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 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.

Biventricular Repair of Double Outlet Right Ventricle: Preoperative Echocardiography and Surgical Outcomes.

OBJECTIVE: To discuss the key anatomic features of double outlet right ventricle (DORV) assessed by preoperative echocardiography among patients treated with different types of biventricular repair. METHODS: Surgical and echocardiographic databases were queried to identify patients who had undergone biventricular repair for DORV and had adequate preoperative echocardiographic imaging. All patients underwent pre- and postoperative echocardiography and clinical evaluation following discharge. RESULTS: Two hundred sixty-two patients with DORV met the inclusion criteria of the study. The patients were divided into two groups-intraventricular tunnel repair (IVR) to the aorta (194 [74%] patients) or to the pulmonary artery with either concomitant arterial switch operation or double-root translocation (68 [26%] patients). Among 68 patients undergoing IVR to the pulmonary artery, 50 patients with transposition of the great arteries (TGA) type of DORV and 7 patients with remote ventricular septal defect (VSD) type underwent IVR plus arterial switch operation and 6 patients with TGA type and 5 patients with remote VSD type underwent IVR plus double-root translocation. There were three hospital deaths and one late death (overall operative mortality: 1.5%). CONCLUSION: Preoperative echocardiography provided crucial data to estimate the feasibility of intraventricular tunnel creation to either the aorta or the pulmonary artery and to guide the selection of either arterial switch or double-root translocation. Biventricular repair could be achieved with favorable outcomes in most patients with DORV.

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.

Echocardiographic Classification and Surgical Approaches to Double-Outlet Right Ventricle for Great Arteries Arising Almost Exclusively from the Right Ventricle.

Selecting an appropriate surgical approach for double-outlet right ventricle (DORV), a complex congenital cardiac malformation with many anatomic variations, is difficult. Therefore, we determined the feasibility of using an echocardiographic classification system, which describes the anatomic variations in more precise terms than the current system does, to determine whether it could help direct surgical plans. Our system includes 8 DORV subtypes, categorized according to 3 factors: the relative positions of the great arteries (normal or abnormal), the relationship between the great arteries and the ventricular septal defect (committed or noncommitted), and the presence or absence of right ventricular outflow tract obstruction (RVOTO). Surgical approaches in 407 patients were based on their DORV subtype, as determined by echocardiography. We found that the optimal surgical management of patients classified as normal/committed/no RVOTO, normal/committed/RVOTO, and abnormal/committed/no RVOTO was, respectively, like that for patients with large ventricular septal defects, tetralogy of Fallot, and transposition of the great arteries without RVOTO. Patients with abnormal/committed/RVOTO anatomy and those with abnormal/noncommitted/RVOTO anatomy underwent intraventricular repair and double-root translocation. For patients with other types of DORV, choosing the appropriate surgical approach and biventricular repair techniques was more complex. We think that our classification system accurately groups DORV patients and enables surgeons to select the best approach for each patient's cardiac anatomy.

The role of myocardial viability assessed by perfusion/F-18 FDG imaging in children with anomalous origin of the left coronary artery from the pulmonary artery.

PURPOSE: Although surgical treatments evolved, the short-term postoperative mortality is still high in children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), and long-term survivors may suffer from restrained functional recovery. Therefore, an optimal means in predicting postoperative reversal is demanded. In this study, we assess the utility of myocardial perfusion/F-18 fluorodeoxyglucose (FDG) imaging in the evaluation of myocardial viability and postsurgery functional recovery in children with ALCAPA. MATERIALS AND METHODS: A retrospective study was performed in 7 children with diagnosed ALCAPA who underwent myocardial perfusion/F-18 FDG imaging preoperatively. Global viability index was used to evaluate myocardial viability and was compared with the preoperative deviations of left ventricular ejection fraction (LVEF) and left ventricular end-diastolic diameter (LVEDD) from age-matched healthy children and with the postoperative durations of intensive care. RESULTS: Children with more viable myocardium had less severe clinical symptoms. The viability index was correlated well with the preoperative deviations of LVEF (r = -0.98, P = 0.001) and LVEDD (r = 0.87, P = 0.02) and postoperative durations of intensive care hospitalization (r = 0.77, P = 0.04) and mechanical ventilation (r = 0.83, P = 0.02). LVEF and LVEDD reached normal range within 5 months in viable children, whereas incomplete reversal was observed in partial- and nonviable children. CONCLUSIONS: In children with ALCAPA, myocardial viability evaluated by perfusion/F-18 FDG imaging is related to the preoperative clinical manifestations and cardiac function. Additionally, it may predict functional recovery after surgical repair.

[Surgical treatment of aortic coarctation with intracardiac anomaly in infants and toddlers].

OBJECTIVE: To review the experience in repair of aortic coarctation with intracardiac anomaly in infants and toddlers. METHODS: 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. RESULTS: There were 8 hospital deaths. The mortality is 9.5%. Among 8 deaths, 3 patients were misdiagnosed. CONCLUSIONS: 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.