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J Thorac Cardiovasc Surg 2008;136:1095-1097
© 2008 The American Association for Thoracic Surgery

Brief Communication

Mitral annuloplasty in an infant with Barth syndrome and severe mitral insufficiency: First case report and determination of annular diameter

^a Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan
^b Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
^c Department of Genetics, National Taiwan University Hospital, Taipei, Taiwan

Received for publication November 25, 2007; accepted for publication January 17, 2008.

^_* Address for reprints: Shoei-Shen Wang, MD, PhD, Department of Surgery, National Taiwan University Hospital, 7 Chung-Shan S Rd, Taipei 100, Taiwan. (Email: sswang@ha.mc.ntu.edu.tw).

The first 20% of the full text of this article appears below.

Barth syndrome, an X-linked recessive disorder, is caused by mutations in the taffazin gene (TAZ) at Xq28, leading to severe cardiolipin deficiency in the mitochondrial membrane.¹ The most common presentation is cardiomyopathy in infancy, including left ventricular (LV) dilation, hypertrophy, and noncompaction, with congestive heart failure.

In the case of refractory heart failure, a heart transplant is considered to be required.² We performed a successful mitral valve repair on an infant with Barth syndrome with a presentation of cardiogenic shock. We describe the techniques and consideration for mitral valve repair for this infant.

Clinical Summary

An 11-month-old boy had Barth syndrome, which had been diagnosed during his early infancy. Echocardiography revealed LV noncompaction with hypertrophy and impaired systolic function (LV ejection fraction 36%) with severe mitral regurgitation (MR). He was treated with furosemide, captopril, and aspirin. He was also on the waiting list for a heart transplant.

The patient was admitted to the intensive care unit with the impression of cardiogenic shock. Echocardiography showed severe MR with dilated left atrium and LV ( Figure 1, A). A short period of cardiac massage was . . . [Full Text of this Article]