title: 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.
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Michael P
authors.initials: MP
authors.lastname: Robich
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Robert D
authors.initials: RD
authors.lastname: Stewart
authors.affiliation: Pediatric Cardiology, Pediatric Institute, Cleveland Clinic Children's, Cleveland, OH, USA.
authors.forename: Kenneth G
authors.initials: KG
authors.lastname: Zahka
authors.affiliation: Department of Cardiovascular Medicine, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Richard A
authors.initials: RA
authors.lastname: Krasuski
authors.affiliation: Department of Cardiovascular Medicine, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Mazen
authors.initials: M
authors.lastname: Hanna
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Eugene H
authors.initials: EH
authors.lastname: Blackstone
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA PETTERG@ccf.org.
authors.forename: Gosta B
authors.initials: GB
authors.lastname: Pettersson
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D003657:Decision Making; D004452:Echocardiography; D005260:Female; D016027:Heart Transplantation; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000151:congenital / Q000175:diagnosis / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D008946:Mitral Valve Stenosis / Q000151:congenital / Q000175:diagnosis / Q000601:surgery*; D013577:Syndrome; D014655:Vascular Resistance; D055815:Young Adult
keywords: adult congenital heart disease; mitral valve replacement; pulmonary vascular resistance/hypertension; reoperation