PV annular enlargement with valve repair has reasonable long-term results and yields a lower long-term incidence of significant PR compared with the simple transannular patch enlargement technique.

No abstract

Background Primary Ewing sarcoma of the cranial bone is rare, accounting for only 1% of all Ewing sarcomas. Primary Ewing sarcoma arising in the squamous temporal bone is particularly rare. Methods A 16‐year‐old male was seen with signs of sudden sensorineural hearing loss (SSNHL). After 1 week of SSNHL and new‐onset headache, imaging studies showed a mass that originated in the left squamous temporal bone with intracranial and extracranial extension. Histopathological study revealed that the mass was a Ewing sarcoma. Results The patient manifested the diagnostic EWSR1 mutation and was treated with adjuvant multidrug chemotherapy and focal radiotherapy after surgery according to the Children's Oncology Group interval compression arm of AEWS0031 with a regimen of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide/etoposide. Conclusions This case showed an extremely uncommon location, as well as unusual symptoms of primary Ewing sarcoma.

Key words coarctation of the aorta, diverticulum, Kabuki syndrome, left ventricle.The present patient was an 11-month-old male infant, who had undergone surgical repair of coarctation of the aorta (CoA) and atrial septal defect (ASD) at 7 days of age. He was clinically diagnosed with Kabuki syndrome (KS) on the presence of characteristic facial features, clinodactyly of the fifth finger, persistent finger fetal pads, postnatal growth retardation, and developmental delay. At the time of initial computed tomography (CT), small crypt (diameter, 2.6 mm) was noted on the lateral side of the left ventricle (LV), and we decided to follow up this lesion. When he visited the outpatient clinic for a periodic exam at 7 months old, we noted an LV diverticulum (LVD) where the small crypt was located on 2-D echocardiography. On subcostal view, an accessory tongue-shaped chamber with a narrow neck (diameter of the body, 5 mm) was seen in the LV apex. The contraction of the diverticulum was synchronous with that of the LV, and color flow mapping indicated rapid diastolic filling inside the diverticulum. Because the patient was asymptomatic, we decided to do medical follow up frequently. On 2-D echocardiography at 9 months of age ( Fig. 1a), the LVD had increased in size to approximately 9.6 mm (diameter of the body) compared with the previous imaging. On cardiac catheterization, we gained more precise imaging of the location, size and morphology of the LVD (Fig. 1b). Although the patient had no symptoms, we were concerned about life-threatening complications of LVD such as cardiac rupture because the LVD was increasing rapidly. Finally, surgery was decided upon, to avoid life-threatening complications. At the age of 11 months, he underwent redo median sternotomy with preparation for cannulation. The diverticulum was identified arising from the LV near the left anterior descending artery. Tenting sutures were placed at the diverticular sac, and the diverticular sac was then incised (Fig. 1c). The diverticular stump was identified with coronary probe, and obliterated with purse-string sutures. The diverticular sac was closed with bovine pericardium, pledget-buttressed horizontal mattress sutures and

No abstract