Quick Fire Cases
Shawn Shaji, MD
Cardiac Advanced Imaging Fellow
University of Utah/Primary Children's Hospital
Shawn Shaji, MD
Cardiac Advanced Imaging Fellow
University of Utah/Primary Children's Hospital
Andrea Harman, RT
MRI technologist
Primary Children's Hospital
Mckenzie Foreman, RT
MRI technologist
Primary Children's Hospital
Spencer B. Barfuss, MD
Assistant Professor of Pediatrics
Boston Children's Hospital
Sanja Dzelebdzic, MD
Pediatric Cardiologist
University of Utah/Primary Children's Hospital
Kevin K. Whitehead, MD, PhD
Cardiologist
Children's Hospital of Philadelphia
Edem Binka, MD
Assistant Professor
University of Utah
Barbara K. Han, MD
Professor
University of Utah
A 14-year-old male with no significant past medical history presented with facial droop and left-sided weakness and was found to have occlusion of the inferior division of the right middle cerebral artery. He was transferred for mechanical thrombectomy with successful reperfusion. Stroke workup included transthoracic and transesophageal echocardiography which showed normal intracardiac anatomy and systolic function. Agitated saline studies were consistent with pulmonary arteriovenous malformations (AVMs). This was confirmed on a chest computed tomography (CT) scan which revealed extensive right-sided pulmonary arteriovenous malformations (AVMs). Genetic testing confirmed the diagnosis of hereditary hemorrhagic telangiectasia (HHT). He underwent pulmonary AVM embolization but on repeat TTE was found to have continued positive agitated saline study and new findings of hypokinesis and focal hypertrophy of the left ventricular (LV) posterior wall with myocardial tissue heterogeneity (Figure 1). The patient underwent cardiac magnetic resonance imaging (CMR) for further assessment of these findings.
Diagnostic Techniques and Their Most Important Findings:
CMR was completed on a 1.5T Siemens Magnetom Sola (Siemens Healthcare, Erlangen, Germany). Standard functional assessment was done with balanced SSFP cines. 3D whole heart angiography, along with parametric mapping sequences, were performed. After administration of gadolinium, first pass perfusion, delayed enhancement (DE), post-contrast T1 mapping, and volumetric interpolated breath-hold examination (VIBE) were completed. There was mildly decreased LV systolic function (ejection fraction 48%) with focal wall thickening and wall motion abnormality of the basal anterolateral and inferolateral LV wall (Figure 2). There was elevated native T1 value (1780±845 ms), no perfusion from the epicardial to mid-myocardial layers, and transmural DE with central foci of hypoenhancement. VIBE and DE with an inversion time (TI) of 600 ms showed dark epicardial to mid-myocardial LV wall. The coronary origins and proximal courses were normal.
Learning Points from this Case:
These findings were consistent with transmural myocardial infarction in the basal anterolateral and inferolateral LV in the circumflex distribution. In addition, areas of central hypoenhancement were evident on DE consistent with microvascular obstruction (MVO) with hemorrhage. High sensitivity troponin I was followed and peaked to 5569 ng/L and down trended prior to discharge. ECG showed normal sinus rhythm with nonspecific T wave abnormality. Cardiac CT showed no intervenable lesions or thrombi. He was discharged on anticoagulation, beta blocker, and statin with plans for close cardiology follow up. This is the first reported case of myocardial infarction with MVO noted after pulmonary AVM embolization in a pediatric patient with HHT. This case highlights the importance of multimodality imaging with complimentary diagnostic utility.
