Oral Case
Ashish Shrivastava, MD
Pediatric Cardiology Fellow
Cincinnati Children's Hospital Medical Center
Ashish Shrivastava, MD
Pediatric Cardiology Fellow
Cincinnati Children's Hospital Medical Center
Hieu Ta, MD
Cardiologist
Cincinnati Children's Hospital Medical Center
Aki Tanimoto, MD
Assistant Professor
Cincinnati Children's Hospital Medical Center
Paul Critser, MD
Cardiologist
Cincinnati Children's Hospital Medical Center
Clayton T. Smith, MD
Assistant Professor of Pediatrics
University of Cincinnati
Amr Matoq, MD
Cincinnati Children's Hospital Medical Center
Russel Hirsch, MD
Cardiologist
Cincinnati Children's Hospital Medical Center
Sean M. Lang, MD
Cardiologist
Cincinnati Children's Hospital Medical Center
Kimberley Miles, MD
Pediatric Cardiology Fellow
Cincinnati Children's Hospital Medical Center
Cardiac MRI (1.5 Tesla Philips Ingenia) was performed with standard cine imaging, parametric mapping, resting first pass perfusion (FPP), and late gadolinium enhancement (LGE) imaging 9 minutes post-administration of ~0.4mmol/kg of gadoterate meglumine (Dotarem, Guerbet, Villepinte, France) utilizing PSIR sequences with a “dark blood” approach, adjusting the TI to null the LV blood pool and improve visualization of sub-endocardial LGE. She had severe LV systolic dysfunction (LVEF 29%) with hypokinesis of the basal- and mid-inferior segments and mid-inferolateral wall, mild RV systolic dysfunction (RVEF 43%), elevated T1/ECV and T2 values, hypoperfusion on FPP along the right coronary distribution (Image 1), and corresponding transmural LGE with evidence of microvascular occlusion (Image 2).
Learning Points from this Case: Catastrophic antiphospholipid syndrome is a rare, life-threatening presentation of antiphospholipid syndrome (APLS), characterized by critical illness and thrombotic complications. While rare in children, acute myocardial dysfunction due to MINOCA has been described in up to 20% of adults with APLS and myocardial ischemia. After excluding coronary pathology, first pass perfusion and delayed gadolinium enhancement cardiac MRI techniques can identify evidence of microvascular obstruction and myocardial ischemia/infarction, which supported MINOCA in the setting of CAPS in our patient. Acute myocardial dysfunction due to MINOCA is a rare complication of CAPS in children. This case highlights the importance of cardiac MRI in identifying mechanisms of non-atherosclerotic myocardial ischemia.
Image 1. During resting first pass perfusion, there is subendocardial hypoperfusion in a right coronary distribution of the (A) basal- and (B) mid-inferoseptal, inferior, and inferolateral segments.
Image 2: Transmural LGE of the (A) basal-inferoseptal and (B) inferior and mid-inferoseptal segments with limited enhancement of the basal inferoseptal subendocardial and mid-myocardial segments, corresponding to microvascular occlusion (arrowheads).