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a Division of Cardiac Surgery, King Abdulaziz Medical City, Riyadh, Saudi Arabia
b Division of Cardiac Anesthesia, King Abdulaziz Medical City, Riyadh, Saudi Arabia
c Division of Pediatric Cardiology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
Accepted for publication February 9, 2009.
* Address correspondence to Dr Al-Khaldi, Division of Cardiac Surgery, King Abdulaziz Medical City, PO Box 22490, Riyadh, 11426, Saudi Arabia (Email: khaldiab{at}ngha.med.sa).
The outcomes of surgical repair for diffuse pulmonary artery stenoses due to arterial tortuosity syndrome are unknown. We report a 1-year-old girl with tortuosity syndrome who presented with suprasystemic right ventricular pressure due to distortion and stenoses of the pulmonary artery branches. We describe the operative findings of this unique pathology and the surgical techniques used to completely reconstruct the pulmonary arterial tree, with normalization of the right ventricular pressure.
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