title: Cardiovascular abnormalities in Taiwanese patients with mucopolysaccharidosis.
abstract: BACKGROUND
  The mucopolysaccharidoses (MPS) are a group of rare inherited metabolic diseases that can cause damages in various organs including the heart. This study aimed to review the medical records of Taiwanese patients with MPS in order to evaluate the cardiovascular involvement in those patients.
  
  
  METHODS
  From 2000 to 2012, the medical records of 60 patients with MPS in a tertiary medical center in Taiwan were retrospectively reviewed. Data on cardiac measurements and functions were obtained from previously performed echocardiograms and electrocardiograms. Cardiac parameters were analyzed according to MPS types and patients' age.
  
  
  RESULTS
  The most frequent MPS type was type II (43%). Overall, heart conditions such as thick interventricular septum (55%), asymmetric septal hypertrophy (42%) and mitral valve prolapse (33%) were common, while cardiac enlargement was infrequently seen. Valvular stenosis/regurgitation and cardiac hypertrophy were more common in patients with MPS I, II, and VI when compared with other MPS types. Cardiovascular abnormalities including valvular deformation and thickening, thick interventricular septum and diastolic dysfunction were found to progress with age.
  
  
  CONCLUSIONS
  The anatomical changes of cardiovascular systems were common in all types of MPS patients, especially in MPS I, II, and VI. Echocardiography and electrocardiography can provide us good tools for early detection and long-term follow-up for these patients.
authors.affiliation: Department of Medicine, Mackay Medical College, New Taipei City, Taiwan; Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan; Mackay Junior College of Medicine, Nursing and Management, Taipei, Taiwan.
authors.forename: Shan-Miao
authors.initials: SM
authors.lastname: Lin
authors.affiliation: Department of Medicine, Mackay Medical College, New Taipei City, Taiwan; Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan; Mackay Junior College of Medicine, Nursing and Management, Taipei, Taiwan; Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan.
authors.forename: Hsiang-Yu
authors.initials: HY
authors.lastname: Lin
authors.affiliation: Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan; Medical College, Fu-Jen Catholic University, Taipei, Taiwan.
authors.forename: Chih-Kuang
authors.initials: CK
authors.lastname: Chuang
authors.affiliation: Department of Medicine, Mackay Medical College, New Taipei City, Taiwan; Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan; Mackay Junior College of Medicine, Nursing and Management, Taipei, Taiwan; Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan; Department of Infant and Child Care, National Taipei University of Nursing and Health Sciences, Taipei, Taiwan. Electronic address: zsplin14@gmail.com.
authors.forename: Shuan-Pei
authors.initials: SP
authors.lastname: Lin
authors.affiliation: Department of Medicine, Mackay Medical College, New Taipei City, Taiwan; Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan; Mackay Junior College of Medicine, Nursing and Management, Taipei, Taiwan. Electronic address: mingren44@gmail.com.
authors.forename: Ming-Ren
authors.initials: MR
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D018376:Cardiovascular Abnormalities / Q000150:complications* / Q000000981:diagnostic imaging; D002648:Child; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications* / Q000000981:diagnostic imaging; D010808:Physical Examination; D013624:Taiwan; D014463:Ultrasonography; D055815:Young Adult
keywords: Cardiovascular; Echocardiography; Electrocardiography; Mucopolysaccharidosis; Taiwan