{"title":"Radiculomegaly as a key clinical feature in oculo-facio-cardio-dental (OFCD) syndrome: a case report with a novel truncating variant in <i>BCOR</i> gene.","authors":"Clément Sauvestre, Marie-José Boileau, Camille Caule, Didier Griffiths, Florian Schrub, Nicolas Chassaing, Caroline Rooryck","doi":"10.1017/S104795112402660X","DOIUrl":"https://doi.org/10.1017/S104795112402660X","url":null,"abstract":"<p><p>Radiculomegaly is a rare dental anomaly characterised by the enlargement of the root canals of teeth. It is usually associated with oculo-facio-cardio-dental (OFCD) syndrome due to truncating variants in BCL-6 transcriptional corepressor (<i>BCOR</i>) (MIM*300485). We present the case of a 21-year-old female patient who was referred to genetics for a polymalformative syndrome including bilateral glaucoma and dental anomalies, especially radiculomegaly. Some others dysmorphic features were right superior lip notch, ogival palate, long philtrum, difficulty in pronation, café-au-lait spots, II-III toe bilateral syndactyly, and macrocephaly. Cone-beam CT confirmed radiculomegaly. The genetic analysis identified a heterozygous pathogenic variant NM_001123385.1:c.2093del (p.Pro698Glnfs*17) in the <i>BCOR</i> gene. After genetic diagnosis of OFCD syndrome, cardiac CT-scan revealed a large asymptomatic atrial septal defect that was subsequently surgically closed. Reviews of the literature have previously highlighted the prevalence of radiculomegaly in OFCD syndrome with a positive predictive value of 88.23% and a sensitivity of 75.94%. This case report highlights the importance of radiculomegaly as a clinical sign of OFCD syndrome, emphasising the rarity of non-syndromic radiculomegaly and the benefits of its diagnosis in clinical management, especially in cardiac screening.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2024-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142399489","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Faiz Rasool, Amna Zafer Qureshi, Asim Khan, Tehmina Kazmi, Salman Ahmad Shah
{"title":"Role of BT shunt in tetralogy of Fallot.","authors":"Faiz Rasool, Amna Zafer Qureshi, Asim Khan, Tehmina Kazmi, Salman Ahmad Shah","doi":"10.1017/S1047951124025836","DOIUrl":"https://doi.org/10.1017/S1047951124025836","url":null,"abstract":"<p><strong>Introduction: </strong>Modified Blalock-Taussig shunt (BT shunt) is a palliative operation used for cyanotic heart diseases with decreased pulmonary blood supply. The definitive management of tetralogy of Fallot (TOF) is total corrective surgery, but these patients can be palliated with BT shunt. In the modern world, the BT shunt is getting out of favour in patients with TOF. In this article, we will share our 5-year experience at our institute, which also shows a decreasing trend.</p><p><strong>Patients and methods: </strong>It is a retrospective study. Files of all the patients admitted in our department from January 2019 to December 2023 were reviewed. Age, weight, hospital stay, inotropic support duration, mechanical ventilation duration, and outcomes were studied.</p><p><strong>Results: </strong>From January 2019 to December 2023, 173 patients underwent BT shunt for TOF. The mean age was 31 months, and the mean weight was 9.3 kg. The overall mortality for BT shunt was 15% after BT shunt. Hypercyanotic spell not controlled by medical management was the most common indication for BT shunt in our setup. Most of the patients with hypercyanotic spells were also candidates for total correction but due to the emergency, BT shunt was performed.</p><p><strong>Conclusion: </strong>The role of BT shunt in patients with TOF is decreasing due to PDA/RVOT stenting, it is likely that the BT shunt in TOF will become a thing of the past in the future even in developing countries like ours.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142388310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kota Suzuki, Masaki Nii, Takehiro Tanabe, Mizuhiko Ishigaki, Keisuke Sato, Jun Yoshimoto, Sung-Hae Kim, Norie Mitsushita, Yasuhiko Tanaka
{"title":"Global longitudinal strain is a surrogate marker for time constant of isovolumic relaxation in post-Fontan operation patients with single right ventricle and preserved ejection fraction.","authors":"Kota Suzuki, Masaki Nii, Takehiro Tanabe, Mizuhiko Ishigaki, Keisuke Sato, Jun Yoshimoto, Sung-Hae Kim, Norie Mitsushita, Yasuhiko Tanaka","doi":"10.1017/S1047951124026428","DOIUrl":"https://doi.org/10.1017/S1047951124026428","url":null,"abstract":"<p><strong>Background: </strong>The time constant of isovolumic relaxation is an established index of ventricular relaxation, a major component of diastolic function, even in a single right ventricle. However, the specific echocardiographic parameters for estimating diastolic dysfunction are insufficient for a single right ventricle. This study aimed to investigate the echocardiographic indices associated with time constant of isovolumic relaxation in post-Fontan operation patients with a single right ventricle.</p><p><strong>Methods: </strong>We included 39 patients with hypoplastic left heart syndrome after Fontan operation with an ejection fraction ≥45% and preserved valve function. First, the correlation between echocardiographic parameters and time constant of isovolumic relaxation was examined, and partial correlation coefficients were calculated using age and heart rate as covariates. Next, univariate regression analysis was performed using time constant of isovolumic relaxation as the objective variable and echocardiographic parameters as independent variables, followed by multivariate regression analysis incorporating parameters with <i>p</i> < 0.10.</p><p><strong>Results: </strong>Among the echocardiographic parameters, global longitudinal strain correlated most strongly with time constant of isovolumic relaxation (<i>r</i> = 0.778, <i>p</i> < 0.001). This was consistent with the partial correlation coefficients (<i>r</i> = 0.707, <i>p</i> < 0.001). Using stepwise multivariate regression analysis, only global longitudinal strain was found to be an independent predictor of time constant of isovolumic relaxation (adjusted <i>R</i><sup>2</sup> = 0.551).</p><p><strong>Conclusions: </strong>Global longitudinal strain could be used as a surrogate marker of time constant of isovolumic relaxation, an invasive indicator of relaxation impairment, in post-Fontan operation patients with a single right ventricle, preserved ejection fraction, and valve function.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-9"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142458651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Longitudinal echocardiographic parameters for evaluation of pulmonary hypertension in preterm infants with very low birth weight.","authors":"Kwannapas Saengsin, Varangthip Khuwuthayakorn, Yupada Prongprot, Rekwan Sittiwangkul, Phichayut Phinyo, Krittai Tanasombatkul, Munranee Langu","doi":"10.1017/S1047951124026040","DOIUrl":"https://doi.org/10.1017/S1047951124026040","url":null,"abstract":"<p><strong>Background: </strong>Echocardiography is essential for the evaluation of pulmonary hypertension. We determined the feasible quantitative parameter for screening and monitoring pulmonary hypertension in preterm infants.</p><p><strong>Methods: </strong>This secondary analysis of a prospective cohort single-centre study was conducted between August 2019 and September 2020. Serial echocardiography was performed 7 and 28 days after birth and at 36 weeks postmenstrual age. The data of infants who developed pulmonary hypertension at 36 weeks postmenstrual age were compared with those without pulmonary hypertension. We also modelled the parameters' trend and performed an interaction test using multi-level Gaussian regression.</p><p><strong>Results: </strong>Out of 30 infants enrolled in the study, 79 echocardiograms were analysed. Left ventricular eccentric index was obtainable in all infants, while tricuspid jet velocity was measurable in 44.1%. Left ventricular eccentric index correlated well with tricuspid regurgitation jet velocity (<i>r</i> = 0.77, <i>P</i> < 0.001). Six infants were diagnosed with newly developed or persistent pulmonary hypertension at 36 weeks postmenstrual age. Serial left ventricular eccentric index showed a significantly different increasing trend in the pulmonary hypertension group (change per day: +0.004; <i>P</i> = 0.090) from the decreasing trend among a non-pulmonary hypertension group (change per day: -0.001; <i>P</i> = 0.041) (<i>P</i> for interaction = 0.007). Right ventricular systolic function and right ventricular isovolumic systolic velocity revealed a reducing trend in the pulmonary hypertension group, which was different from the improving trend in non-pulmonary hypertension infants. Infants with low current weight, low postmenstrual age, and requiring high-flow oxygen therapy at day 28 of life trended to increase the risk of late pulmonary hypertension.</p><p><strong>Conclusion: </strong>Left ventricular eccentric index and right ventricular isovolumic systolic velocity were feasible for assessing pulmonary hypertension and should be incorporated into pulmonary hypertension evaluation. Serial left ventricular eccentric index and right ventricular isovolumic systolic velocity may help predict late pulmonary hypertension and early detection of right ventricular dysfunction.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-10"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142458656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Recurrent acute kidney injury is associated with delayed language development in infants with Congenital Heart Disease.","authors":"Chetna K Pande, Ayse Akcan-Arikan, Sonia Monteiro, Danielle Guffey, Faridis Serrano, Natasha Afonso, Kriti Puri, Barbara-Jo Achuff, Lara Shekerdemian, Lisa Noll","doi":"10.1017/S1047951124025873","DOIUrl":"https://doi.org/10.1017/S1047951124025873","url":null,"abstract":"<p><strong>Objective: </strong>Neurodevelopmental delay is common in children who undergo surgery for Congenital Heart Disease (CHD) in infancy. Cardiac surgery associated acute kidney injury (CS-AKI) occurs frequently in the paediatric cardiac Intensive care unit (ICU). Cardiac surgery associated acute kidney injury (CS-AKI) is associated with worse neurodevelopmental scores and delay in cognitive, language, and motor domains in children with CHD. No known data exist regarding the association of CS-AKI and motor and language subscales. In this study, we explored the relationship between CS-AKI and receptive and expressive language, as well as gross and fine motor delay.</p><p><strong>Methods: </strong>This was a single centre retrospective observational cohort study. Children who underwent surgery for CHD and developed recurrent CS-AKI in the first year of life who had follow-up neurodevelopmental testing using the Bayley Scale of Infant Development Version III were included. Neurodevelopmental delay subscales assessed included: receptive and expressive language, fine and motor skills.</p><p><strong>Results: </strong>The study cohort included 203 children. Recurrent CS-AKI was significantly associated with lower scores in receptive and expressive language, as well as fine and gross motor on unadjusted analyses. On adjusted analyses, recurrent CS-AKI was significantly associated with severe receptive language delay.</p><p><strong>Conclusion: </strong>The independent association of recurrent CS-AKI with severe language delay in children who undergo surgery for CHD in infancy is novel. Our findings may contribute to the understanding of language impairment in this population. Further studies are required to better understand this relationship and any potentially modifiable factors.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-5"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142458665","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Safety and feasibility of transcatheter closure of atrial septal defects in small children weighing less than 10 kg.","authors":"Thejaswi Puthiyedath, Pramod Sagar, Kothandam Sivakumar","doi":"10.1017/S1047951124025897","DOIUrl":"https://doi.org/10.1017/S1047951124025897","url":null,"abstract":"<p><strong>Background: </strong>When young patients with an oval fossa defect present early with symptoms of heart failure and pulmonary hypertension, surgical treatment is recommended in small bodyweight rather than transcatheter closure.</p><p><strong>Methods: </strong>Outcomes of device closure in consecutive symptomatic children weighing under 10 kg were compared with young children aged below 4 years but weighing above 10 kg. Transthoracic echocardiography under conscious sedation guided the procedure in all without need for balloon sizing, transesophageal echocardiogram, and intubation anaesthesia. Symptoms, anthropometry, shunt ratio, pulmonary pressures, defect and device size, percentage oversizing, device/body weight ratio, complications, and post-procedural growth spurt were compared.</p><p><strong>Results: </strong>Ninety-six patients weighing under 10 kg were compared with 160 patients weighing above 10 kg. In total, 83.3% of patients in the study group and 25% of controls were severely malnourished. The median indexed defect size was 35.2 mm/sq.m and 27.4 mm/sq.m, and the device was oversized by 8.7% and 14.2% in the study group and controls, respectively. The device/body weight ratio was 1.93 in study group and 1.4 in controls. Procedure was successful in all except one patient weighing under 10 kg who had a device embolisation. Both groups showed significant growth spurts and proportion, with severe malnutrition reduced to 42% and 11% in the two groups.</p><p><strong>Conclusions: </strong>Device closure was feasible and safe in patients under 10 kg. Transthoracic echocardiographic imaging on conscious sedation provided adequate guidance. Symptoms and growth significantly improved after intervention. Despite a larger defect size, smaller patients had comparable outcomes. In symptomatic children under 10 kg needing early closure, transcatheter intervention should not be deferred.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-8"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142458666","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Thomas Holm-Weber, Sheyanth Mohanakumar, Thora Wesenberg Helt, Lotte Borgwardt, Lise Borgwardt, Klaus Juul, Vibeke B Christensen, Vibeke E Hjortdal
{"title":"Lymphatic magnetic resonance imaging abnormalities in children with repaired tetralogy of Fallot.","authors":"Thomas Holm-Weber, Sheyanth Mohanakumar, Thora Wesenberg Helt, Lotte Borgwardt, Lise Borgwardt, Klaus Juul, Vibeke B Christensen, Vibeke E Hjortdal","doi":"10.1017/S1047951124025435","DOIUrl":"https://doi.org/10.1017/S1047951124025435","url":null,"abstract":"<p><strong>Introduction: </strong>Tetralogy of Fallot patients face an elevated risk of developing chylothorax and pleural effusions post-surgery. This patient group exhibits risk factors known to compromise the lymphatic system, such as elevated central venous pressure, pulmonary flow changes, and hypoxia. This study investigates the morphology and function of the lymphatic system in tetralogy of Fallot patients through lymphatic magnetic resonance imaging and near-infrared fluorescence imaging, respectively.</p><p><strong>Methods: </strong>Post-repair tetralogy of Fallot patients aged 6-18 years were recruited, along with age and gender-matched controls. Magnetic resonance imaging was used to assess the morphology of the thoracic lymphatic vessels and the thoracic, while near-infrared fluorescence imaging was used to assess lymphatic activity utilising lymph rate, velocity, and pressure.</p><p><strong>Results: </strong>Nine patients and 10 controls were included. Echocardiography revealed that 2/3 of the patients had moderate-severe pulmonary regurgitation, while none displayed signs of elevated central venous pressure. Magnetic resonance imaging identified three patients with type 3 (out of 4 types) lymphatic abnormalities, while controls had none. The thoracic ducts showed severe (one patient) and moderate (one patient) tortuosity. Mean thoracic duct diameters were 3.3 mm ±1.1 in patients and 3.0 mm ± 0.8 in controls (<i>p</i>-value = 0.53). Near-infrared fluorescence imaging revealed no anomalous patterns.</p><p><strong>Conclusion: </strong>Despite no presence of clinical lymphatic disease, 3/9 of the repaired tetralogy of Fallot patients exhibited lymphatic morphological abnormalities. The significance of these anomalies remains uncertain currently. Further research is needed to determine whether these lymphatic alterations in this patient cohort are a result of congenital malformations, haemodynamic shifts, or prenatal and early-life saturation levels.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-7"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142458658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ryan D Byrne, Keila N Lopez, Christopher R Broda, Stephen J Dolgner
{"title":"Outcomes in infective endocarditis among adults with CHD: a comparative national study.","authors":"Ryan D Byrne, Keila N Lopez, Christopher R Broda, Stephen J Dolgner","doi":"10.1017/S1047951124026507","DOIUrl":"https://doi.org/10.1017/S1047951124026507","url":null,"abstract":"<p><strong>Background: </strong>Given increased survival for adults with CHD, we aim to determine outcome differences of infective endocarditis compared to patients with structurally normal hearts in the general population.</p><p><strong>Methods: </strong>We conducted a retrospective cross-sectional study identifying infective endocarditis hospitalisations in patients 18 years and older from the National Inpatient Sample database between 2001 and 2016 using International Classification of Disease diagnosis and procedure codes. Weighting was used to create national annual estimates indexed to the United States population, and multivariable logistic regression analysis determined variable associations. Outcome variables were mortality and surgery. The primary predictor variable was the presence or absence of CHD.</p><p><strong>Results: </strong>We identified 1,096,858 estimated infective endocarditis hospitalisations, of which 17,729 (1.6%) were adults with CHD. A 125% increase in infective endocarditis hospitalisations occurred for adult CHD patients during the studied time period (<i>p</i> < 0.001). Adults with CHD were significantly less likely to experience mortality (5.4% vs. 9.5%, OR 0.54, CI 0.47-0.63, <i>p</i> < 0.001) and more likely to undergo in-hospital surgery (31.6% vs. 6.7%, OR 6.49, CI 6.03-6.98, <i>p</i> < 0.001) compared to the general population. CHD severity was not associated with increased mortality (<i>p</i> = 0.53). Microbiologic aetiology of infective endocarditis varied between groups (<i>p</i> < 0.001) with <i>Streptococcus</i> identified more commonly in adults with CHD compared to patients with structurally normal hearts (36.2% vs. 14.4%).</p><p><strong>Conclusions: </strong>Adults with CHD hospitalised for infective endocarditis are less likely to experience mortality and more likely to undergo surgery than the general population.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-10"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142388307","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Luis F Castillo-Aguilar, Leonardo Rivera-Rodríguez, Jorge L Cervantes-Salazar
{"title":"The hidden dangers of the coronary anomalies: an ARCAPA case report.","authors":"Luis F Castillo-Aguilar, Leonardo Rivera-Rodríguez, Jorge L Cervantes-Salazar","doi":"10.1017/S1047951124026179","DOIUrl":"https://doi.org/10.1017/S1047951124026179","url":null,"abstract":"<p><p>An anomalous origin of the right coronary artery from the pulmonary artery case report. The diagnosis was made by angiotomography. Reimplantation of the right coronary artery into the ascending aorta and reconstruction of the pulmonary artery were conducted.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-2"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142458684","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Megan E LeBlanc, Sarah Tallent, Christoph P Hornik, Michael G W Camitta, Anne C Schmelzer, Lillian Kang, Kevin D Hill
{"title":"Mobile health monitoring of children with CHDs.","authors":"Megan E LeBlanc, Sarah Tallent, Christoph P Hornik, Michael G W Camitta, Anne C Schmelzer, Lillian Kang, Kevin D Hill","doi":"10.1017/S1047951124026222","DOIUrl":"https://doi.org/10.1017/S1047951124026222","url":null,"abstract":"<p><strong>Background: </strong>Mobile health has been shown to improve quality, access, and efficiency of health care in select populations. We sought to evaluate the benefits of mobile health monitoring using the KidsHeart app in an infant CHD population.</p><p><strong>Methods: </strong>We reviewed data submitted to KidsHeart from parents of infants discharged following intervention for high-risk CHD lesions including subjects status post stage 1 single ventricle palliation, ductal stent or surgical shunt, pulmonary artery band, or right ventricular outflow tract stent. We report on the benefits of a novel mobile health red flag scoring system, mobile health growth/feed tracking, and longitudinal neurodevelopmental outcomes tracking.</p><p><strong>Results: </strong>A total of 69 CHD subjects (63% male, 41% non-white, median age 28 days [interquartile range 20, 75 days]) were included with median mobile health follow-up of 137 days (56, 190). During the analytic window, subjects submitted 5700 mobile health red flag notifications including 245 violations (mean [standard deviation] 3 ± 3.96 per participant) with 80% (55/69) of subjects submitting at least one violation. Violations precipitated 116 interventions including hospital admission in 34 (29%) with trans-catheter evaluation in 15 (13%) of those. Growth data (<i>n</i> = 2543 daily weights) were submitted by 63/69 (91%) subjects and precipitated 31 feed changes in 23 participants. Sixty-eight percent of subjects with age >2 months submitted at least one complete neurodevelopment questionnaire.</p><p><strong>Conclusion: </strong>In our initial experience, mobile health monitoring using the KidsHeart app enhanced interstage monitoring permitting earlier intervention, allowed for remote tracking of growth feeding, and provided a means for tracking longitudinal neurodevelopmental outcomes.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-6"},"PeriodicalIF":0.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142458659","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}