DOI: 10.1161/circ.148.suppl_1.17504 ISSN: 0009-7322

Abstract 17504: Revisiting the Etiology of a Non-Ischemic Cardiomyopathy

Alexandra B Steverson, Masataka Kawana
  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Introduction: In the era of REVIVED-BCIS2 and ISCHEMIA clinical trials, the role of routine invasive coronary angiography for treatment of coronary artery disease (CAD) in the setting of left ventricular dysfunction has come into question. While the majority of heart failure (HF) patients undergo some form of non-invasive ischemic evaluation, there are a wide range of opinions on which testing modality is the most appropriate.

Case: This is a 53 year-old man with a history of HTN, HLD, and presumed non-ischemic cardiomyopathy and LVEF of 15-20%, first diagnosed in 2009. He had a family hx of heart disease in several distant relatives, thus the etiology was presumed familial. In 2022, he had NYHA class II symptoms and a routine echo showed a LVEF of 25% with anteroseptal hypokinesis. Given these findings, a coronary CTA was obtained. This demonstrated infarct in the LAD territory and a proximal left main to mid-LAD aneurysm measuring up to 7 mm with a partially calcified shell and peripheral thrombus, consistent with prior Kawasaki’s disease (KD). He was initiated on a DOAC to prevent further embolic events and aspirin. He is now undergoing serial monitoring of the aneurysm size, thrombus burden, and functional testing to monitor for ischemia.

Discussion: Non-invasive anatomic testing to evaluate for CAD in HF patients can provide useful information over a functional assessment. KD is a rare disease with an estimated incidence rate of ≈25 per 100,000 children less than 5 years of age. KD can lead to coronary artery aneurysms, which often contain thrombi, and subacute/chronic myocarditis. The affected arteries can become stenotic over time or even occlude, leading to ischemic injury later in life. Early recognition of this rare disease is important to initiate appropriate treatment and prevent thromboembolic complications.

Conclusion: KD is a rare cause of adult cardiomyopathy. Anatomic testing was essential to appropriately diagnose and treat this patient’s cardiomyopathy.

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