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Case Reports in Cardiology
Volume 2013, Article ID 418565, 5 pages
Case Report

Heart Transplantation in a 14-Year-Old Boy in the Presence of Severe Out-of-Proportion Pulmonary Hypertension due to Restrictive Left Heart Disease: A Case Report

1Department of Pediatrics, Division of Cardiology, Pulmology, Allergology and Cystic Fibrosis, Innsbruck Medical University, 6020 Innsbruck, Austria
2Department of Pediatrics, Pediatric Intensive Care Unit, Innsbruck Medical University, 6020 Innsbruck, Austria
3Department of Anaesthesiology and Critical Care Medicine, Innsbruck Medical University, 6020 Innsbruck, Austria
4Department of Pediatric Cardiology, Medical University of Vienna, 1090 Vienna, Austria
5Department of Pediatrics, Salzburg State Hospital, 5020 Salzburg, Austria
6Department of Pathology, Innsbruck Medical University, 6020 Innsbruck, Austria

Received 28 February 2013; Accepted 25 March 2013

Academic Editors: C. D. Barrett and A. Movahed

Copyright © 2013 Martin Schwienbacher et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


A 14-year-old boy after balloon valvuloplasty of severe aortic valve stenosis in the neonatal period was referred for heart-lung transplantation because of high grade pulmonary hypertension and left heart dysfunction due to endocardial fibroelastosis with severe mitral insufficiency. After heart catheterization, hemodynamic parameters were invasively monitored: a course of levosimendan and initiation of diuretics led to a decrease of pulmonary capillary wedge pressure (from maximum 35 to 24 mmHg). Instead of an expected decrease, mean pulmonary artery pressures (mPAP) increased up to 80 mmHg with increasing transpulmonary pressure gradient (TPG) up to 55 mmHg. Oral bosentan and intravenous epoprostenol then led to a ~50% decrease of mPAP (TPG between 16 and 22 mmHg). The boy was listed solely for heart transplantation which was successfully accomplished 1 month later.