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Left ventricular non-compaction cardiomyopathy with coronary artery anomaly complicated by ventricular tachycardia
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Medicinska och farmaceutiska vetenskapsområdet, centrumbildningar mm, Centre for Research and Development, Gävleborg.
Kiruna Sjukhus, Dept Med, SE-98128 Kiruna, Sweden..
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Medicinska och farmaceutiska vetenskapsområdet, centrumbildningar mm, Centre for Research and Development, Gävleborg.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Medicinska och farmaceutiska vetenskapsområdet, centrumbildningar mm, Centre for Research and Development, Gävleborg.
2017 (English)In: BMC Cardiovascular Disorders, ISSN 1471-2261, E-ISSN 1471-2261, Vol. 17, article id 263Article in journal (Refereed) Published
Abstract [en]

Background: Non-compaction cardiomyopathy (NCCM) is characterized by prominent trabeculations, deep intertrabecular recesses, and a thick non-compacted endocardial myocardium. Prevalence in the general population remains unclear, but echocardiography series report 0.05%. During fetal development muscle fibers and trabeculae should compact into a solid myocardium and when this fails, NCCM occurs. The condition is genetic, even though acquired forms have been described. Worsening myocardial dysfunction may lead to heart failure and/or arrhythmias. Case presentation: A 52-year-old man presented with heart failure. The diagnosis of NCCM was confirmed after echocardiography and cardiac magnetic resonance tomography. Interestingly, the angiogram revealed a coronary anomaly, in which the circumflex artery rose aberrantly from the right coronary artery. Due to left ventricular ejection fraction being less than 35% despite optimal pharmacological therapy, an implantable cardioverter defibrillator (ICD) was implanted and four years later a ventricular tachycardia was terminated by antitachycardia pacing. Conclusion: We describe a case of NCCM with a concomitant coronary anomaly, in which systolic myocardial dysfunction developed. The ICD subsequently terminated a life-threatening ventricular arrhythmia, which supports risk stratification based on low ejection fraction and possibly coronary anomaly.

Place, publisher, year, edition, pages
BIOMED CENTRAL LTD , 2017. Vol. 17, article id 263
Keywords [en]
Cardiac imaging, Cardiac magnetic resonance, Cardiomyopathy, Coronary artery anomaly, Echocardiography, Heart failure, implantable cardioverter defibrillator, Non-compaction cardiomyopathy, Sudden cardiac death, Ventricular tachycardia
National Category
Cardiac and Cardiovascular Systems
Identifiers
URN: urn:nbn:se:uu:diva-340130DOI: 10.1186/s12872-017-0699-7ISI: 000413276600002PubMedID: 29037169OAI: oai:DiVA.org:uu-340130DiVA, id: diva2:1178470
Available from: 2018-01-29 Created: 2018-01-29 Last updated: 2018-01-29Bibliographically approved

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