Two Cases of Late Shone Syndrome With Pulmonary Hypertension: Heart-Lung Transplant or Valve Surgery?

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Robich, MP; Stewart, RD; Zahka, KG; Krasuski, RA; Hanna, M; Blackstone, EH; Pettersson, GB

Published Date

  • January 2016

Published In

Volume / Issue

  • 7 / 1

Start / End Page

  • 100 - 103

PubMed ID

  • 26715002

Electronic International Standard Serial Number (EISSN)

  • 2150-136X

Digital Object Identifier (DOI)

  • 10.1177/2150135115577671


  • eng

Conference Location

  • United States