Successful Orthotopic Heart Transplant in an Infant With an Inflammatory Myofibroblastic Tumor of the Left Ventricle Michael V. Di Maria, MD, a David N. Campbell, MD, b Maxwell B. Mitchell, MD, b Mark A. Lovell, MD, c Biagio A. Pietra, MD, d and Shelley D. Miyamoto, MD d A female infant was diagnosed with an inflammatory myofibroblastic tumor involving the left ventricle, which compromised cardiac function. The tumor was endocardial in location and resection was not possible. In this study we report the first successful cardiac transplant and long-term follow-up for this indication. J Heart Lung Transplant 2008;27:792– 6. Copyright © 2008 by the International Society for Heart and Lung Transplantation. Primary cardiac tumors are extremely rare in infants and children, with a prevalence of 0.1%. 1 The majority of cardiac tumors in children are benign, present in in- fancy, and consist of rhabdomyomas, followed by fibro- mas. 2 Inflammatory myofibroblastic tumors (IMTs) of the heart are extremely uncommon, occurring primar- ily in children and young adults. Reports of cardiac IMTs consist of case reports and small series. 3–10 The exact IMT incidence has not been well described due to the lesion’s rarity and a still-evolving understanding of its histologic properties. The tumor occurs more fre- quently in the organs and soft tissues of the thorax and abdomen than in the heart. 11 This report describes the first successful cardiac transplantation of an infant with intracardiac IMT. CASE REPORT A 3-month-old infant girl presented to a local hospital emergency room with a low-grade fever and cough. In retrospect, the parents noted that tachypnea and in- creased effort of breathing had been intermittently present since birth. The infant was born at full term after an uncomplicated pregnancy and delivery. She had been feeding less vigorously and was being moni- tored for failure to thrive but was on no medications. Family history was remarkable for the mother and maternal grandmother having uterine fibroids and a maternal uncle with a pituitary tumor. Chest X-ray revealed an enlarged cardiothymic silhou- ette (Figure 1A). A transthoracic echocardiogram dem- onstrated a large mass on the left ventricular free wall, extending into the pericardial space (Figure 2A, B). Ventricular function was normal but a large pericardial effusion was present. Chest and abdominal computed tomography (CT) scans confirmed that the mass was adherent to the left ventricle; no other masses were identified (Figure 1B). She was taken to the operating room for pericardial drainage and biopsy of the tumor through a median sternotomy. On direct visualization, the coronary arteries were noted to course over the surface of the tumor making safe resection impossible. After pericardial drainage and low-dose inotropic sup- port the patient had a marked improvement in clinical symptoms. Biopsy results were diagnostic for IMT and she was referred to our institution for transplant con- sideration. At the time of transfer, she was 4 months old and was on milrinone at 0.5 g/kg/min, digoxin and lasix. After extensive evaluation revealed no evidence of systemic involvement or end-organ dysfunction she was deemed to be an acceptable candidate and was listed for transplant. Serial echocardiograms demon- strated that the mass was increasing in size with distortion of the mitral annulus, resulting in moderate mitral regurgitation and mild mitral stenosis (5 mm Hg mean gradient) (Figure 2C, D). Although there was some flow acceleration noted in the left ventric- ular outflow tract, there was no obstruction. One week after being listed she was noted to have premature atrial contractions (PACs), unifocal prema- ture ventricular contractions (PVCs) and several runs of non-sustained ventricular tachycardia. No electro- lyte abnormalities were identified and she was clini- cally stable during these episodes. Amiodarone ther- apy was initiated and digoxin discontinued with a marked improvement in the frequency of PACs and PVCs and complete resolution of the ventricular tachycardia. She continued to feed ad libitum by mouth and gained approximately 20 g/day. She did From the Departments of a Pediatrics, b Cardiovascular Surgery, c Pa- thology and d Cardiology, University of Colorado at Denver and Health Sciences Center, The Children’s Hospital, Aurora, Colorado. Submitted December 17, 2007; revised March 6, 2008; accepted March 12, 2008. Reprint requests: Shelley Miyamoto, MD, Department of Pediatrics, University of Colorado at Denver and Health Sciences Center, The Children’s Hospital, 13123 East 16th Avenue, B100, Aurora, CO 80045. Telephone: 720-777-5682. Fax: 720-777-7290. E-mail: miyamoto. shelley@tchden.org Copyright © 2008 by the International Society for Heart and Lung Transplantation. 1053-2498/08/$–see front matter. doi:10.1016/ j.healun.2008.03.005 792