Primary cardiac rhabdomyosarcoma developed after receiving radiotherapy for left breast cancer 18 years prior.

The heart is an organ where primary malignant tumors rarely develop. In particular, the incidence of cardiac rhabdomyosarcoma (RMS) is extremely low. It has been reported that the risk of second malignant tumors in mediastinum is increased by radiotherapy in women with breast cancer. However, little is known about the association between irradiation to heart and cardiac RMS. Here, we report a case of a 68-year-old woman with primary cardiac RMS. She suddenly presented syncope at a workplace, and was taken to the emergency room at our hospital. Several imaging tests, including echocardiogram and cine magnetic resonance imaging, detected two tumors in the right ventricle (RV) and its outflow tract, which had almost obstructed the main trunk of the pulmonary artery (PA). To avoid sudden PA occlusion by the tumor, we emergently performed surgical excision of the tumors from the RV. Pathological analysis revealed that these tumors were embryonal type RMS. She had received radiotherapy after mastectomy for left breast cancer 18 years previously, and no recurrence of breast cancer had been detected. This cardiac RMS is considered as a second malignant tumor related to radiotherapy for breast cancer. .

Complete repair of a total defect type aortopulmonary window in an extremely low birth weight neonate.

The authors performed a complete repair for a total defect type aortopulmonary window (APW) in an extremely low birth weight neonate weighing 1.1 kg. The APW was repaired using an autologous pericardial patch. A novel cardiopulmonary bypass system with low priming volume allowed a safe surgery for the premature patient.

Does a bidirectional Glenn shunt improve the oxygenation of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum?

OBJECTIVE: There is a risk of myocardial ischemia in patients with pulmonary atresia and intact ventricular septum associated with the right ventricle-dependent coronary circulation. In this patient group, the oxygen delivery to the myocardium depends on the oxygen saturation of the right ventricular cavity. We hypothesized that bidirectional Glenn shunt would improve the oxygenation of right ventricle-dependent coronary circulation relative to a systemic-pulmonary artery shunt. The reduction of systemic venous return to the right atrium due to a bidirectional Glenn shunt could increase the oxygen saturation of the right ventricle in the clinical setting, when the mixture of systemic and pulmonary venous blood is unchanged at the atrial level. METHODS: Patients with right ventricle-dependent coronary circulation were defined as those with right ventricle-coronary artery fistulas plus stenoses of the right or left coronary arteries. For 7 patients with right ventricle-dependent coronary circulation before and after bidirectional Glenn shunt, cardiac catheterization was performed and the oxygen saturation of the right ventricular cavity was measured. RESULTS: For all 7 patients, the bidirectional Glenn shunt was performed at a mean age of 18 months. Ischemic changes in the electrocardiogram before the bidirectional Glenn shunt improved after the procedure in 2 patients. The oxygen saturation of the right ventricular cavity before the bidirectional Glenn shunt was 54.6 +/- 8.8%, and that after the BGS significantly increased to 75.6% +/- 5.8% (P < .01). All 7 patients have subsequently undergone the Fontan procedure with excellent results. CONCLUSION: Early bidirectional Glenn shunt could prevent progression of myocardial ischemia in pulmonary atresia with intact ventricular septum with right ventricle-dependent coronary circulation.

One-lung ventilation for video-assisted thoracoscopic interruption of patent ductus arteriosus.

PURPOSE: Video-assisted endoscopic techniques have recently been employed in congenital heart surgery for patent ductus arteriosus (PDA) interruption. We report our preliminary experience of using a new technique of single-lung ventilation to perform video-assisted thoracoscopic PDA interruption (VATS-PDA) in small infants and children. METHODS: Sixteen infants with a mean body weight of 6.5 +/- 2.4 kg (range 2.6-12.8 kg) underwent VATS-PDA under selective right-lung ventilation using a 2-F balloon catheter for arterial embolectomy. RESULTS: We did not need to reposition the retractor or reinflate the atelectatic lung, as there was no transient hypoxia or hypercarbia. The mean procedure time was 81 +/- 27 min (range 45-145 min) and all patients, with the exception of one with a total anomalous pulmonary venous connection, were extubated in the operating room. CONCLUSION: This technique using single-lung ventilation for infants and small children was safe and effective in providing pediatric thoracic access and exposure within confined and delicate anatomic spaces.