Two Cases of Late Shone Syndrome With Pulmonary Hypertension: Heart-Lung Transplant or Valve Surgery?
Abstract
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.
Type
Journal articleSubject
adult congenital heart diseasemitral valve replacement
pulmonary vascular resistance/hypertension
reoperation
Abnormalities, Multiple
Adult
Decision Making
Echocardiography
Female
Heart Transplantation
Heart Valve Prosthesis Implantation
Humans
Hypertension, Pulmonary
Lung Transplantation
Male
Mitral Valve Stenosis
Syndrome
Vascular Resistance
Young Adult
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https://hdl.handle.net/10161/11575Published Version (Please cite this version)
10.1177/2150135115577671Publication Info
Robich, 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.This is constructed from limited available data and may be imprecise. To cite this
article, please review & use the official citation provided by the journal.
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Richard Andrew Krasuski
Professor of Medicine

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