• Journal of Chest Surgery
• /
• v.26 no.10
• /
• pp.804-807
• /
• 1993

We experienced a case of cardiac rhabdomyoma,which is incidentally found at perinatally checked sonography.The cardiac rhabdomyoma is the most common cardiac tumor of infants & children, and second most common cardiac tumor of all age groups, which is usually multiple in the case of 90%, invariably involves the ventricles affecting the left & right side equally. In more than fifty percents, the size of cardiac rhabdomyoma is enough large to threatening the life of newborn within 24hrs of birth. Cardiac rabdomyoma is actually a myocardial harmatoma rather than a true neoplasm,because of the finding of complete lack of mitotic activities. Recently,more advanved instruments such as ultrasonography or echocardiography allows to us early detection & surgical intervention of this tumor. In our case, the tumor was found at both ventricles, which occupied nearly total chambers of both ventricles. The patient was operated on 3 day after birth. The operation was removal of the tumor through left ventriculotomy and right ventriculotomy respectively. He expired in the immediate postoperative period due to low cardiac output syndrome, despite of massive inotropic agents.

• Journal of Chest Surgery
• /
• v.24 no.11
• /
• pp.1138-1143
• /
• 1991

Primary cardiac tumors are rare, being found in approximately 1 in 10, 000 routine autopsies in patients of all ages. of the primary cardiac tumors, 75% to 80% of patients are benign lesion, of the benign cardiac tumors, myxoma is the most common type in adults, whereas rhabdomyoma predominates in infants and children. About 50% of cardiac rhabdomyoma patients, die in the first 6 months of life and 80% by 1 year of age. We report on the one infant and the one neonate operated upon successfully for removal of cardiac rhabdomyoma.

• Journal of Chest Surgery
• /
• v.35 no.6
• /
• pp.467-470
• /
• 2002

The most common primary heart tumor in infants is the rhabdomyoma. It is known that tuberous sclerosis is found in half of the patients with cardiac rhabdomyoma. Because the prognosis of this tumor associated with subaortic stenosis is poor, the surgical intervention is indicated. We report one case of left ventricular rhabdomyoma with tuberous sclerosis.

• Journal of Chest Surgery
• /
• v.29 no.6
• /
• pp.655-659
• /
• 1996

Primary cardiac tumors are known to be rare. Of the benign primary tumors, rhabdomyoma predominates in neonates and infants. Tuberous sclerosis is found in hall of the patients with rhabdomyomas. Rhabdomyomas demonstrate benign pathological characteristics and may regress spontaneously that resection of the intracavitary obstructing portion of these tumors are recommended. We performed resection of intraatrial rhabdomyoma which had been obstructing mitral valve orifice and caused heart failure in a neonate. Postoperatively, fungal endocarditis developed and after 3 days, rapid growth and extension of the vegetation in the aorta obstructed the flow through the ascending aorta and resulted in hemodynamic deterioration.

• Journal of Chest Surgery
• /
• v.30 no.2
• /
• pp.205-208
• /
• 1997

Cardiac rhabdomyomas are the moil common primary tumor in infancy and childhood and are frequently associated with tuberous sclerosis. Although spontaneous regression of cardiac rhabdomyoma has been reported, prognosis of this tumor associated with subaortic stenosis is still considered to be poor and surgery continues to be indicated. A 4-day-old female was admitted due to tachypnea and cyanosis. Single rhabdomyoma arising from the interventricular septum associated with severe subaortic stcnosls as partially removed under cardiopulmonary bypass. Excised tumor sizc was 0.7$\times$0.9$\times$0.4cm in dimension. The postoperative course was uneventful and the infant discharged on the 14th postoperative day.

• Journal of Chest Surgery
• /
• v.43 no.6
• /
• pp.725-728
• /
• 2010

Rhabdomyoma is the most common benign cardiac neoplasm in neonates. Most patients with rhabdomyoma experienced spontaneous regression. Yet some of them need surgical therapy because of hemodynamic problems of the heart such as arrhythmia, outflow tract obstruction and valvular dysfunction. We found multiple masses in both ventricles on the patient's fetal echocardiogram. Heart failure caused by severe left ventricular outflow tract obstruction quickly presented after birth. The mass interfering with the outflow tract was resected via the transaortic approach at the first day of birth. Postoperative echocardiography showed complete release of the outflow tract obstruction. He was discharged on the postoperative day 8. During the 3 years of follow up, we found that the sizes of the remnant masses had gradually decreased.

• Clinical and Experimental Pediatrics
• /
• v.51 no.7
• /
• pp.766-770
• /
• 2008

Primary tumors of the heart are uncommon among pediatric patients. Rhabdomyoma is the most frequent cardiac tumor in infants and children, which is commonly associated with tuberous sclerosis. Tuberous sclerosis is a neurocutaneous syndrome affecting the brain, heart, skin, and other organs. Cardiac rhadomyomas are reported in 50-64% of infants with tuberous sclerosis. Tuberous sclerosis involves multiple locations in the atrium, ventricle and septum, and may induce mechanical obstruction of the outflow tract and heart failure depending on the location, number, size, and degree of invasion of tumors. Arrhythmias may also develop in infants with cardiac rhadomyomas, but only a few of these patients require prolonged anti-arrhythmic therapy because arrhythmia often disappears with spontaneous regression of the tumors, and the ultimate prognosis may be decided by the cerebral manifestations.

• Journal of Chest Surgery
• /
• v.35 no.10
• /
• pp.745-749
• /
• 2002

Cardiac rhabdomyoma is a rare type of benign tumor affecting the heart. There are a few previous reports of intracardiac rhabdomyomas causing ventricular arrythmia. We describe a 1-year-old female tuberous sclerosis patient who was presented with a ventricular tachycardia. Diagnostic echocardiography revealed two masses in the left ventricular outflow tract originating from the ventricular septum. The masses were surgically resected through aortotomy using cardiopulmonary bypass and the masses appeared benign. The pathology was that of a cardiac rhabdomyomas. Postoperative course was uneventful and the ventricular tachycardia was controlled.

• Clinical and Experimental Pediatrics
• /
• v.46 no.9
• /
• pp.913-917
• /
• 2003

Cardiac rhabdomyomas are relatively uncommon and associated with tuberous sclerosis in 40-50% cases. We report a 10-month-old infant with tuberous sclerosis who presented with ventricular arrythmias and status epilepticus. There were hypopigmented macules on the body, periventricular calcifications, renal cyst and cardiac rabdomyomas just below the aortic valve. The patient required resection of left ventricular subaortic masses due to sustained arrythmia in spite of intravenous amiodarone therapy. The pathologic examination confirmed the diagnosis of rhabdomyoma. The patient had no more arrythmia during the 14 month follow up period. Although cardiac rhabdomyomas may spontaneously regress, surgery is often necessary and frequently resolves the underlying arrythmia.

• Journal of Chest Surgery
• /
• v.42 no.6
• /
• pp.770-773
• /
• 2009

Rhabdomyoma has been reported to be the most common type of cardiac tumors in fetuses and children, and multiple cardiac rhabdomyomas almost certainly signify the association with tuberous sclerosis. We report here on a case of Tetralogy of Fallot (ToF) that was associated with multiple rhabdomyomas and tuberous sclerosis. A two-year-old boy, who had undergone systemic-pulmonary shunt during the neonatal period, received total correction of his ToF after the complete regression of the cardiac tumor. The postoperative course was uneventful, and he has been follow-up for 2 months. The boy is currently in an excellent condition.