Two Cases of Late Shone Syndrome With Pulmonary Hypertension: Heart-Lung Transplant or Valve Surgery?
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Two cases of Shone syndrome with severe mitral and aortic valve problems and pulmonary hypertension were referred for heart-lung transplantation. Severely elevated pulmonary vascular resistance (PVR) was confirmed as was severe periprosthetic mitral and aortic regurgitation. Based on the severity of the valve lesions in both patients, surgery was decided upon and undertaken. Both experienced early pulmonary hypertensive crises, one more than the other, that gradually subsided, followed by excellent recovery and reversal of pulmonary hypertension and PVR. These cases illustrate Braunwald's concept that pulmonary hypertension secondary to left-sided valve disease is reversible.
Subjectadult congenital heart disease
mitral valve replacement
pulmonary vascular resistance/hypertension
Heart Valve Prosthesis Implantation
Mitral Valve Stenosis
Published Version (Please cite this version)10.1177/2150135115577671
Publication InfoRobich, Michael P; Stewart, Robert D; Zahka, Kenneth G; Krasuski, Richard A; Hanna, Mazen; Blackstone, Eugene H; & Pettersson, Gosta B (2016). Two Cases of Late Shone Syndrome With Pulmonary Hypertension: Heart-Lung Transplant or Valve Surgery?. World J Pediatr Congenit Heart Surg, 7(1). pp. 100-103. 10.1177/2150135115577671. Retrieved from https://hdl.handle.net/10161/11575.
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Richard Andrew Krasuski
Professor of Medicine
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