The Cardiac Tumor Program at Boston Children’s Hospital Heart Center is a comprehensive program that specializes in the diagnosis and treatment of pediatric cardiac tumors. Tumors originating in the heart are rare in children, and many hospitals see only a few, if any, each year. The team at Boston Children’s treats approximately one each month.
Depth of expertise
The team includes Tal Geva, MD, chief of Cardiology; Pedro del Nido, MD, chief of Cardiac Surgery; Edward Walsh, MD, chief of Electrophysiology; and cardiologists Ashwin Prakash, MD; and Elizabeth Dewitt, MD — all of whom have extensive experience in the diagnosis and treatment of pediatric cardiac tumors.
“What sets us apart is substantial experience with complex pediatric cardiac tumors — and results, many of which have been published in peer-reviewed medical journals.”
Because cardiac tumors are so rare, and because there are a number of different types of cardiac tumors, each requiring unique treatment plans, there is not a standardized process for treating them. Diagnosing these tumors and determining the specific type, takes experience with imaging, knowing exactly what to look for, such as the source of tumor, location, extent, involvement of adjacent cardiac structures and tumor tissue type.
“In the last 10 years, the program has maintained a 100-percent survival rate,” Geva says.
The advanced imaging available at Boston Children’s — cardiac MRI, 2-D and 3-D echocardiography, ultrafast CT, and catheter angiography — provides better accuracy in cardiac tumor evaluation. Once the tumor has been accurately diagnosed, each child’s care plan is designed under the direction of the program’s team from the comprehensive diagnostic evaluation to the surgical approach.
“In order to perform these complex operations, and to decide when it is appropriate to operate, takes a significant amount of expertise, especially when it comes to a rare condition like cardiac tumors,” says Geva. “We’ve had about 30 cardiac-fibroma surgical resections — and growing — which is relatively large.”
Cardiac tumor diagnostics
The team has developed methodology to identify tumor type by MRI in conjunction with echocardiography.
- Kiaffas MG, Powell AJ, Geva T. Magnetic resonance imaging evaluation of cardiac tumor characteristics in infants and children. Am J Cardiol 2002;89:1229-1233.
- Mackie AS, Kozakewich HPW, Geva T, Perez-Atayde AR, Mulliken JB. Vascular tumors of the heart in infants and children: Case series and review of the literature. Ped Cardiol 2005;26:344-349.
- Beroukhim RS, Prakash A, Valsangiacomo Buechel ER, Cava JR, Dorfman AL, Festa P, Hlavacek AM, Johnson TR, Keller MS, Krishnamurthy R, Misra N, Moniotte S, Parks J, Powell AJ, Soriano BD, Srichai MB, Yoo SJ, Zhou J, Geva T. Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience. J Am Coll Cardiol 2011;58:1044-1054.
- Beroukhim RS, Geva T. Primary and secondary cardiac tumors. In: da Cruz EM, Ivy D, and Juggers J (eds.). Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. London: Springer-Verlag 2014;2275-2295
Potentially life-threatening arrhythmias often complicate cardiac tumors. Our team has extensive experience with management of atrial and ventricular arrhythmias in these patients and has published on treatment strategies in patients with rhabdomyomas and fibromas.
Miyake CY, del Nido PJ, Alexander ME, Cecchin F, Berul CI, Triedman JK, Geva T, Walsh EP. Cardiac Tumors and Associated Arrhythmias in Pediatric Patients, With Observations on Surgical Therapy for Ventricular Tachycardia J Am Coll Cardiol 2011;58:1903-1909
Surgical management and techniques
Surgery is the most comprehensive and contemporary approach in carefully selected patients with pediatric cardiac tumors. However, the management strategy and, in particular, the surgical approach for ventricular fibromas can pose a challenge. The large tumor size in relationship to the ventricular chambers and encroachment on vital adjacent structures has often been considered a limiting factor.
“In our experience, all but one tumor could be resected in its entirety, independent of the tumor size, tumor location or patient age,” says Geva. “Large ventricular fibromas can be safely managed with primary total or near total excision, with relief of life-threatening arrhythmias and excellent clinical results.”
Nathan M, Fabozzo A, Geva T, Walsh E, del Nido JP. Successful surgical management of ventricular fibromas in children. J Thorac Cardiovasc Surg 2014;148:2602-8