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J Thorac Cardiovasc Surg 2006;131:471-473
© 2006 The American Association for Thoracic Surgery
Brief Communication |
Department of Paediatric Cardiac Surgery, Diana, Princess of Wales Children's Hospital, Birmingham, United Kingdom.
Received for publication June 20, 2005; revisions received September 19, 2005; accepted for publication September 27, 2005. * Address for reprints: W. J. Brawn, FRCS, FRACS, Department of Paediatric Cardiac Surgery, Diana, Princess of Wales Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK (Email: william.brawn@bch.nhs.uk).
| The first 20% of the full text of this article appears below. |
After relief of obstructive lesions in Shone syndrome, left ventricular dysfunction might persist, with high left ventricular end-diastolic pressure (LVEDP) and pulmonary hypertension (PHT).
1
We reasoned that the right ventricle (RV), having been trained by means of PHT, could support the systemic circulation. The left ventricle (LV), although dysfunctional in the systemic circulation, would be adequate to support the pulmonary circulation. With this arrangement, PHT might resolve. This was achieved in 2 patients with the combined atrial and arterial switch procedure (double-switch procedure), which is usually used in congenitally corrected transposition.
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Clinical Summary
Patient 1
An 18-month-old boy (7.5 kg) with Shone syndrome and previous coarctation and parachute mitral valve repair presented with failure to thrive and PHT. His aortic valve was bicuspid. Preoperative evaluation showed severe mitral regurgitation and a nonapex-forming LV with endomyocardial fibroelastosis, although its end-diastolic diameter was normal (25 mm; predicted range, 21-31 mm). LVEDP was 17 mm Hg. Pulmonary artery (PA) pressures were 120/70 mm Hg (mean, 85 mm Hg), decreasing to 65/50
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