ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49 days (range: 4 days, 12.8 years). Cardiac lesions associated with ALCAPA were HLHS (n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSD with right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF (n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.

Long-term outcomes of tricuspid valve intervention during stage 2 palliation in patients with a single right ventricle.

OBJECTIVES: In patients with single ventricle physiology and a systemic right ventricle, tricuspid valve regurgitation increases the risk of adverse outcomes, and tricuspid valve intervention at the time of staged palliation further increases that risk in the postoperative period. However, long-term outcomes of valve intervention in patients with significant regurgitation during stage 2 palliation have not been established. The purpose of this study is to evaluate the long-term outcomes after tricuspid valve intervention during stage 2 palliation in patients with right ventricular dominant circulation in a multicenter study. METHODS: The study was performed using the Single Ventricle Reconstruction Trial and Single Ventricle Reconstruction Follow-up 2 Trial datasets. Survival analysis was performed to describe the association among valve regurgitation, intervention, and long-term survival. Cox proportional hazards modeling was used to estimate the longitudinal association of tricuspid intervention and transplant-free survival. RESULTS: Patients with tricuspid regurgitation at stage 1 or 2 had worse transplant-free survival (hazard ratio, 1.61; 95% confidence interval, 1.12-2.32; hazard ratio, 2.3; 95% confidence interval 1.39-3.82). Those with regurgitation who underwent concomitant valve intervention at stage 2 were significantly more likely to die or undergo heart transplantation compared with those with regurgitation who did not (hazard ratio, 2.93; confidence interval, 2.16-3.99). Patients with tricuspid regurgitation at the time of the Fontan had favorable outcomes regardless of valve intervention. CONCLUSIONS: The risks associated with tricuspid regurgitation in patients with single ventricle physiology do not appear to be mitigated by valve intervention at the time of stage 2 palliation. Patients who underwent valve intervention for tricuspid regurgitation at stage 2 had significantly worse survival compared with patients with tricuspid regurgitation who did not.

Application of 3D Printing Technology in Heart Failure.

Advanced imaging is often used to supplement clinical information in guiding management for patients with heart failure. 3 dimensional (3D) imaging datasets allow for a better understanding of the relevant cardiac spatial anatomic relationships. 3D printing technology takes this one step further and allows for the creation of patient-specific physical cardiac models. In this review, we summarize some of the recent innovative applications of this technique to patients with heart failure from different etiologies, to provide more patient-directed care.

Assessment of Anomalous Coronary Arteries by Imagers and Surgeons: Comparison of Imaging Modalities.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is associated with sudden cardiac death. High-risk characteristics are most commonly assessed using a 2-dimensional (2D) echocardiogram (echo) or cardiac computed tomography (CT). We hypothesize that these characteristics will be more accurately assessed when they are presented in the form of a 3D digital model. METHODS: Fourteen participants, including cardiothoracic surgeons and cardiac imaging specialists, assessed image representations, including echo, CT images, and a 3D digital model, from 6 patients who had undergone AAOCA repair. Accuracy of assessment was evaluated by comparing responses with operative findings (the gold standard). RESULTS: The reported type of AAOCA was most accurately assessed on CT (100%) and 3D models (92.31%) compared with echo (80.77%). The accuracy of the AAOCA course was highest on CT (91.03%), followed by the 3D model (80.77%), and lowest on echo (61.54%). The accuracy of intramurality was low across all imaging modalities (17.95% echo, 29.49% CT, and 21.79% 3D model). Accurate assessment of a separate AAOCA ostium was highest on 3D models (97.40%). Ostial stenosis was more accurately assessed on 3D models (56.41%). When accuracy was separated by subspecialty, CT and 3D models were more accurately assessed by all participants regardless of training. CONCLUSIONS: Cardiac imagers and congenital cardiothoracic surgeons most accurately assessed AAOCA presence, type, and course on cardiac CT and 3D models. 3D models were superior in representation of ostial characteristics. CT and 3D models are overall more accurately assessed by specialists regardless of training.

Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study.

We sought to describe the clinical course and outcomes of patients who are diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) after infancy. We conducted a retrospective evaluation of patients who underwent ALCAPA surgery between January 2009 to March 2018 at 21 US centers. Clinical presentation, inpatient management, and postoperative outcomes of patients repaired ≥1 year of age were described. To characterize this cohort, we compared these data to patients repaired before 1 year of age. Of 248 ALCAPA patients, 71 (29%) underwent repair ≥1 year of age. Among this subset, the median age at diagnosis was 8.3 years. Chronic arrhythmia occurred in 7%. Patients had good postoperative recovery of left ventricle (LV) dysfunction (90%) and LV dilation (75%), although a low incidence of recovery of mitral regurgitation (40%). Compared to infants, older patients were more likely to present with cardiac arrest (11% vs 1%) and less likely to have moderate or worse LV dysfunction or mitral regurgitation. Older patients had significantly less postoperative extracorporeal membrane oxygenation use, and shorter ICU and hospital stay. In the older cohort, operative mortality occurred in only 1 patient and no patient died after discharge (median follow-up 2.7 years). Survival of patients who presented with ALCAPA beyond infancy was excellent, although chronic mitral regurgitation and chronic arrhythmia were not uncommon. Patients who underwent ALCAPA repair ≥1 year of age were less likely to present with LV dysfunction but more likely to present with cardiac arrest than younger patients.

Utility of 3D Printed Cardiac Models for Medical Student Education in Congenital Heart Disease: Across a Spectrum of Disease Severity.

The most common modes of medical education for congenital heart disease (CHD) rely heavily on 2-dimensional imaging. Three-dimensional (3D) printing technology allows for the creation of physical cardiac models that can be used for teaching trainees. 3D printed cardiac models were created for the following lesions: pulmonic stenosis, atrial septal defect, tetralogy of Fallot, d-transposition of the great arteries, coarctation of the aorta, and hypoplastic left heart syndrome. Medical students participated in a workshop consisting of different teaching stations. At the 3D printed station, students completed a pre- and post-intervention survey assessing their knowledge of each cardiac lesion on a Likert scale. Students were asked to rank the educational benefit of each modality. Linear regression was utilized to assess the correlation of the mean increase in knowledge with increasing complexity of CHD based on the Aristotle Basic Complexity Level. 45 medical students attended the CHD workshop. Students' knowledge significantly improved for every lesion (p < 0.001). A strong positive correlation was found between mean increase in knowledge and increasing complexity of CHD (R2 = 0.73, p < 0.05). The 3D printed models, pathology specimens and spoken explanation were found to be the most helpful modalities. Students "strongly agreed" the 3D printed models made them more confident in explaining congenital cardiac anatomy to others (mean = 4.23, ± 0.69), and that they recommend the use of 3D models for future educational sessions (mean = 4.40, ± 0.69). 3D printed cardiac models should be included in medical student education particularly for lesions that require a complex understanding of spatial relationships.

Deficient maternal zinc intake-but not folate-is associated with lower fetal heart rate variability.

OBJECTIVE: Few studies of maternal prenatal diet and child development examine micronutrient status in relation to fetal assessment. METHODS: Twenty-four-hour dietary recall of zinc and folate and 20min of fetal heart rate were collected from 3rd trimester pregnant adolescents. RESULTS: Deficient zinc was associated with less fetal heart rate variability. Deficient folate had no associations with HRV. Neither deficient zinc nor deficient folate was related to fetal heart rate. CONCLUSIONS: These findings, from naturalistic observation, are consistent with emerging data on prenatal zinc supplementation using a randomized control design. PRACTICAL IMPLICATION: Taken together, the findings suggest that maternal prenatal zinc intake is an important and novel factor for understanding child ANS development.