Diagnostic Approach to Recurrent Intracardiac Masses That Present With Systemic Symptoms.

Cardiac masses are difficult to diagnose in the pediatric population, especially in the setting of systemic symptoms. Although multiple imaging modalities are available to characterize cardiac masses, it is important to consider a different diagnostic approach in the setting of recurrent cardiac masses and nonspecific systemic symptoms. We present a case involving a previously healthy adolescent with multiple hospitalizations because of persistent fevers, cachexia, and recurrent cardiac masses. Echocardiography and cardiac computed tomography imaging suggested endocarditis, but the patient failed to respond to multiple intravenous antibiotic treatments. He developed recurrent cardiac masses in the right atrium and right ventricle that were debulked and biopsied. The biopsy did not yield a conclusive diagnosis. The patient returned to the hospital with hemoptysis and large pulmonary pseudoaneurysms that had to be occluded during cardiac catheterization. Given his constellation of symptoms and improvement with steroids during surgical procedures, he was ultimately diagnosed with a variant of Behcet's disease known as Hughes-Stovin syndrome. His symptoms resolved completely with steroids and immunosuppression therapy. Our report reveals the limitations of the standard diagnostic approach toward cardiac masses and the importance of considering response to treatment as a clue to the etiology of an unusual cardiac mass.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

BACKGROUND: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria. OBJECTIVES: The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. METHODS: CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. RESULTS: Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. CONCLUSIONS: CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.