Long-Term Outcomes Following the Ross Procedure in Neonates and Infants: A Multi-Institutional Analysis.

OBJECTIVES: For neonates and infants with aortic valve pathology, the Ross procedure has historically been associated with high rates of morbidity and mortality. Data regarding long-term durability are lacking. METHODS: The international, multi-institutional Ross Collaborative included six tertiary-care centers. Infants who received a Ross operation between 1996-2016 (allowing a minimum five years of follow-up) were retrospectively identified. Serial echocardiograms were examined to study evolution in neoaortic size and function. RESULTS: Primary diagnoses for the 133 patients (n=30 neonates) included isolated aortic stenosis (AS; 14%, n=19), Shone complex (14%, n=19), and AS+other (excluding Shone complex; n=95, 71%) including arch obstruction (n=55), left ventricular hypoplasia (n=9), and mitral disease (>moderate stenosis or regurgitation, n=31). At the time of Ross, median age was 96 (IQR 36-186) days and median weight was 4.4 (3.6-6.5) kg. In-hospital mortality occurred in 13/133 (10%) patients (4/30 [13%] neonates). Post-discharge mortality occurred in 10/120 (8%) patients at a median 298 days post-Ross. Post-Ross neoaortic dilatation occurred, peaking at 4-5 standard deviations above normal at 2-3 years before returning to near-baseline z-score at a median follow-up of 11.5 [6.4-17.4] years. Autograft/LVOT reintervention was required in 5/120 (4%) patients at a median 10.3 [4.1-12.8] years. Freedom from >moderate neoaortic regurgitation (AR) was 86% at 15 years. CONCLUSIONS: Neonates and infants experience excellent post-discharge survival and long-term freedom from autograft reintervention and AR following Ross. Neoaortic dilatation normalizes in this population in the long-term. Increased consideration should be given to Ross in neonates and infants with aortic valve disease.

Iron deficiency and anemia in pediatric dilated cardiomyopathy are associated with clinical, biochemical, and hematological markers of severe disease and adverse outcomes.

BACKGROUND: There is limited evidence regarding the prevalence and impact of iron deficiency (ID) in children with dilated cardiomyopathy (DCM). METHODS: Retrospective single-center review of all children between 2010 and 2020 with a diagnosis of DCM and complete iron studies. ID was defined as ≥2 of ferritin <20 µg/liter, iron <9 µmol/liter, transferrin >3 g/liter, or transferrin saturation (TSat) <15%. Clinical and laboratory indices and freedom from a composite adverse event (CAE) of mechanical circulatory support (MCS), heart transplant, or death were compared between children with and without ID. RESULTS: Of 138 patients with DCM, 47 had available iron studies. Twenty-nine (62%) were iron deficient. Children with ID were more likely to be receiving inotropes (17, 59%, p = 0.005) or invasive/noninvasive ventilation (13, 45%, p = 0.016) than those who were iron replete. They had a higher incidence of anemia (22, 76%, p = 0.004) and higher NT-proBNP (1,590 pmol/liter, IQR 456-3,447, p = 0.001). Children with ID had significantly less freedom from the CAE at 1-year (54% ± 10%), 2-years (45 ± 10), and 5-years (37% ± 11%) than those without (p = 0.011). ID and anemia were the only significant predictors of the CAE on univariate Cox regression. CONCLUSIONS: ID is highly prevalent in children with DCM. Iron studies are undermeasured in clinical practice, but ID is associated with severe heart failure (HF) and an increased risk of the CAE. The need for iron replacement therapy should be considered in children who present in HF with DCM.

An Opportunity to See the Heart Defect Physically: Medical Student Experiences of Technology-Enhanced Learning with 3D Printed Models of Congenital Heart Disease.

Three-dimensional (3D) printing is increasingly used in medical education and paediatric cardiology. A technology-enhanced learning (TEL) module was designed to accompany 3D printed models of congenital heart disease (CHD) to aid in the teaching of medical students. There are few studies evaluating the attitudes and perceptions of medical students regarding their experience of learning about CHD using 3D printing. This study aimed to explore senior medical students' experiences in learning about paediatric cardiology through a workshop involving 3D printed models of CHD supported by TEL in the form of online case-based learning. A mixed-methods evaluation was undertaken involving a post-workshop questionnaire (n = 94 students), and focus groups (n = 16 students). Focus group and free-text questionnaire responses underwent thematic analysis. Questionnaire responses demonstrated widespread user satisfaction; 91 (97%) students agreed that the workshop was a valuable experience. The highest-level satisfaction was for the physical 3D printed models, the clinical case-based learning, and opportunity for peer collaboration. Thematic analysis identified five key themes: a variable experience of prior learning, interplay between physical and online models, flexible and novel workshop structure, workshop supported the learning outcomes, and future opportunities for learning using 3D printing. A key novel finding was that students indicated the module increased their confidence to teach others about CHD and recommended expansion to other parts of the curriculum. 3D printed models of CHD are a valuable learning resource and contribute to the richness and enjoyment of medical student learning, with widespread satisfaction. Supplementary Information: The online version contains supplementary material available at 10.1007/s40670-023-01840-w.

Right Ventricular Outflow Tract Stenting is a Safe and Effective Bridge to Definitive Repair in Symptomatic Infants With Tetralogy of Fallot1.

INTRODUCTION: Right ventricular outflow tract (RVOT) stent angioplasty is a palliative procedure for neonates and infants with symptomatic tetralogy of Fallot prior to surgical repair. We review our institutional outcomes of RVOT stenting. METHODS: Retrospective review of all infants with tetralogy of Fallot under 3 months of age who underwent primary native RVOT stent angioplasty at The Children's Hospital at Westmead, Sydney, Australia between January 2010 and December 2020. Demographics and echocardiographic pulmonary artery dimensions were collected pre-stent angioplasty and prior to surgical repair. RESULTS: Twenty (20) infants underwent primary RVOT stenting. Median age at stent was 14 days (interquartile range [IQR] 7-32) and median weight 2.7 kg (IQR 2.1-3.4). Three patients underwent hybrid per-ventricular procedures. Indication for RVOT stenting was recurrent hyper-cyanotic spells in 12 (60%) and duct-dependent pulmonary blood flow in 8 (40%). Saturations increased from a median of 80% (IQR 75-85) to 91% (IQR 90-95) post procedure (P<0.001). A single major complication occurred: transient complete atrioventricular dissociation requiring isoprenaline infusion for <24 hours. Twelve (12, 60%) required catheter re-intervention prior to definitive repair for further augmentation of pulmonary blood flow. There were two non-cardiac deaths distant from the stent procedure, but prior to surgical repair. Median right and left pulmonary artery Z-scores increased respectively from -2.06 (IQR -2.99 to -0.17) and -1.2 (IQR -2.59 to -0.14) prior to RVOT stent, to -0.74 (IQR [-1.21 to 0.26], P=0.01) and 0.06 (IQR [-1.87 to 1.15], P=0.006) by the time of definitive repair. Eighteen (18) patients achieved definitive repair at a median age of 6.1 months (IQR 4.7-7.3). Palliation with more than one RVOT stent was associated with an increased duration of cardiac bypass (P=0.035) and cross-clamp (P=0.044) time at definitive repair. CONCLUSIONS: In symptomatic neonates and infants with tetralogy of Fallot at high-risk of peri-operative complications, RVOT stent angioplasty can safely and effectively augment pulmonary blood flow prior to definitive repair.

The Ross/Ross-Konno procedure in infancy is a safe and durable solution for aortic stenosis.

OBJECTIVE: The objective of this study was to characterize early and midterm outcomes after the Ross/Ross-Konno procedure performed in infancy for severe aortic valve disease. METHODS: Between January 1995 and December 2018, 35 infants younger than 1 year (13 neonates) underwent a Ross/Ross-Konno procedure. Patients were followed up to a median of 4.1 years (interquartile range [IQR], 2.6-9.5). Primary outcome measures were survival, early morbidity, freedom from reintervention and long-term functional and echocardiographic status. RESULTS: Median age at operation was 49 days (IQR, 17-135) and weight was 4 kg (IQR, 3.4-5.2). Thirty-one (89%) had undergone a previous procedure, including balloon valvuloplasty in 26 (74%). Thirty (86%) required annular enlargement (Konno incision). Five required concomitant aortic arch surgery (2 neonates, 3 infants). There were no early deaths, and 1 late death at 18 months. Freedom from reoperation was 85% (95% confidence interval [CI], 68%-93%) at 1 year, 76% (95% CI, 54%-88%) at 5 years, and 62% (95% CI, 36%-79%) at 10 years. One modified Konno was performed at 5 years after a Ross in infancy. Ten right ventricle to pulmonary artery conduits have required reintervention (2 percutaneous pulmonary valve implantations). One child required a permanent pacemaker for complete heart block. At latest follow-up, 32 (94%) of 34 survivors were asymptomatic. There was no significant change in neoaortic Z-scores between 6 weeks and latest follow-up. CONCLUSIONS: The neonatal and infant Ross/Ross-Konno procedure can be performed with low mortality and achieves a stable left ventricular outflow tract. Significant early morbidity reflects the preoperative condition of the patients but definitive surgery of this type can be considered as a primary approach.

Neonatal Ebstein Anomaly: A 30-year Institutional Review.

This study aimed to review our 30-year, single-center experience of neonates admitted with Ebstein anomaly. Between January 1985 and August 2015, 80 neonates with Ebstein anomaly were managed. The primary outcome measures were early and late survival, freedom from reoperation, and functional status. Pulmonary atresia or critical stenosis occurred in 18 neonates. Twenty-seven (34%) patients required intervention: 13 systemic-to-pulmonary shunts, 5 balloon pulmonary valvotomy, 3 relief of right ventricular outflow tract obstruction, 3 Starnes procedures, and 3 other procedures. Sixty-nine (86%) of the neonates survived to hospital discharge. Overall 15-year survival estimate was 67% (SE = 6.5), with a superior prognosis for those able to be managed medically (15-year survival of 79%, SE = 7.0) compared with those in whom surgical or catheter intervention was undertaken (15-year survival of 45%, SE = 11.2, P = 0.005). For early survivors of neonatal surgery, freedom from reoperation at 10 years was 16% (SE = 8.5). For long-term survivors, 96% were classified as New York Heart Association Class I or II. Neonates with Ebstein anomaly who can be managed without intervention have a good prognosis. Substantial mortality risk remains in those who require intervention, especially those complicated by pulmonary atresia.