{"id":"https://openalex.org/W2132145238","doi":"https://doi.org/10.1152/ajpheart.00479.2010","title":"Impaired cardiac reserve and severely diminished skeletal muscle O 2 utilization mediate exercise intolerance in Barth syndrome","display_name":"Impaired cardiac reserve and severely diminished skeletal muscle O 2 utilization mediate exercise intolerance in Barth syndrome","publication_year":2011,"publication_date":"2011-08-26","ids":{"openalex":"https://openalex.org/W2132145238","doi":"https://doi.org/10.1152/ajpheart.00479.2010","mag":"2132145238","pmid":"https://pubmed.ncbi.nlm.nih.gov/21873497"},"language":"en","primary_location":{"id":"doi:10.1152/ajpheart.00479.2010","doi":"https://doi.org/10.1152/ajpheart.00479.2010","is_oa":false,"landing_page_url":"https://doi.org/10.1152/ajpheart.00479.2010","pdf_url":null,"source":{"id":"https://openalex.org/S87338489","display_name":"American Journal of Physiology-Heart and Circulatory Physiology","issn_l":"0363-6135","issn":["0363-6135","1522-1539"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","dongbi-a","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320261","host_organization_name":"American Physical Society","host_organization_lineage":["https://openalex.org/P4310320261"],"host_organization_lineage_names":["American Physical Society"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"American Journal of Physiology-Heart and Circulatory Physiology","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5112673610","display_name":"Carolyn T. Spencer","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I186335123","display_name":"East Carolina University","ror":"https://ror.org/01vx35703","country_code":"US","type":"education","lineage":["https://openalex.org/I186335123"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Carolyn T. Spencer","raw_affiliation_strings":["Division of Cardiology, Department of Pediatrics, East Carolina University, Greenville, North Carolina;","Division of Cardiology, Department of Pediatrics, East Carolina University, Greenville, North Carolina, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Cardiology, Department of Pediatrics, East Carolina University, Greenville, North Carolina;","institution_ids":["https://openalex.org/I186335123"]},{"raw_affiliation_string":"Division of Cardiology, Department of Pediatrics, East Carolina University, Greenville, North Carolina, USA","institution_ids":["https://openalex.org/I186335123"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5072518895","display_name":"Barry J. Byrne","orcid":"https://orcid.org/0000-0002-7302-1756","observed_orcids":["https://orcid.org/0000-0002-7302-1756"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Barry J. Byrne","raw_affiliation_strings":["Congential Heart Center;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Congential Heart Center;","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5110404559","display_name":"Randall M. Bryant","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Randall M. Bryant","raw_affiliation_strings":["Congential Heart Center;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Congential Heart Center;","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5080840894","display_name":"Renee E. Margossian","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1288882113","display_name":"Boston Children's Hospital","ror":"https://ror.org/00dvg7y05","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1288882113"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Renee Margossian","raw_affiliation_strings":["Children's Hospital, Boston, Massachusetts;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Children's Hospital, Boston, Massachusetts;","institution_ids":["https://openalex.org/I1288882113"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5033747839","display_name":"Melissa K. Maisenbacher","orcid":"https://orcid.org/0000-0001-7907-2004","observed_orcids":["https://orcid.org/0000-0001-7907-2004"]},"institutions":[{"id":"https://openalex.org/I2800717037","display_name":"University of Florida Health","ror":"https://ror.org/04tk2gy88","country_code":"US","type":"education","lineage":["https://openalex.org/I2800717037","https://openalex.org/I33213144"]},{"id":"https://openalex.org/I33213144","display_name":"University of Florida","ror":"https://ror.org/02y3ad647","country_code":"US","type":"education","lineage":["https://openalex.org/I33213144"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Melissa Maisenbacher","raw_affiliation_strings":["Division of Genetics and Metabolism, Department of Pediatrics, University of Florida School of Medicine, Gainesville, Florida;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Genetics and Metabolism, Department of Pediatrics, University of Florida School of Medicine, Gainesville, Florida;","institution_ids":["https://openalex.org/I2800717037","https://openalex.org/I33213144"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5081149920","display_name":"Petar Breitenger","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Petar Breitenger","raw_affiliation_strings":["Congential Heart Center;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Congential Heart Center;","institution_ids":[]}]},{"author_position":"middle","author":{"id":null,"display_name":"Paul B. Benni","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Paul B. Benni","raw_affiliation_strings":["CAS Medical Systems Incorporated, Branford, Connecticut; and"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"CAS Medical Systems Incorporated, Branford, Connecticut; and","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5015761759","display_name":"Sharon Redfearn","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Sharon Redfearn","raw_affiliation_strings":["Congential Heart Center;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Congential Heart Center;","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5073734159","display_name":"Edward N. Marcus","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1288882113","display_name":"Boston Children's Hospital","ror":"https://ror.org/00dvg7y05","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1288882113"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Edward Marcus","raw_affiliation_strings":["Children's Hospital, Boston, Massachusetts;"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Children's Hospital, Boston, Massachusetts;","institution_ids":["https://openalex.org/I1288882113"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5090146098","display_name":"W. Todd Cade","orcid":"https://orcid.org/0000-0002-2329-7623","observed_orcids":["https://orcid.org/0000-0002-2329-7623"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"W. Todd Cade","raw_affiliation_strings":["Program in Physical Therapy and Department of Medicine, Washington University School of Medicine, St. Louis, Missouri"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Program in Physical Therapy and Department of Medicine, Washington University School of Medicine, St. Louis, Missouri","institution_ids":["https://openalex.org/I204465549"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":5,"corresponding_author_ids":["https://openalex.org/A5090146098"],"corresponding_institution_ids":["https://openalex.org/I204465549"],"apc_list":null,"apc_paid":null,"fwci":1.2972,"has_fulltext":false,"cited_by_count":88,"citation_normalized_percentile":{"value":0.79032077,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":94,"max":99},"biblio":{"volume":"301","issue":"5","first_page":"H2122","last_page":"H2129"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10301","display_name":"Mitochondrial Function and Pathology","score":0.5327000021934509,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T10301","display_name":"Mitochondrial Function and Pathology","score":0.5327000021934509,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11209","display_name":"Cardiovascular and exercise physiology","score":0.396699994802475,"subfield":{"id":"https://openalex.org/subfields/2707","display_name":"Complementary and alternative medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.02969999983906746,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/barth-syndrome","display_name":"Barth syndrome","score":1.0},{"id":"https://openalex.org/keywords/exercise-intolerance","display_name":"exercise intolerance","score":0.9430000185966492},{"id":"https://openalex.org/keywords/mitochondrial-myopathy","display_name":"mitochondrial myopathy","score":0.8180000185966492},{"id":"https://openalex.org/keywords/oxygen-extraction","display_name":"oxygen extraction","score":0.7419999837875366},{"id":"https://openalex.org/keywords/cardiac-reserve","display_name":"cardiac reserve","score":0.8930000066757202},{"id":"https://openalex.org/keywords/ejection-fraction","display_name":"ejection fraction","score":0.8579999804496765},{"id":"https://openalex.org/keywords/peak-oxygen-uptake","display_name":"peak oxygen uptake","score":0.7540000081062317},{"id":"https://openalex.org/keywords/muscle-oxygen-saturation","display_name":"muscle oxygen saturation","score":0.5410000085830688},{"id":"https://openalex.org/keywords/deoxyhemoglobin","display_name":"deoxyhemoglobin","score":0.9179999828338623},{"id":"https://openalex.org/keywords/near-infrared-spectroscopy","display_name":"near-infrared spectroscopy","score":0.7889999747276306},{"id":"https://openalex.org/keywords/cardiac-function","display_name":"cardiac function","score":0.3529999852180481}],"concepts":[{"id":"https://openalex.org/C2777321227","wikidata":"https://www.wikidata.org/wiki/Q5420062","display_name":"Exercise intolerance","level":3,"score":0.7891595363616943},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.7670297622680664},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.7261704206466675},{"id":"https://openalex.org/C2779959927","wikidata":"https://www.wikidata.org/wiki/Q1048687","display_name":"Skeletal muscle","level":2,"score":0.6336861252784729},{"id":"https://openalex.org/C2778198053","wikidata":"https://www.wikidata.org/wiki/Q181754","display_name":"Heart failure","level":2,"score":0.5974840521812439},{"id":"https://openalex.org/C164705383","wikidata":"https://www.wikidata.org/wiki/Q10379","display_name":"Cardiology","level":1,"score":0.5853574275970459},{"id":"https://openalex.org/C78085059","wikidata":"https://www.wikidata.org/wiki/Q641303","display_name":"Ejection fraction","level":3,"score":0.5344428420066833},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.441556841135025},{"id":"https://openalex.org/C133165541","wikidata":"https://www.wikidata.org/wiki/Q472094","display_name":"Exercise physiology","level":2,"score":0.43485406041145325},{"id":"https://openalex.org/C181588539","wikidata":"https://www.wikidata.org/wiki/Q219067","display_name":"Physical exercise","level":2,"score":0.41389143466949463}],"mesh":[{"descriptor_ui":"D000293","descriptor_name":"Adolescent","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000319","descriptor_name":"Adrenergic beta-Antagonists","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D000704","descriptor_name":"Analysis of Variance","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001794","descriptor_name":"Blood Pressure","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002311","descriptor_name":"Cardiomyopathy, Dilated","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":false},{"descriptor_ui":"D002311","descriptor_name":"Cardiomyopathy, Dilated","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":false},{"descriptor_ui":"D002311","descriptor_name":"Cardiomyopathy, Dilated","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":true},{"descriptor_ui":"D002311","descriptor_name":"Cardiomyopathy, Dilated","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D002311","descriptor_name":"Cardiomyopathy, Dilated","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":false},{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D003430","descriptor_name":"Cross-Sectional Studies","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004452","descriptor_name":"Echocardiography","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004562","descriptor_name":"Electrocardiography","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005080","descriptor_name":"Exercise Test","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006339","descriptor_name":"Heart Rate","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006454","descriptor_name":"Hemoglobins","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D009119","descriptor_name":"Muscle Contraction","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D010100","descriptor_name":"Oxygen","qualifier_ui":"Q000097","qualifier_name":"blood","is_major_topic":false},{"descriptor_ui":"D010100","descriptor_name":"Oxygen","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D010101","descriptor_name":"Oxygen Consumption","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D015415","descriptor_name":"Biomarkers","qualifier_ui":"Q000097","qualifier_name":"blood","is_major_topic":false},{"descriptor_ui":"D015656","descriptor_name":"Respiratory Mechanics","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D016022","descriptor_name":"Case-Control Studies","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D016277","descriptor_name":"Ventricular Function, Left","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D017079","descriptor_name":"Exercise Tolerance","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D019265","descriptor_name":"Spectroscopy, Near-Infrared","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D052097","descriptor_name":"Quadriceps Muscle","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D052097","descriptor_name":"Quadriceps Muscle","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":false},{"descriptor_ui":"D055815","descriptor_name":"Young Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D056889","descriptor_name":"Barth Syndrome","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":true},{"descriptor_ui":"D056889","descriptor_name":"Barth Syndrome","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":false},{"descriptor_ui":"D056889","descriptor_name":"Barth Syndrome","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D056889","descriptor_name":"Barth Syndrome","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":false}],"locations_count":2,"locations":[{"id":"doi:10.1152/ajpheart.00479.2010","doi":"https://doi.org/10.1152/ajpheart.00479.2010","is_oa":false,"landing_page_url":"https://doi.org/10.1152/ajpheart.00479.2010","pdf_url":null,"source":{"id":"https://openalex.org/S87338489","display_name":"American Journal of Physiology-Heart and Circulatory Physiology","issn_l":"0363-6135","issn":["0363-6135","1522-1539"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","dongbi-a","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320261","host_organization_name":"American Physical Society","host_organization_lineage":["https://openalex.org/P4310320261"],"host_organization_lineage_names":["American Physical Society"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"American Journal of Physiology-Heart and Circulatory Physiology","raw_type":"journal-article"},{"id":"pmid:21873497","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/21873497","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"American journal of physiology. Heart and circulatory physiology","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.9537000060081482,"display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/observational-study","display_name":"Observational Study"}],"awards":[],"funders":[{"id":"https://openalex.org/F4320307186","display_name":"Barth Syndrome Foundation","ror":"https://ror.org/00p5z3m34"}],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":48,"referenced_works":["https://openalex.org/W1487623199","https://openalex.org/W1515514301","https://openalex.org/W1524830325","https://openalex.org/W1535969389","https://openalex.org/W1551009226","https://openalex.org/W1962028868","https://openalex.org/W1963698855","https://openalex.org/W1968617084","https://openalex.org/W1972172777","https://openalex.org/W1974145389","https://openalex.org/W1978977765","https://openalex.org/W1991550546","https://openalex.org/W2001425543","https://openalex.org/W2002950502","https://openalex.org/W2004693594","https://openalex.org/W2007318429","https://openalex.org/W2011550515","https://openalex.org/W2012168559","https://openalex.org/W2017458348","https://openalex.org/W2019524569","https://openalex.org/W2027122450","https://openalex.org/W2027253641","https://openalex.org/W2029777615","https://openalex.org/W2033072021","https://openalex.org/W2036827645","https://openalex.org/W2037361684","https://openalex.org/W2044756377","https://openalex.org/W2057092244","https://openalex.org/W2074179637","https://openalex.org/W2077637487","https://openalex.org/W2078789786","https://openalex.org/W2080197336","https://openalex.org/W2089437414","https://openalex.org/W2099103099","https://openalex.org/W2113426999","https://openalex.org/W2114510322","https://openalex.org/W2115238669","https://openalex.org/W2119250250","https://openalex.org/W2132084610","https://openalex.org/W2144243947","https://openalex.org/W2145139856","https://openalex.org/W2147739814","https://openalex.org/W2147868953","https://openalex.org/W2152354140","https://openalex.org/W2165361329","https://openalex.org/W2170693788","https://openalex.org/W2171013289","https://openalex.org/W4290422017"],"related_works":["https://openalex.org/W1595465629","https://openalex.org/W2587859487","https://openalex.org/W4412094193","https://openalex.org/W2424939044","https://openalex.org/W1712277796","https://openalex.org/W2988417392","https://openalex.org/W2934602283","https://openalex.org/W2095666202","https://openalex.org/W3157555905","https://openalex.org/W2738100013"],"abstract_inverted_index":{"Barth":[0],"syndrome":[1],"(BTHS)":[2],"is":[3,34,242],"a":[4,72],"mitochondrial":[5,258],"myopathy":[6],"characterized":[7],"by":[8],"reports":[9],"of":[10,23,92,102,268],"exercise":[11,30,69,154,188,238],"intolerance.":[12],"We":[13],"sought":[14],"to":[15,21,36,152,186,244,265],"determine":[16],"if":[17],"1)":[18],"BTHS":[19,33,47,143,173,223,241],"leads":[20],"abnormalities":[22],"skeletal":[24,103,230,248,257],"muscle":[25,96,104,179,231,249],"O(2)":[26,38,105,111,181],"extraction/utilization":[27],"and":[28,56,78,86,124,204,247,256],"2)":[29],"intolerance":[31,239],"in":[32,142,155,172,222,233,240],"related":[35],"impaired":[37,40,229],"extraction/utilization,":[39],"cardiac":[41,246,255],"function,":[42],"or":[43],"both.":[44],"Participants":[45],"with":[46,75,175,225,254],"(age:":[48,59],"17":[49],"\u00b1":[50,61,114,118,129,133,160,165,194,199,212,217],"5":[51],"yr,":[52,63],"n":[53,64],"=":[54,65],"15)":[55],"control":[57,145,176,226],"participants":[58],"13":[60],"4":[62],"9)":[66],"underwent":[67],"graded":[68],"testing":[70],"on":[71],"cycle":[73],"ergometer":[74],"continuous":[76],"ECG":[77],"metabolic":[79],"measurements.":[80],"Echocardiography":[81],"was":[82,97,170,189,208],"performed":[83],"at":[84,87],"rest":[85,151,185],"peak":[88,110,125,153,187],"exercise.":[89],"Near-infrared":[90],"spectroscopy":[91],"the":[93,205,266],"vastus":[94],"lateralis":[95],"continuously":[98],"recorded":[99],"for":[100,108],"measurements":[101],"extraction.":[106],"Adjusting":[107],"age,":[109],"consumption":[112],"(16.5":[113],"4.0":[115],"vs.":[116,131,162,196,214],"39.5":[117],"12.3":[119],"ml\u00b7kg(-1)\u00b7min(-1),":[120],"P":[121,136,167,201,219],"<":[122,137,168,202,220],"0.001)":[123,138],"work":[126],"rate":[127],"(58":[128],"19":[130,164],"166":[132],"60":[134],"W,":[135],"were":[139],"significantly":[140],"lower":[141],"than":[144],"participants.":[146,177],"The":[147,178],"percent":[148],"increase":[149],"from":[150,184],"ejection":[156],"fraction":[157],"(BTHS:":[158,192,210],"3":[159],"10":[161,216],"control:":[163,197,215],"4%,":[166],"0.01)":[169],"blunted":[171,209],"compared":[174,224],"tissue":[180],"saturation":[182],"change":[183,207],"paradoxically":[190],"opposite":[191],"8":[193],"16":[195],"-5":[198],"9,":[200],"0.01),":[203],"deoxyhemoglobin":[206],"0":[211],"12":[213],"8,":[218],"0.09)":[221],"participants,":[227],"indicating":[228],"extraction":[232],"BTHS.":[234,269],"In":[235],"conclusion,":[236],"severe":[237],"due":[243],"both":[245],"impairments":[250],"that":[251],"are":[252],"consistent":[253],"myopathy.":[259],"These":[260],"findings":[261],"provide":[262],"further":[263],"insight":[264],"pathophysiology":[267]},"counts_by_year":[{"year":2026,"cited_by_count":2},{"year":2025,"cited_by_count":6},{"year":2024,"cited_by_count":4},{"year":2023,"cited_by_count":7},{"year":2022,"cited_by_count":4},{"year":2021,"cited_by_count":11},{"year":2020,"cited_by_count":5},{"year":2019,"cited_by_count":12},{"year":2018,"cited_by_count":7},{"year":2017,"cited_by_count":9},{"year":2016,"cited_by_count":6},{"year":2015,"cited_by_count":5},{"year":2014,"cited_by_count":4},{"year":2013,"cited_by_count":4},{"year":2012,"cited_by_count":2}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2016-06-24T00:00:00"}