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Objectives: We aimed to investigate the safety, feasibility and early and mid-term outcomes of total repair of elective tetralogy of Fallot through a right subaxillary thoracotomy., Methods: Between May 2010 and September 2015, a retrospective review of patients with elective tetralogy of Fallot undergoing primary repair through a right subaxillary thoracotomy was performed. This study included 47 patients [27 males, 20 females; the mean age of 1.7 ± 1.5 years (ranging from 3 to 106 months); body weight of 9.7 ± 2.8 kg (ranging from 5 to 16 kg); McGoon ratio ≥1.5]. The ventricular septal defects were approached through a right atriotomy in 30 patients, a right ventriculotomy in 8 patients and the combination of a right atriotomy and ventriculotomy in 9 patients. Transannular and non-transannular patches were used in 20 and 27 patients, respectively., Results: There was 1 operative death (2.1%), and the others recovered uneventfully. The median cardiopulmonary bypass and aortic cross-clamping times was 79 ± 20.8 (45-136) and 50 ± 15.7 (25-94) min, respectively. The mechanical ventilation time, length of intensive care unit and postoperative hospital stay were 0.9 ± 0.6, 2.6 ± 1.1 and 10.7 ± 2.7 days, respectively. The main complications involved low cardiac output syndrome in 5 patients, arrhythmia in 6 patients, pulmonary infection in 4 patients, small residual shunt in 2 patients, mild residual right ventricular outflow tract obstruction in 2 patients, mild tricuspid regurgitation in 3 patients and atelectasis in 1 patient. The mean follow-up time was 82 ± 22.0 months, and no late deaths occurred during the follow-up. One (2.1%) patient received reintervention for residual significant pulmonary artery stenosis. That was in addition to 2 (4.3%) patients having mild residual right ventricular outflow tract, 1 (2.1%) patient with moderate tricuspid regurgitation and 3 (6.4%) patients with moderate pulmonary valve regurgitation to be followed up., Conclusions: The right subaxillary thoracotomy, providing a safe and feasible alternative, can be performed with a low mortality rate and satisfactory early and mid-term outcomes for total repair of elective tetralogy of Fallot., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: We sought to investigate the impact of 22q11.2 deletion on perioperative outcome in tetralogy of Fallot., Methods: We conducted a retrospective review of patients with tetralogy of Fallot who underwent complete surgical reconstruction at The Children's Hospital of Philadelphia between 1995 and 2006. Inclusion criteria included diagnosis of tetralogy of Fallot and known genotype. Fisher exact and Mann-Whitney tests were used for categoric and continuous variables, respectively. Regression analysis was used to determine whether deletion status predicts outcome., Results: We studied 208 subjects with tetralogy of Fallot, 164 (79%) without and 44 (20%) with 22q11.2 deletion syndrome. There were no differences in sex, race, gestational age, age at diagnosis, admission weight, and duration of mechanical ventilation. Presenting anatomy, survival, complications and reoperations were also comparable between patients with and without 22q11.2 deletion syndrome. Those with 22q11.2 deletion syndrome had more aortopulmonary shunts preceding complete surgical repair (21% vs 7%, P = .02). This association was present after adjustment for presenting anatomy (stenosis, atresia, or absence of pulmonary valve and common atrioventricular canal) and surgical era. In addition, those with 22q11.2 deletion syndrome had longer cardiopulmonary bypass time (84 vs 72 minutes, P = .02) and duration of intensive care (6 vs 4 days, P = .007)., Conclusions: Genotype affects early operative outcomes in tetralogy of Fallot resulting, in particular, in longer duration of intensive care. Future studies are required to determine factors contributing to such differences in this susceptible population., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Patients with repaired tetralogy of Fallot are monitored for pulmonary regurgitation (PR) and right ventricular (RV) function. We sought to compare measures of PR and RV function on echocardiogram to those on cardiac magnetic resonance (CMR) and to develop a new tool for assessing PR by echocardiogram., Methods and Results: Patients with repaired tetralogy of Fallot (n=143; 12.5±3.2 years) had an echocardiogram and CMR within 3 months of each other. On echocardiogram, RV function was assessed by (1) Doppler tissue imaging of the RV free wall and (2) myocardial performance index. The ratio of diastolic and systolic time-velocity integrals measured by Doppler of the main pulmonary artery was calculated. CMR variables included RV ejection fraction, RV volumes, and pulmonary regurgitant fraction (RF). Pulmonary regurgitation was graded as mild (RF<20%), moderate (RF=20-40%), and severe (RF>40%). On CMR, RF was 34+17% and RV ejection fraction was 61+8%. Echocardiography had good sensitivity identifying cases with RF>20% (sensitivity 97%; 95% CI: 92-99%) but overestimated the amount of PR when RF<20% (false-positive rate 36%; 95% CI: 18-57%). The diastolic and systolic time-velocity integrals on echocardiogram showed moderate correlation with RF on CMR (R=0.60; P<0.0001). On CMR, RF of 20% and 40% corresponded with a diastolic and systolic time-velocity integral of 0.49 (95% CI: 0.44-0.56) and 0.72 (95% CI: 0.68-0.76), respectively. RV myocardial performance index correlated modestly with RV ejection fraction (r=-0.33; P<0.001)., Conclusions: This study suggests that the diastolic and systolic time-velocity integrals ratio may make a modest contribution to the overall assessment of PR in patients with repaired tetralogy of Fallot and warrants further investigation. However, echocardiography continues to have a limited ability to quantify PR and RV function as compared with CMR.

To explore the detection rate, types, accompanying abnormalities and prognosis of fetal congenital heart disease (CHD) in a single centre of northwest China, in order to lay a foundation for further studying the epidemiological characteristics of fetal CHD and integrated management of FCHD in the prenatal and postnatal. The number of abortion cases, intrauterine and postnatal natural deaths of fetal CHD accounted for 60.3% (1357/2251) of the total follow-up, of which complex CHD and chromosomal abnormalities accounting for the majority. The fetal complex CHD were more common while the prognosis were worse than simple CHD, but some types of complex CHD could be corrected by surgery by pregnancy monitoring and evaluation. [Extracted from the article]

To explode ultrasonographic pregnatal diagnosing value through retrospective study on absent pulmonary valve syndrome (APVS). 66558 examinations cases within 10 years from our centre were retrospectively studied by fetal echocardiography (FECG) include two dimension and Doppler imaging. APVS is typical shown by FECG include 2D and spectral Doppler and colour Doppler imaging, but isolate absent of pulmonary valve should be examed carefully to determine and exclude the other intracardiac malformations. [Extracted from the article]