiac involvement is rare, conduction abnormalities, diastolic dysfunction, and wall motion abnormalities have been reported. Severe cardiomyopathy from necrotizing
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Journal of Cardiac Failure Vol. 24 No. 8S August 2018
autoimmune myopathy resulting in end-stage biventricular failure is extremely rare without a clear role for guideline-directed medical therapy. A 45-year-old man with hypothyroidism was admitted with several months of worsening proximal muscular weakness. He was found to have new severe biventricular dysfunction with conduction abnormalities during diagnostic work-up. Physical exam was notable for symmetric proximal muscle weakness in upper and lower extremities, sclerodactyly, elevated jugular venous distension, and lower extremity edema. Laboratory testing showed a creatine kinase of 3101 U/L, ANA 1:640, positive RNP antibodies, positive PM/Scl 100 antibodies. Anti-HMGCR antibodies and anti-SRP antibodies were negative. Lower extremity MRI showed diffuse bilateral symmetric myositis around the pelvis consistent with an inflammatory myopathy. EMG suggested irritable myopathy. Left thigh skeletal muscle biopsy showed necrotizing autoimmune myopathy with a paucity of inflammatory cells. Electrocardiogram revealed a right bundle branch block and 1st degree AV block. Frequent atrial and ventricular ectopic beats were seen on telemetry. Transthoracic echocardiogram showed biventricular failure with LVEF 23% and severe global hypokinesis. Cardiac MRI showed patchy foci of delayed subendocardial delayed enhancement. Endomyocardial biopsy did not reveal evidence of lymphocytic infiltration or ongoing myocarditis. Right heart catheterization was notable for elevated biventricular pressures with a cardiac index of 2.18. No obstructive coronary artery disease was seen on coronary angiogram. He was treated with intravenous immunoglobulins, methotrexate, and high-dose prednisone with improvement in proximal weakness. Low-dose spironolactone and lisinopril were started but could not be increased due to degree of hypotension. This case highlights a rare manifestation of an exceedingly rare autoimmune condition associated with high mortality. Given the variability of cardiac involvement in the disorder, a high clinical suspicion is needed to establish an early diagnosis and pursue aggressive treatment with up-front combination immunomodulating therapy, which has been associated with improved outcomes.
Figure 1. A: CMR demonstrates pericardial effusion (black asterisk) and subendocardial enhancement (white arrows). B: Eosinophilic infiltration of endomyocardium (Black arrows). C: Hypercellular Bone marrow.
215 Multi-modality Imaging in a Hypereosinophilic Syndrome with Cardiac Involvement Karuppiah Arunachalam, Charles Beale, Liz Edmund, Michael Atalay, Daniel Levine, Karen Aspry, Rayan Yousefzai; Warren Alpert Medical School of Brown University, Providence, RI Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare, multi-system auto-immune vasculitic disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. Cardiac involvement is the primary contributor to mortality. Case Report: Mr. W is a 53-year-old man with a history of asthma and environmental allergies presented to the emergency department with sudden onset of blurred vision in his left eye and evidence of optic neuritis. He endorsed a 2-week history of night sweats, arthralgias, anorexia, transient ankle edema, and a recent rash. His exam was notable for purpuric lesions in extremities and left visual field deficits. Laboratory data showed a WBC’s of 36,000 cells/mm3 with 69% eosinophils, CRP of 63 mg/dl, ESR of 122 mm/min, IgE of 1,741 IU/ml, and troponin of 11 ng/ml. An Electrocardiogram showed low voltage and q waves in anteroseptal leads. An infectious work-up was negative.A brain MRI revealed acute punctate infarcts. A transthoracic echocardiogram (TTE) revealed a severely dilated left ventricle with an ejection fraction of 25%. Cardiac catheterization showed normal coronary arteries, elevated filling pressures, and normal cardiac output. Cardiac Magnetic Resonance (CMR) showed moderate to severe biventricular dysfunction, a moderate pericardial effusion, and patchy bi-ventricular subendocardial enhancement (Figure 1A). So he underwent endomyocardial biopsy, which showed increased perivascular eosinophils/eosinophilic degranulation and fibrin (Figure 1B).
The differential diagnosis was EGPA, hypersensitivity myocarditis or a myeloproliferative variant of hypereosinophilic syndrome. A bone marrow biopsy was performed which showed hypereosinophilia (Figure 1C), without evidence of malignancy, confirming EGPA. He was treated with high dose steroids with a prolonged taper and cyclophosphamide, which rapidly resolved his eosinophilia. A repeat TTE 7 weeks later showed near normalization of cardiac function and resolution of pericardial fluid. Conclusion: Cardiac involvement in EGPA is up to 60% and early development of myocardial fibrosis. Prompt recognition via multi-modality imaging and endomyocardial biopsy and rapid treatment with high dose steroids or other immunotherapies, may prevent early progression to dilated or restrictive cardiomyopathy.
216 Specialized Resident Force in Internal Medicine Residency Program Helps Modify Risk of Heart Failure Readmissions: A Performance Improvement Project T.S. Dharmarajan1, S. Singh2, N. Bulcha2, S. Virdi2, R. Gali2, D. Yang2, S. Venkatraman2, S. Saleem2, G. Bhardwaj2, S. Zareef2, Ke Di Palo2, P. Shah2; 1Montefiore Medical Center (Wakefield Campus), Scarsdale, NY; 2Montefiore Medical Center, Bronx, NY Introduction: Heart Failure (HF) is a leading cause of hospitalization, mortality and health care costs in the U.S. HF is the most common cause of readmissions in Medicare patients, with >20% readmitted within 30 days of hospital discharge. Several interventions have been tried to lower readmissions involving patient, provider and related disciplines, without significant long-term impact. Hypothesis: Development of a specialized team of internal medicine residents trained to educate and counsel HF patients, will enhance disease state knowledge of the disease, improve self-care and reduce hospital readmissions among patients admitted with acute decompensated HF (ADHF). Methods: In August 2017, a team of 9 Internal Medicine residents were recruited to create a Specialized Resident Force (SRF). The residents received individual hands-on training sessions in counseling HF patients and met with a mentor weekly initially, later monthly. Patients on 2 selected medicine units with a high volume of HF admissions were counseled by the SRF on diet, activity, medication use, weight monitoring and outpatient follow-up. Demographic data, comorbidities and HF specific data was gathered and compared to patients receiving standard education. Patients were evaluated for cognition and advance directives. In the cognitively impaired, education was offered to the caregiver, if available. A tool was developed to document data; a booklet on HF education and a 2 page easy to use script to help counsel patients at bedside was provided to each resident. Results: Enhanced SRF education was provided to 44 patients between September and December 2017. Education provided by the SRF resulted in 55.7% reduction in the 30 day all cause readmission rate (see Table 1) Observations by the mentor suggested that knowledge base of SRF trainees improved as a result of participation. Conclusions: Specialized internal medicine resident teams providing bedside education to patients with ADHF may favorably influence HF readmissions, while simultaneously enhancing resident knowledge base on HF management; Academic medical centers can easily incorporate resident driven patient education into existing readmission reduction efforts TABLE 1. Bedside Eduction
Admissions
Readmissions
Readmission Rate
Standard care (usual care: nurses, attendings Enhanced (SRF driven)
78
19
25.7%
44
5
11.36%
217 Symmetrical Peripheral Gangrene Associated with Low Output Cardiac Failure Sijan Basnet1, Priya Rajagopalan2, Rashmi Dhital1, Ataul Qureshi2; 1Reading Hospital, West Reading, PA; 2Jefferson Medical College, Philadelphia, PA Introduction: Symmetrical peripheral gangrene (SPG) is a rare clinical condition with acute, symmetrical ischemia of two or more extremities leading to gangrene in the absence of large vessel obstruction or vasculitis. We present a case of SPG presenting in the setting of severely depressed cardiac ejection fraction. Case Description: Patient is a 64-year-old man referred to our emergency department from an outside hospital for management of liver failure and bilateral necrotic toes. His past medical history and surgical history were unremarkable. He drank 3-4 beers and 2-3 glasses of hard liquor daily. He had dry and gangrenous second through fifth toes and densely cyanotic first toes bilaterally. (Figure 1) Dorsalis pedis and posterior tibialis pulses were present bilaterally on palpation. His liver function test (LFT) was deranged (Total bilirubin: 35.0 mg/dL, direct bilirubin: >10.0 mg/dL, AST: 309 IU/L, ALT: 295 IU/L). Echo showed ejection fraction of 10 percent with global hypokinesis and severe left ventricular enlargement but no embolus. His brain natriuretic peptide was 18,984 pg/mL (reference range: < 125 pg/
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Performance Improvement Initiatives / Case Studies 212 Progressive Cardiomyopathy in TTR Amyloidosis after Liver Transplant Jenna Kay1, Jonathan Menachem2, Rupal O’Quinn1, Brian Drachman1; 1Hospital of the University of Pennsylvania, Philadelphia, PA; 2Vanderbilt University, Nashville, TN Introduction: Familial transthyretin (TTR) amyloidosis is a rare disorder characterized primarily by progressive neuropathy and cardiomyopathy. The source of amyloid protein is the liver, thus early liver transplantation can significantly slow disease progression. Unfortunately, for some patients without significant cardiomyopathy at the time of transplant, cardiac disease can subsequently progress due to enhanced deposition of wild-type amyloid protein. Promising new medical therapies may soon replace liver transplant as the gold standard treatment. Case report: A 67 year-old male with bilateral carpal tunnel syndrome presented with hematuria and was found to have bladder masses that stained positive for amyloidosis. A work-up for systemic amyloidosis included a normal bone marrow biopsy and an echocardiogram with LVH in the absence of increased voltage on ECG. Two years later he had a cardiac arrest in the setting of sustained VT. A left heart catheterization revealed normal coronary arteries. His endomyocardial biopsy Congo red stain was positive. He was diagnosed with a previously undiscovered mutation of familial TTR amyloidosis (Glu61Gly) and five years later underwent liver transplantation. He was NYHA Class I and did well for ten years before his functional capacity gradually declined. Serial echocardiograms showed a slow progression of LV wall thickness from 1.3 to 1.7 cm and a decline in LVEF from 50% to 30%. In addition, the defibrillation threshold of his ICD progressively increased. He underwent heart transplantation due to end stage amyloid cardiomyopathy. Postoperatively he developed ulcerative esophagitis and ultimately expired due to fungal pericarditis. Discussion: Although liver transplantation can slow the progression of TTR amyloidosis and is an appropriate treatment for select individuals, new alternative medical therapies may replace early transplantation. Diflunisal, an anti-inflammatory drug, stabilizes the TTR tetramer in vitro and in a randomized trial slowed progression of neuropathy in familial TTR. Combined doxycycline and tauroursodeoxycholic acid disrupt TTR amyloid fibrils and appear to slow disease progression. Tafamadis, a small molecule that inhibits the dissociation of TTR tetramers has been shown in phase 2 and 3 trials to slow neuropathy progression and decrease mortality and heart failure hospitalizations. Patisiran is a small interfering RNA that blocks TTR synthesis and has demonstrated stabilization of neuropathy. Inotersen is an antisense oligonucleotide that has also shown promise in familial TTR patients with polyneuropathy. Most recently, an investigational monoclonal antibody has been designed to target and remove amyloid deposits. Conclusion: Familial TTR amyloidosis is characterized by progressive neuropathy and cardiomyopathy for which patients may undergo liver transplant, though cardiac disease can subsequently progress. Promising new medical therapies may soon replace liver transplant as the gold standard.
213 Don’t Go Breaking My Heart Again: A Case of Recurrent Myocarditis Tishangi Kumar, Christina L. Dunbar Matos; Carilion Clinic, Roanoke, VA Background: Myocarditis refers to inflammation of the heart muscle due to infectious and non-infectious triggers.The most common infectious pathogens are coxsackievirus, adenovirus and parvovirus B-19. Myocarditis is the third most common cause of sudden cardiac death - identified in 8.6% to 12% of autopsies. We present a case of recurrent myocarditis, in a patient with a history of coxsackie myocarditis and the importance of cardiac magnetic resonance (CMR) in its diagnosis. Case report: 45-year-old female with a history of coxsackie myocarditis - occurred in 2012 and again in 2014. During both episodes patient underwent a coronary angiogram that revealed normal coronary arteries (FIG 1).In 2014, the angiogram noted hypokinesis of the mid-ventricle with an estimated left ventricular function of 40%. Prior treatments included Aspirin and Colchicine. In 2017, she presented to the emergency department with sharp chest pain radiating to the back. Associated symptoms included two-week history of chills, sore throat and cervical lymphadenopathy. Initial vital signs and physical exam were unremarkable. A 12 lead electrocardiogram showed sinus rhythm with septal q waves. Laboratory results were notable for mildly elevated troponin at 0.134, erythrocyte sedimentation rate at 32 and C-reactive protein at 1.05. Cardiac magnetic resonance (CMR) revealed left ventricular systolic function of 40% with hypokinesis and T2 enhancement of the mid-septum and the mid lateral segments (FIG 2) - there was no late gadolinium enhancement (LGE). Based on this information a diagnosis of focal myocarditis was made. She was successfully treated with Aspirin 81mg, Colchicine 0.6mg twice per day, Metoprolol Succinate 25mg daily and a two week tapering dose of Prednisone 20mg daily (for suspected underlying pericarditis component). Discussion: Myocarditis is an under diagnosed cardiac disease that carries high morbidity and mortality. Diagnosis remains challenging due to nonspecific clinical signs and symptoms and low accuracy of endomyocardial biopsy. However, CMR can provide information about cardiac anatomy and tissue characterization. The presence and extent of LGE and abnormal T2 weighted imaging carry prognostic implication in predicting major cardiovascular events. This case highlights the importance of CMR in diagnosis and risk stratification of chronic myocarditis.
214 End-Stage Biventricular Failure from Necrotizing Autoimmune Myopathy Janet I. Ma, Nicholas Wettersten, Paul Kim, Eric Adler, Marcus A. Urey; University of California San Diego, San Diego, CA Necrotizing autoimmune myopathy is a rare neuromuscular disorder characterized clinically by severe proximal limb muscle weakness and pathologically by necrotic muscle fibers with minimal inflammation. It is often associated with statin therapy, connective tissue diseases, and malignancy. Most have characteristic autoantibodies although a small subset present without myositis-specific autoantibodies. While cardiac involvement is rare, conduction abnormalities, diastolic dysfunction, and wall motion abnormalities have been reported. Severe cardiomyopathy from necrotizing
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Journal of Cardiac Failure Vol. 24 No. 8S August 2018
autoimmune myopathy resulting in end-stage biventricular failure is extremely rare without a clear role for guideline-directed medical therapy. A 45-year-old man with hypothyroidism was admitted with several months of worsening proximal muscular weakness. He was found to have new severe biventricular dysfunction with conduction abnormalities during diagnostic work-up. Physical exam was notable for symmetric proximal muscle weakness in upper and lower extremities, sclerodactyly, elevated jugular venous distension, and lower extremity edema. Laboratory testing showed a creatine kinase of 3101 U/L, ANA 1:640, positive RNP antibodies, positive PM/Scl 100 antibodies. Anti-HMGCR antibodies and anti-SRP antibodies were negative. Lower extremity MRI showed diffuse bilateral symmetric myositis around the pelvis consistent with an inflammatory myopathy. EMG suggested irritable myopathy. Left thigh skeletal muscle biopsy showed necrotizing autoimmune myopathy with a paucity of inflammatory cells. Electrocardiogram revealed a right bundle branch block and 1st degree AV block. Frequent atrial and ventricular ectopic beats were seen on telemetry. Transthoracic echocardiogram showed biventricular failure with LVEF 23% and severe global hypokinesis. Cardiac MRI showed patchy foci of delayed subendocardial delayed enhancement. Endomyocardial biopsy did not reveal evidence of lymphocytic infiltration or ongoing myocarditis. Right heart catheterization was notable for elevated biventricular pressures with a cardiac index of 2.18. No obstructive coronary artery disease was seen on coronary angiogram. He was treated with intravenous immunoglobulins, methotrexate, and high-dose prednisone with improvement in proximal weakness. Low-dose spironolactone and lisinopril were started but could not be increased due to degree of hypotension. This case highlights a rare manifestation of an exceedingly rare autoimmune condition associated with high mortality. Given the variability of cardiac involvement in the disorder, a high clinical suspicion is needed to establish an early diagnosis and pursue aggressive treatment with up-front combination immunomodulating therapy, which has been associated with improved outcomes.
Figure 1. A: CMR demonstrates pericardial effusion (black asterisk) and subendocardial enhancement (white arrows). B: Eosinophilic infiltration of endomyocardium (Black arrows). C: Hypercellular Bone marrow.
215 Multi-modality Imaging in a Hypereosinophilic Syndrome with Cardiac Involvement Karuppiah Arunachalam, Charles Beale, Liz Edmund, Michael Atalay, Daniel Levine, Karen Aspry, Rayan Yousefzai; Warren Alpert Medical School of Brown University, Providence, RI Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare, multi-system auto-immune vasculitic disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. Cardiac involvement is the primary contributor to mortality. Case Report: Mr. W is a 53-year-old man with a history of asthma and environmental allergies presented to the emergency department with sudden onset of blurred vision in his left eye and evidence of optic neuritis. He endorsed a 2-week history of night sweats, arthralgias, anorexia, transient ankle edema, and a recent rash. His exam was notable for purpuric lesions in extremities and left visual field deficits. Laboratory data showed a WBC’s of 36,000 cells/mm3 with 69% eosinophils, CRP of 63 mg/dl, ESR of 122 mm/min, IgE of 1,741 IU/ml, and troponin of 11 ng/ml. An Electrocardiogram showed low voltage and q waves in anteroseptal leads. An infectious work-up was negative.A brain MRI revealed acute punctate infarcts. A transthoracic echocardiogram (TTE) revealed a severely dilated left ventricle with an ejection fraction of 25%. Cardiac catheterization showed normal coronary arteries, elevated filling pressures, and normal cardiac output. Cardiac Magnetic Resonance (CMR) showed moderate to severe biventricular dysfunction, a moderate pericardial effusion, and patchy bi-ventricular subendocardial enhancement (Figure 1A). So he underwent endomyocardial biopsy, which showed increased perivascular eosinophils/eosinophilic degranulation and fibrin (Figure 1B).
The differential diagnosis was EGPA, hypersensitivity myocarditis or a myeloproliferative variant of hypereosinophilic syndrome. A bone marrow biopsy was performed which showed hypereosinophilia (Figure 1C), without evidence of malignancy, confirming EGPA. He was treated with high dose steroids with a prolonged taper and cyclophosphamide, which rapidly resolved his eosinophilia. A repeat TTE 7 weeks later showed near normalization of cardiac function and resolution of pericardial fluid. Conclusion: Cardiac involvement in EGPA is up to 60% and early development of myocardial fibrosis. Prompt recognition via multi-modality imaging and endomyocardial biopsy and rapid treatment with high dose steroids or other immunotherapies, may prevent early progression to dilated or restrictive cardiomyopathy.
216 Specialized Resident Force in Internal Medicine Residency Program Helps Modify Risk of Heart Failure Readmissions: A Performance Improvement Project T.S. Dharmarajan1, S. Singh2, N. Bulcha2, S. Virdi2, R. Gali2, D. Yang2, S. Venkatraman2, S. Saleem2, G. Bhardwaj2, S. Zareef2, Ke Di Palo2, P. Shah2; 1Montefiore Medical Center (Wakefield Campus), Scarsdale, NY; 2Montefiore Medical Center, Bronx, NY Introduction: Heart Failure (HF) is a leading cause of hospitalization, mortality and health care costs in the U.S. HF is the most common cause of readmissions in Medicare patients, with >20% readmitted within 30 days of hospital discharge. Several interventions have been tried to lower readmissions involving patient, provider and related disciplines, without significant long-term impact. Hypothesis: Development of a specialized team of internal medicine residents trained to educate and counsel HF patients, will enhance disease state knowledge of the disease, improve self-care and reduce hospital readmissions among patients admitted with acute decompensated HF (ADHF). Methods: In August 2017, a team of 9 Internal Medicine residents were recruited to create a Specialized Resident Force (SRF). The residents received individual hands-on training sessions in counseling HF patients and met with a mentor weekly initially, later monthly. Patients on 2 selected medicine units with a high volume of HF admissions were counseled by the SRF on diet, activity, medication use, weight monitoring and outpatient follow-up. Demographic data, comorbidities and HF specific data was gathered and compared to patients receiving standard education. Patients were evaluated for cognition and advance directives. In the cognitively impaired, education was offered to the caregiver, if available. A tool was developed to document data; a booklet on HF education and a 2 page easy to use script to help counsel patients at bedside was provided to each resident. Results: Enhanced SRF education was provided to 44 patients between September and December 2017. Education provided by the SRF resulted in 55.7% reduction in the 30 day all cause readmission rate (see Table 1) Observations by the mentor suggested that knowledge base of SRF trainees improved as a result of participation. Conclusions: Specialized internal medicine resident teams providing bedside education to patients with ADHF may favorably influence HF readmissions, while simultaneously enhancing resident knowledge base on HF management; Academic medical centers can easily incorporate resident driven patient education into existing readmission reduction efforts TABLE 1. Bedside Eduction
Admissions
Readmissions
Readmission Rate
Standard care (usual care: nurses, attendings Enhanced (SRF driven)
78
19
25.7%
44
5
11.36%
217 Symmetrical Peripheral Gangrene Associated with Low Output Cardiac Failure Sijan Basnet1, Priya Rajagopalan2, Rashmi Dhital1, Ataul Qureshi2; 1Reading Hospital, West Reading, PA; 2Jefferson Medical College, Philadelphia, PA Introduction: Symmetrical peripheral gangrene (SPG) is a rare clinical condition with acute, symmetrical ischemia of two or more extremities leading to gangrene in the absence of large vessel obstruction or vasculitis. We present a case of SPG presenting in the setting of severely depressed cardiac ejection fraction. Case Description: Patient is a 64-year-old man referred to our emergency department from an outside hospital for management of liver failure and bilateral necrotic toes. His past medical history and surgical history were unremarkable. He drank 3-4 beers and 2-3 glasses of hard liquor daily. He had dry and gangrenous second through fifth toes and densely cyanotic first toes bilaterally. (Figure 1) Dorsalis pedis and posterior tibialis pulses were present bilaterally on palpation. His liver function test (LFT) was deranged (Total bilirubin: 35.0 mg/dL, direct bilirubin: >10.0 mg/dL, AST: 309 IU/L, ALT: 295 IU/L). Echo showed ejection fraction of 10 percent with global hypokinesis and severe left ventricular enlargement but no embolus. His brain natriuretic peptide was 18,984 pg/mL (reference range: < 125 pg/