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Evaluation of the extent of left ventricular trabeculations and association with imaging findings and clinical outcomes in pediatric patients with possible left ventricular noncompaction cardiomyopathy 评估可能患有左心室非充盈性心肌病的儿科患者的左心室小梁范围及其与成像结果和临床预后的关系
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-29 DOI: 10.1016/j.ppedcard.2024.101719
Ian Milligan , Sassan Hashemi , Denver Sallee , Ritu Sachdeva , Erik Michelfelder , Timothy Slesnick , Hunter Wilson
{"title":"Evaluation of the extent of left ventricular trabeculations and association with imaging findings and clinical outcomes in pediatric patients with possible left ventricular noncompaction cardiomyopathy","authors":"Ian Milligan ,&nbsp;Sassan Hashemi ,&nbsp;Denver Sallee ,&nbsp;Ritu Sachdeva ,&nbsp;Erik Michelfelder ,&nbsp;Timothy Slesnick ,&nbsp;Hunter Wilson","doi":"10.1016/j.ppedcard.2024.101719","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101719","url":null,"abstract":"<div><h3>Background</h3><p>Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent trabeculations in the left ventricle and has been associated with adverse outcomes; yet, there is difficulty in discerning pathologic trabeculations from normal variation.</p></div><div><h3>Objectives</h3><p>This study sought to test the hypothesis that a ratio of noncompacted to compacted (NC:C) myocardium of &gt;2.3 in diastole by cardiac magnetic resonance imaging (CMR) is associated with adverse clinical outcome and imaging findings in children and young adults referred for CMR to assess for presence of LVNC cardiomyopathy.</p></div><div><h3>Methods</h3><p>A retrospective cohort study was performed of patients who were referred for CMR at our center for suspected LVNC between 1/1/11 and 12/31/21. An NC:C ratio was calculated for each CMR, and patients were classified as CMR+ or CMR− using a NC:C cutoff of &gt;2.3. Demographic, electrocardiographic, echocardiographic, CMR, and clinical outcome data were compared.</p></div><div><h3>Results</h3><p>Fifty-one patients were identified, with 30 (59 %) being CMR+. Patients were 12.9 ± 4.7 years old at the time of CMR and followed for a median of 1.7 (IQR 0.1–4.4) years. Baseline characteristics were similar between the CMR+ and CMR− groups. Of the cohort, 1/51 (2 %) who was CMR−, had a stroke, and 5/51 (10 %) experienced significant arrhythmia or ectopy with no significant difference between the groups. There was no mortality, transplant, or mechanical support. LV systolic dysfunction was noted in 8/51 (16 %) of patients by CMR, with no significant difference between the CMR+ and CMR− groups. There was no correlation between the NC:C ratio and left or right ventricular systolic function by CMR.</p></div><div><h3>Conclusion</h3><p>NC:C ratio, as measured by CMR, alone may be an inadequate marker of pathologic left ventricular trabeculations in children and young adults.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"73 ","pages":"Article 101719"},"PeriodicalIF":0.9,"publicationDate":"2024-02-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140103788","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Valve repair for tricuspid valve regurgitation in pediatric patients: Midterm outcomes 小儿三尖瓣反流的瓣膜修复术:中期疗效
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-27 DOI: 10.1016/j.ppedcard.2024.101715
Toshi Maeda, Kosuke Yoshizawa, Otohime Mori
{"title":"Valve repair for tricuspid valve regurgitation in pediatric patients: Midterm outcomes","authors":"Toshi Maeda,&nbsp;Kosuke Yoshizawa,&nbsp;Otohime Mori","doi":"10.1016/j.ppedcard.2024.101715","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101715","url":null,"abstract":"<div><h3>Background</h3><p>Tricuspid valve regurgitation in pediatric patients has heterogeneous lesions, and surgical intervention is sometimes necessary. Tricuspid valve repair is the preferred treatment for tricuspid valve regurgitation.</p></div><div><h3>Objectives</h3><p>This study aimed to review the midterm outcomes of tricuspid valve repair for tricuspid valve regurgitation in pediatric patients.</p></div><div><h3>Methods</h3><p>A total of 26 patients aged &lt; 18 years who underwent tricuspid valve repair for tricuspid valve regurgitation at our institute between January 2005 and September 2022 were retrospectively reviewed. Their median age was 3.7 years (range, 1 month–16.2 years), and 15 were males. 23 had congenital heart diseases. Among them, 35 % of patients had tetralogy of Fallot or pulmonary atresia with ventricular septal defect (9/26), followed by ventricular septal defect (23 %, 6/26).</p></div><div><h3>Results</h3><p>Several reparative techniques were used according to valve lesions. Commissural edge-to-edge suture was the most frequently used technique. There were two cases of hospital death, and no late death occurred. During the median follow-up period of 7.8 years (range, 5 months–17.5 years), reoperation was performed in one patient with pulmonary atresia with intact ventricular septum. At the last follow-up, all survivors had mild or less tricuspid valve regurgitation. No patient had tricuspid valve stenosis. Postoperative tricuspid annular diameter increased within the normal range in all patients. Survival and reoperation-free rates at both 5 and 10 years were 92.3 % and 95.8 %, respectively.</p></div><div><h3>Conclusion</h3><p>Tricuspid valve repair for tricuspid valve regurgitation in pediatric patients is safe and feasible with satisfactory midterm outcomes.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"73 ","pages":"Article 101715"},"PeriodicalIF":0.9,"publicationDate":"2024-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139999886","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Use of Sacubitril/Valsartan in atypical hemolytic uremic syndrome induced dilated cardiomyopathy in a pediatric patient 萨库比特利/缬沙坦在非典型溶血性尿毒症综合征诱发的扩张型心肌病儿科患者中的应用
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-24 DOI: 10.1016/j.ppedcard.2024.101714
Terence Truong, Swati Sehgal
{"title":"Use of Sacubitril/Valsartan in atypical hemolytic uremic syndrome induced dilated cardiomyopathy in a pediatric patient","authors":"Terence Truong,&nbsp;Swati Sehgal","doi":"10.1016/j.ppedcard.2024.101714","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101714","url":null,"abstract":"<div><p>Dilated cardiomyopathy is a rare but dangerous cardiac manifestation of hemolytic anemia that can lead to heart failure. We report a 3-year-old male with dilated cardiomyopathy refractory to angiotensin converting enzyme inhibitor treated successfully with Sacubitril/Valsartan combination.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"73 ","pages":"Article 101714"},"PeriodicalIF":0.9,"publicationDate":"2024-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140290594","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Surgery for tricuspid regurgitation associated with ostium secundum atrial septal defects in adults: Three cases 成人三尖瓣反流伴房间隔缺损(ostium secundum atrial septal defects)的手术治疗:三个病例
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-22 DOI: 10.1016/j.ppedcard.2024.101713
Nsiri Salma, Khedija Soumer, Houcine Horchani, Nadia Azabou, Mouna Bousnina, Amine Jemel
{"title":"Surgery for tricuspid regurgitation associated with ostium secundum atrial septal defects in adults: Three cases","authors":"Nsiri Salma,&nbsp;Khedija Soumer,&nbsp;Houcine Horchani,&nbsp;Nadia Azabou,&nbsp;Mouna Bousnina,&nbsp;Amine Jemel","doi":"10.1016/j.ppedcard.2024.101713","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101713","url":null,"abstract":"<div><p>Small atrial septal defects are usually asymptomatic and routinely followed in young patients. However, closure of atrial septal defects becomes necessary if defects fail to close or if size of the defects results in hemodynamically significant shunting. Patients with untreated left to right shunting are at risk for long term complications. Including enlargement of right heart structures, tricuspid annular dilatation with secondary valvular insufficiency, and heart failure. Older adult patients may be at risk for paradoxical embolism. Patients are generally referred for surgery before school age. Older patients may be considered for device closure. Nonetheless, in some cases, patients are not diagnosed early or are not followed up sufficiently, leading to progression of disease and discovery in adulthood. We present three adult cases with large atrial septal defects associated with tricuspid valve regurgitation managed surgically.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"72 ","pages":"Article 101713"},"PeriodicalIF":0.9,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139944945","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A rare association of congenital diaphragmatic eventration in complex cyanotic heart defect: Should we miss? 复杂性紫绀型心脏畸形中罕见的先天性膈肌分离:我们是否应该错过?
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-11 DOI: 10.1016/j.ppedcard.2024.101711
Anis Munirah MK
{"title":"A rare association of congenital diaphragmatic eventration in complex cyanotic heart defect: Should we miss?","authors":"Anis Munirah MK","doi":"10.1016/j.ppedcard.2024.101711","DOIUrl":"10.1016/j.ppedcard.2024.101711","url":null,"abstract":"<div><p>Congenital diaphragmatic eventration is relatively uncommon. The association with congenital cyanotic heart defect is even more uncommon. The identification of these conditions at an early stage is a significant challenge due to the similarities in symptoms exhibited by cyanotic heart disease and diaphragmatic eventration. We present a case of a two-year-old child with an underlying Ebstein anomaly, pulmonary atresia, and ventricular septal defect. Despite a few previous hospitalizations due to the underlying cardiac defect, the child was diagnosed with left diaphragmatic eventration at the age of 13 months. A decision was made to perform diaphragmatic plication before cardiac surgery following a multidisciplinary team discussion to optimize lung function and prevent complications after cardiac surgery.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"72 ","pages":"Article 101711"},"PeriodicalIF":0.9,"publicationDate":"2024-02-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139830257","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Fontan hepatic fibrosis and serial liver biopsies 丰坦肝纤维化和连续肝活检
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-06 DOI: 10.1016/j.ppedcard.2024.101709
William N. Evans, Ruben J. Acherman, Alvaro Galindo, Abraham Rothman
{"title":"Fontan hepatic fibrosis and serial liver biopsies","authors":"William N. Evans,&nbsp;Ruben J. Acherman,&nbsp;Alvaro Galindo,&nbsp;Abraham Rothman","doi":"10.1016/j.ppedcard.2024.101709","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101709","url":null,"abstract":"<div><h3>Background</h3><p>The value and timing of liver biopsy, as a component of monitoring Fontan-associated liver disease progression, is unknown.</p></div><div><h3>Objective</h3><p>We compared liver total fibrosis scores (TFS) derived from post-Fontan patients who underwent an initial and subsequent transvenous hepatic biopsy.</p></div><div><h3>Methods</h3><p>We identified post-Fontan patients who had transvenous hepatic biopsy during cardiac catheterization between January 2012 and September 2023. We selected those that had both an initial and subsequent study. We calculated a TFS (0–8) by summing the portal fibrosis scores (0–4) and the sinusoidal fibrosis scores (0–4).</p></div><div><h3>Results</h3><p>We identified 181 patients, of which 56 underwent an initial and subsequent study. Of the 56, 33 (58.9) were male. The average time between studies was 5.2 ± 1.5 years. The average age at biopsy 1 was 17.6 ± 9.6 years and 23.9 ± 9.8 years at biopsy 2 (<em>p</em> = .002), and the average Fontan duration at biopsy 1 was 14.0 ± 8.1 years and 19.4 ± 7.8 years at biopsy 2 (<em>p</em> = .0003). Overall, the average TFS at biopsy 1 was 2.6 ± 1.4 and 2.7 ± 1.5 at biopsy 2 (<em>p</em> = .56). However, for the 56 patients, 23 had an increase in TFS (average increase of 1.7), 15 had a decrease in TFS (average decrease of 1.8), and 18 had no change in TFS; further, there were no differences in average Fontan mean pressures between the first and second biopsy regardless of whether TFS increased, decreased or were unchanged.</p></div><div><h3>Conclusion</h3><p>In a cohort of primarily young, stable Fontan patients, despite a significant five-year average interval between transvenous hepatic biopsies, we found no significant change in average TFS for the cohort as a whole. However, some patients demonstrated an increase, decrease, or no change in TFS between biopsies. Such differences could be related to hepatic fibrosis heterogenicity and subjective biopsy grading. To our knowledge, this is the largest cohort of post-Fontan patients who have undergone a comparison of liver fibrosis scoring between initial and subsequent liver biopsies.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"72 ","pages":"Article 101709"},"PeriodicalIF":0.9,"publicationDate":"2024-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139714197","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Modified microvascular plug as a flow restrictor in a single ventricle patient with MAPCAs 将改良微血管栓作为单心室 MAPCA 患者的限流器
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-06 DOI: 10.1016/j.ppedcard.2024.101712
Chelsea Miller , Michael J. Shorofsky , Michael Hainstock , Samuel Casella
{"title":"Modified microvascular plug as a flow restrictor in a single ventricle patient with MAPCAs","authors":"Chelsea Miller ,&nbsp;Michael J. Shorofsky ,&nbsp;Michael Hainstock ,&nbsp;Samuel Casella","doi":"10.1016/j.ppedcard.2024.101712","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101712","url":null,"abstract":"<div><p>Balancing systemic and pulmonary circulation is paramount to the management of single ventricle heart disease. Insufficient pulmonary blood flow has been managed with a variety of surgical and transcatheter therapeutic options, whereas excessive pulmonary blood flow has been limited to surgical management. Recently, reports have described the use of a modified Microvascular Plug as a pulmonary flow restrictor. We report the use of this technique in a 5-month-old with single ventricle physiology and major aortopulmonary collateral arteries whose clinical course had been complicated by pulmonary over-circulation and necrotizing enterocolitis.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"72 ","pages":"Article 101712"},"PeriodicalIF":0.9,"publicationDate":"2024-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139718630","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Fast-track extubation guided by pulmonary artery pressure monitoring after ventricular septal defect closure in infants with pulmonary hypertension 肺动脉高压婴儿室间隔缺损封堵术后在肺动脉压力监测指导下快速拔管
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-04 DOI: 10.1016/j.ppedcard.2024.101710
Haruna Araki , Fumiaki Shikata , Shinzo Torii , Tadashi Kitamura , Toshiaki Mishima , Masaomi Fukuzumi , Yusuke Motoji , Rihito Horikoshi , Yoshimi Tamura , Daiki Ishiwaki , Yoichiro Hirata , Kagami Miyaji
{"title":"Fast-track extubation guided by pulmonary artery pressure monitoring after ventricular septal defect closure in infants with pulmonary hypertension","authors":"Haruna Araki ,&nbsp;Fumiaki Shikata ,&nbsp;Shinzo Torii ,&nbsp;Tadashi Kitamura ,&nbsp;Toshiaki Mishima ,&nbsp;Masaomi Fukuzumi ,&nbsp;Yusuke Motoji ,&nbsp;Rihito Horikoshi ,&nbsp;Yoshimi Tamura ,&nbsp;Daiki Ishiwaki ,&nbsp;Yoichiro Hirata ,&nbsp;Kagami Miyaji","doi":"10.1016/j.ppedcard.2024.101710","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101710","url":null,"abstract":"<div><h3>Background</h3><p>The fast-track extubation approach in pediatric cardiac surgery shortens hospital stays and reduces medical costs.</p></div><div><h3>Objectives</h3><p>This study aims to evaluate the feasibility of the fast-track extubation approach and the role of intraoperative pulmonary artery pressure (PAP) monitoring in managing patients with preoperative pulmonary hypertension (PH) who underwent ventricular septal defect (VSD) closure.</p></div><div><h3>Methods</h3><p>This retrospective study included 102 infants with VSD and preoperative PH (mean PAP &gt; 20 mmHg on cardiac catheterization). We excluded four cases that required complex procedures with long cardiopulmonary bypass time for associated lesions. Intraoperative systolic PAP monitoring was performed to evaluate the suitability of the fast-track extubation approach (&lt;35 mmHg). We defined the fast-track extubation approach as extubation in the operation room after VSD closure. This study also aimed to assess the predictors of postoperative adverse events. Postoperative adverse events included in-hospital death, PH crisis and prolonged mechanical ventilation time, and nitric oxide (NO) administration.</p></div><div><h3>Results</h3><p>The fast-track extubation approach was carried out in 39 patients (38 %). One in-hospital death (1 %) occurred in a non-fast-track patient due to postoperative PH crisis. Two patients (5.1 %) required re-intubation after the fast-track extubation approach due to upper airway issues. The frequency of NO inhalation and administration of pulmonary hypertensive medicines was significantly lower in the fast-track group than in the non-fast-track group (<em>P</em> &lt; 0.001). Multivariate analysis revealed that body weight of &lt;4 kg at surgery (<em>P</em> = 0.006), surgery by trainees (<em>P</em> = 0.003), and greater intraoperative systolic PAP (<em>P</em> &lt; 0.001) were significant risk factors for postoperative adverse events.</p></div><div><h3>Conclusions</h3><p>Our findings showed that the fast-track extubation approach after VSD closure in infants with preoperative PH was feasible, as indicated by the low frequency of re-intubation and postoperative adverse events. Intraoperative systolic PAP measurement was useful for selecting patients to be included in the fast-track extubation approach.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"72 ","pages":"Article 101710"},"PeriodicalIF":0.9,"publicationDate":"2024-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139710042","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Favorable long term clinical outcomes in two patients with neonatal Marfan syndrome 两名新生儿马凡氏综合征患者的长期临床治疗效果良好
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-01 DOI: 10.1016/j.ppedcard.2024.101707
Keerthana Reddy Banala , Dean Karahalios , Yamuna Sanil , Swati Sehgal
{"title":"Favorable long term clinical outcomes in two patients with neonatal Marfan syndrome","authors":"Keerthana Reddy Banala ,&nbsp;Dean Karahalios ,&nbsp;Yamuna Sanil ,&nbsp;Swati Sehgal","doi":"10.1016/j.ppedcard.2024.101707","DOIUrl":"10.1016/j.ppedcard.2024.101707","url":null,"abstract":"<div><p>Neonatal Marfan syndrome is a rare condition with poor prognosis. It is genotypically and phenotypically distinct from classical Marfan syndrome, as it presents with a higher incidence of atrioventricular valve regurgitation and early mortality due to fibrillin 1 (<em>FBN1)</em> gene mutations in exons 23–32. This case series describes two cases of neonatal Marfan syndrome with favorable clinical status at 4 and 2.5 years of age (Patient 1 and Patient 2, respectively). Both patients were noted to have typical Marfanoid physical characteristics after birth, including arachnodactyly and dilated aortic root. Genetic analysis confirmed <em>FBN1</em> mutation in exons 26 and 27 in patient 1 and patient 2, respectively. Both patients are treated with atenolol and losartan. Patient 2 required valve-sparing aortic root replacement at 24 months of age. Both patients are doing well clinically past the first year of life, which is a rare but favorable clinical outcome for patients with neonatal Marfan syndrome. Our cases highlight that <em>FBN1</em> mutations result in a broad range of phenotypes and clinical severity, even if the mutations are located in the neonatal region.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"72 ","pages":"Article 101707"},"PeriodicalIF":0.9,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139885933","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cardiopulmonary exercise testing among pediatric patients with severe obesity prior to bariatric surgery 减肥手术前重度肥胖儿科患者的心肺运动测试
IF 0.9
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-02-01 DOI: 10.1016/j.ppedcard.2024.101708
Joanna E. Nelson , Kimberly M. Sanchez , Yujing Yao , Zhezhen Jin , Jeffrey L. Zitsman , Meyer Kattan , Robert P. Garofano , Aimee M. Layton , Aliva De
{"title":"Cardiopulmonary exercise testing among pediatric patients with severe obesity prior to bariatric surgery","authors":"Joanna E. Nelson ,&nbsp;Kimberly M. Sanchez ,&nbsp;Yujing Yao ,&nbsp;Zhezhen Jin ,&nbsp;Jeffrey L. Zitsman ,&nbsp;Meyer Kattan ,&nbsp;Robert P. Garofano ,&nbsp;Aimee M. Layton ,&nbsp;Aliva De","doi":"10.1016/j.ppedcard.2024.101708","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101708","url":null,"abstract":"<div><h3>Background</h3><p>Cardiopulmonary exercise testing (CPET) is used prior to bariatric surgery in children with severe obesity to assess surgical risk factors. Lack of data for peak oxygen consumption (pVO<sub>2</sub>) for this population limits CPET interpretation and establishing surgical risk.</p></div><div><h3>Objectives</h3><p>We aimed to use lean tissue mass (LM) to better define pVO<sub>2</sub> and developed predictive equations for use in this cohort.</p></div><div><h3>Methods</h3><p>Participants with obesity age 9–20 years underwent CPET, using Bruce treadmill protocol, prior to bariatric surgery from January 1, 2006, to December 31, 2019. Bioelectrical impedance analysis (BIA) helped calculate LM from total body weight (TBW). Achieving peak heart rate ≥ 90 % predicted, RER of ≥1.1, and patient volition were considered satisfactory effort.</p></div><div><h3>Results</h3><p>Of tests performed by 446 participants, CPET studies (29 %) met inclusion criteria as peak exercise. For this group, body mass index was 45.2 ± 6.1 kg/m<sup>2</sup>, body fat % 49.7 ± 6.8, and LM 63 ± 10 kg. Mean pVO<sub>2</sub> was 22.2 ± 3.2 mL/kg/min, which was 51 % ± 7 % predicted when using conventional prediction methods. Mean pVO<sub>2</sub> using LM was 44.8 ± 8.7 mL/kg/min, which was 102.3 ± 17.6 % predicted. Linear regression yielded reference equations pVO<sub>2</sub> = 1571.6 + 12.2 ∗ TBW (males) and pVO2 = 1301.8 + 10.6 ∗ TBW (females).</p></div><div><h3>Conclusion</h3><p>This report is the largest dataset of pVO<sub>2</sub> in the pediatric population with severe obesity. Adolescents with severe obesity had normal pVO<sub>2</sub> when LM was applied rather than TBW. Given BIA or other methods of calculating LM are not readily available to all labs, a novel set of predictive pVO<sub>2</sub> equations using TBW was developed for labs to integrate into their CPET interpretations when evaluating youth with severe obesity prior to surgery.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"72 ","pages":"Article 101708"},"PeriodicalIF":0.9,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139710041","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","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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