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Aortopulmonary collaterals (APCs) develop universally, but to varying degrees, in patients with single ventricle congenital heart disease (CHD). Despite their ubiquitous presence, APCs remain poorly understood. We sought to evaluate the association between APC burden and common non-invasive clinical variables. We conducted a single center, retrospective study of patients with single ventricle CHD and previous Glenn palliation who underwent pre-Fontan cardiac magnetic resonance (CMR) imaging from 3/2018 to 3/2021. CMR was used to quantify APC flow, which was normalized to aortic (APC/Q

We sought to characterize the clinical course and outcomes of intervention for Tetralogy of Fallot (TOF) with associated conal septal hypoplasia (CSH) compared to those with identifiable conal septum on initial newborn echocardiogram. We performed a retrospective, 1:2 case-control study of children with TOF anatomy, 33 with CSH and 66 with typical TOF, who underwent surgical repair from 1991-2019 at Children's Wisconsin. Data on echocardiographic anatomic features, systemic oxygen saturations, medical therapies, admissions, palliative interventions, operative strategies, and long-term follow-up were compared. The CSH group had fewer hypercyanotic spells (6% vs 42%, p 0.001), beta-blockers prescribed (12% vs 41%, p = 0.005), and hospital admissions for cyanosis (12% vs 44%; p = 0.001) prior to any intervention. Of 14 who required palliative intervention, 8 had balloon pulmonary valvuloplasty (BPV) (7 from the CSH group and 1 from the control group), and 6 had systemic-to-pulmonary artery shunts (all from the control group). Definitive repair was performed at a significantly older age in the CSH group (10.2 ± 10 vs 5.6 ± 5.9 months, p = 0.011), with less subpulmonary muscle resection (57.6% in vs 92.4%, p 0.001) and higher use of a transannular patch (84.8% vs 65.2%, p = 0.040). The average time to surgical reintervention was similar in both groups (9.7 ± 5.9 vs 8.6 ± 6.4 years in controls). We conclude that infants with TOF and CSH have a milder preoperative clinical course with fewer hypercyanotic spells or need for medical therapy. They also respond well to palliative BPV and can safely undergo later definitive repair compared to typical TOF with a well-developed conal septum.

It is unclear if neonatal tetralogy of Fallot repair offers better outcomes compared to repair later in infancy. We therefore conducted a meta-analysis comparing outcomes of neonatal and non-neonatal repair. Manuscripts were identified and reviewed for quality and bias with favorably scored manuscripts being included in the final meta-analysis. Several perioperative and postoperative variables were compared. A total of 8 studies with 3858 patients were included in the analysis. Of these patients, 19% underwent neonatal repair. Neonatal repair was associated with increased mortality, longer intensive care unit stays, and longer total hospital length of stay.

We present our experience for patients who have undergone an anatomic repair (AR) for congenitally corrected transposition of the great arteries (CCTGA) at the Children’s Hospital of Wisconsin. A retrospective chart review of patients who underwent AR for CCTGA from 2001 to 2015 was performed. The cohort consisted of 15 patients (74% male). Median age of anatomic repair was 15 months (range 4.5–45.6 months). Four patients had a bidirectional Glenn (BDG) prior to AR. At the time of AR,—9 (60%) underwent Senning/Rastelli procedure, 4 (26.6%) had double switch operation, and 2 (13.3%) underwent only Senning with VSD closure. Median duration of follow-up was 5.5 years (0.05–14 years). Reoperations prior to discharge included BDG, revision of pulmonary venous baffle, closure of residual VSD, and pacemaker placement. Late reoperations included left ventricular outflow tract obstruction repair, conduit replacement, melody valve placement, and pacemaker implantation. At their most recent follow-up, no patient had heart failure symptoms and only 1 had severely diminished function that improved with cardiac resynchronization therapy. Moderate mitral regurgitation was noted in 15% (2/13), and severe in 7% (1/13). Moderate tricuspid regurgitation was noted in 15% (2/13). One patient, 7% (1/13), developed moderate aortic insufficiency. There was a 100% survival at the time of the most recent follow-up. Patients with CCTGA who have undergone AR have excellent functional status and mid-term survival but reinterventions are common. Longer term studies are needed to determine both the extent and spectrum of reinterventions as well as long term survival.

Cor triatriatum dexter is a rare congenital heart defect that can lead to cyanosis in a newborn with an otherwise normal exam. The initial evaluation of these patients typically focuses on searching for a pulmonary etiology for arterial desaturation, which often leads to a negative work up. When cardiac evaluation is performed, it may be challenging because the heart lesion can be difficult to visualize on an echocardiogram. The diagnosis requires a high index of suspicion and thorough echocardiographic imaging. Once diagnosed, surgical repair can alleviate the shunt created by the defect. This case series describes all patients (3) with cor triatriatum dexter seen at Children's Hospital of Wisconsin from 2000 to 2013.