Abstract
Double Trouble: A Case Of Combined Amyloid And Ischemic Cardiomyopathy
Journal of cardiac failure, v 28(5), S30
Apr 2022
Abstract
The management of concurrent cardiac amyloidosis (CA) and ischemic cardiomyopathy (ICM) is not well established due to the rarity of this phenomenon. We present a case of mixed cardiomyopathy with challenging clinical decision-making.
A 76-year-old African American woman with hypertension, diabetes, and longstanding carpal tunnel syndrome presented with progressive dyspnea and was found to have decompensated heart failure attributable to a new cardiomyopathy. Initial workup revealed left ventricular ejection fraction (LVEF) of 20% and severe triple vessel coronary artery disease. Echocardiogram also demonstrated an apical-sparing strain pattern (Figure 1A) prompted concern for coexisting CA given her demographic and carpal tunnel history. A cardiac magnetic resonance (CMR) raised further concern for CA given restrictive cardiomyopathy, pericardial effusion, and characteristic late gadolinium enhancement (Figure 1B and 1C). Ultimately, transthyretin CA (ATTR) was confirmed with a positive technetium-99m-Pyrophosphate Scintigraphy (PYP) scan (Figure 1D) paired with normal serum immunofixation electrophoresis. She was evaluated by cardiothoracic surgery but deemed a prohibitively high-risk candidate for CABG due to her advanced age, ATTR diagnosis, and significant systolic dysfunction. Instead, she was diuresed and started on guideline-directed medical therapy, which she tolerated unusually well despite her diagnosis of ATTR. Using Impella CP for temporary mechanical circulatory support, she underwent high-risk percutaneous coronary intervention to the distal left main artery extending into the proximal left circumflex. She was discharged on dual antiplatelet therapy. During outpatient follow-up in the Multidisciplinary CA Clinic, genetic testing revealed a pathogenic V122I mutation indicative of hereditary ATTR. She was started on tafamidis after nerve conduction studies did not demonstrate neuropathy. The combination of guideline-directed medical therapy, successful percutaneous revascularization, and ATTR-directed treatment resulted in remarkable improvement (NYHA Class II, LVEF 50% from NYHA IV, LVEF 20%).
Further investigation is needed to define optimal treatment strategies in patients with coexisting ischemic and amyloid cardiomyopathy. Until then, a multidisciplinary approach must be utilized to individualize the care of such patients.
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Details
- Title
- Double Trouble: A Case Of Combined Amyloid And Ischemic Cardiomyopathy
- Creators
- Arinze Bosah - Allegheny Health NetworkAmel Tobaa - Allegheny Health NetworkKushani Gajjar - Allegheny Health NetworkKartikeya Kashyap - Allegheny Health NetworkMithun Chakravarthy - Allegheny Health NetworkCraig Alpert - Allegheny Health Network
- Publication Details
- Journal of cardiac failure, v 28(5), S30
- Publisher
- Elsevier
- Resource Type
- Abstract
- Language
- English
- Academic Unit
- General Internal Medicine
- Other Identifier
- 991022053781004721