Cardiac Implantable Electronic Devices In Advanced Heart Failure Patients On Palliative Inotropes
HFSA ePoster Library. Tolia S. 09/10/21; 343381; 156
Sanika Tolia
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Abstract
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Introduction Giant cell myocarditis (GCM) classically presents in a dramatic fashion. Initial manifestations may include fulminant heart failure, refractory ventricular arrhythmias, and high degree AV block. Treatment includes aggressive immunosuppression, but even with proper treatment, the risk of progression to end stage heart failure is high. Although the clinical scenario can strongly suggest GCM, endomyocardial biopsy (EMB) remains the gold diagnostic standard.
Case Report A 70-year-old female with hypertension and hyperlipidemia presented to the hospital with syncope. She was found to have new systolic heart failure with a left ejection fraction of 32%. She had a new first degree AV block with intermittent 2:1 AV block. Cardiac MRI showed multiple areas of delayed enhancement thought to be consistent with RCA territory infarct pattern versus infiltrative cardiomyopathy, specifically, sarcoidosis. Subsequent coronary angiogram demonstrated mild, non-obstructive coronary artery disease. EMB at this time showed focal histiocytic infiltrate with scattered eosinophils. No granulomas or giant cells were identified.
Given the clinical picture and EMB findings, presentation was felt to be most consistent with cardiac sarcoidosis, although other inflammatory etiologies remained in the differential. Prednisone and neurohormonal blockade were started. Given her intermittent AV block, a CRT-D device was implanted. The remainder of the patient’s index admission was uneventful and she had NYHA Class II symptoms on discharge.
However, the patient quickly returned to the emergency department after 17 appropriate ICD discharges in the setting of VT storm. She quickly deteriorated requiring emergent peripheral VA-ECMO cannulation. A second EMB was obtained at this time which showed profound inflammation as well as giant cells (Figure 1). She was treated for giant cell myocarditis with aggressive immunosuppression but had worsening end-organ dysfunction and continued to deteriorate clinically. Ultimately, the decision was made to transition to comfort measures and the patient expired.
Discussion Both GCM and cardiac sarcoidosis can present with new onset heart failure, refractory ventricular arrhythmias, and life-threatening conduction abnormalities. Endomyocardial biopsy is critical to establish the diagnosis, yet the yield is not 100%. Given the exceedingly high mortality without proper treatment, repeat EMB may be necessary to establish the diagnosis of GCM.
Case Report A 70-year-old female with hypertension and hyperlipidemia presented to the hospital with syncope. She was found to have new systolic heart failure with a left ejection fraction of 32%. She had a new first degree AV block with intermittent 2:1 AV block. Cardiac MRI showed multiple areas of delayed enhancement thought to be consistent with RCA territory infarct pattern versus infiltrative cardiomyopathy, specifically, sarcoidosis. Subsequent coronary angiogram demonstrated mild, non-obstructive coronary artery disease. EMB at this time showed focal histiocytic infiltrate with scattered eosinophils. No granulomas or giant cells were identified.
Given the clinical picture and EMB findings, presentation was felt to be most consistent with cardiac sarcoidosis, although other inflammatory etiologies remained in the differential. Prednisone and neurohormonal blockade were started. Given her intermittent AV block, a CRT-D device was implanted. The remainder of the patient’s index admission was uneventful and she had NYHA Class II symptoms on discharge.
However, the patient quickly returned to the emergency department after 17 appropriate ICD discharges in the setting of VT storm. She quickly deteriorated requiring emergent peripheral VA-ECMO cannulation. A second EMB was obtained at this time which showed profound inflammation as well as giant cells (Figure 1). She was treated for giant cell myocarditis with aggressive immunosuppression but had worsening end-organ dysfunction and continued to deteriorate clinically. Ultimately, the decision was made to transition to comfort measures and the patient expired.
Discussion Both GCM and cardiac sarcoidosis can present with new onset heart failure, refractory ventricular arrhythmias, and life-threatening conduction abnormalities. Endomyocardial biopsy is critical to establish the diagnosis, yet the yield is not 100%. Given the exceedingly high mortality without proper treatment, repeat EMB may be necessary to establish the diagnosis of GCM.
Introduction Giant cell myocarditis (GCM) classically presents in a dramatic fashion. Initial manifestations may include fulminant heart failure, refractory ventricular arrhythmias, and high degree AV block. Treatment includes aggressive immunosuppression, but even with proper treatment, the risk of progression to end stage heart failure is high. Although the clinical scenario can strongly suggest GCM, endomyocardial biopsy (EMB) remains the gold diagnostic standard.
Case Report A 70-year-old female with hypertension and hyperlipidemia presented to the hospital with syncope. She was found to have new systolic heart failure with a left ejection fraction of 32%. She had a new first degree AV block with intermittent 2:1 AV block. Cardiac MRI showed multiple areas of delayed enhancement thought to be consistent with RCA territory infarct pattern versus infiltrative cardiomyopathy, specifically, sarcoidosis. Subsequent coronary angiogram demonstrated mild, non-obstructive coronary artery disease. EMB at this time showed focal histiocytic infiltrate with scattered eosinophils. No granulomas or giant cells were identified.
Given the clinical picture and EMB findings, presentation was felt to be most consistent with cardiac sarcoidosis, although other inflammatory etiologies remained in the differential. Prednisone and neurohormonal blockade were started. Given her intermittent AV block, a CRT-D device was implanted. The remainder of the patient’s index admission was uneventful and she had NYHA Class II symptoms on discharge.
However, the patient quickly returned to the emergency department after 17 appropriate ICD discharges in the setting of VT storm. She quickly deteriorated requiring emergent peripheral VA-ECMO cannulation. A second EMB was obtained at this time which showed profound inflammation as well as giant cells (Figure 1). She was treated for giant cell myocarditis with aggressive immunosuppression but had worsening end-organ dysfunction and continued to deteriorate clinically. Ultimately, the decision was made to transition to comfort measures and the patient expired.
Discussion Both GCM and cardiac sarcoidosis can present with new onset heart failure, refractory ventricular arrhythmias, and life-threatening conduction abnormalities. Endomyocardial biopsy is critical to establish the diagnosis, yet the yield is not 100%. Given the exceedingly high mortality without proper treatment, repeat EMB may be necessary to establish the diagnosis of GCM.
Case Report A 70-year-old female with hypertension and hyperlipidemia presented to the hospital with syncope. She was found to have new systolic heart failure with a left ejection fraction of 32%. She had a new first degree AV block with intermittent 2:1 AV block. Cardiac MRI showed multiple areas of delayed enhancement thought to be consistent with RCA territory infarct pattern versus infiltrative cardiomyopathy, specifically, sarcoidosis. Subsequent coronary angiogram demonstrated mild, non-obstructive coronary artery disease. EMB at this time showed focal histiocytic infiltrate with scattered eosinophils. No granulomas or giant cells were identified.
Given the clinical picture and EMB findings, presentation was felt to be most consistent with cardiac sarcoidosis, although other inflammatory etiologies remained in the differential. Prednisone and neurohormonal blockade were started. Given her intermittent AV block, a CRT-D device was implanted. The remainder of the patient’s index admission was uneventful and she had NYHA Class II symptoms on discharge.
However, the patient quickly returned to the emergency department after 17 appropriate ICD discharges in the setting of VT storm. She quickly deteriorated requiring emergent peripheral VA-ECMO cannulation. A second EMB was obtained at this time which showed profound inflammation as well as giant cells (Figure 1). She was treated for giant cell myocarditis with aggressive immunosuppression but had worsening end-organ dysfunction and continued to deteriorate clinically. Ultimately, the decision was made to transition to comfort measures and the patient expired.
Discussion Both GCM and cardiac sarcoidosis can present with new onset heart failure, refractory ventricular arrhythmias, and life-threatening conduction abnormalities. Endomyocardial biopsy is critical to establish the diagnosis, yet the yield is not 100%. Given the exceedingly high mortality without proper treatment, repeat EMB may be necessary to establish the diagnosis of GCM.
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