Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.     

64层螺旋CT诊断先天性冠状动脉起源异常的价值

目的：探讨64层螺旋CT冠状动脉造影诊断冠状动脉起源异常的临床价值。材料和方法：回顾性总结2005年12月至2006年12月间1800例64层螺旋CT冠脉造影的结果，统计冠状动脉起源异常的发生率。结果：1800例检查者中发现冠脉起源异常51例，发病率2．8％。包括右冠状动脉变异11例（21．6％）、左冠状动脉变异8例（15．7％）、左右冠均发生变异1例（2％）、单支冠状动脉1例（2％）、分支变异28例（54．9％）。结论：64层螺旋CT是诊断冠状动脉起源异常的安全有效方法，对临床治疗有重要的意义。     

合并异常冠状动脉法洛四联症和右心室双出口一期根治术

目的 总结合并异常冠状动脉的法洛四联症和右心室双出口一期根治术的经验，探讨有关外科技术的改进。方法1995年6月至2002年6月完成该类一期根治术12例，其中2例采取了肺动脉移位、3例在游离的左前降支下加宽右室流出道、5例改变右室切口并在冠状动脉下缝合、2例经肺动脉和右房疏通流出道。结果无手术死亡。随访3个月～6年，无晚期死亡和并发症发生。结论合并异常冠状动脉的法洛四联症和右心室双出口的一期根治手术是可行的，且效果良好，但必须采用适当的外科技术以保护异常的冠状动脉。     

Successful triplet pregnancy in a patient with a unicornuate uterus with a cavitary communicating rudimentary horn

In a patient with primary infertility, ovulation was inducedby monitored stimulation with human menopausal gonadotrophins(HMG) because of polycystic ovarian disease. Infertility work-uphad shown a unicornuate uterus with a cavitary communicatingrudimentary horn. The husband showed a varicocele-related moderateoligoasthenoterato-zoospermia. A triplet pregnancy occurredin a third HMG ovulation induction cycle combined with intra-uterineinsemination of the husband's washed semen. The pregnancy wascarefully monitored, and measures to prevent premature deliverywere taken. Because of the patient's obvious discomfort in thepresence of premature labour, Caesarean section was performedat 33 weeks gestation and three healthy infants were delivered.This is the first report of a successful triplet pregnancy ina women with a unicornuate uterus. The reproductive and obstetricoutcome of this condition in general, and in the case of multiplepregnancy, is discussed.     

Congenital defects of lower limbs and associated malformations: A population based study

As part of a detailed study of limb defects and associated patterns of congenital malformations, cases with lower limb deficiencies were analysed separately. We identified a total of 130 cases with deficiencies of the lower limbs without defects of the upper limbs. This gives an incidence of 1.07/10,000 livebirths, or 1/9,337 for this group of limb defects. Most common were femur deficiencies and deficiencies of the foot. A preponderance of males was found in the group of transverse defects of the leg (fibula/tibia deficiencies) and central axis deficiencies, while females had significantly more often longitudinal tibia defects and preaxial ray defects. © 1993 Wiley-Liss, Inc.