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Late Onset of Pulmonary Hypertension Following Successful Mustard Surgery for Transposition of the Great Arteries. Eric S. Ebenroth, MD, Roger A. Hurwitz, MD, FAAP, Department of Pediatrics, James Whitcomb Riley Hospital for Children, Indiana University, Indianapolis, IN Background: The development of pulmonary hypertension in adolescent and adult patients following successful Mustard surgery for d-transposition of the great arteries (dTGA) is a concern not completely answered. The present study was designed to evaluate long term Mustard patients for the development of pulmonary hypertension. Methods: The records of all patients followed at Riley Hospital for Children with dTGA and operated 7-25 years before were reviewed: 93 patients repaired between 1972 and 1989 were identified and 60 patients satisfied all inclusion criteria. Results: Serial echocardiography and/or cardiac catheterization over a 25 year period demonstrated pulmonary pressures to remain normal in 52 of 60 (87%), but identified 8 patients (13%) with significant pulmonary hypertension (pulmonary pressure > 50% systemic pressure): 4 of these had anatomic explanations for their pulmonary hypertension. The remaining 4 patients (7% of the study group) had pulmonary hypertension in the absence of anatomic abnormalities: 3 of them had mildly elevated pulmonary pressures (33 - 50% of systemic pressures) at initial post-operative catheterization following surgery. Conclusions: This study demonstrates that patients with dTGA who have undergone Mustard surgery are at increased risk of developing pulmonary hypertension even in the face of normal or slightly elevated pulmonary arterial pressures early after surgery. The complete article was published in The American Journal of Cardiology 85; 127-30, 2000 Jan. Published in abstract form in Pediatrics. 102(3 Pt 2): 682, 1998 Sep. Presented at the 7th World Congress on Heart Failure, July 2000, Vancouver. Presented at the Section of Cardiology at the Annual American Academy of Pediatrics Meeting, October 1998, San Francisco. |