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Objectives: To compare the effect between the right ventricle to pulmonary artery connection and the systemic-to-pulmonary artery shunt palliative surgery in patients with pulmonary atresia and ventricular septal defect. Methods: A total of 92 consecutive patients (mean age [1.69 ± 1.72] years, range 0.2-8.1 years) diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index [87.51 ± 36.97] mm2/m2; McGoon ratio 0.97±0.28) from December 2009 to August 2012 in our hospital were included in this retrospective study. 45 patients underwent the procedure of right ventricle to pulmonary artery connection (RV-PA) and 47 underwent the systemic-to-pulmonary artery shunt surgery, some patients also received simultaneous transcatheter occlusion of major aortopulmonary collaterals and/or pulmonary angioplasty. The mechanical ventilation time and ICU monitoring time, the pulmonary vascular growth and the anatomical repair rate were compared between the two groups. Results: The mean follow up time was (2.01±1.02)years. Nakata index and McGoon ratio equally increased significantly post operation as compared to baseline level (P<0.001). Incidence of final anatomical repair was significantly higher in the RV-PA group than in the systemic-to-pulmonary artery shunt group (62.2% vs 31.9%,P<0.01). During the anatomical repair operation, the incidence of cyanosis improvement was significantly higher, while the operation time was significantly shorter in the RV-PA group than in the systemic-to-pulmonary artery shunt group(both P<0.05). Mechanical ventilation time and ICU monitoring time also tended to be shorter in the RV-PA group (P>0.05). Conclusions: The palliative right ventricle to pulmonary artery connection surgery is related to higher rate of final anatomical repair in patients with pulmonary atresia and ventricular septal defect and may help the patients recover after the anatomical repair.
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<p><b>BACKGROUND</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>In infants and children with ALCAPA, 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.</p>
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<p><b>BACKGROUND</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>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.</p>
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Objective To retrospectively study 50 children with anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA) and find the simple and practical indexes that may predict myocardial viability and the possible risk factors that may affect the choice of operation and the recovery after surgery.Methods A retrospective study was performed in 50 children with ALCAPA [29 male,21 female,aged from 4 months to 18 years,average (4.49 ±4.30) years] between Apr.1999 and Mar.2013.The preoperative examination included electrocardiogram (ECG),echocardiography,chest X-ray,mutislice spiral computerized tomography (MSCT),angiocardiography et al.Follow-up data were obtained by reviewing the records made in out-patient and recent telephone call.According to myocardial 18F-FDG imaging,the extent of myocardial viability of 15 patients with ALCAPA was classified into 4 grades.0 normal myocardial; 1 viable myocardial ;2 partial viable myocardial;3 myocardial infarction.Meanwhile,the global clinical scores were calculated and divided into 10 levels according to the deviations of clinical manifestations which included abnormal Q waves,left ventricular ejection fraction (LVEF),left ventricular end-diastolic dimension (LVED),cardiothoracic ratio(CTR),intercoronary collaterals(ICC),mitral regurgitation(MR) and aneurysm.The relationship of the extent of myocardial viability and clinical features were studied.All patients with ALCAPA were classified into groups by the global clinical scores and the preoperative,perioperative and post operative clinical manifestations were compared among groups.Results The extent of myocardial viability was related well to the global clinical scores (r =0.936,P ＜0.001),LVEF(r =0.783,P ＜ 0.001),CTR (r =0.770,P ＜ 0.002),abnormal Q waves (r =0.667,P ＜ 0.01) and LVED (r =0.637,P ＜ 0.02),but was not related to age,MR and ICC (r =-0.206,-0.268,-0.342,all P ＞ 0.05).The results showed that grade 0-1 equaled scores 0-3,grade 2 equaled scores 3-5,grade 3 equaled scores ＞ 5.Chil-dren with less viable myocardium had more severe clinical symptoms.Among abnormal Q waves,LVEF ＜ 50％,CTR ＞0.65,ICC dysplasia,median to severe MR and the global clinical scores ＞ 3,the global clinical scores ＞ 3 and LVEF ＜50％ showed a good predict of myocardial infarction.The preoperative,perioperative and post operative clinical manifestations were compared between 2 groups divided by the global clinical scores (group A,scores ≤ 3 and group B,scores ＞3) in all patients.More infants and toddlers,abnormal Q waves,ICC dysplasia,RCA/AO ＜0.2,larger CTR and lower LVEF were found in group B than those in group A.The time of cross-clamp and CPB was not different between the 2 groups,but the postoperative ventilation time and postoperative ICU stay were longer in group B than in group A.Fortyseven patients underwent operation and there were no operative deaths.One infant with scores 9 died while waiting heart transplantation.Follow-ups from 1 to 168 months were conducted in 38 patients(80.9％,38/47 cases) and 1 patient with ligation of the left coronary artery (LCA) had a sudden death after 8 months of surgery.All patients had gotten smaller LVED after surgery.Nineteen patients had mild MR and 3 patients had mild supravalvular pulmonary stenosis in following-up.Of the 16 patients with LVEF ＜ 50％,14 had a recovery of LVEF,2 patients still had LVEF ＜ 50％.Conclusions In children with ALCAPA,the extent of myocardial viability evaluated by myocardial 18 F-FDG imaging is related closely to the preoperative clinical manifestations.The global clinical scores ＞ 3 and LVEF ＜ 50％ have a good predict of myocardial infarction.Even in young children with severely depressed left ventricular function,higher global clinical scores and more myocardial infarction,median and long-term follow-ups showed satisfactory recovery of cardiac function after successful restoration of a dual coronary arterial system.
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<p><b>OBJECTIVE</b>To review the experience in repair of aortic coarctation with intracardiac anomaly in infants and toddlers.</p><p><b>METHODS</b>From January 2000 to December 2006, 84 infants and children diagnosed as aortic coarctation with intracardiac anomaly underwent surgical treatment. Mean age of the patients was 13.5 months, with a range from 1 month to 3 years. Mean body weight was 7.3 kg, with a range from 3.3 to 15 kg. Twelve patients complicated with complex intracardiac anomaly. Seventy-two patients complicated with ventricular septal defect and other simple anomaly. Twenty-one patients had hypoplasia of the aortic arch. Sixty-two patients had one-stage repair. Median sternotomy was used to simultaneously repair coarctation and intracardiac defect in 49 patients. Left thoracotomy and median sternotomy were applied to repair aortic coarctation and intracardiac anomaly respectively in 13 patients. Twenty-two patients had staged repair. Operational techniques for aortic coarctation include 42 patients of patch aortoplasty, 30 patients of resection and end-to-end anastomosis, 6 patients of subclavian flap aortoplasty, 3 patients of vascular bypass, and 1 patient of balloon dilation. In all 49 patients of one-stage operation through median sternotomy, selective cerebral perfusion was used in 43 patients, deep hypothermia low flow was applied in 4 patients, deep hypothermia circulatory arrest was performed in 2 patients.</p><p><b>RESULTS</b>There were 8 hospital deaths. The mortality is 9.5%. Among 8 deaths, 3 patients were misdiagnosed.</p><p><b>CONCLUSIONS</b>Surgeries for aortic coarctation with intracardiac anomaly have satisfactory short-term results in infants and toddlers. One-stage repair through median sternotomy can be applied to most of the patients. Selective cerebral perfusion with deep hypothermia and circulatory arrest in lower body can protect the brain and other vital organs.</p>