Rebecca S Beroukhim1, Sunil Ghelani2, Ravi Ashwath3, Sowmya Balasubramanian4, David M Biko5, Sujatha Buddhe6, M Jay Campbell7, Russell Cross8, Pierluigi Festa9, Lindsay Griffin10, Heynric Grotenhuis11, Keren Hasbani12, Sassan Hashemi13, Sanjeet Hegde14, Tarique Hussain15, Supriya Jain16, Maria Kiaffas17, Shelby Kutty18, Christopher Z Lam19, Gabriela Liberato20, Anthony Merlocco21, Nilanjana Misra22, Katie L Mowers23, Juan Carlos Muniz24, Arni Nutting25, David A Parra26, Jyoti K Patel27, Antonio R Perez-Atayde2, Deepa Prasad28, Carlos F Rosental29, Amee Shah30, Margaret M Samyn31, Lynn A Sleeper2, Timothy Slesnick13, Emanuela Valsangiacomo32, Tal Geva2. 1. Boston Children's Hospital, Boston, Massachusetts, USA. Electronic address: rebecca.beroukhim@cardio.chboston.org. 2. Boston Children's Hospital, Boston, Massachusetts, USA. 3. University of Iowa Stead Family Children's Hospital, Iowa City, Iowa, USA. 4. CS Mott Children's Hospital, Ann Arbor, Michigan, USA. 5. Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. 6. Seattle Children's Hospital, Seattle, Washington, USA. 7. Duke Children's Hospital, Durham, North Carolina, USA. 8. Children's National Medical Center, Washington, DC, USA. 9. Fondazione G. Monasterio C.N.R. Regione Toscana, Pisa, Italy. 10. Ann & Robert H Lurie Children's Hospital of Chicago, Chicago, Illinois, USA. 11. University of Utrecht, Utrecht, the Netherlands. 12. Dell Children's Medical Center, Austin, Texas, USA. 13. Children's Healthcare of Atlanta, Atlanta, Georgia, USA. 14. Rady Children's Hospital San Diego, San Diego, California, USA. 15. Children's Medical Center Dallas, Dallas, Texas, USA. 16. Maria Fareri Children's Hospital at Westchester Medical Center, Valhalla, New York, USA. 17. Children's Mercy Hospital, Kansas City, Missouri, USA. 18. Johns Hopkins Children's Center, Baltimore, Maryland, USA; Children's Hospital and Medical Center, Omaha, Nebraska, USA. 19. Hospital for Sick Children, Toronto, Canada. 20. Heart Institute, InCor, Sao Paulo, Brazil. 21. Le Bonheur Children's Hospital, Memphis, Tennessee, USA. 22. Cohen Children's Medical Center of New York, Northwell Health, New Hyde Park, New York, USA. 23. CS Mott Children's Hospital, Ann Arbor, Michigan, USA; St Louis Children's Hospital, St Louis, Missouri, USA. 24. Nicklaus Children's Hospital, Miami, Florida, USA. 25. Medical University of South Carolina, Charleston, South Carolina, USA. 26. Vanderbilt Children's Hospital, Nashville, Tennessee, USA. 27. Riley Children's Hospital, Indianapolis, Indiana, USA. 28. Banner Children's Hospital, Mesa, Arizona, USA. 29. Hospital Pediatria Garrahan, Buenos Aires, Argentina. 30. Children's Hospital of New York, New York, New York, USA. 31. Medical Collect of Wisconsin/Children's Wisconsin, Milwaukee, Wisconsin, USA. 32. University Children's Hospital Zurich, Zurich, Switzerland.
Abstract
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. Crown
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. Crown