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- 1. Congenital Cardiac Fibroma: A Case Report E.R. Bonner 1, S.J. Crapp 2, D.A. Parra 2 1 Meharry Medical College, Nashville, TN 37208, 2 Vanderbilt University, Nashville, TN 37232 Case Report Discussion A 5 week old male presented to his pediatrician with a II/VI systolic ejection murmur along the left sternal border. He had mild Cardiac fibromas are the second most common pediatric tachypnea without cyanosis. His oral intake was adequate with no primary cardiac tumor. Ninety percent of these tumors occur evidence of failure to thrive. in children younger than twelve years old. Fibromas are He was referred to a pediatric cardiologist who performed an ECG made up of fibroblasts and collagen, and often contain small and a transthoracic echocardiogram. The ECG showed normal sinus calcifications. They are solitary lesions and are most often rhythm at 135 beats per minute with no abnormalities. The found in the left ventricular free wall or the ventricular transthoracic echocardiogram showed a 25 x 25 x 14 mm septum, but as seen in this patient, they are occasionally homogenous mass originating from the anterior free wall of the located in the right ventricle. Patients typically present with right ventricle, and mild dilation of the right ventricle. Mild dynamic subpulmonary stenosis and a secundum atrial septal defect were heart failure, arrhythmias, syncope, or blood flow also noted. Although the murmur was significantly louder at one obstructions; but, a third of patients will be asymptomatic. month follow-up, a repeat echocardiogram did not reveal any Ten percent of patients may present with sudden death. increase in the size of the mass. Cardiac fibromas can be associated with Gorlin Syndrome, At 2 months of age, a cardiovascular magnetic resonance imaging also called Basal Cell Nevus Syndrome. Gorlin syndrome is (CMRI) study under general anesthesia was performed. CMRI an autosomal dominant disorder that predisposes patients to revealed a 16 x 21 x 22 mm intracardiac tumor that was causing a host of different neoplasms, neurological symptoms, and mass effect and narrowing of the right ventricular outflow tract. bone abnormalities. The tumor was isointense on T2-weighted imaging (figure 2) and hyperintense on T1-weighted imaging, with positive delayed Figure 1. CMRI ECG-gated SSFP Figure 2. CMRI ECG-gated T2- enhancement (figure 4). These findings, along with the size and TSE Surgical resection of these tumors is generally curative location of the mass, were consistent with a diagnosis of a cardiac because they do not usually recur. Resection is even fibroma. Chest MRA, that was also performed, showed normal recommended for most asymptomatic patients. Tumors that extracardiac vascular anatomy with no evidence of peripheral infiltrate the myocardium and grow around coronary arteries branch pulmonary stenosis (not shown). and conduction tissue are considered unresectable. Cardiac fibromas do not usually increase in size; however, the Unresectable tumors that cause arrhythmias can be managed concern is the child’s risk of arrhythmias. Frequent Holter with an implantable cardioverter defibrillator (ICD). Those monitoring was recommended for this patient. Considerations were patients may later need a heart transplant. also made for an electrophysiology study within 1-2 years to determine the risk of ventricular ectopy. At that point, the patient References can be assessed for possible surgical resection of the fibroma. Key Points 1.Beroukhim, R et al. "Characterization of cardiac tumors in children by cardiovascular MRI". JACC Vol 58, No 10, 2011: 1044-54 2.Greenberg, S. McDonald, C., (2008) “Cardiac Tumors: Intrinsic •Cardiac fibroma is the 2nd most common primary tumors.” T. Slovis et al. Caffey’s Pediatric Diagnostic Imaging, pp. 1707-1708. Philadelphia, PA: Mosby cardiac tumor of childhood •Frequently detected in the first year of life but 3.Morelli JG. Tumors of the skin. In: Kliegman RM, Behrman RE, Jenson may present throughout childhood HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. •Solitary tumor of the myocardium composed of Philadelphia, Pa: Saunders Elsevier;2007:chap 669. fibroblasts and collagen 4.Myers KA, Wong KK, Tipple M, Sanatani S. Benign Cardiac Tumours, •CT imaging shows calcifications and Figure 3. CMRI ECG-gated Turbo Figure 4. CMRI Myocardial Malignant Arrhythmias. Can J Cardiol 2010;26(2):e58-e61. heterogeneous enhancement Spin Black Blood Slice Delayed Enhancement 5.Sparrow PJ, Kurian JB, Jones TR, Sivananthan MU. MR Imaging of •MR shows T2 signal, early enhancement, Cardiac Tumors. RadioGraphics 2005;25(5):1255-1276. Delayed enhancement 6.Stratemann S, Dzurik Y, Fish F, Parra D. Left Ventricular Cardiac Fibroma in a Child Presenting with Ventricular Tachycardia. Pediatr Cardiol 2008;29:223-226. 7.Syed IS, Feng D, Harris SR, et al. MR Imaging of Cardiac Masses. Magn Reson Imaging Clin N Am 2008;16:137-164. Wednesday, March 7, 12