Methodologic comparison of left ventricular stroke volumes in the early neonatal period by echocardiography.

Several methods for evaluating left ventricular stroke volume (SV) in neonates using echocardiography have been reported. However, no studies on methodologic comparison of SV with three-dimensional (3D) echocardiography are available. This is the first detailed report on a methodologic comparison of SV in the early neonatal period. The study group included 70 normal neonates (35 boys and 35 girls). An iE33 echocardiograph and Q-LAB supplied by Philips Electronics were used to examine and calculate volumes. Comparisons of SV were performed using Teichholz (T), the velocity time integral (VTI), Pombo (P), modified Simpson (MS), and 3D methods with normal neonates who had no persistent ductus arteriosus less than 7 days after birth. The mean SVs were 33.7 mL/m(2) (T), 30.6 mL/m(2) (VTI), 22.0 mL/m(2) (P), 17.5 mL/m(2) (3D), and 14.9 mL/m(2) (MS) using Haycock's formula of body surface area. The stroke volumes differed significantly depending on the different methods. The correlation coefficient was highest between the MS and 3D methods. The SVs of the T and VTI methods were significantly greater than those previously reported, and it seemed inappropriate to evaluate volumes in neonates. The 3D and MS methods were appropriate for measuring SV in neonates during the early neonatal period.

Spondylocostal dysostosis with tetralogy of Fallot and herniation of the spleen through the diaphragm.

Spondylocostal dysostosis (SCD) is a very rare syndrome characterized by vertebral malformation and rib deformity. Some of the patients with SCD have other birth defects in the central nervous system, the genitourinary tract, diaphragm or heart and so forth. There have been reported SCD with complex congenital heart disease, such as pulmonary atresia, double outlet right ventricle, and d-transposition of great arteries. However, there have been no reported SCD patients with confirmed tetralogy of Fallot (TOF). Here, a patient with SCD having a very rare combination of rib defects on the right side and left-sided scoliosis, tetralogy of Fallot, and diaphragmatic spleen herniation, which had not been reported before, was described.

Novel treatment criteria for persistent ductus arteriosus in neonates.

BACKGROUND: The indications for ductus arteriosus ligation in very-low-birth-weight infants (VLBWIs) with persistent ductus arteriosus (PDA) are unclear. Increased left ventricular end-diastolic dimension (LVDd) is commonly found in patients with PDA. Here, the enlargement of LVDd in term and preterm neonates without congenital heart disease was estimated by two-dimensional echocardiography. METHODS: The value of the measured LVDd was divided by the normal LVDd as an index (LVDd ratio) to compare 30 patients who underwent PDA ligation with 30 patients treated with indomethacin and 30 patients who did not undergo radical therapy. RESULTS: An LVDd ratio between 122% and 197% (mean, 142%) was considered to be an indication for the ligation procedure. The proportion of patients exceeding 130% in the LVDd ratio was 87% (26/30) in those patients who underwent ligation. Catecholamines and/or vasodilators were required in 83% patients for the treatment of low ejection fraction or hypertension after operations, suggesting that patients had been in preload and/or afterload remodeling failure during the operation. The percentage of patients with less than 115% in the LVDd ratio was 90% in the non-radical-therapy patients. The LVDd ratios of 130% and 115% were regarded as cut-off values for surgical ligation and indomethacin treatment. CONCLUSION: The LVDd ratio is a useful measure to determine the treatment of VLBWIs with PDA.

Paclitaxel-based chemotherapy for aggressive kaposiform hemangioendothelioma of the temporomastoid region: Case report and review of the literature.

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy and childhood. This tumor results in poor prognosis, and therefore, development of a more effective treatment is needed. METHODS AND RESULTS: We describe an 11-year-old boy presenting with left facial palsy caused by aggressive KHE of the left temporomastoid region. He was treated with paclitaxel-based chemotherapy, because of the difficulty with complete surgical resection for anatomic factor, multiple lung metastases on diagnosis, and no response to conventional treatments. This treatment reduced the volume of primary tumor and lung metastatic lesions, but the efficacy was transitory. CONCLUSIONS: Paclitaxel-based chemotherapy for aggressive KHE may be effective, therefore the multimodality therapy including paclitaxel of aggressive KHE, particularly in the head and neck, needs to be investigated in further studies.

Translocation (1;10)(p34;p15) in infant acute myeloid leukemia with extramedullary infiltration in multiple sites.

Primary acute myeloid leukemia (AML) with extramedullary infiltration (EMI) in multiple sites is rare. Herein, we describe a case of infant AML with EMI in multiple sites. She had dyspnea and hypofibrinogenemia at diagnosis, and systemic computed tomography revealed EMI in the orbit, gingiva, bronchial pathway, and urinary tract. Bone marrow examination confirmed the diagnosis of AML with t(1;10)(p34;p15). No case of AML with t(1;10)(p34;p15) has been reported in the literature. At the present time, she remains free of disease 12 months after bone marrow transplantation (BMT) in the second complete remission.