Predictors of post-operative left atrioventricular valve regurgitation in pediatric patients with complete atrioventricular canal defects.

BACKGROUND: In infants with complete atrioventricular canal (CAVC) defects, post-operative left atrioventricular valve regurgitation (LAVVR) is a known major cause of morbidity and mortality and a common indication for re-operation. However, there is scarce data to identify risk factors for poor outcomes. Our study aims to find echocardiographic characteristics that predict post-operative LAVVR at discharge and 1-year follow-up. METHODS: Retrospective cohort study of patients with initial CAVC repair at our hospital who were followed for 1 year between 2013 and 2022. Patients with major co-morbid conditions were excluded. Serial echocardiograms were reviewed. Anatomic details, quantitative and qualitative measure of LAVVR including the number of regurgitant jets, regurgitant jet length and vena contracta width, and ventricular function were collected. The time points measured include pre-operative transthoracic echocardiogram (TTE), post-operative transesophageal echocardiogram (PO-TEE), routine protocol based post-operative day 1 (POD1) TTE, discharge TTE and 1-year post-operative (1yPO) TTE. Paired t-tests, chi-square analysis, and linear regression analysis were performed comparing measured variables to LAVVR outcomes. RESULTS: Fifty-two patients were included; 92% had Trisomy 21. The majority were classified as Rastelli A (71%), others Rastelli C (29%). Only two patients had moderate or greater LAVVR pre-operatively. The mean age at repair was 125 ± 44 days. Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year after backward stepwise regression. Of those with < moderate LAVVR on PO-TEE, 20% had worsening to ≥ moderate at discharge, but only 9% remained that way at 1 year. Of those with ≥ moderate LAVVR on PO-TEE, 40% improved to < moderate by 1 year. Two patients who worsened at 1 year, both secondary to likely cleft suture dehiscence. Only one patient required reoperation in the immediate post-operative period secondary to severe LAVVR due to suture dehiscence. Routine protocol-based POD1 echo did not have any association with altered outcomes. CONCLUSION: Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year. A significant percentage (40%) of patient with ≥ moderate LAVVR on PO-TEE improved to < moderate by 1 year. Furthermore, routine protocol-based POD1 echo did not have any association with altered outcomes.

Enhanced congenital cardiac surgery knowledge with multidisciplinary collaborative education series for paediatric cardiology fellows and nurse practitioners.

INTRODUCTION: Paediatric cardiologists and nurse practitioners lack structured education tools focused on basic cardiac surgery principles. However, non-surgical specialties caring for surgical patients require this knowledge for comprehensive clinical care. We created a cardiac surgical educational curriculum focused on improving knowledge and attitudes towards communication for non-surgical trainees and advanced practice providers. METHODS: Over one academic year, six paediatric cardiology fellows and seven paediatric cardiac surgery nurse practitioners at Seattle Children's Hospital participated in this study. With surgical supervision, six lectures were prepared by each fellow and delivered monthly. Sessions were hybrid and recorded for later viewing. Pre- and post-intervention survey of attitudes regarding surgical topics and pre- and post- test-based knowledge assessments were administered. RESULTS: Participants positively rated the usefulness of the lecture series (4.2/5) and would recommend it to a colleague (4.5/5). Self-reported confidence discussing surgical concepts with patients increased from 2.3 to 3.4 among paediatric cardiology fellows (p < 0.001) and from 2.8 to 3.9 among nurse practitioners (p < 0.001), out of 5. In both groups, knowledge assessment scores improved from 54 to 79% post-intervention (p < 0.001). CONCLUSIONS: After a six-part educational series taught by paediatric cardiology fellows, both paediatric cardiology fellows and paediatric cardiac surgery nurse practitioners demonstrated improved knowledge and reported increased comfort counselling families on basic cardiac surgery topics. Structured, active-learning lessons taught by fellows for non-surgical audiences can improve attitudes and build clinically relevant knowledge. Creating an effective level-appropriate multidisciplinary curriculum accessible to various types of medical providers could enhance comprehensive care of complex congenital cardiac surgery patients.

Living with Congenital Aortic Stenosis: Exercise Restriction, Patterns of Adherence, and Quality of Life.

Modern consensus panel guidelines recommend restriction from most organized sports for patients with moderate or severe aortic stenosis (AS). However, there is little published data on how frequently physicians deviate from guidelines, how well patients adhere to exercise restrictions, or the effect of restriction on patient-reported quality of life. In this study, we surveyed 93 subjects with AS and their cardiologists regarding participation in organized sports, physical activity, weightlifting, and exercise restriction. Subjects completed the pediatric quality of life inventory (PedsQL) and the pediatric cardiac quality of life inventory (PCQLI). We found that subjects with severe AS (n = 3) were commonly, but not universally, restricted from organized sports (n = 2, 66%). Subjects with moderate AS (n = 40) were rarely restricted from organized sports (n = 6, 17%). No physician-specific characteristics were associated with increased likelihood of recommending exercise restriction. Subjects were more likely to be restricted if they were older (16 years vs. 13 years, p 0.02) and had moderate versus mild AS (p 0.013). PCQLI scores for teens and young adults with AS (age 13-25) were lower than a comparison group of patients with mild congenital heart disease. For all age groups, the PedsQL social functioning score was lower for subjects with exercise restriction (p 0.052). In summary, cardiologists apply consensus guidelines leniently when restricting patients with moderate/severe AS from organized sports and weightlifting. Patients with AS routinely adhere to exercise restriction recommendations. Children and young adults with AS and exercise restriction have lower QOL scores in the social functioning domain.

Pulmonary Vein Stenosis Associated with Germline PIK3CA Mutation.

Pulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include surgery, interventional cardiac catheterization, and more recently, medications targeting cell proliferation, which are not uniformly effective. We present a patient with PVS and a PIK3CA mutation, who demonstrated a good response to the targeted inhibitor, alpelisib.

Validation of Prenatal Aortic Arch Angle Measurements in the Diagnosis of Neonatal Coarctation of the Aorta.

Prenatal prediction of coarctation of the aorta (CoA) is challenging. Methods identifying prenatal CoA have high sensitivity with significant false positives. We previously derived prenatal aortic arch angles for identifying CoA with high sensitivity and specificity and aim to validate these angles and compare them with a model utilizing ascending aorta (AAo) and isthmus (Aoi) measures. Retrospective case/cohort study of fetuses with prenatal suspicion for CoA. 35 fetuses were included. Measurements included: ascending-descending aortic angle (AAo.DAo), transverse-descending aortic angle (TAo.DAo); diameters and z-scores of Aoi from sagittal (Aoi-sag), three-vessel (Aoi-3VV) view and AAo. Discriminant functions for the 5 variables were compared using histograms and positive/negative predictive values (PPV/NPV). CoA was confirmed in 28/35 neonates. The PPV and NPV for angle measures were 100% and 77%. The AAo + Aoi-3VV model PPV and NPV were 92% and 80% and Aoi-sag + Aoi-3VV model were 82% and 71%. A linear discriminant model utilizing the 3 most predictive variables improved NPV to 90% and PPV to 100%. In conclusion, we validate that angle measures are superior to standard models of predicting CoA. An optimized 3 variable model maintains accuracy of identifying CoA while eliminating false positives.

Spatiotemporal expression of activin receptor-like kinase-5 and bone morphogenetic protein receptor type II in the ovary of shortfinned eel, Anguilla australis.

In the eel ovary, the expression of growth differentiation factor-9 (Gdf9) appears to be largely confined to the germ cell in early stages of oogenesis. However, both the target tissue and the function of Gdf9 in fish remain unknown. This study aimed to describe the abundance and localization of activin receptor-like kinase-5 (Alk5) and bone morphogenetic protein receptor type II (Bmpr2), which together mediate the Gdf9 signal, in the ovary of a basal teleost, the shortfinned eel, Anguilla australis, during early folliculogenesis. The cDNA encoding eel alk5 and bmpr2 genes were cloned, characterized and the transcript abundances of these receptors quantified by quantitative real-time PCR. Ovarian transcript abundance for both receptors, along with that of gdf9 and of its paralogue bmp15, increased from the previtellogenic to early vitellogenic stage. Localization of receptor mRNAs by in situ hybridization revealed that these receptors are located in the somatic cells surrounding the oocyte. Furthermore, tissue distribution analysis showed that the expression of alk5 and bmpr2 were highest in ovary and thyroid, respectively. Unexpectedly, however, bmpr2 mRNA levels were lower in the ovary than in any of the other 17 tissues examined, and indeed, lower than ovarian gdf9 transcript abundance. These findings, together with the ovarian expression pattern of Gdf9, suggest that Gdf9, and conceivably, Bmp15, from the oocyte can signal through receptors that are located on the somatic cells surrounding the oocyte; this, in turn, facilitates elucidation of the function of these growth factors during oogenesis in teleost fish.