Two Cases of Late Shone Syndrome With Pulmonary Hypertension: Heart-Lung Transplant or Valve Surgery?
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2016-01
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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.
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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 https://hdl.handle.net/10161/11575.
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Richard Andrew Krasuski
Dr. Richard Krasuski is an Adjunct Professor of Medicine and Pediatrics at Duke University Medical Center. He was formerly the Director of the Adult Congenital Heart Center, Director of Hemodynamic Research, and the Medical Director of the CTEPH Program at Duke. 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 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 100 students, residents and fellows and has published over 350 peer reviewed articles, book chapters and meeting abstracts. He is also the Chief Editor of Advances in Pulmonary Hypertension and on the editorial boards of >20 leading medical journals. He has been an invited speaker at over 100 medical centers worldwide.
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