Pediatric Cardiology最新文献

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Modifiable Health Behaviours in Children with HCM: Lessons from the Heart Health Survey. HCM儿童可改变的健康行为:来自心脏健康调查的经验教训
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-08 DOI: 10.1007/s00246-024-03746-9
Mitchell J Wagner, Aamir Jeewa, Tara Pidborochynski, Samuel Lemaire-Paquette, Michael Khoury, Chentel Cunningham, Santokh Dhillon, Nassiba Alami Laroussi, Laurence Vaujois, Frederic Dallaire, Daryl Schantz, Kathryn Armstrong, Wadi Mawad, Timothy J Bradley, Jennifer Conway
{"title":"Modifiable Health Behaviours in Children with HCM: Lessons from the Heart Health Survey.","authors":"Mitchell J Wagner, Aamir Jeewa, Tara Pidborochynski, Samuel Lemaire-Paquette, Michael Khoury, Chentel Cunningham, Santokh Dhillon, Nassiba Alami Laroussi, Laurence Vaujois, Frederic Dallaire, Daryl Schantz, Kathryn Armstrong, Wadi Mawad, Timothy J Bradley, Jennifer Conway","doi":"10.1007/s00246-024-03746-9","DOIUrl":"https://doi.org/10.1007/s00246-024-03746-9","url":null,"abstract":"<p><p>Obesity factors into hypertrophic cardiomyopathy (HCM)-related risk as a disease modifying environmental factor. Behaviours such as diet and sleep are seldom reported upon in children with HCM. It was our aim to report on these factors in this population. A multicenter cross-sectional study recruited children and teens with HCM aged 10-19 years old from 10 Canadian pediatric care sites. Patient demographics were obtained from review of medical charts. Participants completed the Healthy Hearts Survey, involving 92 questions related to family health history, personal health, smoking, nutrition, and physical or sedentary activity. A cohort of n = 56 patients with median (IQR) age of 15.5 (13.8-16.8) years were enrolled. Over half (53.6%) were classified as overweight or obese based on BMI, with 75% under activity restriction. Consumption of sugar-rich and starchy foods alongside skipping of meals (43.6%) and snacking (87.5%) were reported. A desire to lose weight was prevalent (48.2%) despite general perceptions of good health. Participants slept a median duration of 8 (7.4-9.0) hours on weekdays, however, sleep disturbances were widely reported. A majority (96.4%) reported less than 20 min of exercise per day over the course of the week. Unhealthy patterns regarding diet, sleep, and physical or sedentary activities that associate with cardiovascular risk are present within children with HCM. These habits may worsen disease burden in children with HCM. It is unclear how activity restriction factors into such patterns. There are modifiable lifestyle factors in children with HCM which, if addressed, could positively impact cardiovascular risk.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142952819","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}
引用次数: 0
Cerebral Protection in Pediatric Cardiac Surgery. 儿童心脏外科的脑保护。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-08 DOI: 10.1007/s00246-024-03748-7
Sheida Shams-Molkara, Vitor Mendes, François Verdy, Maria-Helena Perez, Stefano Di Bernardo, Matthias Kirsch, Amir-Reza Hosseinpour
{"title":"Cerebral Protection in Pediatric Cardiac Surgery.","authors":"Sheida Shams-Molkara, Vitor Mendes, François Verdy, Maria-Helena Perez, Stefano Di Bernardo, Matthias Kirsch, Amir-Reza Hosseinpour","doi":"10.1007/s00246-024-03748-7","DOIUrl":"https://doi.org/10.1007/s00246-024-03748-7","url":null,"abstract":"<p><p>Cardiac surgery, both adult and pediatric, has developed very rapidly and impressively over the past 7 decades. Pediatric cardiac surgery, in particular, has revolutionized the management of babies born with congenital heart disease such that now most patients reach adult life and lead comfortable lives. However, these patients are at risk of cerebral lesions, which may be due to perioperative factors, such as side effects of cardiopulmonary bypass and/or anesthesia, and non-perioperative factors such as chromosomal anomalies (common in children with congenital heart disease), the timing of surgery, number of days on the intensive care unit, length of hospitalization and other hospitalizations in the first year of life. The risk of cerebral lesions is particularly relevant to pediatric cardiac surgery given that cerebral metabolism is about 30% higher in neonates, infants and young children compared to adults, which renders their brain more susceptible to ischemic/hypoxic injury. This issue has been a major concern throughout the history of cardiac surgery such that many preventive measures have been implemented over the years. These measures, however, have had only a modest impact and cerebral lesions continue to be a major concern. This is the subject of this review article, which aims to outline these protective measures, offer possible explanations of why these have not resolved the issue, and suggest possible actions that ought to be taken now.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142952814","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}
引用次数: 0
Preoperative Oral Feeding in Infants with Congenital Heart Disease Within the First Month of Life is Associated with a Higher Likelihood of Freedom From Tube Feeding at Time of Postoperative Discharge. 先天性心脏病婴儿在出生后第一个月内术前口服喂养与术后出院时免于管饲的可能性较高相关
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-08 DOI: 10.1007/s00246-024-03750-z
Aseel Dabbagh, Sarah Miller, Michael McCulloch, Geoffrey Rosenthal, Mark Conaway, Shelby White
{"title":"Preoperative Oral Feeding in Infants with Congenital Heart Disease Within the First Month of Life is Associated with a Higher Likelihood of Freedom From Tube Feeding at Time of Postoperative Discharge.","authors":"Aseel Dabbagh, Sarah Miller, Michael McCulloch, Geoffrey Rosenthal, Mark Conaway, Shelby White","doi":"10.1007/s00246-024-03750-z","DOIUrl":"https://doi.org/10.1007/s00246-024-03750-z","url":null,"abstract":"<p><p>Though several studies have demonstrated that preoperative oral feeding (PO) can be safe in patients with congenital heart disease, they are commonly prohibited from doing so, potentially precluding the development of such skills. We sought to determine whether preoperative oral feeding is associated with freedom from tube feeding at postoperative discharge. Single-center, observational study including patients in the first month of life (≤ 30 days of age) who underwent a single cardiac surgery between 7/1/2017-6/30/2022 and survived to discharge. Preoperative PO was defined as any oral intake up to the day of cardiac surgery. General demographics and peri-operative characteristics were analyzed. A total of 235 patients were included of which 178 (78%) PO fed preoperatively, and 171 (73%) were discharged taking full PO. Those discharged without tube feeds received more preoperative oral feeds, were less likely to have a chromosomal abnormality/genetic syndrome, had lower STAT categories, and were less likely to have postoperative complications, vocal cord dysfunction or prolonged mechanical ventilation. Multivariate analysis found that any preoperative PO [odds ratio (OR) of 2.78 (CI 1.48, 5.24, p-value = 0.002)] and increasing amounts of PO were predictive of full PO intake at discharge [≤ 20 ml/kg/day (OR 2.06, CI 1.03, 4.14, p-value = 0.042) and > 20 ml/kg/day (OR 4.07, CI 1.88, 8.84, p-value = 0.004)]. Preoperative oral feeding is a strong predictor of discharging with full PO intake after cardiac surgery in the first month of life and that it may also improve with increasing volumes. Multi-institutional analyses are warranted.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142952822","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}
引用次数: 0
Proposal: Bold New Indications for Transcatheter Pulmonary Flow Restrictors. 建议:大胆的经导管肺限流器新适应症。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-05 DOI: 10.1007/s00246-024-03759-4
Dietmar Schranz
{"title":"Proposal: Bold New Indications for Transcatheter Pulmonary Flow Restrictors.","authors":"Dietmar Schranz","doi":"10.1007/s00246-024-03759-4","DOIUrl":"https://doi.org/10.1007/s00246-024-03759-4","url":null,"abstract":"<p><p>This proposal presents a proof of concept for the use of pulmonary flow restrictors (PFRs) based on MVP™-devices, drawing from clinical experience, and explores their potential role in the management of newborns with hypoplastic left heart syndrome (HLHS), other complex left heart lesions, and infants with end-stage dilated cardiomyopathy (DCM). At this early stage of age, manually adjusted PFRs can be tailored to patient's size and hemodynamic needs. Although currently used off-label, PFRs have substantial potential to improve outcomes in these vulnerable patient populations. When integrated into a holistic treatment strategy, they represent a promising advancement in care. There is significant potential to reduce mortality and improve the quality of life for neonates with HLHS and variants, as well as to support age-dependent functional regeneration in DCM, all while avoiding the need for advanced surgical procedures, including general anesthesia. The main barrier to broader adoption is the limited availability of appropriately sized MVP devices. However, with ongoing patient-centered knowledge sharing and refinement of transcatheter techniques, there is reason for optimism that PFRs, tailored for individual patients, could provide significant benefits to thousands of infants with congenital heart disease and DCM worldwide.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932382","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}
引用次数: 0
Clarifying the Cardiovascular Morphology and Associated Abnormalities in Patients with Double Inlet Right Ventricle Using Multidetector CT Angiography. 利用多层螺旋CT血管造影研究双入口右心室患者的心血管形态及相关异常。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-05 DOI: 10.1007/s00246-024-03766-5
Niraj Nirmal Pandey, Mansi Verma, Sheetal Sharma, Vineeta Ojha, Sanjeev Kumar, Sivasubramanian Ramakrishnan, Priya Jagia
{"title":"Clarifying the Cardiovascular Morphology and Associated Abnormalities in Patients with Double Inlet Right Ventricle Using Multidetector CT Angiography.","authors":"Niraj Nirmal Pandey, Mansi Verma, Sheetal Sharma, Vineeta Ojha, Sanjeev Kumar, Sivasubramanian Ramakrishnan, Priya Jagia","doi":"10.1007/s00246-024-03766-5","DOIUrl":"https://doi.org/10.1007/s00246-024-03766-5","url":null,"abstract":"<p><p>We sought to evaluate the intracardiac morphology and associated cardiovascular anomalies in patients with double inlet right ventricle (DIRV) on multidetector CT angiography. A retrospective search of our departmental database was conducted from January 2014 to January 2023 to identify patients with a diagnosis of DIRV on CT angiography. The intracardiac anatomy and associated cardiovascular abnormalities were systematically evaluated. Patients with a common atrioventricular valve were excluded. DIRV was diagnosed in 41 patients (36 males, 5 females; mean age at imaging: 7.4 years [range: 4 months-30 years]). Mirror-imaged viscero-atrial arrangement was observed in 12/41 (29.3%) while right isomerism and left isomerism were seen in 6/41 (14.6%) and 2/41 (4.9%) patients respectively. Hypoplastic left ventricular cavity was seen in 39/41 (95.1%) patients. AV valve straddling was observed in 40/41 (97.6%) patients. Atrial septal defect was seen in 28/41 (68.3%) patients. All (100%) patients had presence of ventricular septal defect. Twisted atrioventricular connections were seen in 4/41 (9.8%) patients. The most common ventriculo-arterial morphology was presence of double outlet right ventricle (DORV) seen in 25/41 (61%) patients. Some degree of pulmonary outflow obstruction was seen in 24/41 (58.5%) patients. Systemic venous anomalies, pulmonary venous anomalies and coronary artery anomalies were observed in 13/41 (31.7%), 6/41 (14.6%) and 8/41 (19.5%) patients. DIRV is a type of single ventricle congenital heart disease associated with an array of cardiovascular abnormalities, most commonly DORV and pulmonary outflow tract obstruction. The knowledge of associated anomalies is imperative for preoperative planning. CT angiography allows for detailed anatomical evaluation of DIRV and associated cardiovascular anomalies which may positively impact surgical management in these patients.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932353","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}
引用次数: 0
Medical & Socioeconomic Risk Factors Associated with Lack of Neurodevelopmental Evaluation Following Neonatal Cardiac Surgery. 新生儿心脏手术后缺乏神经发育评估的医疗和社会经济风险因素
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-04 DOI: 10.1007/s00246-024-03761-w
Luma Essaid, Kelly Haque, Amanda Shillingford, Lauren Zimmerman, Alisa Burnham, Lyla Hampton, Oluwatimilehin Okunowo, J William Gaynor, Nicholas S Abend, Maryam Y Naim, Monique M Gardner
{"title":"Medical & Socioeconomic Risk Factors Associated with Lack of Neurodevelopmental Evaluation Following Neonatal Cardiac Surgery.","authors":"Luma Essaid, Kelly Haque, Amanda Shillingford, Lauren Zimmerman, Alisa Burnham, Lyla Hampton, Oluwatimilehin Okunowo, J William Gaynor, Nicholas S Abend, Maryam Y Naim, Monique M Gardner","doi":"10.1007/s00246-024-03761-w","DOIUrl":"https://doi.org/10.1007/s00246-024-03761-w","url":null,"abstract":"<p><p>Neonates with congenital heart disease (CHD) who undergo cardiopulmonary bypass (CPB) are at high-risk for unfavorable neurodevelopmental (ND) outcomes and are recommended for ND evaluation (NDE); however, poor rates have been reported. We aimed to identify risk factors associated with lack of NDE. This single-center retrospective observational study included neonates < 30 days old who underwent CPB and survived to discharge between 2012 and 2018. Primary outcome (NDE) was ≥ 1 appointment at our center's dedicated cardiac, neonatal, or general ND clinics before the 3rd birthday. Predictor variables included demographic, medical, and social factors. Social disorganization index (SDI) was obtained with geocoding based on address at time of discharge. Logistic regression identified risk factors associated with lack of NDE. The cohort included 594 patients, predominantly male (59%) and white (59%). A majority (63%) had NDE. Lack of NDE was more common in patients with postnatal CHD diagnosis, CHD without arch obstruction, absence of postoperative seizures, living below 100% poverty level, lack of insurance, younger parental age, and overall higher SDI (p < 0.03). In multivariable analysis, lack of NDE was associated with single-ventricle CHD without arch obstruction (OR 2.17; 95% CI 1.08-4.55), two ventricle CHD without arch obstruction (OR 2.56; 95% CI 1.59-4.17), and higher SDI (OR 1.25; 95% CI 1.05-1.49); all p < 0.05. This study identifies medical and neighborhood-level socioeconomic factors that may help address care gaps in this high-risk population. Patients with socioeconomic disparities may benefit from increased care coordination upon discharge.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142927660","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}
引用次数: 0
Correction: Short and Mid-term Outcomes of Ductal Graft with Bilateral Pulmonary Banding in Hypoplastic Left Heart Syndrome and Variants. 更正:左心发育不全综合征及变异型的导管移植与双侧肺束带术的短期和中期疗效。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-01 DOI: 10.1007/s00246-024-03640-4
Ayla Oktay, Ahmet Arnaz, Canan Ayabakan, Tayyar Sarioglu, Yusuf Kenan Yalcinbas
{"title":"Correction: Short and Mid-term Outcomes of Ductal Graft with Bilateral Pulmonary Banding in Hypoplastic Left Heart Syndrome and Variants.","authors":"Ayla Oktay, Ahmet Arnaz, Canan Ayabakan, Tayyar Sarioglu, Yusuf Kenan Yalcinbas","doi":"10.1007/s00246-024-03640-4","DOIUrl":"10.1007/s00246-024-03640-4","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"246"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142292933","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}
引用次数: 0
Correction: Ewing's Sarcoma Metastazing from the Right Pelvis via the Inferior Vena Cava into the Heart: Diagnosis and Successful Surgical Treatment. 更正:从右骨盆经下腔静脉进入心脏的尤文肉瘤转移:诊断和成功的手术治疗。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-01 DOI: 10.1007/s00246-024-03690-8
Joachim Peter Sturmberg, Hans Meyer, Reiner Körfer, Wolfgang Matthies, Wolf-Rüdiger Thies, Alfred Schauer, Peter Weinel
{"title":"Correction: Ewing's Sarcoma Metastazing from the Right Pelvis via the Inferior Vena Cava into the Heart: Diagnosis and Successful Surgical Treatment.","authors":"Joachim Peter Sturmberg, Hans Meyer, Reiner Körfer, Wolfgang Matthies, Wolf-Rüdiger Thies, Alfred Schauer, Peter Weinel","doi":"10.1007/s00246-024-03690-8","DOIUrl":"10.1007/s00246-024-03690-8","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"253"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142583597","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}
引用次数: 0
Correction: Screening for Life-Threatening Arrhythmia in Asymptomatic Patients After Paediatric Cardiac Surgery: A Single-Centre Retrospective Analysis of 790 Pre-hospital-discharge 24-h Holter Electocardiogram Recordings. 更正:筛查儿科心脏手术后无症状患者中危及生命的心律失常:对 790 份出院前 24 小时 Holter 心电图记录的单中心回顾性分析。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-01 DOI: 10.1007/s00246-024-03709-0
Evangelia Blana, Matthias Gass, Florian Berger, Hitendu Dave, Christian Balmer
{"title":"Correction: Screening for Life-Threatening Arrhythmia in Asymptomatic Patients After Paediatric Cardiac Surgery: A Single-Centre Retrospective Analysis of 790 Pre-hospital-discharge 24-h Holter Electocardiogram Recordings.","authors":"Evangelia Blana, Matthias Gass, Florian Berger, Hitendu Dave, Christian Balmer","doi":"10.1007/s00246-024-03709-0","DOIUrl":"10.1007/s00246-024-03709-0","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"254"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142681916","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}
引用次数: 0
Left Atrial Phasic Function via Cardiac Magnetic Resonance Imaging in Patients with Duchenne Muscular Dystrophy. Duchenne肌营养不良患者的心脏磁共振成像左心房时相功能。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-01-01 Epub Date: 2023-11-08 DOI: 10.1007/s00246-023-03327-2
Taylor M Trussell, Tyler Brown, Elisa Marcuccio, Anna Mullikin, Huaiyu Zang, Nicholas J Ollberding, Chet Villa, Sean M Lang
{"title":"Left Atrial Phasic Function via Cardiac Magnetic Resonance Imaging in Patients with Duchenne Muscular Dystrophy.","authors":"Taylor M Trussell, Tyler Brown, Elisa Marcuccio, Anna Mullikin, Huaiyu Zang, Nicholas J Ollberding, Chet Villa, Sean M Lang","doi":"10.1007/s00246-023-03327-2","DOIUrl":"10.1007/s00246-023-03327-2","url":null,"abstract":"<p><p>Cardiac dysfunction is a leading cause of morbidity and mortality in Duchenne muscular dystrophy (DMD). Left atrial (LA) function is a poorly understood concept in this patient population, and research suggests underlying structural changes that could affect atrial function. Cardiac magnetic resonance (CMR) imaging may provide an important non-invasive approach to evaluating LA function. This study was a single center retrospective review of consecutive CMR studies over a 1 year period comparing LA phasic function within a cohort of DMD patients, and to those with structurally and functionally normal hearts. LA strain measurements including global reservoir, conduit, boost-pump strain, and LA volumes were obtained retrospectively. Spearman correlation analyses were performed on atrial strain measurements. 107 DMD and 79 normal CMR studies were included. The DMD cohort had worse systolic function (p < 0.001), smaller indexed max LA and left ventricular (LV) volumes (p < 0.001), and greater LA emptying fraction (p < 0.001). In the DMD cohort, emptying fraction decreased with advanced patient age (p < 0.001) and diminishing systolic function (p < 0.001). DMD patients with moderate or severe LV dysfunction demonstrated lower LA emptying fraction (p = 0.002), more impaired 2-chamber LA reservoir (p = 0.003), and LA pump (p = 0.006) and conduit strain (p = 0.018). DMD patients with preserved function have lower indexed LA volumes with higher LA emptying fractions than controls. Progression of disease and age is associated with decreased LA emptying fraction with early manifestations in reservoir and conduit strain. These findings suggest that strain markers of LA compliance and early left ventricular relaxation are associated with worsening cardiomyopathy in the DMD population.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"72-80"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71522321","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}
引用次数: 0
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