CJC Pediatric and Congenital Heart Disease最新文献

{"title":"Exercise Pathophysiology and Testing in Individuals With a Fontan Circulation","authors":"Arjun K. Mahendran MD,&nbsp;David Katz MD,&nbsp;Alexander R. Opotowsky MD, MMSc,&nbsp;Adam M. Lubert MD","doi":"10.1016/j.cjcpc.2023.01.001","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2023.01.001","url":null,"abstract":"<div><p>The Fontan circulation, a surgical palliation for single-ventricle congenital heart disease, profoundly impacts the cardiopulmonary response to exercise. Reliant on passive pulmonary blood flow, the Fontan circulation has limited capacity to augment cardiac output as necessary to supply working muscles during exercise. Cardiopulmonary exercise testing (CPET) objectively assesses cardiorespiratory fitness and provides insight into the etiology of exercise intolerance. Furthermore, CPET variables, such as peak oxygen consumption and submaximal variables, have prognostic value and may be used as meaningful endpoints in research studies. CPET is also useful in clinical research applications to assess the effect of pharmacologic or other interventions. Medical therapies to improve exercise tolerance in individuals with a Fontan circulation, such as pulmonary vasodilators, may modestly improve peak oxygen consumption. Exercise training focused on aerobic fitness and lower extremity strength may have a more consistent and larger impact on these measures of aerobic fitness. CPET is a valuable diagnostic and prognostic tool for those with a Fontan circulation. Newer ancillary assessments, such as noninvasive peripheral venous pressure monitoring and cardiac output measurements, hold promise to provide a more nuanced insight into the underlying pathophysiology.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49751422","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}

{"title":"Successful Treatment of Pulmonary Artery Aneurysm With Atrioventricular Septal Defect and Pulmonary Hypertension: Clinical and Pathologic Features","authors":"Naofumi F. Sumitomo MD, PhD ,&nbsp;Kazuhiro Yamanoi MD, PhD ,&nbsp;Sho Akiyama MD ,&nbsp;Naritaka Kimura MD, PhD ,&nbsp;Kazuki Kodo MD, PhD ,&nbsp;Hiroyuki Yamagishi MD, PhD","doi":"10.1016/j.cjcpc.2023.02.001","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2023.02.001","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49751426","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}

{"title":"Determining Research Priorities With Teen and Adult Congenital Heart Disease Patients: A Mixed-Methods Study","authors":"Michelle Keir MD, MSc ,&nbsp;Cheryl Tarr BScN ,&nbsp;Chanda McFadden MSW, RSW ,&nbsp;Glenda Durupt RN, BN ,&nbsp;Lori Newman BN ,&nbsp;Yvonne Balon BN, MN ,&nbsp;Timothy Prieur MD ,&nbsp;David J. Patton MD ,&nbsp;Jessica Jenkins BN, NP ,&nbsp;Nanette Alvarez MD ,&nbsp;Jillian Colbert MD ,&nbsp;Namrata Guron MD ,&nbsp;Stephen Reynolds MD, MSc ,&nbsp;Kim Myers MD","doi":"10.1016/j.cjcpc.2022.12.002","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2022.12.002","url":null,"abstract":"<div><h3>Background</h3><p>Children with congenital heart disease (CHD) are living longer than ever before. This growing cohort of adults with CHD has high medical and psychosocial needs. Also, patients and advocacy groups are justifiably demanding that their voices be heard in all phases of clinical and health services research.</p></div><div><h3>Methods</h3><p>We conducted a first of its kind research priority–setting exercise with teens and adults with moderate-to-complex CHD. Focus groups were held using a fixed, mixed methods, exploratory sequential design. Objectives were to include the patient voice in all phases of the research process, determine the key needs of patients living with CHD, to guide health services research, and identify the “top 10” research priorities of teens and adults living with CHD.</p></div><div><h3>Results</h3><p>Thirty-five patients participated in one of nine 3-hour focus groups where they shared their experiences living with CHD. They expressed a desire for connection with others living with CHD and altruistic motives for participating. Patients with CHD identified a need for information about their disease and prognosis, a need for connection through physical activity and mentorship programmes, and a need for advanced communication with health care teams. Qualitative results correlated well with quantitative ratings to create a patient-derived “top 10” research priorities list.</p></div><div><h3>Conclusions</h3><p>Patients affected by a chronic disease like CHD want to be included in all phases of research. Our research priority–setting exercise in teens and adults with CHD has created a roadmap for clinicians and researchers to investigate issues most important to those living with CHD.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49726759","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}

{"title":"Unexplained Cardiovascular Collapse After Scoliosis Surgery in a Patient With Loeys-Dietz Syndrome","authors":"Abdulrahman Al Ghamdi MD ,&nbsp;Marina Mir Parramón MD ,&nbsp;Tíscar Cavallé-Garrido MD, FRCPC","doi":"10.1016/j.cjcpc.2022.11.004","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2022.11.004","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49726368","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}

{"title":"Clinical Characteristics and Outcomes in Adults With Moderate-to-Severe Complexity Congenital Heart Disease Undergoing Palliation or Surgical Repair","authors":"Edgar García-Cruz MD ,&nbsp;Daniel Manzur-Sandoval MD ,&nbsp;Rodrigo Gopar-Nieto MD ,&nbsp;Stephanie Teresa Angulo-Cruzado MD ,&nbsp;Sheila Vania Sánchez-López MD ,&nbsp;José Miguel Torres-Martel MD ,&nbsp;Samuel Ramírez-Marroquín MD ,&nbsp;Jorge Luis Cervantes-Salazar MD ,&nbsp;Antonio Benita-Bordes MD ,&nbsp;Juan Calderon-Colmenero MD ,&nbsp;José Antonio García-Montes MD ,&nbsp;Linda Guieniza Díaz-Gallardo MD ,&nbsp;Isis Guadalupe Montalvo-Ocotoxtle MD ,&nbsp;Lizeth Estefanía Escobar-Sibaja MD ,&nbsp;Cristopher Candido Sánchez-Rodríguez MD ,&nbsp;Ricardo Leopoldo Barajas-Campos MD ,&nbsp;Juan Carlos García-Cruz MD ,&nbsp;Montserrat Villalobos-Pedroza MD ,&nbsp;Jorge Sánchez-Nieto MD ,&nbsp;Elisa Mier y Terán-Morales MD ,&nbsp;Francisco Martín Baranda-Tovar MD","doi":"10.1016/j.cjcpc.2022.10.005","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2022.10.005","url":null,"abstract":"<div><h3>Background</h3><p>Congenital heart disease (CHD) survival rate has improved dramatically due to advances in diagnostic and therapeutic techniques. However, concerning the unrepaired CHD population of moderate and severe complexity, the data regarding risk predictors and surgical outcomes are scarce. Our aim was to describe the surgical results and predictors of in-hospital outcomes in adult patients with moderate-to-severe complexity CHD that were not repaired in childhood.</p></div><div><h3>Methods</h3><p>We conducted a retrospective cohort study that included 49 adult patients with moderate-to-complex CHD who were treated in a single medical centre. Clinical and echocardiographic variables were obtained on admission, after surgical procedures and during follow-up.</p></div><div><h3>Results</h3><p>Most of the patients were female (66%). Left ventricular ejection fraction and right ventricular outflow tract fractional shortening were within the normal range. The median pulmonary artery systolic pressure was 37 (27-55) mm Hg. The median time was 118 (80–181) minutes for extracorporeal circulation and 76 (49-121) minutes for aortic cross-clamping. The most frequent complication was postoperative complete atrioventricular block (12.2%). In-hospital survival rate was 87.7%. The development of low cardiac output syndrome with predominant right ventricle failure in the postoperative period was the most important predictor of in-hospital death (<em>P</em> = 0.03).</p></div><div><h3>Conclusions</h3><p>Deciding to treat adults with CHD is challenging in moderate and severe unrepaired cases. Adequate clinical, functional, and imaging evaluation is essential to determine each patient’s suitability for surgical management and to achieve the best clinical outcome for this population.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49762624","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}

{"title":"Information for Readers","authors":"","doi":"10.1016/S2772-8129(23)00041-6","DOIUrl":"https://doi.org/10.1016/S2772-8129(23)00041-6","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49726845","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}

{"title":"Call for Original Papers: Special Issue on Tetralogy of Fallot Across the Lifespan","authors":"","doi":"10.1016/S2772-8129(23)00067-2","DOIUrl":"https://doi.org/10.1016/S2772-8129(23)00067-2","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49738671","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}

{"title":"Left Cardiac Sympathetic Denervation as an Acute Treatment of Torsades in a Paediatric Case of Long QT","authors":"Jennifer Shortland MBBCH, MRCPCH ,&nbsp;Diego Marquez MD ,&nbsp;Osman Al-Radi MD, FRCSC ,&nbsp;Shubhayan Sanatani MD, FRCPC","doi":"10.1016/j.cjcpc.2022.10.008","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2022.10.008","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49726818","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}

{"title":"Cut Left or Left Alone, What to Do in the Middle of a Storm!","authors":"Joseph Atallah MD CM, SM","doi":"10.1016/j.cjcpc.2022.11.006","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2022.11.006","url":null,"abstract":"","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49726784","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}

{"title":"The Ross Operation in Young Patients: A 15-Year Experience Focused on Right Ventricle to Pulmonary Artery Conduit Outcomes","authors":"Nabil Dib MD, MSc ,&nbsp;Xavier Iriart MD ,&nbsp;Yaniss Belaroussi MD, MSc ,&nbsp;Waleed Albadi MD ,&nbsp;Nadir Tafer MD ,&nbsp;Jean-Benoit Thambo MD, PhD ,&nbsp;Paul Khairy MD, PhD ,&nbsp;François Roubertie MD, PhD","doi":"10.1016/j.cjcpc.2022.12.003","DOIUrl":"https://doi.org/10.1016/j.cjcpc.2022.12.003","url":null,"abstract":"<div><h3>Background</h3><p>Data on long-term outcomes of the Ross operation in children and young adult patients are limited. The best pulmonary valve substitute for the right ventricular outflow tract reconstruction remains uncertain. This study aimed to assess the outcomes of right ventricular outflow tract reconstruction in the Ross operation in young patients using various pulmonary valve substitutes at a single institution. In addition, a comparison of reintervention rates between patients younger than 18 years and those older than 18 years was performed.</p></div><div><h3>Methods</h3><p>The study assessed all patients (N = 110) who underwent the Ross operation at the University Hospital of Bordeaux, France, between 2004 and 2020.</p></div><div><h3>Results</h3><p>The median follow-up time was 4.2 years, and the median age at operation was 15.9 years. There was no operative mortality and 1 late noncardiac death (0.8%). The overall survival rate at 10 years was 99.2%. The need for right ventricular outflow tract reoperation was lower with the pulmonary homograft compared with the Contegra conduit and Freestyle bioprosthesis: 94.3%, 93.8%, and 80% at 5 years, respectively, and 94.3%, 72.3%, and 34.3% at 10 years, respectively (<em>P</em> = 0.011). The probability of reintervention was not significantly different at 10 years among children vs adults (<em>P</em> = 0.22).</p></div><div><h3>Conclusions</h3><p>The Ross procedure in children and young adults was associated with a lower requirement for right ventricular outflow tract reoperation when pulmonary homografts were used instead of xenografts.</p></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49738666","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}