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


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.





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Publication Info

Robich, Michael P, Robert D Stewart, Kenneth G Zahka, Richard A Krasuski, Mazen Hanna, Eugene H Blackstone and Gosta B Pettersson (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

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Richard Andrew Krasuski

Professor of Medicine

Dr. Richard Krasuski is Director of the Adult Congenital Heart Center at Duke University Medical Center, the Director of Hemodynamic Research, and the Medical Director of the CTEPH Program. He is considered a thought leader in the fields of pulmonary hypertension and congenital heart disease. His research focus is in epidemiologic and clinical studies involving patients with pulmonary hypertension and patients with congenital heart disease. He is involved in multiple multicenter studies through the Alliance for Adult Research in Congenital Cardiology (AARCC). He has also helped to develop multiple research databases in these patient populations. He is Co-PI in the upcoming EPIPHANY Study examining the impact of medical and transcatheter interventions on RV-PA coupling in patients with chronic thromboembolic pulmonary hypertension. Over his career he has mentored over 80 students, residents and fellows and has published over 300 peer reviewed publications, book chapters and meeting abstracts. He is also the Chief Editor of Advances in Pulmonary Hypertension and on the editorial boards of several leading medical journals.

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