A 16-year-old female with acute decompensated heart failure and subsequently diagnosed with severe multi-vessel coronary disease and myocardial infarct, initially suspected through findings on cardiac magnetic resonance imaging (QF_FR_084)

A 16-year-old female with Evan syndrome, type 2 diabetes mellitus, primary hypertension, and previously normal heart structure and function, was admitted for management of acute decompensated heart failure. She reported months of increasing dyspnea, orthopnea, and low-grade fever with persistent cough, unresponsive to oral antibiotics, without reported chest pain. She was noted to have cardiomegaly on chest x-ray with viral panel positive for rhinovirus/enterovirus. Transthoracic echocardiography (TTE) showed right ventricular (RV) hypertension, left ventricular (LV) dilation, and diminished LV systolic function, with LV ejection fraction (LVEF) of 19%. She required supplemental oxygen, milrinone, and diuretics. Cardiac magnetic resonance (CMR) was obtained to risk stratify and clarify an underlying diagnosis for heart failure.

Diagnostic Techniques and Their Most Important Findings:

CMR was performed using a torso phased-array coil. Multiplanar stack of short axis and two-chamber and four-chamber long axis views of the heart were obtained using cine steady-state free precession (SSFP) imaging to assess wall motion. Tissue characterization was performed using pre-contrast T2 weighted fast turbo spin echo images, and late gadolinium (10 minutes postcontrast) inversion recovery imaging. CMR demonstrated gross LV dilation with severely diminished systolic function (LVEF 25%), diminished RV systolic function, regional LV wall motion abnormalities (hypokinesia of basal anterior and inferior walls, as well as the basal/mid ventricular septum), dyskinesia/hypokinesia of true LV apex, with associated wall thinning and transmural and some subendocardial late gadolinium enhancement (LGE) of the ventricular septum. Of note, no LGE was noted at the LV apex. The patient underwent diagnostic cardiac catheterization revealing severe multi-vessel coronary disease, including complete occlusion of the ramus intermedius and left anterior descending (LAD). Hours after catheterization, she developed significant ST-segment changes and became pulseless for which resuscitation was initiated. Given significant disease, without good options to bridge to long-term survival, resuscitation was stopped. Autopsy was significant for cardiomegaly, mild to moderate biventricular dilation, and severe coronary artery stenosis with 80-100% stenosis of proximal right coronary (RCA), proximal and distal portions of the left circumflex (LCX), and distal LAD.

Learning Points from this Case:

Chronic cardiac ischemia is an uncommon finding in children and was not highly suspected. CMR demonstrated wall motion abnormalities and transmural LGE with some subendocardial involvement, spanning multiple coronary artery distribution, concerning for severe multivessel coronary artery disease and myocardial infarct, making a previously unsuspected diagnosis clear with a high degree of accuracy, later confirmed on cardiac catheterization and autopsy. CMR continues to have an essential role in the evaluation of heart failure, especially cases with an unknown etiology.