Preoperative Evaluation and Midterm Outcomes after the Surgical Correction of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in 50 Infants and Children

Background： Anomalous origin of the left coronary artery （LCA） from the pulmonary artery （ALCAPA） is a rather rare congenital anomaly that has a profound effect on heart function. This study aimed to retrospectively illustrate the perioperative clinical features, therapy experience, and midterm outcomes after surgical correction, and to determine the value of left ventricular ejection fraction （LVEF） and myocardial viability in differentiating critically ill patients among infants and children with ALCAPA. Methods： From April 1999 to March 2013, infants and children patients diagnosed with ALCAPA in Beijing Fuwai Hospital were analyzed. Clinical data of patients were summarized and retrospectively analyzed. All patients were divided into two groups according to LVEF level （Group 1： LVEF 〉50%, or Group 2： LVEF ≤50%） to compare perioperative and follow-up variables. Effect of myocardial viability evaluated according to myocardial perfusion/18F-fluorodeoxyglucose （FDG） imaging on the clinical variables was also analyzed. Results： A total of 50 patients with ALCAPA （male/female： 29/21; median age： 3.1 years [range： 4 months to 18 years]） were included. Younger age, lower weight, intercoronary collaterals （ICC） dysplasia, ratio of the proximal right coronary artery diameter to the aortic root diameter 〈0.2, and larger cardiothoracic ratio （CTR） were more frequently found in Group 2 than those in Group 1. Forty-seven patients underwent cardiac surgery. The times of cross-clamp and cardiopulmonary bypass were not different between the two groups; however, the duration of mechanical ventilation and postoperative Intensive Care Unit stay were longer in Group 2 than those in Group 1. Follow-ups were possible in 38 patients （80.9%）; median time： 84.5 months （range： 49 months to 216 months）. There was one late sudden death with simple ligation of the LCA at 8 months after surgery. No severe complications and reoperation occurred. The relationship of the grades of myocardial viability and clinical features was analyzed in 15 patients with myocardial perfusion/18F-FDG imaging, and the results showed that myocardial viability correlated well with LVEF, CTR, abnormal Q waves, and left ventricular end-diastolic dimension. It was not correlated with age, mitral regurgitation, and ICC. Heart implantation was decided in one patient with little viable myocardium; however, this patient died 2 months after the diagnosis while waiting for transplantation. Two patients with no viable myocardium in the area of aneurysm had aneurysmectomy concomitantly. Conclusions： In infants and children withALCAPA, heart function and myocardial viability are closely related to clinical features. LVEF and the grades of myocardial viability can differentiate high-risk patients before surgery and in the early stage of recovery after surgery. The area and extent of myocardial infarction are also crucial in making preoperative clinical decisions. However, even in patients with depressed ventricular function and severe myocardial infarction, the midterm follow-up showed satisfactory recovery of cardiac function after the successful restoration of a dual-coronary arterial system.

Coarctation of the Aorta with Aortic Arch Hypoplasia：Midterm Outcomes of Aortic Arch Reconstruction with Autologous Pulmonary Artery Patch

Background： Coarctation of the aorta （CoA） with aortic arch hypoplasia （AAH） is a relatively common congenital heart disease in clinical practice. Nonetheless, the corrective surgical technique for infants and children is a clinical problem that remains controversial. In this study, we sought to evaluate the surgical effects of aortic arch （AA） reconstruction with coarctation resection and aortoplasty with autologous pulmonary artery patch for infants and young children with CoA and AAH. Methods： Between January 2009 and December 2015, a total of 22 infants and young children with CoA and AAH who underwent coarctation resection and aortoplasty with autologous pulmonary artery patch were enrolled in this study. The median age of patients was 4.5 （Q1, Q3： 2.0, 14.0） months and the median body weight was 5.75 （Q1, Q3： 4.10, 9.38） kg. All patients were diagnosed with CoA and AAH, and concomitant cardiac anomalies were corrected in one stage. Perioperative and postoperative data were collected and analyzed using the paired sample t-test. Results： No perioperative deaths occurred. No residual obstruction was detected by echocardiography. The postoperative pressure difference across the repaired segment of CoA was 14.05 ± 4.26 mmHg （1 mmHg = 0.133 kPa）, which was smaller than the preoperative pressure difference （48.30 ± 15.73 mmHg; t = -10.119, P 〈 0.001）. The median follow-up time was 29.0 （Q1, Q3： 15.5, 57.3） months. There was no death during the follow-up period, and all patients experienced obvious clinical improvement. Only one child underwent subsequent aortic balloon angioplasty due to restenosis. Computed tomography angiography showed that the AA morphology was smooth, with no aortic aneurysm or angulation deformity. Conclusion： AA reconstruction with coarctation resection and aortoplasty with autologous pulmonary artery patch could effectively correct CoA with AAH, and the rate of reintervention for restenosis is low.