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Article: Myocardial structural alteration and systolic dysfunction in preclinical hypertrophic cardiomyopathy mutation carriers

TitleMyocardial structural alteration and systolic dysfunction in preclinical hypertrophic cardiomyopathy mutation carriers
Authors
KeywordsCalibration
Cardiovascular risk
Coronary artery disease
Diabetes mellitus
Electrocardiogram
Issue Date2012
PublisherPublic Library of Science. The Journal's web site is located at http://www.plosone.org/home.action
Citation
PLoS One, 2012, v. 7 n. 5, article no. e36115 How to Cite?
AbstractBACKGROUND: To evaluate the presence of myocardial structural alterations and subtle myocardial dysfunction during familial screening in asymptomatic mutation carriers without hypertrophic cardiomyopathy (HCM) phenotype. METHODS AND FINDINGS: Sixteen HCM families with pathogenic mutation were studied and 46 patients with phenotype expression (Mut+/Phen+) and 47 patients without phenotype expression (Mut+/Phen-) were observed. Twenty-five control subjects, matched with the Mut+/Phen- group, were recruited for comparison. Echocardiography was performed to evaluate conventional parameters, myocardial structural alteration by calibrated integrated backscatter (cIBS) and global and segmental longitudinal strain by speckle tracking analysis. All 3 groups had similar left ventricular dimensions and ejection fraction. Basal anteroseptal cIBS was the highest in Mut+/Phen+ patients (-14.0+/-4.6 dB, p<0.01) and was higher in Mut+/Phen- patients as compared to controls (-17.0+/-2.3 vs. -22.6+/-2.9 dB, p<0.01) suggesting significant myocardial structural alterations. Global and basal anteroseptal longitudinal strains (-8.4+/-4.0%, p<0.01) were the most impaired in Mut+/Phen+ patients as compared to the other 2 groups. Although global longitudinal strain was similar between Mut+/Phen- group and controls, basal anteroseptal strain was lower in Mut+/Phen- patients (-14.1+/-3.8%, p<0.01) as compared to controls (-19.9+/-2.9%, p<0.01), suggesting a subclinical segmental systolic dysfunction. A combination of >-19.0 dB basal anteroseptal cIBS or >-18.0% basal anteroseptal longitudinal strain had a sensitivity of 98% and a specificity of 72% in differentiating Mut+/Phen- group from controls. CONCLUSION: The use of cIBS and segmental longitudinal strain can differentiate HCM Mut+/Phen- patients from controls with important clinical implications for the family screening and follow-up of these patients.
Persistent Identifierhttp://hdl.handle.net/10722/159657
ISSN
2015 Impact Factor: 3.057
2015 SCImago Journal Rankings: 1.395
PubMed Central ID
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorYiu, KHen_US
dc.contributor.authorAtsma, DEen_US
dc.contributor.authorDelgado, Ven_US
dc.contributor.authorNg, ACen_US
dc.contributor.authorWitkowski, TGen_US
dc.contributor.authorEwe, SHen_US
dc.contributor.authorAuger, Den_US
dc.contributor.authorHolman, ERen_US
dc.contributor.authorvan Mil, AMen_US
dc.contributor.authorBreuning, MHen_US
dc.contributor.authorTse, HFen_US
dc.contributor.authorBax, JJen_US
dc.contributor.authorSchalij, MJen_US
dc.contributor.authorMarsan, NAen_US
dc.date.accessioned2012-08-16T05:53:48Z-
dc.date.available2012-08-16T05:53:48Z-
dc.date.issued2012en_US
dc.identifier.citationPLoS One, 2012, v. 7 n. 5, article no. e36115en_US
dc.identifier.issn1932-6203-
dc.identifier.urihttp://hdl.handle.net/10722/159657-
dc.description.abstractBACKGROUND: To evaluate the presence of myocardial structural alterations and subtle myocardial dysfunction during familial screening in asymptomatic mutation carriers without hypertrophic cardiomyopathy (HCM) phenotype. METHODS AND FINDINGS: Sixteen HCM families with pathogenic mutation were studied and 46 patients with phenotype expression (Mut+/Phen+) and 47 patients without phenotype expression (Mut+/Phen-) were observed. Twenty-five control subjects, matched with the Mut+/Phen- group, were recruited for comparison. Echocardiography was performed to evaluate conventional parameters, myocardial structural alteration by calibrated integrated backscatter (cIBS) and global and segmental longitudinal strain by speckle tracking analysis. All 3 groups had similar left ventricular dimensions and ejection fraction. Basal anteroseptal cIBS was the highest in Mut+/Phen+ patients (-14.0+/-4.6 dB, p<0.01) and was higher in Mut+/Phen- patients as compared to controls (-17.0+/-2.3 vs. -22.6+/-2.9 dB, p<0.01) suggesting significant myocardial structural alterations. Global and basal anteroseptal longitudinal strains (-8.4+/-4.0%, p<0.01) were the most impaired in Mut+/Phen+ patients as compared to the other 2 groups. Although global longitudinal strain was similar between Mut+/Phen- group and controls, basal anteroseptal strain was lower in Mut+/Phen- patients (-14.1+/-3.8%, p<0.01) as compared to controls (-19.9+/-2.9%, p<0.01), suggesting a subclinical segmental systolic dysfunction. A combination of >-19.0 dB basal anteroseptal cIBS or >-18.0% basal anteroseptal longitudinal strain had a sensitivity of 98% and a specificity of 72% in differentiating Mut+/Phen- group from controls. CONCLUSION: The use of cIBS and segmental longitudinal strain can differentiate HCM Mut+/Phen- patients from controls with important clinical implications for the family screening and follow-up of these patients.-
dc.languageengen_US
dc.publisherPublic Library of Science. The Journal's web site is located at http://www.plosone.org/home.action-
dc.relation.ispartofPLoS Oneen_US
dc.rightsCreative Commons: Attribution 3.0 Hong Kong License-
dc.subjectCalibration-
dc.subjectCardiovascular risk-
dc.subjectCoronary artery disease-
dc.subjectDiabetes mellitus-
dc.subjectElectrocardiogram-
dc.titleMyocardial structural alteration and systolic dysfunction in preclinical hypertrophic cardiomyopathy mutation carriersen_US
dc.typeArticleen_US
dc.identifier.emailYiu, KH: khkyiu@hku.hken_US
dc.identifier.emailTse, HF: hftse@hkucc.hku.hken_US
dc.identifier.authorityYiu, KH=rp01490en_US
dc.identifier.authorityTse, HF=rp00428en_US
dc.description.naturepublished_or_final_version-
dc.identifier.doi10.1371/journal.pone.0036115-
dc.identifier.pmid22574137-
dc.identifier.pmcidPMC3344846-
dc.identifier.scopuseid_2-s2.0-84860524301-
dc.identifier.hkuros203997en_US
dc.identifier.volume7en_US
dc.identifier.issue5, article no. e36115-
dc.identifier.isiWOS:000305349800033-
dc.publisher.placeUnited States-

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