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Abnormal origin of left coronary artery from the pulmonary artery (ALCAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. This study aimed to determine the correlation between age at clinical presentation, level of ventricular dysfunction, and post-repair outcomes. This is retrospective study from 1993 to 2018 including thirty-one patients. The study cohort was divided into two groups according to age ( 6 months). The significance level was set at p 0.05.The median follow-up time was 72 [24–168] months. Median age was 4.7 [2.3–16] months. Median weight was 6.2 [4.3–9] kg. There was severe left ventricular (LV) dysfunction (ejection fraction

Aim Transposition of the great arteries (TGA) associated with ventricular septal defect (VSD) could present an intracardiac anatomy making the neonatal repair very challenging: multiple/apical VSD, straddling, tortuous intraventricular tunnel repair. To delay the repair, the isolated pulmonary artery banding (PAB) has the disadvantage to endanger the future aortic valve and to create a double obstacle in the frequent association with aortic annulus and arch hypoplasia. We propose a 2-step strategy with an initial palliative arterial switch operation (ASO) associated with PAB (± aortic arch repair) and, later, an intracardiac repair with debanding. The Aim of this retrospective study is to compare this strategy to a classic isolated PAB. Methods Among the 715 neonatal TGA admitted in our institution between 2007 and 2018, 10 complex TGA benefited either from a palliative ASO with PAB (group A, n = 5) or a PAB (group B, n = 5). Results At palliation, the duration of inotropic support and the length of stay in intensive care unit were significantly shorter in group A (respectively 4.75 ± 1.7 and 5.5 [4.25–6.75] days versus 13 ± 6.9 and 16 [10.5–30] days in group B; P = 0,029 and P = 0,008). At complete repair, age and weight were significantly higher in group A (P = 0,018) and the aortic cross-clamping and bypass times were shorter (P = 0,018). No patient required a delayed sternal closure in group A. Mean length of stay in intensive care unit and hospital duration were respectively of 2 ± 1 days and 5.6 ± 1.53 days in group A versus 21.4 ± 16.9 days and 24.5 ± 19.9 days in the group B (P = 0.018 and P = 0.029), showing reduced morbidity. Conclusion Differing the neonatal repair for TGA is exceptional. When we should consider it due to particular intracardiac anatomy, it seems legitimate to practice a palliative ASO rather than the classic isolated PAB.

Objective Abnormal origin of coronary artery from the pulmonary artery (ACAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. Immediate surgical correction can provide excellent results. This study aimed to determine the surgical outcome of ACAPA. Methods From 1993 to 2016, 31 consecutive patients underwent coronary re-implantation. Concomitant mitral valvuloplasty was performed in two. The study cohort was divided into two groups according to age (group 1: ≤ 6 months and group 2: > 6 months). Data from demographic characteristics, electrocardiography, echocardiography, surgery, intensive care unit stay and follow-up were evaluated. Results The study included 29 patients with anomalous left coronary artery from the pulmonary artery and 2 with anomalous right coronary artery from the pulmonary artery. The median age at repair was 4.75 [2.3–16.3] months and the median weight was 6.2 [4.3–9.4] kg. Preoperative echocardiography showed severe LV dysfunction in 56.7% of patients. The median preoperative LVEF was 33.5% [20–55]. Mitral regurgitation (MR) was moderate to severe in 13 patients (41.9%). Requirement to extracorporeal membrane oxygenation (ECMO) support systems was necessary in 2 cases (6.4%) before surgery and 5 cases (16.1%) after correction. Postoperative mortality rate was 9.7%. Younger age ( Conclusions Young age is significantly associated with a more severe presentation, more ECMO requirement and mortality. Lesser development of coronary collateral circulation can explain these findings. After establishment of a two-coronary circulation both ventricular function and MR tend to be normalized over time. This ‘recovery’ usually occurs within 6 months but may require more time (maximum of thirty months in our population).

Objective: To report our experience with perventricular closure of muscular (apical) ventricular septal defects (VSDs) in small infants, with echocardiographic guidance only, in a nonhybrid suite. Methods: Eight infants with nine large muscular (apical) VSDs underwent perventricular device closure in a nonhybrid operating room, with transesophageal and epicardial echocardiography guidance, at a mean age and weight of 3.07 (0.3-7.28) months and 3.7 (2.5-6.2) kg, respectively. Five patients had multiple VSDs. Four had associated cardiac defects. Results: Nine Amplatzer muscular VSD devices with a mean size of 10 (4-14) mm were deployed. Seven patients were discharged from the intensive care unit with a mean length of stay of 8.6 days. Four patients had minimal postprocedural residual shunt; no one had a residual shunt at six-month follow-up. Mid-term results are excellent. Conclusion: Perventricular closure is feasible under echocardiographic guidance only in small patients, even without hybrid suite. This may be a good approach for very symptomatic low-weight infants with apical VSD and may also be useful, in any center, at any time, and in any operating room, to treat an associated apical VSD, even unexpected.

A three-year-old boy was referred for persistent arterial duct. Transthoracic echocardiography showed a right aortic arch and an unusual Doppler flow in the arch vessels and the pulmonary artery. The tomodensitometry showed a right-sided aortic arch, with successive origin of the right common carotid, the right subclavian artery, and an aberrant (lusoria) left subclavian artery. The left common carotid took origin from the pulmonary trunk. During surgery, a fibrous cord independent from the anomaly was identified. An end-to-side anastomosis between the left carotid and the ascending aorta was done and the fibrous cord was divided. Was this fibrous cord a ductal ligament?