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J Korean Pediatr Cardiol Soc. 2006 Dec;10(4):426-435. Korean. Original Article.
Kim SH , Huh J , Kang IS , Lee HJ , Park SW .
Department of Pediatric Cardiology, Sejong General Hospital, Bucheon, Korea.
Department of Pediatrics, Sungkyunkwan University School of Medicine, Seoul, Korea. herzhuh@smc.samsung.co.kr
Department of Internal Medicine, Sungkyunkwan University School of Medicine, Seoul, Korea.
Abstract

PURPOSE: In patients with the congenitally corrected transposition of great arteries (CCTGA), systemic ventricle tends to be hypertrophic due to longstanding systemic high pressure that may lead to dysfunction of ventricle. The aim of this study was to assess the global and segmental wall function of systemic right ventricle in CCTGA. METHODS: Twelve patients with the CCTGA patients were enrolled. Ejection fraction of systemic right ventricle, myocardial performance index (MPI), strain and strain rate were assessed. RESULTS: The median age was 32.5 years with a range of 4 to 66 years. Ejection fraction (EF) using M-mode and Simpsons method was significantly lower in the CCTGA patients (48.5+/-4.6% and 51.2+/-4.1%, respectively, vs 63.7+/-3.6% and 66.6+/-3.8% respectively). 5 patients had diastolic dysfunction (3/12; pseudo-normalization pattern, 2/12; relaxation abnormality). Strain and strain rate revealed segmental systemic right ventricle dysfunction, and were lower in the CCTGA patients at mid and apical segment of septum and apical segment of anterior wall. The extent of dysfunctional segment was related to the age and MPI. CONCLUSION: There was global dysfunction in the CCTGA patients and it was related with age. Segmental dysfunction was especially shown at mid and apical segment of septum and apical segment of anterior wall and more frequently at apical segment of each wall. The extent of dysfunctional segment was related with age.

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