Pediatric Cardiology最新文献

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Cognitive Biases in High-Stakes Decision-Making: Implications for Joint Pediatric Cardiology and Cardiothoracic Surgery Conference. 高风险决策中的认知偏差:小儿心脏病学和心胸外科联合会议的意义。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-03-01 Epub Date: 2024-03-24 DOI: 10.1007/s00246-024-03462-4
Joshua A Daily, Stephen Dalby, Lawrence Greiten
{"title":"Cognitive Biases in High-Stakes Decision-Making: Implications for Joint Pediatric Cardiology and Cardiothoracic Surgery Conference.","authors":"Joshua A Daily, Stephen Dalby, Lawrence Greiten","doi":"10.1007/s00246-024-03462-4","DOIUrl":"10.1007/s00246-024-03462-4","url":null,"abstract":"<p><p>Extensive research has consistently demonstrated that humans frequently diverge from rational decision-making processes due to the pervasive influence of cognitive biases. This paper conducts an examination of the impact of cognitive biases on high-stakes decision-making within the context of the joint pediatric cardiology and cardiothoracic surgery conference, offering practical recommendations for mitigating their effects. Recognized biases such as confirmation bias, availability bias, outcome bias, overconfidence bias, sunk cost fallacy, loss aversion, planning fallacy, authority bias, and illusion of agreement are analyzed concerning their specific implications within this conference setting. To counteract these biases and enhance the quality of decision-making, practical strategies are proposed, including the implementation of a no-interruption policy until all data is reviewed, leaders refraining from immediate input, requiring participants to formulate independent judgments prior to sharing recommendations, explicit probability estimations grounded in base rates, seeking external opinions, and promoting an environment that encourages dissenting perspectives.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"536-543"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140194382","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
PDA Stent Placement *6: High-Impact Limitations of Angiography to Delineate a Complex PDA. PDA 支架置入术 *6:血管造影术在确定复杂 PDA 方面的高度局限性。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-03-01 Epub Date: 2024-06-06 DOI: 10.1007/s00246-024-03527-4
Ahmed Deniwar, Mohammad Alnoor, Ahmadreza Ghasemiesfe, Frank F Ing, Timothy J Pirolli, Ralf J Holzer
{"title":"PDA Stent Placement *6: High-Impact Limitations of Angiography to Delineate a Complex PDA.","authors":"Ahmed Deniwar, Mohammad Alnoor, Ahmadreza Ghasemiesfe, Frank F Ing, Timothy J Pirolli, Ralf J Holzer","doi":"10.1007/s00246-024-03527-4","DOIUrl":"10.1007/s00246-024-03527-4","url":null,"abstract":"<p><p>This is a case of an infant with duct-dependent pulmonary circulation, who required 6 stents delivered over three procedures to fully stent the arterial duct, which originated in a very unusual fashion. The attainable angiographic projections were unable to profile its origin, and only a CT scan was ultimately able to delineate the (stenotic) ductal origin from the aorta.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"736-739"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141262522","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
Response to "Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome". 对 "针对左心发育不全综合征右室-肺动脉分流,采用止血钳和球囊血管成形术联合疗法进行肺血流管理 "的回应
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-03-01 Epub Date: 2024-10-05 DOI: 10.1007/s00246-024-03667-7
Mukesh Kumar, F N U Nandni, Sangeeta Davi, F N U Venjhraj, Falak Rehan
{"title":"Response to \"Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome\".","authors":"Mukesh Kumar, F N U Nandni, Sangeeta Davi, F N U Venjhraj, Falak Rehan","doi":"10.1007/s00246-024-03667-7","DOIUrl":"10.1007/s00246-024-03667-7","url":null,"abstract":"<p><p>The article \"Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome\" offers valuable insights into Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome. This letter commends the study for its relevance and potential to improve patient care but highlights several limitations. Notably, the study overlooks the impact of Balloon angioplasty is a useful treatment for recoarctation in individuals with HLHS, but it frequently necessitates further operations. Following Norwood or Fontan palliation, stenting is safe, adaptable, and can be used to control uncommon neointimal growth. The risk of pulmonary artery stenosis may be decreased by the hemi-Fontan the technique. BA is better for recoarctation because it has fewer risks, whereas endovascular stenting helps high-risk patients avoid surgery.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"744-745"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378075","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
Comment on: Early Post-operative ECG Changes as a Predictor of Post-pericardiotomy Syndrome Following Atrial Septal Defect Repair. 评论术后早期心电图变化作为心房室间隔缺损修复术后心包切开综合征的预测指标。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-03-01 Epub Date: 2024-11-12 DOI: 10.1007/s00246-024-03710-7
Huzafa Ali
{"title":"Comment on: Early Post-operative ECG Changes as a Predictor of Post-pericardiotomy Syndrome Following Atrial Septal Defect Repair.","authors":"Huzafa Ali","doi":"10.1007/s00246-024-03710-7","DOIUrl":"10.1007/s00246-024-03710-7","url":null,"abstract":"<p><p>In response to the article \"Early Postoperative ECG Changes as a Predictor of Post-Pericardiotomy Syndrome Following Atrial Septal Defect Repair\" by Hyberg et al. I commend the authors for their valuable insights into early ECG changes predicting postoperative complications. However, I raise several concerns regarding their study's methodology. Specifically, the use of European Society of Cardiology (ESC) criteria without incorporating pediatric-specific criteria, such as those proposed by Heching et al. potentially limits the study's applicability to younger patients. Additionally, reliance solely on ST-segment elevation and PR segment depression for assessing pericardial inflammation overlooks the significance of Spodick's sign. The lack of a standardized timing for postoperative ECGs and the omission of independent risk factors for Post-Pericardiotomy Syndrome (PPS), including history of pericarditis and pneumonia, further limit the study's comprehensiveness. I suggest that future research should address these aspects to refine diagnostic and monitoring strategies.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"747-748"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142625525","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
The Estimated Fick Method Systematically Over-Estimates Cardiac Index Compared to Thermodilution in Children. 与热稀释法相比,估计菲克法系统性地高估了儿童的心脏指数。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-03-01 Epub Date: 2024-05-18 DOI: 10.1007/s00246-024-03491-z
Andrew A Lawson, Paul Tannous, Christina Laternser, Karl Migally
{"title":"The Estimated Fick Method Systematically Over-Estimates Cardiac Index Compared to Thermodilution in Children.","authors":"Andrew A Lawson, Paul Tannous, Christina Laternser, Karl Migally","doi":"10.1007/s00246-024-03491-z","DOIUrl":"10.1007/s00246-024-03491-z","url":null,"abstract":"<p><p>Cardiac index (CI) may be derived from the Fick method, using measured or estimated oxygen consumption (VO<sub>2</sub>), or from thermodilution. In children, LaFarge VO<sub>2</sub> estimates correlate poorly with measured VO<sub>2</sub> values. In a large adult cohort, there was only modest correlation between estimated Fick CI (eFick CI) and thermodilution CI (TDCI). We evaluated the extent of agreement between eFick CI using LaFarge estimates of VO<sub>2</sub> and TDCI in a pediatric cohort. A retrospective, single-center chart review of patients 3-18 years of age who underwent cardiac catheterization with documented eFick CI and TDCI from 2004 to 2020 included 201 catheterizations from 161 unique patients. The mean patient age at catheterization was 12.2y (SD 4.4y). The most frequent diagnosis was cardiomyopathy, followed by congenital heart disease and pulmonary hypertension. TDCI and eFick CI differed by > 20% in 49% of catheterizations. eFick CI systematically exceeded TDCI by a mean percentage difference of 24% (SD 31%). Higher mean CI ((eFick CI + TDCI)/2) and older age were predictive of greater percent difference between eFick CI and TDCI. For each increase in mean CI by 1.0 L/min/m<sup>2</sup>, the expected percent difference in CI increased by 9.9% (p < 0.001). In pediatric patients undergoing cardiac catheterization, eFick CI with LaFarge VO<sub>2</sub> systematically exceeds TDCI. The difference between methods is frequently > 20%, which may have clinically significant implications. Discrepancies between eFick CI and TDCI increase at higher mean CI.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"713-718"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140958743","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
Ductal Stenting in Low-Resource Environments. 低资源环境下的乳腺导管支架植入术
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-03-01 Epub Date: 2024-05-02 DOI: 10.1007/s00246-024-03496-8
Navaneetha Sasikumar, Pranoti Toshniwal, Praveen Reddy Bayya, Abish Sudhakar, Raman Krishna Kumar
{"title":"Ductal Stenting in Low-Resource Environments.","authors":"Navaneetha Sasikumar, Pranoti Toshniwal, Praveen Reddy Bayya, Abish Sudhakar, Raman Krishna Kumar","doi":"10.1007/s00246-024-03496-8","DOIUrl":"10.1007/s00246-024-03496-8","url":null,"abstract":"<p><p>Duct-dependent pulmonary circulation has traditionally been addressed by the Blalock-Taussig-Thomas shunts (BTTS). Recently, catheter-based alternatives such as ductal stenting have emerged as a particularly advantageous option, especially in resource-constrained settings. This article delves into the nuances of ductal stenting within low-resource environments, highlighting its relative ease of application, reduced morbidity, and cost-effectiveness as key factors in its favor. Comparisons in mortality between the two procedures are however likely to be confounded by selection biases. Ductal stenting appears to be particularly beneficial for palliating older infants and children with cyanotic congenital heart disease and diminished pulmonary blood flow who present late. Additionally, it serves as a valuable tool for left ventricular training in late-presenting transposition with an intact ventricular septum. A meticulous pre-procedure echocardiographic assessment of anatomy plays a pivotal role in planning access and hardware, with additional imaging seldom required for this purpose. The adaptation of adult coronary hardware has significantly enhanced the technical feasibility of ductal stenting. However, challenges such as low birth weight and sepsis specifically impact the performance of ductal stenting and patient recovery in low-resource environments. There is potential for systematic application of quality improvement processes to optimize immediate and long-term outcomes of ductal stenting. There is also a need to prospectively examine the application of ductal stenting in low-resource environments through multi-center registries.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"525-535"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140870802","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
Comment on: Evaluation of Cardiac Function in Children Undergoing Liver Transplantation.
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-02-27 DOI: 10.1007/s00246-025-03818-4
Neha Bansal, Nadia Ovchinsky, Joseph Mahgerefteh, Jacqueline M Lamour, Debora Kogan-Liberman, Nadine Choueiter
{"title":"Comment on: Evaluation of Cardiac Function in Children Undergoing Liver Transplantation.","authors":"Neha Bansal, Nadia Ovchinsky, Joseph Mahgerefteh, Jacqueline M Lamour, Debora Kogan-Liberman, Nadine Choueiter","doi":"10.1007/s00246-025-03818-4","DOIUrl":"https://doi.org/10.1007/s00246-025-03818-4","url":null,"abstract":"<p><p>The authors recognize the limitations of our study, most of which are due to being a single-center study and the small sample size of our cohort. We tried to keep our population as homogeneous as possible and included patients only with cirrhosis. Imaging modalities like cardiac MRI, 3D echocardiography, and tissue Doppler imaging as well as exercise testing often give us significantly more information about cardiac function but come with their inherent limitations of requiring general anesthesia, limited availability, and age-dependent variability. We agree with both reviewers that our study lacks the longitudinal aspect which would allow us to study the reversibility of findings post-liver transplantation. However, the purpose of our study was to correlate the pre-transplantation echocardiographic findings as a risk stratification method for post-transplantation clinical measures like duration of mechanical ventilation and length of hospital stay. We believe that the results of our study can serve as a basis for further prospective studies in the evaluation of patients prior to liver transplantation as well as demonstrating reversal of findings in long-term post-transplant period.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143524045","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
Early and Midterm Reintervention in Valve-Sparing Tetralogy of Fallot Repair: Role of Residual Lesion Score.
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-02-27 DOI: 10.1007/s00246-025-03813-9
Javeria Tariq, Asefa Shariq Ansari, Akbar Mistry, Qalab Abbas, Waris Ahmad, Muneer Amanullah, Mahim Malik, Shazia Mohsin
{"title":"Early and Midterm Reintervention in Valve-Sparing Tetralogy of Fallot Repair: Role of Residual Lesion Score.","authors":"Javeria Tariq, Asefa Shariq Ansari, Akbar Mistry, Qalab Abbas, Waris Ahmad, Muneer Amanullah, Mahim Malik, Shazia Mohsin","doi":"10.1007/s00246-025-03813-9","DOIUrl":"https://doi.org/10.1007/s00246-025-03813-9","url":null,"abstract":"<p><p>Tetralogy of Fallot (TOF) is a common congenital heart defect with a traditionally high reintervention rate of post-surgical correction. The Residual Lesion Score (RLS) is a tool used to evaluate the quality of surgical outcomes by assessing postoperative echocardiography findings and the need for unplanned reinterventions. The study assesses the association between the Residual Lesion Score (RLS) and early and midterm outcomes, including reintervention rates, in patients undergoing valve-sparing TOF repair at a tertiary care center in a low- and middle-income country (LMIC). We conducted a retrospective analysis of 62 patients who underwent valve-sparing TOF repair between January 2017 and December 2019 at Aga Khan University, Pakistan. The RLS was calculated based on postoperative echocardiograms and unplanned surgical or catheter-based reinterventions. Patients were categorized into RLS 1 (trivial or no residual lesions), RLS 2 (minor residual lesions), and RLS 3 (major residual lesions or reinterventions before discharge). Two (3.2%) patients lacked postoperative echocardiography, were assigned an RLS score of 5, and excluded from further analysis. Of the 60 patients, 13 (21%) had an RLS of 3, indicating major residual lesions, and 29 (48.3%) had an RLS of 2, indicating minor residual lesions. Early reinterventions were required in 11.7% of patients. Patients with RLS 3 had a significantly higher reintervention rate compared to those with RLS 2 (p = 0.003) reintervention and longer hospital stay (p-value = 0.3). Implementing RLS can help optimize postoperative management and patient outcomes by identifying patients at higher risk of early reintervention.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516288","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
Significant Practice Variation in Oxygen Consumption Values in Congenital Cardiac Catheterization: Cross Sectional Survey in the United States.
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-02-26 DOI: 10.1007/s00246-025-03817-5
Daisuke Kobayashi, Dominic B Zanaboni, Andrew C Glatz, David Balzer
{"title":"Significant Practice Variation in Oxygen Consumption Values in Congenital Cardiac Catheterization: Cross Sectional Survey in the United States.","authors":"Daisuke Kobayashi, Dominic B Zanaboni, Andrew C Glatz, David Balzer","doi":"10.1007/s00246-025-03817-5","DOIUrl":"https://doi.org/10.1007/s00246-025-03817-5","url":null,"abstract":"<p><p>Oxygen consumption (VO<sub>2</sub>) is an essential component to calculate cardiac output using the Fick principle in the congenital cardiac catheterization laboratory (CCCL). Although VO<sub>2</sub> can be measured, the value of VO<sub>2</sub> is frequently assumed based on predictive equations. The objective of this study was to investigate current practice variation in measurement and estimation of VO<sub>2</sub> in CCCL. This was a cross-sectional survey study conducted in January-February 2024. The survey (n = 114) was sent to one interventional cardiologist for each CCCL program in the United States utilizing REDCap. The responses were summarized in a descriptive fashion. Eighty eight programs completed the survey (response rate 77%). Only 13 (15%) programs measured VO<sub>2</sub>, though 67 programs (76%) thought VO<sub>2</sub> should be measured for all cases (n = 41) and selective cases (n = 26) and 17 programs thought assumed VO<sub>2</sub> was satisfactory. When VO<sub>2</sub> is referred from predictive equations, most programs primarily used LaFarge (n = 64, 73%), followed by Seckeler (n = 23, 26%). For patients aged < 3 years (where LaFarge equation does not provide VO2 value) in 64 programs using LaFarge, 16 used Seckeler and the remaining 48 programs used a wide range of arbitrary VO<sub>2</sub> values. Most CCCL thought VO<sub>2</sub> should be measured but only 13 (15%) programs actually measured VO<sub>2</sub> in the United States. LaFarge remains frequently used as a predictive equation. There is no standardization of clinical practice in VO<sub>2</sub> measurement/estimation among CCCL in the United States.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516290","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
Heart Transplantation Outcomes in Pediatric Patients with Noonan Syndrome: An Institutional Case Series.
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2025-02-25 DOI: 10.1007/s00246-025-03812-w
Joseph Andreola, Svetlana Shugh, Robert Winchester, Fredrick Fricker, Maryanne Chrisant, Laura D'Addese
{"title":"Heart Transplantation Outcomes in Pediatric Patients with Noonan Syndrome: An Institutional Case Series.","authors":"Joseph Andreola, Svetlana Shugh, Robert Winchester, Fredrick Fricker, Maryanne Chrisant, Laura D'Addese","doi":"10.1007/s00246-025-03812-w","DOIUrl":"https://doi.org/10.1007/s00246-025-03812-w","url":null,"abstract":"<p><p>Noonan syndrome is an autosomal dominant genetic condition associated with cardiac manifestations that may necessitate heart transplantation. This case series describes the short- and medium-term outcomes in five patients with Noonan syndrome status-post heart transplant followed at our institution. Retrospective, single center chart review of the electronic medical record in post-heart transplant patients with a diagnosis of Noonan syndrome. Five out of 88 heart transplant patients at our institution had genetically confirmed Noonan syndrome with either RAF1 or PTPN11 mutations. All patients were noted to have hypertrophic cardiomyopathy. Severe outflow tract obstruction, in conjunction with comorbidities such as intractable arrhythmias, recurrent syncope, and failure to thrive were leading indications for heart transplant. The most common complications post-heart transplant included recurrent viral infections and pleural and pericardial effusions. Isolated complications included lymphangiectasias, posterior reversible encephalopathy syndrome, and aspergillus pneumonia. Feeding difficulties were common. All patients are alive at the time of this publication. Noonan syndrome is highly associated with hypertrophic cardiomyopathy, and severe cases may necessitate heart transplantation. The post-heart transplant complications seen in our cohort are not unique to the Noonan syndrome population. Survival post-heart transplant is high.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143502433","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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